Tam Metin - Marmara Medical Journal
Transkript
Tam Metin - Marmara Medical Journal
Marmara Medical Journal Marmara Üniversitesi Tıp Fakültesi Dergisi Editör Prof. Dr. Mithat Erenus Koordinatörler Seza Arbay, MA Dr. Vera Bulgurlu Editörler Kurulu Prof. Dr. Mehmet Ağırbaşlı Prof. Dr. Serpil Bilsel Prof. Dr. Safiye Çavdar Prof. Dr. Tolga Dağlı Prof. Dr. Haner Direskeneli Prof. Dr. Kaya Emerk Prof. Dr. Mithat Erenus Prof. Dr. Zeynep Eti Prof. Dr. RainerVV. Guillery Prof. Dr. Oya Gürbüz Prof. Dr. Hande Harmancı Prof. Dr. Hızır Kurtel Prof. Dr. Ayşe Özer Prof. Dr. Tülin Tanrıdağ Prof. Dr. Tufan Tarcan Prof. Dr. Cihangir Tetik Prof. Dr. Ferruh Şimşek Prof. Dr. Dr. Ayşegül Yağcı Prof. Dr. Berrak Yeğen Doç. Dr. İpek Akman Doç. Dr. Gül Başaran Doç. Dr. Hasan Batırel Doç. Dr. Nural Bekiroğlu Doç. Dr. Şule Çetinel Doç. Dr. Mustafa Çetiner Doç. Dr. Arzu Denizbaşı Doç. Dr. Gazanfer Ekinci Doç. Dr. Dilek Gogas Doç. Dr. Sibel Kalaça Doç. Dr. Atila Karaalp Doç. Dr. Bülent Karadağ Doç. Dr. Handan Kaya Doç. Dr. Gürsu Kıyan Doç. Dr. Şule Yavuz Asist. Dr. Asım Cingi Asist. Dr. Arzu Uzuner Marmara Medical Journal Marmara Üniversitesi T p Fakültesi Dergisi DERGİ HAKKINDA Marmara Medical Journal, Marmara Üniversitesi Tıp Fakültesi tarafından yayımlanan multidisipliner ulusal ve uluslararası tüm tıbbi kurum ve personele ulaşmayı hedefleyen bilimsel bir dergidir. Marmara Üniversitesi Tıp Fakültesi Dergisi, tıbbın her alanını içeren özgün klinik ve deneysel çalışmaları, ilginç olgu bildirimlerini, derlemeleri, davet edilmiş derlemeleri, Editöre mektupları, toplantı, haber ve duyuruları, klinik haberleri ve ilginç araştırmaların özetlerini , ayırıcı tanı, tanınız nedir başlıklı olgu sunumlarını, , ilginç, fotoğraflı soru-cevap yazıları (photo-quiz) ,toplantı, haber ve duyuruları, klinik haberleri ve tıp gündemini belirleyen güncel konuları yayınlar. Periyodu: Marmara Medical Journal -Marmara Üniversitesi Tıp Fakültesi Dergisi yılda 3 sayı olarak OCAK,MAYIS VE EKİM AYLARINDA yayınlanmaktadır. Yayına başlama tarihi:1988 2004 Yılından itibaren yanlızca elektronik olarak yayınlanmaktadır Yayın Dili: Türkçe, İngilizce eISSN: 1309-9469 Temel Hedef Kitlesi: Tıp alanında tüm branşlardaki hekimler, uzman ve öğretim üyeleri, tıp öğrencileri İndekslendiği dizinler: EMBASE - Excerpta Medica ,TUBITAK - Türkiye Bilimsel ve Teknik Araştırma Kurumu , Türk Sağlık Bilimleri İndeksi, Turk Medline,Türkiye Makaleler Bibliyografyası ,DOAJ (Directory of Open Access Journals) Makalelerin ortalama değerlendirme süresi: 8 haftadır Makale takibi -iletişim Seza Arbay Marmara Medical Journal (Marmara Üniversitesi Tıp Fakültesi Dergisi) Marmara Üniversitesi Tıp Fakültesi Dekanlığı, Tıbbiye cad No:.49 Haydarpaşa 34668, İSTANBUL Tel: +90 0 216 4144734 Faks: +90 O 216 4144731 e-posta: mmj@marmara.edu.tr Yayıncı Plexus BilişimTeknolojileri A.Ş. Tahran Caddesi. No:6/8, Kavaklıdere, Ankara Tel: +90 0 312 4272608 Faks: +90 0312 4272602 Yayın Hakları: Marmara Medical Journal ‘in basılı ve web ortamında yayınlanan yazı, resim, şekil, tablo ve uygulamalar yazılı izin alınmadan kısmen veya tamamen herhangi bir vasıtayla basılamaz. Bilimsel amaçlarla kaynak göstermek kaydıyla özetleme ve alıntı yapılabilir. www.marmaramedicaljournal.org Marmara Medical Journal Marmara Üniversitesi Tıp Fakültesi Dergisi YAZARLARA BİLGİ Marmara Medical Journal – Marmara Üniversitesi Tıp Fakültesi Dergisine ilginize teşekkür ederiz. Derginin elektronik ortamdaki yayınına erişim www.marmaramedicaljournal.org adresinden serbesttir. Marmara Medical Journal tıbbın klinik ve deneysel alanlarında özgün araştırmalar, olgu sunumları, derlemeler, davet edilmiş derlemeler, mektuplar, ilginç, fotoğraflı soru-cevap yazıları (photo-quiz), editöre mektup , toplantı, haber ve duyuruları, klinik haberleri ve ilginç araştırmaların özetlerini yayınlamaktadır. Yılda 3 sayı olarak Ocak, Mayıs ve Ekim aylarında yayınlanan Marmara Medical Journal hakemli ve multidisipliner bir dergidir.Gönderilen yazılar Türkçe veya İngilizce olabilir. Değerlendirme süreci Dergiye gönderilen yazılar, ilk olarak dergi standartları açısından incelenir. Derginin istediği forma uymayan yazılar, daha ileri bir incelemeye gerek görülmeksizin yazarlarına iade edilir. Zaman ve emek kaybına yol açılmaması için, yazarlar dergi kurallarını dikkatli incelemeleri önerilir. Dergi kurallarına uygunluğuna karar verilen yazılar Editörler Kurulu tarafından incelenir ve en az biri başka kurumdan olmak üzere iki ya da daha fazla hakeme gönderilir. Editör, Kurulu yazıyı reddetme ya da yazara(lara) ek değişiklikler için gönderme veya yazarları bilgilendirerek kısaltma yapmak hakkına sahiptir. Yazarlardan istenen değişiklik ve düzeltmeler yapılana kadar, yazılar yayın programına alınmamaktadır. Marmara Medical Journal gönderilen yazıları sadece online olarak http://marmaramedicaljournal.org/submit. adresinden kabul etmektedir. Yazıların bilimsel sorumluluğu yazarlara aittir. Marmara Medical Journal yazıların bilimsel sorumluluğunu kabul etmez. Makale yayına kabul edildiği takdirde Yayın Hakkı Devir Formu imzalanıp dergiye iletilmelidir. Gönderilen yazıların dergide yayınlanabilmesi için daha önce başka bir bilimsel yayın organında yayınlanmamış olması gerekir. Daha önce sözlü ya da poster olarak sunulmuş çalışmalar, yazının başlık sayfasında tarihi ve yeri ile birlikte belirtilmelidir. Yayınlanması için başvuruda bulunulan makalelerin, adı geçen tüm yazarlar tarafından onaylanmış olması ve çalışmanın başka bir yerde yayınlanmamış olması da yayınlanmak üzere ya değerlendirmede olmaması gerekmektedir. Yazının son halinin bütün yazarlar tarafından onaylandığı ve çalışmanın yürtüldüğü kurum sorumluları tarafından onaylandığı belirtilmelidir.Yazarlar tarafından imzalanarak onaylanan üst yazıda ayrıca tüm yazarların makale ile ilgili bilimsel katkı ve sorumlulukları yer almalı, çalışma ile ilgili herhangi bir mali ya da diğer çıkar çatışması var ise bildirilmelidir.( * ) ( * ) Orijinal araştırma makalesi veya vaka sunumu ile başvuran yazarlar için üst yazı örneği: "Marmara Medical Journal'de yayımlanmak üzere sunduğum (sunduğumuz) "…-" başlıklı makale, çalışmanın yapıldığı laboratuvar/kurum yetkilileri tarafından onaylanmıştır. Bu çalışma daha önce başka bir dergide yayımlanmamıştır (400 sözcük – ya da daha az – özet şekli hariç) veya yayınlanmak üzere başka bir dergide değerlendirmede bulunmamaktadır. Yazıların hazırlanması Derginin yayın dili İngilizce veya Türkçe’dir. Türkçe yazılarda Türk Dil Kurumu Türkçe Sözlüğü (http://tdk.org.tr) esas alınmalıdır. Anatomik terimlerin ve diğer tıp terimlerinin adları Latince olmalıdır. Gönderilen yazılar, yazım kuralları açısından Uluslararası Tıp Editörleri Komitesi tarafından hazırlanan “Biomedikal Dergilere Gönderilen Makalelerde Bulunması Gereken Standartlar “ a ( Uniform Requirements For Manuscripts Submittted to Biomedical Journals ) uygun olarak hazırlanmalıdır. (http://www. ulakbim.gov.tr /cabim/vt) Makale içinde kullanılan kısaltmalar Uluslararası kabul edilen şeklide olmalıdır (http..//www.journals.tubitak.gov.tr/kitap/ma www.marmaramedicaljournal.org knasyaz/) kaynağına başvurulabilir. Birimler, Ağırlıklar ve Ölçüler 11. Genel Konferansı'nda kabul edildiği şekilde Uluslararası Sistem (SI) ile uyumlu olmalıdır. Makaleler Word, WordPerfect, EPS, LaTeX, text, Postscript veya RTF formatında hazırlanmalı, şekil ve fotoğraflar ayrı dosyalar halinde TIFF, GIF, JPG, BMP, Postscript, veya EPS formatında kabul edilmektedir. Yazı kategorileri Yazının gönderildiği metin dosyasının içinde sırasıyla, Türkçe başlık, özet, anahtar sözcükler, İngilizce başlık, özet, İngilizce anahtar sözcükler, makalenin metini, kaynaklar, her sayfaya bir tablo olmak üzere tablolar ve son sayfada şekillerin (varsa) alt yazıları şeklinde olmalıdır. Metin dosyanızın içinde, yazar isimleri ve kurumlara ait bilgi, makalede kullanılan şekil ve resimler olmamalıdır. Özgün Araştırma Makaleleri Türkçe ve İngilizce özetler yazı başlığı ile birlikte verilmelidir. (i)özetler: Amaç (Objectives), Gereç ve Yöntem (Materials and Methods) ya da Hastalar ve Yöntemler (Patients and Methods), Bulgular (Results) ve Sonuç (Conclusion) bölümlerine ayrılmalı ve 200 sözcüğü geçmemelidir. (ii) Anahtar Sözcükler Index Medicus Medical Subject Headings (MeSH) ‘e uygun seçilmelidir. Yazının diğer bölümleri, (iii) Giriş, (iv) Gereç ve Yöntem / Hastalar ve Yöntemler, (v) Bulgular, (vi) Tartışma ve (vii) Kaynaklar'dır. Başlık sayfası dışında yazının hiçbir bölümünün ayrı sayfalarda başlatılması zorunluluğu yoktur. Maddi kaynak , çalışmayı destekleyen burslar, kuruluşlar, fonlar, metnin sonunda teşekkürler kısmında belirtilmelidir. Olgu sunumları İngilizce ve Türkçe özetleri kısa ve tek paragraflık olmalıdır. Olgu sunumu özetleri ağırlıklı olarak mutlaka olgu hakkında bilgileri içermektedir. Anahtar sözcüklerinden sonra giriş, olgu(lar) tartışma ve kaynaklar şeklinde düzenlenmelidir. Derleme yazıları İngilizce ve Türkçe başlık, İngilizce ve Türkçe özet ve İngilizce ve Türkçe anahtar kelimeler yer almalıdır. Kaynak sayısı 50 ile sınırlanması önerilmektedir. Kaynaklar Kaynaklar yazıda kullanılış sırasına göre numaralanmalıdır. Kaynaklarda verilen makale yazarlarının sayısı 6 dan fazla ise ilk 3 yazar belirtilmeli ve İngilizce kaynaklarda ilk 3 yazar isminden sonra “ et al.”, Türkçe kaynaklarda ise ilk 3 yazar isminden sonra “ ve ark. “ ibaresi kullanılmalıdır. Noktalamalara birden çok yazarlı bir çalışmayı tek yazar adıyla kısaltmamaya ve kaynak sayfalarının başlangıç ve bitimlerinin belirtilmesine dikkat edilmelidir. Kaynaklarda verilen dergi isimleri Index Medicus'a (http://www.ncbi.nim.nih.gov/sites/entrez/qu ery.fcgi?db=nlmcatalog) veya Ulakbim/Türk Tıp Dizini’ne uygun olarak kısaltılmalıdır. Makale: Tuna H, Avcı Ş, Tükenmez Ö, Kokino S. İnmeli olguların sublukse omuzlarında kas-sinir elektrik uyarımının etkinliği. Trakya Univ Tıp Fak Derg 2005;22:70-5. Kitap: Norman IJ, Redfern SJ, (editors). Mental health care for elderly people. New York: Churchill Livingstone, 1996. Kitaptan Bölüm: Phillips SJ, Whisnant JP Hypertension and stroke. In: Laragh JH, Brenner BM, editors. Hypertension: Pathophysiology, Diagnosis, and Management. 2nd ed. New York: Raven Pres, 1995:465-78. Kaynak web sitesi ise: Kaynak makalerdeki gibi istenilen bilgiler verildikten sonra erişim olarak web sitesi adresi ve erişim tarihi bildirilmelidir. Kaynak internet ortamında basılan bir dergi ise: Kaynak makaledeki gibi istenilen bilgiler verildikten sonra erişim olarak URL adresi ve erişim tarihi verilmelidir. Kongre Bildirileri: Bengtsson S, Solheim BG. Enforcement of data protection, privacy and security in medical informatics. In: Lun KC, Degoulet P, Piemme TE, Rienhoff O, editors. MEDINFO 92. Proceedings of the 7th World Congress on Medical Informatics; 1992 Sep 6-10; Geneva, Switzerland. Amsterdam: North-Holland; 1992:1561-5. Tablo, şekil, grafik ve fotoğraf Tablo, şekil grafik ve fotoğraflar yazının içine yerleştirilmiş halde gönderilmemeli. Tablolar, her sayfaya bir tablo olmak üzere yazının gönderildiği dosya içinde olmalı ancak yazıya ait şekil, grafik ve fotografların her biri ayrı bir imaj dosyası (jpeg yada gif) olarak gönderilmelidir. www.marmaramedicaljournal.org Tablo başlıkları ve şekil altyazıları eksik bırakılmamalıdır. Şekillere ait açıklamalar yazının gönderildiği dosyanın en sonuna yazılmalıdır. Tablo, şekil ve grafiklerin numaralanarak yazı içinde yerleri belirtilmelidir. Tablolar yazı içindeki bilginin tekrarı olmamalıdır. Makale yazarlarının, makalede eğer daha önce yayınlanmış alıntı yazı, tablo, şekil, grafik, resim vb var ise yayın hakkı sahibi ve yazarlardan yazılı izin almaları ve makale üst yazısına ekleyerek dergiye ulaştırmaları gerekmektedir. Tablolar Metin içinde atıfta bulunulan sıraya göre romen rakkamı ile numaralanmalıdır. Her tablo ayrı bir sayfaya ve tablonun üst kısmına kısa ancak anlaşılır bir başlık verilerek hazırlanmalıdır. Başlık ve dipnot açıklayıcı olmalıdır. Sütun başlıkları kısa ve ölçüm değerleri parantez içinde verilmelidir. Bütün kısaltmalar ve semboller dipnotta açıklanmalıdır. Dipnotlarda şu semboller: (†‡¶§) ve P değerleri için ise *, **, *** kullanılmalıdır. SD veya SEM gibi istatistiksel değerler tablo veya şekildin altında not olarak belirtilmelidir. Grafik, fotoğraf ve çizimler ŞEKİL olarak adlandırılmalı, makalede geçtiği sıraya gore numaralanmalı ve açıklamaları şekil altına yazılmalıdır Şekil alt yazıları, ayrıca metinin son sayfasına da eklenmelidir. Büyütmeler, şekilde uzunluk birimi (bar çubuğu içinde) ile belirtilmelidir. Mikroskopik resimlerde büyütme oranı ve boyama tekniği açıklanmalıdır. Etik Marmara Medical Journal’a yayınlanması amacı ile gönderilen yazılar Helsinki Bildirgesi, İyi Klinik Uygulamalar Kılavuzu,İyi Laboratuar Uygulamaları Kılavuzu esaslarına uymalıdır. Gerek insanlar gerekse hayvanlar açısından etik koşullara uygun olmayan yazılar yayınlanmak üzere kabul edilemez. Marmara Medical Journal, insanlar üzerinde yapılan araştırmaların önceden Araştırma Etik Kurulu tarafından onayının alınması şartını arar. Yazarlardan, yazının detaylarını ve tarihini bildirecek şekilde imzalı bir beyan ile başvurmaları istenir. Çalışmalar deney hayvanı kullanımını içeriyorsa, hayvan bakımı ve kullanımında yapılan işlemler yazı içinde kısaca tanımlanmalıdır. Deney hayvanlarında özel derişimlerde ilaç kullanıldıysa, yazar bu derişimin kullanılma mantığını belirtmelidir. İnsanlar üzerinde yapılan deneysel çalışmaların sonuçlarını bildiren yazılarda, Kurumsal Etik Kurul onayı alındığını ve bu çalışmanın yapıldığı gönüllü ya da hastalara uygulanacak prosedürlerin özelliği tümüyle kendilerine anlatıldıktan sonra, onaylarının alındığını gösterir cümleler yer almalıdır. Yazarlar, bu tür bir çalışma söz konusu olduğunda, uluslararası alanda kabul edilen kılavuzlara ve TC. Sağlık Bakanlığı tarafından getirilen ve 28 Aralık 2008 tarih ve 27089 sayılı Resmi Gazete'de yayınlanan "Klinik araştırmaları Hakkında Yönetmelik" ve daha sonra yayınlanan 11 Mart 2010 tarihli resmi gazete ve 25518 sayılı “Klinik Araştırmalar Hakkında Yönetmelikte Değişiklik Yapıldığına Dair Yönetmelik” hükümlerine uyulduğunu belirtmeli ve kurumdan aldıkları Etik Komitesi onayını göndermelidir. Hayvanlar üzerinde yapılan çalışmalar için de gereken izin alınmalı; yazıda deneklere ağrı, acı ve rahatsızlık verilmemesi için neler yapıldığı açık bir şekilde belirtilmelidir. Hasta kimliğini tanıtacak fotoğraf kullanıldığında, hastanın yazılı onayı gönderilmelidir. Yazı takip ve sorularınız için iletişim: Seza Arbay Marmara Universitesi Tıp Fakültesi Dekanlığı, Tıbbiye Caddesi, No: 49, Haydarpaşa 34668, İstanbul Tel:+90 0 216 4144734 Faks:+90 0 216 4144731 e-posta: mmj@marmara.edu.tr www.marmaramedicaljournal.org İÇİNDEKİLER Orjinal Araştırma FROM THE ANESTHESIOLOGIST’S PERSPECTIVE RETROSPECTIVE ANALYSIS OF PERIOPERATIVE COMPLICATIONS OF TRANSSPHENOIDAL PITUITARY SURGERY Arzu Gerçek, Deniz Konya, Zafer Toktaş, Türker Kılıç, M. Necmettin Pamir……………………….…104 THE ROLE OF CONTRASTED DYNAMIC MULTI-DETECTOR COMPUTERIZED TOMOGRAPHY IMAGING ON SURGICAL DECISION MAKING IN BREAST CANCER PATIENTS Taner Yiğit, Unsal Coskun, Mentes Oner, Cengizhan Yigitler, Gulec Bulent, Müjdat Balkan, Orhan Kozak, Tufan Turgut……………………………………..…………………………………………...………...109 COMPARING OF THE IMMUNOHISTOCHEMICAL AND MORPHOLOGICAL FEATURES OF THE TESTICULAR SEMİNOMA AND INTRACRANIAL GERMNOMA Ahmet Midi, Süheyla Bozkurt, Aydın Sav, M Memet Özek, Necmettin Pamir………………………......116 EVALUATION OF INITIATING, CONTINUING AND WEANING TIME OF BREASTFEEDING Ruhusen Kutlu, Kamile Marakoğlu......................................................................................................121 IDIOPATHIC SUDDEN SENSORINEURAL HEARING LOSS Ufuk Derinsu, Şengül Terlemez, Ferda Akdaş……………………………………………………………....127 Olgu Sunumu RADIONUCLIDE IMAGING IN DIFFERENTIAL DIAGNOSIS OF TORSION AND INFECTIONS OF TESTIS AND EPIDIDYMIS REVISITED Yasemin Şanlı, Işık Adalet, Handan Tokmak, Öner Şanlı, Orhan Ziylan, Sema Cantez…………….…132 SPINOCEREBELLAR ATAXIA TYPE 2 IN A TURKISH FAMILY Kadriye Ağan, Deniz Kutlu, Nazlı Başak, Önder Us, Dilek İnce-Günal…….…………………...……...135 AN UNUSUAL CASE OF MULTIFOCAL MOTOR NEUROPATHY WITH CRANIAL NERVE INVOLVEMENT AND HYPERREFLEXIA Hande Türker, Oytun Bayrak, Levent Güngör, Murat Sarıca, Musa Onar………………..……....……139 NON-FUNCTIONING KIDNEY RESULTED FROM PRIMARY HYDATID CYST OF THE PSOAS MUSCLE Engin Kandıralı, Atilla Semerciöz, Ahmet Metin, Muzaffer Eroglu, Bülent Uysal……………………..145 GALL BLADDER TORSION: A CASE REPORT Sabahattin Aslan, Nemci Yücekule, Bahadır Çetin, Melih Akıncı, Ahmet Seki, Aybala Ağaç, Recep Çetin, Abdullah Çetin…………………………………………………………………………………………...147 ORIGINAL RESEARCH FROM THE ANESTHESIOLOGIST’S PERSPECTIVE RETROSPECTIVE ANALYSIS OF PERIOPERATIVE COMPLICATIONS OF TRANSSPHENOIDAL PITUITARY SURGERY Arzu Gerçek1, Deniz Konya2, Zafer Toktaş2, Türker Kılıç2, M. Necmettin Pamir2 1 Marmara Üniversitesi Nörolojik Bilimler Enstitüsü, Anesteziyoloji ve Reanimasyon, İstanbul, Türkiye 2 Marmara Üniversitesi Nörolojik Bilimler Enstitüsü, Beyin Cerrahisi, İstanbul, Türkiye ABSTRACT Objective: Pituitary surgery presents unique challenges for the anesthesiologist due to the distinct medical co-morbidities associated with various adenomas. The aim of this study was to investigate the perioperative complications throughout the transsphenoidal pituitary surgery from the anesthesiologist’s perspective. Methods: Retrospectively, 82 ASA physical status I-II patients, who underwent transsphenoidal surgery between 1st Jan 2002-1st Jan 2006, were included in the study. The following general information was recorded for each patient: demographic data, airway management, cardiovascular and electrocardiographic abnormalities, duration of procedures, pituitary pathology, and any complications during the perioperative period. Results: After induction, four patients developed severe bradycardia and ventricular premature beats with bizarre QRS complex with hypotension, non-responsive to atropine and ephedrine. Three patients experienced intubation problems.In 12 patients, following submucosal injection, a hypertensive response was observed.Only two patients (2.4%) had experienced temporary diabetes mellitus after surgery. Overall, 21 patients (25.6%) experienced complications during the perioperative period of transsphenoidal pituitary surgery. Conclusion: Anesthesiologists must be wary of the possibility of difficult intubation, hypertensive episode at the time of intranasal submucosal injection of vasoconstrictor-supplemented local anesthetic, and hemodynamic and electrocardiographic abnormalities related to the underlying overlooked cardiac pathologies at any time during surgery. Keywords:Anesthesia, Cardiomyopathy, Complications, Difficult intubation, Transsphenoidal pituitary surgery ANESTEZİST GÖZÜYLE TRANSSFENOİDAL HİPOFİZ CERRAHİSİNDE PERİOPERATİF DÖNEMDE GÖRÜLEN KOMPLİKASYONLARININ GERİYE DÖNÜK DEĞERLENDİRİLMESİ ÖZET Amaç: Pitüiter cerrahi adenomların tiplerine göre, farklılık gösteren morbiditeler nedeniyle anestezi doktoru için yoğun uğraş gerektiren girişimlerdir. Bu çalışmanın amacı, transsfenoidal cerrahi sırasında ortaya çıkan komplikasyonları anestezi doktorunun bakış açısıyla değerlendirmektir. Yöntem: 1 Ocak 2002-1 Ocak 2006 tarihleri arasında transsfenoidal hipofiz cerrahisi geçiren ve ASA skoru I-II olan 82 hasta retrospektif olarak çalışmaya alındı. Her hasta için; demografik özellikler, havayolu sağlanması, cerrahi süre, kardiyovasküler ve elektrokardiyografik anormallikler, patolojik tanı ve perioperatif dönemde ortaya çıkan komplikasyonlar kaydedildi. Bulgular: Anestezi indüksiyonunu takiben 4 hastada medikal tedaviye cevap vermeyen ağır bradikardi ve geniş QRS kompleksin eşlik ettiği ventriküler premature atımlar görüldü. Üç hasta entübasyon sırasında problem yaşadı. Oniki hastada ise submukozal enjeksiyon sonrasında hipertansif yanıt gözlendi. Erken postoperatif dönemde 2 hastada (%2.4) geçici diabetes insipid görüldü. Hastaların hiçbirinde rinore veya nazal kanama görülmedi. Bu bağlamda 21 hastada (%25.6) perioperative dönemde komplikasyon oldu. Sonuç: Anestezistler zor entübasyon, vazokonstriktör eklenmiş lokal anesteziğin submukozal enjeksiyonuna bağlı hipertansif yanıt ve anestezi indüksiyonu sonrasında subklinik kardiyak patolojilerin klinik bulgu verir hale gelme olasılığını göz önünde bulundurmalıdır. Anahtar Kelimeler: Anestezi, Kardiyomiyopati, Komplikasyon, Zor entübasyon, Transsfenoidal hipofiz cerrahisi İletişim Bilgileri: Arzu Gerçek e-mail: agercek@hotmail.com Marmara Üniversitesi Nörolojik Bilimler Enstitüsü, Anesteziyoloji ve Reanimasyon, İstanbul, Türkiye 104 Marmara Medical Journal 2006;19(3);104-108 Marmara Medical Journal 2006;19(3);104-108 Arzu Gerçek, et al. From the anesthesiologist’s perspective retrospective analysis of perioperative complications of transsphenoidal pituitary surgery any further cardiovascular investigation was not conducted. Anesthesia management Standard anesthesia was applied to all patients: Normoventilation was instituted following loss of consciousness with IV remifentanil 1 µg kg-1 and propofol 1-2 mg kg-1. Once an adequate mask airway was assured, IV vecuronium bromide 0.15 mg kg-1, dexamethasone 0.2 mg kg-1 and ondansetron 0.1 mg kg-1 were administered. In case of inadequate mask airway ventilation, vecuronium bromide was not injected, and patients were intubated with or without 1.5mg kg-1 succinylcholine. Anesthesia was maintained with 0.5% isoflurane 50% N2O in O2 and remifentanil 0.25 µg kg-1 min-1 infusion. Aspiration of postnasal blood drainage was prevented with a wet sponge placed into the oropharynx. During surgery, the lungs were normoventilated in order not to displace the pituitary gland with hyper- and hypo-ventilation. At the end of the surgery, a three-point headrest was taken off. Remifentanil infusion was stopped and the lungs were ventilated with 100% O2. The wet sponge was taken off from the oropharynx, and IV neostigmine 30µg kg-1 and atropine 10µg kg-1 were administered for the reversal of neuromuscular blockade. After spontaneous adequate respiration and the patient’s response to the verbal comment was achieved, the lungs were extubated. The patient was reminded to breathe orally, as told before the operation. After 24-hour follow up in the Intensive Care Unit the patient was discharged on postop day 7. Visual analogue scale was used for the evaluation of the patient’s postoperative pain. Pethidine hydrochloride (Aldolan, Liba, Turkey) was titrated intravenously in order to keep the patient’s score equal or below to 3. Surgical management After induction, the patient was positioned for surgery with the head fixed in a three-point headrest and secured with pins anchored into the cranium itself. To reduce venous engorgement, the operation table was adjusted to fifteen-degree Fowler position. After nasal cleaning, the patient received submucosal injections of 2 ml of 2% lidocaine and 1:100,000 epinephrine mixture to reduce bleeding and facilitate dissection. Then, transsphenoidal surgery was performed under INTRODUCTION The pituitary gland has a very important role in human life. It regulates the function of the thyroid gland, the adrenal glands, the ovaries and the testes. Besides controlling lactation, uterine contractions during labor and the linear growth, it regulates the osmolality and volume of intravascular fluid by providing absorption of water in the kidney1. Tumors of the pituitary gland frequently originate from the anterior lobe. They account for approximately 10-15% of diagnosed brain neoplasms2,3. Particular problems in such patients relate to primary hormonal hypersecretion and its complications, and mass effects of the macro adenomas. Although medical therapy is available for most hyperfunctioning states, it is not curative4,5. Transsphenoidal pituitary surgery has become common due to its safety and effectiveness in the management of various problems associated with the region of the sella turcica. Pituitary surgery presents unique challenges for the anesthesiologist due to the distinct medical co-morbidities associated with various adenomas. This retrospective study analyzed the correlation between the perioperative events and the pituitary pathology from the anesthesiologist’s perspective. PATIENTS AND METHODS Retrospectively, 82 ASA physical status I-II patients, who underwent transsphenoidal surgery between 1st Jan 2002-1st Jan 2006, were included in the study. Preoperative evaluation Upon receiving the history and physical examination of the patient, the Mallampati classification, thyromental distance and mouth opening were evaluated in order to predict the possibility of difficult intubation. None of these patients had thyromental distance under 5 cm, Mallampati Class III-IV and mouth opening lesser than 1 cm as an indicator of difficult intubation6. Preoperative blood levels of the growth hormone (GH), luteizing hormone (LH), follicule stimulating hormone (FSH), prolactin (PRL), adrenocorticotropin hormone (ACTH), and thyroid hormone levels (free T3 and T4) were measured. In the absence of known-cardiovascular pathology and electrocardiographic abnormality, 105 Marmara Medical Journal 2006;19(3);104-108 Arzu Gerçek, et al. From the anesthesiologist’s perspective retrospective analysis of perioperative complications of transsphenoidal pituitary surgery progress, therefore, surgery was cancelled, and the patients were awakened. Cardiology consultation revealed that these four patients had moderate cardiomyopathy. In one (4%) of the patients with PRL secreting adenoma, intubation was attempted four times.The laryngoscopic appearance of the vocal cord was Cormack-Lehane grade IV. During these attempts, the patient never experienced hypoxia. Because a fiberoptic bronchoscope was not available, surgery was cancelled. In two (13%) of the patients with GH secreting adenoma, endotracheal intubation was achieved at the third attempt due to Cormack-Lehane grade III vocal cord appearance. In 12 patients (14%), following submucosal injection, a hypertensive response was observed (Table I). The patients’ blood pressure increased by 30%. In one patient, systolic blood pressure rose as high as 260 mm Hg. Esmelol and remifentanil were used to control these increases in blood pressure. Nineteen patients (23%) had experienced intraoperative complications during transsphenoidal pituitary surgery. After cessation of anesthetic agents, all patients responded to verbal comment within 5 min. And their lungs were extubated. None of the patients experienced nausea and vomiting during emergence from the anesthesia and the postoperative 24-hour Intensive Care Unit follow up. Only two patients (2.4%) had experienced temporary diabetes mellitus after surgery. Overall, 25.6 % of the 82 patients had experienced perioperative complications during the transsphenoidal pituitary surgery. microscope and recorded on compact disc. Fatty tissue taken from the abdominal wall was placed onto the sella turcica. Nasal tampon was placed. Data analysis The following general information was recorded for each patient: demographic data, airway management (such as difficult intubation requiring more than three attempts and inability to intubate, etc.), duration of procedures, cardiovascular and electrocardiographic abnormalities, immunohistochemical diagnosis of pituitary pathology, and any complications during the perioperative period. RESULTS Eighty-two patients (39 female / 43 male) were included in the study. The mean age of the patients was 42.69±14.63 years (range: 18-75 years). The mean operation duration was 105±15 min. The patient distribution based on the pathologies and intraoperative complications, is provided in Table I. After induction, three (20%) of the patients with GH secreting adenoma, and one (6%) of the patients with non-secreting adenoma developed severe bradycardia (range: 25-35 beat per min) and ventricular premature beats with bizarre QRS complex. Apart from cardiac arrhythmia, they were also slightly hypotensive. Blood pressure values in these patients were 40% lower than the previous value. Patients’ cardiovascular statuses were non-responsive to the medical treatment with repeated doses of atropine and ephedrine. Twenty minutes after the induction of anesthesia, the patients’ cardiovascular statuses did not show any Table 1: The patients’ demographic features, pathologies and intraoperative complications Intraoperative Etiology Pt. (n) Cardiovascular Hypertension Airway management Arrhythmias+Hypotension Apoplexy 3 Benign cystic Adrenocorticotropic hormone secreting adenoma 2 2 Plurihormonal secreting adenoma 11 3 Prolactin secreting adenoma 27 4 Luteizing hormone secreting adenoma 4 1 Growth hormone secreting adenoma 15 1 3 Null cell (non-secreting adenoma) 17 2 1 Malignant melanoma Total 1 12 4 82 Postoperative Difficult* Enabled Hormonal Temporary Diabetes Insipidus 1 1 1 1 1 2 2 2 *: Difficult intubation was described as a case in that more than three attempts were needed for intubation of the trachea. 106 Marmara Medical Journal 2006;19(3);104-108 Arzu Gerçek, et al. From the anesthesiologist’s perspective retrospective analysis of perioperative complications of transsphenoidal pituitary surgery Intranasal submucosal injection of vasoconstrictor-supplemented local anesthetics may result in a hypertensive episode, as in these 12 patients12,13. All patients who underwent transsphenoidal pituitary surgery received a prophylactic dose of dexamethasone 0.2 mg kg-1 IV at the induction of anesthesia. While acute,a synthetic corticosteroid injection may cause a side effect of systemic hypertension, the magnitude of which is typically small compared to the responses in these 12 cases14,15. Chelliah et al16 reported a case of postoperative myocardial infarct related to the intraoperative submucasal epinephrine-induced hypertension. We believe that close proximity of the injection side of vasoconstrictor-supplemented local anesthetics to the hypothalamus-pituitary axis, results in the increase in the incidence tachycardia and hypertension even after a small dose. DISCUSSION The perioperative care of patients presenting for pituitary surgery requires careful preoperative assessment and meticulous intraoperative management using principles common to all intracranial procedures7. A safe airway supply is essential for the management of anesthesia. In literature, the incidence of difficult intubation shows great variability and ranges between 0.05 and 18%6,8,9. In this study, overall incidence of difficult intubation was 3.9%, while in patients with GH secreting adenoma, its incidence increased to 13%. Excessive GH results in the coarsening of features with bony proliferation that can concomitantly involve macroglossia, prognathia with malocclusion and hypertrophy of soft tissues (esp. the tongue), epiglottis and aryepiglottis folds10. All these changes make tracheal intubation difficult in these patients. Colao et al11 have shown that adolescents with prolactinoma have osteopenia or osteoporosis. In our study, the patient whose tracheal intubation could not be achieved had a problem at her neck due to the osteoporotic changes. Neck extension was limited to 300. The patient was 50 years-old. These osteoporotic changes might be related to PRL secreting adenoma, not age. However, the literature does not provide information on increased incidence of difficult intubation in patients with PRL secreting adenoma11. In patients undergoing transsphenoidal surgery, balanced anesthesia with remifentanil provides faster awakening time compared with high concentration of volatile anesthetics, without the risk of postoperative opioids respiratory depression17. In conclusion, transsphenoidal pituitary surgery entails careful preoperative evaluation.The anesthesiologist must be wary of the possibility of difficult intubation, hypertensive episode at the time of intranasal submucosal injection of vasoconstrictor-supplemented local anesthetic and haemodynamic and electrocardiographic abnormalities related to the underlying overlooked cardiac pathologies at any time during surgery. In acromegalic patients, cardiac muscles are also affected, and the incidence and severity of cardiac hypertrophy is related to the duration of the disease4. After induction, three patients had experienced severe bradycardia ventricular premature beats with bizarre QRS complex and hypotension, non-responsive to the medical treatment with repeated doses of atropine and ephedrine. In the preoperative visit, these patients did not have any problems related to the cardiovascular system, and both auscultation and electrocardiographic findings were within normal range. Therefore, preoperative echocardiography was not planned. Our results showed that intraoperative incidence of clinically presentedcardiomyopathy was 13% in acromegalic patients, and overall incidence in patients who had undergone pituitary surgery was 2.4%. In the light of these results, routine echocardiography should be performed, particularly on acromegalic patients, to rule out the cardiac disorders. REFERENCES 1. 2. 3. 4. 5. 6. 7. 107 Nemergut EJ, Dumont AS, Barry UT, Laws ER. Perioperative management of patients undergoing transsphenoidal pituitary surgery. Anesth Analg 2005;84:545-550. Faglia G, Ambrosi B. Hypotalamic and pituitary tumours: general principles. In: Grosman A, ed. Clinical Endocrinology. Oxford: Blackwell, 1992:113-122. Whitworth JA, Saines D, Scoggins BA. Blood pressure and metabolic effects of cortisol and deoxycorticosterone in man. Clin Exper Hyper 1984;6:795-809. Smith M, Hirsch NP. Pituitary disease and anaesthesia. Br J Anaesth 2000;85:3-14. Rose DK, Cohen MM. The airway: problems and predictions in 18,500 patients. Can J Anaesth 1994;41:372-383. Baykan N, Gercek A. Supratentorial tumour surgery and anesthesia. J Turk Anesthesiol Reanim Soc 2004; 32:167-179. Ezri T, Warters RD. The incidence of Class “Zero” airway and the Impact of Mallampati Score, Age, Sex Marmara Medical Journal 2006;19(3);104-108 Arzu Gerçek, et al. From the anesthesiologist’s perspective retrospective analysis of perioperative complications of transsphenoidal pituitary surgery and Body Mass Index on prediction of Laryngoscopy Grade. Anesth Analg 2001;93:1073-1075. 8. Gercek A, Lim S, Isler FB, Eti Z, Gogus FY. Prediction of difficult intubation with bedside scoring systems. MarmaraMed J 2003;16:36-39. 9. Rossi L, Thiene G, Caregano L, Giardano R, Laura S. Dysrhythmias and sudden death in acromegalic heart disease. Chest 1977;72:495-498. 10. Pasternak JJ, Atkinson JL, Kasperbauer JL, Lanier WL. Hemodynamic responses to epinephrine-containing local anesthetic injection and to emergence from general anesthesia in transsphenoidal hypophysectomy patients. J Neurosurg Anesthesiol 2004;16:189-195. 11. Colao A, Di Somma C, Loche S, et al. Prolactinomas in adolescents: persistent bone loss after 2 years of prolactin normalization. Clin Endocrinol 2000;52:319327. 12. Horrigan RW, Eger EI, Wilson C. Epinephrine-induced arrhythmias during enflurane anesthesia in man: a nonlinear dose-response relationship and dosedependent protection from lidocaine. Anesth Analg 1978;57:547-550. 13. Randell T. Prediction of difficult intubation. Acta Anaesthesiol Scand 1998;42:136-137. 14. Oyesiku NM. Assessment of pituitary function. In: Recgachary SS, Ellenbagen RG, eds. Principles of Neurosurgery. 2nd ed. Edinburg: Elsewier-Mosby, 2005:559-591. 15. Keegan MT, Atkinson JLD, Kasperbauer JL, Lanier WL. Exaggerated hemodynamic responses to nasal injection and awakening from anesthesia in a cushingoid patient having transsphenoidal hypophysectomy. J Neurosurg Anesthesiol 2000;12:225-229. 16. Chelliah YR, Manninen PH. Hazards of epinephrine in transsphenoidal pituitary surgery. J Neurosurg Anesthesiol 2002;14:43-46. 17. Gemma M, Tommasino C, Cozzi S, et al. Remifentanil provides hemodynamic stability and faster awakening time in transsphenoidal surgery. Anesth Analg 2002;94:163-168. 108 ORIGINAL RESEARCH THE ROLE OF CONTRASTED DYNAMIC MULTI-DETECTOR COMPUTERIZED TOMOGRAPHY IMAGING ON SURGICAL DECISION MAKING IN BREAST CANCER PATIENTS Taner Yiğit1, Ünsal Coşkun2, Öner Menteş1, Cengizhan Yiğitler1, Bülent Güleç1, Müjdat Balkan1, Orhan Kozak1, Turgut Tufan1 1 Gülhane Askeri Tıp Akademisi, Genel Cerrahi AD, Ankara, Türkiye 2Gülhane Askeri Tıp Akademisi, Rehabilitasyon Hastanesi, Ankara, Türkiye ABSTRACT Objective:. The success of surgical treatment of breast carcinoma depends mainly on prevention of local recurrence. Nowadays magnetic resonance imaging is being intensively used for the diagnosis of breast lesions. In this study we investigated the effect of the multi-detector computed tomography as an alternative to magnetic resonance in the evaluation of satellite breast cancer and the relation of the tumor margins with surrounding tissue, which plays an important role in local recurrence. Methods: In an 18 months' period, 30 patients with breast cancer, for whom breast-conserving surgery was planned, were given a multi-detector computed tomography in the preoperative period. Results: In two (10%) patients a very close correlation between the tumor and pectoral fascia was found; in three (15%) patients a satellite tumor focus was detected. It was also reported that one of these patients had both satellite lesions and a primary tumor-pectoral fascia connection. Four patients received a modified radical mastectomy in accordance with their informed consent. Conclusion: Multi-detector computed tomography is a good alternative for magnetic resonance imaging in some cases and provides useful data in the determination of satellite tumor focus and the relation of the primary tumor to the pectoral fascia. Keywords: Breast cancer, Multi-detector CT, Diagnosis, Treatment MEME KANSERLİ HASTALARDA CERRAHİ TEDAVİNİN PLANLANMASINDA KONTRASTLI DİNAMİK MULTİ-DEDEKTÖR TOMOGRAFİ GÖRÜNTÜLEMENİN ROLÜ ÖZET Giriş: Meme kanserinin cerrahi tedavisinin başarısı temel olarak hastalık tekrarının önlenmesi prensibine dayanmaktadır. Günümüzde manyetik rezonans görüntüleme meme kanserinin tanısında yoğun olarak kullanılmaktadır. Buna karşın kapalı alan korkusu olan hastalara bu tetkikin uygulanmasında güçlükler ve kolay ulaşılabilirliğin kısıtlı olması manyetik rezonans görüntülemenin uygulanabilirliğinin sınırlamaları olarak karşımıza çıkmaktadır. Bu çalışmada bölgesel hastalık tekrarı gelişiminde önemli etkenlerden olan tümörün çevre yapılarla olan ilişkisi ve uydu tümör odaklarının varlığının incelenmesinde kontrastlı dinamik çok detektörlü tomografinin manyetik rezonans görüntülemeye alternatif olarak kullanılabilirliğini araştırdık. Materyal Yöntem: 18 aylık dönem içinde meme kanseri tanısı konmuş ve meme koruyucu cerrahi planlanan toplam 30 hastaya kontrastlı dinamik çok detektörlü tomografi uygulandı. Sonuçlar: İki hastada (10%) tümörün pektoral kas kılıfına çok yakın sonlandığı, üç hastada (15%) uydu tümör odağı varlığı tespit edildi. Bu hastaların birinde ise uydu tümör odağına ilave olarak pektoral kas kılıfına yakın sonlanma tespit edildi. Hastaların dördüne de aydınlatılmış onamları alınarak meme koruyucu cerrahi yerine modifiye radikal mastektomi ameliyatı uygulandı. Tartışma: Kontrastlı dinamik çok detektörlü tomografi, seçilmiş vakalarda manyetik rezonans görüntülemeye iyi bir alternatiftir. Kontrastlı dinamik çok detektörlü tomografi ile ana tümörün pektoral kas kılıfına ile olan ilişkisi ve uydu tümör odakları hakkında yararlı veriler elde edilebilmektedir. Anahtar Kelimeler: Meme kanseri, Çok detektörlü CT, Tanı, Tedavi İletişim Bilgileri: Taner Yiğit e-mail: yigittaner@yahoo.com Gülhane Askeri Tıp Akademisi, Genel Cerrahi AD, Ankara, Türkiye 109 Marmara Medical Journal 2006;19(3);109-115 Marmara Medical Journal 2006;19(3);109-115 Taner Yiğit, et al. The role of contrasted dynamic multi-detector computerized tomography imaging on surgical decision making in breast cancer patients mammography and pathology), c) patient's choice d) surgeon’s experience. All patients were given a thorough physical examination at the time of first admission.The dimensions of the lesion, condition of the contra lateral breast and both axilla were noted along with a detailed patient history. All patients were given bilateral breast and axillary USG and mammography as a standard procedure for screening. Patients with a suspicious malignant breast lesion were reevaluated with focused USG and also given a fine needle aspiration biopsy (FNB) or open biopsy. Patients given and diagnosed with open biopsy were excluded from the study in order to prevent possible side effects of the procedure on the contrasted dynamic MDCT results. Patients having T1-T2 tumor and candidates for BCS were assigned to the study group. Patients’ preference of treatment as to which operation they wanted was determined after giving detailed information concerning complications, recurrence and disease-free-overall survival rates, and effect on their quality of life after surgery and radiotherapy following the BCS. According to these data, patients who selected mastectomy were excluded from the study. Finally, patients with T1 and T2 tumors, who chose BCS, were included in the study and all data of these patients including age, USG, mammography, MDCT and FNC results, tumor size, histology, localization and TNM classification were recorded. INTRODUCTION Breast cancer (BC) occurs in more than 8% of women during their lifetime and this ratio continues to increase 1,2. Thus, it is one of the major causes of death among women 40-55 years of age 3,4. Despite the advantages in both diagnostic tool and treatment strategies, the mortality rates of BC still remain significant. Currently, the overall survival rate is 73% at five years and 59% at 10 years 2. While mastectomy was formerly the mainstay of treatment, today breast-conserving surgery (BCS), which offers nearly similar survival rates, is extensively used. Even if disease-free and overall survival rates are nearly similar, local recurrence rates are higher after BCS than after mastectomy5-7. Many factors such as the patient’s age, tumor size, pregnancy, collagen vascular disease, resection margin, satellite tumor focus, tumor nature, axillary lymph node involvement, tumor grade, mononuclear cell infiltration, were reported as accounting for local recurrence in previous studies 8-12. Among the factors listed above, resection margin and satellite tumor focus are unique factors which can be controlled during the preoperative period. In this prospective study, we aimed to evaluate the potential role of contrasted dynamic multidetector computed tomography (MDCT) as an additional imagining modality in the detection of occult satellite tumors in the targeted breast tissue and the relationship of the tumors with surrounding tissue and pectoral fascia. All of these are important regarding local recurrence in patients with a diagnosis of BC. Furthermore, we also evaluated the effect of the contrasted dynamic MDCT on the treatment planning of breast cancer. All patients who were recruited for the study were given a contrasted dynamic MDCT two days before the surgery. Patients were examined before and after the contrast injection. Patients who were known to have an allergic reaction for contrast dye were not given the contrasted dynamic MDCT and were also excluded from the study. Before the contrasted images of the breast tissue were made, plain images were collected. Using an MDCT scanner (Picker, MX Twin, Israel) set for 1-mm collimation and a pitch of 5.5, we scanned our patients from the level of the axilla to the lower edge of the breast. Patients were asked to hold their breath four times both before and 1, 3, and 8 min after an IV rapid bolus administration of nonionic contrast material. We infused 100 mL of nonionic contrast material (Ultravist [iopromid], Schering, Germany) at a rate of 3.0 mL/sec. The data were reconstructed at 0.6 mm increments. Patients underwent MDCT in the supine position because it allowed surgical PATIENTS AND METHODS The study comprises all patients who applied to the breast clinic between September 2003 and February 2005 either for routine breast checkups or for a breast mass diagnosed by selfexamination. Among these patients, women who were diagnosed with BC by physical examination, ultrasonography (USG), mammography and pathological analysis were identified as potential candidates for BCS and were recruited for the study. Candidacy of the patients for BCS was determined using four major criteria; a) physical examination, b) laboratory tests ( USG, 110 Marmara Medical Journal 2006;19(3);109-115 Taner Yiğit, et al. The role of contrasted dynamic multi-detector computerized tomography imaging on surgical decision making in breast cancer patients simulation on three-dimensional data displays. After reconstruction, the images were transferred to a workstation. Multiplanar reconstructions (axial, coronal, and sagittal) and maximal intensity projections were used for the evaluation of tumors. One radiologist reviewed all detected lesions for morphologic features and time-density curve patterns. All detected lesions were classified as either mass or nonmass enhancing lesions. For mass lesions, margin, shape and enhancement patterns were evaluated. Irregular contour and shape, early contrasting and washout were assigned as malignancy criteria. Clinical TNM classification of the patients revealed that 11(36.6%) patients had T1N1M0 tumors, six (20%) patients had T1N2M0 tumors, five (16.7%) patients had T2N0M0 tumors, three (10%) patients had T2N1M0 tumors and five (16.7%) patients had T2N2M0 tumors (Fig. 2). Mean tumor size was 21.7 (range10-40) mm.The tumor was localized in the upper outer lobe in 17(56.6%) patients, lower outer lobe in 4 (13.3%) patients, lower inner lobe in 2 (6.6%) patients and upper inner lobe of the left breast in 7 (23.5%) patients. Contrasted dynamic MDCT results revealed that 2 (6.7%) patients had a tumor with a very close margin or connection to the pectoral fascia (Fig.3), 3(10%) patients had a satellite tumor, which had not been noticed in the USG and mammography during the preoperative period (Fig. 4) (Table-I). One of these two patients with pectoral connection also had satellite tumor focus. Detailed specifications of those four patients have been given in table. (Table-II) Finally, these 4 (13.3%) patients who seemed to have contraindication for BCS were given a modified radical mastectomy (MRM) with their informed consent. Patients’ candidacy for BCS was reevaluated according to contrasted dynamic MDCT results. Patients with a satellite tumor requiring large breast tissue excision and / or patients with a lesion which was related to the pectoral fascia were designated as patients who were not eligible for BCS. These groups of patients were also informed about the potential risks of BCS under these conditions and they were prepared to undergo mastectomy with their informed consent. Accuracy of the contrasted dynamic MDCT results was controlled by retrospectively comparing these data with excision materials’ pathology results. Primary outcomes of this study were finding a ratio of the patients who were given MDCT and diagnosed having additional tumor with/or pectoral muscle infiltration to the total number of cohort who were recruited to the study. Hereby, we aimed to investigate of the accuracy of MDCT in protection to the patients from local recurrence. All steps of this study design were approved by the Gulhane Military Medical Academy Human Subject Review Committee and meet the guidelines of this governmental agency. Fig. 1: Distribution of the tumor according the pathological classification. RESULTS Thirty elegible patients were identified and recruited for the study. The mean age of the patients was 46.7 (range 33-66) years old. All patients were reported as having one solid lesion in their breast detected by USG and mammography. Malignancies in all patients were diagnosed using FNB. According to the FNB results, three (10%) patients had infiltrative lobular carcinoma, two patients (6.6%) had infiltrative ductal + infiltrative lobular carcinoma (mixed type) and 25 (83,4%) patients had infiltrative ductal carcinoma (Fig. 1). Fig. 2: Distribution of the tumor according the TNM classification 111 Marmara Medical Journal 2006;19(3);109-115 Taner Yiğit, et al. The role of contrasted dynamic multi-detector computerized tomography imaging on surgical decision making in breast cancer patients Fig. 3: Pectoral invasion of the primary tumor. Fig. 4: Conglomerate LAP and satellite tumor. Table 1: Primary tumor and axillary assessment results according to the contrasted dynamic MDCT Single lesion Satellite lesion Pectoral Invasion 26(%86.7) 2(%6.7%) 1(3.3%) Satellite lesion + Pectoral Invasion 1(3.3%) Table II: Detailed specifications of patients diagnosed satellite tumor and pectoral invasion detected with MDCT Patient 1 Patient 2 Patient 3 Patient 4 Age 35 35 48 40 Tumor Localization Upper outer Upper outer Upper outer Lower inner Satellite No Yes Yes No Pectoral invasion Yes Yes No Yes and although mastectomy continues to be appropriate for some patients, nowadays many authors recommend BCS for surgical treatment of BC. Clinical trials have revealed that overall and disease-free survival rates are equal or nearly equal for mastectomy and BCS with postoperative radiotherapy.5-7,13,14 Patients who had abnormal findings in contrasted dynamic MDCT and who were given MRM were reevaluated during the postoperative period using pathology examination results. Two pectoral fascia connections and two satellite tumors, noticed in contrasted dynamic MDCT, were confirmed with pathology results. As for other satellite tumor focus, it was not confirmed by pathology. But this patient also had a pectoral fascia involvement, which was identified by contrasted dynamic MDCT and further confirmed by pathology examination. BCS has advantages in helping the patient retain a better body image, better sexual condition and less negative effect on a patient’s emotional state. It also offers better quality of life 15,16. Although it has many advantages, local recurrence rates have been reported as 3-17% after the BCS and 2-10% for mastectomy 7. Even local recurrence rates have been reported higher than for mastectomy with similar rates for distant metastasis. Overall mortality and BC specific mortality rates have already been reported 17. DISCUSSION Surgical treatment of breast carcinoma has improved in recent decades. While mastectomy has been the historical mainstay of surgical treatment of stage-I and stage-II BC for decades 112 Marmara Medical Journal 2006;19(3);109-115 Taner Yiğit, et al. The role of contrasted dynamic multi-detector computerized tomography imaging on surgical decision making in breast cancer patients examination due to certain contraindications, serious claustrophobic syndrome or lack of a machine. In this study we aimed to verify the diagnostic value of contrasted dynamic MDCT in patients with certain conditions listed above. Data obtained in this study revealed that 4 patients for whom a BCS was planned were not suited for this operation due to satellite tumor focus and /or close margin of tumor to the pectoral fascia. These 4 patients’ operation strategies were changed from BCS to MRM. We think that by doing so we prevented our patients from local recurrence, which may have developed in the future. Bilateral thoracic wall examination capacity, suitability for easier approach to imaging-guided fine needle aspiration and hookwire localization are the other advantages provided by contrasted dynamic MDCT 33-35. The main problem related to the use of contrasted dynamic MDCT in the diagnosis of BC is the X-ray exposure. A previous study, comparing the radiation doses delivered to the breast during conventional breast CT with those involved in mammography, revealed that for conventional CT the doses are almost 10 times higher than those administrated in standard mammographic examination 36. In our opinion,this is sufficient reason to choose MRI over CT in the diagnosis of BC. On the other hand, extra X-Ray exposure of healthy breasts and lungs seems negligent in patients diagnosed with BC because these patients are usually going to have postoperative radiotherapy after having undergone BCS. Additionally, a study performed by Boone et al 37 in 2001 revealed that the total X-Ray dose in the CT performed with 80 kVp, 300 mAs and 5 mm slice are nearly equal with X-Ray dose in mammography performed to the 5 cm compressed breast tissue. They also reported that the X-Ray dose is one-third less in CT than when using mammography of a large breast, which is thicker than 5 cm with compression. Allergic reaction to the contrast agent used for CT is another side effect of the CT. But we believe that this possibility is not a contraindication for CT because it is not any higher in CT than in other radiological contrasted procedures. Local recurrence has a negative effect on a patient’s quality of life. Furthermore, additional operation requirements make this clinical dilemma difficult to accept for the patient. In order to prevent local recurrence of the cancer, everything possible will be attempted according to the patient’s wishes and, hopefully, such efforts will increase the patient's satisfaction with treatment. Multicentricity and positive or close resection margin are two important factors in the development of local recurrence. Patients with satellite tumors are poor candidates for BCS 16. Satellite tumors usually require extensive resections and negatively affect cosmetic outcome. Additionally in case of misdiagnosis, satellite tumors cause local recurrence after BCS. Liberman et al.18 reported that magnetic resonance imaging identified additional occult lesions in addition to the index breast lesion in 19(27%) of 70 consecutive women. Studies have shown unacceptably high false-negative rates for both USG and mammography, with detection of very few clinically occult carcinomas 19,20. To minimize the risk of local recurrence, the surgical management of breast cancer relies on complete excision of the tumor with a margin of normal tissue 21. The local recurrence rates at 5 years have been reported as between 2% and 24% for patients with invaded margins, 6%-16% with close margins,and 2%-7% with clear margins 22-24. The initial surgical biopsy of breast cancer results with positive margins were between 45% to 54% of the patients 25,26. Although Schmitt-Urlich et al 25 demonstrated that local recurrences could be virtually eliminated by adjusting the magnitude of the extension site boost dose to the relative degree of margin clearance, some authors claimed that with proper selection criteria, it may be safe to omit adjuvant radiotherapy in patients with adequate margins 27,28. It is obvious that a clear margin is important in preventing local recurrence and intensive radiotherapy protocols. Additional diagnostic tools aim to identify any occult satellite lesions or penetration of the tumor to the deeper tissues (pectoral fascia) and are beneficial in preventing patients from local recurrence. In conclusion, contrasted dynamic MDCT offers valuable data in the determination of satellite tumor focus and the relation of the primary tumor to the pectoral fascia especially for patients with certain contraindications for MRI and claustrophobia and in case of absence of the MRI unit. We believe that MDCT is a good alternative to MRI in patients who are planning to have a BCS and requiring additional radiologic Recently magnetic resonance imagining (MRI) of the breast gained a role in clarifying uncertain cases (suspicious tumor focus which can not be identified with conventional techniques) after mammography and / or USG, and in detecting multifocal, multisentric, and bilateral breast cancers 29-32. Nevertheless, a not negligible number of patients cannot undergo MRI 113 Marmara Medical Journal 2006;19(3);109-115 Taner Yiğit, et al. The role of contrasted dynamic multi-detector computerized tomography imaging on surgical decision making in breast cancer patients 16. Wolmark N, Wang J, Mamounas E, Bryant J, Fisher B. Preoperative chemotherapy in patients with operable breast cancer: nine-year results from National Surgical Adjuvant Breast and Bowel Project B-18. J Natl Cancer Inst Monogr 2001;30:96-102. 17. Meric-Bernstam F. 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Radiology 2001;22 :657-667. 115 ARAŞTIRMA YAZISI TESTİS SEMİNOMU VE İNTRAKRANYAL GERMİNOMUN İMMÜNHİSTOKİMYASAL VE HİSTOMORFOLOJİK ÖZELLİKLERİNİN KARŞILAŞTIRILMASI Ahmet Midi1, Süheyla Bozkurt1, Aydın Sav1, M Memet Özek2, Necmettin Pamir2 Marmara Üniversitesi Nörolojik Bilimler Enstitüsü, Talia Balı Aykan Patoloji Laboratuarı, İstanbul, Türkiye 2Marmara Üniversitesi Tıp Fakültesi, Nöroşirürji Anabilim Dalı, İstanbul, Türkiye 1 ÖZET Amaç: Kranyal germinom, testis seminomu ve over disgerminomu histolojik olarak benzer özelliklere sahip ancak farklı lokalizasyonlarda yerleşen tümörlerdir. Bu çalışmada testis seminomu ile kranyal germinomlar tümör içerisindeki lenfosit ve eozinofillerin oranı, Ki–67, PLAP, vimentin immünhistokimyasal boyanma özellikleri bakımından karşılaştırılmıştır. Gereç ve Yöntem: M.Ü. Nörolojik Bilimler Enstitüsünde 1995–2002 yılları arasında tanı almış 10 adet intrakranyal germinom ve 2 adet testis seminomuyla Marmara Üniversitesi Tıp. Fakültesi Patoloji Anabilim Dalı’nda 1998–2003 yılları arasında tanı almış 6 adet testis seminomu olgusu çalışma kapsamına alınmıştır. Olgulara PLAP, vimentin, Ki–67, tümör içerisindeki lenfosit yoğunluğunu saptamak için CD45 immünhistokimyasal boyamalar yapılmıştır. Bulgular ve Sonuç: Germinom olgularının yaş aralığı 9–30 olup ortalama yaş 17 dir. Seminom olgularının yaş aralığı 15–49 olup ortalama yaş 32,6’dır. Çalışmamızda kranyal germinom ve seminomlarda tümör içi lenfosit ve eozinofil varlığı yanısıra PLAP ve vimentin immünhistokimyasal boyanma özellikleri bakımından istatistiksel olarak anlamlı fark bulunmamıştır. MIB-1 (Ki-67) proliferasyon indeksi seminomda %44, germinomda %25 olarak bulunmuştur ve fark istatistiksel olarak anlamlıdır. Anahtar Kelimeler: Kranyal germinom, Testis seminomu, Lenfosit, Eozinofil, Ki–67, PLAP COMPARING OF THE IMMUNOHISTOCHEMICAL AND MORPHOLOGICAL FEATURES OF THE TESTICULAR SEMİNOMA AND INTRACRANIAL GERMNOMA ABSTRACT Objective: Cranial germinoma, testicular seminoma and ovarian dysgerminoma are tumours with similar histological features but located at different localizations. In this study; cranial germinoma and testicular seminoma, has been compared for the ratio of the tumor infiltrating lymphocytes, eosinophils, and immunohistochemical staining features for the Ki–67, PLAP, and vimentin. Materials and Methods: Cases were obtained from the archives of the Pathology Laboratory, Marmara University Hospital, Institute of Neurological Science (10 IKG and 2 testicular seminoma in the time period of 1995 to 2002 ), and Pathology Laboratory, Marmara University Hospital (6 testicular seminoma, in the time period of 1998 to 2003 ). We performed immunohistochemical staining for PLAP, vimentin, CD45 and Ki–67. Results and Conclusion: The germinoma cases were aged between 9–30, (median age 17). The seminoma cases were aged between 15–49, (median age 32, 6). Keywords: Cranial germinoma, Testicular seminoma, Lymphocite, Eosinophils, Ki-67, PLAP morfolojik ve immünhistokimyasal olarak karşılaştıran 2 çalışma bulunmaktadır 5,6. Germinom primer intrakranyal tümörlerin yaklaşık % 0,5-2'ini oluşturan en sık görülen intrakranyal germ hücre kaynaklı tümördür. GİRİŞ Seminoma ve kranyal germinom (KG) benzer histolojik özellikler gösteren farklı yerlerde oluşan germ hücreli tümörlerdir1-4. Kaynaklarda seminoma ve germinom'u İletişim Bilgileri: Ahmet Midi e-mail: ahmetmidi@yahoo.com Marmara Üniversitesi Nörolojik Bilimler Enstitüsü, Talia Balı Aykan Patoloji Laboratuarı, İstanbul, Türkiye 116 Marmara Medical Journal 2006;19(3);116-120 Marmara Medical Journal 2006;19(3);116-120 Ahmet Midi ve ark. Testis seminomu ve intrakranyal germinomun immünhistokimyasal ve histomorfolojik özelliklerinin karşılaştırılması En sık pineal bölge ve suprasellar bölgede yerleşirler 2,3,7. KG seminomlar gibi radyoterapiye oldukça duyarlı tümörlerdir 4. Germinom iri, yuvarlak veziküler nükleuslu, düzensiz, pleomorfik nükleolu olan, PAS pozitif boyanan geniş sitoplazmalı, iri neoplastik hücreler ile değişen yoğunlukta lenfositlerden meydana gelen iki hücre popülâsyonundan oluşmaktadır 2. Germ hücreleri alkalen fosfataz pozitif olup nadir olgularda ‘'ß-human chorionic gonadotrophin'' (ß-HCG) pozitif boyanan sinsisyotrofoblastlarda bulunabilir 2. Seminoma tüm germ hücreli tümörler arasında en sık görülen germ hücreli tümördür. Mikroskopik olarak birbirlerine benzeyen hücreler tabakalar oluşturur ve fibröz septumlarla lobüllere ayrılır. Hücreler iri, yuvarlak ya da çok kenarlı olup belirgin hücre membranlı olan sitoplâzmaları berrak görünümdedir. Hücrelerin büyük kısmının 1 ila 3 belirgin nükleolu olan iri ve merkezi yerleşimli nükleusları bulunur 6,8. Kaynaklarda ekstra gonad kökenli germ hücreli tümörlerin kemoterapiye testisin germ hücreli tümörlerinden daha az yanıt verdiği belirtilmektedir 2. Bu durum iki farklı lokalizasyonla yerleşmiş germ hücreli tümörün morfolojik ve immünhistokimyasal özelliklerinin farklı olabileceği ihtimalini akla getirmektedir. Bu nedenle bu çalışmada testis seminomu ile KG'nin tümör içi lenfosit, eozinofil yoğunluğu bakımından karşılaştırılması ve immünhistokimyasal olarak PLAP, vimentin Ki–67 immünekspresivitelerinin belirlenmesi amaçlanmıştır. GEREÇ VE YÖNTEM On KG ve sekiz testis seminomu olgusu çalışmaya alınmıştır. Olgulara PLAP, vimentin ve Ki–67 immünhistokimya boyaları uygulanmıştır. Değerlendirmeler kalitatif yapılmıştır. Buna göre PLAP, vimentin ve Ki–67 boyalarının boyanan hücre yaygınlığına göre (negatif, fokal pozitif, dağınık pozitif, yaygın pozitif) ve boyanma şiddetine göre 0–3 arasında sayısal değerlendirme yapılmıştır. ( 0:boyanma izlenmedi, 1: zayıf boyanma, 2: orta şiddette boyanma, 3: şiddetli boyanma). Tüm olgularda tümör içi lenfosit, eozinofil yoğunluğu kantitatif değerlendirilmiştir ve 0– 3 arası sayısal değere dönüştürülmüştür. (0: yok, 1: seyrek, 2:orta yoğunlukta, 3 yoğun). Sonuçların değerlendirmesinde istatistiksel olarak Mann-Whitney U-testi kullanılmıştır. İmmünhistokimya İmmünohistokimyasal Boyama Yöntemi Rezeksiyon materyalleri %4'lük tamponlanmış formaldehitte fikse edildikten sonra parafinle bloklanmıştır. Parafin bloklardan 3 aminopropyltriethoxysilane kaplı lamlara 2µ kalınlıkta kesitler alınarak immünohistokimyasal boyama yapılmıştır. Boyamada sırası ile şu işlemler yapılmıştır. 1. Kesitler etüvde 60ºC de 1 saat deparafinize edildi. 2. Daha sonra ksilende 10 dakika, %100'lük alkolde 5 dakika bekletildi ve su ile yıkandı. 3. %10'luk sitratla tamponlanmış solüsyon içine yerleştirilen lamlar mikrodalgada maksimum güçte (800 watt) 15 dakika bekletildi. 4. Daha sonra güç yarıya düşürülerek 20 dakika daha mikrodalgada bekletildi. 5. Mikrodalgadan çıkarılan lamlar oda sıcaklığında 20 dakika bekletildi. 6. Endojen peroksidaz aktivitesi %3'lük hidrojen peroksitte 20 dakika bekletilerek giderildi. 7. Distile su ile yıkanan lamlar 3x5 PBS ile muamele edildikten sonra üzerlerine protein blokajı damlatıldı. 8. Beş dakika sonra blokaj yıkanmadan lamların üzerine PLAP, vimentin, Ki–67 antikoru damlatıldı. 9. Primer antikorda 30 dakika bekletildikten sonra PBS'e alınarak 5 dakika yıkandı. 10. Daha sonra biyotinlenmiş sekonder antikor ile 20 dakika muamele edildi ve PBS'te 5 dakika yıkandı. 11. Peroksidaz konjuge antikor ile 20 dakika bekletildi. Daha sonra 5 dakika PBS'te yıkandı. 12. Kromojende (DAB) 5 dakika bekletildi. 13. Musluk suyu altında yıkanan lamlar hematoksilen ile karşıt boyama yapıldı. 14. Dehidrate edildi, kurutuldu ve entellan ile kapatıldı. 117 Marmara Medical Journal 2006;19(3);116-120 Ahmet Midi ve ark. Testis seminomu ve intrakranyal germinomun immünhistokimyasal ve histomorfolojik özelliklerinin karşılaştırılması yoğunluğu bakımından gruplar arasında istatisitksel olarak anlamlılık saptanmamıştır (p= 0.96). Ki–67 ortalama değeri seminomda %44 (%15-%76), germinomda %25 (%0%62) olarak bulunmuştur ve fark istatistiksel olarak anlamlıdır (p=0.026). PLAP immünhistokimya boyası ile 3 germinoma ve 1 seminoma olgusunda boyanma olmazken, 7 germinoma (%70) ve 7 (%87) seminoma olgusunda değişen yoğunlukta boyanma izlenmiştir. PLAP boyanma özelliği bakımından gruplar arasında istatistiksel olarak anlamlılık saptanmamıştır (p= 0.49). Vimentin ile 4 (%40) germinoma, 2 (%25) seminoma olgusunda fokal ya da dağınık boyanma izlenmiştir. Altı germinoma ve 6 seminoma olgusunda boyanma olmamıştır. Vimentin boyanma özelliği bakımından gruplar arasında istatistiksel olarak anlamlı fark saptanmamıştır (p= 0.57). BULGULAR KG olgularının yaş aralığı 9–30 olup ortalama yaş 17 dir. Olguların 1'i kadın 9' u erkektir. Seminom olgularının yaş aralığı 15–49 olup ortalama yaş 32 dir. Tablo-1'de germinomların (Resim 1,2,3), Tablo-2'de seminomların (Resim 4,5) demografik, immünhistokimyasal özellikleri, lenfosit ve eozinofil yoğunlukları belirtilmiştir. CD45 immünhistokimya boyası ile saptanan lenfosit her iki grupta tüm olgularda değişik yoğunlukta (+ veya +++) izlenmiş olup gruplar arasında istatisitksel olarak anlamlılık saptanmamıştır (p= 0.14). Eozinofil germinoma olgularının 7'sinde değişik yoğunlukta (+ veya ++) saptanırken 3 olguda izlenmemiştir. Seminoma olgularının ise 6'sında değişen oranda izlenirken (+ veya ++) 2 tanesinde görülmemiştir. Eozinofil Tablo 1: Germinomaların demografik, morfolojik ve İHK özellikleri No 1 2 3 4 5 6 7 8 9 10 Yaş/Cin siyet 15/E ?/E ?/E 28/E 11/E 12/E 12/E 19/E 30/E 9/K Lenfosit Eozinofil +++ +++ ++ + +++ ++ +++ ++ ++ +++ + + + + + ++ ++ Ki–67 (%) 7 28 50 1 55 1 0 20 62 28 PLAP Vimentin Diffüz ++ Diffüz +++ Diffüz +++ Diffüz ++ Fokal ++ Diffüz +++ Dağınık ++ - Fokal +++ Dağınık ++ Dağınık ++ Dağınık ++ Tablo 2: Seminomalarin demografik, morfolojik ve İHK özellikleri No 1 2 3 4 5 6 7 8 Yaş ? 21 40 15 38 35 49 31 Lenfosit ++ +++ + + ++ +++ + + Eozinofil + ++ + + + + Ki–67 (%) 76 48 52 36 46 15 29 50 118 PLAP Diffüz ++ Diffüz +++ Diffüz ++ Diffüz +++ Diffüz ++ Diffüz +++ Diffüz +++ Vimentin Dağınık ++ Dağınık ++ - Marmara Medical Journal 2006;19(3);116-120 Ahmet Midi ve ark. Testis seminomu ve intrakranyal germinomun immünhistokimyasal ve histomorfolojik özelliklerinin karşılaştırılması Resim 1: Germinoma H&E X 400; germ hücreleri, lenfosit ve eozinofilller. Resim 2: Germinoma Ki-67 X 200; germ hücrelerinde nükleer boyanma. Resim 4: Seminoma H&E X 100; lenfositik zeminde tabakalar halinde germ gücreleri. Resim 3: Germinoma CD45 X 400; lenfositlerde yoğun boyanma. TARTIŞMA KG histolojik olarak over disgerminomu ve testis seminomuna benzer. Nispeten düşük dereceli bir malignite özellikleri gösterir. Ancak çevre yapıları infiltre edebilir. Ependim boyunca ventriküllere ve subaraknoid alana yayılma eğilimi gösterirler 7 . Yüksek proliferatif aktiviteye sahip olabilirler. Martinazzi M ve ark.'nın çalışmasında Ki–67 proliferatif indeksi seminomada %23,8 bulunmuştur 8. Mevcut çalışmada 3 KG ve 3 seminoma olgusunda Ki–67 proliferatif indeksi %50 veya üstünde bulunmuştur. Resim 5: Seminoma PLAP X 200; germ hücrelerinde boyanma. 119 Marmara Medical Journal 2006;19(3);116-120 Ahmet Midi ve ark. Testis seminomu ve intrakranyal germinomun immünhistokimyasal ve histomorfolojik özelliklerinin karşılaştırılması Vimentin Nakagawa Y ve ark'nın çalışmasında 7 germinomanın 3'ünde pozitif iken, gonadal germ hücreli tümörlerde negatif olarak bulunmuştur 5. Bentley AJ ve arkadaşlarının çalışmasında germinoma ve seminoma olgularında vimentin ile boyanma izlenmemiştir 6. Mevcut çalışmada vimentin ile 4 (%40) germinoma, 2 seminoma (% 25) olgusunda fokal ya da dağınık boyanma izlenmiştir. Hattab EM ve ark.'nın çalışmasında KG'de PLAP % 92 olguda pozitif izlenmiştir 9. Klasik seminomada ise %100 pozitif boyanma bildirilmiştir 10. Mevcut çalışmada PLAP ile 7 germinoma (%70) ve 7 (%87) seminoma olgusunda değişen yoğunlukta boyanma izlenmiştir. Kaynaklarda KG ve seminomada eozinofil yoğunluğu ile ilgili çalışma bulunmamaktadır. Çalışmamızda KG'da %70 olguda değişen yoğunlukta eozinofil izlenmiştir. KG ayırıcı tanısına giren histiyositozis x'de yoğun eozinofil izlenir. Bir çalışmada eozinofil varlığının histiyositozis x'e spesifik olmadığı KG'da da bulunabilmesi nedeniyle bu lezyonların ayırıcı tanısında, özellikle iğne biyopsilerinde eozinofil varlığının histiyositozis x'e spesifik bir bulgu olarak yorumlanmaması gerektiği vurgulanmıştır11. Ingilizce literatürde seminom ve KG'da lenfositlerin immünohistokimyasal özellikleri ile ilgili çalışmalar olmakla birlikte lenfosit yoğunlu ile ilgili 1 çalışmaya rastlanmıştır 1,12,13 . Mevcut çalışmada değişen yoğunlukta lenfosit tüm olgularda izlenmiş olup lenfosit yoğunluğu bakımından seminom ve KG benzer özellikler göstermiştir. İntrakranyal germinomun ve testis seminomunun aynı hücreden köken almaları nedeniyle Ki–67 proliferatif indeksinin mevcut çalışmada farklı çıkmasının anlamı açık değildir. Hsu YJ ve arkadaşlarının çalışmasında ekstra gonad kökenli germ hücreli tümörlerin kemoterapiye testisin germ hücreli tümörlerinden daha az yanıt verdiği belirtilmektedir 2. Her ne kadar seminomda Ki–67 proliferatif indeksin germinomdan yüksek çıkması tedavilere farklı yanıt vermelerini destekleyen bulgu olarak gözükse de, serimizin az sayıda olgudan oluşması nedeniyle bu farklılığı seminomdaki hücre proliferasyonunun fazlalığına ve bu nedenle kemo-radyoterapiye duyarlılığının artışına bağlanamamıştır. Bu konuda bölgesel ve çevresel etkenlerin proliferatif indeksin üzerine etkisinin araştırılmasına gerek vardır. KAYNAKLAR 1. Parker C, Milosevic M, Panzarella T, Banerjee D, Jewett M, Catton C, et al. The prognostic significance of the tumor infiltrating lymphocyte count in stage 1 testicular seminoma managed by surveillance. Eur J Cancer 2002; 38 (15):2014-9. 2. Hsu YJ, Pai L, Chen YC, Ho CL, Kao WY, Chao TY. Extragonadal germ cell tumors in Taiwan: an analysis of treatment results of 59 patients. Cancer 2002; 95:766– 74. 3. El Abbadi N, Maagili MR, Arkha Y, Amarti A, Bellakhdar F. Cerebellar primary germinom. Case report. Neurochirurgie 2002; 48:351-4. 4. Wolden SL, Wara WM, Larson DA, Prados MD, Edwards MSB, Sneed PK. Radiation therapy for primary intracranial germ-cell tumors. Int J Radiat Oncol Biol Phys. 1995; 32:943–47. 5. Nakagawa Y, Perentes E, Ross GW, Ross AN, Rubinstein LJ. immunohistochemical differences between intracranial germinomas and their gonadal equivalents. An immunoperoxidase study of germ cell tumors with epithelial membrane antigen, cytokeratin, and vimentin. J Pathol. 1988; 156(1):67-72. 6. Bentley AJ, Parkinson MC, Harding BN, Bains RM, Lantos PL. A comparative morphological and immunohistochemical study of testicular seminomas and intracranial germinomas. Histopathology. 1990;17(5):443-9. 7. Tekkok IH, Sav A. Aggressive spinal germinom with ascending metastases. J Neurooncol. 2005;75(2):135– 41. 8. Martinazzi M, Zampieri A, Martinazzi S, Crivelli F, Mauri MF, Calandra C.Proliferative activity of stage I testicular neoplasms: evaluation by image analysis of immunoreactive MIB-1. Pathologica. 1998; 90(6):783-7. 9. Hattab EM, Tu PH, Wilson JD, Cheng L. OCT4 immunohistochemistry is superior to placental alkaline phosphatase (PLAP) in the diagnosis of central nervous system germinoma. Am J Surg Pathol. 2005 ;29(3):36871. 10. Bailey D, Marks A, Stratis M, Baumal R. Immunohistochemical staining of germ cell tumors and intratubular malignant germ cells of the testis using antibody to placental alkaline phosphatase and a monoclonal anti-seminoma antibody. Mod Pathol. 1991; 4(2):167-71. 11. Midi A, Bozkurt S, Yapıcıer Ö, Sav A.. Langerhans Cell Histiocytosis and Intracranial Germinoma: Are Immunohistology Techniques Helpful in Distinguishing Two Entities?. Journal oj Neurological Sciences (Türkish) . 2006; 23 (3): 209-214 12. Bell DA, Flotte TJ, Bhan AK. Immunohistochemical characterization of seminoma and its inflammatory cell infiltrate. Hum Pathol. 1987; 18(5):511-20. 13. Grobholz R, Verbeke CS, Schleger C, Kohrmann KU, Hein B, Wolf G,et al. Expression of MAGE antigens and analysis of the inflammatory T-cell infiltrate in human seminoma. Urol Res. 2000; 28(6):398-403. 120 ORIGINAL RESEARCH EVALUATION OF INITIATING, CONTINUING AND WEANING TIME OF BREASTFEEDING Ruhusen Kutlu, Kamile Marakoğlu Selcuk Üniversitesi, Meram Tıp Fakültesi, Aile Hekimliği Anabilim Dalı, Konya, Türkiye ABSTRACT Objective: The aim of this study was to evaluate the knowledge and behavior of mothers related to the initiation time, duration and affecting factors of breastfeeding. Methods: This descriptive and cross-sectional study included 214 mothers who had children aged between 1-72 months. These mothers were selected from the pediatric policlinics of the Meram Medical Faculty. Data were obtained via questionnaire form by interviewing. Statistical analyses were performed using the software package SPSS version 10.0. Results: The mean age of the mothers was 28.96 (SD±6.06). 187 mothers (87.4 %) delivered in the hospital. 20 mothers (9.3 %) delivered in a primary health care center. The rate of exclusive breast-feeding for at least 6 months was 37.7 %. The rate of breast-feeding initiation time in the first 1-2 hours after delivery was 78.9 %. The mean breast-feeding weaning duration was 10.6 months (SD±5.69). Conclusion: In our study, 96.7 % of the mothers delivered their babies in a hospital or primary health care center under the control of a nurse or a doctor. All health professionals should have the necessary knowledge and skills in order to protect, promote and support breastfeeding. Consequently, prenatal and postnatal breastfeeding education and support courses should be provided everywhere. Keywords: Breastfeeding, Education program, Mother’s health ANNE SÜTÜNE BAŞLAMA, DEVAM VE KESME ZAMANININ DEĞERLENDİRİLMESİ ÖZET Amaç: Bu çalışmada anne sütüne ilk başlama zamanı, süresi ve bunu etkileyen faktörlerle ilgili annelerin bilgi ve davranışlarını değerlendirmeyi amaçladık. Yöntem: Tanımlayıcı ve kesitsel tipteki bu çalışma 1-72 aylık bebeği olan 214 annede yapıldı. Çalışmaya katılan anneler Meram Tıp Fakültesi çocuk kliniğinden seçildi. Veriler anket yoluyla ve yüz yüze görüşülerek elde edildi. İstatistik analizler bilgisayarda SPSS 10.0 programı kullanılarak yapıldı. Bulgular: Annelerin yaş ortalaması 28,86 (SD±6.06) olup, 187 anne (%87.4) hastanede, 20 anne(%9.3) sağlık ocağında doğum yapmıştı. En az 6 ay süre ile etkili anne sütü verme sıklığı %37.7. idi. Doğumdan sonraki ilk 1-2 saat içinde anne sütüne başlama sıklığı %78,9 olup, anne sütünü kesme süresinin ortalama değeri 10,6 ay (SD±5.69) idi. Sonuç: Bizim çalışmamızda tüm annelerin %96,7’si hastane veya bir sağlık ocağında hekim ya da bir hemşire kontrolü altında doğum yapmıştı. Bu nedenle, sağlık personeli anne sütünün desteklenmesi, korunması ve devam ettirilmesi konularında gerekli bilgi ve beceriye sahip olmak zorundadır. Bu yüzden, her yerde doğum öncesi ve sonrası anne sütü eğitimi ve destekleme kursları düzenlenmelidir. Anahtar Kelimeler: Anne sütü, Eğitim programı, Anne sağlığı active compounds including hormones, cytokines and enzymes which are important not only for the maturation of the immune system but also for neurological development, especially in premature infants. Breastfeeding is the healthiest way to feed INTRODUCTION Breast milk is the ideal food for newnates and infants. Breastfeeding also has many advantages in regard to the mother’s health1-4.Breast milk contains many different kinds of biologically İletişim Bilgileri: Ruhusen Kutlu e-mail: ruhuse@yahoo.com Selcuk Üniversitesi Meram Tıp Fakültesi, Aile Hekimliği AD, Konya, Türkiye 121 Marmara Medical Journal 2006;19(3);121-126 Marmara Medical Journal 2006;19(3);121-126 Ruhusen Kutlu, et al Evaluation of initiating, continuing and weaning time of breastfeeding a baby. It is possible, but difficult, for mothers to reverse a decision not to breastfeed or to re-start breastfeeding once they have stopped. Introducing partial bottle feeding will reduce a mother's breast milk supply5-7. METHODS This descriptive and cross-sectional study was carried out among 214 mothers who had 1-72 months old babies between July 10th and December 30 th 2003. The hospitals where the deliveries took place were not taken into consideration but the types of delivery were listed. After finishing this study, the midwives, nurses, pediatricians and gynecologists were trained in a three-day course on the Ten Steps to successful breastfeeding. In this training and promotion course, the techniques, benefits, initiation, continuing and weaning time of breastfeeding were explained. It is emphasized that breastfeeding should begin as soon as possible after birth, usually within the first half hour, and exclusive breastfeeding should continue for the first 6 months. It is also recommended that breastfeeding continue for at least 24 months.1,14 In the pediatric hospital, a breastfeeding room was organized. Posters supporting breastfeeding were placed on the walls of the hospital. Booklets informing all pregnant women and lactating mothers about the benefits and management of breastfeeding were delivered. An exclusive handout about breastfeeding policy for all health care staff was prepared. We held public meetings to advertise the Baby Friendly Hospital Initiative. Then we organized breastfeeding support groups between mothers. We plan to carry out another study to evaluate the results obtained from the breastfeeding education program later on. Epidemiologic research shows that human milk and breastfeeding of infants provides advantages in terms of general health, growth and development. Breastfeeding also significantly decreases the risk of a large number of acute and chronic diseases. In addition to reducing childhood infections, breastfeeding may also protect against sudden infant death syndrome, insulin-dependent diabetes mellitus, Crohn’s disease, ulcerative colitis, lymphoma, allergic diseases and other chronic digestive diseases.5,8,9 Breastfeeding has also been related to possible enhancement of cognitive development.6,10 There are also a number of studies that indicate some possible health benefits of breastfeeding for mothers. It has been known for a long time that breastfeeding increases the level of oxytocin, resulting in less postpartum bleeding and more rapid uterine involution. Lactational amenorrhea causes less menstrual blood loss over the months after delivery. A lactating woman returns to her normal weight much earlier. The risk of ovarian cancer and pre-menopausal breast cancer decreases.6,11 The Baby Friendly Hospital Initiative (BFHI) is a global program initiated in 1991 by the World Health Organization (WHO) and the United Nations Children’s Fund (UNICEF) in response to the Innocent Declaration (1990)12. WHO and UNICEF recommend exclusive breastfeeding until the sixth month and breastfeeding combined with a gradually diversified food intake until the age of 2.1,6,13,14 A pediatrician interviewed mothers who were included in this study. Information was recorded on a standardized form. The questionnaire included 21 items and revealed the women’s sociodemographic characteristics, education and details of the pregnancy, economic status, smoking habit during pregnancy, type of delivery, place of delivery, smoking status of their husbands, child’s weight and height characteristics at birth, initiation of breastfeeding, duration of exclusive breastfeeding, the weaning of breastfeeding, and the use of a pacifier. In practical terms, a Baby Friendly Hospital/Maternity facility encourages and helps women to successfully initiate and continue to breastfeed their babies. Since the inception of the program, over 15,000 hospitals worldwide have received the Baby Friendly designation12. The commercial SPSS 10.0 software was used for data entry on the computer. This software was also used in the classification and statistical analyses. The variables were described by frequency, mean and standard deviation (SD). The Chi–square test was used for statistical analysis. P<0.05 was considered significant. We planned this study as a pre-test before starting education related to BFHI. In this study, we aimed to evaluate the knowledge and behavior of mothers related to the initiation time, duration and affecting factors of breastfeeding. 122 Marmara Medical Journal 2006;19(3);121-126 Ruhusen Kutlu, et al Evaluation of initiating, continuing and weaning time of breastfeeding sociodemographic characteristics and perinatal conditions of mothers and infants are shown in Table I. Three mothers refused to breastfeed. The rate of breastfeeding initiating time in the first 1-2 hours after delivery was 78.9 %. 175 babies were aged 6 months and older. In this group, the rate of exclusive breastfeeding for at least 6 months was 37.7 %. The mean weaning time was 10.6 months (SD±5.69). The breastfeeding characteristics of the babies are shown in Table II. RESULTS The mean age of the mothers was 28.96 (SD±6.06). Sixty-one mothers were primiparous and one hundred and eighty-seven mothers delivered their babies in a hospital. All the women were married. Twenty babies were delivered by the help of a midwife in a primary health care center,and seven mothers delivered spontaneously at home. The mean age (month) of the last baby was 20.9 (SD±16,89) and the mean birth weight (g) of the last baby was 3075.2 (SD±399,13). The Table 1: Sociodemographic characteristics and perinatal conditions of mothers and infants Characteristics n % 28 105 47 34 13.1 49.1 22.0 15.8 10 102 60 42 4.7 47.7 28.0 19.6 175 34 5 81.8 15.9 2.3 Consanguinity with her husband To share the same house with a grandparent Presence of handicapped child among close relatives Smoking status of husbands Perinatal conditions 45 55 22 113 21.0 25.7 10.3 52.8 Smoking during pregnancy and lactation Number of prenatal visits during the last pregnancy None 1-3 4-6 7 and ↑ Primiparous Place and type of delivery Home,self-delivery Primary health care center, by a midwife Hospital Delivery by cesarean section 19 8.9 18 65 48 83 61 8.4 30.4 22.4 38.8 28.5 7 20 187 63 3.3 9.3 87.4 29.4 Last child’s characteristics Age (month) 0-6 7-12 13-18 19-24 25 and over 50 50 25 27 62 23.4 23.4 11.7 12.5 29.0 Maternal demographics Age (years) 18-23 24-29 30-35 36 and over Education Illiteracy Primary School Middle and High School University Employment Housewife White-collar Blue-collar worker 123 Marmara Medical Journal 2006;19(3);121-126 Ruhusen Kutlu, et al Evaluation of initiating, continuing and weaning time of breastfeeding The status of breastfeeding initiation after delivery between normal spontaneous delivery and cesarean section was significantly different (X2=32.653, p=0.000) as shown in Table III. In the 6 month or over exclusive breastfeeding group, the prevalence of initiation of breastfeeding within half an hour was higher than in the group who was exclusively breastfed for less than 4 months (p=0.004). There was no significant difference between the educational levels of the mothers and the rate of exclusive breastfeeding for at least 6 months (p=0.159). There was no significant difference between the employment status of the mothers and the rate of exclusive breastfeeding for at least 6 months (p=0.996). In the group of babies aged over 1 year (n=131), the rate of the continuing breastfeeding until the 12th month and breastfeeding combined with a gradually diversified food intake was 38.9%. The rate of using pacifier was significantly lower among children exclusively breast-fed until the sixth month than among the children exclusively breastfed until the fourth month (p=0.001). Table II: The breastfeeding characteristics of the babies Breastfeeding Characteristics Initiation of breastfeeding Within 30 minutes after delivery Within 1-2 hours after delivery Later Interval of breastfeeding Whenever baby wants Specific interval At least 6 months exclusive breastfeeding Breast discomfort or infection Artificial nipple Weaning of breastfeeding (x) 0-5 months 6-11 months 12-17 months 18-23 months 24 months and over Duration of exclusive breastfeeding (month) 1 month 3 months 6 months x n % 85 84 45 39.7 39.2 21.1 193 21 66 79 100 90.2 9.8 37.7 36.9 46.7 32 53 31 10 12 23.2 38.4 22.5 7.2 8.7 172 142 66 98.3 81.1 37.7 current breastfeeding (n=76) were not included Table III: The status of breastfeeding initiation after delivery and kind of delivery Breastfeeding status Within 30 minutes Within 1-2 hours Later Total Normal spontaneous delivery % n 81.2 69 67.9 57 35.6 16 66.4 142 Cesarean section % 16.8 32.2 64.4 33.6 n 16 27 29 72 X2=32.653, p=0.000 was not entirely representative of the Turkish population. The rates of breastfeeding may be considered low, as this study was carried out before BFHI. DISCUSSION Before discussing the results, the limitations of the study must be considered. The cases who applied to pediatric policlinics for any reason within a certain period were included in this study. The research period can be found as short by some. In addition, although quite a close match, the sample Breastfeeding is the best form of nutrition for infants. Family physicians and healthcare workers can have a significant role in the initiating and 124 Marmara Medical Journal 2006;19(3);121-126 Ruhusen Kutlu, et al Evaluation of initiating, continuing and weaning time of breastfeeding maintenance of breastfeeding if they have sufficient knowledge of the benefits of breastfeeding and the necessary clinical management skills or habits. Over one million infants worldwide die every year because they are not breastfed or because they are given other foods too early. Millions of people live in poor health, exposed to preventable diseases and malnutrition. A woman’s ability to feel self confidence and secure with her decision to breastfeed are challenged by her family and friends, the media, and health care providers1,13,15. ratio of breastfeeding until the 12th month combined with a gradually diversified food intake was 38.9 %. Whereas, breastfeeding education, lactation consultation, and other postpartum supports may improve a woman’s chance of starting and continuing to breast-feed her baby.5,11,15 Breast discomfort or infection was seen in 36.9% of all mothers. If the mothers are well-informed this negative rate can decrease. The rate of using an artificial nipple was 46.7 % (n=100). This rate was significantly lower in exclusively breastfed babies for six months, than among babies exclusively breastfed for less than four months (p=0.001). The use of an artificial nipple is an unhealthy habit.14,16 Unfortunately, the nonexclusive breastfeeding group has a tendency to use a pacifier. The rate of breastfeeding initiation within the first 1-2 hours after delivery was 78.9 % and after two hours it was 21.1 %. The rate of breastfeeding initiation within the first 1-2 hours in normal spontaneous delivery significantly showed higher prevalence than in cesarean section cases (X2=32.653, p=0.000). Cesarean delivery (C/S) has a negative effect on early, successful breastfeeding.16,17 We recommend that an attempt be made to initiate lactation before the end of the first hour (and no later than two hours) after the birth following cesarean delivery. Positioning may be more difficult because of postoperative pain after C/S. The lateral decubitus position for breastfeeding might be preferable. Special pillows may also be used to provide support for breastfeeding.16 In our study, most of the mothers were from middle/low educational levels. The educational level and working status did not significantly affect the rate of exclusive breastfeeding for at least 6 months. The rate of smoking during pregnancy and lactation was 8.9 %. This rate was lower than in other countries.20-23 Smoking is an important public health problem. Especially, during pregnancy and lactation, smoking negatively affects both the mother’s and the baby’s health. To prevent and reduce tobacco use in the young and in pregnancy, further research should be carried out to develop more effective smoking cessation programmes.23,24 The rate of breastfeeding whenever the baby wanted was 90.2 %, and the ratio of breastfeeding with certain intervals was 9.8 %. This finding shows similarity to the recommended breastfeeding practices of WHO.11,14 In our study, in the group of babies aged 6 months and older , exclusive breastfeeding rates in the 1st, 3rd and 6th months postpartum were 98.3 %, 81.1 % and 37.7 % respectively. According to Anne Merewood, MA, IBCLC, in 2001, the mean breastfeeding initiation rate in US Baby- Friendly hospitals was 84.8%, compared to a national breastfeeding initiation rate of 69.5%.18 According to Kramer MS. et al, the exclusive breastfeeding rate at 6th month was 7.9% in the Republic of Belarus in 1997.19 Durand M. et al evaluated a training program about breast feeding at the maternity section of Obstetrics, Gynecology and Reproductive Medicine at Grenoble University Hospital Center in 2002. The exclusive breastfeeding rates in a pre-and post sample survey were 14.0% and 28.0% respectively. In the same study, the initiating rate of breastfeeding was 76.0%.1 In our study, this rate was higher. In the group of babies older than 1 year (n=131), the In our country, traditional marriages between relatives are common. In this study, the rate of relativity among couples was 21 %. To share the same house with grandparents is also a traditional custom. This rate was 25.7 %. Having a grandparent in the same house positively supports and encourages this ideal form of nutrition for infants. Human milk is the preferred feeding for all infants, including premature and sick newborns.11 Prenatal support, hospital management and subsequent pediatric and maternal visits are allimportant components of breastfeeding promotion.2,5,15,17 Therefore, health professionals should have the necessary knowledge and skills for managing the different stages of lactation. REFERENCES 1. 125 Durand M, Labarere J, Brunet E, Pons JC. Evaluation of a training program for healthcare professionals about Marmara Medical Journal 2006;19(3);121-126 Ruhusen Kutlu, et al Evaluation of initiating, continuing and weaning time of breastfeeding 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. breastfeeding. Eur J Obstet Gynecol Reprod Biol. 2003; 106:134-138. Moran VH, Bramwell R, Dykes F, Dinwoodie K. An evaluation of skills acquisition on the WHO/UNICEF Breastfeeding Management Course using the prevalidated Breastfeeding Support Skills Tool (BeSST). Midwifery 2000; 16:197-203. Egemen A, Kusin N, Aksit S, Emek M, Kurugol Z. A generally neglected threat in infant nutrition: incorrect preparation of infant formulae. Turk J Pediatr 2002; 44:298-303. Giugliani ER. Breastfeeding in clinical practice. J Pediatr (Rio J) 2000; 76:238-252. Earle S. Factors affecting the initiation of breastfeeding: implications for breastfeeding promotion. Health Promot Int 2002; 17: 205-214. L.Parker. Breastfeeding and cancer prevention. Eur J Cancer 2001; 37: 155-158. Yngve A, Sjostrom M. Breastfeeding determinants and a suggested framework for action in Europe. Public Health Nutr 2001; 4: 729-739. Booth I. Does the duration of breastfeeding matter? BMJ 2001; 322: 625-626. Moreland J, Coombs J. Promoting and supporting breastfeeding. Am Fam Physician 2000; 61: 2103-2104. Gomez-Sanchez M, Canete R, Rodero I, Baeza JE, Avila O. Influence of breastfeeding on mental and psychomotor development. Clin Pediatr (Phila) 2003; 42: 35-42. Rea MF. [Breastfeeding and the use of human milk: what the American Academy of Pediatrics recommends] J Pediatr (Rio J) 1998; 74: 171-172. Baby-Friendly in BC Survey Results. http://www.bcbabyfriendly.ca/bfhi.html. was reached at 10 July 2004 Breastfeeding: Ideal for Infants. http://www.cdc.gov/breastfeeding/compendbabyfriendlywho.htm.was reached at 12 July 2004. 14. Moreland J, Coombs J. Promoting and Supporting Breast-feeding. Am Acad Pediatr 2000; 61: 2093-2100. 15. Deshpande AD, Gazmararian JA. Breastfeeding education and support: association with the decision to breast-feed. Eff Clin Pract 2000; 3: 116-122. 16. Sınusas K, Gaglıardı A. Initial management of breastfeeding. Am Fam Physician 2001; 64: 981-988. 17. de la Torre MJ, Martin-Calama J, Hernandez-Aguilar MT; Spanish Committee on Human Lactation, Spanish Paediatric Association. Breastfeeding in Spain. Public Health Nutr 2001; 4: 1347-1351. 18. Merewood A, Philipp BL, Mehta S, Chamberlain LB. Baby-Friendly Hospital Initiative in the US:Rates, barriers, and associations from the first national survey.(Abstract) Submitted for presentation in the 132nd Annual Meeting (November 6-10, 2004) of APHA. 19. Kramer MS, Chalmers B, Hodnett ED, Sevkovskaya Z, Dzikovich I, Shapiro S, Collet JP, Vanilovich I, Mezen I, Ducruet T, Shishko G, Zubovich V, Mknuik D, et all. Promotion of Breastfeeding Intervention Trial (PROBIT): a randomized trial in the Republic of Belarus. JAMA 2001;285:413-420 20. Balle J, Olofssson MJ, Hilden J. Cannabis and pregnancy. Ugeskr Laeger 1999; 161: 5024-5028. 21. Ebrahim SH, Floyd RL, Merritt RK 2nd, Decoufle P, Holtzman D. Trends in pregnancy-related smoking rates in the United States. 1987-1996. JAMA 2000; 283: 361366. 22. Pagano R, La Vecchia C, Decarli A. Smoking in Italy, 1995. Tumori 1998; 84: 456- 459. 23. Chatenoud L, Chiaffarino F, Parrazini F, Benzi G, Vecchia CL. Letters. Prevalence of smoking among pregnant women is lower in Italy than England. Br Med J 1999; 318: 1012. 24. Altman DG, Wheelis AY, McFarlane M, Lee H, Fortmann SP. The relationship between tobacco access and use among adolescents: a four community study. Soc Sci Med 1999; 48: 759-775. 126 ORIGINAL RESEARCH IDIOPATHIC SUDDEN SENSORINEURAL HEARING LOSS Ufuk Derinsu, Şengül Terlemez, Ferda Akdaş Marmara Üniversitesi Tıp Fakültesi, Odyoloji Bilim Dalı, İstanbul, Türkiye ABSTRACT Objective: The purpose of this study is to present our patients with ISSHL and to assess the shape of the audiogram and the recovery incidence of the patients. Methods: 28 patients who were referred to our clinic under diagnosis as idiopathic sudden sensorineural hearing loss during two years (between 2004 - 2006) were included in the study. Routine audiological evaluation was performed to all patients. Shape of initial audiograms was classified in 3 groups: (1) flat audiogram; (2) upsloping audiogram; (3) downsloping audiogram. Differences in PTA and WDS between pre and post-treatment were calculated to assess the recovery of hearing. Results: In this study, recovery rate was high in each of the three groups. There was no difference in the recovery rate between the groups. There was no relation between recovery and early referral to therapy. The groups were very small for statistical analysis. Conclusion: It is necessary to enlarge the study group to compare the effects of the factors on idiophatic sudden sensorineural hearing loss. Keywords: Idiopathic sudden hearing loss, sudden hearing loss, sensori-neural İDİOPATİK ANİ SENSORİNÖRAL İŞİTME KAYBI ÖZET Amaç: Bu çalışmanın amacı idiopatic ani sensorinöral işitme kayıplı hastalarımızı sunmak ve bu hastaların odyogram şekillerini, iyileşme oranlarını değerlendirmektir. Metot: 2004-2006 yılları arasında idiopatic ani sensorinöral işitme kaybı tanısı alan hastalar çalışmaya alındı. Bütün hastalara rutin odyolojik değerlendirmeler yapıldı. İlk başvurudaki odyogram şekilleri (1) düz odyogram, (2) yüksek frekanslara doğru eğimli odyogram ve (3) alçak frekanslara doğru eğimli odyogram olmak üzere sınıflandırıldı. İşitmedeki düzelmeyi değerlendirmek için tedavi öncesi ve sonrasında saptanan saf ses eşikleri ve ayırdetme puanları arasındaki farklılıklar hesaplandı. Sonuçlar: Bu çalışmada bütün gruplarda iyileşme oranı yüksek bulundu. Gruplar arasında iyileşme oranı açısından farklılık görülmedi. İyileşme ile tedaviye erken başlama olgusu arasında bir ilişki saptanmadı. İstatistiksel analizler için gruplardaki hasta sayıları yetersiz bulundu. Tartışma: İdiyopatik ani sensorinöral işitme kaybına ilişkin özellikleri ve etken faktörleri ortaya koyabilmek için, çalışmanın sürdürülmesine karar verildi. Anahtar Kelimeler: İdiopatik ani işitme kaybı, ani işitme kaybı, sensorinöral generally used to refer to hearing losses of sensorineural origin, which have evolved over a period of a few days2. Sudden hearing loss is known to be causally related to viral infection, circulatory disturbance in the area of the anterior inferior cerebellar artery, acoustic tumor and perilymphatic fistula and so on3,4. As there is no single known etiology for sudden hearing loss, the treatment of this disease is largely empirical and involves a wide variety of therapies5. INTRODUCTION Idiopathic sudden sensorineural hearing loss (ISSHL) is characterized by sudden hearing impairment up to deafness within a short period of time and is possibly associated with vestibular dysfunction, tinnitus and pressure sensation in the affected ear. In a majority of cases, ISSHL is unilateral1. There is no universal consent on the standard definition of sudden sensorineural hearing loss. The term sudden hearing loss is İletişim Bilgileri: Ufuk Derinsu e-mail: uderinsu@yahoo.com Marmara Üniversitesi Tıp Fakültesi, Odyoloji Bilim Dalı, İstanbul, Türkiye 127 Marmara Medical Journal 2006;19(3);127-131 Marmara Medical Journal 2006;19(3);127-131 Ufuk Derinsu, et al. Idiopathic sudden sensorineural hearing loss immitancemeter audiometer. Clinically, sudden sensorineural hearing loss has been seen in association with other autoimmune diseases such as rheumotoid arthritis, inflammatory bowel disease, and polyarteritis nodosa. The improvement in hearing after corticostereoid and immunosuppressive therapy, as well as plasmapheresis, further suggests an autoimmune response as the cause of hearing loss in these conditions6. and Interacoustics AC 40 Tympanometry was performed on all patients to verify the pure sensorineural hearing loss. Tympanograms were accepted as normal, when middle ear pressure was ≥ 75 mm H2O. Air- and bone-conducted pure tone audiometry was performed in the range of 250 Hz to 8000 Hz and 500Hz to 4000 Hz respectively. Speech tests included speech reception thresholds and monosyllabic speech discrimination tests. Marmara University Monosyllabic Word Lists in Turkish were used for evaluating the speech discrimination. Spontaneous complete hearing recovery is reported in The literature 7-10. The likelihood of recovery is influenced by the shape of the audiogram, the age of the patient, and the presence or absence of vertigo2. The purpose of this paper is to present patients with ISSHL and to assess the shape of the audiogram and the recovery incidence of the patients. The shape of initial audiograms was classified in 3 groups: (1) flat audiogram; (2) upsloping audiogram; (3) downsloping audiogram. Eighteen of the patients had flat audiogram, 7 had upsloping and 3 had downsloping audiogram. 2 patients with flat audiogram had bilateral hearing loss. Characteristics of the patients are shown in Table I. METHODS Subjects: Twenty-eight patients who were referred to our clinic diagnosed as having ISSHLfor two years (between 2004 - 2006) were included in the study. Biochemical blood analyses, serological tests and MRI had been performed for all patients before they were referred to our clinic. There was no clearly defined cause for hearing loss in these cases. Table 1: Characteristics of the patients. The age of the patients ranged between 7 and 69 years old. Mean age was 46 years. 19 Patients were female and 9 patients were male. The inclusion criteria were as follows: (1) sudden hearing loss with a history not exceeding 10 days; (2) sensorineural hearing loss for at least three contiguous frequencies, averaging 30 dB or more compared with that for normal hearing. The interval between onset and initiation of therapy ranged from 22 hours to eleven days. All patients had tinnitus at the beginning of the hearing loss. None of them complained of vertigo. Two patients’ hearing losses were bilateral; so 30 ears of 28 patients were included in the study. 13 of them were left ears and 17 ears were right. The degree of hearing loss ranged from mild to profound at the first audiological evaluation. Routine audiological evaluation was carried out in standard soundproof booths (Industrial Acoustic Company), using Interacoustics AZ 7 128 Marmara Medical Journal 2006;19(3);127-131 Ufuk Derinsu, et al. Idiopathic sudden sensorineural hearing loss Table 2: Changes in PTA, SRT and WDS between Pre and Post-Treatment The assessment of hearing recovery was made on the basis of the patient’s impression and audiological criteria. Audiological improvement was defined as a 10 dB decrease at least in puretone average (PTA) or 16% improvement at least in word discrimination score (WDS). PTA was calculated from 0.5, 1, 2 and 4 kHz. The same therapeutic protocol (corticosteroid) was applied by Marmara University Otorhinolaryngology Department for all patients. RESULTS Differences in PTA and WDS between pre and post-treatment were calculated. These data are shown in Table II. Ten of 20 ears of the patients with flat audiogram showed improvement in PTA and 6 of them in WDS (Figure 1). There were positive changes in PTA for one ear, but they were not enough to accept as improvement, whereas SRT had an improvement of 20 dB. Furthermore one ear had an improvement of 35 dB in PTA, but speech tests showed no changes (Table 2). Five patients with upsloping audiogram showed improvement in PTA whereas one of them had an insignificant improvement and one of them showed no change. Although one patient had improvement of 12 dB in PTA, deterioration was observed in SRT, but no change was found for WDS (Figure 2). Improvement in PTA and WDS was observed in two patients with downsloping audiogram while there was no difference in the other patient (Figure 3). Figure 1: Changes in PTA and WDS in Patients with Flat Audiogram 129 Marmara Medical Journal 2006;19(3);127-131 Ufuk Derinsu, et al. Idiopathic sudden sensorineural hearing loss Figure 2: Changes in PTA and WDS in Patients with Upsloping Audiogram Figure 3: Changes in PTA and WDS in Patients with Downsloping Audiogram patient with vertigo in this study. The high recovery rate could be explained by this factor. DISCUSSION In this study, the recovery rate was high in each of the three groups. There was no difference in the recovery rate between the groups. Mattox and Simmons reported that recovery was influenced by the shape of the audiogram9. This finding was not compatible with our study. However, it is necessary to emphasize that the number of subjects in the downsloping and the upsloping groups was very small. Therefore, statistical analysis of the data was not possible. In the literature, vertigo has been accepted as one of the poor prognostic indicators9,12. There was no There were slightly more females than males in this study, unlike to the equal gender distribution in literature11,13. Some studies reported that the duration between onset of the hearing loss and referral to therapy was essential14-16. We did not observe this relationship. Our observation is compatible with Huy and Sauvaget’s findings17. Hearing recovery was obtained in the patient referred 11 days after the onset of hearing loss, whereas there was no 130 Marmara Medical Journal 2006;19(3);127-131 Ufuk Derinsu, et al. Idiopathic sudden sensorineural hearing loss improvement in the patient referred on the same day of the onset of hearing loss. 7. In the data analysis stage, we concluded that our findings can give an idea about ISSHL, but it is necessary to enlarge the study group in order to compare the effects of the factors on ISSHL. 8. 9. 10. REFERENCES 1. 2. 3. 4. 5. 6. 11. Chen CY, Emmerling O, Ilgner J, Westhofen M. Idiopathic sudden sensorineural hearing loss in children. Int J Pediatr Otorhinolaryngol 2005;69:817-821. Cole RR, Jahrsdoerfer R. Sudden hearing loss. Am J Otol 1988;9:211-215. Nakashima T, Tanabe T, Yanagita N, Wakai K, Ohno Y. Risk factors for sudden deafness: a case control study. Aurus Nasus Larynx 1997;24:265-270. Zadeh M H, Storper I.S, Spitzer JB. Diagnosis and treatment of sudden-onset sensorineural hearing loss: A study of 51 patients. Otolaryngol Head Neck Surg 2003;128:93-98. Kroenenberg J. Vasoactive therapy versus plasebo in the treatment of sudden hearing loss: A double-blind clinical study. Laryngoscope 1992;102. Matteson EL Use of methotrexate for autoimmune hearing loss. Ann Otol Rhinol Laryngol 2000;109:710714. 12. 13. 14. 15. 16. 17. 131 Chen CY, Halpin C, Rauch SD. Oral steroid treatment of sudden sensorineural hearing loss: a ten year retrospective analysis. Otol Neurotol 2003;24:728-733. Weinaug P. Spontaneous remission in sudden deafness. HNO 1984;32:346-351. Mattox D E, Simmons FB. Natural history of sudden sensorineural hearing loss. Ann Otol Rhinol Laryngol 1977;86:463-480. SnowJB Jr. Sudden deafness. In: Paparella MM, Shumrick DA, eds. Otolaryngology. Philadelphia: W.B. Saunders, 1980:1757-1766. Mattox DE, Lyles CA.Idiopathic sudden sensorineural hearing loss. Am J Otol 1989; 10:242-247. Psifidis AD, Psillas GK, Daniilidis JCh. Sudden sensorineural hearing loss: Long-term follow-up results. Otolaryngol Head Neck Surg 2006;134:809-815. Shaia FT, Sheehy JL. Sudden sensorineural hearing impairment: A report of 1220 cases. Laryngoscope 1976;86:389-398. Laird N, Wilson WR. Predicting recovery from idiopathic sudden hearing loss. Am J Otolaryngol 1983;4:161-165. Byl FM. Sudden hearing loss: eight years' experience and suggested prognostic table. Laryngoscope 1984;94:647-661. Fetterman B, Saunders J, Luxford W. Prognosis and treatment of sudden hearing loss. Am J Otol 1996;17:529-536. Huy PT, Sauvaget E. Idiopathic sudden sensorineural hearing loss is not an otologic emergency. Otol Neurootol 2005;26:896-902. CASE REPORT RADIONUCLIDE IMAGING IN DIFFERENTIAL DIAGNOSIS OF TORSION AND INFECTIONS OF TESTIS AND EPIDIDYMIS REVISITED 1 Yasemin Şanlı1, Işık Adalet1, Handan Tokmak1, Öner Şanlı2, Orhan Ziylan2, Sema Cantez1 Departments of Nuclear Medicine Medical Faculty of Istanbul, University of Istanbul 2Departments of Urology, Medical Faculty of Istanbul, University of Istanbul ABSTRACT Differential diagnosis of acute scrotum especially with acute epididymis and testicular torsion should be made promptly, as testicular torsion is a true surgical emergency of the highest order. In this manuscript, two demonstrative cases introducing with acute scrotum and diagnosed with scintigraphy and color Doppler ultrasonography were presented to recall the knowledge. Keywords: Testicular torsion, radionuclide imaging, color Doppler ultrasonography RADYONUKLİD GÖRÜNTÜLEME METODU İLE TESTİS TORSİYONU TEŞHİSİ VE TESTİS VE EPİDİDİMİS ENFEKSİYONLARI ÖZET Akut skrotumun ayırıcı tanısı özellikle akut epididimit ve testis torsiyonu için hızla yapılmalıdır. Çünkü testiküler torsiyon gerçek bir cerrahi acildir. Bu makalede akut skrotal ağrısı olan ve tanısında testis sintigrafisi ve Doppler Ultrasonografisi kullanılan iki vakayı sunmayı ve bu konudaki bilgileri gözden geçirmeyi amaçladık. Anahtar Kelimeler: Testis torsiyonu, radynüklid görüntüleme, renkli Doppler Ultrasonografi ultrasonography (CDU) demonstrated an heterogenious right testis with hypoechoic paranchyma. And vascularity was diminised in the body of testis but increased in the peritesticular area. Left testis was completely normal in physical examination and CDU. An immediate testicular scintigraphy was performed due to the insignificant evidence of the testicular torsion on CDU. While patient was in the supine position in the gamma camera, 10 mCi Tc-99 m O4 was injected intravenously and 60 frames for 2 seconds were taken as a dynamic study. After that, statical images were recorded for 10 minutes. Testicular scintigraphy demonstrated an increased activity (perfusion) in the dynamical phase and a hypoactive center with a hyperactive hologram in the late static phase (Figure 1). Prompt scrotal exploration revealed the torsion of right testis with 540º, both testis and epididiymis were purple (necrotic) in color and showed no bleeding or significant change in the color after detorsion. Cord was clamped and orchiectomy was performed (Figure 2). INTRODUCTION Scrotal pain is an urgent condition that Urologists, Pediatricians and Pediatric surgeons frequently encounter and may have various etiologies. However, the most common two causes of this acute symptom are torsion of testicles and acute epididymitis. Differentiation of torsion from infections of testis and epididymis has a great importance because of the major differences in treatment modalities 1 Mostly, physical examination combined with color Doppler ultrasonography or testicular scintigraphy is used in differential diagnosis 2,3. In this manuscript, we aimed to recall the utility of testicular scintigraphy in these two acute conditions using two cases. CASE REPORT Case 1 Twenty two years old white male presenting with right scrotal pain extending to the right inguinal region for 36 hours administered to our outpatient Urology clinic. Physical examination revealed right testicular tenderness and hyperemia in scrotal skin. Assessment with color Doppler İletişim Bilgileri: Yasemin Şanlı e-mail: yaseminsanli75@yahoo.com İstanbul Tıp Fakültesi, Nükleer Tıp AD, Çapa, İstanbul Marmara Medical Journal 2006;19(3);132-134 132 Marmara Medical Journal 2006;19(3);132-134 Yasemin Şanlı, et al. Radionuclide imaging in differential diagnosis of torsion and infections of testis and epididymis revisited Case 2 An eight year old boy presenting with pain and tenderness in the left testicle administered to the outpatient clinic of department of Pediatric Surgery. His CDU revealed an expanded and heterogenious left testis and epididiymis with a slightly increased vascularity. These findings were demonstrating an acute epididimo-orchitis. In order to confirm the diagnosis, testicular scintigraphy was performed. In dynamic phase, the activity was increased in the area suiting to the left testicle compared with the other side. In the static phase, the activity was diffusely increased on the left testis (Figure 3). This image was interpreted as epididymoorchitis. He had received an antibiotherapy regimen for 10 days and his symptoms improved within a few days. Figure 1: Testicular scintigraphy demonstrated an increased activity in the dynamical phase and a hypoactive center with a hyperactive hologram in the late static phaseon the right testis. DISCUSSION Torsion is the twisting of the testis on the spermatic cord, resulting in strangulation of the blood supply and infarction of the testis. The patient usually presents with the sudden onset of pain and swelling of the involved testis. The pain may radiate through groin and abdomen. On physical examination, it is difficult to distinguish testis from epididymis because of localised swelling. For this reason the condition is frequently misdiagnosed as epididymitis. Therefore, imaging of the scrotum is very important for the diagnosis of the specific etiology of the patients presenting with acute scrotum. Prompt differential diagnosis should be made between acute testicular torsion and acute infections of testis and epididimytis, since torsion of the testicle is a true surgical emergency of the highest order. An immediate surgical exploration (and detorsion if needed) should be performed to the patients diagnosed with acute testicular torsion. Irreverible ischemic injury to the testicular paranchyma may begin as soon as 4 hours after occlusion of the cord 4. Figure 2: Scrotal exploration revealed torsion of the testis with 540º, all testis and epididymis were purple in color (arrows). Color duplex ultrasonography has proved to be a valuable tool in the differential diagnosis of epididymis and torsion. This method uses visual color coding of flow velocities in blood vessels superimposed on the grey scale ultrasound to determine increases and decreases. Wilbert and collegues found that CDU had a sensitivity of 82% and specificity of 100% for torsion 5. In addition, the sensitivity and specificity for epididymis were found to be70% and 88% respectively. False-negative CDU imaging in torsion was generally due to partial torsion with some residual blood flow in the testicle and Figure 3: In dynamic phase, the reactivity was increased in the area suiting to the left testicle compared with the other side. In the static phase, the activity was diffusely on the left testis (arrows) 133 Marmara Medical Journal 2006;19(3);132-134 Yasemin Şanlı, et al Radionuclide imaging in differential diagnosis of torsion and infections of testis and epididymis revisited epididymitis. Thus, in this imaging modality, operator should be highly experienced. In our first case, diminished blood flow (not complete loss) was detected. However, specific etiology of the acute scrotum couldn’t be diagnosed as testicular torsion, while the second case was diagnosed accurately as epididymo-orchitis. Radionuclide scintigraphy of the scrotum is the most accurate method of diffrential diagnosis. Levy and collegues found the study to have a positive predictive value of 75%, a sensitivity of 90% and specificity of 89% 6. In the early phase of torsion ( <5 to 7 hours after onset of symptoms) in which there is only a minimal edema or congestion and minimal or no reactive hyperemia in dartos, the angiographic phase of the scintigraphy demonstrates normal or diminished blood flow to the affected side. Static images taken for 5-10 minutes demonstrate a central photopenic defect in the involved hemiscrotum7. Inflammation has not yet occurred in the dartos, therefore no rim of activity is identified in the hemiscrotum. In missed torsion, the angiographic phase of the radionuclide scintigraphy may show normal or increased blood flow to the affected scrotum. Occasionally a nubbin sign may be present. This represents increased blood flow secondary to hyperemia in the dartos. Static images demonstrate a central photopenic defect with a variable degree of reactive hyperemia appearing as a rim of activity surrounding the testicle. The scan is interpreted as missed torsion because testicular viability has been jeopardised. On the other hand, in acute epididymitis, the radionuclide angiogram shows increased perfusion to the affected hemiscrotum. If the inflammation involves only the epididymis, radionuclide activity is seen principally at the superolateral pole of the testis and if inflammation has progressed to involve the entire scrotum (epididymo-orchitis), the entire hemiscrotum will have increased activity when compared with the contralateral side7. Likewise in the second case, diagnosis of left epididymo-orchitis was supported by scintigraphic findings. In conclusion, evidence from literature reveals that sonography and scintigraphy show similar sensitivity for diagnosing testicular torsion. One should keep in mind that, false negatives of sonography which is inherently operator dependent, may be compansated with scintigraphy. REFERENCES 1. 2. 3. 4. 5. 6. 7. 134 Schneck FX, Bellinger MF, Abnormalities of the testes and scrotum and their surgical management. In:Retik AB, Vaughan ED Jr, Wein AJ (eds). Campbell’s Urology. Philedelphia: Saunders 2002: 2353-2394. Nussbaum Blask AR, Bulas D, Shalaby-Rana E, Rushton G, Shao C, Majd M. Color Doppler sonography and scintigraphy of the testis: a prospective, comparative analysis in children with acute scrotal pain. Pediatr Emerg Care 2002;18: 67-71. Wu H, Sun SS, Kao A et al, Comparison of radionuclide imaging and ultrasonography in the differentiation of acute testicular torsion and inflammatory testicular disease. Clin Nuc Med 2002; 27: 490-493. Bartsch G, Frank S, Marberger H, Mikuz G.Testicular torsion: late results with special regard to fertility and endocrine function. J Urol 1980; 124: 375-378. Wilbert DM, Schaerfe CW, Stern WD, Strohmaier WL, Bichler KH Evaluation of the acute scrotum by colorcoded Doppler ultrasonography. J Urol 1993; 149: 14751477. Levy OM, Gittelman MC, Strashun AM, Cohen EL, Fine EJ.Diagnosis of acute testicular torsion using radionuclide scanning. J Urol 1983; 129: 975-977. Beltran MR, Testicular Imaging. In: Henkin RE, Boles MA, Dillehay CL (eds). Nuclear Medicine Vol II. Mosby-Year book, Inc. 1996: 1110-1121. CASE REPORT SPINOCEREBELLAR ATAXIA TYPE 2 IN A TURKISH FAMILY 1 Kadriye Ağan1, Deniz Kutlu1, Nazlı Başak2, Önder Us1, Dilek İnce-Günal1 Marmara University, School of Medicine, Department of Neurology, Istanbul, Türkiye 2Boğaziçi University, Molecular Biology and Genetics, Istanbul, Türkiye ABSTRACT Spinocerebellar ataxias are classified according to the clinical signs, affected neuroanatomical regions and genetic features. Spinocerebellar ataxia type II (SCA 2) is characterized by gait and limb ataxia, dysarthria, ophthalmoplegia, and polyneuropathy . Extrapyramidal system signs and dementia are observed at late clinical stages. SCA 2 is caused by an expanded (CAG) trinucleotide repeat on the chromosome 12 resulting in production of abnormal protein called ataxin-2. Here we report a family who was affected by SCA 2 for three generations. Gait ataxia was the first symptom in all cases, followed by dysarthria and ophthalmoplegia respectively. None of the patients had extrapyramidal signs or cognitive decline. Axonal polyneuropathy was established with EMG in one patient and brain MRI showed pure cerebellar atrophy in all patients. An autosomal dominant cerebellary ataxia was the preliminary diagnosis on the virtue of family history, neurological examination and laboratory and scaning techniques. Genetic studies disclosed a mutation on the SCA 2 locus. Spinocerebellary ataxias are a group of disorders classified according to associating clinical signs and symptoms.Therefore, it is important to establish an accurate clinical classification as it would lead to a clue for the discovery of new gene locuses. Keywords: Ataxia, Spinocerebellar ataxia, Autosomal dominant SPİNOSEREBELLAR ATAKSİ TİP 2 İLE İZLENEN AİLE SUNUMU ÖZET Spinoserebellar ataksiler, ataksiye eşlik eden diğer nöroanatomik yapıların etkilenip etkilenmemesine ve genetik özelliklerine göre sınıflandırılır. Spinoserebellar ataksi tip II (SCA-2) yürüyüş ve ekstremite ataksisi ile başlayan, dizartri, oftalmopleji, periferik sinir tutulumu ile şekillenen geç klinik evrelerde ekstrapiramidal sistem bulguları ve demansında eklendiği bir SCA tipidir. İlk bulguların 3-4. dekadda başladığı SCA-2 12. kromozoma lokalize edilmiş, ataksin-2 genini kodlayan bir trinükleotid tekrar hastalığıdır. Bu yazıda 3 kuşak boyunca etkilenimin olduğu bir aile sunulacaktır. Tüm olgularda yürüyüş ataksisi ile başlayan semptomlara sırası ile dizartri ve oftalmoplejinin eklendiği gözlendi. Piramidal, ekstrapiramidal ve kognitif etkilenim saptanmadı. Bir hastanın EMG’sinde aksonal tutulumun ön planda olduğu polinöropati saptandı. Üç hastada yapılan kranial görüntülemelerde saf serebellar atrofi bulundu. Klinik, radyolojik ve elektrofizyolojik çalışmaların sonucu ve aile öyküsü nedeni otozomal dominant spinoserebellar ataksi düşünülen hastalarda gerçekleştirilen genetik çalışmada SCA-2 lokusunda mutasyon saptandı. Spinoserebellar ataksiler, serebellar bulgulara eşlik eden nörolojik semptomlara göre sınıflanan ve her yeni tanımlanan semptoma göre yeni bir lokusun bulunduğu bir hastalık grubudur. Hastaların klinik bulgularının iyi sınıflandırılması lokusun saptanması için önemlidir. Anahtar Kelimeler: Ataksi, Spinoserebellar ataksi, Otozomal dominant INTRODUCTION SCA 2 is caused by an expanded (CAG) trinucleotide repeat on the chromosome 12 resulting in production of abnormal protein called ataxin-22. The symptoms usually begin in the third or fourth decade of life 2. Spinocerebellar ataxias are classified according to the clinical signs, affected neuroanatomical regions and genetic features. Spinocerebellar ataxia type II (SCA 2) is characterized by gait and limb ataxia, dysarthria, ophtalmoplegia, and polyneuropathy1,2. Extrapyramidal system signs and dementia are observed at late clinical stages. Here we will report a Turkish family who was affected by SCA 2 for three generations. İletişim Bilgileri: Kadriye Ağan e-mail: kagan@marmara.edu.tr Marmara University Faculty of Medicine, Neurology, Istanbul, Türkiye 135 Marmara Medical Journal 2006;19(3);135-138 Marmara Medical Journal 2006;19(3);135-138 Kadriye Ağan, et al. Spinocerebellar ataxia type 2 in a turkish family The second patient (III-1) was a 26 year old son of the index case. He was complaining of head titubation which started approximately a year ago. Neurological examination revealed dysarthria, bilaterally restricted lateral eye movements and limb ataxia. MRI showed cerebellar atrophy (Figure 3) CASE REPORT The index case (II-1) was a 49 year old female. Her symptoms began in the fourth decade of life with dysequilibrium and dysarthria respectively. Two years later clumsiness in fine finger movements and writing has been developed. The family history is depicted in Figure 1. Her minimental status test score was 29/30. Neurological examination revealed dysarthria, bilaterally restricted upward and lateral eye movements, limb and gait ataxia. Magnetic resonance imaging (MRI) showed cerebellar atrophy (Figure 2). Electrophysiological studies revealed axonal sensory polyneuropathy. The brother (II-3) of the index case was a 47 year old man. He experienced dysequilibrium which began in the fourth decade. The mother (I-4) of the index case was 69 years old. Her history of dysequilibrium and explosive speech began in the sixth decade. MRI of this case showed cerebellar atrophy, too. Figure 1: Family history Figure 3: MRI shows cerebellar atrophy Figure 2: MRI shows cerebellar atrophy 136 Marmara Medical Journal 2006;19(3);135-138 Kadriye Ağan, et al. Spinocerebellar ataxia type 2 in a turkish family type 2 was confirmed by genetic study. One patient (III-1) showed head titubation, which was rarely reported in the literature7 . Acquired vitamin E deficiency, vitamin B 12 deficiency, hypothyroidism, other familial and neurodegenerative diseases (multi-system atrophy, cerebellary malformations, stroke, multiple sclerosis, psychogenic disorders, CreutzfeldtJakob Disease, Behçet’s Disease) were excluded by neurologic examination, laboratory and scanning techniques. The SCAs show anticipation and different degrees of expansion in maternal or paternal transmission.7. There is a negative correlation between CAG repeat size and age of disease onset, therefore young patients suffer from more severe and rapidly progressing disease8. DNA analysis showed a normal CAG repeat of 23 on one chromosome and abnormally amplified CAG repeat of over 41 on the other chromosome in index case and her son III-1. Blood samples from the mother and brother of the index case were not available. Therefore, the degree of anticipation and the existence of paternal imprinting were not analyzed. The widest range of age onset is observed in individuals with allele numbers between 38-45 CAG repeats9 . The family showed autosomal dominant inheritance and anticipation resulting in onset of symptoms, in the latest generation, in the third decade; earlier generations had onset in the fifth or sixth decade. The testing showed a normal CAG repeat of 23 on one chromosome and abnormally amplified CAG repeat of over 41 on the other chromosome in index case and her son III-1. Genetic studies disclosed an autosomal dominant cerebellary ataxia, SCA 2. DISCUSSION Testing for dominant ataxias should be included in the evaluation of patients with ataxia, especially in cases with a positive family history for spinocerebellar ataxia. New gene locuses which are linked to SCA‘s, are discovered every other day. Genetic study is significant in defining SCA types which are common in our country. Spinocerebellar ataxia (SCA) type 2 is a CAG trinucleotide repeat disorder characterized by progressive ataxia. In 1982, Harding classified autosomal dominant cerebellar ataxias (ADCA) into three types1. ADCA I is characterized by ataxia with signs of neurodegeneration outside the cerebellum including pyramidal and extrapyramidal involvement, ophthalmoplegia, peripheral neuropathy, and dementia1. ADCA II represents ataxia with extracerebellar neurologic findings, plus retinal degeneration. ADCA III is a pure form of ataxia and degeneration restricted to the cerebellum1. SCA – 1, 2, 3 and 6 fit into the ADCA I clinical category1. SCA 2, 15% (13-40%) is the second common form of ADCA I and characterized by slowly progressive ataxia and dysarthria associated with the ocular findings of nystagmus, slow saccadic eye movements, and in some individuals ophthalmoparesis3,4. We describe a family with ataxia, dysarthria, ophthalmoplegia and peripheral neuropathy. There are studies which revealed dementia, Ldopa responsive Parkinsonism, fasciculations, dystonia, and/or chorea at SCA 2 patients5. The Parkinsonian phenotype is associated predominantly with a shorter abnormal range of CAG repeat lengths and older onset age6. In the present report all of the four patients had cerebellar dysfunction. Most patients began with dysarthria and gait ataxia. As the disease progressed, limb ataxia became more pronounced. Family members who were examined showed ophthalmoparesis and peripheral nerve involvement, and the clinical suspicion of SCA REFERENCES 1. 2. 3. 4. 5. 6. 7. 137 Harding AE. The clinical features and classification of the late onset autosomal dominant cerebellar ataxias. A study of 11 families, including descendants of the ‘the Drew family of Walworth’. Brain 1982; 105: 1–28. Pulst SM, Nechiporuk A, Nechiporuk T, et al. Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. Nat Genet. 1996; 14: 269–276. Geschwind DH, Perlman S, Figueroa C P, Treiman L J, Pulst S M. The prevalence and wide clinical spectrum of the spinocerebellar ataxia type 2 trinucleotide repeat in patients with autosomal dominant cerebellar ataxia. Am J.Hum Genet 1997; 60: 842-850. Cancel G, Durr A, Didierjean O, et al. Molecular and clinical correlations in spinocerebellar ataxia 2: a study of 32 families. Hum Mol Genet 1997; 6:709-715. Belal S, Cancel G, Stevanin G, et al. Clinical and genetic analysis of a Tunisian family with autosomal dominant cerebellar ataxia type 1 linked to the SCA2 locus. Neurology 1994; 44: 1423-1426. Lu CS, Wu Chou YH, Kuo PC, Chang HC, Weng YH. The Parkinsonian phenotype of spinocerebellar ataxia type 2. Arch Neurol 2004; 61:35-38. Kim JM, Shin S, Kim JY, et al. Spinocerebellar ataxia type 2 in seven Korean families: CAG trinucleotide expansion and clinical characteristics. J Korean Med Sci 1999; 14:659-664. Marmara Medical Journal 2006;19(3);135-138 Kadriye Ağan, et al. Spinocerebellar ataxia type 2 in a turkish family 8. Babovic-Vuksanovic D, Snow K, Patterson M C, Michels V V. Spinocerebellar ataxia type 2 (SCA 2) in an infant with extreme CAG repeat expansion. Am J.Med Genet 1998; 79: 383-387. 9. 138 Giunti P, Sabbadini G, Sweeney MG, Davis MB, Veneziano L, Mantuano E, Federico A, Plasmati R, Frontali M , Wood NW. The role of the SCA2 trinucleotide repeat expansion in 89 autosomal dominant cerebellar ataxia families. Frequency, clinical and genetic correlates. Brain 1998; 121: 459–467. CASE REPORT AN UNUSUAL CASE OF MULTIFOCAL MOTOR NEUROPATHY WITH CRANIAL NERVE INVOLVEMENT AND HYPERREFLEXIA Hande Türker, Oytun Bayrak, Levent Güngör, Murat Sarıca, Musa Onar Ondokuzmayıs University, Faculty of Medicine,Department of Neurology, Samsun, Türkiye ABSTRACT Multifocal motor neuropathy is characterized by progressive, asymmetric weakness of the limbs with persistent conduction blocks (CB). Sensory loss is very rare and it also rarely presents with cranial nerve involvement and hyperreflexia. Here we described a 32-year-old woman with progressive weakness of hand muscles associated with weakness of orbicularis oculi muscles and fasciculations of tongue. The electrophysiological examination revealed persistent conduction blocks in both of the ulnar, right median and left posterior tibial nerves together with F-waves with abnormal persistence. These findings and the persistency of conduction blocks along with a response to IVIG made it likely that the diagnosis was multifocal motor neuropathy. The case seemed to be worth reporting because of her unusual clinical findings. Keywords: Multifocal motor neuropathy, Cranial nerve involvement, Hyperreflexia KRANİAL SİNİR TUTULUMU VE HİPERREFLEKSİ İLE GİDEN MULTİFOKAL MOTOR NÖROPATİ: OLGU SUNUMU ÖZET Multifokal motor nöropati (MMN), kalıcı iletim bloklarının eşlik ettiği, ilerleyici, asimetrik ekstremite güçsüzlüğüyle karakterizedir. Duysal etkilenim çok seyrek olarak görülür. MMN’nin kranial sinir tutulumu ve refleks artışı ile birlikteliği nadiren bildirilmiştir. Burada, el kaslarındaki ilerleyici güçsüzlüğe, orbikularis okuli kaslarında güçsüzlük ve dilde fasikülasyonun eşlik ettiği 32 yaşında MMN’li bir kadın hasta sunulmuştur. Elektrofizyolojik incelemede her iki ulnar, sağ median ve sol posterior tibial sinirlerde iletim blokları ve F dalgalarında persistans anormalliği saptandı. Bu bulgularla birlikte, ileti bloklarının persistansı ve tablonun İVİG’e yanıtlı olması bize multifokal motor nöropati tanısını düşündürttü. Hasta, kliniğindeki seyrek rastlanır öğeler nedeniyle rapor edilmeye değer bulundu. Anahtar Kelimeler: Multifokal motor nöropati, Kranial sinir tutulumu, Refleks artışı cranial nerve symptoms4,5. In this report, we describe a case of MMN with hyperreflexia and cranial nerve symptoms and we want to emphasize that the lesion distribution may be more widespread than it is considered. INTRODUCTION Multifocal motor neuropathy (MMN) is characterized by slowly progressive, asymmetrical weakness of the limbs without sensory loss. The upper extremities are often affected in the first place and distal muscles are affected more than proximal muscles. On electrophysiological examination there may be evidence of demyelination and persistent conduction blocks (CB)1-3. Differentiation of MMN from motor neuron disease is important because MMN is a treatable disorder. CASE REPORT A 32-year-old woman was admitted to our department because of the progressive weakness of her hand muscles. Her complaints had started in her left hand, one year before her admittance and had progressed to involve her right hand 4 months later. Two prior electromyograms, the first 10 months and the second 5 months prior to admission, were reported as carpal tunnel syndrome and C8-T1 root involvement. She had Upper motor neuron signs and cranial nerve involvement are usually absent1 although MMN is rarely reported presenting with hyperreflexia and İletişim Bilgileri: Hande Türker e-mail: drhande@ttnet.net.tr Ondokuzmayıs University, Faculty of Medicine, Department of Neurology, Samsun, Türkiye Marmara Medical Journal 2006;19(3);139-144 139 Marmara Medical Journal 2006;19(3);139-144 Hande Türker, et al An unusual case of multifocal motor neuropathy with cranial nerve involvement and hyperreflexia had surgery on her left hand but her symptoms continued to progress. Her cervical computerized tomography (CT) revealed only a C5-6 posterior central bulging. On neurological examination the orbicularis oculi muscles were bilaterally weak and there were fasciculations on her tongue. The muscle strength of the thenar abductors and hypothenar abductors were 2/5, wrist extensors, elbow extensors and elbow flexors were 4/5, wrist flexors were 3/5, and arm abductors were 3/5 bilaterally (According to MRC). She had a claw finger deformity of the second digit of her left hand (Fig. 1). The strength of the ankle dorsalflexors, plantar-flexors, knee extensors and knee flexors were 5/5 bilaterally and hip flexors were 4/5 on the left, 5/5 on the right side. Her deep tendon reflexes were increased. The sensory examinations were normal. Plantar responses were flexor. She did not have any other systemic diseases or history of usage of any drugs. Laboratory examinations including blood count, renal and liver function tests, thyroid function tests and erythrocyte sedimentation rate were all within normal limits. Antiganglioside antibodies (anti GM1 Ab) were negative. Electrophysiological studies: The electrophysiological studies including nerve conduction studies (NCs) and needle electromyography (EMG) were performed on Nihon Kohden Neuropack 8 at our neurophysiology laboratory. The patient’s skin temperature was kept between 31-32ºC. least 40% or 50% reduction of proximal versus distal CMAP amplitude with minimal dispersion (less than 30 % increase in CMAP duration) or at least 50% or 60% reduction of amplitude with moderate dispersion (31-60 % increase in CMAP duration)6. Median, ulnar and sural sensory nerve action potentials (SNAPs) were obtained antidromically, recording from digit III for median and from digit V for ulnar nerves. Fig. 1: Claw finger deformity of left hand Table I and II show the results of the NCs. The median, ulnar and sural SNAPs were all in normal limits bilaterally. On the left side the amplitudes of the median and ulnar CMAPs were reduced and CMAPs could not be recorded at the Erb’s point although the stimulus was supramaximal (0.1-0.2 ms duration at 50 mA) and the recordings were repeated. There was an amplitude reduction of 64 % in the ulnar nerve at the axilla and prolongation of duration was lower than 30 %. The distal latency of the ulnar CMAP was normal and the latency of the median CMAP was prolonged. Ulnar CV was normal. Persistences of F-waves of the ulnar and median nerves were reduced. The latency and the amplitude of the peroneal CMAP were normal but persistence of F-waves was again reduced. The latency and the amplitude of the posterior tibial CMAP and the persistence of Fwaves were normal. Peroneal and posterior tibial CVs were normal. There was a reduction of CMAP amplitude of the posterior tibial nerve by 74 % at the popliteal fossa. According to the consensus criteria for the diagnosis of partial conduction block, the tibial nerve may only have probable partial conduction block and amplitude reduction should be over 50 percent when there is minimal temporal dispersion (duration increased by 30% or less). Our patient’s NCs of posterior tibial nerve fulfilled this condition. Nerve conduction studies: Median, ulnar, peroneal and posterior tibial motor NCs including F-waves were performed bilaterally. NCs were performed comprising the stimulation of the Erb’s point and the popliteal fossa for median and ulnar nerves and peroneal and posterior tibial nerves respectively. For compound muscle action potentials (CMAPs), we measured the latency, amplitude and motor conduction velocity (CV). We also measured amplitude reduction and percentage of prolongation of duration on proximal versus distal stimulation. The American Association of Electrodiagnostic Medicine has proposed the criteria for the definition of definite and probable CB as follows: a) Definite CB: Presence of at least 50% or 60% reduction of proximal versus distal CMAP amplitude in the nerves of the upper and lower extremities with minimal temporal dispersion (less than 30 % increase in CMAP duration). b) Probable CB: presence of either at 140 Marmara Medical Journal 2006;19(3);139-144 Hande Türker, et al. An unusual case of multifocal motor neuropathy with cranial nerve involvement and hyperreflexia Table I: Motor nerve conduction studies of the patient Side Nerve Recording Latency N Amplitude N Duration (mv-µV) NCV N (m/s) F N min. (mv-µV) latenc y Left* Ulnar 2.7 <3.2 4 elbow 6.8 <7.0 above 8.0 <10.3 ADM >6.0 5.5 54.3 4 5.1 76.9 3.9 4.5 76 1.4 6.4 wrist >50. 26 6 4/16 >50. 35 6 6/16 >47. 28 <27. 3 7/16 7 >47. 29.6 <27. <28 elbow DPCB Right* axilla 9.7 DPCB erb No CMAP at erb Ulnar ADM 3.5 <3.2 4.2 elbow 7.5 <7.0 above 9.3 >6.0 7.0 50.5 3.4 6.3 59.1 <10.3 1.4 6.2 >80 1.2 6.9 wrist DPCB <28 elbow Left* DPCB axilla 10.2 DPCB erb No CMAP at erb Median APB 4.8 <3.6 0.7 elbow 8.3 <7.8 axilla 11.1 erb No CMAP at erb APB 4.1 <3.6 1 >6.0 4.3 57 0.7 4.6 61 0.7 5.0 wrist Right* Median >6.0 4.6 31 3 wrist DPCB Left Peroneal elbow 10.2 1 4.6 90 axilla 11.5 <7.8 1 5.0 38 erb 13.0 0.5 4.5 EDB 4.2 <5.1 3 >2 5.7 53 >41 ankle Right Peroneal cap. fib. 10.2 3 6.6 poplitea 10.9 3 6.9 EDB 3.4 <5.1 4 >2 7 42.9 <63. 4/16 4 42 <63. 90 5.8 42 90 <41 ankle Left* Post.tibial 4 cap. fib. 9.5 4 6.7 poplitea 10.2 4 7.0 AHL 4.2 <5.0 13.7 >3 6.6 57.8 >38 48.8 ankle Right 6 PPCB poplitea 10.0 Post.tibial AHL 3.6 6.9 3.5 <5.0 6.7 >3 6.1 ankle poplitea <55. 40.2 >38 46 <55. 6 12.3 4.8 141 4.8 Marmara Medical Journal 2006;19(3);139-144 Hande Türker, et al An unusual case of multifocal motor neuropathy with cranial nerve involvement and hyperreflexia Table II: Sensory nerve conduction studies of the patient Side Nerve Recording Latency N Amplitude N (mv-µV) Distance NCV (cm) (m/s) N (MvµV) Left Ulnar Sensory 2.7 <3.6 65 µV >10.0 12 44 >39 Sensory 3.2 <3.5 90 µV >16.0 14 43 >40 Sensory 2.7 <3.6 87 µV >11.0 12 43 >40 Sensory 3.4 <3.5 52 µV >10.0 14 40 >40 5th finger Left Median 3rd finger Right Ulnar 5th finger Right Median 3rd finger Left Sural Sensory 3.0 <4 20 >9 14 46 >32 Right Sural Sensory 2.9 <4 22 >9 14 47 >32 Footnotes: Abbreviations for both tables APB: Abductor pollicis brevis NCV: Nerve conduction velocity EDB: Extensor digitorum brevis ADM: Abductor digiti minimi Min. latency: Minimal latency AHL: Adductor hallucis longus N: Normal value (According to our lab. normals in the same age group) cap.fib: Caputulum fibulum mv: milivolt DPCB: Definite partial conduction block PPCB: Probable partial conduction block * Pathologic values are written in bold fasciculation potentials and positive sharp waves at rest. We found a moderate neurogenic involvement of orbicularis oculi, sternocleidomastoid and lingual muscles. The latency of the right ulnar CMAP was prolonged, the amplitudes were reduced and an amplitude reduction of 58 % was found at the axilla (Fig. 2). The persistence of F-waves was reduced. The latency of the median CMAP was prolonged, the amplitudes and the CV were reduced and an amplitude reduction of 50 % was found at the Erb’s point. The minimal F-wave latency was prolonged. The latencies, amplitudes and CVs of the peroneal and posterior tibial nerves together with the minimal latencies and the persistence of F-waves were normal. Needle EMG: Tibialis anterior, extensor digitorum communis, first dorsal interosseous, deltoid, biceps brachii, genioglossus and lingual muscles were examined on the left side whereas deltoid, extensor digitorum communis, first dorsal interosseous, orbicularis oculi and sternocleidomastoid muscles were examined on the right. All extremity muscles that were examined, except tibialis anterior, showed a severe neurogenic involvement with reduced recruitment of polyphasic, large amplitude, long duration motor unit potentials with a moderate number of fibrillation and Fig. 2: Motor nerve conduction study of right ulnar nerve. There is definite conduction block at the axillary and Erb stimulation points 142 Marmara Medical Journal 2006;19(3);139-144 Hande Türker, et al An unusual case of multifocal motor neuropathy with cranial nerve involvement and hyperreflexia The asymmetrical onset of weakness of distal hand muscles in a young patient together with the conduction blocks in proximal segments in NCs made the diagnosis of MMN very likely although there was cranial nerve involvement and hyperreflexia. The patient was given 0.4g/kg/day intravenous immunoglobulins (IVIG) for 7 days. Three weeks after the administration of IVIG, conduction block in the left ulnar nerve quite improved although improvement of muscle weakness was slight (the muscle strength of the thenar abductors and hypothenar abductors became 3/5, wrist extensors, elbow extensors and elbow flexors were 4/5, wrist flexors were 3/5, and arm abductors were 4/5 bilaterally, according to MRC, in the follow-up examination). We planned to continue IVIG administration once a month for a year and the patient was admitted to the physical rehabilitation program. indicated that corticospinal tract was at least functionally involved in some patients with MMN showing hyperreflexia5. Our patient was negative for anti Gm1 Ab. Although other antiganglioside antibodies such as antiGD1b were not sought for, a diagnosis of MMN does not require that every patient should have these antibodies. About one half of MMN patients lack elevated levels of these antibodies and many others have only modest elevations, to a degree often seen in other neurological and even non-neurological disorders9. The electrophysiological examination of our patient revealed CBs in proximal segments with reduction in the persistence of the F-waves. However, in our patient, the amplitudes of the median CMAPs were significantly reduced. This progression could have resulted because of distal conduction failure leading to axonal degeneration. Similar cases in which initially reduced CMAPs were found without major indication of demyelination were reported. The authors of these reports concluded that the conduction blocks could have resulted from immune mediated conduction failure at the nodes of Ranvier without underlying demyelination in the cases10-12. There are also patients of MMN in whom CB may decrease or even disappear after several years of the disease because of a progressive reduction of the distal CMAP amplitude, which may reflect either secondary axonal degeneration or the appearance of previously unrecognized very distal CB13. Our patient may also have a similar situation. DISCUSSION The clinical diagnosis of MMN is based on the presence of chronic and progressive asymmetrical limb weakness with a multineuropathic distribution pattern affecting the muscles of at least two different motor nerves and lasting for at least two months. There is also minimal or no sensory loss. Absence of upper motor neuron involvement is almost a rule7. Our case had distal, progressive weakness in her hand muscles with an asymmetrical onset for approximately one year. Her neurological examination revealed muscle weakness in the distribution of more than two different motor nerves besides hyperreflexia and cranial nerve involvement. Axelsson and Liedholm reported a similar case to ours, diagnosed as MMN with cranial nerve symptoms: an old man with a slight atrophy in the hands and right-sided hypoglossal palsy4. They suspected motor neuron disease at first, but the electrophysiological studies revealed bilateral ulnar CBs indicative of MMN. They treated the patient with IVIG. Leon et al reported a case of MMN with an abnormal blink reflex8. Their findings allowed them to suggest that the distribution of involvement pattern in MMN seemed to be more widespread than generally considered. Motor conduction velocities are usually normal or slightly reduced outside areas of CB and are sometimes normal even at the level of segments of CB or adjacent segments confirming the predominantly focal nature of the demyelination13. This situation also takes place in our case and it is noteworthy that it did not happen because of the technical artifacts. The clinical presentation of our case and electrophysiological findings as CBs in both ulnar nerves, right median and left posterior tibial nerves with reduction in the persistence of the Fwaves together with the response to IVIG therapy were thought to be consistent with MMN. In our patient the progression of the disease took a year. Such acute or subacute progression is unusual but cases similar to ours were also reported11. There are 9 cases reported in the literature as acute multifocal motor neuropathy (AMMN) and the Oshima et al. investigated patients with hyperreflexia who exhibited paralysis with CB. To determine whether hyperreflexia corresponded to corticospinal tract dysfunction, they evaluated central motor conduction time with magnetic stimulation in these patients. Their findings 143 Marmara Medical Journal 2006;19(3);139-144 Hande Türker, et al An unusual case of multifocal motor neuropathy with cranial nerve involvement and hyperreflexia pathophysiological mechanisms of AMMN probably overlap with those of chronic MMN11. The differential diagnosis of MMN from motor neuron disease is important because MMN is a treatable disorder. There may still be some patients in whom the differential diagnosis of MMN from motor neuron disease is quite hard. The literature contains some examples of these patients. Parry et al reported 5 cases of MMN who had muscle atrophy, cramps and fasciculations with preserved reflexes. Although the clinical picture of these cases led to an initial diagnosis of motor neuron disease, nerve conduction studies revealed multifocal CBs predominantly involving proximal nerve segments14. Kaji et al reported 2 patients with MMN whose clinical signs included atrophy of the tongue and limb muscles closely resembling that of motor neuron disease and nerve conduction studies revealed multifocal CBs without sensory abnormalities15. However the classical motor neuron disease presents with needle EMG findings, which cannot be seen in MMN. The most important of these findings are the reinnervation motor unit action potentials seen together with chronic denervated motor unit action potentials and this provides evidence for a continuous degeneration and regeneration pattern of involvement16. Our patient lacked these EMG findings. Her age and sex, the neurological examination findings and the clinical progression which were related with a multineuropathic distribution pattern together with the response to IVIG therapy were not found consistent with motor neuron disease. Nevertheless, it can still be debated that the definite diagnosis of our patient may become more clear in the upcoming followups. We emphasize that in patients with distal, progressive asymmetrical weakness, MMN should be kept in mind even if they have cranial nerve involvement and hyperreflexia. A detailed electrophysiological examination should also be performed for detection of CBs in proximal segments of the nerves, especially at the upper extremities. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. REFERENCES 1. Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2nd ed. Philadelphia: Elsevier, 2005: 389-420. 144 Biessels GJ, Franssen H, van den Berg LH, et al. Multifocal motor neuropathy. J Neurol 1997; 244:143152. Nobile- Orazio E. Multifocal motor neuropathy. J Neuroroimmunol 2000; 115:4-18. Axelsson G, Liedholm LJ. Multifocal motor neuropathyunusual cause of hypoglossal palsy. Lakartidningen 2002; 99:1448-1450. Oshima Y, Mitsui T, Yoshino H, et al. Central motor conduction in patients with anti-ganglioside antibody associated neuropathy syndromes and hyperreflexia. J Neurol Neurosurg Psychiatry 2002; 73: 568-573. Olney RK. Consensus criteria for the diagnosis of partial conduction block. Muscle Nerve 1999; 22: 225-229. Nobile-Orazio E, Cappellari A, Meucci N, et al. Multifocal motor neuropathy: clinical and immunological features and response to IVIG in relation to the presence and degree of motor conduction block. J Neurol Neurosurg Psychiatry 2002; 72:761-766. Leon-S FE, Pradilla G, Chavez AM, DeLeon MA, Rueda OL, Bayona J. Contralateral early blink reflex in multifocal motor neuropathy. Electromyogr Clin Neurophysiol 1998; 38:419-422. Parry GJ. Antiganglioside antibodies do not necessarily play a role in multifocal motor neuropathy. Muscle Nerve 1994; 17:97-99. Caposso M, Caporale CM, Pomilio F, Gandolfi P, Lugaresi A, Uncini A. Acute motor conduction block neuropathy. Another Guillian- Barre syndrome variant. Neurology 2003; 61: 617-622. Lefaucheur JP, Gregson NA, Gray I, von Raison F, Bertocchi M, Creange A. A variant of multifocal motor neuropathy with acute, generalized presentation and persistent conduction blocks. J Neurol Neurosurg Psychiatry 2003; 74: 1555-1561. Rajabally YA, Strens LHA, Abbott RJ. Acute motor conduction block neuropathy followed by axonal degeneration and poor recovery. Neurology 2006; 2: 287-288. Orazio EN, Terenghi F. Other dysimmune neuropathies. peripheral neuropathy. American Academy of Neurology Continuum. 2003; 9:70-76. Parry GJ, Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle and Nerve. 1988; 11: 103-107. Kaji R, Shibasaki H, Kimura J. Multifocal demyelinating motor neuropathy: cranial nerve involvement and immunoglobulin therapy. Neurology 1992; 42: 506-509. Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2nd ed. Philadelphia: Elsevier, 2005: 423-437. CASE REPORT NON-FUNCTIONING KIDNEY RESULTED FROM PRIMARY HYDATID CYST OF THE PSOAS MUSCLE Engin Kandıralı, Atilla Semerciöz, Ahmet Metin, Muzaffer Eroglu, Bülent Uysal Abant İzzet Baysal Üniversitesi, İzzet Baysal Tıp Fakültesi, Üroloji Anabilim Dalı, Bolu, Türkiye ABSTRACT Hydatid cyst is rarely located in the psoas muscle. Herein, we report a case with non-functioning kidney resulted from a primary hydatid cyst located at the psoas muscle obstructing upper segment of the ureter. Keywords: Psoas muscle; Kidney; Hydatid cyst; Treatment PRİMER PSOAS KİST HİDATİĞİ SONUCU OLUŞAN NONFONKSİYONE BÖBREK ÖZET Psoas kasında nadiren kist hidatik görülür. Bu vakada primer psoas kası kist hidatiği sonucu oluşmuş nonfonksiyone böbrek vakası bildirilmiştir. Anahtar Kelimeler: Psoas kası, Böbrek, Hidatik kist, Tedavi INTRODUCTION Hydatid cyst is a parasitic infection prevalent in most sheep raising in the Mediterranean countries. Primary cyst may localize anywhere in the body but commonly they are found in the liver (5570%) and lungs (20-30%), location in the muscular tissue accounts for 2-3 % of all cases1. Herein, we report a case with non-functioning kidney resulted from a primary hydatid cyst located at the psoas muscle obstructing upper segment of the ureter. CASE REPORT A 42-year-old woman presented with right loin pain for 2 years. She did not complain of any voiding symptoms and history was unremarkable. Physical examination was normal. Ultrasonography revealed a well defined right retroperitoneal cystic lesion adjacent to the right hydronephrotic kidney. Intravenous urogram showed a non-functioning right kidney and normal left kidney (Fig. 1). Computerized tomography revealed a cystic formation having vesicles with well defined wall suggestive of hydatid disease in the right retroperitoneum (Fig. 2). Also right ureter was compressed by cystic lesion. Imaging studies detected no other cyst anywhere in the body. Serological studies were normal. Fig. 1: Intravenous urogram shows a nonfunctioning right kidney. Medical therapy with oral albendazole (10mg/kg/day) was administered 7 days before surgery. Right flank incision was made and a cystic lesion obstructing right ureter was observed. After the operative field was protected with swabs soaked with 3% cetrimide, the area around the cyst was incised and the cyst content, İletişim Bilgileri: Engin Kandıralı e-mail: ekandirali@yahoo.com Abant İzzet Baysal Üniversitesi, İzzet Baysal Tıp Fakültesi, Üroloji AD, Bolu, Türkiye 145 Marmara Medical Journal 2006;19(3);145-146 Marmara Medical Journal 2005;19(3);145-146 Engin Kandıralı, et al Non-functioning kidney resulted from primary hydatid cyst of the psoas muscle composed of numerous daughter cysts and a water-like fluid with debris, was evacuated. Drainage was followed by partial cystectomy and simple right nephrectomy of the non-functioning kidney. A prophylactic large-caliber passive tube drain was placed in the cyst cavity and removed at the sixth postoperative day. Postoperative stay was uneventful and the patient was discharged 8 days after surgery with continuous albendazole therapy for 6 months. No recurrence of hydatid disease was observed by the same examinations done after 1 year follow up period. Fig. 2: CT shows a cystic formation having vesicles with well defined wall suggestive of hydatid cyst adjacent to the right hydronephrotic kidney. DISCUSSION Hydatid cyst may occur in any part of the body. When the hydatid cyst located in the muscle tissue, the diagnosis of hydatid cyst may be difficult and late, because this localization is unexpected and the cyst is asymptomatic until it enlarges and compresses adjacent organs. Although skin and serological tests (i.e. specific antibodies titres) are widely used to confirm diagnosis, they are often negative because the capsule isolates the parasite from the host’s immune system. Hydatid cyst is rarely located in the psoas muscle. If it does not compress the kidney, ureter or vertebra, it is generally asymptomatic and may be diagnosed incidentally. Radiologic investigations (particularly ultrasonography and CT) are used to establish the diagnosis when daughter cysts are identified and are very helpful in determining the extent of the disease. Recently, magnetic resonance imaging (MRI) has been gaining popularity for investigating hydatid disease involving soft tissues2. Cystic or complex retroperitoneal tumor, pyogenic abscess of the psoas and even tuberculosis must be considered in the differential diagnosis of retroperitoneal hydatid cyst3. Although the diagnosis of hydatid disease is mainly clinical and radiologic, clinic suspicion is the most important factor for its diagnosis. After preoperative diagnosis of hydatid cyst is confirmed, adequate surgical excision of cyst, and sterilization of the cavity with scolocidal agents (such as hypertonic saline, cetrimide, hydrogen peroxide) for preventing the seeding should be done. Percutaneous drainage and alcoholization could be used to reduce the size of the cyst and sterilizing, but Melis et. al. reported a case with no significant changes within the size of the cyst after this procedure1. Medical therapy with mebendazole or albendazole is used as prophylaxis and postoperatively to prevent recurrences. Preoperative medical therapy should be initiated at least 4 days before the operative procedure and continued for at least a month or preferably several months postoperatively4,5. Extraperitoneal approach is preferred in order to avoid intraperitoneal dissemination and partial cystectomy, simple nephrectomy, and combined with medical therapy was the treatment choice in our case. Total cystectomy is not required in the muscular hydatid disease, because disease is diffuse in the muscle3. Angulo et al. recommended irrigation of the cavity with scolocidal agents via drain tube for several weeks following surgery and some authors did not advise postoperative irrigation1,3. In summary, the case presented here is notable for two reasons; one is the rarity of it and the other is that of its compression leading to a non-functional kidney. REFERENCES 1. 2. 3. 4. 5. 146 Melis M, Marongiu L, Scintu F et al. Primary hidatid cysts of psoas muscle. ANZ J Surg 2002; 27: 443-445. Claikens B, Van Hoe L, Vansteenbergen W. Images in clinical radiology. MRI features of hydatid disease. JBR-BTR 1999; 82:167. Angulo JC, Granell J, Muguerza J, Sanchez-Chapado M. Primary bilateral hydatidosis of the psoas muscle. J Urol 1999; 161:1557-1558. Morovic M Human hydatidosis in Dalmatia, Croatia Epidemiol Infect 1997; 119:271–276. Gluncic I, Roje Z, Bradaric N, Petricevic A, Pisac VP, Gluncic V. Primary Echinococcosis of the sternocleidomastoid muscle. Croat Med J 2001;42:196– 198. OLGU SUNUMU SAFRA KESESİ TORSİYONU: OLGU SUNUMU Sabahattin Aslan, Nemci Yücekule, Bahadır Çetin, Melih Akıncı, Ahmet Seki, Aybala Ağaç, Recep Çetin, Abdullah Çetin Ankara Onkoloji Eğitim Araşırma Hastanesi, I. Cerrahi Kliniği, Ankara, Türkiye ÖZET Safra kesesi torsiyonu nadir rastlanan ve sıklıkla intraoperatif tanı konulan bir akut batın nedenidir. Wendel tarafından ilk kez tanımlanmasından sonra yaklaşık 300 olgu bildirilmiştir. Olgumuzda ayırıcı tanıda akılda tutulması gerektiği sonucuna ulaştığımız inkomplet torsiyone olmuş safra kesesinin yol açtığı tablo sunulmuş ve ek olarak hastalığın etyolojisi, klinik prezentasyonu ve tedavisi değerlendirilmiştir. Anahtar Kelimeler: Safra kesesi, Torsiyon, Kolesistit, Akut batın GALL BLADDER TORSION: A CASE REPORT ABSTRACT Gall bladder torsion is a rare cause of acute abdomen and usually diagnosed intraoperatively. After Wendel reported the first case, nearly 300 cases have been reported in the literature. Here, we present a clinical case caused by an incomplete torsion of the gall bladder which should be kept in mind in the differential diagnosis and we review the etiology, clinical presentation and the treatment of the disease. Keywords: Gall bladder, Torsion, Cholesystitis, Acute abdomen ultrasonografide; karaciğerin normal olduğu, safra kesesi içerisinde 15 mm taş saptandığı, safra kesesinin hidropik olduğu ve arka duvar lokalizasyonunda safra çamuru izlendiği rapor edildi. Tiroid fonksiyon testlerinde subklinik hipotiroidisi bulunan hasta operasyona alındı. Sağ subkostal kesi ile batına girildiğinde safra kesesinin hidropik ve sistik kanalın uzun olduğu, safra kesesinin sistik kanal etrafında saat yönünde yaklaşık 180º torsiyone olduğu görüldü (Resim 1-2). GİRİŞ Çok sık görülen bir klinik durum olmayan ve etiyolojisi tam anlaşılmayan safra kesesi torsiyonuna ait ilk vaka 1898’de Wendel tarafından bildirilmiştir1-5. Günümüze kadar literatürde 300 den fazla vaka rapor edilmiştir1. Bununla beraber vaka raporlarındaki son zamanlardaki artış insidansın aslında tahmin edilenden yüksek olduğunu düşündürmektedir3. Safra kesesi torsiyonunun tedavisi cerrahidir ve tedavi seçimi kolesistektomidir. Günümüzde çoğu vakaya cerrahi sırasında tanı konmaktadır. Bununla beraber hastalığın klinik paterni şüphe uyandırmalı ve yapılacak radyolojik tetkiklerle ayırıcı tanıda akılda bulundurulmalıdır. OLGU SUNUMU 29 yaşında bayan hasta iki gündür olan sağ üst kadran ağrısı, bulantı ve kusma şikâyeti ile kliniğimize başvurdu. Tıbbi geçmişinde hipotiroidizm mevcut olan hasta 50 mcg levotiroksin tb kullanıyordu. Başka bir sistemik hastalık öyküsü saptanmadı. Laboratuar tetkiklerinde WBC 6400 cell/µL, hemoglobin 14,1 g/dl, glikoz 71 mg/dl, böbrek ve karaciğer fonksiyon testleri ve elektrolitler normal sınırlarda rapor edildi. Hastaya yapılan abdominal Resim 1: 180 derece torsiyone olmuş hidropik safra kesesi uzun sistik kanal nedeniyle abdominal kesi açıklığından rahatlıkla görülmekte İletişim Bilgileri: Sabahattin Aslan e-mail: seboaslan@yahoo.co.uk Ankara Onkoloji Eğitim Araşırma Hastanesi, I. Cerrahi Kliniği, Ankara, Türkiye Marmara Medical Journal 2006;19(3);147-148 147 Marmara Medical Journal 2006;19(3);147-148 Sabahattin Aslan ve ark. Safra kesesi torsiyonu: olgu sunumu Akut batın kliniği ile başvuran hastalarda preoperatif safra kesesi torsiyonu ayırıcı tanısı yapmak oldukça güçtür ve sıklıkla operasyon sırasında tanı konulur. Yapılan abdominal USG’lerde yüzen safra kesesi karaciğer altında ancak karaciğere temas etmiyor şekilde izlenebilir. Safra kesesi boynunda konileşme, safra kesesi duvarında ödem ve kalınlaşma, inflamasyon ve gangrenöz değişiklikler gözlenebilir ancak hiç birisi safra kesesi torsiyonu için özellikli değildir. Yaklaşık %20–33 hastada safra kesesi taşı ile birliktelik bulunur5. Safra kesesi torsiyonu gangren ve perforasyon ile katastrofik bir kinik tabloya yol açabilir. Gangrenöz safra kesesinde erken tanı ile perforasyondan korunulur ve cerrahi mortalite %5 den azdır6. Safra kesesi detorsiyone edilerek kolesistektomi uygulandı ve postoperatif ikinci gün hasta problemi olmadan taburcu edildi. Resim 2: Torsiyone hidropik kesenin uzun sistik kanalı görülmekte En sık görülen klinik durum bulantı ve kusma ile sağ üst kadran ağrısıdır. Otuz hastanın birinde sağ üst kadranda kitle palpe edilir. Kitle defans nedeni ile palpe edilemeye bilir ancak perküsyonla matite alınabilir.Lökositoz sıklıkla izlenir. Hastada sarılık ve toksemi bulguları genellikle izlenmez. Incomlete torsiyon safra kesesi koliğini taklit edebilir2,3. TARTIŞMA Safra kesesi torsiyonu nadir görülen akut batın nedenlerindendir ve hastanede yatan 365.520 hastadan birinde görülür. İleri yaş kadın hastalarda insidansı daha yüksektir ancak her yaş gurubunda görülebilir. Erkek/kadın oranı 1/3 ‘dür2. Safra kesesi torsiyonu insidansı beklenen yaşam süresinin artması ile artmaktadır. Bizim hastamızda sağ üst kadran ağrısı ile birlikte safra kesesinde taş izlenmiş ve akut kolesistit atağı düşünülerek opere edilmiştir. Operasyon sırasında görülen incomplete torsiyonun uzun sistik kanal varlığına bağlı olduğunu düşündürmektedir. Sarılık olmadan başlayan ani karın ağısı, bulantı, kusma ve palpabl safra kesesi bulgusu olan hastaların ayırıcı tanısında safra kesesi torsiyonu da akılda tutularak değerlendirilmelidir. Safra kesesi torsiyonunun etyolojisi tam bilinmemektedir, birçok faktörün rol oynadığı ileri sürülmektedir. Gross tarafından iki tip safra kesesi konjenital anomalisi tanımlanmıştır3. Bu anomaliler toplumun %5’inde bulunur ve safra kesesi uzun bir mezenter ile asılı durumdadır. Yaşlılarda bu mezenter ile ilgili ameliyat bulguları konjenital deformite ve/veya kazanılmış generalize visseropitozla ilişkili olabilir. Bu durumda anatomik yerleşim safra kesesi mobilitesini arttırır sonradan ortaya çıkan tetikleyici olaylar safra kesesinin sistik kanal etrafında rotasyonuna neden olur. Karaciğer atrofisi, yaşlanma ile visseral yağ ve elastisite kaybı, kilo kaybı ve spinal deformite safra kesesinin farklı konumlarda bulunmasına ve torsiyonuna neden olur. Ateroskleroz, sistik kanal tortisitesi ve kolelithiazis ile akut dilatasyon torsiyon için dayanak noktası olabilir. Ani hareketler ve yoğun komşu organ peristaltizmi torsiyona yol açabilir3. KAYNAKLAR 1. 2. 3. 4. 5. 6. Safra kesesi torsiyonu complete (270º-360º) veya incomplete (180º) olabilir2,4. Torsiyon meydana geldiğinde venöz kan akımının blokajına bağlı kanlanma azalır ve gangrene yol açar. Safra kesesi kan akımı doppler USG ile değerlendirilebilir4. 148 Jorge OG, Martha PR, Francisco GC, Ricardo Gerardo VC. Volvulus of the gallbladder. Digest Dis Scien 2003;48:116-117. Vosswinkel JA, Colantonio AL. Torsion of the gallbladder laparascopic identification and treatment. Surg Endscop 1999;13:1154-1156. Losken A, Wilson BW, Sherman R Torsion of the gallbladder. Am Surg 1997;63:975-978. Jeffrey M Nicholas Gallbladder volvulus Arch Surg 2002;137:741-742 Abie H, Honda H, Kuroiwa T, et al. Galbladder torsion. Abdm Imaging 2002;27:51-53 Lyons Kenneth P, Challa S, et al. Floating galbladder: A Questionable prelude to torsion, Clin Nucl Med 2000;25:182-83