Topuzlu Gonca TEKAND, Ebru YEŞİLDAĞ, Daver YEKER, Sergülen DERVİŞOĞLU, Ergun ERDOĞAN, Nüvit SARIMURAT, Afaq Alhaq MOBİN, Temurçin ÜNAL

Ülkemiz koşullarında nöronal intestinal displazi tanısı konabilir mi?

Hirschsprung hastalığının klinik bulguları ile başvuran hastaların yalnızca % 50'sinde klasik aganglionosis belirlenirken, bu grup hastaların %20-40'ında nöronal intestinal displazi (NİD) sorumlu tutulan gerçek patolojidir. Bu çalışmada, NİD tanısı alan ve barsak dismotilitesi yakınmalarıyla başvuran hastaların sonuçları değerlendirilmiştir. Cerrahpaşa Tıp Fakültesi Çocuk Cerrahisi Anabilim Dalı'nda 1990-1999 yılları arasında 4 kız ve 4 erkek toplam 8 olguda patolojik inceleme sonucunda NİD tanısı konmuştur. Kliniğimize yatırılan tüm olgulara gastrointestinal dekompresyon, lavman opak grafisi ve anorektal manometri rutin olarak uygulandı. Tam kat rektal biyopsiler, peroperatif kolon biyopsileri ve anal miyektomi örnekleri histopatolojik olarak hematoksilen eosin boyasıyla (HE) değerlendirildi. Histopatolojik olarak NİD tanısı alan olgular oral cisapride veya cerrahi tedavi uygulanarak izlendiler. Sonuç olarak, ülkemiz koşullarında immünohistokimyasal olanakların olmaması nedeniyle, NİD'nin tanısı patologun HE boyası ile deneyimi ve olguların klinik başvurularının birlikte değerlendirilmesi ile konulmalıdır. Anorektal manometri ve lavman opak grafilari NİD için nonspesifik bilgi verdikleri için patoloji, tanı kriteri olarak önemini korumaktadır.

Can neural intestinal dysplasia be diagnosed in our domestic environment?

Background and Design.- Classic aganglionosis is detected in only 50% of patients presenting with clinical symptoms of Hirschsprung's disease; neuronal intestinal dysplasia (NID) is the real pathology in 20-40% of this group. Herein, the outcome of cases presenting with complaints related to bowel dysmotility and diagnosed to have NID, is evaluated. Results.- 8 NID cases, admitted to our unit had gastrointestinal decompression, barium enema and anorectal manometry, performed routinely. The full thickness rectal biopsies, peroperative colonic biopsy and anal myectomy samples were histopathologically evaluated with hematoxylin eosin stain (HE). Those cases histopathologically labeled as NID were either given oral Cisapride or surgically treated and followed up. In our domestic environment deprived of immunohistochemical facilities, diagnosis of NID is dependent on the combined experience of the pathologist, using the HE stain, and the clinical presentation of the cases. Conclusion.- Anorectal manometry and barium enema, provide nonspecific information for NID and pathology reveals the most important diagnostic criteria.

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