Kolestazın nadir bir nedeni: Konjenital sağ diafragma hernisi

Öz

82 günlük kız hasta ani gelişen sarılık, akolik dışkılama olması nedeniyle yapılan Abdominal Ultrasonografisinde safra kesesinin görülmemesi üzerine kliniğimize yönlendirildi. Akciğer grafisinde sağ diyafram elevasyonu mevcut idi. Hastanın muayenesinde aynı tarafta solunum sesleri azalmış olarak alınıyor idi. Çekilen Tomografisinde sağ diyafragmatik herni mevcut ve karaciğer ile safra kesesi toraks boşluğuna herniye idi. Olgumuzda sağ Bochdalek hernisi mevcut idi ve safrayollarının torasik kavitede sıkışmasına bağlı obstrüktif sarılık gelişmiş idi. Operasyon sırasında alınan karaciğer biyopsisinde fibroz bulguları oluşmaya başlamış idi. Operasyona alınan hastanın abdominal organları yerine alınarak, kolesistektomi ve hepatikojejunostomi operasyonu yapıldı. Erken tanı ve tedavi ile hastanın takibinde yapılan karaciğer biyopsisinde karaciğerde gelişen bulguların düzelmeye başladığı görüldü. Kolestaz, çok çeşitli etyolojik nedenlere bağlı olabilmekle birlikte cerrahi nedenlerin ivedilikle tanı ve tedavisinin yapılması prognoz açısıdan önem arz etmektedir. Bu olgu ile Bochdalek hernisinin çok farklı bir prezantasyon şeklini sunmak istedik. Kolestaz ile gelen akolik dışkısı mevcut olan hastaların ayırıcı tanısında diafragma hernisi de akılda tutulmalıdır.

Anahtar Kelimeler

• Obstrüktif sarılık
• Akolik gaita
• Kolestaz
• Diyafragmatik hernia

A rare cause of cholestasis: Congenital right diaphragmatic hernia

Öz

An 82-day-old infant, investigated for sudden onset jaundice and acholic feces, was referred to our department, as no gallbladder was observed by abdominal ultrasonography. Her chest x-ray revealed an elevated right diaphragm, and the breath sounds were diminished on the right. Suspicious arteriovenous malformation in the fifth segment of the right lobe accompanied by a rotation anomaly of liver was reported. Tomography scans showed right diaphragmatic hernia where the left lobe of liver and gallbladder herniated to the thoracic space. In this case with right Bochdalek hernia, the bile ducts had kinked in the thoracic cavity, causing obstructive jaundice. If the kinking continues despite relocation of organs into the abdominal cavity, cholecystectomy-hepaticojejunostomy is an effective and reliable surgical method. Myriad pathologies may cause cholestasis, and surgical reasons should be investigated and diagnosed without delay to improve prognosis. For closure of the diaphragmatic defect, primary repair should be the preferred method. With this case, we wanted to emphasize a highly different presentation of Bochdalek hernia and remind that diaphragmatic hernia should be kept in mind in patients presenting with cholestasis and acholic stools.

Anahtar Kelimeler

• obstructive jaundice
• acholic feces
• cholestasis
• Diaphragmatic Hernia

Kaynakça

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