Makroskopik Hematüri ile Bulgu Veren Konjenital Portal Ven Agenezisi: Olgu Sunumu ve Literatür İncelemesi

Portal venin konjenital yokluğu, portal kan akışının kısmen veya tamamen sistemik dolaşıma yönlendirildiği nadir bir klinik tablodur. Bu durum, sayısız klinik semptomlara neden olur ve çoğunlukla kardiyak ve vasküler kaynaklı diğer konjenital anormalliklerle ilişkilidir. Burada makroskopik hematüri ile başvuran tip IB Abernethy malformasyonlu 14 yaşında erkek hasta sunulmuş olup, olgu canlı donör karaciğer transplantasyonu ile tedavi edilmiştir. Hastanın tıbbi kayıtları geriye dönük olarak gözden geçirilerek olgu, literatürün kısa bir gözden geçirmesi ile birlikte sunulmuştur. Hastada fokal nodüler hiperplaziye ait karaciğer kitleleri ile birlikte nutcracker sendromu da mevcuttu. Hasta postoperatif dönemde sorunsuz seyretmiştir. Karaciğer nakli, bu çok nadir görülen anatomik malformasyon için güvenli ve radikal bir tedavi sunar. Sadece anormal portal akışı düzeltmekle kalmaz, aynı zamanda hepatopulmoner sendrom ve diğer olası karaciğer maligniteleri gibi diğer yaşamı tehdit eden komplikasyonları da önler.

Congenital Absence of Portal Vein Presenting with Macroscopic Hematuria: A Case Report with a Brief Review of the Literature

Congenital absence of the portal vein is a rare clinical entity in which portal blood flow is partially or totally diverted to the systemic circulation. This condition causes a myriad of clinical symptoms and is associated with other congenital abnormalities mostly of cardiac and vascular origin. A 14-year-old male patient with type IB Abernethy malformation who presented with macroscopic hematuria was treated with live donor liver transplantation. The medical recordings of the patient were retrospectively reviewed, and the case was presented with a brief review of the literature. The patient had associating nut-cracker syndrome and liver masses that were revealed to be focal nodular hyperplasia. The patient is doing well in the postoperative course. Liver transplantation offers a safe and radical treatment for this very rare anatomic malformation. It not only corrects aberrant portal flow but also prevents other life-threatening complications such as hepatopulmonary syndrome and possible liver malignancies.

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