Smira G, Matei E, Gheorghe L, Popescu I
Center of Gastroenterology and Hepatology, Fundeni Clinical Institute, Bucharest, Romania.
Chirurgia (Bucur). 2013 May-Jun;108(3):418-21.
Polycystic Liver Disease (PLD) is a rare progressive disease characterized by increased liver volume due to many cysts, with symptoms related mainly to the size of the liver and the compression on adjacent organs. Most patients who have PLD require no medical or surgical intervention. On the other hand, massive hepatomegaly with severe symptoms which cannot be managed conservatively requires surgical procedures.Liver transplantation (LT) offers the only curative option for the relief of symptoms arising from cyst enlargement and compression of abdominal structures. We presented a rare case of a young man with highly symptomatic isolated PLD due to liver volume - 23,200 cm3, which provokes severe physical and social handicaps and we considered that only total hepatectomy and LT provides a chance of definitive treatment.To our knowledge this is the largest specimen from a PLD patient who was transplanted.
多囊肝病（PLD）是一种罕见的进行性疾病，其特征是由于多个囊肿导致肝脏体积增大，症状主要与肝脏大小及对邻近器官的压迫有关。大多数患有PLD的患者无需药物或手术干预。另一方面，出现严重症状的巨大肝肿大且无法通过保守治疗控制时，则需要进行手术。肝移植（LT）是缓解因囊肿增大和腹部结构受压引起症状的唯一治愈选择。我们报告了一例罕见病例，一名年轻男性患有症状严重的孤立性PLD，肝脏体积达23,200立方厘米，这给他带来了严重的身体和社会障碍，我们认为只有全肝切除术和肝移植才有根治的机会。据我们所知，这是接受移植的PLD患者切除的最大标本。
