de Agustín Asensio J C, Rivilla Parra F, Torres Nieto A, Díez Pardo J
Departamento de Cirugía Pediátrica, Hospital Infantil La Paz, Madrid.
An Esp Pediatr. 1988 Dec;29(6):467-9.
Spontaneous perforation of extrahepatic bile duct is a rare entity, but is the second cause of surgical jaundice in the newborn period after biliary atresia. We review the etiological theories and three patients with this anomaly. Centrolobulillar cholestasis was present in all cases. Parietal ischemic necrosis was seen in one case, obstructive lesions (extrahepatic biliary atresia) in two and malformative lesions (choledochal cyst) in the last patient. A developmental error and a small circumscribed area of duct wall weakness have been the most accepted factors involved in its pathogenesis. However we have found histologic and morphologic evidence that ratify the obstructive and vascular lesions as relevant elements in the etiology of the spontaneous perforation of the biliary tract.
肝外胆管自发性穿孔是一种罕见的病症，但却是新生儿期继胆道闭锁之后外科性黄疸的第二大病因。我们回顾了其病因学理论并报道三例患有该异常的病例。所有病例均存在小叶中心性胆汁淤积。其中一例可见壁层缺血性坏死，两例存在阻塞性病变（肝外胆道闭锁），最后一例存在畸形性病变（胆总管囊肿）。发育异常和一小片局限性的胆管壁薄弱区域是其发病机制中最被认可的因素。然而，我们发现了组织学和形态学证据，证实阻塞性和血管性病变是胆道自发性穿孔病因中的相关因素。
