Stringel G, Mercer S
J Pediatr Surg. 1983 Oct;18(5):546-50. doi: 10.1016/s0022-3468(83)80356-x.
Idiopathic perforation of the bile duct is rare in children. Sixty-seven cases were reported in the English literature to 1980. It is, nevertheless, the second commonest surgical cause of jaundice in the neonate. The etiology is unknown though distal obstruction and weakness in the bile duct wall have been postulated. Limited surgical treatment with external drainage is the preferred therapy. In isolated cases internal drainage procedures or repeated aspiration have been successful. The diagnosis should be suspected in the presence of jaundice and ascites with or without abdominal pain and signs of peritoneal irritation. We describe a 3-month-old girl presenting with anemia, vomiting, jaundice, and ascites. This was initially diagnosed as hepatitis but bilious fluid was found on paracentesis. Computerized tomography with cholangiography and 99 MTC Diisopropyl IDA cholescintigraphy confirmed the diagnosis. The latter seems to be more accurate than I-131 Rose Bengal. The perforation was at the junction of the hepatic and cystic ducts. It was treated successfully by external drainage and a cholecystostomy. Direct attempts to close the perforation, or more complicated surgical procedures, are unnecessary while nonoperative treatment carries a high mortality. At follow-up after 1 year the IV cholangiogram and liver-function tests are normal. Cholecystostomy provided good drainage of the biliary ducts as well as easy access for follow-up cholangiography.
小儿特发性胆管穿孔较为罕见。至1980年，英文文献中共报道了67例。然而，它却是新生儿黄疸的第二大常见外科病因。病因尚不清楚，不过曾有人推测是胆管远端梗阻及胆管壁薄弱所致。首选的治疗方法是采用外引流进行有限的手术治疗。在个别病例中，内引流手术或反复抽吸也取得了成功。出现黄疸和腹水，无论有无腹痛及腹膜刺激征，均应怀疑该病的诊断。我们报道了一名3个月大的女童，她出现贫血、呕吐、黄疸和腹水。最初诊断为肝炎，但腹腔穿刺抽出了胆汁样液体。计算机断层扫描胆管造影和99锝二异丙基乙基亚氨基二乙酸胆管闪烁造影确诊了该病。后者似乎比I-131玫瑰红更准确。穿孔位于肝管和胆囊管交界处。通过外引流和胆囊造口术成功治愈。无需直接尝试闭合穿孔或进行更复杂的外科手术，而非手术治疗的死亡率很高。1年后随访时，静脉胆管造影和肝功能检查均正常。胆囊造口术能很好地引流胆管，也便于后续进行胆管造影检查。
