Clifton Matthew S, Goldstein Ruth B, Slavotinek Anne, Norton Mary E, Lee Hanmin, Farrell Jody, Nobuhara Kerilyn K
Department of Surgery, University of California, San Francisco, CA 94143-0570, USA.
Pediatrics. 2006 Mar;117(3):e596-600. doi: 10.1542/peds.2005-1411. Epub 2006 Feb 1.
Familial choledochal cysts are extremely rare. High-resolution ultrasound now allows for the antenatal diagnosis of these anomalies. After delivery, elective surgical resection should be planned; however, increases in size, deterioration of liver function, and ascending cholangitis frequently force early intervention. We report an unusual occurrence of siblings with type I choledochal cysts and review the existing literature on cause, genetics, classification, diagnosis, and management of this disease.
家族性胆总管囊肿极为罕见。高分辨率超声现在能够对这些异常情况进行产前诊断。分娩后，应计划进行择期手术切除；然而，囊肿大小增加、肝功能恶化以及化脓性胆管炎常常迫使进行早期干预。我们报告了一例I型胆总管囊肿在兄弟姐妹中罕见发生的病例，并综述了关于该疾病的病因、遗传学、分类、诊断和治疗的现有文献。
