Pintér Andrew B, Farkas Andrew, Pár Alajos, Kövesi Tamás, Appelshoffer Sarolta
Department of Paediatric Surgery, Faculty of Medicine, University of Pécs, Pécs, Hungary.
J Pediatr Surg. 2004 Jul;39(7):1050-4. doi: 10.1016/j.jpedsurg.2004.03.053.
BACKGROUND/PURPOSE: Description of the long-term follow-up of 5 patients operated on for atresia of the common bile duct.
During a 25-year period (1960 to 1985) 45 infants underwent surgical exploration for biliary atresia (BA), of which, 5 (11.1%) were found to have atresia of the common bile duct. The children were followed up into adult life by pediatric surgeons, pediatricians, and later, adult hepatologists with the range of 19 to 36 years. Liver function tests, histology, complications, and somatic development (including sexual maturation and mental development) were obtained continuously.
Liver function test results were normal in all but 1 patient. Repeated ultrasound scan and postoperative liver biopsies were normal in 2 patients and moderately and mildly abnormal in 3 patients, respectively, suggesting hepatic fibrosis. HIDA hepatic scans in all but 1 patient showed prompt uptake by the liver with passage into the bowel within 30 minutes. Endoscopic retrograde cholangiography (ERCP; 1 patient) and duodenography (4 patients) showed, at most, mild reflux of contrast material into the extra- or intrahepatic bile ducts. One patient with cholecysto-duodenostomy had 3 episodes of clinically proven ascending cholangitis. All 5 patients had normal physical growth and mental development, they are all age-appropriate schooled, and they are working and living a normal life.
Long-term favorable outcome has been suggested to be more influenced by anatomic and biological features rather than the surgical correction. The hypothesis is supported that BA is not a static congenital malformation but a progressive inflammatory panductular obliterative disease of the bile ducts starting in the antenatal period, which might cease, either early at birth or any time in infancy. The destructive inflammatory process might involve only the distal part of the extrahepatic bile duct causing obstruction and leaving the proximal ducts patent.
背景/目的：描述5例因胆总管闭锁接受手术治疗患者的长期随访情况。
在1960年至1985年的25年期间，45例婴儿因胆道闭锁（BA）接受了手术探查，其中5例（11.1%）被发现患有胆总管闭锁。这些儿童由小儿外科医生、儿科医生随访至成年期，后来由成人肝病学家随访，随访时间为19至36年。持续获取肝功能检查、组织学、并发症及身体发育情况（包括性成熟和智力发育）。
除1例患者外，所有患者的肝功能检查结果均正常。2例患者的重复超声扫描及术后肝脏活检结果正常，3例患者分别为中度和轻度异常，提示肝纤维化。除1例患者外，所有患者的肝胆动态显像均显示肝脏迅速摄取，30分钟内造影剂排入肠道。内镜逆行胆管造影（ERCP；1例患者）和十二指肠造影（4例患者）显示，最多只有少量造影剂反流至肝外或肝内胆管。1例行胆囊十二指肠吻合术的患者发生了3次经临床证实的上行性胆管炎。所有5例患者身体生长和智力发育正常，均接受了适龄教育，工作和生活正常。
长期良好预后似乎更多地受解剖和生物学特征而非手术矫正的影响。这支持了以下假说：BA不是一种静态的先天性畸形，而是一种始于产前的进行性炎症性全胆管闭塞性疾病，可能在出生时或婴儿期的任何时候停止。破坏性炎症过程可能仅累及肝外胆管的远端，导致梗阻，而近端胆管保持通畅。
