Dorney S F, Middleton A W, Martin H C, Cummins G, Shun A, Gaskin K J, O'Loughlin E V, Kamath K R
Department of Gastroenterology, Children's Hospital, Camperdown, New South Wales, Australia.
Aust N Z J Surg. 1989 Nov;59(11):855-8. doi: 10.1111/j.1445-2197.1989.tb07027.x.
Forty-seven infants (26 male, 21 female) with biliary atresia under- went hepatic portoenterostomy during the 16-year period 1971-87. Twenty-six patients (55%) are alive 1-17 years after surgery, with 21 (45%) being jaundice-free. For children who became jaundice-free, the mean age at surgery was 78 days (range: 34-125 days), compared with 97 days (range: 48-224 days) for those who did not. Of 39 patients operated on at less than 120 days of age, 24 (60%) are alive. All four patients operated on after 125 days of life died. Of 31 patients operated on more than 5 years ago, 12 (39%) have survived, the oldest being 17 years. Ten (32%) have normal serum bilirubin concentrations, have non-active cirrhosis on liver biopsy, have had normal growth and development, and lead normal lives. The oldest two patients suffered variceal haemorrhage in their teenage years. In our recent experience, 11 of 16 patients (69%) have had complete clearing of jaundice, lead normal lives and do not currently require assessment for liver transplantation. It is believed that early referral of children with biliary atresia to experienced surgical units for portoenterostomy will lead to long-term survival, without the need for liver transplantation in a majority of cases. Liver transplantation should be offered in infancy only after failed portoenterostomy, except for patients presenting after 120 days in whom transplantation may be considered primary therapy.
在1971年至1987年的16年期间，47例患有胆道闭锁的婴儿（26例男性，21例女性）接受了肝门肠吻合术。26例患者（55%）在手术后1至17年存活，其中21例（45%）无黄疸。对于无黄疸的儿童，手术时的平均年龄为78天（范围：34至125天），而未无黄疸的儿童为97天（范围：48至224天）。在39例120日龄以下接受手术的患者中，24例（60%）存活。所有4例在125日龄后接受手术的患者均死亡。在5年多前接受手术的31例患者中，12例（39%）存活，年龄最大的为17岁。10例（32%）血清胆红素浓度正常，肝活检显示无活动性肝硬化，生长发育正常，过着正常生活。年龄最大的两名患者在青少年时期发生了静脉曲张出血。根据我们最近的经验，16例患者中有11例（69%）黄疸完全消退，过着正常生活，目前不需要进行肝移植评估。据信，将患有胆道闭锁的儿童尽早转诊至经验丰富的外科单位进行肝门肠吻合术将导致长期存活，大多数情况下无需进行肝移植。仅在肝门肠吻合术失败后才应在婴儿期提供肝移植，但120日龄后就诊的患者除外，对于这些患者可考虑将移植作为主要治疗方法。
