Grosfeld J L, Fitzgerald J F, Predaina R, West K W, Vane D W, Rescorla F J
Department of Surgery, Indiana University School of Medicine, Indianapolis.
Surgery. 1989 Oct;106(4):692-700; discussion 700-1.
This report describes the treatment and outcome of 66 infants with biliary atresia. Mean age was 79.8 +/- 33.2 days. Diagnosis was achieved by 99mTc DISIDA scanning. Hepatoportoenterostomy (HPE) was performed in 48 cases and hepatoportocholecystostomy in four, with microscopic ducts at the porta hepatis. Fourteen infants without microscopic ducts did not undergo HPE. Patients were staged according to the postoperative result. HPE was successful in 25% of patients (group A), resulted in improvement in 19% (group B), failed in 43% (group C), and was short-term in 13% (group D). In patients less than 90 days of age, the HPE success rate was 31%; 23% improved, and 33% showed no improvement. Age (less than 90 days) and bile clearance were prognostic determinants of success. Reoperation was useful only in patients with a previously successful HPE. Ten of 20 patients referred for liver transplantation survived (50%) (7/11) survived after liver transplantation and 3/9 on the waiting list). Fourteen of 15 patients in group A remain anicteric and well without liver transplantation. Patients in group B have had extended survival (greater than 3 years) but eventually required transplantation. Patients in group C and children more than 90 days old at diagnosis require early liver transplantation. HPE is a useful procedure when performed in infants less than 90 days of age who have biliary atresia.
本报告描述了66例胆道闭锁婴儿的治疗情况及预后。平均年龄为79.8±33.2天。通过99mTc二异丙基乙基亚氨基二乙酸扫描确诊。48例患者进行了肝门肠吻合术（HPE），4例肝门部有微小胆管的患者进行了肝门胆囊吻合术。14例无微小胆管的婴儿未接受HPE。根据术后结果对患者进行分期。HPE在25%的患者中成功（A组），19%的患者病情改善（B组），43%的患者失败（C组），13%的患者为短期有效（D组）。年龄小于90天的患者中，HPE成功率为31%；23%的患者病情改善，33%的患者无改善。年龄（小于90天）和胆汁清除率是成功的预后决定因素。再次手术仅对先前HPE成功的患者有用。转诊进行肝移植的20例患者中有10例存活（50%）（肝移植后7/11存活，等待名单上3/9存活）。A组15例患者中有14例无黄疸且情况良好，无需肝移植。B组患者生存期延长（大于3年），但最终需要移植。C组患者及诊断时年龄大于90天的儿童需要早期肝移植。对于年龄小于90天的胆道闭锁婴儿，HPE是一种有效的手术方法。
