Senniappan Senthil, Pitt Katherine, Shah Pratik, Arya Ved, Jaiswal Sanjay, Haddad Munther, Hind Jonathan, Dhawan Anil, Davenport Mark, Hussain Khalid
Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children, London, UK.
Horm Res Paediatr. 2015;83(3):217-20. doi: 10.1159/000369014. Epub 2015 Jan 21.
Portosystemic shunts (PSS) are abnormal vascular connections between the portal vein or its tributaries and the systemic vein that allow mesenteric blood to reach the systemic circulation without first passing through the liver. PSS can be associated with various syndromes and can lead to serious complications. We report a rare case of a child with PSS and recurrent hypoglycaemia.
A 20-month-old girl with Down's syndrome presented with recurrent hypoglycaemic episodes. She had multiple anomalies including a ventricular septal defect, oesophageal atresia and tracheo-esophageal fistula, gastro-oesophageal reflux, and conjugated hyperbilirubinaemia. The initial investigations suggested hyperinsulinaemic hypoglycaemia (HH). She did not respond to diazoxide. An oral glucose tolerance test suggested postprandial HH. Further vascular imaging showed a side-to-side portocaval shunt (Abernethy malformation) with relative hypoperfusion of the liver. Hypoglycaemia resolved following surgical closure of the portocaval shunt.
PSS can rarely be associated with HH, possibly due to lack of insulin degradation in the liver. Surgical closure of the shunt resolves the hypoglycaemia.
门体分流（PSS）是门静脉或其分支与体循环静脉之间的异常血管连接，使肠系膜血液无需先通过肝脏即可进入体循环。PSS可与多种综合征相关，并可导致严重并发症。我们报告一例患有PSS和反复低血糖的儿童罕见病例。
一名20个月大的唐氏综合征女孩出现反复低血糖发作。她有多种异常情况，包括室间隔缺损、食管闭锁和气管食管瘘、胃食管反流以及结合胆红素血症。初步检查提示高胰岛素性低血糖（HH）。她对二氮嗪无反应。口服葡萄糖耐量试验提示餐后HH。进一步的血管成像显示侧侧门腔分流（阿伯内西畸形）伴肝脏相对灌注不足。门腔分流手术关闭后低血糖得到缓解。
PSS很少与HH相关，可能是由于肝脏中胰岛素降解不足。分流手术关闭可缓解低血糖。
