title: Postprandial hyperinsulinaemic hypoglycaemia secondary to a congenital portosystemic shunt.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children, London, UK.
authors.forename: Senthil
authors.initials: S
authors.lastname: Senniappan
authors.forename: Katherine
authors.initials: K
authors.lastname: Pitt
authors.forename: Pratik
authors.initials: P
authors.lastname: Shah
authors.forename: Ved
authors.initials: V
authors.lastname: Arya
authors.forename: Sanjay
authors.initials: S
authors.lastname: Jaiswal
authors.forename: Munther
authors.initials: M
authors.lastname: Haddad
authors.forename: Jonathan
authors.initials: J
authors.lastname: Hind
authors.forename: Anil
authors.initials: A
authors.lastname: Dhawan
authors.forename: Mark
authors.initials: M
authors.lastname: Davenport
authors.forename: Khalid
authors.initials: K
authors.lastname: Hussain
mesh_terms: D005260:Female; D006801:Humans; D006946:Hyperinsulinism / Q000097:blood / Q000000981:diagnostic imaging; D007003:Hypoglycemia / Q000097:blood / Q000000981:diagnostic imaging; D007223:Infant; D011169:Portal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011859:Radiography; D054079:Vascular Malformations / Q000097:blood / Q000000981:diagnostic imaging