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. Case: 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. Conclusion: 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.

Congenital intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism: a case report and review of the literature

“…The authors described these effects as a result of reduced insulin degradation in the liver [ 6 ]. This effect has also been described in a few other case reports [ 9 , 10 ]. Bas et al postulated an insufficient postprandial hepatic glucose uptake, resulting in early systemic hyperglycemia, leading to an exaggerated insulin secretion [ 9 ].…”

Pulmonary arterial hypertension caused by congenital extrahepatic portocaval shunt: a case report

“…The clinical presentations of CEPS may vary and depend on associated conditions: (1) liver dysfunction and hepatic neoplasms, which may be benign (focal nodular hyperplasia and nodular regenerative hyperplasia) or malignant (liver cancer), may be due to the lack of perfusion and nutrient delivery to the liver [9–11]. (2) Congenital hepatic shunts can also present together with hepatic encephalopathy, hepatopulmonary syndrome [12], and hypoglycaemia [13]. (3) Increased pressure of the splenic vein and superior mesenteric vein system with complete or partial occlusion of the portal vein can lead to hypersplenism and haemorrhage of the digestive tract.…”

Portosystemic shunt as a cause of congenital hyperinsulinemic hypoglycemia

“…6,7 Hence, it is possible that patients with CPSS have high insulin concentration, especially after meals, because insulin secreted from beta cells flows directly into the systemic circulation. Senniappan et al 4 reported on a 20-month-old girl with CPSS who had hyperinsulinemic hypoglycemia. The hypoglycemia was completely cured after surgical closure of the shunt, and OGTT after surgery showed no hyperinsulinemia.…”

“…Some CPSS patients also have hypoglycemia. Recently, Senniappan et al 4 reported on the case of a 20-month-old girl with Down syndrome and CPSS who presented with hyperinsulinemic hypoglycemia, possibly due to lack of insulin degradation in the liver. Herein we describe the case of a 5-month-old boy with post-prandial CHH and type 1 extrahepatic CPSS.…”