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

Abstract

BackgroundCongenital portosystemic shunts present with various associated complications, such as other congenital malformations, hyperammonemia, or hepatopulmonary syndrome. Few cases of associated hypoglycemia have been reported so far and our case, to the best of our knowledge, describes the most severe extent of hypoglycemia.Case presentationWe describe the case of a newborn Arab boy with two intrahepatic portosystemic shunts, resulting in severe and persistent hypoglycemia, due to which one of the shunts was closed by interventional radiology whereas the other shunt had already closed spontaneously.ConclusionsBecause he showed elevated levels for insulin and prolonged high insulin levels in an oral glucose tolerance test, our case supports the theory that portocaval shunts cause a reduced hepatic insulin reduction due to the high blood volume bypassing the liver. This case provides further insights into glucose regulation mechanisms of the liver and we suggest a consistent screening for hypoglycemia in patients with congenital portosystemic shunts.

Abernethy syndrome in Slovenian children: Five case reports and review of literature

“…One of our patients (case 4) also began experiencing hypoglycemic episodes when he was four years old, which is one of the rarer clinical complications of CPSS and has been described mostly in newborns, in whom it can be clinically very severe and persistent and is most likely due to hyperinsulinism from reduced hepatic degradation of insulin[ 28 ]. In our case, the patient responded well to increased oral glucose intake.…”

Congenital intrahepatic portosystemic venous shunt embolization: A two‐case experience

“…Intrahepatic shunts have a higher rate of spontaneous closure . In contrast, extrahepatic shunts almost never show spontaneous closure . Morgan and Superina classified the extrahepatic portosystemic shunts in two types considering the type I, a shunt that causes complete diversion of portal flow into the inferior vena cava with congenital absence of a portal vein.…”

“…There are four morphological types of congenital IPSVS described in the literature: Type 1 is the most common and consists of a single vessel directly communicating the right portal vein with the inferior vena cava (IVC) . Type II shunts involve one hepatic segment and consist of either a single communication or multiple communications between peripheral portal and hepatic venous branches . The third type of shunt is the least common and involves direct communication between peripheral portal and hepatic venous branches through an aneurysmal segment .…”

“…2 In contrast, extrahepatic shunts almost never show spontaneous closure. 3 Morgan and Superina classified the extrahepatic portosystemic shunts in two types considering the type I, a shunt that causes complete diversion of portal flow into the inferior vena cava with congenital absence of a portal vein. In addition, the splenic and mesenteric veins end up each one directly or in a common trunk into the vena cava.…”

“…5 Type II shunts involve one hepatic segment and consist of either a single communication or multiple communications between peripheral portal and hepatic venous branches. 3 The third type of shunt is the least common and involves direct communication between peripheral portal and hepatic venous branches through an aneurysmal segment. 4 The fourth type of shunt involves more than one hepatic segment and consists of multiple communicating peripheral portal and hepatic venous branches.…”