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Published in: Journal of Medical Case Reports 1/2018

Open Access 01-12-2018 | Case report

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

Authors: Alexander Weigert, Jeanette Bierwolf, Heiko Reutter, Ulrich Gembruch, Joachim Woelfle, Rainer Ganschow, Andreas Mueller

Published in: Journal of Medical Case Reports | Issue 1/2018

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Abstract

Background

Congenital 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 presentation

We 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.

Conclusions

Because 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.
Literature
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go back to reference Duprey J, Gouin B, Benazet MF, Le Gal J. Intolerance au glucose et hypoglycemie post-stimulative secondaires a une anastomose porto-cave intrahepatique vraisemblablement congenitale. Ann Med Interne (Paris). 1985;136:655–8. Duprey J, Gouin B, Benazet MF, Le Gal J. Intolerance au glucose et hypoglycemie post-stimulative secondaires a une anastomose porto-cave intrahepatique vraisemblablement congenitale. Ann Med Interne (Paris). 1985;136:655–8.
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go back to reference Bas S, Guran T, Atay Z, Haliloglu B, Abalı S, Turan S, Bereket A. Premature pubarche, hyperinsulinemia and hypothyroxinemia: Novel manifestations of congenital portosystemic shunts (Abernethy malformation) in children. Horm Res Paediatr. 2015;83:282–7. https://doi.org/10.1159/000369395.CrossRef Bas S, Guran T, Atay Z, Haliloglu B, Abalı S, Turan S, Bereket A. Premature pubarche, hyperinsulinemia and hypothyroxinemia: Novel manifestations of congenital portosystemic shunts (Abernethy malformation) in children. Horm Res Paediatr. 2015;83:282–7. https://​doi.​org/​10.​1159/​000369395.CrossRef
Metadata
Title
Congenital intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism: a case report and review of the literature
Authors
Alexander Weigert
Jeanette Bierwolf
Heiko Reutter
Ulrich Gembruch
Joachim Woelfle
Rainer Ganschow
Andreas Mueller
Publication date
01-12-2018
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2018
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-018-1881-y

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