Congenital intrahepatic portosystemic shunt and neonatal hyperinsulinaemic hypoglycaemia

An incidental finding of a congenital intrahepatic portosystemic shunt (CPSS) on an abdominal ultrasound scan identified the cause of neonatal hyperinsulinaemic hypoglycaemia (HH). We present the development of clinical presentation, differential diagnosis and subsequent management of a case of CPSS. We provide an explanatory illustration of the pathophysiology of CPSS and HH, highlighting the importance of long-term follow-up of infants who suffer HH early in life.

Natalie Fairhurst
Advanced Neonatal Nurse Practitioner
nfairhurst@sidra.org

Ahmad Alhamad
Radiologist

Haytham Ali
Attending Neonatologist, Division of Neonatology

Sidra Medicine, Doha, Qatar

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Keywords

portosystemic shunt; hypoglycaemia; hyperinsulinism

Key points

Intrahepatic portosystemic shunt is a recognised cause of refractory neonatal hyperinsulinaemic hypoglycaemia.
An early abdominal ultrasound can assist in rapid diagnosis and more aggressive treatment thereby minimising the risks of persistent hypoglycaemia.
Infants with this diagnosis benefit from close follow-up of their neurodevelopment.

Also published in Infant:

VOLUME 14/ISSUE 6, NOVEMBER 2018

A two-year comparative study of admissions of term neonates for hypoglycaemia: what have the BAPM recommendations changed?

Hypoglycaemia is a leading cause of term admissions to neonatal units but many term babies admitted for hypoglycaemia could be cared for in an alternative hospital or community setting. The British Association of Perinatal Medicine (BAPM) Framework for Practice aims to reduce national variation in detection and management of term babies with hypoglycaemia. The study presented here compares the epidemiology of neonatal hypoglycaemia in term neonates admitted to a tertiary neonatal intensive care unit for a one-year period before and after implementation of a BAPM recommendations-based guideline.