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 21 ISSUE 2/APRIL 2025

Is moderate to late prematurity an independent risk factor for neonatal hypoglycaemia and subsequent neurodevelopmental impairment?

Neonatal hypoglycaemia is the most frequent metabolic disorder in newborns and is associated with poorer neurodevelopment. Consensuses regarding definition of neonatal hypoglycaemia and management are debated. Infant factors increasing risk of hypoglycaemia include premature birth. This is the first systematic review considering whether moderate to late prematurity is an independent risk factor for hypoglycaemia <2.6mmol/L (<47mg/dL) and associated impact on neurodevelopment.