1Karthikeyan Muthugounder Athiyappan, 1Mathew Cherian, 1Pankaj Mehta, 1Santhosh Poyyamoli, 1Nikhil Handihal Reddy, 1Akhil Monga

1Kovai Medical Center and Hospitals, Coimbatore, India

Background:

Congenital porto systemic shunt (CPSS) can present with hyperammonemia, hepatopulmonary syndrome and porto-pulmonary hypertension. CPSS can be intra-hepatic or extra-hepatic. Severe persistent hypoglycemia is one of the rare manifestations of CPSS which is hypothesized due to decreased clearance of insulin in the liver. Closure of CPSS should be considered for all symptomatic extra-hepatic or intra-hepatic shunt.

Material(s) and Method(s):

A 12-day-old neonate (weight of 3.6 kg) presented to us with complaints of seizures, persistent hyperinsulinemic hypoglycemia, un-responsive to i.v. diazoxide, hydrocortisone and octreotide. On evaluation she had congenital porto-systemic shunt (patent ductus venosus) associated with dilated right atrium, right ventricle, severe pulmonary hypertension and severe tricuspid regurgitation. Due to severe persistent hypoglycemia and associated severe pulmonary hypertension, endovascular embolization of CPSS was suggested.

Under general anaesthesia, right jugular vein access was secured with 5F long sheath.  A Buddy wire was placed in the infrarenal IVC for stability of sheath since the distal end of porto-systemic shunt was close to cavo-atrial junction. Under ultrasound and fluoroscopy guidance, the shunt was cannulated using triaxial system of 4F multipurpose catheter-progreat microcatheter –microwire and obliterated using Amplatzer IV vascular plug.

Result(s):

Following the procedure serum insulin level decreased and the hypoglycemic episodes reduced, requiring minimal medications. Follow up echo showed resolution of pulmonary hypertension. On further evaluation, the neonate had underlying genetic mutation (ABCC8) positive for familial hyperinsulinemic hypoglycemia which contributed as a synergistic factor.

Conclusion(s):

Neonates with persistent and treatment resistant hyperinsulinemic hypoglycemia should be screened for CPSS as a causative or synergistic factor which can be treated by endovascular embolization.

Buddy wire in the IVC offers stability of sheath during placement of vascular plug and USG guidance during placement of vascular plug offers optimal positioning.