https://pubmed.ncbi.nlm.nih.gov/35442217/

J Pediatr Gastroenterol Nutr. 2022 May 30.
doi: 10.1097/MPG.0000000000003450.Online ahead of print.

PRESENTATION, MANAGEMENT AND OUTCOME OF CONGENITAL PORTOSYSTEMIC SHUNTS IN CHILDREN: THE BOSTON CHILDREN'S HOSPITAL EXPERIENCE

Doaa M Fahmy 1 2, Paul D Mitchell 3, Maureen M Jonas 1

Abstract

Objectives: Congenital portosystemic shunts (CPSS) are rare vascular malformations. We describe presentations, complications, associations, and outcomes of CPSS at Boston Children's Hospital (BCH).

Methods: This was a retrospective review of children with CPSS at BCH from 2000-2020.

Results: 29 patients had CPSS (17F): 14 extrahepatic (EH) and 15 intrahepatic (IH). At diagnosis, 15 were ≤ 5d, 7 < 1y and 7 >1y (range 1-19). Median follow-up duration was 5.2y (IQR 1.6-10.9) in EH and 2.2y (0.2-4.2) in IH CPSS. The most common presentation was antenatal ultrasound 13 (45%) followed by hyperammonemia 10 (34%), while 6 (21%) were asymptomatic. Complications were noted in 17 (12/14 EH vs 6/15 IH, P=0.008). Associated anomalies were present in 25 (14/14 EH vs 11/15 IH, P=0.10). Spontaneous closure was observed in 8 (28%) patients with IH CPSS, all <12 months of age. 10 patients underwent shunt closure 3 (30%) by Interventional radiology (IR) and 5 (50%) by surgery, while 2 (20%) required both. After therapeutic closure; 8 had improvement, 1 had portal hypertension and 1 had sepsis and thrombosis. The remaining 11 patients, 8 (42%) were followed without closure: 6/8 (75%) EH vs 2/11 (18%) IH (P=0.02), 2 lost follow-up and 1 with complicated EH CPSS died, unsuitable for therapeutic closure.

Conclusions: CPSS may be asymptomatic or present with complications. Spontaneous closure of IH shunts may occur in infancy, thus therapeutic closure may be deferred until age ≥ 2y. IR and surgical closure of CPSS are associated with improvement in the majority of cases.