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Presentation, Management, and Outcome of Congenital Portosystemic Shunts in Children: The Boston Children's Hospital
Doaa M Fahmy1,2, Paul D Mitchell3, Maureen M Jonas1
1From the Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Insights
Congenital portosystemic shunts (CPSS) are rare vascular malformations. Therapeutic closure of CPSS via interventional radiology or surgery often leads to improved outcomes in pediatric patients.
Area of Science:
- Vascular Malformations
- Pediatric Surgery
- Interventional Radiology
Background:
- Congenital portosystemic shunts (CPSS) are uncommon vascular anomalies.
- Understanding their presentations, complications, and outcomes is crucial for patient management.
Purpose of the Study:
- To describe the clinical presentations, associated anomalies, complications, and outcomes of pediatric patients with CPSS.
- To evaluate the effectiveness of different management strategies, including spontaneous and therapeutic closure.
Main Methods:
- Retrospective review of pediatric patients diagnosed with CPSS at Boston Children's Hospital between 2000 and 2020.
- Analysis of patient demographics, clinical presentations, associated conditions, management approaches, and outcomes.
Main Results:
- Twenty-nine patients with CPSS were identified, with varying presentations including antenatal detection and hyperammonemia.
- Complications were more frequent in extrahepatic (EH) shunts compared to intrahepatic (IH) shunts.
- Spontaneous closure occurred in some IH shunts in infancy; therapeutic closure by interventional radiology (IR) or surgery showed improvement in most cases.
Conclusions:
- CPSS can present asymptomatically or with significant complications.
- Spontaneous closure of IH shunts in infancy may allow for delayed therapeutic intervention.
- Both IR and surgical closure of CPSS are effective management options, leading to improved patient outcomes.
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 to 2020.
Results:
Twenty-nine patients had CPSS (17 girls): 14 extrahepatic (EH) and 15 intrahepatic (IH). At diagnosis, 15 were ≤5 days, 7 <1 year, and 7 >1 year (range 1-19). Median follow-up duration was 5.2 years (interquartile range [IQR] 1.6-10.9) in EH and 2.2 years (0.2-4.2) in IH CPSS. The most common presentation was antenatal ultrasound 13 (45%) followed by hyperammonemia 10 (34%), whereas 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. Ten patients underwent shunt closure 3 (30%) by interventional radiology (IR) and 5 (50%) by surgery, whereas 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 of 8 (75%) EH versus 2 of 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 ≥ 2 years. IR and surgical closure of CPSS are associated with improvement in the majority of cases.
