Clinical characteristics, associated anomalies and outcomes of congenital portosystemic shunts in children: a single-center experience
Abstract
Aims: Congenital portosystemic shunts (CPSS) are rare vascular malformations diverting portal venous blood into the systemic circulation. Data describing their clinical spectrum, associated congenital anomalies, and natural history in children remain limited.
Methods: We retrospectively evaluated nine pediatric patients diagnosed with CPSS at the Department of Pediatric Gastroenterology, Ankara Etlik City Hospital, between January 2023 and October 2025. Demographic, clinical, laboratory, radiological, and follow-up data were analyzed descriptively.
Results: Seven patients (77.8%) had intrahepatic and two (22.2%) had extrahepatic (Abernethy type II) shunts. The most common presentations were neonatal hypoxemia/cyanosis (4/9), cholestasis (3/9), and hypoglycemia (2/9). Associated congenital anomalies, most frequently cardiovascular, hepatobiliary, and renal, were identified in eight of nine patients (88.9%). During follow-up (mean 21.6±14.1 months), complete spontaneous closure occurred in four of seven intrahepatic shunts (57.1%) and regression in two (28.6%), whereas both extrahepatic shunts remained patent.
Conclusion: In this small series, intrahepatic CPSS diagnosed in infancy frequently demonstrated spontaneous closure or regression, whereas both extrahepatic shunts remained patent during follow-up, consistent with previous reports, although this finding should be interpreted cautiously given the small number of extrahepatic cases. Associated congenital anomalies were frequently observed, supporting consideration of multidisciplinary multisystem evaluation in children diagnosed with CPSS.
Keywords
- Congenital portosystemic shunt
- Abernethy malformation
- children
- spontaneous closure
- congenital anomalies
Supporting Institution
no
Ethical Statement
This study was approved by the Institutional Ethics Committee (Clinical Research Ethics Committee) of Ankara Etlik City Hospital (Approval No: AEŞH-BADEK2-2025-482, Date: 04.11.2025).
References
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Details
Primary Language
English
Subjects
Pediatric Gastroenterology
Journal Section
Research Article
Publication Date
October 25, 2026
Submission Date
August 13, 2026
Acceptance Date
October 5, 2026
Published in Issue
Year 2026 Volume: 9 Number: 6
APA
Cebe Tok, A., Akpınar, A., Yılmaz, Ş., Araslı Yılmaz, A., & Dinlen Fettah, N. (2026). Clinical characteristics, associated anomalies and outcomes of congenital portosystemic shunts in children: a single-center experience. Journal of Health Sciences and Medicine, 9(6), 1799-1805. https://doi.org/10.32322/jhsm.2016215
AMA
1.Cebe Tok A, Akpınar A, Yılmaz Ş, Araslı Yılmaz A, Dinlen Fettah N. Clinical characteristics, associated anomalies and outcomes of congenital portosystemic shunts in children: a single-center experience. J Health Sci Med / JHSM. 2026;9(6):1799-1805. doi:10.32322/jhsm.2016215
Chicago
Cebe Tok, Ayşegül, Alper Akpınar, Şükriye Yılmaz, Aslıhan Araslı Yılmaz, and Nurdan Dinlen Fettah. 2026. “Clinical Characteristics, Associated Anomalies and Outcomes of Congenital Portosystemic Shunts in Children: A Single-Center Experience”. Journal of Health Sciences and Medicine 9 (6): 1799-1805. https://doi.org/10.32322/jhsm.2016215.
EndNote
Cebe Tok A, Akpınar A, Yılmaz Ş, Araslı Yılmaz A, Dinlen Fettah N (October 1, 2026) Clinical characteristics, associated anomalies and outcomes of congenital portosystemic shunts in children: a single-center experience. Journal of Health Sciences and Medicine 9 6 1799–1805.
IEEE
[1]A. Cebe Tok, A. Akpınar, Ş. Yılmaz, A. Araslı Yılmaz, and N. Dinlen Fettah, “Clinical characteristics, associated anomalies and outcomes of congenital portosystemic shunts in children: a single-center experience”, J Health Sci Med / JHSM, vol. 9, no. 6, pp. 1799–1805, Oct. 2026, doi: 10.32322/jhsm.2016215.
ISNAD
Cebe Tok, Ayşegül - Akpınar, Alper - Yılmaz, Şükriye - Araslı Yılmaz, Aslıhan - Dinlen Fettah, Nurdan. “Clinical Characteristics, Associated Anomalies and Outcomes of Congenital Portosystemic Shunts in Children: A Single-Center Experience”. Journal of Health Sciences and Medicine 9/6 (October 1, 2026): 1799-1805. https://doi.org/10.32322/jhsm.2016215.
JAMA
1.Cebe Tok A, Akpınar A, Yılmaz Ş, Araslı Yılmaz A, Dinlen Fettah N. Clinical characteristics, associated anomalies and outcomes of congenital portosystemic shunts in children: a single-center experience. J Health Sci Med / JHSM. 2026;9:1799–1805.
MLA
Cebe Tok, Ayşegül, et al. “Clinical Characteristics, Associated Anomalies and Outcomes of Congenital Portosystemic Shunts in Children: A Single-Center Experience”. Journal of Health Sciences and Medicine, vol. 9, no. 6, Oct. 2026, pp. 1799-05, doi:10.32322/jhsm.2016215.
Vancouver
1.Ayşegül Cebe Tok, Alper Akpınar, Şükriye Yılmaz, Aslıhan Araslı Yılmaz, Nurdan Dinlen Fettah. Clinical characteristics, associated anomalies and outcomes of congenital portosystemic shunts in children: a single-center experience. J Health Sci Med / JHSM. 2026 Oct. 1;9(6):1799-805. doi:10.32322/jhsm.2016215
