Congenital portosystemic shunt: characterization of a multisystem disease

Sokollik, Christiane; Bandsma, Robert H. J.; Gana, Juan C.; van den Heuvel, Meta; Ling, Simon C. (2013). Congenital portosystemic shunt: characterization of a multisystem disease. Journal of pediatric gastroenterology and nutrition, 56(6), pp. 675-681. Lippincott Williams & Wilkins 10.1097/MPG.0b013e31828b3750

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OBJECTIVES

Congenital portosystemic shunts (CPSSs) are rare but increasingly recognized as a cause of important multisystem morbidity. We present new cases and a systematic literature review and propose an algorithm for the identification and care of affected patients.

METHODS

We reviewed the charts of consecutive patients seen in our pediatric liver clinic between 2003 and 2010 and systematically reviewed the literature of cases with CPSS.

RESULTS

We identified 316 published cases and 12 patients in our own clinic. Of the published cases (177 male), 185 had an extrahepatic and 131 an intrahepatic portosystemic shunt. Diagnosis was made at any age, from prenatal to late adulthood. Cardiac anomalies were found in 22% of patients. The main complications were hyperammonemia/neurological abnormalities (35%), liver tumors (26%), and pulmonary hypertension or hepatopulmonary syndrome (18%). The spectrum of neurological involvement ranged from changes in brain imaging, subtle abnormalities on neuropsychological testing, through learning disabilities to overt encephalopathy. Spontaneous shunt closure occurred mainly in infants with intrahepatic shunts. Therapeutic interventions included shunt closure by surgery or interventional radiology techniques (35%) and liver transplantation (10%) leading to an improvement of symptoms in the majority. These findings mirror the observations in our own patients.

CONCLUSIONS

In this largest review of the reported clinical experience, we identify that children with CPSS may present with otherwise unexplained developmental delay, encephalopathy, pulmonary hypertension, hypoxemia, or liver tumors. When CPSS is diagnosed, children should be screened for all of these complications. Spontaneous closure of intrahepatic shunts may occur in infancy. Closure of the shunt is indicated in symptomatic patients and is associated with a favorable outcome.

Item Type:	Journal Article (Original Article)
Division/Institute:	04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine 04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine > Paediatric Gastroenterology
UniBE Contributor:	Sokollik, Christiane
Subjects:	600 Technology > 610 Medicine & health
ISSN:	0277-2116
Publisher:	Lippincott Williams & Wilkins
Language:	English
Submitter:	Anette van Dorland
Date Deposited:	12 Jun 2014 10:40
Last Modified:	05 Dec 2022 14:30
Publisher DOI:	10.1097/MPG.0b013e31828b3750
PubMed ID:	23412540
URI:	https://boris.unibe.ch/id/eprint/45617