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
Full text not available from this repository. (Request a copy)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 |
Actions (login required)
 |
Edit item |