Cargando…

A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations that result in blood bypassing the liver and re-entering the systemic circulation unfiltered. The outcomes of CPSS diagnosed prenatally are unknown. Previously published data is related to patients diagnosed after sym...

Descripción completa

Detalles Bibliográficos
Autores principales: Steg Saban, O, Weissbach, T, Achiron, R, Pekar Zlotin, M, Haberman, Y, Kassif, E, Weiss, B
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8859223/
http://dx.doi.org/10.1093/jcag/gwab049.220
_version_ 1784654408401813504
author Steg Saban, O
Weissbach, T
Achiron, R
Pekar Zlotin, M
Haberman, Y
Kassif, E
Weiss, B
author_facet Steg Saban, O
Weissbach, T
Achiron, R
Pekar Zlotin, M
Haberman, Y
Kassif, E
Weiss, B
author_sort Steg Saban, O
collection PubMed
description BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations that result in blood bypassing the liver and re-entering the systemic circulation unfiltered. The outcomes of CPSS diagnosed prenatally are unknown. Previously published data is related to patients diagnosed after symptom appearance, including hepatic encephalopathy, liver tumors and portal hypertension. AIMS: To describe a cohort of prenatally diagnosed children with CPSS and report on the natural history and outcomes. METHODS: The study was conducted between 2006 and 2019. Prenatal diagnosis was performed during routine prenatal ultrasound. Patients were followed by the pediatric gastroenterologists’ unit. RESULTS: 32 patients were identified; 28 patients with intrahepatic shunts and four patients with extrahepatic shunts. During follow up, Failure to Thrive (FTT) was observed in three of the patients with one shunt (16.7%), and five of the patients with two shunts or more (55.6%). The difference is significant (p- value=0.037). 24 patients with intrahepatic shunts had their shunts closed spontaneously. Median time for closure of the shunt was seven months (IQR 2–14 months, range 0–35 months). No predicting factor was detected for closure of the shunt before the age of one year. All extrahepatic shunts required surgical closure. CONCLUSIONS: Congenital intrahepatic shunts usually close spontaneously and do not need intervention. All patients in our prenatally diagnosed cohort survived with limited to no sequelae. FUNDING AGENCIES: None
format Online
Article
Text
id pubmed-8859223
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Oxford University Press
record_format MEDLINE/PubMed
spelling pubmed-88592232022-02-22 A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME Steg Saban, O Weissbach, T Achiron, R Pekar Zlotin, M Haberman, Y Kassif, E Weiss, B J Can Assoc Gastroenterol Poster of Distinction BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations that result in blood bypassing the liver and re-entering the systemic circulation unfiltered. The outcomes of CPSS diagnosed prenatally are unknown. Previously published data is related to patients diagnosed after symptom appearance, including hepatic encephalopathy, liver tumors and portal hypertension. AIMS: To describe a cohort of prenatally diagnosed children with CPSS and report on the natural history and outcomes. METHODS: The study was conducted between 2006 and 2019. Prenatal diagnosis was performed during routine prenatal ultrasound. Patients were followed by the pediatric gastroenterologists’ unit. RESULTS: 32 patients were identified; 28 patients with intrahepatic shunts and four patients with extrahepatic shunts. During follow up, Failure to Thrive (FTT) was observed in three of the patients with one shunt (16.7%), and five of the patients with two shunts or more (55.6%). The difference is significant (p- value=0.037). 24 patients with intrahepatic shunts had their shunts closed spontaneously. Median time for closure of the shunt was seven months (IQR 2–14 months, range 0–35 months). No predicting factor was detected for closure of the shunt before the age of one year. All extrahepatic shunts required surgical closure. CONCLUSIONS: Congenital intrahepatic shunts usually close spontaneously and do not need intervention. All patients in our prenatally diagnosed cohort survived with limited to no sequelae. FUNDING AGENCIES: None Oxford University Press 2022-02-21 /pmc/articles/PMC8859223/ http://dx.doi.org/10.1093/jcag/gwab049.220 Text en ڣ The Author(s) 2022. Published by Oxford University Press on behalf of the Canadian Association of Gastroenterology. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Poster of Distinction
Steg Saban, O
Weissbach, T
Achiron, R
Pekar Zlotin, M
Haberman, Y
Kassif, E
Weiss, B
A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME
title A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME
title_full A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME
title_fullStr A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME
title_full_unstemmed A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME
title_short A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME
title_sort a221 congenital portosystemic shunts: from prenatal diagnosis to long- term postnatal outcome
topic Poster of Distinction
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8859223/
http://dx.doi.org/10.1093/jcag/gwab049.220
work_keys_str_mv AT stegsabano a221congenitalportosystemicshuntsfromprenataldiagnosistolongtermpostnataloutcome
AT weissbacht a221congenitalportosystemicshuntsfromprenataldiagnosistolongtermpostnataloutcome
AT achironr a221congenitalportosystemicshuntsfromprenataldiagnosistolongtermpostnataloutcome
AT pekarzlotinm a221congenitalportosystemicshuntsfromprenataldiagnosistolongtermpostnataloutcome
AT habermany a221congenitalportosystemicshuntsfromprenataldiagnosistolongtermpostnataloutcome
AT kassife a221congenitalportosystemicshuntsfromprenataldiagnosistolongtermpostnataloutcome
AT weissb a221congenitalportosystemicshuntsfromprenataldiagnosistolongtermpostnataloutcome