Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period

Objective: The aim of this single-center retrospective study was to analyze the clinical characteristics, treatment options, and course of neonatal-onset congenital portosystemic shunts (CPSS).Methods: We included all patients with CPSS who presented with clinical symptoms within the neonatal period...

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Autores principales: Suhua Xu, Peng Zhang, Liyuan Hu, Wenhao Zhou, Guoqiang Cheng
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Publicado: Frontiers Media S.A. 2021
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spelling oai:doaj.org-article:d981e654d84c49deb138225c666fb41f2021-12-02T10:38:08ZCase Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period2296-236010.3389/fped.2021.778791https://doaj.org/article/d981e654d84c49deb138225c666fb41f2021-12-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fped.2021.778791/fullhttps://doaj.org/toc/2296-2360Objective: The aim of this single-center retrospective study was to analyze the clinical characteristics, treatment options, and course of neonatal-onset congenital portosystemic shunts (CPSS).Methods: We included all patients with CPSS who presented with clinical symptoms within the neonatal period in our institution between 2015 and 2020.Results: Sixteen patients were identified, including 13 patients with intrahepatic portosystemic shunts (IPSS) and three patients with extrahepatic portosystemic shunts (EPSS). The median age of diagnosis was 16 days (range prenatal 24 weeks−12 months). Hyperammonemia (60%), neonatal cholestasis (44%), elevated liver enzyme (40%), hypoglycemia (40%), thrombocytopenia (38%), and coagulation abnormalities (23%) appeared in neonatal CPSS. Twelve patients (75%) presented with congenital anomalies, of which congenital heart disease (CHD) (44%) was the most common. Thirteen patients with IPSS initially underwent conservative treatment, but two of them were recommended for the catheter interventional therapy and liver transplantation, respectively, due to progressive deterioration of liver function. Spontaneous closure occurred in nine patients with IPSS. The shunt was closed using transcatheter embolization in one patient with EPSS type II. Another patient with EPSS type II underwent surgical treatment of CHD firstly. The remaining patient with EPSS type Ib received medical therapy and refused liver transplantation.Conclusion: Hyperammonemia, neonatal cholestasis, elevated liver enzyme, hypoglycemia, and thrombocytopenia are the main complications of neonatal CPSS. Moreover, CPSS is associated with multiple congenital abnormalities, especially CHD. Intrahepatic portosystemic shunts may close spontaneously, and conservative treatment can be taken first. Extrahepatic portosystemic shunts should be closed to prevent complications.Suhua XuPeng ZhangLiyuan HuWenhao ZhouGuoqiang ChengFrontiers Media S.A.articlecongenital portosystemic shuntsabernethy syndromevascular malformationsneonatecase reportPediatricsRJ1-570ENFrontiers in Pediatrics, Vol 9 (2021)
institution DOAJ
collection DOAJ
language EN
topic congenital portosystemic shunts
abernethy syndrome
vascular malformations
neonate
case report
Pediatrics
RJ1-570
spellingShingle congenital portosystemic shunts
abernethy syndrome
vascular malformations
neonate
case report
Pediatrics
RJ1-570
Suhua Xu
Peng Zhang
Liyuan Hu
Wenhao Zhou
Guoqiang Cheng
Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period
description Objective: The aim of this single-center retrospective study was to analyze the clinical characteristics, treatment options, and course of neonatal-onset congenital portosystemic shunts (CPSS).Methods: We included all patients with CPSS who presented with clinical symptoms within the neonatal period in our institution between 2015 and 2020.Results: Sixteen patients were identified, including 13 patients with intrahepatic portosystemic shunts (IPSS) and three patients with extrahepatic portosystemic shunts (EPSS). The median age of diagnosis was 16 days (range prenatal 24 weeks−12 months). Hyperammonemia (60%), neonatal cholestasis (44%), elevated liver enzyme (40%), hypoglycemia (40%), thrombocytopenia (38%), and coagulation abnormalities (23%) appeared in neonatal CPSS. Twelve patients (75%) presented with congenital anomalies, of which congenital heart disease (CHD) (44%) was the most common. Thirteen patients with IPSS initially underwent conservative treatment, but two of them were recommended for the catheter interventional therapy and liver transplantation, respectively, due to progressive deterioration of liver function. Spontaneous closure occurred in nine patients with IPSS. The shunt was closed using transcatheter embolization in one patient with EPSS type II. Another patient with EPSS type II underwent surgical treatment of CHD firstly. The remaining patient with EPSS type Ib received medical therapy and refused liver transplantation.Conclusion: Hyperammonemia, neonatal cholestasis, elevated liver enzyme, hypoglycemia, and thrombocytopenia are the main complications of neonatal CPSS. Moreover, CPSS is associated with multiple congenital abnormalities, especially CHD. Intrahepatic portosystemic shunts may close spontaneously, and conservative treatment can be taken first. Extrahepatic portosystemic shunts should be closed to prevent complications.
format article
author Suhua Xu
Peng Zhang
Liyuan Hu
Wenhao Zhou
Guoqiang Cheng
author_facet Suhua Xu
Peng Zhang
Liyuan Hu
Wenhao Zhou
Guoqiang Cheng
author_sort Suhua Xu
title Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period
title_short Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period
title_full Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period
title_fullStr Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period
title_full_unstemmed Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period
title_sort case report: clinical features of congenital portosystemic shunts in the neonatal period
publisher Frontiers Media S.A.
publishDate 2021
url https://doaj.org/article/d981e654d84c49deb138225c666fb41f
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