Hypoxia due to intrapulmonary vascular dilatation in a toddler with a congenital portacaval shunt: case report

Mohammed T Alsamri,Mohamed Sulaiman,Abdul-Kader Souid,Hassib Narchi,Mohamed A Hamdan

doi:10.1186/s12890-019-0788-8

Mohammed T Alsamri, Mohamed Sulaiman + Show 3 more

Open Access

https://doi.org/10.1186/s12890-019-0788-8

Copy DOI

Abstract

BackgroundThe term hepatopulmonary syndrome typically applies to cyanosis that results from “intrapulmonary vascular dilatation” due to advanced liver disease. Similar findings may result from a congenital portosystemic shunt without liver disease. An adverse consequence of such shunts is intrapulmonary vascular dilatation, which affects the microvascular gas exchange units for oxygen.Case presentationHere, we describe a toddler with chronic cyanosis, exercise intolerance, and finger clubbing due to a malformation shunt between the portal vein and the inferior vena cava. A transcatheter embolization of the shunt resulted in resolution of his findings.ConclusionsCongenital portosystemic shunts need to be considered in the differential diagnosis of cyanosis.

Highlights

The term hepatopulmonary syndrome typically applies to cyanosis that results from “intrapulmonary vascular dilatation” due to advanced liver disease
Other rare causes include abnormal hemoglobin or hepatopulmonary syndrome. The latter entity may result from a hepatic parenchymal pathology or, more rarely, from a congenital portosystemic shunt that causes intrapulmonary vascular dilatation leading to hypoxia [1, 2]
The presence of cyanosis and dyspnea without respiratory or cardiac pathology should lead to a careful evaluation of platypnea and orthodeoxia

Summary

Introduction

The term hepatopulmonary syndrome typically applies to cyanosis that results from “intrapulmonary vascular dilatation” due to advanced liver disease. Similar findings may result from a congenital portosystemic shunt without liver disease. Case presentation: Here, we describe a toddler with chronic cyanosis, exercise intolerance, and finger clubbing due to a malformation shunt between the portal vein and the inferior vena cava. Other rare causes include abnormal hemoglobin (e.g., methemoglobinemia) or hepatopulmonary syndrome. The latter entity may result from a hepatic parenchymal pathology or, more rarely, from a congenital portosystemic shunt that causes intrapulmonary vascular dilatation leading to hypoxia [1, 2]. We describe a 2-year-old boy with chronic cyanosis due to a large portosystemic shunt between the portal vein and the inferior vena cava. This report aims to increase awareness of this rare entity and emphasize that embolization of the shunt is curative

Objectives

Discussion

Conclusion