Li Cheng, Shuwen Feng, Yin Shen, Xia Wang, Bingyan He, Dongchi Zhao, Pu Yang
Women and Children’s Hospital and Zhongnan Hospital of Wuhan University.
China
Frontiers in Pediatrics
Front Pediatr 2026; 14:
DOI: 10.3389/fped.2026.1836875
Abstract
A congenital portosystemic shunt (CPSS), a rare developmental malformation of the portal venous system, often presents with atypical clinical manifestations in the neonatal period that are prone to be missed or misdiagnosed. Herein, we report two neonatal CPSS cases. Case 1 initially manifested as respiratory distress, diffuse ecchymosis, and petechial hemorrhages and later caused progressive liver dysfunction, disseminated intravascular coagulation (DIC), and patent ductus arteriosus. Case 2 initially exhibited respiratory distress and multiple cutaneous hemangiomas, subsequently leading to progressive liver dysfunction, an increase in the number and size of hemangiomas, and severe pulmonary hypertension. Alongside our two neonatal CPSS cases, this article reviews 34 other neonatal cases reported in the literature over the past decade, aiming to enhance clinical recognition by summarizing the disease’s clinical characteristics, diagnosis, treatment, and prognostic features during the neonatal period. Particularly, the early onset of idiopathic DIC, multiple cutaneous hemangiomas, progressive liver dysfunction, and pulmonary hypertension in neonates may be suggestive of CPSS. Furthermore, it underscores the critical importance of prenatal ultrasound screening, comprehensive multisystem assessment, and multidisciplinary care for the early diagnosis, individualized treatment, and improved prognosis of neonatal CPSS.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes