A congenital portosystemic shunt in the neonatal period: case report and literature review.

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.