A congenital portosystemic shunt in the neonatal period: case report and literature review - Report - MDSpire

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

  • By

  • Li Cheng

  • Shuwen Feng

  • Yin Shen

  • Xia Wang

  • Bingyan He

  • Dongchi Zhao

  • Pu Yang

  • July 15, 2026

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Clinical Report: Neonatal Presentation of Congenital Portosystemic Shunt

Background

Congenital portosystemic shunt (CPSS) is a rare vascular malformation with an incidence estimated at 1 in 30,000 to 50,000 live births. Clinical manifestations during the neonatal period can be vague and non-specific.

Data Highlights

No numerical data or trial data are presented in the article.

Key Findings

  • Case 1 presented with respiratory distress, diffuse ecchymosis, and petechial hemorrhages, leading to progressive liver dysfunction and DIC.
  • Case 2 exhibited respiratory distress and multiple cutaneous hemangiomas, progressing to severe pulmonary hypertension.
  • Clinical manifestations of CPSS in neonates may include abnormal liver function, hyperammonemia, and hypoglycemia.
  • CPSS is associated with conditions such as congenital heart disease, Down syndrome, and pulmonary hypertension.
  • Early prenatal ultrasound screening is important for the diagnosis of CPSS.

Clinical Implications

Healthcare professionals should be aware of the atypical presentations of CPSS in neonates to ensure timely diagnosis.

Related Resources & Content

  1. George Y. Wu, Congenital Portosystemic Shunts – A Review, 2026 -- Congenital portosystemic shunts (CPSS) are rare vascular anomalies.
  2. Frontiers in Pediatrics, Endovascular closure of a large intrahepatic portosystemic shunt in a three-month-old infant: a case report and literature review, 2026 -- Introduction of CPSS and its implications.
  3. Frontiers in Pediatrics, Single-center experience of interventional therapy for congenital portal-systemic shunt in children, 2026 -- Evaluation of interventional treatment for CPSS.
  4. Pediatric Cardiology — Pulmonary Interstitial Glycogenosis: A Potential Overlooked Cause of Persistent Pulmonary Hypertension in Newborns with Congenital Heart Defects?
  5. Frontiers in Pediatrics — Case Report: Imaging findings of neonatal horseshoe lung with multiple system malformations
  6. Congenital Portosystemic Shunts – A Review
  7. https://pure.amsterdamumc.nl/ws/portalfiles/portal/150099936/Expert-management-of-congenital-portosystemic-shunts-and-their-complications.pdf
  8. Impact of Portal Flow on the Prognosis of Children With Congenital Portosystemic Shunt: A Multicentric Observation Study in Japan - ScienceDirect

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