To summarize clinical characteristics, diagnosis, treatment, and prognostic features of congenital portosystemic shunt (CPSS) during the neonatal period.
Approach:
Case Reports: We report two neonatal CPSS cases and review 34 additional cases from the literature over the past decade to enhance clinical recognition.
Key Findings:
Case 1 presented with respiratory distress, diffuse ecchymosis, petechial hemorrhages, and later developed progressive liver dysfunction and DIC.
Case 2 exhibited respiratory distress and multiple cutaneous hemangiomas, leading to progressive liver dysfunction and severe pulmonary hypertension.
Early onset of idiopathic DIC, multiple cutaneous hemangiomas, progressive liver dysfunction, and pulmonary hypertension may suggest CPSS.
Interpretation:
The clinical manifestations of CPSS in neonates are often vague and non-specific, complicating early diagnosis.
Limitations:
The rarity of CPSS may limit the generalizability of findings across different populations.
The retrospective nature of the analysis may introduce bias in the interpretation of results.
Conclusion:
Systematic assessment and timely intervention are essential for improving outcomes in neonatal CPSS.
