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A 14-year-old girl, with no medical history except for a preterm birth at 7 months, presented with clinical and laboratory signs of pancytopenia. Radiological investigation, including abdominal ultrasound and magnetic resonance imaging revealed a focal irregular main portal vein wall and splenomegaly. Endoscopy showed major esophageal and gastric varices. Definitive diagnosis was made by direct, transhepatic portography revealing a focal stenosis associated with a dissection of the main portal vein. Percutaneous treatment including balloon angioplasty and stent placement resulted in complete re-expansion of the portal vein main branch. Clinical and radiological follow-up over 5 years showed complete disappearance of all clinical signs and normalization of laboratory data and splenic volume.

Focal portal vein stenosis in an adolescent potentially related to complicated umbilical catheter insertion in the neonatal period