Focal portal vein stenosis in an adolescent potentially related to complicated umbilical catheter insertion in the neonatal period
A 14-year-old girl presented with a gradual onset of fatigue and apathy. Laboratory analysis revealed a pancytopenia as summarized in Table . Liver function tests were within normal limits. Her medical history was non-specific except for a preterm birth at 7 months and observation at the neonatal intensive care. At that time a venous umbilical catheter was placed for intravenous fluid administration. However, catheter position was not documented by abdominal plain film. There was no history of hepatitis or other diseases in this otherwise healthy girl. Screening abdominal ultrasound was within normal limits, except for a splenomegaly with a maximal splenic diameter of 17 cm. In order to exclude portal venous and hepatic parenchymal disorders a magnetic resonance angiography (MRA) as well as a transjugular liver biopsy and pressure measurements were performed. MRA revealed a discrete, focal irregularity of the extrahepatic portal vein main branch. The liver biopsy was within normal limits without signs of fibrosis or cirrhosis. Pressure measurements showed a wedged hepatic venous pressure of 11 mmHg and inferior vena cava pressure of 9 mmHg. Further, a gastroscopy was performed, revealing major varices in the lower esophagus and signs of hypertensive gastropathy. The varices were endoscopically ligated, as it was suggested that the anemia could be associated with occult or intermittent bleeding from these varices. Finally, additional laboratory analysis could not identify any thrombophilic parameter disorder. Based on these findings, it was decided to perform direct transhepatic portography and percutaneous treatment.\nUnder general anesthesia, access to the right portal vein was made using ultrasound guidance. Venography clearly revealed a moderate focal stenosis of the main portal vein, potentially associated with a focal intimal dissection. Additionally very large gastric and esophageal variceal collaterals were also clearly visible (Fig. a). Pressure measurements over the stenosis were: 16 mmHg proximal and 6 mmHg distal to the stenosis resulting in a pressure gradient of 10 mmHg. Balloon angioplasty with a 10-mm diameter balloon did not result in sufficient re-expansion of the stenosed segment. It was decided to insert a self-expanding nitinol stent with a length of 4 cm and a nominal diameter of 12 mm (Zilverstent, Cook Medical, Bloomington, IN, USA) which was postdilated up to 10 mm diameter. Pressure measurements after stenting were: 12 mmHg proximal and 10 mmHg distal to the stented segment resulting in a residual pressure gradient of 2 mmHg. Completion venography confirmed the correct appositioning and expansion of the stent and absence of any collateral flow (Fig. b). Patient recovered well after the procedure and could leave the hospital 2 days later. Follow-up was performed by clinical, ultrasound, and laboratory evaluation at regular intervals: every 3 months in the first year and yearly thereafter. Clinically, all signs of chronic anemia disappeared and gastroscopy was normal without visible esophageal varices or hypertensive gastropathy. Ultrasound evaluation could demonstrate a normal volume of the spleen with maximal diameters after 1, 2, 3, 4, and 5 years of 13.2 cm, 13.2 cm, 13.6 cm, 13.8 cm, and 14 cm, respectively, which is at the upper limit of normal values (12–14 cm). Duplex-ultrasound flow measurements were on all evaluations between 40 and 60 cm/s over a 5-year follow-up period. Finally, values of red blood cell, white blood cell, and platelet count normalized during follow-up (Table).