We describe the case of an ex 25/40 gestation infant initially referred with failure to thrive to her local hospital. She had a background of tracheo-oesophageal fistula and had a degree of chronic lung disease for which she had home oxygen. She was the first born child to unrelated caucasian parents, and was placed in foster care prior to discharge due to parental drug abuse. Examination at referral centre revealed marked splenomegaly. Ultrasound scan revealed portal cavernoma and she was referred to a tertiary unit for investigation and management.
Prior to review, the patient suffered a significant PR bleed at a corrected gestational age of 4 months. Following transfer she suffered 4 further bleeds, 2 of which resulted in cardiac arrest. This is an unusual presentation in a child so young. She had endoscopy and banding, as well as sclerotherapy on 4 separate ocasions, each time suffering a further bleed 3 days later. She was commenced on anti-hypertensive medications with minimal effect. Octreotide did control the bleeding but each time this was weaned she suffered further bleeding. She went on to have splenorenal shunt surgery which abated the bleeds temporarily prior to eventual splenectomy. She had a prolonged intensive care admission and was eventually discharged home on.
This case report highlights the sequelae of prematurity as well as management of an unusual presentation of portal cavernoma in a patient of this age.
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