A100 CAVERNOUS TRANSFORMATION OF THE PORTAL VEIN IN A PEDIATRIC PATIENT WITH GOLDENHAR SYNDROME: A CASE REPORT

Abstract Background Goldenhar syndrome is a rare congenital condition thought to arise from the first and second branchial arches typically asymmetrically affects eyes, ears, spine. Portal vein thrombosis/cavernous transformation one of main causes portal hypertension in children. It often associated with risk factors such as catheterization umbilical neonatal period, omphalitis, intra-abdominal infections period prothrombotic conditions. This challenging clinical scenario it leads variceal bleed no chronic liver disease stigmata function essentially normal. While previous European literature early-2000s reported anomalies syndrome, we are not aware any recent and/or Canadian pediatric cases. Purpose We report case cavernous resulting gastrointestinal bleeding child syndrome. Method Case Result(s) A patient postnatal suspicion confirmation by 6 months age, presented an acute 3-day history melena, on context viral illness ibuprofen use. Laboratory testing showed normocytic anemia (Hb 68) normal INR (1.1). The rest workup was unremarkable. There nor other hematological complications. To assess for potential duplication cyst differential diagnosis, computerized tomography chest, abdomen pelvis ordered revealed suspected varices near gastroesophageal junction within abdomen, hepatomegaly spleen size at upper limit Octreotide started after CT results. gastroscopy done 24h presentation found hypertensive gastropathy esophageal (2 Grade II 1 I). Sclerotherapy performed. Post-gastroscopy ultrasound supported earlier radiographic findings hepatosplenomegaly. Moreover, noted flow direction hepatic veins. has re-presented further episodes bleeding. Conclusion(s) supports previously association between venous anomalies. Early consideration may lead prompt diagnosis management potentially life-threatening complications this population. Please acknowledge all funding agencies checking applicable boxes below None Disclosure Interest Declared