C1-esterase inhibitor deficiency in pediatric heart transplant recipients: incidence and findings on ultrasound

Abstract

Acquired angioedema of the bowel caused by a deficiency of C1-esterase inhibitor can lead to severe abdominal pain with sudden onset, mimicking an acute surgical abdomen. In contrast to hereditary angioedema, which usually manifests in childhood, acquired angioedema is broadly recognized to affect people older than 40years. To determine the incidence of acquired angioedema in a cohort of pediatric heart transplant recipients and assess imaging findings on ultrasonography. A cohort of 207 children and adolescents who had undergone heart transplantation were assessed at regular follow-up examinations for incidence of acquired angioedema. All patients received ACE inhibitors and immune inhibitors. Control examinations carried out in 3-month intervals included history, assessment of clinical symptoms, physical examination, US of the abdomen and laboratory blood analysis. In addition, if clinical symptoms were newly encountered, children were admitted between regularly scheduled intervals. We analyzed results of abdominal US for pathological findings of the bowel, and we assessed imaging findings in children diagnosed with acquired angioedema. Acquired angioedema was diagnosed in 3/207 patients (2 girls ages 16 and 17years and 1 boy age 9months). These children presented with sudden onset of severe abdominal pain. The bowel wall was thickened in the presence of ascites. After a mean of 72h, abdominal pains resolved. Thickening of bowel loops dissolved and ascites disappeared. Single episodes of acquired angioedema were encountered in 1.4% of our series of pediatric heart transplant recipients. Radiologists should be familiar with this disease so they can diagnose it on US imaging.