RESUMO
BACKGROUND Hereditary angioedema (HAE) is an autosomal disease caused either by deficiency or presence of a non-functioning C1 inhibitor. The lack or non-functionality of said inhibitors leads to activation of an inflammatory cascade, which result in cutaneous and mucosal edema. Most patients with HAE present with either cutaneous, laryngeal/pharyngeal, or gastrointestinal exacerbations. An uncommon gastrointestinal manifestation of HAE is an intussusception, which in most cases require invasive/surgical management. CASE REPORT A 17-year-old Hispanic female patient with past medical history of HAE, presented with a 4-day history of episodic abdominal pain, worsening during the last 2 days with associated nausea, vomiting, and bright red blood per rectum. The abdominal ultrasound performed at our institution showed an elongated region of hypoechoic and hyperechoic concentric rings, raising suspicion of an intussusception. The patient was treated conservatively, with 30 mg of ecallantide and a unit of fresh frozen plasma (FFP). Follow-up abdominopelvic computed tomography scan was performed approximately 20 hours after the administration of fresh frozen plasma revealing complete interval resolution of the colo-colonic intussusception. Subsequently, the patient was kept under hospital care for the next 4 days with adequate progression of diet and without recurrence of intussusception. CONCLUSIONS To the best of our knowledge, most cases of patient with HAE presenting with intussusception have been treated with invasive/surgical procedures. In our case, conservative management has proven successful to reduce edema with subsequent non-surgical reduction of the intussusception. By directly targeting the pathophysiologic aspects of HAE, an unnecessary invasive procedure, as well as its potential complications, were avoided.