Non-Surgical Management of Colo-Colonic Intussusception in Patients with Hereditary Angioedema

Abstract

Patient: Female, 17Final Diagnosis: Colo-colonic intussusceptionSymptoms: Abdominal painMedication: —Clinical Procedure: —Specialty: RadiologyObjective:Unusual clinical courseBackground: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.

Bowel obstruction secondary to internal hernia in a hereditary angioedema patient: a case report

“…In a review of 153 patients and 33,671 episodes of gastrointestinal attacks, less frequent symptoms were reported, including hemorrhagic diarrhea, tetany, and a single documented case of bowel intussusception [ 8 ]. There have been similar reports of small bowel obstruction and intussusceptions that resolved after infusion of C1-INH concentrate, but few have reported a bowel with impending ischemia [ 9 , 10 ]. In this case, the surgical procedure revealed strangulation of the distal jejunum to ileum incarcerated as an internal hernia due to adhesions forming a hernial band.…”

Gastrointestinal manifestations of angioedema: a potential area of misdiagnosis

“…Hence, given the presumed diagnosis of acute appendicitis, she underwent an appendicectomy. [28] Ascites Case report Cohen et al [29] Hypovolemic shock Case report Bork et al [30] Hypovolemic shock 4.4% Wu et al [31] Retroperitoneal angioedema Rare Witschi et al [32] Intussusception Rare Patel et al [13] Intussusception Case report Figueroa-Diaz et al [33] Intussusception Case report Cheng et al [34] Intussusception 0.05% Farkas et al [35] Elevated transaminase levels; Rare Hepatic parenchymal changes Cutler et al [36] Pancreatitis Case report Matesic et al [37] Pancreatitis Case report Cancian et al [38] Pancreatitis Case report Czaller et al [39] Pancreatitis Case report Berger et al [40] Pancreatitis Case report Aksoy et al [41] Pancreatitis Case report Lopes Veronez et al [42] Pancreatitis Case report…”