Hereditary angio-oedema as a rare cause of small-bowel obstruction

Jamil, Bilal; Naeem, Muhammad Saulat; Anachebe, Tochukwu; Majeed, Muhammad Hamid

doi:10.1136/bcr-2019-231186

Cited by 4 publications

(3 citation statements)

References 9 publications

(29 reference statements)

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“…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.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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Background Gastrointestinal attacks are frequent symptoms in patients diagnosed with hereditary angioedema (HAE). Cases of self-limited bowel intussusception and unneeded exploratory laparotomy due to lack of knowledge about HAE have been reported. Furthermore, after the introduction of C1-esterase inhibitor (C1-INH) concentrate, the recommended medication for HAE attacks, treatment has become typically medical in nature. We share a rare case where operative exploration was indicated to resolve a mechanical small bowel obstruction secondary to an HAE attack. Case report An 80-year-old woman with HAE presented with lower left abdominal pain, vomiting, and nausea. Computed tomography (CT) showed edema of the small bowel and stomach as well as possible signs of mechanical small bowel obstruction. The patient was treated with C1-INH concentrate but showed only mild signs of relief, warranting diagnostic laparoscopy. Intraoperative findings showed internal herniation and strangulation of the small bowel caused by adhesions forming a band. After surgical intervention, no bowel resection was needed. Conclusion Although C1-INH concentrate remains the principal treatment for HAE, gastrointestinal attacks may potentially cause surgical emergencies.

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Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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“…Evidence of the HAE-GI symptom overlap comes from both observational 57 and single-case studies 27,38,42,50,[67][68][69][70] (Table 2). HAE should be suspected when a patient presents with a history of recurrent cutaneous angioedema attacks.…”

Section: When Should Hae Be Suspected? Practical Guidancementioning

confidence: 99%

Consider Hereditary Angioedema in the Differential Diagnosis for Unexplained Recurring Abdominal Pain

Staller

Lembo

Banerji

et al. 2022

Journal of Clinical Gastroenterology

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Health care providers are likely to encounter patients with recurrent unexplained abdominal pain. Because hereditary angioedema (HAE) is a rare disease, it may not be part of the differential diagnosis, especially for patients who do not have concurrent skin swelling in addition to abdominal symptoms. Abdominal pain is very common in patients with HAE, occurring in up to 93% of patients, with recurrent abdominal pain reported in up to 80% of patients. In 49% of HAE attacks with abdominal symptoms, isolated abdominal pain was the only symptom. Other abdominal symptoms that commonly present in patients with HAE include distension, cramping, nausea, vomiting, and diarrhea. The average time from onset of symptoms to diagnosis is 6 to 23 years. Under-recognition of HAE in patients presenting with predominant gastrointestinal symptoms is a key factor contributing to the delay in diagnosis, increasing the likelihood of unnecessary or exploratory surgeries or procedures and the potential risk of related complications. HAE should be considered in the differential diagnosis for patients with unexplained abdominal pain, nausea, vomiting, and/or diarrhea who have complete resolution of symptoms between episodes. As highly effective targeted therapies for HAE exist, recognition and diagnosis of HAE in patients presenting with isolated abdominal pain may significantly improve morbidity and mortality for these individuals.

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“…Generally, clinical symptoms are not specific and may be ignored by patients as they are self-limiting (1). HA rarely mimics an acute abdomen, leading to unnecessary diagnostic laparotomy (3). Abdominal CT reveals the presence of peri-intestinal free fluid, bowel wall edema, and the "bowel target sign" and differentiates HA from other acute abdominal causes.…”

Section: Answermentioning

confidence: 99%

A 25-year-old female with recurrent abdominal pain

Ufuk¹,

Celik²

2022

AGEB

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A 25-year-old female patient, who had a history of medical treatment with the diagnosis of asthma for the last two years, presented with complaints of abdominal pain, nausea, and vomiting. Similar complaints recurred several times over the previous two months and regressed spontaneously in his medical history. These symptoms were acute onset and lasted for 6-8 hours. She had no history of weight loss or fever. Family history revealed the patient’s mother had a history of recurrent swelling of the face and lips, which resolved spontaneously, and her father had coronary artery disease. Physical examination revealed epigastric tenderness without guarding. Labora- tory test results were within normal limits, except for low hemoglobin level (13.2 g/dL; reference range 14-16 g/ dL). Abdominal ultrasound showed mild peri-intestinal free fluid, and contrast-enhanced abdominal computed tomography (CT) was obtained for further evaluation. Abdominal CT showed an edematous thickening of the duodenum wall and peri-intestinal free fluid (Figure 1). The remaining abdominal CT findings were within normal limits.

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Hereditary angio-oedema as a rare cause of small-bowel obstruction

Cited by 4 publications

References 9 publications

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

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

Consider Hereditary Angioedema in the Differential Diagnosis for Unexplained Recurring Abdominal Pain

A 25-year-old female with recurrent abdominal pain

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