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An unusual complication of familial Mediterranean fever: intestinal volvulus and necrosis

Gülay A Tireli1, Murat Unal, Serdar Sander

  • 1Department of Pediatric Surgery, Bakirköy Maternity and Children's Hospital, Istanbul, Turkey. gultireli@yahoo.com

The Turkish Journal of Gastroenterology : the Official Journal of Turkish Society of Gastroenterology
|August 31, 2006
PubMed

Insights

Familial Mediterranean fever (FMF) can cause rare, life-threatening abdominal emergencies like bowel necrosis and obstruction. Early recognition is crucial to prevent unnecessary surgeries and manage this severe complication of FMF.

Area of Science:

  • Internal Medicine
  • Genetics
  • Gastroenterology

Background:

  • Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder.
  • It typically presents with recurrent inflammatory attacks, often involving the peritoneum (over 95% of patients).
  • Peritoneal involvement can mimic acute abdomen, leading to potential misdiagnosis and unnecessary surgical interventions.

Observation:

  • The study highlights two cases of FMF patients who experienced rare abdominal complications.
  • Both patients underwent laparotomy due to FMF-related abdominal issues.
  • Surgical intervention included segmental small bowel resection necessitated by necrosis.

Findings:

  • Adhesive intestinal obstruction, bowel strangulation, and volvulus are identified as rare but serious complications of FMF.
  • These complications arose in patients with diagnosed Familial Mediterranean fever.
  • The cases underscore the potential for severe gastrointestinal manifestations in FMF.

Implications:

  • Physicians should consider FMF in the differential diagnosis for patients presenting with acute abdominal symptoms, especially those with a known history.
  • Awareness of these rare, life-threatening complications can guide timely and appropriate management.
  • Prompt diagnosis and management of FMF-related abdominal emergencies are critical to improve patient outcomes and avoid surgical complications.

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