Sickle cell 'girdle syndrome' progressing to ischaemic colitis and colonic perforation

Clin Lab Haematol. 2006 Feb;28(1):60-2. doi: 10.1111/j.1365-2257.2006.00739.x.

Abstract

Abdominal pain of presumed vasocclusive origin, often termed 'girdle syndrome' because of the circumferential distribution of the pain, is common in sickle cell anaemia (SCA). Evidence of progression to bowel infarction is rare. A 27-year-old man with SCA developed chest and abdominal pain unresponsive to opiate analgesia. Abdominal X-ray showed dilated bowel loops because of partial obstruction. Despite reduction of HbS to 23% by automated red cell exchange, abdominal pain worsened. A CT scan was the most informative investigation and showed free peritoneal air. He underwent emergency hemicolectomy and reversible ileostomy formation. Histology of the resected colon was consistent with acute ischaemic colitis. Early surgical intervention remains essential in SCA when abdominal pain does not respond to maximal therapy including red cell exchange: as this case illustrates, sickle girdle syndrome has the capacity to progress to irreversible ischaemic colitis and necrotic perforation of the bowel wall.

Publication types

  • Case Reports

MeSH terms

  • Abdominal Pain / drug therapy
  • Abdominal Pain / etiology*
  • Abdominal Pain / pathology
  • Abdominal Pain / surgery
  • Adult
  • Analgesics, Opioid / administration & dosage
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / pathology
  • Anemia, Sickle Cell / therapy
  • Colitis, Ischemic / drug therapy
  • Colitis, Ischemic / etiology*
  • Colitis, Ischemic / pathology
  • Colitis, Ischemic / surgery
  • Colostomy / methods
  • Drug Resistance
  • Erythrocyte Transfusion / methods
  • Humans
  • Ileostomy / methods
  • Intestinal Perforation / drug therapy
  • Intestinal Perforation / etiology*
  • Intestinal Perforation / pathology
  • Intestinal Perforation / surgery
  • Male
  • Syndrome

Substances

  • Analgesics, Opioid