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Case Study: Distal Intestinal Obstruction in Cystic Fibrosis

Uncover a unique case of distal intestinal obstruction syndrome in a 20-year-old man with cystic fibrosis, exploring imaging findings and diagnostic considerations. Learn about abdominal manifestations and management strategies in CF patients.

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Case Study: Distal Intestinal Obstruction in Cystic Fibrosis

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  1. An interesting case……BSGAR 2015 Dr James Burn SpR Radiology – Imperial College NHS Trust drjburn@gmail.com

  2. History • 20 year old man • Presented to the Hammersmith Hospital with increasing pain and distension. • Similar episode 6 months previously – resolved spontaneously with conservative management – not imaged at that time • History of previous ‘gastric surgery’ • No other history provided at time of referral – language barrier • ? Obstruction

  3. Review 1 – Plain radiographs at Presentation Multiple distended small and large bowel loops. Suggestion of faecalised material within small bowel. No gas demonstrated within the rectum NB: Midline sternotomy wires and left paramediastinal surgical clips

  4. Review 2 - CT • Multiple dilated loops of large and small bowel • Multiple fluid levels and small bowel faecalisation • Dilated to distal sigmoid with no transition point seen

  5. Review 2 - CT • The appendix is abnormally distended but not overtly inflammed 11 mm

  6. Review 2 - CT • Fundus of stomach wrapped around the gastro-oesophageal junction – the position of which is confirmed anteriorly by the indwelling NG tube Appearances in keeping with a previous Nissen Fundoplication

  7. Review 2 - CT • Pancreatic atrophy and lipomatosis. • Pancreatic calcification suggestive of previous pancreatitis. Low attenuation throughout the small volume pancreas in keeping with complete fatty atrophy. Small focus of calcification within the pancreatic tail.

  8. Review 2 - CT • Bilateral renal calculi

  9. Key Findings • Widespread small and large bowel loop dilatation with multiple fluid levels and small bowel faecalisation – no transition point • Abnormally distended appendix without apparent concomitant inflammation • Previous Nissen fundoplication • Pancreatic calcification and fatty atrophy • Renal cacluli • Evidence of previous cardiothoracic surgery

  10. Final Diagnosis Distal Intestinal Obstruction Syndrome in Cystic Fibrosis

  11. Learning Points: Abdominal Manifestations of CF • Distal Intestinal Obstruction Syndrome (DIOS) • Thought to be caused by pancreatic insufficiency, thickened intestinal secretions, undigested food remnants, poor motility, and faecal stasis • Distal intestinal obstruction syndrome occurs in approximately 15% of CF patients – highest incidence in 20s and 30s. • Pancreatic insufficiency in 90% of CF patients. • Adults with CF more likely to have residual pancreatic function and prone to recurrent pancreatitis.

  12. Abdominal Manifestations of CF • Gastroesophageal reflux very common in CF - 30% by 5 years. May relate to elevated abdominal pressures from chronic cough, hyperinflation, and diaphragm depression. • The appendix may be chronically distended from inspissated secretions – can be difficult to distinguish from appendicitis – the incidence of which is actually reduced in CF. • CFTR protein is expressed in abundance in the kidney - abnormal gene expression results in alterations in the formation of urine - resulting in nephrocalcinosis / urolithiasis • Liver disease: (Not demonstrated on this study) • 2nd leading cause of death in patients with CF. • Approx. 40% of CF patients develop liver disease, but only 1%–5% of these cases progress to portal hypertension and end-stage liver disease.

  13. Final Diagnosis • Cystic fibrosis later confirmed with the acquisition of a comprehensive collateral history. • Patient was again managed conservatively with hydration and conventional phosphate enema. • Treatment options can include: • Water-soluble (e.g. Gastrograffin) contrast enemas can help by resulting in osmotic influx of water into the lumen of the bowel • Intestinal lavage: Reserved for recurrent but not complete obstruction. The aim is to washout the accumulated secretions • Colonoscopy or surgery are rarely necessary

  14. References • Patient registry 2003 annual report. Bethesda, Md: Cystic Fibrosis Foundation, 2004. • Efrati O, Barak A, Modan-Moses D, et al. Liver cirrhosis and portal hypertension in cystic fibrosis. Eur J Gastroenterol Hepatol 2003;15:1073–1078. • Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors, and outcome. Hepatology 2002;36:1374–1382. • Graham N, Manhire AR, Stead RJ, Lees WR, Hodson ME, Batten JC. Cystic fibrosis: ultrasonographic findings in the pancreas and hepatobiliary system correlated with clinical data and pathology. Clin Radiol 1985;36:199–203. • Gilljam M, Chaparro C, Tullis E, Chan C, Keshavjee S, Hutcheon M. GI complications after lung transplantation in patients with cystic fibrosis. Chest 2003;123:37–41. • Littlewood JM. Cystic fibrosis: gastrointestinal complications. Br Med Bull 1992;48:847–859. • Gibney EM, Goldfarb DS. The association of nephrolithiasis with cystic fibrosis. Am J Kidney Dis 2003;42:1–11. • Review of the Abdominal Manifestations of Cystic Fibrosis in the Adult Patient. Radiographics 2006; 26: 3 May-June

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