Intussusception of the Appendix in a Young Adult with Cystic Fibrosis: An Important Differential Diagnosis of Abdominal Pain in Cystic Fibrosis Patients? (2022)

Type of publication:
Conference abstract

*Venkatasami M.; *Cobby E.

British Journal of Surgery. Conference: ASiT Surgical Conference 2022. Aberdeen United Kingdom. 109(Supplement 6) (pp vi57), 2022. Date of Publication: September 2022.

Background: Cystic fibrosis (CF) is commonly associated with gastrointestinal manifestations from infancy to adulthood. Intussusception in the paediatric CF population is widely reported, whereas in the adult, distal intestinal obstruction syndrome (DIOS) is common affecting 20%, of which, intussusception is rare and appendiceal intussusception is rarer, affecting 1%. Case-Description: A 20-year-old male with CF presented with 3 days of right iliac fossa pain and diarrhoea. On admission, he was hypotensive and afebrile. Clinical examination revealed tenderness of the right flank with a palpable mass in the right iliac fossa. Serum investigations showed mildly raised inflammatory markers. Contrast CT of the abdomen-pelvis confirmed intussusception of the appendix into the ascending colon. Histological analysis of the appendix further demonstrated intussusception, with intraluminal mucinous material. Fascinatingly, no transmural inflammation was present. The patient underwent a laparotomy-open appendicectomy. Interestingly, intraoperative findings showed the intussusception resolved, no longer requiring surgical reduction. Patient postoperative recovery was complicated by pneumonia for which he was transferred for specialist CF respiratory care and thereafter, discharged. Discussion(s): Literature review highlighted a paucity of data, with 10 reported cases of appendiceal intussusception in adult CF patients. Interestingly, we report the intussusception had reduced by the time of operation. This is in-keeping with previous case reports of transient intussusception which spontaneously resolved. Conclusion(s): From this rare case presentation, we have learnt it is imperative to carry a high index of suspicion for gastrointestinal manifestations in CF patients where acute appendicitis is seen less often and differential diagnoses like DIOS and intussusception should be considered.

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