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We refer to the article by Wilschanski and Durie (Gut 2007;56:1153–63) in which they addressed the challenges of establishing or excluding a cystic fibrosis (CF) diagnosis in patients who present de novo in adulthood.
We present a 44 year old male CF patient who presented with a 24 hour history of intermittent and colicky central abdominal pain and vomiting. Chronic constipation remained a persistent problem for him for several years despite regular use of laxatives. There was no history of any abdominal surgery.
On examination he was apyrexial but tachycardiac. His abdomen was soft, distended, with localised tenderness in the right iliac fossa. There were no palpable abdominal masses and bowel sounds were normal as was rectal examination. Blood results showed white cell count of 17.3, C reactive protein of 34 with normal urea and electrolytes, and liver function tests. Abdominal and pelvic CT scan (fig 11)) demonstrated several dilated loops of small bowel in the left upper quadrant, with a transitional zone in the right side of the abdomen that appeared to contain a stricture. No free fluid or air was seen. No mass lesion was identified.
He underwent an emergency laparotomy for obstruction at which there was no evidence of a small bowel stricture but there was evidence of an inflamed and grossly distended appendix. Viscid, semi‐solid secretions were palpable in the lumen of the ileum up to the ileo‐caecal junction. Appendicectomy was performed and the lumen showed thick inspissated mucus. Histological appearances were consistent with early acute appendicitis and mucus retention with evidence of crypt abscesses.
The post‐operative period was complicated by prolonged ileus and he was finally discharged from hospital on day 13 postoperatively.
Studies showed that there is an associated increased risk of certain colonic disorders and the emergence of other previously unrecognised disorders in adult CF patients, like distal intestinal obstruction syndrome ,which may be complicated by intussusception, appendiceal disease, volvulus, Crohn's disease, fibrosing colonopathy and colonic carcinoma.1
The diagnosis of acute appendicitis although uncommon in patients with CF is often delayed, and appendiceal abscess is a frequent complication and remains a diagnostic challenge. The large numbers of intra‐abdominal conditions in CF, which can cause pain and be confused with acute appendicitis, are contributing factors in the misdiagnosis of the early stages of acute appendicitis. The true underlying condition is further masked by the concurrent use of antibiotics for pulmonary infection.2
In a retrospective autopsy review of 51 CF patients it was shown that in 49 of 51 instances the mucosa of the appendix was hyperplastic, and the mucosal glands were distended with eosinophilic secretions. In 12 cases (24%) the appendix itself was grossly firm, dilated and distended, although the mucosal wall was free of inflammation. This lends credence to the suggestion that these inspissated secretions may be protective against the occurrence of appendicitis, the incidence of which may be as low as 1–2% among CF patients.3
Appendiceal disease in CF patients represents a spectrum ranging from simple mucous distension to acute appendicitis with perforation. CF patients with pain secondary to a non‐inflamed distended appendix represent a distinct syndrome cured by appendicectomy.4
Following laparotomy in CF patients, prolonged postoperative ileus is not uncommon and hence a conservative approach with or without total parenteral nutrition should be considered.
In conclusion, adult CF patients with refractory or unexplained intestinal symptoms merit thorough investigations and a higher index of suspicion. Diagnostic abdominal CT and/or ultrasonography should be considered when patients with CF present with pain, prolonged fever and abdominal mass or fluid discharge from the right flank.
Competing interests: None.