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Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e3181c15ef8
Image of the Month

Intussusception of the Appendix Causing Small Bowel Obstruction in a Patient With Cystic Fibrosis

Fishman, Douglas S*; Sailhamer, Elizabeth A; Cohen, Daniel T; Mino-Kenudson, Mari§; Doody, Daniel P; Winter, Harland S

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*Section of Pediatric Gastroenterology, Nutrition and Hepatology, Texas Children's Hospital; Houston, USA

Department of Pediatric Surgery, Massachusetts General Hospital, Boston, USA

Radiologic Imaging Consultants, St. Charles, MO, USA

§Department of Pathology, Massachusetts General Hospital, Boston, USA

Department of Pediatric Gastroenterology and Nutrition, MassGeneral Hospital for Children, Boston, USA.

Received 8 June, 2009

Accepted 10 September, 2009

Address correspondence and reprint requests to Douglas S. Fishman, 6621 Fannin St, Clinical Care Center 1010.20, Texas Children's Hospital, Baylor College of Medicine, Houston, TX 77019 (e-mail: dougfishman@gmail.com).

The authors report no conflicts of interest.

Submissions for the Image of the Month should include high-quality TIF endoscopic images of unusual or informative findings. In addition, 1 or 2 other associated photographs, such as radiological or pathological images, can be submitted. A brief description of no more than 200 words should accompany the images. Submissions are to be made online at www.jpgn.org, and will undergo peer review by members of the NASPGHAN Endoscopy and Procedures Committee, as well as by the Journal.

An 18-year-old male with cystic fibrosis (CF) developed acute emesis and abdominal pain. Treatment for distal intestinal obstruction syndrome (DIOS) resolved the acute symptoms, but his pancreatic enzyme dose (>50,000 U · kg−1 · day−1) raised suspicion for fibrosing colonopathy. A prior computed tomography scan showed chronic appendiceal thickening (Fig. 1). Colonoscopy was performed and the right colon appeared thickened with a 5-cm intussuception of the appendix into the cecum (Fig. 2).

Figure 1
Figure 1
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Figure 2
Figure 2
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At elective appendectomy, there was prolapse and intussusception of the appendiceal mucosa and submucosa into the cecal lumen (Fig. 3). This was likely the lead point for a cecal-ascending colon intussuception with intermittent obstruction of the ileocecal valve. Histologically, there was a thickened muscularis mucosae and fibrous expansion of the lamina propria with acute and chronic inflammation (Fig. 4).

Figure 3
Figure 3
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Figure 4
Figure 4
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Intestinal complications of CF include meconium ileus, rectal prolapse, chronic constipation, DIOS, fibrosing colonopathy, and intussusception and volvulus. Fibrosing colonopathy is associated with excess pancreatic enzyme supplementation leading to colonic strictures, frequently right sided (1,2). In the largest series, 9 of 803 patients with CF had appendicitis (3). Intussusception in CF is common, with a prevalence of 1%, primarily ileocolic, but appendiceal intussusception is rare (4). DIOS may mimic intussusception or appendicitis, illustrating the role of endoscopy in establishing an unsuspected diagnosis.

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REFERENCES

1. FitzSimmons SC, Burkhart GA, Borowitz D, et al. High-dose pancreatic-enzymes and supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med 1997; 336:1283–1289.

2. Borowitz DS, Grand RJ, Durie PR. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. J Pediatr 1995; 127:681–684.

3. Shields MD, Levison H, Reisman JJ, et al. Appendicitis in cystic fibrosis. Arch Dis Child 1990; 65:307–310.

4. Holsclaw D, Rocmans C, Shwachman H. Intussusception in patients with cystic fibrosis. Pediatrics 1971; 48:51–58.

© 2010 Lippincott Williams & Wilkins, Inc.

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