Ileal Peduculated Tumor in a Patient With Crohn’s Disease

2009

Question: A 42-year-old man presented to the emergency department with a 2-day history of crampy abdominal pain, abdominal distention, and vomiting. He had undergone emergent operation for perforation followed by small bowel obstruction 2 years earlier in another hospital, when he was diagnosed with Crohn’s ileitis for the first time. He was referred to our hospital 1 year later for further examination and management, and underwent fluoroscopic enteroclysis and abdominal contrastenhanced computed tomography, which revealed multiple mild strictures with wall thickness, longitudinal ulcers, and cobblestone appearance in the intrapelvic ileum. He was prescribed 50 mg (0.82 mg/kg body weight) azathioprine for 4 months in addition to 5-aminosalycylate and an elemental diet.

On examination, he was afebrile and had abdominal distention with tenderness on palpation. Laboratory tests detected elevated C-reactive protein level (5.66 mg/dL; normal, 0.00 – 0.30). His hemoglobin was 14.1 g/dL (normal, 13.0 –18.0), and white cell count 4,300/L (normal, 3,800 – 8,500). Contrast-enhanced computed tomography demonstrated a 25-mm pedunculated enhancing polypoid lesion in the dilated ileum (Figure A). This was not accessible on double-balloon endoscopy (DBE) via the anal route owing to stricture with longitudinal ulceration in the terminal ileum, but was delineated with Gastrografin injected through the working channel of the endoscope, appearing relatively soft with compression (Figure B). After fasting and total parenteral nutrition for 3 weeks, he underwent the second anal DBE, which passed the resolved stricture and then detected the polypoid lesion with partial occlusion of the mid-ileum accompanied by longitudinal scar (Figure C). What is the diagnosis and appropriate management for this lesion?

Answer to the Clinical Challenges and Images in GI Question: Image 2 (page 1223): Ileal Capillary Hemangioma in a Patient With Crohn’s Disease
The 30-mm mass was successfully resected in 2 pieces after submucosal injection with a saline-epinephrine solution and retrieved at DBE. One of those 2 pieces was cut in 2 during retrieval, and the specimens consisted of 3 pieces 25 x 25 x 10 mm, 20 x 15 x 10 mm, and 15 x 10 x 10 mm in diameter. The largest piece is shown in Figure D. Hematoxylin and eosin-stained sections (Figure E) showed lobular arrangement of small, thin-walled, closely packed vessels immunoreactive to vascular endothelial markers CD31 and CD34 (Figure F), which confirmed capillary hemangioma. On postpolypectomy days 4 and 5, bleeding occurred with a slight decrease in the hemoglobin level to 12.8 g/dL, but resolved by fasting only. He was discharged 11 days later.

Intestinal hemangiomas are rare, with an incidence of about 1 per 15,000 patients. About one half of these hemangiomas are associated with cutaneous hemangiomas, most commonly in the blue rubber bleb nevus syndrome and rarely in Klippel–Trenaunay syndrome. Intestinal hemangiomas are capillary, cavernous, or mixed. Bleeding lesions are generally large and more likely to be cavernous than capillary hemangiomas. Gastrointestinal lesions occasionally intussuscept and cause gastrointestinal obstruction. Most cases were treated operatively, but recently endoscopic hemostasis and polypectomy have been reported.1 The association between capillary hemangioma and Crohn's disease treated by immunosuppressant remains unclear, although there are some reports that cutaneous tufted angioma developed in the recipient of a liver transplant and in a patient with Crohn's disease treated by infliximab and azathioprine.2 To avoid multiple operations in a patient with Crohn's disease, the less invasive enteroscopic resection of the pedunculated polypoid lesion was possible and may be preferable to surgery.3

References

  1. Cappell M. Gastrointestinal vascular malformations or neoplasms: arterial, venous, arteriovenous, and capillary. In Yamada T, ed: Textbook of Gastroenterology (4th ed). Philadelphia: Lippincott Williams & Wilkins; 2003:2722–2741.
  2. Al-Za’abi AM, Ghazarian D, Greenberg GR, et al. Eruptive tufted angiomas in a patient with Crohn's disease. J Clin Pathol 2005;58:214 –216.
  3. Ohmiya N, Taguchi A, Shirai K, et al. Endoscopic resection of Peutz-Jeghers polyps throughout the small intestine at double-balloon enteroscopy without laparotomy. Gastrointest Endosc 2005;61:140 –147.