Successful Surgical Treatment of Pediatric Intestinal Behçet's Disease with Ileocecal Ulcer Refractory to Medical...

High postoperative recurrence and reoperation rates following surgical treatment for intestinal Behçet's disease (BD) have been reported in adults. In pediatric patients, reports on surgical treatment and clinical course are extremely rare.

An 11-year-old girl was diagnosed with intestinal BD with an ileocecal ulcer at the age of 2 and had been maintained on medical therapy. At the age of 8, she was hospitalized due to exacerbation of the intestinal lesions. Despite adjustment of immunosuppressive agents and other medical management, her condition did not improve. One month after admission, she developed a fever of 40°C, vomiting, and elevated inflammatory markers. Contrast-enhanced CT revealed marked wall thickening from the ileocecal region to the transverse colon, accompanied by intramural abscess formation and surrounding inflammation. She was diagnosed with a refractory ileocecal ulcer resistant to medical therapy, and laparoscopic right hemicolectomy with stoma creation was performed. Her postoperative course was uneventful, and she was discharged. As she remained in remission without recurrence, stoma closure was performed 17 months after the initial surgery following steroid tapering. She has remained relapse-free for approximately 3 years since the first operation.

In this case, postoperative recurrence was a substantial concern. However, surgical intervention ultimately led to marked improvement of the disease, and the patient was able to maintain a more stable daily life compared with the preoperative period. This case represents a valuable example in which surgical treatment was remarkably effective for pediatric intestinal BD.