Intest Res.  2022 Apr;20(2):274-277. 10.5217/ir.2021.00029.

Endoscopic balloon dilations for strictures of rectum, ileocecal valve and duodenum in a patient with X-linked inhibitor of apoptosis deficiency: a case report

• Otagiri S
• Katsurada T
• Sakurai K
• Sugita J
• Sakamoto N

• KMID: 2529574
• DOI: http://doi.org/10.5217/ir.2021.00029

Abstract

X-linked inhibitor of apoptosis (XIAP) deficiency is a rare primary immunodeficiency and gastrointestinal (GI) lesions in XIAP deficiency are similar to Crohn’s disease. For patients with Crohn’s disease, endoscopic balloon dilation (EBD) is known to be a standard procedure for intestinal strictures including upper GI tract. However, there are no articles which mention the efficacy of EBDs for the strictures in upper GI tract in patients with XIAP deficiency. Herein, we describe an 18-year-old male with XIAP deficiency in whom EBDs for the rectum, ileocecal valve (ICV), and duodenum were performed. Before hematopoietic stem cell transplantation (HSCT), GI endoscopy revealed strictures of the rectum, ICV and duodenum with active ulcers. Although these ulcers healed after HSCT, the strictures progressed. Therefore, we performed EBDs for the strictures of the rectum, ICV, and duodenum. In contrast studies, we did not find any other strictures in the small intestine. Throughout the patient’s clinical course, no complications of EBD occurred. He started eating after EBDs, but abdominal symptoms did not relapse without any dietary restrictions. Our case suggests that EBD could be an effective and safe procedure for intestinal strictures including upper GI tract after HSCT in patients with XIAP deficiency.

Keyword

Endoscopy; Gastrointestinal diseases; Hematopoietic stem cell transplantation; X-linked inhibitor of apoptosis protein; Case reports

Figure

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  Fig. 1. Endoscopic images of the large intestine. Before hematopoietic stem cell transplantation (HSCT), multiple longitudinal ulcers in the transverse colon (A) and descending colon (B) were observed. After HSCT, these ulcers were healing in the transverse colon (C) and descending colon (D).

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  Fig. 2. Endoscopic and fluoroscopic images of the rectum. Before hematopoietic stem cell transplantation (HSCT), the rectum was mildly stenosed with active ulcers (A). After HSCT, active ulcers were healing; however, the stricture of the rectum progressed (B). We performed endoscopic balloon dilations for the rectum up to 12 mm (C, D).

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  Fig. 3. Endoscopic and fluoroscopic images of the ileocecal valve (ICV). Before hematopoietic stem cell transplantation (HSCT), the ICV was severely stenosed with an active ulcer (A). After HSCT, the active ulcer was healing, and the stricture of the ICV remained severe (B). We performed endoscopic balloon dilations for the ICV up to 10 mm (C, D).

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  Fig. 4. Endoscopic and fluoroscopic images of the duodenum. Before hematopoietic stem cell transplantation (HSCT), the duodenum bulb was mildly stenosed with an active ulcer (A). After HSCT, the active ulcer was healing; however, the stricture of the duodenum progressed (B). We performed endoscopic balloon dilations for the rectum up to 10 mm (C, D).

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  Fig. 5. Fluoroscopic images of the small intestine (A, B). In contrast studies, no other stricture in the small intestine was observed.

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