Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Inflammatory Bowel Disease Center, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Korean J Radiol. 2023 Apr;24(4):305-312. doi: 10.3348/kjr.2022.0684. Epub 2023 Mar 7.
Chronic enteropathy associated with gene (CEAS) is a recently recognized disease. We aimed to evaluate the enterographic findings of CEAS.
Altogether, 14 patients with CEAS were confirmed based on known mutations. They were registered in a multicenter Korean registry between July 2018 and July 2021. Nine of the patients (37.2 ± 13 years; all female) who underwent surgery-naïve-state computed tomography enterography (CTE) or magnetic resonance enterography (MRE) were identified. Two experienced radiologists reviewed 25 and 2 sets of CTE and MRE examinations, respectively, regarding the small bowel findings.
In initial evaluation, eight patients showed a total of 37 areas with mural abnormalities in the ileum on CTE, including 1-4 segments in six and > 10 segments in two patients. One patient showed unremarkable CTE. The involved segments were 10-85 mm (median, 20 mm) in length, 3-14 mm (median, 7 mm) in mural thickness, circumferential in 86.5% (32/37), and showed stratified enhancement in the enteric and portal phases in 91.9% (34/37) and 81.8% (9/11), respectively. Perienteric infiltration and prominent vasa recta were noted in 2.7% (1/37) and 13.5% (5/37), respectively. Bowel strictures were identified in six patients (66.7%), with a maximum upstream diameter of 31-48 mm. Two patients underwent surgery for strictures immediately after the initial enterography. Follow-up CTE and MRE in the remaining patients showed minimal-to-mild changes in the extent and thickness of the mural involvement for 17-138 months (median, 47.5 months) after initial enterography. Two patients required surgery for bowel stricture at 19 and 38 months of follow-up, respectively.
CEAS of the small bowel typically manifested on enterography in varying numbers and lengths of abnormal ileal segments that showed circumferential mural thickening with layered enhancement without perienteric abnormalities. The lesions caused bowel strictures that required surgery in some patients.
慢性肠病相关基因（CEAS）是一种新认识的疾病。我们旨在评估 CEAS 的肠影像学表现。
共有 14 例 CEAS 患者基于已知的基因突变得到确认。他们于 2018 年 7 月至 2021 年 7 月在一个多中心韩国注册中心登记。确定了 9 名未接受手术的计算机断层肠造影术（CTE）或磁共振肠造影术（MRE）的患者（37.2±13 岁；均为女性）。两名经验丰富的放射科医生分别对 25 组和 2 组 CTE 和 MRE 检查的小肠表现进行了回顾。
在初步评估中，8 例患者在 CTE 上共显示 37 个回肠壁异常区域，其中 6 例有 1-4 个节段，2 例有>10 个节段。1 例 CTE 未见异常。受累节段长度为 10-85mm（中位数 20mm），壁厚度为 3-14mm（中位数 7mm），86.5%（32/37）呈环形，肠期和门静脉期呈分层强化分别为 91.9%（34/37）和 81.8%（9/11）。2.7%（1/37）和 13.5%（5/37）分别有肠周浸润和明显的直血管。6 例（66.7%）患者存在肠狭窄，最大上游直径为 31-48mm。2 例患者在初始肠造影后立即因狭窄而行手术治疗。其余患者在初始肠造影后 17-138 个月（中位数 47.5 个月）的随访 CTE 和 MRE 显示肠壁受累的范围和厚度有最小至轻度变化。2 例患者分别在随访 19 个月和 38 个月时因肠狭窄而再次手术。
小肠 CEAS 在肠造影上的表现通常为数个和多个长度不同的异常回肠段，这些段表现为环形壁增厚，伴有分层强化，无肠周异常。这些病变导致一些患者需要手术的肠狭窄。
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