Child Life and Health, University of Edinburgh.
Department of Paediatric Gastroenterology and Nutrition.
J Pediatr Gastroenterol Nutr. 2019 Nov;69(5):539-543. doi: 10.1097/MPG.0000000000002444.
Stricturing duodenal Crohn disease (CD) is a rare but serious presentation of CD causing significant morbidity. We aim to provide the first robust incidence data and case studies on this severe presentation in children.
A regional cohort of prospectively acquired incident cases of paediatric CD diagnosed <16 years of age in South-East Scotland was captured over a 19-year period (1999-2018). A retrospective review was conducted on the medical records of all patients together with a review of the available literature and consensus guidelines. Incidence rates for all CD and for duodenal stricturing CD were calculated.
A total of 247 new cases of paediatric CD were diagnosed within the study period. Median age at diagnosis was 12.5 years with 62% male predominance. Overall paediatric CD incidence rate was 5.70/100,000/year with a specific duodenal B2 phenotype disease incidence rate of 0.05/100,000/year; representing 0.8% of incident cases at diagnosis. Two incident cases of stricturing duodenal CD presented with systemic symptoms of weight loss, abdominal pain, anorexia, and lethargy, together with persistent vomiting suggestive of obstruction. Both cases partially responded to intensive medical therapy but eventually required laparoscopic gastroduodenostomy. A detailed literature search confirmed there are no paediatric incidence data, guidelines, or case reports relating to duodenal stricture as either a presentation or complication of CD.
Duodenal structuring disease is a rare but serious presentation of CD causing significant morbidity and not currently covered in the paediatric literature or consensus guidelines. Best practice medical and surgical management remain uncertain and require further research.
狭窄性十二指肠克罗恩病（CD）是一种罕见但严重的 CD 表现形式，会导致严重的发病率。我们旨在提供有关这种严重儿童表现形式的首次可靠发病率数据和病例研究。
在苏格兰东南部，通过前瞻性获取的 19 年时间（1999-2018 年），对<16 岁诊断为小儿 CD 的患者进行了区域性队列研究。对所有患者的病历进行了回顾性审查，并对现有文献和共识指南进行了审查。计算了所有 CD 和十二指肠狭窄性 CD 的发病率。
在研究期间共诊断出 247 例新的小儿 CD 病例。中位诊断年龄为 12.5 岁，男性占 62%。总体小儿 CD 发病率为 5.70/100,000/年，特定十二指肠 B2 表型疾病发病率为 0.05/100,000/年；占诊断时发病例的 0.8%。2 例狭窄性十二指肠 CD 病例表现为体重减轻、腹痛、食欲不振和乏力等全身症状，以及持续性呕吐提示梗阻。这两个病例对强化药物治疗有部分反应，但最终需要腹腔镜胃十二指肠吻合术。详细的文献检索证实，目前尚无有关儿童发病率数据、指南或病例报告涉及 CD 的十二指肠狭窄作为表现或并发症。
十二指肠结构疾病是一种罕见但严重的 CD 表现形式，会导致严重的发病率，目前在儿科文献或共识指南中并未涵盖。最佳的医疗和手术管理实践仍然不确定，需要进一步研究。
