IV型埃勒斯-当洛综合征：儿童自发性乙状结肠穿孔和肠腔瘘罕见病因的病例报告

Kodikara Hemal, King Sebastian K, McLeod Elizabeth

Department of Paediatric Surgery, The Royal Children's Hospital, Melbourne, Australia.

Department of Paediatric Surgery, Christchurch Hospital, Christchurch, New Zealand.

Surg Case Rep. 2020 Dec 8;6(1):312. doi: 10.1186/s40792-020-01051-0.

BACKGROUND

Ehlers-Danlos syndrome (EDS) type IV is a rare subtype of EDS, but has important surgical implications. Case presentation Here, we present a case of a spontaneous sigmoid perforation in a 14-year-old boy. He was initially treated with laparotomy, oversew of the sigmoid perforation and a diverting ileostomy. He developed a complete wound dehiscence and enteroatmospheric fistulae. These were managed with a combination of negative pressure wound therapy and Eakin (TG Eakin™) pouch changes. We discuss the clinical features and presentation of EDS type IV, the surgical implications of managing patients with the condition, and the challenges in management of enteroatmospheric fistulae in children.

CONCLUSIONS

Ehlers-Danlos syndrome type IV should be considered as a cause of any spontaneous colonic perforation in children.

背景

IV型埃勒斯-当洛综合征（EDS）是EDS的一种罕见亚型，但具有重要的手术意义。病例报告在此，我们报告一例14岁男孩自发性乙状结肠穿孔的病例。他最初接受了剖腹手术、乙状结肠穿孔缝合及转流性回肠造口术。他出现了完全性伤口裂开和肠-腹壁瘘。这些情况通过负压伤口治疗和更换伊金（TG Eakin™）袋进行处理。我们讨论了IV型EDS的临床特征和表现、治疗该疾病患者的手术意义以及儿童肠-腹壁瘘管理中的挑战。