Surgery, Princess Alexandra Hospital, Woolloongabba, Queensland, Australia.
Surgery, Greenslopes Private Hospital, Brisbane, Queensland, Australia
BMJ Case Rep. 2020 Sep 23;13(9):e234670. doi: 10.1136/bcr-2020-234670.
A 35-year-old man with Ehlers-Danlos syndrome type IV (EDS IV) underwent surgical repair of an enteroatmospheric fistula. Despite the substantially increased operative risk, repair was undertaken in view of his poor quality of life and severe nutritional deficits. Dense adhesions and extremely fragile bowel and vasculature characteristic of EDS IV were encountered intraoperatively. Multiple traction enterotomies and faecal matter leaking from suture holes necessitated leaving the abdomen open for a prolonged period. An Abdominal Reapproximation Anchor device was applied to prevent lateral retraction of the abdominal wall during this time. At relook on day 6, no leak was found, and the abdomen was closed. Two years postoperatively, the patient has an intact abdominal wall, with a vastly improved quality of life. This case illustrates the challenges of operating on patients with EDS IV, and presents a novel technique in managing fistulas in these patients.
一位 35 岁的 Ehlers-Danlos 综合征 IV 型（EDS IV）患者接受了肠气瘘的手术修复。尽管手术风险显著增加，但鉴于他的生活质量差和严重的营养不足，仍进行了修复。术中发现了 EDS IV 特有的致密粘连和极其脆弱的肠管和血管。多次牵引肠切开术和从缝线孔漏出的粪便使腹部需要长时间敞开。在此期间，应用腹壁重新接近锚定装置以防止腹壁侧向回缩。在第 6 天再次检查时，未发现漏出，遂关闭了腹部。术后两年，患者腹壁完整，生活质量大大改善。该病例说明了在 EDS IV 患者中进行手术的挑战，并提出了一种管理这些患者瘘管的新方法。
