Soucy P, Eidus L, Keeley F
Department of Surgery, Children's Hospital of Eastern Ontario, Ottawa, Canada.
J Pediatr Surg. 1990 Nov;25(11):1180-2. doi: 10.1016/0022-3468(90)90759-3.
A 15-year-old girl presented with severe fecal peritonitis due to a large spontaneous colonic perforation. The sigmoid colon was the site of a cluster of white serosal lesions with omental adhesions, of an appearance identical to that of the edges of the perforation. Her father had died at 30 years of age of spontaneous rupture of an iliac artery aneurysm, preceded by rupture of a splenic artery aneurysm and a spontaneous carotid-cavernous fistula. The clinical diagnosis of Ehlers-Danlos syndrome type IV was made, and confirmed by demonstrating that the patient's cultured fibroblasts are not producing or secreting type III collagen. Spontaneous perforation of the colon is a well-described complication of this syndrome, with a high incidence of recurrence. We recommend total abdominal colectomy to minimize the latter possibility.
一名15岁女孩因巨大自发性结肠穿孔出现严重粪性腹膜炎。乙状结肠处有一簇白色浆膜病变并伴有网膜粘连，其外观与穿孔边缘相同。她的父亲30岁时死于髂动脉瘤自发性破裂，之前还有脾动脉瘤破裂和自发性颈动脉海绵窦瘘。临床诊断为IV型埃勒斯-当洛综合征，并通过证明患者培养的成纤维细胞不产生或分泌III型胶原得以确诊。结肠自发性穿孔是该综合征一种广为人知的并发症，复发率很高。我们建议行全腹结肠切除术以尽量降低后者的可能性。
