McEntyre R L, Raffensperger J G
J Pediatr Surg. 1977 Aug;12(4):531-5. doi: 10.1016/0022-3468(77)90192-0.
The Ehlers-Danlos syndrome is a special challenge to the surgeon because of its relative infrequency and the late onset of overt symptoms, such as hyperelastic skin. We should consider this diagnosis in every child with recurrent inguinal herniae and search for its stigmata. A child with a known Ehlers-Danlos syndrome who requires an elective operation should be carefully studied for coagulation disorders. If there is a history of past injuries or operations in which there was poor healing, the benefits of the proposed operation must be carefully weighed against possible complications. Operations must be carried out with great care to avoid tearing the skin and fascia. Meticulous attention is given to hemostasis to prevent postoperative bleeding or hematomas. Inguinal hernias in these children resemble those seen in elderly patients. The transversalis fascia is thin and the internal ring is hugely dilated. An adult-type repair, with the use of mesh or felt may result in a lower incidence of recurrence. In addition, the Ehlers-Danlos syndrome may be yet another etiology for obscure gastrointestinal bleeding in children. Specific enzyme defects have been identified in 2 of the 7 subtypes of this disease. Further, biochemical study of connective tissue defects may contribute to our knowledge of normal collagen synthesis and wound healing.
埃勒斯-当洛综合征对外科医生来说是一项特殊挑战，因为其相对罕见且明显症状出现较晚，如皮肤弹性过度。对于每例复发性腹股沟疝患儿，我们都应考虑这一诊断并寻找其体征。对于需要择期手术的已知埃勒斯-当洛综合征患儿，应仔细检查是否存在凝血障碍。如果有既往受伤或手术愈合不良的病史，必须仔细权衡拟行手术的益处与可能的并发症。手术必须极其小心地进行，以避免撕裂皮肤和筋膜。要特别注意止血，以防止术后出血或血肿形成。这些患儿的腹股沟疝与老年患者的相似。腹横筋膜薄，内环口极大扩张。采用网片或毡片进行成人型修补可能会降低复发率。此外，埃勒斯-当洛综合征可能是儿童不明原因胃肠道出血的另一种病因。在该疾病的7个亚型中的2个亚型中已发现特定的酶缺陷。此外，对结缔组织缺陷的生化研究可能有助于我们了解正常的胶原蛋白合成和伤口愈合。
