Shian W J, Chi C S, Wong T T
Department of Pediatrics, Taichung Veterans General Hospital, Taiwan, R.O.C.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1994 Jan-Feb;35(1):57-62.
Fourteen infants and children, 6 males & 8 females, with lipomyelomeningoceles were reviewed from October 1982 to December 1991 at Taichung Veterans General Hospital. The age at diagnosis ranged from 3 days to 5 years. The chief problems included mass on the back, urinary distention, weakness of lower limbs and poor bowel control. The cutaneous lesions over the lumbosacral region were subcutaneous lipoma, dimples and hemangioma. There were several associated anomalies, such as occult spina bifida, syringomyelia, sacrococcygeal dysgenesis, high-types imperforate anus, genitourinary anomalies, congenital heart disease, talipes equinovarus and annular pancreas. Among the 12 cases who were operated on, 8 had preoperative neurological deficits, but there was only little postoperative improvement in 2. It is important to recognize at an early stage the defect underlying skin lesions and its associated anomalies, rather than to be concerned about cosmetic factors. Early surgical repair before the appearance of neurological deficits is recommended.
1982年10月至1991年12月期间,台中荣民总医院对14例患有脂肪脊髓脊膜膨出的婴幼儿进行了回顾性研究。其中男性6例,女性8例。诊断时的年龄范围为3天至5岁。主要问题包括背部肿块、尿潴留、下肢无力和排便控制不佳。腰骶部的皮肤病变包括皮下脂肪瘤、酒窝和血管瘤。还存在一些相关的异常情况,如隐性脊柱裂、脊髓空洞症、骶尾发育不全、高位肛门闭锁、泌尿生殖系统异常、先天性心脏病、马蹄内翻足和环状胰腺。在接受手术的12例病例中,8例术前存在神经功能缺损,但术后仅有2例有轻微改善。重要的是要早期识别皮肤病变及其相关异常的潜在缺陷,而不是关注美容因素。建议在神经功能缺损出现之前尽早进行手术修复。