Atala A, Bauer S B, Dyro F M, Shefner J, Shillito J, Sathi S, Scott R M
Division of Urology, Children's Hospital, Boston, Massachusetts.
J Urol. 1992 Aug;148(2 Pt 2):592-4. doi: 10.1016/s0022-5347(17)36662-4.
From 1986 to 1991, 12 boys and 23 girls underwent surgery for lipomyelomeningocele removal. Of these patients 29 were 15 months old or younger (average age 3 months), while 6 were 4.5 to 19 years old (average age 10 years). Preoperative and postoperative urodynamic studies, including external urethral sphincter electromyography, were done on everyone. All 29 infants had a cutaneous lesion overlying the lower back and 14 had an abnormal neurological examination. Preoperative urodynamic studies were abnormal in 11 patients, consisting of an upper motor neuron lesion in 6, and a mixed upper and lower motor neuron lesion in 5. Postoperatively, 10 of 14 children with an abnormal neurological examination improved, while 9 of 11 with abnormal lower urinary tract function normalized. In 1 of 18 children (6%) with normal preoperative urodynamic studies detrusor-sphincter dyssynergia developed postoperatively. In all 6 older children urinary incontinence developed, and this led to the diagnosis. Everyone had an abnormal neurological examination and abnormal preoperative urodynamic studies. One child had a lower motor neuron lesion, and 5 had a mixed upper and lower motor neuron lesion. Postoperatively, the neurological examination improved in only 1 patient (16%), and the urological symptoms and urodynamic findings improved in another child. Lipomyelomeningocele has a progressive effect on lower spinal cord function because infants tend to present with fewer urinary manifestations and physical findings than older children. Individuals who escape early detection tend to have a more subtle cutaneous abnormality. As a result, older children are more likely to present with urological and neurological complaints. Surgical correction in infancy provides a degree of reversibility not seen in older children. It is imperative that early identification, evaluation and treatment be undertaken to prevent this progression and permanency of neurological changes and urinary dysfunction.
1986年至1991年期间，12名男孩和23名女孩接受了脂肪瘤型脊髓脊膜膨出切除术。这些患者中，29名年龄在15个月及以下（平均年龄3个月），6名年龄在4.5至19岁（平均年龄10岁）。对所有患者均进行了术前和术后尿动力学研究，包括尿道外括约肌肌电图检查。所有29名婴儿在下背部均有皮肤病变，14名神经学检查异常。术前尿动力学研究异常的患者有11例，其中6例为上运动神经元损伤，5例为上、下运动神经元混合损伤。术后，14名神经学检查异常的儿童中有10名有所改善，11名下尿路功能异常的儿童中有9名恢复正常。18名术前尿动力学研究正常的儿童中有1名（6%）术后出现逼尿肌-括约肌协同失调。所有6名年龄较大的儿童均出现尿失禁，由此得以确诊。所有人神经学检查均异常，术前尿动力学研究也均异常。1名儿童为下运动神经元损伤，5名儿童为上、下运动神经元混合损伤。术后，只有1名患者（16%）神经学检查有所改善，另1名儿童的泌尿系统症状和尿动力学检查结果有所改善。脂肪瘤型脊髓脊膜膨出对脊髓下段功能有渐进性影响，因为婴儿比年龄较大的儿童往往表现出较少的泌尿系统症状和体征。未被早期发现的个体往往有更隐匿的皮肤异常。因此，年龄较大的儿童更有可能出现泌尿系统和神经学方面的症状。婴儿期进行手术矫正可提供一定程度的可逆性，这在年龄较大的儿童中未见。必须尽早进行识别、评估和治疗，以防止神经学改变和泌尿系统功能障碍的进展及永久性损害。
