Bowman R M, McLone D G, Grant J A, Tomita T, Ito J A
Division of Pediatric Neurosurgery, Children's Memorial Hospital, Northwestern University Medical School, Chicago, IL 60614, USA.
Pediatr Neurosurg. 2001 Mar;34(3):114-20. doi: 10.1159/000056005.
Open spina bifida is the most complex congenital abnormality compatible with long-term survival. This report outlines the 20- to 25-year outcome for our original cohort of patients with a myelomeningocele treated in a nonselective, prospective manner.
Of the initial 118 children, 71 patients were available for our most recent review. Nineteen patients have been lost to follow-up and 28 patients have died. Data were collected on: motor level, shunt status, education/employment, seizure history, mobility, bladder/bowel continence, tethered cord, scoliosis, latex allergy, posterior cervical decompression, tracheostomy and/or gastrostomy tube.
Mortality (24%) continues to climb into young adulthood. Eighty-six percent of the cohort have cerebrospinal fluid diversion, with 95% having undergone at least one shunt revision. Thirty-two percent have undergone a tethered cord release, with 97% having an improvement or stabilization in their preoperative symptoms. Forty-nine percent have scoliosis, with 43% eventually requiring a spinal fusion. Sixteen patients (23%) have had at least one seizure. Eighty-five percent are attending or have graduated from high school and/or college. More than 80% of young adults have social bladder continence. Approximately 1/3 of patients are allergic to latex, with 6 patients having experienced a life-threatening reaction.
At least 75% of children born with a myelomeningocele can be expected to reach their early adult years. Late deterioration is common. One of the greatest challenges in medicine today is establishing a network of care for these adults with spina bifida.
开放性脊柱裂是最复杂的先天性异常疾病，但患者仍可长期存活。本报告概述了我们最初以非选择性、前瞻性方式治疗的脊髓脊膜膨出患者队列在20至25年的随访结果。
最初的118名儿童中，71名患者可供我们进行最新评估。19名患者失访，28名患者死亡。收集的数据包括：运动水平、分流状态、教育/就业情况、癫痫病史、活动能力、膀胱/肠道控尿情况、脊髓栓系、脊柱侧弯、乳胶过敏、颈椎后路减压、气管造口术和/或胃造口术。
死亡率（24%）在青年期仍持续上升。该队列中86%的患者进行了脑脊液分流，其中95%至少进行过一次分流器翻修。32%的患者进行了脊髓栓系松解术，97%的患者术前症状得到改善或稳定。49%的患者患有脊柱侧弯，43%最终需要进行脊柱融合术。16名患者（23%）至少发作过一次癫痫。85%的患者正在上学或已从高中和/或大学毕业。超过80%的青年成人膀胱能自主控尿。约1/3的患者对乳胶过敏，6名患者曾发生危及生命的过敏反应。
预计至少75%患有脊髓脊膜膨出的儿童能够活到成年早期。晚期病情恶化很常见。当今医学面临的最大挑战之一是为这些患有脊柱裂的成年人建立一个护理网络。
