Stark G D, Drummond M
Arch Dis Child. 1973 Sep;48(9):676-83. doi: 10.1136/adc.48.9.676.
In a 6-year period 163 unselected newborn infants with open myelomeningocele were assessed. 48%, considered to have the most favourable prognosis, were selected for early back closure and vigorous subsequent treatment. The more severely affected remainder were given only symptomatic treatment unless long-term survival seemed likely. The major adverse criteria were severe paralysis, gross head enlargement, and spinal deformity such as kyphosis. More than 70% of selected infants were alive at 6 years, whereas more than 80% of untreated infants were dead at 3 months. The follow-up results are compared with those of routine early operation (Lorber, 1971a). After 1 year, the overall survival levelled out at 40% (cf 60%). The survivors were, however, less severely handicapped. Only 25% were mainly or entirely in wheelchairs (cf 49%). Only 16% had upper urinary tract damage in addition to incontinence (cf 34%). A combination of mental and severe physical handicap occurred in only 20% (cf 36%). Severe disability was encountered mainly in children who had suffered from complications, e.g. ventriculitis, unforeseeable at birth. It is concluded that the overall results of selective early operation compare favourably with those of purely conservative management on the one hand, and with routine early operation on the other. It is suggested that this policy offers the best prospects of independence for the less severely affected child, and the least distress and suffering for the grossly afflicted.
在6年期间,对163例未经挑选的开放性脊髓脊膜膨出新生儿进行了评估。其中48%被认为预后最佳,被选作早期背部闭合手术及后续积极治疗。其余病情较重的婴儿仅给予对症治疗,除非看起来有可能长期存活。主要的不利标准为严重瘫痪、明显的头部增大以及脊柱畸形如脊柱后凸。超过70%的入选婴儿在6岁时存活,而超过80%未经治疗的婴儿在3个月时死亡。将随访结果与常规早期手术的结果(Lorber,1971a)进行了比较。1年后,总体存活率稳定在40%(对比60%)。然而,存活者的残疾程度较轻。只有25%主要或完全依靠轮椅(对比49%)。除了大小便失禁外,只有16%有上尿路损害(对比34%)。智力和严重身体残疾同时存在的情况仅占20%(对比36%)。严重残疾主要出现在患有并发症(如脑室炎)的儿童中,这些并发症在出生时无法预见。结论是,选择性早期手术的总体结果一方面优于单纯保守治疗,另一方面优于常规早期手术。有人认为,这一策略为病情较轻的儿童提供了最佳的独立前景,为病情严重的儿童带来了最少的痛苦和折磨。