Department of Neurological Sciences, Christian Medical College Hospital, Vellore, Tamil Nadu, 632004, India.
Childs Nerv Syst. 2021 May;37(5):1623-1632. doi: 10.1007/s00381-020-05000-y. Epub 2021 Jan 6.
To determine the functional outcomes and risk factors for outcomes following surgery for lipomyelomeningocele (LMMC).
Data from 109 children with LMMC who underwent surgery from January 2008 to December 2017 were retrospectively studied to evaluate functional outcomes and possible risk factors for early- (at discharge from hospital or within 1 month of surgery) and long-term outcomes after surgery.
There were 53 boys and 56 girls with median age of 36 months (IQR 12-90 months; range, 4 months to 18 years) at surgery. At presentation, neurological function was normal (asymptomatic group) in 28 (25.7%) children while there was neurological dysfunction (symptomatic group) in 81 (74.3%). Near total or radical excision of lipoma was done in 71 (65.1%) children. Six (5.8%) children had deterioration of neurological function in the post-operative period with recovery of function in three of them. There were no statistically significant risk factors for early outcomes. At a mean follow-up of 62.5 months (IQR 35-82 months; range, 12-146 months), 21 (27.6%) of the 76 symptomatic group children (including 59.3% with incomplete bladder dysfunction) showed improvement, 52 (68.4%) remained the same, while 3 (4%) deteriorated. In 31 children (symptomatic group) who developed symptoms after 1 year of age, early surgery after development of symptoms was associated with better chances of recovery (p = 0.0008). In the asymptomatic group, 23 (88.5%) had normal neurological function at mean follow-up of 57.5 months (IQR 30-77 months; range, 12-141 months) and 3 (11.5%) had neurological deterioration. The mean time to late deterioration (re-tethering) from surgery was 51.3 months (IQR 24-75 months; range, 24-84 months). Presence of syrinx on initial MRI (p = 0.008) and partial resection of the lipoma (p = 0.02) were independent risk factors for delayed deterioration.
Radical resection of LMMC probably helps in preserving neurological function in > 90% of children at long-term follow-up. Fifty-five percent of children > 2 years of age with incomplete bladder dysfunction regained normal function following surgery. In children who develop symptoms after birth, early surgical intervention can reverse the neurological deficits.
确定脂肪脊膜脊髓膨出（LMMC）手术后的功能结果和结局的相关因素。
回顾性分析 2008 年 1 月至 2017 年 12 月期间 109 例接受手术治疗的 LMMC 患儿的数据，以评估术后早期（出院或手术后 1 个月内）和长期的功能结果和可能的相关因素。
患儿中男孩 53 例，女孩 56 例，中位年龄 36 个月（IQR 12-90 个月；范围，4 个月至 18 岁）。术前，28 例（25.7%）患儿神经功能正常（无症状组），81 例（74.3%）患儿神经功能障碍（有症状组）。71 例（65.1%）患儿行脂肪瘤近全切除或根治性切除。术后 6 例（5.8%）患儿神经功能恶化，其中 3 例功能恢复。早期结局无统计学意义的相关因素。中位随访时间 62.5 个月（IQR 35-82 个月；范围，12-146 个月）时，76 例有症状组患儿中有 21 例（27.6%，包括 59.3%有不完全膀胱功能障碍）得到改善，52 例（68.4%）保持不变，3 例（4%）恶化。31 例（有症状组）患儿在 1 岁后出现症状，症状出现后早期手术与恢复机会增加相关（p = 0.0008）。无症状组患儿中，23 例（88.5%）在中位随访 57.5 个月（IQR 30-77 个月；范围，12-141 个月）时神经功能正常，3 例（11.5%）神经功能恶化。术后晚期恶化（再栓系）的平均时间为 51.3 个月（IQR 24-75 个月；范围，24-84 个月）。初始 MRI 上存在脊髓空洞（p = 0.008）和脂肪瘤部分切除（p = 0.02）是延迟恶化的独立相关因素。
脂肪脊膜脊髓膨出的根治性切除可能有助于 90%以上的患儿在长期随访中保持神经功能。55%的大于 2 岁且膀胱功能不全的患儿在手术后恢复正常功能。对于出生后出现症状的患儿，早期手术干预可逆转神经功能缺损。
