Unit of Pediatric Neurosurgery, Department of Neurosurgery, University Medical Centre Ljubljana, Ljubljana, Slovenia -
Unit of Pediatric Neurosurgery, Department of Neurosurgery, University Medical Centre Ljubljana, Ljubljana, Slovenia.
J Neurosurg Sci. 2021 Jun;65(3):369-376. doi: 10.23736/S0390-5616.18.04481-8. Epub 2018 Sep 25.
Myelomeningocele (MMC) is a congenital malformation that results from a failure in the process of neurulation. A multidisciplinary follow-up is required to manage and treat all associated sequelae. The aim of the study was to present the epidemiological data and the results of the multidisciplinary follow-up of children born in Slovenia with myelomeningocele (MMC) between 2007 and 2017.
We presented a retrospective analysis of all children born in Slovenia with between 2007 and 2017. The multidisciplinary follow-up included neurosurgical, urological, neurological, endocrinological and orthopedic expertise.
Twenty children were treated in Slovenia for MMC from 2007 to 2017 (mean follow-up of 7.7 years). 9 MMC were thoracic, 2 higher lumbar, 5 lower lumbar and 4 sacral. Thirteen children needed a CSF shunt, 1 was treated with endoscopic ventriculostomy (ETV). Four children needed a craniocervical decompression and 2 needed a detethering procedure. 14 children had a neurogenic bladder and 17 referred bowel continence. Orthopedic correction of the lower limbs was required in 9 cases. 4 children had seizures, 10 had endocrinological deficits. Among 16 children attending school, 11 were inserted in special educational classes.
The prevalence of MMC in Slovenia between 2007 and 2017 was 1/10000 births. Our follow-up results are comparable with those of previous, larger studies and confirm the efficacy of treating hydrocephalus with ETV in selected cases and with CSF shunt only in cases of clearly increased intracranial pressure. By adopting this strategy, we reduced the CSF shunt rate to 65%.
脊髓脊膜膨出(MMC)是一种先天性畸形,是神经管发育过程中失败的结果。需要多学科随访来管理和治疗所有相关的后遗症。本研究的目的是介绍 2007 年至 2017 年在斯洛文尼亚出生的患有脊髓脊膜膨出的儿童的流行病学数据和多学科随访结果。
我们对 2007 年至 2017 年期间在斯洛文尼亚出生的所有儿童进行了回顾性分析。多学科随访包括神经外科、泌尿科、神经科、内分泌科和矫形科专业知识。
2007 年至 2017 年期间,斯洛文尼亚共治疗了 20 例脊髓脊膜膨出患儿(平均随访 7.7 年)。9 例为胸段,2 例为高腰段,5 例为低腰段,4 例为骶段。13 例需要脑脊液分流,1 例接受了内镜脑室造口术(ETV)。4 例需要颅颈减压,2 例需要松解术。14 例患儿有神经源性膀胱,17 例有肠道控便。9 例下肢需要矫形矫正。4 例患儿有癫痫发作,10 例有内分泌缺陷。在 16 名上学的儿童中,11 名被安置在特殊教育班级。
2007 年至 2017 年期间,斯洛文尼亚脊髓脊膜膨出的患病率为 1/10000 出生。我们的随访结果与之前更大规模的研究相似,证实了在选择病例中使用 ETV 治疗脑积水和仅在颅内压明显升高的情况下使用脑脊液分流的有效性。通过采用这种策略,我们将脑脊液分流率降低到 65%。