ORIGINAL ARTICLE   

Journal of Neurosurgical Sciences 2021 June;65(3):369-76

DOI: 10.23736/S0390-5616.18.04481-8

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

Myelomeningocele in Slovenia: results of a 10-year follow-up

Peter SPAZZAPAN ✉, Tomaž VELNAR

Unit of Pediatric Neurosurgery, Department of Neurosurgery, University Medical Centre Ljubljana, Ljubljana, Slovenia

BACKGROUND: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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%.

KEY WORDS: Myelomeningocele; Hydrocephalus; Arnold-Chiari malformation; Neural tube defects; Urinary bladder, neurogenic; Seizures