Subscribe to RSS
DOI: 10.1055/s-0038-1672991
Hydrocephalus management in myelomeningocele patients: a systematic review of surgical indications, timing and technical options
Publication History
Publication Date:
06 September 2018 (online)
Introduction: There is still much controversy regarding the optimal management of hydrocephalus in myelomeningocele (MMC) patients. The goal of this study was to search the medical literature to establish the need and what are the indications for surgical treatment for MMC-related ventriculomegaly, what is the usual timing of cerebrospinal fluid (CSF) management and which is the ideal method of CSF diversion.
Methods: A systematic review of the medical literature available on the main databases (PubMed, Embase, Central Cochrane Database and Lilacs) was performed on July 09, 2017, using the following Medical Subject Headings (MeSH): “Hydrocephalus” AND “Meningomyelocele” AND “Therapeutics”. A minimum of 30 patients was defined for article selection. Data extracted included number of patients, type of clinical study, proportion of patients, clinical and radiological indications for treatment, modality of surgical treatment, outcome measures, mortality and risk of bias.
Results: Previous literature provides “all or none” evidence (1C) in support of hypertensive hydrocephalus treatment in MMC patients. Regarding indications, 174 articles were selected by their title. No clinical trials were found. After evaluation of the abstracts, 32 studies were fully analysed. The final review included 17 articles. Fontanelle characteristics, head circumference at birth, and head growth velocity along with imaging features such as increased ventricular index, enlarged fronto-occipital horn ratio and thalamo-occipital distance (TOD) and elongated transversal lateral ventricular atrium (LVA) width were associated with shunt placement requirement. Need for CSF diversion occurred mostly before 6 months of age. Most articles reported ventriculoperitoneal shunt (VPS) as the standard surgical method. Only a few articles focused on endoscopic third ventriculostomy and showed a success rate lower than 50%.
Conclusions: Non-treated progressive hydrocephalus in MMC patients has an adverse prognosis. CSF diversion is recommended for MMC patients presenting with an initial HC at birth higher than 2 standard deviations from average, progressive enlarging ventricles on image studies and/or clinical manifestations of intracranial hypertension such as bulging fontanelle or abnormally increasing HC, usually before the 6th month after birth. Currently, evidence points to ventriculoperitoneal shunt as the best method for hydrocephalus treatment in this context.