Elke Januschek1,2*, Andreas Röhrig2, Sandra Kunze2, Christian Fremerey3, Bea Wiebe3 and Martina Messing-Jünger2
1Department of Neurosurgery, Sana Klinikum Offenbach GmbH, Germany
2Department of Pediatric Neurosurgery, Asklepios Children`s Hospital, Germany
3Department of Neonatology and Pediatric Intensive Care Medicine, Asklepios Children`s Hospital, Germany
Object: Before 1960 the survival rate of neural tube defects (NTD) was barely 10%. Due to continuous improvements in treatment regime, the 1-year survival rate has been raised to 87%. Now a days about 78% of the patients with spina bifida irrespective of its severity reach the age of 17. Improved understanding of the pathophysiology of the malformation syndrome and its associated comorbidities (“double hit theory”) finally led to changes in diagnostic and therapeutic procedures (center-based pre- and postnatally surgery), and consequently to a higher life expectancy and better quality of life.Methods: In order to assess Myelomeningocele (MMC) associated comorbidities we analyzed retrospectively 49 newborns with MMC, who had been surgically treated in our institution between January 2007 and January 2016. Children, who were previously operated in utero were excluded.Results: The 49 children had a nearly equal gender distribution. 18% went undetected during pregnancy. The MMC was repaired on the day of delivery in 90%, and there were no initial infection of the cerebrospinal fluid (CSF) observed. The follow-up period range from 5 to 8 months 11/12 years without death over the entire period. 88% of the patients suffered from hydrocephalus (HC) necessitating shunt insertion, while shunt revision was required in 33 patients (67%). Seven times the closure of MMC and the implantation of the ventriculoperitoneal shunt were carried out sequentially during the same anesthesia with no further complications. In 37 children we found significant Chiari malformation type II (CMII), syringomyelia was seen in 24 children. Additional typical magnetic resonance imaging (MRI) findings: beaked tectum (n=36), enlarged mass a intermedia (n=37), dysplasia of the corpus callosum (n=33), polymicrogyria/stenogyria (n=20), and heterotopias (n=9). Based on the results of neuro-urological diagnostics, early clear intermittent catheterization (CIC) was the most common management (85.7%). A Gardner decompression was necessary in two, myelolysis due to secondary tethered cord syndrome (sTCS) in five children.Conclusion: Chiari II malformation and hydrocephalus are the most common diseases associated with MMC. Nevertheless we found numerous CMII features in our patients, mostly without clinical relevance. In order to prevent or delay renal failure, which is known to be a significant complication in the course of the life of MMC patients, we highly recommend continuous close attention to the diagnostics and treatment of neurogenic bladder disorder. In our own series of postnatally repaired myelomeningoceles no death occurred, the rate of foramen magnum decompression and myelolysis due to sTCS was low, and the rate of shunted HC is consistent with the literature. The rationale of prenatal MMC repair is the prevention of second hit conditions. Taking the low rate of secondary comorbidities of our series into consideration, the advantages of any prenatal management need to be discussed controversially.
Neural tube defect; Hydrocephalus; Chiari malformation; Neurogenic bladder disorder
Januschek E, R�hrig A, Kunze S, Fremerey C, Wiebe B, Messing-J�nger M. Myelomeningocele, Chiari & Co. Clin Surg. 2017; 2: 1508.