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Published on: April 17, 2019
Myelomeningocele - a single institute analysis of the years 2007 to 2015
Elke Januschek1,2, Andreas Röhrig3, Sandra Kunze3
1Neurosurgical Department, Sana Klinikum Offenbach GmbH, Starkenburgring 66, 63069, Offenbach, Germany. e.januschek@gmx.de.
Insights
Myelomeningocele (MMC) treatment in newborns shows high rates of associated Chiari II malformations and hydrocephalus. An interdisciplinary approach facilitated independence and social continence in most patients.
Area of Science:
- Pediatric Neurosurgery
- Developmental Neurology
- Congenital Malformations
Background:
- Folate supplementation has decreased open spinal dysraphism rates.
- A dedicated pediatric neurosurgery department was established in 2007.
- Myelomeningocele (MMC), a common open neural tube defect (NTD), is treated in newborns at this institution.
Purpose of the Study:
- To present the treatment concepts and outcomes for myelomeningocele (MMC) in newborns.
- To analyze surgical interventions and associated comorbidities in pediatric spinal dysraphism cases.
Main Methods:
- Retrospective analysis of surgical records for newborns with MMC from January 2007 to August 2015.
- Exclusion of infants with prior in utero surgery.
- Data collection on NTD type, location, comorbidities, and ambulation.
Main Results:
- 48 infants with spinal dysraphism were analyzed; 90% underwent MMC repair on the day of delivery.
- Associated conditions included Chiari malformation type II (75%) and hydrocephalus (85.4%), requiring shunt implantation.
- 70% of patients were ambulatory (with or without orthoses/devices); 83.3% with bladder impairment managed with intermittent catheterization.
Conclusions:
- Neural tube defects can remain undetected during pregnancy despite prenatal care.
- Chiari II malformations and hydrocephalus are common comorbidities with MMC.
- An interdisciplinary approach promotes independence and social continence in MMC patients.
Background:
After the introduction of folate supplementation, the number of open spinal dysraphism was successfully reduced over time. In 2007, the department for pediatric neurosurgery was established in the children's hospital. Since then, newborns with myelomeningocele (MMC), the most common form of open neural tube defects (NTD), are treated here. The aim of this study is to present the concepts applied to and experiences resulting from treatment of MMC.
Methods:
Records of all newborns with MMC treated surgically during the period January 2007 to August 2015 in our institution were analyzed. Children, who were previously operated in utero were excluded. The type of neural tube defect, its location, associated comorbidities, and ambulation were recorded.
Results:
Forty-eight children (25 males, 23 females) with spinal dysraphism were included in the analysis. In nearly 90 % of the cases, the repair of the MMC was done on the day of delivery. The follow-up period ranges from 9 weeks to 8 9/12 years (loss of follow-up in 2 cases). In 19 %, the defect remained undetected during gestation and in one case, carbamazepine was taken despite pregnancy. In 36 children (75 %), we found a Chiari malformation type II (CMII) associated with myelomeningocele. 85.4 % suffered from hydrocephalus and implantation of a shunt was necessary. In cases of bladder impairment, an intermittent catheterization was the most common management (83.3 %); no bladder augmentation was required. Twelve children required orthopedic surgery. Twenty-three of 33 patients (70 %) are ambulatory w/wo orthoses and devices. The 13 children who are younger than 2 years were considered separately to assess the motor activity safely.
Conclusions:
Our data show that neural tube defects to this day can remain undetected despite medical care during pregnancy. The most common associated diseases with MMC are Chiari II malformations and hydrocephalus. In the seven cases of simultaneous repair of MMC with shunt implantation, no additional complications were encountered. An interdisciplinary approach was allowed in a high percentage independence and social continence.
