Cervical myelomeningocele--follow-up of five patients
Andreas D Meyer-Heim1, Andrea Klein, Eugen Boltshauser
1Division of Paediatric Neurology, University Children's Hospital, Steinwiesstrasse 75, CH-8032 Zurich, Switzerland.
Abstract:
Only a few series of patients with cervical myelomenigocele (cMMC) and cervical meningocele (cMC) have been published. Interventions as well as the neurologic, orthopaedic, urologic and intellectual outcomes were analysed in this retrospective description of five patients with cMMC and cMC diagnosed in the period 1984-1999. Four patients suffered from cMMC, one from cMC. The average duration of follow-up was 9.5 years. None of the patients had periconceptual prevention with folic acid. Three had a Chiari II malformation and two a hydrocephalus. Tethering of the cervical cord was demonstrated in three patients at follow-up. All children achieved an independent ambulatory function and urinary continence. Incomplete sensorimotor hemiparesis was present in two children, and a mild unilateral arm paresis in one. Two of five patients had age appropriate cognitive functions. Three patients with mild mental retardation or behavioural problems had to be placed in special classes. The outcome of patients with cMMC is favourable regarding to the neurologic, orthopaedic and urologic problems compared with lower neural tube defects. However, the burden of repeated examinations and therapies is considerable and induces high costs, therefore prevention with periconceptual folic acid is a crucial issue also in cMMC. Spinal cord dysfunction has to be considered in growing children due to persistent tethering or re-tethering, therefore regular neurologic and urodynamic investigations are of particular importance.
Insights
Cervical myelomeningocele (cMMC) and cervical meningocele (cMC) outcomes are favorable for neurological and urological issues. However, prevention with folic acid is crucial due to high costs and potential spinal cord dysfunction.
Area of Science:
- Neurology
- Pediatric Surgery
- Public Health
Background:
- Few patient series on cervical myelomeningocele (cMMC) and cervical meningocele (cMC) exist.
- Outcomes and interventions for these rare spinal defects are not well-documented.
Purpose of the Study:
- To analyze interventions and outcomes (neurologic, orthopedic, urologic, intellectual) in patients with cMMC and cMC.
- To highlight the importance of folic acid prevention and regular follow-up for spinal cord dysfunction.
Main Methods:
- Retrospective analysis of five patients diagnosed between 1984-1999.
- Review of interventions, neurologic status, orthopedic function, urologic continence, and cognitive abilities.
- Average follow-up duration of 9.5 years.
Main Results:
- All patients achieved independent ambulation and urinary continence.
- Neurologic deficits included hemiparesis and arm paresis in some.
- Cognitive functions were age-appropriate in two, with three requiring special education.
- Associated conditions included Chiari II malformation and hydrocephalus.
- Tethering of the cervical cord was observed in three patients.
Conclusions:
- Outcomes for cMMC are generally favorable compared to lower neural tube defects.
- High costs and the need for ongoing care emphasize the importance of folic acid prevention.
- Regular neurologic and urodynamic evaluations are vital to monitor for spinal cord tethering or re-tethering.