Anterior cervical myelomeningocele: a rare malformation of the spinal cord.

Cervical myelomeningocele (MMC) is an uncommon congenital malformation of the spinal cord and accounts for a small proportion of neural tube defects. These lesions mostly occur in the dorsal part of the body. Only a single case of an anterior cervical MMC has been previously reported. The authors report a second case of anterior cervical MMC diagnosed when the patient began to experience symptoms of bilateral hand weakness in adulthood. In this patient, MR imaging of the cervical spine showed an anterior cervical MMC at the C6-7 level with hydrocephalus, thinning of the genu and trunk of the corpus callosum, maldevelopment of the cerebellar tonsils, and expansion of the fourth ventricle, posterior cranial fossa, and subarachnoid space. A CT scan and a 3D CT reconstruction of the cervical spine clearly demonstrated contiguous fusions of multiple lower-cervical vertebrae and neural arches, which was consistent with Type III Klippel-Feil syndrome. The patient was advised to undergo operative treatment to prevent the progression of her neurological deficit. However, after being notified of the potential neurological risks, the patient declined surgery and opted for conservative treatment with a hard neck collar. At 4 months' follow-up, the patient's neurological deficit remains stable with the MMC left untreated. The authors presume that the possible pathogenesis of anterior cervical MMC may greatly differ from that of posterior lesions. This lesion could also be associated with multiple other spinal abnormalities, which highlights the importance of comprehensive preoperative radiological examinations.

[1]  G. Balachandran Klippel-Feil Syndrome and Anterior Cervical Meningomyelocele: A Rare Case Report , 2009, American Journal of Neuroradiology.

[2]  M. Catala,et al.  Septième partie - DÉVELOPPEMENT DE LA MOELLE ÉPINIÈRE, DU RACHIS ET DE LA FOSSE POSTÉRIEURE , 2008 .

[3]  P. Tajik,et al.  Cervical Myelomeningocele , 2006, Neurosurgery.

[4]  S. Andronikou,et al.  Cervical spina bifida cystica: MRI differentiation of the subtypes in children , 2006, Child's Nervous System.

[5]  S. Cavalheiro,et al.  Cystic spinal dysraphism of the cervical and upper thoracic region , 2006, Child’s Nervous System.

[6]  S. Ashwal,et al.  Tethered cord syndrome in childhood: diagnostic features and relationship to congenital anomalies , 2004, Neurological research.

[7]  M. Catala,et al.  [Neurosurgical embryology. Part 7: Development of the spinal cord, the spine and the posterior fossa]. , 2003, Neuro-chirurgie.

[8]  R. Biancheri,et al.  Magnetic resonance imaging of spinal dysraphism. , 2001, Topics in magnetic resonance imaging : TMRI.

[9]  P. Steinbok,et al.  Cervical Myelocystoceles and Meningoceles: Long-Term Follow-Up , 2000, Pediatric Neurosurgery.

[10]  A. Rossi,et al.  Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification , 2000, Neuroradiology.

[11]  J. R. Bowen,et al.  The natural history of Klippel-Feil syndrome: clinical, roentgenographic, and magnetic resonance imaging findings at adulthood. , 1995, Journal of pediatric orthopedics.

[12]  P. Steinbok Dysraphic lesions of the cervical spinal cord. , 1995, Neurosurgery clinics of North America.

[13]  D. Cochrane,et al.  The nature of congenital posterior cervical or cervicothoracic midline cutaneous mass lesions. Report of eight cases. , 1991, Journal of neurosurgery.

[14]  R. Biancheri,et al.  Spinal dysraphism: MR imaging rationale. , 2004, Journal of neuroradiology. Journal de neuroradiologie.

[15]  T. Park,et al.  Cervical meningocele and associated spinal anomalies , 2004, Child's Nervous System.

[16]  M. Dias,et al.  Cervical myelomeningoceles. , 1993, Neurosurgery.