Cervical myelocystocele: rare presentation of spinal dysraphism.

INTRODUCTION

Cervical myelocystocele (CM) is a none common variant of closed spinal dysraphisms. It is caused by partial dorsal myeloschisis at the late stage of neurulation [1, 2].

CASE REPORT

The mother of our patient was a 26-year-old woman, whose pre-existing medical conditions include hereditary xerocytosis and severe gastro-esophageal reflux (GERD). She was unable to use folic acid supplement for more than 1 week because of GERD. Her prenatal ultrasounds were done at 12, 18, 26 and 32 weeks of postmenstrual age and reported as normal. Our patient was delivered at term by spontaneous vaginal delivery and noted to have skin-covered soft mass at C5–C7 along spinal column (Fig. 1a). There was a divot with no fluid leakage. Expect for this finding, his general examination was unremarkable. The patient could move all limbs spontaneously; tone was normal, and no spasticity was present. Magnetic-Resonance Imaging (MRI) of the brain, cervical and lumbar spine was requested. Spinal MRI detected a defect in the posterior element of the spinal canal at the level of C5 vertebra representing spina bifida (Fig. 1b). Small stalk arising from the posterior aspect of the thecal sac was crossing through the spina bifida into the dome of meningocele posteriorly at the same level. The rest of the spinal cord demonstrated normal size and signal characteristics. No intra-spinal masses, mass effect, syrinx or Chiari malformation were found.

Figure 1:

(a) Clinical presentation at birth skin-covered mass measures 12 × 8 × 12 mm. (b) MRI on day of life 5, showing spina bifida at the level of C5.

DISCUSSION

CM can present in two subtypes: Rossi type 1: myelocystocele manqué with a fibroneurovascular stalk attached to the dome of the meningocele and Rossi type 2: when hydromyelia replaced the posterior wall of the spinal cord into the meningocele [3]. Our patient presented with type 1 CN, where dorsal sac is histologically originated from a neuroglia, and neural stalk is a remnant of cutaneous ectoderm [4].

Neurosurgery was consulted immediately after birth, and corrective surgery was performed at the age of 3 months, that included amputation of tethering part of the fibrous tissue between the cord and the stalk. The post-operative course was unremarkable. Currently, patient attends pre-school and doing well. No neurological deficits or motor limitations were detected at the age of 4 years.