Norbert Utz 1 , Ilona Daumer 1 , Cornelia Haas 1 , Martin Weinzirl 3 , Marcus Katoh
HELIOS Kliniken Krefeld, Germany Pediatric Radiology HELIOS Kliniken Krefeld, Germany Radiology Department HELIOS Kliniken Krefeld, Germany Neurosurgery Department
We present the magnetic resonance imaging features, the sonographic and clinical findings of a rare case of a cervical nonterminal myelocystocele of a female termnewborn.
Female term-newborn showed soft tissue swelling at the midline of the dorsal neck. Sonography revealed a cystic lesion with some tiny fibrous-like stalk in the center as well as a bony defect of the posterior spine at this level. MRI scan of the spine confirmed the sonographic findings and the new brain MRI showed no evidence of any additional malformations including am Chiari 2 Malformation. She underwent an operation with six months of age and was followed-up by imaging with one year of age. At that time she showed normal neurological development.
The etiology of this lesion is supposed to represent a partial failure of primary neurulation.
The incomplete fuse of the dorsal folds results in a failure of separation of neuronal and cutaneous ectoderm — a thin fibroneurovascular stalk results (containing neurons, glia, and peripheral nerves) that is connected to the skin. Continuous pulsations of subarachnoid fluid displace CSF through the posterior spina bifida and distend the meninges into a posterior meningocele.
In opposition to closed spinal dysraphism that is more common at the lumbosacral levels, such lesions are quite rare at the cervical and thoracal level.
As Rossi et al. explained, the term nonterminal myelocystocele seems to be more precise because of the striking differences between these lesions and the more common lumbosacral myelomeningoceles.
In opposition to the myelomeningoceles, the neuronal tissue in nonterminal myelocystocele is not exposed to the environment what explains why these patients show no neurological impairment.
• The nonterminal myelocystsocele is a distinct form of closed spinal dysraphism characterized by a skin-covered meningocele.
• Clinically the patients are neurologically intact because the neuronal tissue is not exposed to the environment.
• Two distinct forms have bee described: The abortive form crossed by a fibroneurovascular stalk extending from the dorsal part of the myelon to the dome of the meningocele and the complete form with a hydromelic cavity that is continuous with the ependymal canal.
Rossi, A. et al. Spectrum of nonterminal myelocystoceles. Neurosurgery 58, 509–15 discussion 509–15 (2006).
Gressot, L. V., Mohila, C. A., Jea, A., Luerssen, T. G. & Bollo, R. J. Cervicothoracic nonterminal myelocystocele with mature teratoma. J Neurosurg Pediatr 13, 204–208 (2014)