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Posterior Cranial Fossa Dermoid in Association with Congenital Basilar Invagination, an Craniovertebral Junction Anomaly and Kippel- Feil Syndrome: A Potential Threat of Infective Abscess and Meningitis

2018; Volume: 3; Issue: 5 Linguagem: Inglês

10.15761/bem.1000149

2399-9632

Guru Dutta Satyarthee, Sanjeev Lalwani,

Fetal and Pediatric Neurological Disorders

Intracranial dermoid cysts are uncommon occurrence.The intracranially located dermoid cyst arises from ectoderm, probably derived from cell nest included during the closure of neural tube.The commonest site of intracranial dermoid cyst is posterior fossa.The more common supratentorial tumours are located on skull base, frontal or temporal or suprasellar region.Intracranial dermoid cyst can be associated with complete or incomplete sinus tract, usually ending in an intracranial location of dermoid, which predisposes to risk of meningitis, locally discharging sinus and even abscess formation and associated morbidity.Although the spinal lumbosacral dysraphism association with congenital spinal dermoid tumors is well known, however, the association of craniocervical spinal anomalies and posterior fossa dermoids has been recognized recently, so paucity of literature.Kippel-Feil syndrome may be associated with craniovertebral junction anomaly.In addition, it can be associated with diastematomyelia, syringomyelia, agenesis of corpus callosum, meningocele, ventriculomegaly, porencephalic cyst, cervical spina bifida oculta and spinal stenosis.Authors report an 18-year-old male boy with posterior cranial fossa dermoid with basilar invagination.The association of these two discrete pathologies are extremely uncommon.Both these lesions represent embryological disorder.The pathogenesis and treatment modality along with pertinent literature is reviewed.In extensive PubMed search authors could got only one case report by Goel et al. so, current case represents second such association in the literature.Brief embryological development and management of such association along with pertinent literature is reviewed briefly.