Posterior Fossa Dermoid Cysts in Association with Klippel-Feil Syndrome: Report of Three Cases

Abstract

SUMMARY:Although the association of spinal lumbosacral dysraphism and congenital spinal dermoid tumors is well known, the association of craniocervical spinal anomalies and posterior fossa dermoids has only been recognized recently. Advances in imaging technology and awareness of the association likely contribute to an increase in recently reported cases.

Type III Klippel–Feil syndrome: case report and review of associated craniofacial anomalies

“…A brief description of these anomalies is provided in Table 2. The neurologic abnormalities and neurologic complications described in association with KFS include syringomyelia, agenesis of the corpus callosum, meningocele and spinal stenosis [9]. In 1936, the relationship between posterior fossa dermoid tumors with cervical fusion anomalies such as the KFS was first recognized [10].…”

Management issues in a complex case of basilar invagination associated with a large fourth ventricular dermoid and Klippel-Feil syndrome

“…[1][2][3][4] Failure of formation and rearrangement of cervical sclerotomes results in Klippel-Feil syndrome. [1][2][3][4] Failure of formation and rearrangement of cervical sclerotomes results in Klippel-Feil syndrome.…”

“…[1][2][3][4] The embryological basis of BI is more complex and probably results from a failure of correction of physiological BI during the course of fetal development. [1,3,4,6] In patients with this rare association, excision of the posterior fossa lesion through a limited suboccipital craniectomy with endoscopic assistance followed by occipitocervical fusion has been reported. [1,3,4,6] In patients with this rare association, excision of the posterior fossa lesion through a limited suboccipital craniectomy with endoscopic assistance followed by occipitocervical fusion has been reported.…”

“…[4] Many of the intraspinal dermoid cysts are seen in association with other congenital anomalies including spina bifida occulta, dorsal dermal sinus, myelomeningocoele, tethered cord, hypertrichosis, and other dermal abnormalities. [4] Many of the intraspinal dermoid cysts are seen in association with other congenital anomalies including spina bifida occulta, dorsal dermal sinus, myelomeningocoele, tethered cord, hypertrichosis, and other dermal abnormalities.…”