Posterior Cranial Fossa Dermoid in Association with Craniovertebral and Cervical Spinal Anomaly: Report of Two Cases

Abstract

Two patients, a 12-year-old girl and an 8-year-old boy, with congenital craniovertebral anomaly and Klippel-Feil syndrome also had a posterior cranial fossa dermoid. The association of these two discrete pathological lesions in the same individual is extremely rare. As both lesions are related to an embryological disorder, issues regarding the possible stage of dysgenesis are analyzed. The treatment options in such cases are discussed.

Symptomatic Syringomyelia Associated With a Dermoid Tumor in the Posterior Fossa -Case Report-

“…4,8) In the present case, the tumor was located laterally in the left cerebellar hemisphere without hydrocephalus. Bony abnormalities at the craniovertebral junction or upper cervical portion such as Klippel-Feil anomaly may be discrete clinical syndromes associated with posterior fossa dermoid tumors, 4,8,12) but no bony anomalies or associated congenital anomalies were found related to syrinx formation in the present case.…”

“…Dermoid tumors are a rare clinical entity that account for 0.1-0.7% of all brain tumors, and manifest symptoms mostly during early adolescence. 4,8,12) Dermoid tumors generally grow slowly along the cranial midline axis including the posterior fossa, and are occasionally associated with obstructive hydrocephalus. 4,8) In the present case, the tumor was located laterally in the left cerebellar hemisphere without hydrocephalus.…”

Posterior fossa dermoid: yet another cause of difficult airway?

“…This fact may possibly be responsible for the occurrence, albeit uncommon, of both conditions together. This idea is borne out by a review of the literature, which reveals reports of posterior fossa dermoid associated with Klippel-Feil syndrome [2][3][4] and other craniovertebral anomalies [5,6]. In our patient, the classic triad of Klippel-Feil anomaly was present, though the common feature of fused cervical vertebrae was not evident.…”

“…An association of dermoid and epidermoid cysts in the posterior fossa with Klippel-Feil syndrome [2][3][4] and craniovertebral junction anomalies [5,6] has been reported. Whether or not these anomalies are associated with dermoid cysts, they are known to pose challenges while the airway is being secured.…”

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

“…The related failure of cleavage of ectoderm from neuroectoderm resulting in entrapment of dermal elements within the closing neural tube may contribute to the association of Klippel-Feil anomalies and dermoid cysts. 2 Other proposed theories include overdistention of the neural tube resulting in distortion of the somites and reduced expressivity of the Hox or Pax genes, the highly conserved DNA sequences that control the development of the intervertebral disks. 8 A mechanical basis to explain the relationship between these abnormalities is that during the formation of the cephalic and cervical brain flexures, a shortening of the cervical spine because of a reduction or fusion in the number of somites may result in altered tissue tension, which could lead to entrapment of dermal elements.…”