Klippel-Feil syndrome in association with posterior fossa dermoid tumour

Klippel-Feil syndrome (KFS) is characterized by specific congenital anomalies of segmentation of the cervical spine. On the other hand, dermoid tumour is a rare entity accounting for 0.04-0.7% of all intracranial tumours and the most common location is in the posterior fossa, at or near the midline. A new case with the association of KFS and the posterior fossa dermoid tumour is presented with complaints of progressive headache, occipital lump, and short neck. Plain radiography and 3D computed tomography (CT) of the craniovertebral region revealed a central occipital hole as well as fusion of the C4-7 vertebrae. CT and magnetic resonance imaging (MRI) of the brain demonstrated a well circumscribed midline cystic mass without contrast enhancement in the posterior fossa compressing the vermis and cerebellum. A suboccipital craniectomy was done and dermoid tumour with dermal sinus was removed totally after the opening of the dura mater. Pathological examination confirmed that the mass was a dermoid tumour consisting of stratified squamous epithelium, hair, keratin and sebaceous glands. Control MRI showed no evidence of recurrence and the patient was asymptomatic. The experience prompted me to review reports in the literature since 1936 of posterior fossa dermoid tumour associated with KFS. From my analysis, I highlight early diagnosis and an appropriate surgery to prevent complications such as neural compression and bacterial or aseptic meningitis through the rupture site or dermal sinus in cases of KFS associated with for dermoid tumours of the posterior fossa.