Post-traumatic cervical spondyloptosis: A rare entity with multiple management options.

Post-traumatic cervical spondyloptosis is a rare condition associated with high energy injuries, and to our knowledge only case reports are available. There are no universally accepted treatment paradigms for these cases and management is individualised according to the case and surgeon preference. We retrospectively analysed our management and clinical outcomes of this condition. From January 2007 to August 2014 we treated eight patients with cervical spondyloptosis at our institute. Only two patients had no neurological deficits; all the remaining patients had partial cord injury. Seven were treated surgically with preoperative traction followed by anterior cervical discectomy and fusion with fixation in three patients, and combined anterior and posterior fusion and fixation in four. Depending on the presence of anterior compression by a disc an anterior first or posterior first approach was advocated. All four combined anterior and posterior fusion and fixation patients needed to be turned more than once (540°). There was no neurological deterioration in any of the patients, as they either improved or remained stable neurologically. Post-traumatic cervical spondyloptosis is a challenging entity to manage. Surgery can be done safely with good clinical and radiological outcome and needs to be tailored to the individual patient.

Association of oral-facial-digital syndrome type VI (Varadi-Papp syndrome) with optochiasmatic pilocytic astrocytoma.

INTRODUCTION: Varadi-Papp syndrome (VPS) or oral-facial-digital syndrome type VI (OFDS-VI) is a rare autosomal recessive disorder distinguished from other OFDSs by metacarpal abnormalities with central polydactyly and by cerebellar abnormalities. It can be associated with central nervous system tumors, which most commonly has been a hypothalamic hamartoma. CLINICAL CASE REPORT: The boy had unusual facial features, developmental delay, limb malformations, and other phenotypic anomalies suggestive of VPS. X-ray of the hand and feet showed right hand polydactyly. He also had a deep wider peduncular fossa, thickened superior cerebellar peduncle, and inferior vermian hypoplasia along with optochiasmatic tumor. The patient underwent a right pterional craniotomy and tumor decompression. Histopathology was suggestive of a pilocytic astrocytoma. CONCLUSION: This is the first case in available literature in which the OFDS-VI has been associated with an optochiasmatic pilocytic astrocytoma. We suggest an expansion of the disease spectrum of OFDS-VI to include the association of optochiasmatic pilocytic astrocytoma.