Purpose: To describe the incidence and characterize the clinical manifestations, diagnosis and outcome of spontaneous epidural abscess (SEA) not associated with neurosurgical procedures or instrumentation of the spine.
Methods: Review of cases of SEA over 10 years. Diagnosis was made by imaging-techniques and surgical examination.
We report a 22-year-old female with a variant of the Axenfeld-Rieger Syndrome (ARS) and discuss its relation with the subtelomeric 6p deletion. An ARS variant has been described in two familial cases of Axenfeld-Rieger Anomaly (ARA) featuring specific extra ocular manifestations-hypertelorism, midface hypoplasia, mild sensorial deafness, hydrocephaly, psychomotor delay and flattened femoral epiphyses. We proposed that this set of characteristics represents a separate syndrome within the ARS.
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