Oculo-auriculo-vertebral spectrum (OAVS) is an uncommon congenital disorder of abnormal development of the first and second pharyngeal arches. This spectrum is characterized by craniofacial microsomia, epibulbar dermoids, ear abnormalities, renal and cardiac defects, and a wide range of vertebral segmentation and formation disorders. Frequently, the cervicothoracic spine is involved. Only recently, the morbidity attributed to the spinal abnormalities has gained attention. Strategy and timing of spine surgery has become increasingly important in patients with OAVS. Here, we report a case of OAVS with characteristic vertebral cervical and thoracic involvement and its sequelae requiring multiple spinal procedures, further complexed by an unprecedented occult tethered cord syndrome, which was successfully treated by surgical detethering. In this context, the recent literature on spinal anomalies is reviewed.
Successful fusion of affected segments, including resolution of infection, is reported in over 90% of cases described in the literature. The revision rate among our mostly multimorbid patient group with an average age of 66 years was 16%. Of 39 of the 114 (34%) patients with preoperative neurological deficits, 26 (66%) demonstrated postoperative regression. Nine patients (23%) showed no improvement, whilst exacerbation of existing neurological deficits was seen in four patients (11%). Staphylococcus was the major pathogen in 34% of cases.
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