Oculo-Auriculo-Vertebral Dysplasia With Craniocervical Instability and Occult Tethered Cord Syndrome. An Addition to the Spectrum? First Case Report and Review of the Literature
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Oculo-Auriculo-Vertebral Dysplasia With Craniocervical Instability and Occult Tethered Cord Syndrome. An Addition to the Spectrum? First Case Report and Review of the Literature. / Hansen-Algenstaedt, Nils; Liem, Melanie; Khalifah, Salah; Giese, Alf; Gutenberg, Angelika.
In: JAAOS GLOB RES REV, Vol. 3, No. 7, e805, 07.2019.Research output: SCORING: Contribution to journal › SCORING: Review article › Research
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TY - JOUR
T1 - Oculo-Auriculo-Vertebral Dysplasia With Craniocervical Instability and Occult Tethered Cord Syndrome. An Addition to the Spectrum? First Case Report and Review of the Literature
AU - Hansen-Algenstaedt, Nils
AU - Liem, Melanie
AU - Khalifah, Salah
AU - Giese, Alf
AU - Gutenberg, Angelika
N1 - Copyright © 2019 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Orthopaedic Surgeons.
PY - 2019/7
Y1 - 2019/7
N2 - 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.
AB - 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.
U2 - 10.5435/JAAOSGlobal-D-17-00085
DO - 10.5435/JAAOSGlobal-D-17-00085
M3 - SCORING: Review article
C2 - 31579884
VL - 3
JO - JAAOS GLOB RES REV
JF - JAAOS GLOB RES REV
SN - 2474-7661
IS - 7
M1 - e805
ER -