caa a22 4500 46317996X CHVBK 20180406164836.0 cr unu---uuuuu 170326e20070301xx s 000 0 eng 10.1007/s00381-006-0228-y doi (NATIONALLICENCE)springer-10.1007/s00381-006-0228-y Segmental spinal dysgenesis: report of four cases and proposed management strategy [Elektronische Daten] [Ruth Bristol, Nicholas Theodore, Harold Rekate] Introduction: Segmental spinal dysgenesis, a rare developmental malformation, usually manifests during pregnancy or at birth. The resulting gross spinal instability necessitates spinal stabilization, which is inherently challenging in neonates. Methods: We report four cases of segmental dysgenesis: three in the thoracolumbar region and one at the cervicothoracic junction. The latter was maintained in a custom orthosis that restricted all craniospinal motion while allowing routine care. Two neonates underwent surgical stabilization. The fourth patient will remain in a brace until 12-14months old when fusion is planned. Results: Fusion with rib autografts failed in the two neonates. One patient has been followed for 13years and is paraplegic. The second patient was lost to follow up. The patient with the cervicothoracic dysgenesis maintained normal neurologic function until his death at 8months of cardiac failure. The fourth patient is 12months old and has been maintained in a thoracolumbar orthosis with stable neurologic function. Conclusion: Several factors contribute to the challenge of creating a stable fusion in neonates. Incomplete ossification of the vertebral bodies and poor results with allograft materials restrict fusion options. Neurologic deficits often prevent ambulation and decrease the axial-loading forces that enhance fusion. To allow children to grow and develop, we advocate rigid spinal immobilization for 12-18months before spinal fusion (preferably, rib or fibular autograft). Given the already narrow spinal canal, the use of instrumentation is controversial. We advocate the use of instrumentation in infants only when a sound construct cannot be obtained with the graft alone. Springer-Verlag, 2006 Caudal regression syndrome nationallicence Congenital spinal anomaly nationallicence Segmental spinal dysgenesis nationallicence Spinal dysraphism nationallicence Bristol Ruth Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA aut Theodore Nicholas Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA aut Rekate Harold Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA aut Child's Nervous System Springer-Verlag 23/3(2007-03-01), 359-364 0256-7040 23:3<359 2007 23 381 https://doi.org/10.1007/s00381-006-0228-y text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00381-006-0228-y text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Bristol Ruth Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA aut NATIONALLICENCE 700 1- Theodore Nicholas Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA aut NATIONALLICENCE 700 1- Rekate Harold Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA aut NATIONALLICENCE 773 0- Child's Nervous System Springer-Verlag 23/3(2007-03-01), 359-364 0256-7040 23:3<359 2007 23 381 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer