Spondylocostal dysostosis: thirteen new cases treated by conservative and surgical means

Spine
Marco TeliHilali Noordeen

Abstract

Prospective assessment of a cohort of patients affected by spondylocostal dysostosis. To report on the results of conservative and operative management of spondylocostal dysostosis and, based on this, to propose an assessment and treatment protocol for the condition. Spondylocostal dysostosis and spondylothoracic dysostosis are subtypes of Jarcho-Levin syndrome, a hereditary condition manifested by vertebral body and related rib malformations. Mortality prevails in spondylothoracic dysostosis because of more severe respiratory compromise. Details of prenatal and postnatal diagnosis, history, and management of 13 patients with spondylocostal dysostosis are presented. All patients were treated postnatally with repeated chest physiotherapy. Two patients refractory to conservative treatment underwent surgical intervention: the first had a chest wall reconstruction via a latissimus dorsi flap, the second a posterior spinal instrumented fusion for progressive scoliosis. Prenatal ultrasound in 4 of 13 cases showed full details of vertebral and rib anomalies. Thoracic and lumbar hemivertebrae were most common, leading to congenital scoliosis in 10 of 13 cases. A number of extraskeletal abnormalities were also identified. At an average ...Continue Reading

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Citations

Mar 3, 2006·Journal of Neurosurgery·Seong YiSang Won Lee
Feb 12, 2009·Indian Journal of Pediatrics·Siddram J PatilR S Rama Krishnan
Feb 18, 2009·Archives of Gynecology and Obstetrics·Margarita Alvarez de la RosaJuan Mario Troyano Luque
Apr 17, 2007·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Anne M Slavotinek
Apr 24, 2018·American Journal of Medical Genetics. Part a·Arjan BoumanInge B Mathijssen
Mar 29, 2019·Clinical Dysmorphology·Inusha PanigrahiNiranjan Khandelwal

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