Clinicoradiological correlation of scoliosis in children with Jarcho-Levin and Escobar syndromes: associated “flat bone or wing-like” imaging findings

Congenital or early onset scoliosis may be the lead clinical feature in several rare syndromes. In this paper, we present the imaging findings in two children with early onset scoliosis related to the Jarcho-Levin and Escobar syndromes and an osseous plate or wing-like bar extending along the poster...

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Bibliographic Details
Published in:European journal of pediatrics 2014-10, Vol.173 (10), p.1377-1380
Main Authors: Bosemani, Thangamadhan, Poretti, Andrea, Benson, Jane E., Meyer-Heim, Andreas, Huisman, Thierry A. G. M.
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - diagnosis
Age
Cesarean section
Child, Preschool
Female
Heart Defects, Congenital - complications
Heart Defects, Congenital - diagnosis
Hernia, Diaphragmatic - complications
Hernia, Diaphragmatic - diagnosis
Humans
Infant
Infant, Newborn
Magnetic Resonance Imaging
Malignant Hyperthermia - complications
Malignant Hyperthermia - diagnosis
Medicine
Medicine & Public Health
Pediatrics
Scoliosis
Scoliosis - diagnostic imaging
Scoliosis - etiology
Short Communication
Skin Abnormalities - complications
Skin Abnormalities - diagnosis
Spinal cord
Tomography
Tomography, X-Ray Computed
Ultrasonic imaging
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Summary:Congenital or early onset scoliosis may be the lead clinical feature in several rare syndromes. In this paper, we present the imaging findings in two children with early onset scoliosis related to the Jarcho-Levin and Escobar syndromes and an osseous plate or wing-like bar extending along the posterior elements of the spine on computed tomography. The clinical phenotypes in these syndromes are variable. A thorough clinical evaluation with imaging correlation is essential. The recognition of underlying spinal anomalies is essential in planning treatment and estimating prognosis. In young children with progressive scoliosis, cross-sectional imaging plays a major role in the diagnostic work-up. Conclusion : Congenital scoliosis requires a comprehensive clinical evaluation and imaging work-up. The presence of an osseous plate or wing-like fusion of posterior elements of the spine may suggest the diagnosis of Jarcho-Levin and Escobar syndromes.
ISSN:0340-6199
1432-1076
DOI:10.1007/s00431-014-2324-4