Oman Medical Journal. 2013; 28 (6): 445-447
en Inglés
| IMEMR
| ID: emr-142967
ABSTRACT
Metatropic dysplasia is a rare but severe spondyloepimetaphyseal dysplasia characterized by long trunk and short extremities. The exact incidence is not known; however, 81 cases have been reported in the literature till now. Due to progressive kyphoscoliosis, there is a reversal of proportions in childhood [shortening of trunk with relative long extremities]. The diagnostic radiographic findings include marked platyspondyly [wafer-thin vertebral bodies], widened metaphyses [dumbbell-shaped tubular bones] and small epiphysis and a specific pelvic shape. The severe kyphoscoliosis is relentless and resistant to conservative treatment with bracing. Operative treatment is controversial due to the recurrence of deformity despite aggressive correction. We, here in report a case of this rare dysplasia and its follow-up after corrective surgery for spine and limb deformity. The excellent correction and good functional pulmonary status at 6-year follow-up has never been previously reported.
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Índice:
IMEMR (Mediterraneo Oriental)
Asunto principal:
Anomalías Congénitas
/
Vértebras Cervicales
/
Resultado del Tratamiento
/
Deformidades Congénitas de las Extremidades
/
Enanismo
Tipo de estudio:
Informe de Casos
Límite:
Humanos
/
Masculino
Idioma:
Inglés
Revista:
Oman Med. J.
Año:
2013
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