A case of metatropic dysplasia: operative treatment of severe kyphoscoliosis and limb deformities.
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, herein 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.
Institute of Rare Diseases and Department of Orthopaedic Surgery, Korea University Medical Centre, Guro Hospital, #80 Guro-dong, Guro-gu, Seoul 152703, Korea.
Hae Ryong
HR
Song
Shivam
S
Sinha
Sang Heon
SH
Song
Seung Woo
SW
Suh
Deformity correction; Kyphoscoliosis; Metatropic dysplasia