A case of metatropic dysplasia: Operative treatment of severe kyphoscoliosis and limb deformities

Hae Ryong Song, Shivam Sinha, Sang Heon Song, Seung-Woo Suh

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4 Citations (Scopus)

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, 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.

Original languageEnglish
Pages (from-to)445-447
Number of pages3
JournalOman Medical Journal
Volume28
Issue number6
DOIs
Publication statusPublished - 2013 Nov 15

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Keywords

  • Deformity correction
  • Kyphoscoliosis
  • Metatropic dysplasia

ASJC Scopus subject areas

  • Medicine(all)

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