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.
Keywords
Publication details
- DOI
- 10.5001/omj.2013.123
- Journal
- Oman Medical Journal
- Publisher
- Oman Medical Specialty Board
- Open access
- Gold open access
Cite this article
APA 7
Song, H. R., Sinha, S., Song, S. H., & Suh, S. W. (2013). A Case of Metatropic Dysplasia: Operative Treatment of Severe Kyphoscoliosis and Limb Deformities. Oman Medical Journal. https://doi.org/10.5001/omj.2013.123
MLA 9
Song, Hae Ryong, et al. "A Case of Metatropic Dysplasia: Operative Treatment of Severe Kyphoscoliosis and Limb Deformities." Oman Medical Journal, 2013. https://doi.org/10.5001/omj.2013.123.
Chicago (author–date)
Song, Hae Ryong, Shivam Sinha, Sang Heon Song, and Seung Woo Suh. 2013. "A Case of Metatropic Dysplasia: Operative Treatment of Severe Kyphoscoliosis and Limb Deformities." Oman Medical Journal. https://doi.org/10.5001/omj.2013.123.
Harvard
Song, H. R., Sinha, S., Song, S. H. and Suh, S. W. (2013) 'A Case of Metatropic Dysplasia: Operative Treatment of Severe Kyphoscoliosis and Limb Deformities', Oman Medical Journal. doi:10.5001/omj.2013.123.
Vancouver
Song HR, Sinha S, Song SH, Suh SW. A Case of Metatropic Dysplasia: Operative Treatment of Severe Kyphoscoliosis and Limb Deformities. Oman Medical Journal. 2013. doi:10.5001/omj.2013.123
IEEE
H. R. Song, S. Sinha, S. H. Song, and S. W. Suh, "A Case of Metatropic Dysplasia: Operative Treatment of Severe Kyphoscoliosis and Limb Deformities," Oman Medical Journal, 2013, doi: 10.5001/omj.2013.123.