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 language | English |
|---|---|
| Pages (from-to) | 445-447 |
| Number of pages | 3 |
| Journal | Oman Medical Journal |
| Volume | 28 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - 2013 Nov 15 |
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Keywords
- Deformity correction
- Kyphoscoliosis
- Metatropic dysplasia
ASJC Scopus subject areas
- Medicine(all)
Cite this
A case of metatropic dysplasia : Operative treatment of severe kyphoscoliosis and limb deformities. / Song, Hae Ryong; Sinha, Shivam; Song, Sang Heon; Suh, Seung-Woo.
In: Oman Medical Journal, Vol. 28, No. 6, 15.11.2013, p. 445-447.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - A case of metatropic dysplasia
T2 - Operative treatment of severe kyphoscoliosis and limb deformities
AU - Song, Hae Ryong
AU - Sinha, Shivam
AU - Song, Sang Heon
AU - Suh, Seung-Woo
PY - 2013/11/15
Y1 - 2013/11/15
N2 - 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.
AB - 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.
KW - Deformity correction
KW - Kyphoscoliosis
KW - Metatropic dysplasia
UR - http://www.scopus.com/inward/record.url?scp=84887383092&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84887383092&partnerID=8YFLogxK
U2 - 10.5001/omj.2013.123
DO - 10.5001/omj.2013.123
M3 - Article
C2 - 24223250
AN - SCOPUS:84887383092
VL - 28
SP - 445
EP - 447
JO - Oman Medical Journal
JF - Oman Medical Journal
SN - 1999-768X
IS - 6
ER -