A Case of Metatropic Dysplasia : Operative Treatment of Severe Kyphoscoliosis and Limb Deformities

Hae Ryong Song; Shivam Sinha; Sang Heon Song; Seung Woo Suh

doi:10.5001/omj.2013.123

Article Details

A Case of Metatropic Dysplasia : Operative Treatment of Severe Kyphoscoliosis and Limb Deformities

Authors:

Hae Ryong Song | Shivam Sinha | Sang Heon Song | Seung Woo Suh

Publisher:

Oman Medical Specialty Board

Main Topics:

Medicine

Journal:

Oman Medical Journal 2013 vol.28 Issue 6, pp.445-447

Document Type:

Article

Publication Date:

11/2013

Document Language:

English

Document ID (Org No.):

39097

Frequency:

Bi-Monthly

eISSN:

20705204

pISSN:

1999768X

DOI:

10.5001/omj.2013.123

Descriptors:

Keywords:

Abstract:

 Metatropic dysplasia is a rare but severe spondyloepimetaphysealdysplasia characterized by long trunk and short extremities. Theexact incidence is not known; however, 81 cases have been reportedin the literature till now. Due to progressive kyphoscoliosis, thereis a reversal of proportions in childhood (shortening of trunk withrelative long extremities). The diagnostic radiographic findingsinclude marked platyspondyly (wafer-thin vertebral bodies),widened metaphyses (dumbbell-shaped tubular bones) and smallepiphysis and a specific pelvic shape. The severe kyphoscoliosis isrelentless and resistant to conservative treatment with bracing.Operative treatment is controversial due to the recurrence ofdeformity despite aggressive correction. We, herein report a case ofthis rare dysplasia and its follow-up after corrective surgery for spineand limb deformity. The excellent correction and good functionalpulmonary status at 6-year follow-up has never been previouslyreported.

Article Details

A Case of Metatropic Dysplasia : Operative Treatment of Severe Kyphoscoliosis and Limb Deformities

Authors:

Hae Ryong Song | Shivam Sinha | Sang Heon Song | Seung Woo Suh

Publisher:

Oman Medical Specialty Board

Main Topics:

Medicine

Journal:

Oman Medical Journal 2013 vol.28 Issue 6, pp.445-447

Document Type:

Article

Publication Date:

11/2013

Document Language:

English

Document ID (Org No.):

39097

Frequency:

Bi-Monthly

eISSN:

20705204

pISSN:

1999768X

DOI:

10.5001/omj.2013.123

Descriptors:

DISEASES

DWARFISM

DISTORTION

SCOLIOSIS

SPINE

LIMBS

TOMOGRAPHY

CLINICAL EXAMINATIONS

SURGICAL OPERATIONS

PATIENTS

THERAPY

Kyphoscoliosis

Metatropic dysplasia

Deformity correction

Issues

Article Details

Authors: Hae Ryong Song | Shivam Sinha | Sang Heon Song | Seung Woo Suh

Publisher: Oman Medical Specialty Board

Main Topics: Medicine

Journal: Oman Medical Journal 2013 vol.28 Issue 6, pp.445-447

Document Type: Article

Publication Date: 11/2013

Document Language: English

Document ID (Org No.): 39097

Frequency: Bi-Monthly

eISSN: 20705204

pISSN: 1999768X

DOI: 10.5001/omj.2013.123

Descriptors:

Issues

Authors:

Hae Ryong Song | Shivam Sinha | Sang Heon Song | Seung Woo Suh

Publisher:

Oman Medical Specialty Board

Main Topics:

Medicine

Journal:

Oman Medical Journal 2013 vol.28 Issue 6, pp.445-447

Document Type:

Article

Publication Date:

11/2013

Document Language:

English

Document ID (Org No.):

39097

Frequency:

Bi-Monthly

eISSN:

20705204

pISSN:

1999768X

DOI:

10.5001/omj.2013.123