The 3-dimensional Configuration of the Typical Hip and Knee in Diastrophic Dysplasia
Title
The 3-dimensional Configuration of the Typical Hip and Knee in Diastrophic Dysplasia
Creator
Weiner D S; Jonah D; Kopits S
Publisher
Journal of Pediatric Orthopaedics
Date
2010
2010-06
Description
Purpose: Surgical correction of the hip and knee in patients with diastrophic dysplasia is extremely difficult secondary to the markedly distorted pathoanatomy of both the bone and soft tissues. The objective of this study is to provide a 3-dimensional model and carefully and extensively describe the pathoanatomy of the diastrophic hip and knee. Methods: Three-dimensional computer model reconstructions were developed based on clinical, radiographic, and surgical observations performed "meticulously" by a single surgeon on 110 hips in 55 patients. An additional 13 patients seen by another surgeon were also reviewed. In addition to the clinical and radiographic evaluations, 10 computed tomographic scans were performed of the pelvis and hip areas and 53 arthrograms were done in 13 patients. Sixty-six patients underwent a myriad of operative procedures on the hip and proximal femur. Results: The typical pathoanatomy seen in cases of diastrophic dysplasia reflects the severe and rampantly progressive flexion deformity of the hip and knee with time, and the anatomical aberrations of development of the proximal femur and the knee joint, which are highlighted in our computer model. Replacement arthroplasty at the hip and knee may hold the only solution to adolescents and adults with advanced disease; however, the unavailability of long-term data precludes a reasonable management recommendation. Conclusions: The disturbed pathoanatomy of the diastrophic dysplasia hip and knee reflects the difficulties in achieving any substantial surgical correction even with customization. This report provides the most extensive 3-dimensional pathoanatomical observations as yet presented in the literature.
Subject
arthroplasty; chondrocytes; diastrophic dwarfism; diastrophic dysplasia; dwarfism; joint; Orthopedics; pathoanatomy; Pediatrics; proteoglycans; sulfate transporter
Identifier
Format
Journal Article
URL Address
Search for Full-text
Users with a NEOMED Library login can search for full-text journal articles at the following url: https://libraryguides.neomed.edu/home
Rights
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
Pages
403-410
Issue
4
Volume
30
Citation
Weiner D S; Jonah D; Kopits S, “The 3-dimensional Configuration of the Typical Hip and Knee in Diastrophic Dysplasia,” NEOMED Bibliography Database, accessed April 25, 2024, https://neomed.omeka.net/items/show/7557.