Complex orthopaedic management of patients with skeletal dysplasias

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Skeletal dysplasias are challenging for diagnostics and treatment. We present a series of fifteen patients with different forms of skeletal dysplasias with age ranged from 6 to 17 years with variable clinical presentations managed as a part of the project of scientific cooperation between Turner Paediatric Orthopaedic Institute and Orthopaedic Hospital Vienna-Speising. The spectrum of diagnoses included multiple epiphyseal dysplasia, spondyloepiphyseal dysplasia congenita, diastrophic dysplasia, metaphyseal dysplasia, spondylometaphyseal dysplasia, Stickler syndrome, Kniest dysplasia, and anauxetic dysplasia. Complex treatment, which included axial correction and juxta-articular realignment, was performed as a single-stage, or consecutive surgery. Surgical techniques included corrective osteotomies with internal fixation, guided growth technique and external fixation devices. Best results (full axial correction, normal alignment of the joint) were achieved in 8 patients, including 2 patients with metaphyseal dysplasia, 2 patients with multiple epyphyseal dysplasia, 2 patients with spondyloepyphyseal dysplasia, patient with Stickler syndrome and patient with spondylometaphyseal dysplasia. Good results (partial correction at the present time) were seen in 4 patients (2 patients with Kniest dysplasia, 1 - with multiple epyphyseal dysplasia and 1 - with anauxetic dysplasia). Satisfactory results (non-progressive condition in previous progression) were obtained in 2 patients with diastrophic dysplasia, and poor results (progression of the deformity) - in 1 patient with diastrophic dysplasia. Positive results in most of the cases of our series make promising future for usage of complex approach for orthopedic management of children with skeletal dysplasias; advanced international cooperation is productive and helpful for diagnostics and management of rare diseases.

作者简介

A. Baindurashvili

Turner Research Institute for Children's Orthopedics

编辑信件的主要联系方式.
Email: turner01@mail.ru
俄罗斯联邦

V. Kenis

Turner Research Institute for Children's Orthopedics

Email: kenis@mail.ru
俄罗斯联邦

E. Melchenko

Turner Research Institute for Children's Orthopedics

Email: emelchenko@gmail.com
俄罗斯联邦

F. Grill

Orthopaedic Hospital Vienna-Speising

Email: franz.grill@oss.at
俄罗斯联邦

A. Al-Kaissi

Orthopaedic Hospital Vienna-Speising

Email: ali.alkaissi@oss.at
俄罗斯联邦

参考

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