RESEARCH ARTICLE


Treatment of Varus Deformities of the Lower Limbs in Patients with Achondroplasia and Hypochondroplasia



Ali Al Kaissi*, 1, 2, Sebastian Farr 2, Rudolf Ganger 2, Jochen G Hofstaetter 1 , 3 , Klaus Klaushofer 1, Franz Grill 2
1 Ludwig Boltzmann Institute of Osteology, at the Hanusch Hospital of WGKK and, AUVA Trauma Centre Meidling, First Medical Department, Hanusch Hospital, Vienna, Austria
2 Orthopaedic Hospital of Speising, Paediatric Department, Vienna, Austria
3 Department of Orthopaedic Surgery, Vienna General Hospital, Medical University of Vienna, Vienna, Austria


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© Al Kaissi et al.; Licensee Bentham Open.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.5/) which permits unrestrictive use, distribution, and reproduction in any medium, provided the original work is properly cited.

* Address correspondence to this author at the Ludwig-Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Center Meidling, First Medical Department, Hanusch Hospital Vienna, Austria; Tel: 00 43 01 801 82 217; Fax: 00 43 43 801 82-575; E-mail: ali.alkaissi@osteologie.at


Abstract

Angular deformities of the lower limbs are a common clinical problem encountered in pediatric orthopaedic practices particularly in patients with osteochondrodysplasias. The varus deformity is more common than the valgus deformity in achondroplasia and hypochondroplasia patients because of the unusual growth of the fibulae than that of the tibiae. We retrospectively reviewed six patients (four patients with achondroplasia and two patients with hypochondroplsia) with relevant limb deformities due to the above-mentioned entities. All patients manifested significant varus deformity of the lower limbs. Detailed phenotypic characterization, radiologic and genetic testing was carried out as baseline diagnostic tool. We described the re-alignment procedures, which have been applied accordingly. Therefore, bilateral multi-level procedures, multi-apical planning and limb lengthening have been successfully applied. While recognition of the underlying syndromic association in patients who are manifesting angular deformities is the baseline for proper orthopaedic management, this paper demonstrates how to evaluate and treat these complex patients.

Keywords: Angular deformities, achondroplasia, hypochondroplasia, re-alignment procedures, limb lengthening, FGFR3 mutation.