Limb lengthening in achondroplasia by Ilizarov's method

Abstract

In achondroplasts limb lengthening can restore the normal proportion of the body. The Ilizarov method of limb lengthening has been used in 37 patients, 23 of whom have had an adequate follow-up after operation. In this technique the cortex of the bone is partially cut, leaving the medulla intact; the two bone segments are then subjected to distraction by means of a special apparatus. The bone tissue between the two segments starts regenerating and this allows a considerable increase in length. The results and complications are reported. It is considered that this method is based on sound principles and is free of major risks.

Is Bilateral Lower Limb Lengthening Appropriate for Achondroplasia?: Midterm Analysis of the Complications and Quality of Life

“…Achondroplasia is the most frequently encountered form of nonlethal skeletal dysplasia [4] and is characterized by defective enchondral ossification owing to defective gene encoding for fibroblast growth factor receptor 3 [6,14,20]. It is a type of rhizomelic dwarfism with an incidence of approximately one in 10,000 live births [6].…”

“…It is a type of rhizomelic dwarfism with an incidence of approximately one in 10,000 live births [6]. In addition to short stature, individuals with achondroplasia often have substantial angular deformities of the limbs, spinal problems, and other neurologic and craniofacial abnormalities develop [5,9].…”

“…Achondroplastic dwarfism results in considerable physical and psychologic handicaps owing to the disproportionate stature of the body and difficulty in performing routine activities of daily living [1,6]. These individuals often feel 'different' from their families and circle of friends, often suffer from emotional disturbances, and are prone to have inferiority complexes [6].…”

“…These individuals often feel 'different' from their families and circle of friends, often suffer from emotional disturbances, and are prone to have inferiority complexes [6]. They also have major musculoskeletal problems including symptomatic malalignment of the lower limbs.…”

“…Limb lengthening by the Ilizarov method has been used in patients with achondroplasia by many authors during the past few decades, but with varying amounts of success [2,3,6,8,13,16,18,20]. Cattaneo et al [6] reported a considerable increase in length (14-18 cm) without major risk.…”