Popis: |
Apert's syndrome (acrocephalosyndactyly type 1) is characterised by anomalies of the cranium, hands, and feet. The cranial and hand anomalies have been investigated, and the management of these is well established. In contrast, the anomalies affecting the feet and their management has previously received little attention. Forty-three children with Apert's syndrome underwent investigation of the anomalies affecting their feet. This consisted of history, clinical examination, and where possible, radiographic examination to establish the anomalies present, how these altered during development, and their clinical significance. The conclusion of the study is that there are widespread anomalies of the feet, with defects including both predictable dysmorphic changes and progressive fusions of the skeletal components during skeletal maturity. These fusions and their effect on growth combine to produce increasing deformity during childhood. The clinical significance of the anomalies is that walking is often delayed, and the increasing deformity results in difficulty obtaining footwear. This is the most common reason for surgery to the feet being undertaken during childhood to improve the shape of the feet to facilitate the provision of footwear. The unexpectedly high incidence of surgery in this study suggests that the management of foot deformities may require surgery more frequently than current literature would suggest. |