Correction of the Webbed Neck Deformity in Noonan Syndrome-A Case Report and Review of Literature

Abstract

The Noonan syndrome is a genetic disorder resulting in developmental anomaly of multiple parts of the body. Many features of the syndrome are reminiscent of Turner syndrome, except the later is caused by 45×0 chromosome anomaly, whereas the former has normal chromosome number. Accordingly, the Noonan syndrome is also called ”Turner phenotype”. Among the multiple anomalies of Noonan syndrome, the webbed neck deformity is the most prominent one. The deformity is characterized by a subcutaneous band of thickened fascia in the lateral aspects of neck and a low-neck hairline. The goal of surgical management is to correct the low-lying hairline and to restore the normal contour of the neck. The issue of abolishing the webbing band of the neck without residual obvious scarring is a challenge to the surgeons. We report a female patient of Noonan syndrome and describe the technique of correction. We also discuss a number of major procedures described in the literatures.