Witkop syndrome

Overview

A syndrome that is characterised by the occurrence of nail dysplasia and tooth abnormalities due to a mutation in the MSX1 gene.

Symptoms

* Missing teeth * Missing nails * Hypoplastic nails * Hypodontia

Treatment

In order to provide esthetics and optimum function, a multifaceted approach was planned, keeping in mind the needs and limitations of the adolescent patient. Extractions of 64, 71, 74, 81, and 84 were done in view of their short resorbed roots. Fixed partial dentures were planned for both arches to restore the remaining edentulous spaces. All four second primary molars were restored with stainless steel crowns to establish posterior occlusion [Figure - 5]. In order to give a fixed partial denture for the upper arch, the midline diastema was first closed with a simple fixed orthodontic appliance. In the maxillary arch, 53 and 63 were endodontically treated and restored after post and core build-up, so as to serve as abutments in addition to 11, 21, and 54. To facilitate transverse jaw growth, two independent fixed prosthetic units were given in the upper arch. Similarly, in the mandibular arch 72, 73, 82, and 83 were endodontically treated and restored following post and core build-up. These teeth were also used as abutments for a fixed partial denture extending from the premolar region on one side to the premolar region on the other side