Case report: Denys- Drash syndrome.

BACKGROUND: Denys-Drash Syndrome (DDS) is an uncommon disorder that appears sporadically and in rare cases may be inherited as an autosomal dominant trait It manifests either at birth or within the first year of life and typically consists of the triad of congenital nephropathy, Wilms tumour and intersex disorder. CASE REPORT: A 10 year-old Caucasian girl was referred to the Dental Department, at Glasgow Royal Hospital for Sick Children by her Paediatric Nephrologist Consultant. The patient was being teased by her peers over her markedly discoloured teeth. The dental history revealed that the patient was a regular dental attendee from an early age. She was dentally anxious having only experienced dental treatment under general anaesthesia (GA) when she was 4 years old. Apparently her primary dentition also showed a generalised discolouration. TREATMENT: This consisted of multiple visits for diet analysis and tooth brushing instruction with the use of disclosing tablets. Plaque control significantly improved when using a battery operated toothbrush because of its larger handle which the patient found easier to use and a 0.05% sodium fluoride mouthwash was given for daily use. Dyract(AP) veneers directly bonded onto maxillary permanent incisors and mandibular permanent anterior teeth was carried out. This was an interim measure to improve the patient's appearance while assessing the patient co-operation. The compomer facings were replaced with BelleGlass NG veneers which were cemented onto the maxillary incisors and mandibular anterior teeth using Adhesive By Choice (ABC) system under rubber dam on two separate visits. FOLLOW-UP: At her last visit, 27 months after treatment, the patient was still satisfied with the restorative treatment. However, further teeth had erupted including all the premolars. BelleGlass NG crowns were indicated. It was also decided to replace the preformed chrome cobalt crowns with full coverage BelleGlass NG crowns.