A dental approach to Proteus Syndrome: a case report

Abstract

Proteus syndrome (SP; OMIM #176920) is a rare complex disorder, characterized by the asymmetric postnatal growth of different parts of the body which tends to progress throughout life. Its prevalence is estimated at 1 per 1,000,000 live births. The clinical features are: partial gigantism of extremities, plantar hyperplasia, macrocephaly, cranial hyperostosis and asymmetry of the limbs due to the growth of long bones and soft tissues. Some of those affected may have intellectual impairment. Diagnosis is based on clinical criteria, imaging and genetic exams. The mainstay of treatment is early diagnosis of the disease and medication for symptoms presenting throughout life to improve quality of life. This case report presents a female patient, from 4½ years of age to the present, with asymmetric growth of the face and upper and lower limbs, undergoing dental follow-up at the Dental Service of a Specialist Center for Rehabilitation and Readaptation in Goiânia, Brazil. She presents delay in neuropsychomotor development, pronounced osteopenia, does not speak and is dependent in terms of activities of daily living. The eruption of deciduous teeth began at 4 months and permanent dentition at 4 years of age. During the current clinical examination of the child at 12 years and 2 months we found severe enamel hypoplasia in the teeth on the right side, mandibular prognathism, maxillary atresia, ogival palate, and anterior crossbite. The treatment plan included prevention and maintenance of oral health, monitoring of enamel hypoplasia, rhizolysis of the deciduous dentition and of eruption of permanent dentition, and restoration and extraction of teeth which were causing traumatic lesions on soft tissues. The parents' concerns were the malformations in dental enamel and traumatic lesions in the mucous membranes caused by badly positioned teeth. The interventions performed were composite resin restorations, tooth extractions, oral hygiene guidance and topical application of fluoride. Orthodontic treatment was contraindicated due to the severity of the disease. The dental approach in severe cases of PS must be individualized, with emphasis on oral hygiene and the removal of traumatic factors which damage soft tissues and cause pain. Dental surgeons must be prepared for individualized management.