Dental management and oral manifestations in child with Robinow syndrome: case report

Abstract

Robinow syndrome (RS) is a rare genetic disease that may involve cardiovascular, skeletal and urogenital systems and manifest through short limb dwarfism, defects in vertebral segmentation, hypoplastic genitalia and cranial and facial abnormalities. In this article, we hereby present a patient of a 10-year-old Brazilian boy with RS who presented with a remarkable number of classical general and dental features. Physical examination revealed disproportionate short stature with shortening limbs, syndromic face with normal hair appearance, low-set ears, macrocephaly, prominent forehead, hypertelorism and ocular prominence, nose anomalies, triangular mouth with long/short philtrum, incompetent lip seal, midface hypoplasia, retrognathia, deficient malar prominence, among others. Clinical examination revealed gingival hyperplasia, malocclusion, crowding dental, diastemas, microdontia, enamel hypoplasia, prolonged retention, dental caries, delay of dental eruption v-shaped palate, bifid tongue and ankyloglossia history. Radiographically, dental agenesis, hypertaurodontism and thick roof pulp chambers and delayed tooth eruption were observed. Preventive, restorative and rehabilitative dental procedures are being carried out. Robinow syndrome presents clinical and radiographic findings that may be present from birth and require pediatric dentistry follow-up. Furthermore, the pediatric dentist plays an essential role in developing effective planning and treatment in terms of health promotion, aesthetic rehabilitation and functional interventions in the stomatognathic system.