Case Report

Hypophosphataemic Rickets Secondary to Raine Syndrome: A Review of the Literature and Case Reports of Three Paediatric Patients’ Dental Management

Table 2

The clinical, radiographic, and histological features of Hereditary Hypophosphataemic Rickets (HHR) and Raine Syndrome (RS) as described in the literature [2, 49, 11, 14, 15].

DiagnosisClinical featuresRadiographic featuresHistological features

Hereditary Hypophosphataemic Rickets (HHR)Recurrent spontaneous dental abscesses, spontaneous loss of vitality, sinus tracts, eruption anomalies, defective dentine mineralisation, yellow-to-brown enamel hypoplasia, increased periodontal disease, malocclusionRoot resorption, pulp horns extending to enamel–dentine junction (EDJ), taurodontism, poorly defined lamina dura, hypoplastic alveolar ridgeTubular dentinal clefts, increased and hypomineralised interglobular dentine, reduced secondary dentine, widened predentine, microclefts in enamel surface

Raine Syndrome (RS)Hypoplastic amelogenesis imperfecta (AI), delayed dental eruption, high-vaulted palate, cleft palate, malocclusion, gingival enlargement, thin, yellow, and translucent enamel, incisal notch of central incisorsEctopic eruption, pulpal calcifications, root hypoplasia, periapical pathology, lack of differentiation between enamel and dentineGingival or follicular calcifications, increased interglobular dentine, thin enamel