General and Stomatologic Aspects of Bardet-Biedl Syndrome

Abstract

Many patients with this syndrome have compromised oral hygiene, difficulty in addressing dental cases, developmental delay, and autistic behavior. Thus, it is important to pay attention to these early children from an early age and encourage preventive approach.

Oral and Craniofacial Anomalies of Bardet-Biedl Syndrome: Dental Management in the Context of a Rare Disease

“…Dentofacial anomalies are present in >50% of affected individuals in concert with other clinical features, including intra-and extraoral manifestations (see Table 2). Common extraoral manifestations are orbital hypertelorism, strabismus, philtrum discrepancies, hypotonic upper lip mouth breathing, retrognathia, incompetent lips, and bitemporal narrowing (Moore et al 2005;Drugowick et al 2007;Majumdar et al 2012;Ferreira et al 2014;Hassona et al 2017). Ptosis, palpebral fissure discrepancies, down-turned mouth, brachycephaly, macrocephaly, long ears, frontal balding in males, flat nasal bridge, and maxillary atresia were reported by Moore et al (2005).…”

“…The intraoral manifestations reported to date include hypodontia, predominantly with absent maxillary and mandibular premolars, followed by lower lateral incisors, higharched palate, microdontia, crowding and spacing problems, and short dental roots in anterior and posterior teeth with obliterated pulp chambers (Borgström et al 1996;Urben et al 1999;Moore et al 2005;Drugowick et al 2007;Majumdar et al 2012;Andersson et al 2013;Ferreira et al 2014;Hassona et al 2017). Taurodontism (Andersson et al 2013), unilateral and bilateral posterior crossbite (Ferreira et al 2014;Hassona et al 2017), and thicker mandible (Borgström et al 1996) are less common. Enamel hypoplasia was also reported by Beales et al (1999).…”

“…In most cases, drug-induced gingival hyperplasia is consequential to prescribed pharmaceutical exposures, including calcium channel blockers, anticonvulsants, and immune suppressants (Drugowick et al 2007;Ferreira et al 2014;Hassona et al 2017). Drug-induced gingival hyperplasia is mostly seen in patients suffering from renal and neurologic problems and may cause tooth malposition and delayed tooth eruptions.…”

Bardet Biedl Syndrome: Insights for the Oral Healthcare Provider

“…Orofacial manifestations observed in BBS are listed in Dental crowding is observed in about 46% of affected individuals, placing them at increased risk of poor oral hygiene, plaque accumulation, periodontitis and halitosis [18,20]. A fraction of patients may exhibit hypodontia, with resultant difficulties in speech, mastication, and phonation.…”

“…[1,3,15,[18][19][20]. Mouth breathing is observed in about 60% of patients and could lead to increased anterior facial height, dry mouth, malocclusion, increased dental caries incidence, sleep disturbances and nocturia[1,21].…”