Orofacial Manifestations and Dental Management of Patients With Familial Dysautonomia

Objectives: This study reviews the orofacial manifestation of patients with Familial Dysautonomia (FD) and introduces findings on dental management.
Methods: Suitable literature were identified by electronic search using Medline, Pubmed and Cochrane database. Articles published between 1980 to 2016 were searched, screening abstracts that fulfilled the inclusion criteria.
Results: FD is an inherited autosomal recessive disorder found almost exclusively in children of Jewish Ashkenazi origin. In 2001, the gene responsible for FD was discovered as IkB-Kinase associated protein gene (IKBKAP) leading to a decline in its prevalence by prevention; life expectancy improved from early childhood to late fifties. Clinical characteristics include universal progressive motor, sensory, and autonomic neuropathy, skeletal deformities, respiratory distress, recurrent aspiration pneumonia, orthostatic hypotension and compensatory tachycardia, and profound oro-dento-facial deformities. Oral features are lack of fungiform papillae on dorsum of the tongue, dysgeusia, lack of sensitivity with high rates of trauma and orodental self-mutilation. Periodontal diseases are prevalent due to poor oral hygiene and motor skills. Low risk of caries development as a result of salivary gland hypersalivation and salivary drooling due to muscle-control difficulty, are common.
Conclusions: Orofacial treatment of FD patients should be individualized due to variation in response and autonomic instability. An interdisciplinary approach to care is suggested, integrating the dental and the FD health care teams. It is recommended to promote frequent dental visits, immediate treatment of emergencies and perioperative management of systemic conditions during orofacial treatment under sedation or in the operating room. Use of radiographs and comprehensive intraoral examination are critical since vitality tests are not reliable due to somatosensory abnormalities. Restorations and exodontia are to be completed without local anesthesia due to sensory impairment, unless requested otherwise by the patient; an upright chair position is necessary to prevent the risk of aspiration.