ABSTRACT
PHACES syndrome is an acronym for the syndromic presentation of Posterior fossa malformation, Hemangioma, Arterial anomalies, Coarctation of aorta/cardiac defects, Eye abnormalities and Sternal malformations. Infantile hemangiomas are the most common tumors of infancy. Regional odontodysplasia, commonly referred to as "ghost teeth", is a rare localized developmental malformation of enamel and dentin with varying levels of severity that results in unusual clinical and radiographic appearances of affected teeth. This report describes a rare case of a two-year-old Caucasian male diagnosed with PHACES syndrome also presenting with multi-regional odontodysplasia. Ten of twenty teeth were dysplastic. The patient was treated under general anesthesia in a hospital setting. All affected primary teeth were extracted due to sensitivity, abscess and extremely poor long-term prognosis. Moving forward, a long-term interdisciplinary approach will be necessary to address this child's dentition as it develops.
Subject(s)
Aortic Coarctation , Eye Abnormalities , Neurocutaneous Syndromes , Odontodysplasia , Humans , Male , Aortic Coarctation/complications , Aortic Coarctation/diagnostic imaging , Odontodysplasia/diagnostic imaging , Eye Abnormalities/complications , Child, Preschool , Neurocutaneous Syndromes/complications , Cranial Fossa, Posterior/abnormalities , Cranial Fossa, Posterior/diagnostic imaging , Tooth ExtractionABSTRACT
PURPOSE: The aim of this mixed method study was to identify barriers for children with special health care needs (SHCN) to receiving routine preventive dental care following restorative dental care with general anesthesia (GA). METHODS: Electronic health records were reviewed for inclusion criteria and demographic data. Caregivers of children with SHCN were contacted to participate in qualitative interviews. Interview topics explored child, family, and community level influences to accessing routine dental care. Qualitative analysis identified key themes of barriers and enablers to care. RESULTS: A total of 1708 children received dental care with GA during the 2-year study period, of which 498 (29.16%) had a diagnosis of a SHCN. The most common type of SHCN was neurodevelopmental disorders (28.51%). The mean age at time of GA was 8.6 years. Fifty caregivers completed interviews. Identified barriers to obtaining routine dental care included child stress/anxiety, finding an accepting provider, dismissive providers, and proximity of provider/transportation to dental care. Enablers to obtaining care included effective behavior management, continuity of provider/care, positive provider attitude, and referral to an accepting provider. CONCLUSION: Adequately trained and local providers with an accepting attitude are essential to enabling children with SHCN to obtain equitable access to routine dental care.
