Syndromes/Craniofacial Anomalies
Luisana S. Pagán, DMD
Second Year Resident Advanced Education Program in Pediatric Dentistry
University of Puerto Rico Medical Science Campus
University of Puerto Rico Medical Science Campus
San Juan, Puerto Rico
Rosana Hanke Herrero, DMD
University of Puerto Rico Medical Sciences Campus
Cheryl Paulo Malave, DMD
University of Puerto Rico, Medical Sciences Campus
Emilio Agrait Delfillo, DMD
Program Director
University of Puerto Rico - Medical Sciences Campus
San Juan, Puerto Rico
Introduction: Autoimmune polyglandular syndromes (APS) are polyendocrinopathies characterized by autoimmune dysfunction of multiple endocrine organs. APS Type 2 (APS2) is characterized by primary adrenal insufficiency and either type 1 diabetes mellitus or autoimmune thyroiditis. The cause of APS2 is still unknown, although it may involve a combination of genetic and environmental factors.
Case Report: This case report details the dental rehabilitation under general anesthesia of a 9-year-old male with APS2 with clinical manifestations of Addison Disease, Hyperthyroidism, Hashimoto's Thyroiditis, Diabetes Type 1, and Sjogren Syndrome. Current medications include Prednisone, Lantus, Humalog, Plaquenil, Imuran, and Creon. The patient has no reported drug allergies. Clinical examination revealed multiple dental caries, gingivitis, enamel defects, oral candidiasis and xerostomia. This report will include oral manifestations associated with APS2 and highlights the role of an multidisciplinary team in managing the patient before surgery and the care provided during the immediate postoperative period.