Dental Management of a Pediatric Patient with Congenital Adrenal Hyperplasia: A Case Report

Introduction: Congenital adrenal hyperplasia (CAH) is a rare inherited autosomal recessive disorder that leads to insufficient cortisol production caused by mutations in enzymes involved in cortisol biosynthesis. CAH is the most common cause of primary adrenal insufficiency during childhood. Children with CAH are at risk for adrenal crises and mortality. With proper therapy and management, patients can survive into adulthood. The dental management of a pediatric patient with CAH (21-hydroxylase deficiency) who required extensive dental treatment is presented.

Case Description: : A 7-year-old male with a medical history of CAH presented to Nicklaus Children’s Hospital Outpatient Center, with chief concern of extensive caries, , and inability to cooperate for dental treatment. Patient was taking the following medications: 12.5mg hydrocortisone TID, 0.1mg fludrocortisone daily. Upon clinical examination, extra-oral exam revealed patient had very short neck and obesity. Intra-oral examination revealed generalized caries. Due to a short neck, obesity, generalized caries, and poor behavior, it was decided to complete oral rehabilitation under general anesthesia. Endocrinology medical consultation was ordered, and recommendations were as follows: day before procedure, take hydrocortisone 25mg TID for 24hrs starting night before procedure; day of procedure, take 80mg hydrocortisone bolus. If procedure is well tolerated and able to tolerate PO intake, then discharge home and instruct to take 25mg of hydrocortisone 2 more times and return to maintenance dose the next day if doing well. Patient had no complications.

Discussion: CAH is a very rare disorder that can lead to many long term complications if not properly managed. CAH has two main types of clinical presentations, classic and non-classic. Due to lack of cortisol production in individuals with CAH, there is a failure of negative feedback and excessive secretion of corticotrophin-releasing hormone from the hypothalamus and Adrenocorticotropic Hormone (ACTH) from the anterior pituitary. Continued secretion of ACTH causes unremitting stimulation of the adrenal cortex, leading to hyperplasia.

Conclusion: Children with CAH need careful considerations prior to dental procedures in order to avoid acute adrenal crises. Providers should always obtain a medical consult and consider hospitalizing the patient if major surgical procedure is planned. Anxiety and stress increase risk of adrenal crisis, therefore stress reduction techniques should be used.