Orthodontic Management of a Pediatric Patient Affected by Congenital Adrenal Hyperplasia: Case Report

The aim of this study is to report the case of nonclassical congenital adrenal hyperplasia (NCAH) observed in a female child, and to present the management of the surgical-orthodontic rehabilitation, through clinical and histological oral findings.

A 13-year-old female was referred to our department for dental evaluation. The patient was affected by NCAH, confirmed by hormonal and genetic analysis from the age of 6 years, due to the appearance of premature pubic hair. At the time of the visit, she was in treatment with hydrocortisone, modulated on hormonal doses every 6 months. Clinical intraoral and extraoral examination, as well as radiographic and model analysis, were performed to obtain an accurate diagnosis. The impaction of the upper permanent canines required a surgical-orthodontic intervention for their exposure. Due to orthodontic traction impediment, after 3 months, a second surgery was required: the mucosal and fibrous tissues covering the impacted teeth were also removed and histologically examined, revealing hyperparakeratosis and inflammatory infiltrate.

Two and a half years later, the appliance was removed. A correct occlusal relationship and a good aesthetic profile were achieved.

This clinical case, the first in the literature, showed that a microscopic alteration of the superficial and deep periodontium can be hypothesized in children with CAH, which could result in difficulty in tooth eruption associated with difficulties in orthodontic movement and, therefore, would require more careful management.