266 - Oral Manifestations in a Patient with Rett Syndrome: Case Report

Rett Syndrome is a neurological and developmental disorder that primarily affects females and disrupts normal brain development. It leads to a loss of motor skills and language. Typically, babies with Rett syndrome develop normally during the first 6-9 months, after which they begin to lose previously acquired abilities, such as crawling, walking, and communicating. Oral manifestations of Rett Syndrome can include a high palate, anterior open bite, bruxism, gingivitis, periodontitis, gingival hyperplasia, xerostomia, glossitis, erythema multiforme, and delayed eruption of teeth. Among these, bruxism is the most common oral finding, often attributed to medications used in treatment. 

This presentation focuses on a 15-year-old patient with Rett Syndrome who exhibits delayed eruption of permanent teeth. The patient was originally referred for a dental consultation with a chief complaint of delayed tooth eruption. Upon comprehensive clinical and radiographic examinations, it was found that the patient has generalized unerupted permanent teeth, with most primary teeth still present. Additionally, the permanent teeth exhibited fully formed roots but displayed an abnormal eruption pattern. Generalized delayed unerupted permanent teeth can be an indicator for endocrine imbalance such as: hypothyroidism, hypoparathyroidism, or growth hormone deficiency, which are not uncommon in such cases. This patient’s endocrinologist has concerns about the patient’s small statue and failure to thrive as well. This report aims to explore the dental findings associated with Rett Syndrome and how these oral manifestations may serve as early indicators of systemic issues that the patient may not be aware of. 

Identify Supporting Agency and Grant Number: