Oral manifestations of tuberous sclerosis complex: A systematic review

Background

A heritable neurocutaneous condition known as Tuberous Sclerosis Complex (TSC), is characterized by multisystem involvement with multiple hamartomatous tumors, seizures, intellectual disability, and facial angiofibroma affects the human body.

Aim

We aim to study the literature and highlight all the oral and dental manifestations encountered in TSC patients.

Materials and methods

Published articles having a population of tuberous sclerosis patients with oral manifestations, regardless of age or gender, and articles written in English were included. An electronic search was conducted in the PubMed, Google Scholar, Lilac, EBSCO, and SciELO databases from 1964 to 2023. Google Scholar articles were searched and selected manually. To calculate the risk of bias, the Joanna Briggs Institute critical appraisal criteria for quality evaluation was used.

Results

A total of 37 articles were selected out of 1203 in which only case reports (CR) and cross-sectional studies (CS) [descriptive (CS-D) & analytical (CS-A)] met the eligibility criteria. The present systematic review is qualitative. The risk of bias for case reports, 35.7% (n = 10) received a low grade while 57.14% (n = 16) received a moderate grade. For cross-sectional studies, 33.3% (n = 3) received a moderate grade, while 11.1% (n = 1) received a low score. The primary oral outcomes were dental enamel pitting (60% in CR, 79% in CS-D, and 96% in CS-A). gingival growth/hyperplasia/enlargement, (63% in CR, 37.6% in CS-D, and 14% in CS-A), and intra-oral fibroma (18% in CR, 39% in CS-D, and 14% in CS-A). Other secondary oral manifestations were intra-oral hypopigmented macule (n = 3, CR), enamel hypoplasia of teeth (10% in CR, 11.7% in CS-A), dental anomalies like enamel pearl (9.9% in CS-D), odontogenic tumors like desmoplastic fibromas (n = 1, CR), odontogenic myxoma in the jaws (n = 2, CR), hemangioma of the tongue (n = 1, CR) compound odontoma (n = 1, CR), angiomyolipoma (n = 1, CR), and impacted teeth (10%, CR).

Conclusion

Dental enamel pitting, gingival hyperplasia, and intra-oral fibroma are the most common oral manifestations in TSC patients. Other features included intra-oral hypopigmented macules, enamel hypoplasia of teeth, gingival angiofibroma, and dental anomalies such as enamel pearls.