The impact of dental intervention under general anesthesia in Kohlschutter-Tonz Syndrome: a case series.

ABSTRACT:

Background

Kohlschutter-Tonz syndrome (KTSZ) is a rare, autosomal recessive neurodegenerative disorder. Patients suffer from a triad of developmental delays, epilepsy, and amelogenesis imperfecta (AI). Most reports of KTSZ patients focus on the genetic pattern of the disease and medical features, dental care is not considered a primary part of the initial intervention. Thus, the aim of the current work was to evaluate the impact that dental treatment plans had on both patients and caregivers.

Case presentation

We present a series of five pediatric patients, aged 4-14 years, and diagnosed with KTSZ. All patients were from Druze origin and carried the same ROGDI gene mutation. Patients were suffering from oral and dental disease due to a lack of prior dental treatment as well

SUBMITTER: Absawi MK 

PROVIDER: S-EPMC12261647 | biostudies-literature | 2025 Jul

REPOSITORIES: biostudies-literature