Comprehensive dental management of a patient with Crouzon syndrome: a case report.

Abstract

Crouzon syndrome (CS) is a genetic form of syndromic craniosynostosis inherited in an autosomal dominant manner, with an estimated incidence of approximately 1 in 60,000 live births. It is associated with mutations in the FGFR2 and FGFR3 genes. The condition is characterized by the premature fusion of cranial sutures, particularly the coronal and sagittal sutures, resulting in craniofacial deformities, functional impairments, and psychosocial consequences. Common manifestations include maxillary hypoplasia, exophthalmos, hypertelorism, respiratory obstruction, hearing loss, and speech difficulties. From a dental perspective, patients may exhibit severe malocclusions, crossbite, dental crowding, dental agenesis, a high-arched palate, and an increased susceptibility to dental caries and periodontal disease. This case report aimed to describe the comprehensive dental management of a patient with Crouzon syndrome. This case report describes a 34-year-old female patient with a confirmed diagnosis of CS who received treatment at the Faculty of Dentistry of the Evangelical University of El Salvador between 2024 and 2025. Clinical examination revealed retained primary teeth, multiple carious lesions, bilateral crossbite, dental agenesis, poor oral hygiene, and grade 2 gingivitis. Radiographic examinations and cone-beam computed tomography (CBCT) were essential for assessing dental structures and planning comprehensive treatment. A multidisciplinary treatment approach was implemented. Periodontal treatment included scaling, root planing, and prophylaxis. Restorative treatment involved multiple composite resin restorations. Surgical treatment included extraction of the root remnant of tooth 5.4 using the tell-show-do behavior guidance technique. Finally, a Wipla removable partial denture was fabricated based on the characteristics of the abutment teeth (primary teeth), taking into account the patient’s dental limitations and palatal morphology. The treatment improved esthetics, masticatory function, and the patient’s self-esteem. The findings are consistent with the literature, particularly regarding malocclusions, dental biofilm, and gingivitis. Crouzon Syndrome represents a clinical challenge that requires early diagnosis, comprehensive care, and multidisciplinary management. Dental intervention is essential for preventing complications.