Oculo-facio-cardio-dental (OFCD) syndrome: a case report.

BACKGROUND: Oculo-facio-cardio-dental (OFCD) syndrome is a rare condition that affects the eyes, face, heart, and teeth of patients. One notable dental characteristic of OFCD is radiculomegaly, or root gigantism, which highlights the role of dentists in detecting this syndrome. OFCD is an X-linked dominant syndrome that results from a variant in the BCOR gene. Our study presents the first documented case of OFCD in Vietnam and reports a novel BCOR gene variant observed in this case. CASE PRESENTATION: A 19-year-old Vietnamese female patient with an extremely long root with an abscess was clinically examined for the expression of OFCDs. The radiograph and the variant in BCOR gene were also evaluated. We identified abnormalities in the teeth, as well as ocular, facial, and cardiac features, with radiculomegaly of the canines being a specific symptom for OFCDs. The patient's genetic analysis revealed a pathogenic heterozygous deletion at intron 11 of the BCOR gene, representing a novel variant. CONCLUSION: Oculo-facio-cardio-dental syndrome (OFCD) is an extremely rare condition characterized by abnormalities in the eyes, face, heart, and teeth, often caused by variants in the BCOR gene. Radiculomegaly, or enlarged dental roots, is a key diagnostic feature of OFCD, and early detection is crucial for preventing future dental complications.

Clinical and radiological characteristics of odontomas: A retrospective study of 90 cases.

Purpose: Odontomas represent a common clinical entity among odontogenic tumors, but are not well-addressed in the Vietnamese population. The present study aimed to determine the clinical and preclinical characteristics of odontomas and associated factors in the Vietnamese population. Materials and Methods: This retrospective study retrieved data from histopathological diagnoses from 2 central hospitals of Odonto-Stomatology in Ho Chi Minh City, Vietnam during 2004-2017. The odontomas were classified as complex (CxOD) or compound (CpOD) subtypes. The epidemiological, clinical, and radiological characteristics of the odontomas, stratified by subtype and sex, were obtained and analyzed. Results: Ninety cases, consisting of 46 CxODs and 44 CpODs, were included. The average age of patients was 32.4 (±20.2) years. The patients with CxOD were older than those with CpOD (P<0.05). Clinically, 67% of patients showed an intraoral bone expansion. Approximately 60% of patients with CxOD exhibited a painful symptom, about 3-fold more than those with CpOD (P<0.05), whereas almost all patients with CpOD exhibited perturbations of dentition, unlike those with CxOD (P<0.05). Radiologically, CxOD was characterized by a larger dimension than CpOD in both sexes (P<0.05), and CpOD induced complications in adjacent teeth more often than CxOD (P<0.05). The development of odontoma with advancing age differed significantly in odontoma subtypes related to their pathological origins, and between the sexes, resulting from different physiological states. Conclusion: The findings of this study highlight the value of clinical and radiological features of odontomas and their associated factors for the early diagnosis and adequate treatment of younger patients.