Oral health-related quality of life in Fanconi anemia: a cross-sectional study.

PURPOSE: To evaluate the oral health-related quality of life (OHRQoL) of individuals diagnosed with Fanconi anemia (FA). METHODS: A cross-sectional study was conducted with FA patients from two Brazilian referral centers. Participants underwent a complete dental, periodontal, and oral mucosa examination, as well as assessment of resting salivary flow. The short version of the Oral Health Impact Profile (OHIP-14) questionnaire was administered. Descriptive and bivariate analyses were performed, followed by multivariate analysis to examine the impact of independent variables on OHRQoL. RESULTS: The study included 20 (57.1%) males and 15 (42.9%) females, with a mean age of 18.9 years. Oral leukoplakia (OL) was found in 18 individuals. The overall OHIP-14 score was 9.9 ± 10.5. Individuals aged ≥ 16 years had higher OHIP-14 scores, indicating worse OHRQoL for physical pain (p = 0.007), psychological discomfort (p = 0.001), physical disability (p = 0.03), psychological disability (p = 0.001), handicap (p = 0.004), and overall score (p = 0.007). Females reported more negative OHRQoL than males for physical pain (p = 0.02), psychological discomfort (p = 0.03), psychological disability (p = 0.009), and overall score (p = 0.02). Individuals with OL had an overall OHIP-14 score 1.83 times higher than those without OL (95% CI: 1.02-3.28; p = 0.04). Lower salivary flow correlated with higher overall OHIP-14 scores (95% CI: 0.14-0.84; p = 0.01). CONCLUSION: This study represents the first attempt to evaluate OHRQoL in individuals with FA. The presence of OL and reduced salivary flow were identified as predictors of a negative impact on OHRQoL. It is imperative to integrate patients' quality of life in the clinical treatment protocols for the FA population.

Oral cancer and oral potentially malignant disorders in patients with Fanconi anemia - A systematic review.

The purpose of the present study was to perform a systematic review focusing on oral squamous cell carcinoma (OSCC) and oral potentially malignant disorders (OPMD) in Fanconi anemia (FA) individuals. Electronic searches were undertaken in five databases supplemented by manual scrutiny and gray literature. Case reports and/or cases series were included. The searches yielded 55 studies describing 112 cases of OSCC (n = 107) and/or OPMD (n = 5) in FA individuals. The mean age at diagnosis of OSCC/OPMD was 27.1 (±9.6) years, and females (51.8 %) were slightly more affected. Ulcer (n = 37) or mass (n = 25) were described as clinical presentations for OSCC and OPMD. White lesions (n = 4) were the most common manifestation in OPMD. Tongue (47.2 %) was the most frequent location. Sixty-one (54.5 %) individuals underwent HSCT. Surgical resection (n = 75) was the main treatment adopted. The estimated rate of OPMD malignant transformation was 1.8 % and recurrences following OSCC excision occurred in 26.8 % of individuals. Overall, at 60 months of follow-up, the probability of survival fell to 25.5 % and at 64 months the probability of recurrence increased to 63.2 %. The present data support the need for strict surveillance of patients with FA, even in the absence of OPMD, for early OSCC detection and reduction of mortality.

Manutenção de dentes retidos e associado com cisto dentígero após a marsupialização: série de casos / Maintenance of impacted teeth and associated with dentiger cyst after marsupialization: case series

Objetivo: O objetivo deste trabalho foi apresentar uma série de casos de manutenção de dentes retidos associados ao cisto dentígero (CD), empregando-se a marsupialização ou descompressão cística. Métodos: Realizou-se um estudo epidemiológico transversal, fundamentado na análise retrospectiva e descritiva dos registros do Serviço de Estomatologia e Cirurgia Bucomaxilofacial do HMOB (Hospital Metropolitano Odilon Behrens) entre novembro de 2005 a novembro de 2020. Resultados: Os resultados mostraram doze pacientes que receberam a marsupialização como tratamento do CD. Os dentes mais envolvidos foram pré-molares e incisivos centrais superiores e o tempo médio de erupção foi de 13 meses, sendo que a maioria das erupções ocorreram de forma espontânea. Em todos os casos verificou-se erupção do dente associado e neoformação óssea na região do cisto. Não foram observadas recidivas. Conclusão:Assim, baseado no presente estudo e na literatura, a marsupialização ou descompressão cística mostrou-se uma alternativa terapêutica eficiente na abordagem de pacientes jovens portadores de CD, inclusive, com o aproveitamento do dente associado ao cisto em casos selecionados.

Aim: The objective of this study was to present a series of cases of maintenance of impacted teeth associated with dentiger cyst (DC), using marsupialization or cystic decompression. Methods:A cross-sectional epidemiological study was carried out, based on a retrospective and descriptive analysis of the records of the Stomatology and Oral and Maxillofacial Surgery Service of the HMOB (Hospital Metropolitano Odilon Behrens) between November 2005 and November 2020. Results: The results showed twelve patients who received marsupialization as a treatment for DC. The most involved teeth were premolars and upper central incisors and the mean eruption time was 13 months, with most eruptions occurring spontaneously. In all cases, there was eruption of the associated tooth and new bone formation in the region of the cyst. No recurrences were observed. Conclusion: Based on the present study and the literature, marsupialization or cystic decompression is an efficient therapeutic alternative in approaching young patients with DC, including the use of the tooth associated with the cyst in selected cases.