The prevalence of orofacial clefts in Qatar : a cross-sectional nationwide study.

BACKGROUND: Cleft lip and palate are congenital craniofacial anomalies that significantly impact individuals and their families, both medically and psychosocially. The Qatari population has unique characteristics that are suggestive of a high prevalence of congenital anomalies: high consanguinity rate, large family size, advanced paternal age and high prevalence of certain genetic disorders. The lack of existing data concerning the epidemiology of cleft lip and/or palate in Qatar warrants a descriptive study addressing this topic. METHODS: A retrospective nationwide hospital-based cross-sectional study conducted to determine the prevalence of orofacial clefts in Qatar from 2016 to 2021. Data were extracted from the corporation's Cerner database and electronic health records. Information concerning the cleft's characteristics, type, affected side, patient's gender, presence of associated syndromes, nationality, and maternal age were collected. RESULTS: Out of the 147,727 live births, 148 had an orofacial cleft. The prevalence of cleft lip and/or palate was determined to be 1 per 1000 livebirths (95% CI: 0.85, 1.18). The prevalence of cleft lip was 0.18 (95% CI: 0.12, 0.27), cleft palate 0.39 (95% CI: 0.30, 0.51), and cleft lip and palate 0.43 (95% CI: 0.33, 0.55). Qataris had a prevalence of CL 0.25, CP 0.40, and CLP 0.56, compared to 0.16, 0.39, 0.39 for non-Qataris (p-value 0.186). Unilateral clefts predominated over bilateral (74.4% and 25.6%, respectively). Among the unilateral cases, 70.2% occurred on the left side. Most cases were isolated clefts, with only 10.2% having associated syndromes. CONCLUSIONS: The prevalence of orofacial clefts in Qatar is consistent with the globally reported prevalence. Most cases were unilateral and on the left side. Associated syndromes were infrequent and more common with cleft palate alone. Intriguing patterns were revealed between Qatari nationals and non-Qatari residents, with specific subtypes of orofacial clefts showing higher prevalence among nationals.

Radiological evaluation of odontogenic keratocysts in patients with nevoid basal cell carcinoma syndrome: A review.

Background: Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an autosomal dominant syndrome that has various expressions in each patient. Generally; NBCCS is followed by multiple nevoid basal cell carcinoma of the skin, orbital anomalies, skeletal anomalies, central nervous system anomalies and multiple odontogenic keratocysts (OK). NBCCS is usually diagnosed between the ages of 5-30 years, with multiple basal cell carcinomas of the skin and OKs in the jaws as the initial findings. The purpose of this paper is to describe and compare the radiographic findings of the OKs in NBCCS patients in the literature with additional cases. Materials and Methods: In this study, we evaluated the OKs of the patients with NBCCS in PubMed Database with 5 additional cases from our database. A total of 305 articles were found and the articles in English with full-text access were evaluated. Results: Despite all limitations for a fair discussion; we would like to state that among 59 cases that specified whether a 3D or 2D imaging modality was used, 29 cases were only interpreted with 2D data which should be avoided in OK evaluation. Discussion: According to the World Health Organization's Classification of Head and Neck Tumours Book which was published in 2017, OKs in NBCCS has a higher chance to have small satellite cystic lesions which increase their recurrence possibility post-operatively, thus, a thorough clinical and 3D radiographic evaluation should be performed both to NBCCS patients and non-syndromic OK patients to avoid any recurrence. Conclusion: High recurrence rates of OKs should be reminded all the time. Radiographic examinations with 3D imaging modalities should be done in patients with NBCCS in order to provide a concise diagnosis and optimum treatment.