Síndrome del incisivo central maxilar único: reporte de caso

El síndrome del incisivo central maxilar único es una rara alteración en el desarrollo y formación de órganos ubicados principalmente en la línea media; el cual ocurre de manera temprana entre los días 35 al 38 de vida intrauterina. Su etiología es desconocida, aunque se ha asociado a deleciones de los cromosomas 7 (7q.36.1) y 8, y a mutaciones en el gen Sonic Hedgehog. Presenta una prevalencia de 1/50.000 nacidos vivos y aunque es una anomalía poco frecuente del desarrollo craneofacial, su diagnóstico y tratamiento temprano son importantes para los odontólogos generales o especialistas ya que puede ser un signo de otras anomalías congénitas o del desarrollo graves. Por lo tanto, el objetivo de este caso es reportar la fase inicial de tratamiento en un niño con el síndrome de incisivo central maxilar único quien no había sido diagnosticado anteriormente con este síndrome. Caso Clínico: Paciente masculino de 10 años de edad, procedente de Jamundí, Valle del Cauca- Colombia. Reporta ausencia de órgano dentario superior. En el examen intraoral se observa un incisivo central único sobre la línea media del maxilar, ausencia de frenillo labial y papila incisiva, paladar oval y retrognatismo mandibular. Fue tratado en una primera fase con ortopedia funcional maxilar para mejorar la clase II y está a la espera de iniciar la segunda fase de tratamiento con ortodoncia. Conclusiones: El síndrome de incisivo central maxilar único es un síndrome poco frecuente el cual conlleva múltiples afecciones que interfieren en el normal desarrollo y crecimiento de estructuras anatómicas.

A síndrome do incisivo central superior único é uma alteração rara no desenvolvimento e formação de órgãos localizados principalmente na linha média; que ocorre precocemente entre os dias 35 a 38 de vida intrauterina. Sua etiologia é desconhecida, embora tenha sido associada a deleções dos cromossomos 7 (7q.36.1) e 8, e mutações no gene Sonic Hedgehog. Tem uma prevalência de 1/50.000 nascidos vivos e, embora seja uma anomalia rara do desenvolvimento craniofacial, seu diagnóstico e tratamento precoces são importantes para dentistas gerais ou especialistas, pois pode ser sinal de outras anomalias congênitas ou de desenvolvimento graves. Portanto, o objetivo deste caso é relatar a fase inicial do tratamento em uma criança com síndrome do incisivo central superior único que não havia sido previamente diagnosticada com essa síndrome. Caso clínico: Paciente do sexo masculino, 10 anos, procedente de Jamundí, Valle del Cauca- Colômbia. Relata ausência de órgão dentário superior. O exame intraoral mostra um único incisivo central na linha média maxilar, ausência de frênulo labial e papila incisiva, palato oval e retrognatismo mandibular. Foi tratado numa primeira fase com ortopedia funcional maxilar para melhorar a classe II e aguarda para iniciar a segunda fase do tratamento ortodôntico. Conclusões: A síndrome do incisivo central superior único é uma síndrome rara que envolve múltiplas condições que interferem no desenvolvimento e crescimento normal das estruturas anatômicas.

Solitary maxillary central incisor syndrome is a rare alteration in the development and formation of organs located mainly in the midline; which occurs early between days 35 to 38 of intrauterine life. Its etiology is unknown, although it has been associated with deletions of chromosomes 7 (7q.36.1) and 8, and mutations in the Sonic Hedgehog gene. It has a prevalence of 1/50,000 live births and although it is a rare anomaly of craniofacial development, its early diagnosis and treatment are important for general dentists or specialists since it can be a sign of other serious congenital or developmental anomalies. Therefore, the objective of this case is to report the initial phase of treatment in a child with solitary maxillary central incisor syndrome who had not been previously diagnosed with this syndrome. Clinical case: Male patient, 10 years old, from Jamundí, Valle del Cauca- Colombia. Reports absence of upper dental organ. Intraoral examination shows a solitary central incisor on the maxillary midline, absence of labial frenulum and incisive papilla, oval palate and mandibular retrognathism. He was treated in a first phase with maxillary functional orthopedics to improve class II and is waiting to start the second phase of orthodontic treatment. Conclusions: Solitary maxillary central incisor syndrome is a rare syndrome which involves multiple conditions that interfere with the normal development and growth of anatomical structures.

Relationship between non-syndromic oral clefts and cancer: A systematic review and meta-analysis.

OBJECTIVE: To summarize the clinical evidence on the relationship between cancer and non-syndromic oral cleft (NSOC). METHODS: The review was reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist, and a literature search was conducted in six databases and gray literature. Studies published in any language mentioning cancer in patients with NSOC and their relatives and NSOC in patients with cancer and their relatives were included. Risk of bias was assessed using the Joanna Briggs Institute appraisal tool. The certainty of the evidence was evaluated using the GRADE (Grading of Recommendation, Assessment, Development, and Evaluation) assessment. After a 2-step selection process, 33 studies were included: 17 case-control studies, 13 cross-sectional studies, and 3 case reports. RESULTS: The study evaluated 206,096 patients from 20 countries. Of these, 0.35% of patients with cancer (95% CI: 0.0%-1.1%; I2  = 86%), 3.0% of relatives of patients with cancer (95% CI: 1.19%-5.46%; I2  = 55%), and 0.26% of controls (95% CI: 0.0%-0.83%; I2  = 87%) had NSOC. Among the studies that examined the prevalence of cancer, 2.4% (95% CI: 0.0%-19.3%; I2  = 99%) of patients with NSOC, 15.4% of relatives of patients with NSOC (95% CI: 2.0%-37.6%; I2  = 99%), and 5.3% of controls (95% CI: 0.0%-22.8%; I2  = 99%) had cancer. Although no relationship was observed between the risk of cancer in patients with NSOC and the risk of NSOC in patients with cancer, there was an association for an increased risk of cancer in relatives of patients with NSOC (OR: 9.96, 95% CI: 1.55-63.99; p = 0.01) and a significant association for the NSOC risk in relatives of patients with leukemia (OR: 9.31; 95% CI: 1.13-76.67; p = 0.03). CONCLUSION: Our findings demonstrate an increased risk of cancer in relatives of patients with NSOC and that relatives of patients with leukemia were more frequently affected by NSOC. Together, these findings can help guide cancer screening in patients with NSOC and their relatives and shed light on the risk of NSOC in families with a history of cancer.

Hemifacial microsomia: treatment alternatives-a systematic review of literature.

OBJECTIVE: To systematically review literature on therapeutic options for treating hemifacial microsomia (HFM), in young patients with growth potential, classifying and comparing the different dentofacial treatment methods. STUDY DESIGN: An independent review of databases (Scopus, Embase, Ovid, Cochrane Library and PubMed) following the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA), conducted by four evaluators. The protocol of this study was registered in International prospective register of systematic reviews (PROSPERO), under the number CRD42021293076. RESULTS: Between 1970-2021, a total number of 1137 articles were published of which 27 were included in this study according to the selection criteria: one randomized multicentric trial, two case-control studies, three case series and 21 case reports. CONCLUSIONS: The most common orthopedic treatments provide vertical stimulation of the maxillary process in the affected side. Orthodontic approaches are mainly applied for vertical correction and stabilization of the occlusal plane. Other treatment options include orthognathic surgery, osteogenic distraction, temporomandibular reconstruction and grafting. It is recommended that prospective clinical randomized controlled studies be conducted using homogeneous pediatric groups with long-term follow-up, to establish recommended evidence-based methods for treating each set of hemifacial microsomia symptoms.