Three-Dimensional Evaluation of Dental Arches in Individuals with Syndromic Craniosynostosis.

Objective: Individuals with syndromic craniosynostosis present alterations in the dental arches due to anomalies caused by the early fusion of the craniomaxillary sutures. This study aimed to compare intradental and interdental dimensions between individuals with Apert and Crouzon syndromes and nonsyndromic controls. Materials and Methods: Digital models were obtained from the archive of a public tertiary care hospital. The sample consisted of 34 patients (Apert n = 18, Crouzon n = 16) and 34 nonsyndromic controls matched for gender and age. Measurements of perimeter, length, intercanine and intermolar distances (upper and lower), overjet, and molar ratio were performed. Statistical comparisons were performed using ANOVA and Tukey tests (p < 0.05). Results: Patients with Apert and Crouzon syndromes have severely reduced maxillary transverse dimensions, perimeter, and length of the upper arch compared to the control group (p < 0.001). The lower arch is less impacted. Patients with Apert syndrome had an anterior crossbite (p < 0.001), while patients with Crouzon syndrome had an edge-to-edge bite (p < 0.011). Patients with Apert and Crouzon syndromes do not have serious transverse proportion problems when comparing the upper and lower arches. Conclusions: In this sample, both the Apert and Crouzon groups have severely compromised upper arches compared to the control group. Mild dentoalveolar expansion in the maxilla should be sufficient for the transverse adaptation of the dental arches before frontofacial advancement.

Atresia maxilar associada à mordida aberta anterior tratada por meio de expansão rápida da maxila assistida cirurgicamente (ERMAC) / Transverse maxillary deficiency associated with open bite treated by surgically assisted rapid maxillary expansion (SARME)

Resumo A expansão rápida da maxila assistida cirurgicamente (ERMAC) é uma opção de tratamento segura e eficaz para a correção da mordida cruzada posterior em adultos. O objetivo deste artigo é relatar um caso clínico de uma paciente de 37 anos de idade com atresia maxilar e mordida aberta aberta anterior, tratada por meio da ERMAC. O plano de tratamento consitiu na expansão cirúrgica da maxila utilizando um aparelho de Hyrax. Foi realizada a cirurgia de Le Fort I associada a Osteotomia vestibular entre as raízes dos incisivos centrais superiores. Ao término do período ativo de expansão, foi instalado aparelho fixo corretivo no arco inferior e após 4 meses após a expansão foi removido o disjuntor e instalado aparelho fixo superior. Os fios de nivelamento foram evoluídos até .019"x.025" aço. Nesta fase, elásticos intermaxilares de Classe II e verticais anteriores foram utilizados para corrigir a Classe II sudbivisão esquerda e a mordida aberta anterior, respectivamente. Após a remoção dos aparelhos fixos corretivos, foram instaladas as contenções; placa Hawley no arco superior e 3×3 no arco inferior. A relação transversal foi corrigida de maneira eficiente por meio da ERMAC, alcançando assim, adequada relação interarcos. A oclusão finalizou estável com conformação do arco e do sorriso satisfatórios.(AU)

Abstract The surgically assisted maxilla expansion (ERMAC) is a safe and effective treatment option for correction of posterior crossbite in adults. The objective of this article is to report a clinical case of a 37-year-old patient with maxillary atresia and anterior open bite, treated by ERMAC. The treatment plan consisted of the surgical expansion of the maxilla using a Hyrax device. Le Fort I surgery was performed in association with vestibular osteotomy between the roots of the upper central incisors. At the end of the active expansion period, a fixed corrective device was installed in the lower arc and after 4 months after the expansion the circuit breaker was removed and a fixed upper apparatus was installed. Leveling wires were evolved up to. 019"x.025" steel. In this phase, intermaxillary elastics of Class II and anterior vertical elastics were used to correct Class II left sudbivision and anterior open bite, respectively. After removing the fixed corrective devices, the containments were installed; Hawley board in the upper arch and 3×3 in the lower arch. The cross-sectional relationship was efficiently corrected by means of the ERMAC, thus achieving an adequate interarcol relationship. The occlusion ended stable with conformation of the arch and the smile satisfactory. (AU)

Hyoid Bone Position and Head Posture in Patients With Richieri-Costa Pereira Syndrome (EIF4A3 Mutations).

Robin sequence with cleft mandible and limb anomalies, known as Richieri-Costa-Pereira syndrome (RCPS), is an autosomal recessive acrofacial dysostosis characterized by mandibular cleft and other craniofacial anomalies and respiratory complications. The aim of this cross-sectional study was to describe the hyoid and head posture of 9 individuals with RCPS using cephalometric measurements and provide a discussion about its implications in obstructive sleep apnea syndrome (OSAS). The study was conducted on lateral cephalograms of patients with RCPS and 9 selected age-matched controls in tertiary cleft center in Brazil. The cephalograms were digitized and analyzed on a software to obtain the vertical and horizontal hyoid position, its relationship with the mandible and the relation of the cranial base and postvertebral line. The t test was used for analysis of means and Levene's test for equality of variances.Cephalometric measurements H-S (vertical distance between hyoid bone and sella) (Supplemental Digital Content, Figure 1, http://links.lww.com/SCS/B247) and H-C4lp (horizontal position of the hyoid in relation to the post-pharyngeal space) showed statistically significant difference compared to controls (P < 0.05). Therefore, the hyoid bone was more inferiorly and posteriorly positioned in the study group compared with the control group. The vertebrae measurements did not present differences compared to controls. The described position of hyoid bone could be involved in the severe OSAS of RCPS patients.

Craniofacial Morphology in Patients With Opitz G/BBB Syndrome.

OBJECTIVE: To compare the cephalometric characteristics of patients with and without Opitz G/BBB syndrome type I. DESIGN: Cross-sectional, case-control study. SETTING: Tertiary cleft center in Brazil. PARTICIPANTS: Eighteen individuals with Opitz G/BBB syndrome with complete bilateral cleft lip and palate (BCLP), compared to 18 individuals with nonsyndromic complete cleft lip and palate and 18 individuals without malformations, matched for gender and age. INTERVENTIONS: Pretreatment lateral cephalograms of all patients were manually traced and digitized for achievement of linear and angular measurements. MAIN OUTCOME MEASURES: Analysis of variance or Kruskal-Wallis followed by Tukey tests were used for intergroup comparisons at a significance level of P < .05. RESULTS: Individuals with Opitz G/BBB syndrome exhibited alterations in SNGn, P-Co, and N'-Pr/Po-Or that were not attributable to BCLP. Co-Go, Sella-Nasion-Supramentale, ANB (maxillo-mandibular relationship), and anterior nasal spine-posterior nasal spine (ANS-PNS)/U1A-U1T were significantly different in both G/BBB and BCLP groups compared to control, but not different between G/BBB and BCLP groups. Anterior nasal spine-posterior nasal spine/Go-Gn, ANS-PNS, V-Upper pharyngeal wall, and U-lower pharyngeal wall were different in nonsyndromic BLCP compared to nonsyndromic controls and Opitz G/BBB group. CONCLUSION: Patients with Opitz G/BBB syndrome exhibited some unique cephalometric alterations compared to patients with nonsyndromic complete BCLP and controls.

Cephalometric Findings in Nine Individuals With Richieri-Costa-Pereira Syndrome.

The Richieri-Costa-Pereira syndrome (RCPS) is an autosomal-recessive acrofacial dysostosis caused by mutations in EIF4A3, characterized by mandibular cleft comprising other craniofacial anomalies and limb defects such as cleft palate/Robin Sequence, microstomia, absence of mandibular central incisors, minor ear anomalies, clubfeet and first and 5 ray defects. The findings from this study are useful for better understanding the morphological consequences of disorders of EIF4A3, and having a better picture of the anatomic characteristics of the syndrome for a better therapeutic planning. Twenty-four angular and linear variables were measured to assess anteroposterior and vertical (superior-inferior) position of the cranial base, maxilla, mandible, and facial profile. The cephalometric radiographic analysis was performed on 9 individuals with RCPS, obtained at a mean age of 10.3 years, and compared with randomly selected age-matched 9 controls, without clefts and with well-balanced faces, with mean age of 10.6 years (both groups range 8.1 to 13.7 years). t test was used for analysis of means and Levene test for equality of variances. The syndrome group presented severe mandibular hypoplasia and retrognathism (P = 0.009, P = 0.001), greater facial convexity (N'PnPog and N'SnPog, P < 0.05) in syndrome group compared with the control group (P = 0.003, P = 0.004). In conclusion, in the RCPS group, most craniofacial defects affect the lower facial third, considering the severely affected mandible.

Tratamento em paciente com fissura pré-forame completa: o que o ortodontista precisa saber? / Orthodontic treatment in complete cleft lip: what does the orthodontist must know?

Este trabalho descreve o tratamento ortodôntico corretivo de uma adolescente do sexo feminino com fissura pré-forame incisivo completa. A paciente apresentava Padrão I, má oclusão de Classe I, presença de incisivo lateral superior supranumerário na região da fissura e apinhamento anterossuperior moderado. O reparo do lábio foi realizado aos 6 meses de idade. O defeito ósseo alveolar foi restabelecido por meio da cirurgia de enxerto ósseo alveolar secundário (EOAS) de origem autógena, aos 11,5 anos de idade. Houve boa formação de osso alveolar na região da fissura permitindo movimentação ortodôntica convencional nesta área 3 meses após o EOAS. O tratamento ortodôntico durou 2,5 anos. Foi alcançada oclusão satisfatória e estável. As radiografias finais indicaram aspecto saudável do periodonto na região de canino e incisivo lateral esquerdos. A paciente com fissura pré-forame incisivo apresenta algumas particularidades, como a ruptura da continuidade do rebordo ósseo alveolar, no entanto, o tratamento corretivo convencional pode ser realizado da maneira convencional após o procedimento de enxerto ósseo alveolar. (AU)

This work describes the corrective orthodontic treatment of a female adolescent with alveolar cleft lip. The patient presented Class I skeletal pattern, Class I malocclusion, presence of a supernumerary lateral incisor at the cleft region, and moderate maxillary incisor crowding. Lip repair was executed at 6 months of age. The alveolar cleft was restored through secondary alveolar bone graft surgery (SABG) performed at 11.5 years of age using autogenous bone from the iliac crest. The successful alveolar bone filling permitted conventional orthodontic movement at the maxillary arch three months after SABG. Total orthodontic treatment time was 2.5 years. Adequate occlusion and aesthetic smile were achieved. Final radiographs showed normal aspect of periodontal bone at the left maxillary lateral incisor and canine region. The treatment of an alveolar cleft lip patient has some peculiarities, such as the disruption in continuity of the alveolar bone arch. However, the corrective orthodontic approach can be performed conventionally after the alveolar bone grafting procedure. (AU)

Ortodontia x periodontia em paciente adulto ­ relato de caso / Orthodontics x periodontics in adult patient ­ case report

Este trabalho descreve o tratamento de um indivíduo de 53 anos, xantoderma, Classe I de Angle bilateral, mordida cruzada anterior e doença periodontal avançada com perda óssea significante generalizada. O indivíduo passou por tratamento periodontal e após 6 meses foi possível iniciar a ortodontia. A mecânica foi realizada com fios leves e lançando mão de recursos que permitissem que ocorresse da forma mais atraumática possível. O tratamento foi finalizado e bem sucedido, devolvendo estética e função em dentes que haviam sido condenados por outros profissionais. O tratamento de casos semelhantes deve obrigatoriamente ser interdisciplinar e, sempre que possível, conservador. Este estudo tem o objetivo de relatar um caso clínico bastante comum em pacientes com comprometimento periodontal e discutir aspectos atuais envolvidos no êxito do tratamento desses pacientes. (AU)

This paper describes the treatment of a male, 53 year old, xantonoderma, bilateral Angle Class I, anterior crossbite and advanced periodontal disease with generalized significant bone loss. The patient underwent periodontal treatment and after 6 months, it was possible to start the orthodontics. The mechanics was performed with light wires and using resources that allowed it to occur in the most atraumatic way as possible. The treatment was completed and successful, restoring aesthetics and function in teeth that had been condemned by other professionals. The treatment of similar cases must be interdisciplinary and, whenever possible, conservative. This study aims to report a very common clinical case in patients with periodontal impairment and to discuss current aspects involved in a favorable outcome in these patients treatment. (AU)