Reabilitação com prótese bucomaxilofacial: revisão de literatura / Rehabilitation with maxillofacial prosthetics

A odontologia reabilitadora tem como um dos seus ramos a especialidade de Prótese Bucomaxilofacial (PBMF), que visa restaurar ou substituir estruturas perdidas na região facial e no sistema estomatognático artificialmente, podendo ser ou não removidos pelo paciente. O presente trabalho objetiva revisar a leitura a respeito da reabilitação com PBMF e a sua aplicabilidade na clínica odontológica. Os indivíduos com alguma perda de estrutura na região de cabeça e pescoço, devido a traumas físicos e/ou químicos, defeitos congênitos, doenças autoimunes, neoplasias, infecções e parasitas, são pacientes para os quais há a indicação da reposição da parte ausente. As reconstruções podem ser perdas intraorais (área da maxila, mandíbula), extraorais (oculopalpebral, ocular, nasal, facial extensa e auricular) ou conjugadas. Esse é um trabalho multidisciplinar, com especialistas de áreas abrangentes e todos os especialistas trabalham de forma conjunta. Pode-se concluir que, embora seja uma das especialidades mais nobres da odontologia, ainda é muito desconhecida por parte dos estudantes e profissionais das áreas da saúde e são próteses absolutamente fundamentais para a reabilitação e qualidade de vida dos indivíduos que tem a necessidade do uso da prótese PBMF(AU)

Rehabilitating dentistry has as one of its branches the specialty of Oral and Maxillofacial Prosthesis (PBMF), which aims to restore or replace structures lost in the facial region and in the stomatognathic system artificially, which may or may not be removed by the patient. The present study aims to review the reading about rehabilitation with PBMF and its applicability in dental clinic. Individuals with some loss of structure in the head and neck region, due to physical and/or chemical trauma, birth defects, autoimmune diseases, neoplasms, infections and parasites, are patients in whom there is an indication for replacement of the absent part. Reconstructions can be intraoral (maximal area, mandible), extraoral (oculopalpebral, ocular, nasal, extensive facial and auricular) or conjugated losses. It is a multidisciplinary work, with specialists from the comprehensive areas and that all specialists work together. It can be concluded that although it is one of the noblest specialties of dentistry, it is still very unknown to students and health professionals, and they are absolutely fundamental prostheses for the rehabilitation and quality of life of individuals who need the use the PBMFprosthesis(AU)

Evaluación psicológica y cognitiva de autismo en un paciente con síndrome de MOMO: reporte de caso y revisión de la literatura / Psychological and cognitive evaluation of autism in a patient with MOMO syndrome: a case report and literature review

MOMO es un acrónimo para los términos macrosomía, obesidad, macrocefalia y anomalías oculares. El síndrome fue descrito por primera vez en 1993, con un total de nueve pacientes publicados a la fecha. Todos los casos reportaron discapacidad intelectual y en un caso se describió a un paciente con autismo. Presentamos un nuevo caso de paciente con síndrome de MOMO que consultó por fenómenos alucinatorios. Se completó una evaluación neuropsicológica, clínica y cognitiva, en donde se demostró un cociente intelectual limítrofe y se corroboraron los criterios para trastorno del espectro autista. Ésta es la primera evaluación neurocognitiva de un paciente con MOMO, la que apoya el uso de escalas estandarizadas a fin de evaluar el autismo y otras comorbilidades psiquiátricas en pacientes con síndromes genéticos.

MOMO is an acronym for macrosomia, obesity, macrocephaly and ocular abnormalities. The syndrome was first described in 1993, with a total of nine patients published thus far. All the cases presented intellectual disability and in one case autism was described. We present a new case of a patient with MOMO syndrome, who consulted for hallucinatory phenomena. He completed a neuropsychological, clinical and cognitive evaluation, showing a borderline intelligence quotient and fulfilled the criteria for autism spectrum disorder. This is the first neurocognitive evaluation of a patient with MOMO, supporting the use of standardized scales in order to assess the autism and other psychiatric comorbidities in patients with genetics syndromes.

Quistes epidermoides de cabeza y cuello / Epidermoid cysts of the head and neck

Los quistes epidermoides localizados en cabeza y cuello son poco comunes y pueden ser difíciles de diagnosticar. Se describen los casos de cuatro pacientes con quistes epidermoides de cabeza y cuello, dos con localización en la región sublingual y extensión suprahioidea, otro localizado en la pared orofaríngea posterolateral y otro en la región submaxilar y submentoniana. Fueron tratados con éxito mediante abordajes transorales y transcervical, respectivamente. Se realizó una revisión de la bibliografía y se describieron las características anatómicas, clínicas e histológicas y el tratamiento de estas infrecuentes lesiones. (AU)

Epidermoid cysts of the head and neck are rare and can be difficult to diagnose. Two cases of patients with epidermoid cysts of the floor of the mouth with suprahyoid extension, other located at posterolateral oropharynx wall andother located at the submandibular and submental space with extention to midline are described. They were successfully treated by a transoral and transcervical approach respectively. A review of the literature was performed, and the anatomical, clinical and histological aspects and treatment of these uncommon tumors were reported. (AU)

Tremor de cabeça idiopático em cães - relato de dois casos / Idiopathic head tremor in dogs - two case reports

Tremor de cabeça idiopático é uma forma de discinesia paroxística reconhecida como hereditária e associada a determinadas raças, sendo relatado, sobretudo, em Buldogue Inglês, Dobermann Pinscher e Boxer. Conhecido também como head bobbing ou tremor de cabeça episódico, os cães acometidos apresentam crises caracterizadas por tremores limitados à região de cabeça, de direção horizontal, vertical ou ambos, sendo geralmente relacionados a um evento desencadeante. Foram atendidos, no Hospital Veterinário da UFMS, dois caninos da raça Buldogue Inglês (um macho de um ano e uma fêmea de sete meses) com histórico de tremores episódicos restritos à região de cabeça. Exames físicos e neurológicos completos foram realizados, não sendo constatada qualquer alteração. Com base na raça, no histórico, na ausência de outros sinais clínicos e na exclusão de outras causas de tremores, foi dado o diagnóstico de head bobbing. Tal enfermidade caracteriza-se por tremores restritos à região de cabeça, associados a uma condição genética benigna. Não é considerada uma forma de convulsão, uma vez que o animal permanece alerta e responsivo aos estímulos ambientais, os tremores podem ser interrompidos por distrações ou qualquer interação com o ambiente, não sendo responsivos à terapia com anticonvulsivantes. De acordo com a revisão de literatura realizada, estes são os primeiros casos diagnosticados e relatados no Brasil.(AU)

Idiopathic head tremor is a form of paroxysmal dyskinesia recognized as hereditary or associated with certain races, being reported in English bulldogs, doberman pinschers, and boxers. Also known as head bobbing or episodic head tremor, the affected dogs present with seizures characterized by tremors limited to head region, horizontal direction, vertical or both and are usually related to a triggering event. Two dogs of the English bulldog breed (a male of 1 year and a female of 7 months) with a history of episodic tremors restricted to the head region were seen at the UFMS Veterinary Hospital. Complete physical and neurological examinations were performed, and no alterations were found. Based on race, history, absence of other clinical signs and exclusion of other causes of tremors, the diagnosis of head bobbing was performed. Such a disease is characterized by tremors restricted to the head region, associated with a benign genetic condition. It is not considered a form of seizure, since the animal remains alert and responsive to environmental stimuli, the tremors can be interrupted by distractions or any interaction with the environment, being not responsive to anticonvulsant therapy. According to the literature review, these are the first cases diagnosed and reported in Brazil.(AU)

Aspectos fonoaudiológicos em um caso de artrogripose de cabeça e pescoço pediátrico / Speech-language pathology aspects in a pediatric case of head and neck arthrogryposis

RESUMO A artrogripose é uma síndrome múltipla congênita rara que se caracteriza por uma série de malformações congênitas e enrijecimento e contrações articulares e não possui caráter progressivo. Trata-se de um estudo de caso clínico, cujo objetivo é descrever a manifestação funcional relacionada à fonoaudiologia através do processo de avaliação em um caso de artrogripose em pediatria. Foi realizada uma análise do prontuário clínico de um paciente desde o nascimento, bem como uma avaliação clínica completa de investigação de disfagia pediátrica, na qual foi estabelecido o diagnóstico de disfagia orofaríngea de grau grave, apontada por alterações no exame funcional e estrutural. Associa-se ao quadro, uma perda auditiva.

ABSTRACT Arthrogryposis is a rare, multiple, congenital syndrome of non-progressive nature characterized by a series of genetic malformations, as well as stiffness and joint contractures. This is a clinical case study whose objective is to describe speech-language pathology disorders through the evaluation process in a case of arthrogryposis in Pediatrics. The medical records of a patient were analyzed from birth. A complete clinical evaluation of pediatric dysphagia was performed, establishing a diagnosis of severe oropharyngeal dysphagia evidenced by functional and structural impairments. Hearing loss was detected in association with this condition.

Presentación de un caso de siameses cefalópagos / A case report of cephalopagus siamese

Se denominan siameses a aquellos gemelos que comparten estructuras anatómicas en grado variable. La incidencia varía entre 1:50 000 a 1:100 000 embarazos, 1:4 de los casos fallecen in utero y 40-60 por ciento mueren poco después del parto.Los hallazgos en autopsias médico-científicas de gemelos unidos cefalópagos, que son aquellos que presentan fusión cefálica con dos hemicaras, son el cuarto tipo de siameses en ocurrencia y su incidencia está estimada en 1 por cada 3'000.000 de embarazos o 1/58 siameses. El objetivo es presentar los hallazgos de una autopsia médico-científica de gemelos unidos cefalópagos. Se trata de gemelos cefalópagos, con terminación de embarazo por incompatibilidad con la vida extrauterina. Presentan cordón umbilical único de cinco vasos, tenían cráneos fusionados a nivel frontofacial con dos caras en lados opuestos de la cabeza (Janiceps), con fusión ventro-ventral de los ejes somáticos hasta debajo del ombligo sobre la pelvis, con sistemas respiratorios, cardiaco y genitourinario independientes, el sistema digestivo superior esta fusionado hasta el intestino medio, con duplicación a partir de la tercera porción del duodeno. Los gemelos siameses cefalópagos no son los más infrecuentes, pero es difícil encontrar series de casos y revisiones sistemáticas de sus características y patogénesis en Latinoamérica. Son escasas las estadísticas sobre siameses y no se ha descrito previamente ningún caso como éste. Se hace necesaria la realización de estudios de autopsias para mejorar la descripción y caracterización de estos casos y poder determinar si existen otras condiciones asociadas además de las teorías de unión embriológica(AU)

It is denominated siamese to those twins that share anatomical structures to a variable degree. The incidence varies between 1/50.000 and 1/100.000 pregnancies, 1:4 of the cases die in the uterus and 40-60 percent die shortly after birth. Findings in a medical-scientific autopsies of conjoined cephalopagus twins, those that presenting cephalic fusion with two hemi faces, are the fourth type of Siamese in occurrence and their incidence is estimated in 1 every 3´000.000 pregnancies or 1/58 siamese. It is about cephalopagus twins, with termination of pregnancy due to incompatibility with extrauterine life. They presented a five vessels common umbilical cord, fused craniums at frontofacial level with two faces in opposed sides of the head (Janiceps), ventro-ventral fusion of the somatic axis until below the umbilicus and above the pelvis, independent respiratory, cardiac and genitourinary systems. The superior digestive system was fused until the medium intestine, with duplication parting from the third portion of the duodenum. Conjoined cephalopagus twins are not the most frequent but it is hard to find a series of cases and systemic revisions of their characteristics and pathogenesis in Latin America. Statistics on Siamese twins are scarce and it has not been previously described any case like this one. Autopsy studies becomes necessary to improve the description and characterization of these cases and to determine the existence of any other associated conditions different to those of the embryologic union theory(AU)

[Investigating the prevalence of head and upper extremity deformities in students with special needs]

Objective: Musculoskeletal disorders are the most common childhood problems. The aim of the present study was to investigate the prevalence of head and upper extremity deformities in children with special needs

Materials and Methods: The present research is a descriptive cross-sectional study with stratified sampling method. Ten percent of students with mental retardation, hearing impairment and sight impairment in the academic year of 2012-13 were selected for this study. There were 1194 persons in Tehran, 1526 people in the cities of the Tehran Province and 559 students in Alborz Province. In total, 3279 people participated in the study. Demographic questionnaire and a plummet were used for data collection. Data was reported using descriptive statistics

Results: The results of the study revealed that "dropped shoulder" in girls of Alborz province with visual impairment with 100% had the highest rate and this abnormality in boys with visual impairment in the Tehran had the least prevalence. In the deformities of the head, lateral head bending with 76.6% in girls with visual impairment in Tehran, and forward head posture with 83.3% in boys with visual impairment in cities of Tehran had the highest incidence in comparison with others

Conclusion: The prevalence of upper extremity deformities is high in students with special needs including mental retardation, hearing impairment and sight impairment. It is necessary to diagnose these deformities in time

Attention deficit hyperactivity disorder and intellectual giftedness: a study of symptom frequency and minor physical anomalies

Objective: To evaluate the presence of symptoms of attention deficit and hyperactivity disorder (ADHD) in intellectually gifted adults and children. Methods: Two cross-sectional studies were performed in children and adults whose intelligence quotient (IQ) had been previously evaluated using Raven’s Progressive Matrices (RPM) test. Seventy-seven adults displaying IQ scores above the 98th percentile were assessed using the Adult Self-Report Scale (ASRS-18) for signs of ADHD and a modified Waldrop scale for minor physical anomalies (MPAs). Thirty-nine children (grades 1-5) exhibiting IQ scores above the 99th percentile, as well as an equally matched control group, were assessed for ADHD by teachers using the Swanson, Nolan and Pelham IV Rating Scale (SNAP-IV) as used in the NIMH Collaborative Multisite Multimodal Treatment Study of Children with Attention-Deficit/Hyperactivity Disorder (MTA-SNAP-IV). Results: In gifted adults, the frequency of ADHD-positive cases was 37.8%, and the total MPA score was significantly associated with ADHD (p < 0.001). In children, the ADHD-positive case frequency was 15.38% in the gifted group and 7.69% in the control group (odds ratio [OR] = 2.18, p = 0.288). Conclusions: The high frequency of ADHD symptoms observed, both in gifted adults and in gifted (and non-gifted) children, further supports the validity of this diagnosis in this population. Furthermore, the significant association between MPAs and ADHD suggests that a neurodevelopmental condition underlies these symptoms.

Uso do retalho cérvico-submandibular transverso nas reparações de defeitos do terço médio da face / Use of a transverse submandibular cervical flap in the repair of defects of the middle third of the face

INTRODUÇÃO: As reparações de defeitos extensos do terço médio da face, ao necessitarem de grandes quantidades de pele e subcutâneo, tornam-se complexas. Buscando reduzir morbidade e associar bom resultado estético-funcional, Ariyan & McGrath e, posteriormente, Behan et al. propuseram reconstruções a partir de retalhos transversos cervicais e cérvico-submandibulares, respectivamente. Tais retalhos dispõem de boas quantidades de tecidos e baixa morbidade da área doadora. Os autores propõem e descrevem uma variante do retalho de Behan para reconstruções de grandes defeitos no terço médio de face. MÉTODOS: Foi realizado um estudo retrospectivo de 8 casos de reconstrução do terço médio da face pelo retalho cérvico-submandibular transverso, no período de junho de 2011 a dezembro de 2013. Os parâmetros analisados foram: resultados possíveis e complicações precoces e tardias. RESULTADOS: Foram operados 8 pacientes, com média de idade de 73,5 anos. Todos os pacientes apresentaram resultados estético-funcionais satisfatórios. Dentre as complicações precoces, 3 pacientes apresentaram congestão vascular na primeira semana com resolução espontânea e 1 apresentou fistula salivar consequente à ressecção tumoral. Em relação às complicações tardias, a retração cicatricial foi a complicação observada, manifestada por ectrópio (2 pacientes) e retração labial (1 paciente). CONCLUSÃO: O retalho cérvico-submandibular transverso randomizado e com pedículos axiais é mais uma opção para reconstruções de defeitos do terço médio da face.

INTRODUCTION: The need for large amounts of skin and subcutaneous tissue makes it complex to repair extensive defects of the middle third of the face. Aiming to reduce morbidity and attain good aesthetic-functional outcomes, Ariyan and McGrath and, subsequently, Behan et al. proposed reconstruction techniques that use transverse and submandibular cervical flap, respectively. Such flaps have fair amounts of tissue and are associated with low morbidity in the donor area. The present authors propose and describe a variant of the Behan flap for use in the reconstruction of large defects of the middle third of the face. METHODS: We conducted a retrospective study of 8 cases of reconstruction with transverse submandibular cervical flaps for defects of the middle third of the face, conducted between June 2011 and December 2013. The following parameters were analyzed: possible results, and early and late complications. RESULTS: Eight patients with a mean age of 73.5 years were included. All of the patients presented aesthetic-functional satisfactory results. Among the early complications, vascular congestion occurred in 3 patients in the first week with spontaneous resolution, and salivary fistula occurred after tumor resection in 1 patient. With regard to late complications, scar retraction was observed, manifested by either by ectropion (2 patients) or labial retraction (1 patient). CONCLUSION: Randomized transverse submandibular cervical flaps with axial pedicles may be considered as another option for reconstruction of defects of the middle third of the face.

Maternal antecedents of infants with abnormal head sizes in Southwest Nigeria: a community-based study

To identify the socio-demographic antecedents and pregnancy-related history of infants with abnormal head sizes in a developing country. An observational study of mother-infant pairs attending routine immunization clinics in an inner-city community in Lagos, Nigeria. Age and gender-specific head circumference was determined with the current Child Growth Standards of the World Health Organization [WHO]. Factors independently associated with any abnormal head size [z-score < - 2SD or > 2SD], based on the adjusted odds ratio [OR] and 95% confidence interval [CI], were explored with multiple logistic regression analyses. Of the 5731 mothers studied, 730 [12.7%] had an offspring with an abnormal head size. In the final regression model, teenage mothers [OR:1.86; CI:1.26 - 2.75], mothers with primary or no education [OR:1.65; P = 0.007], multiple pregnancies [OR:3.88; CI:2.53 - 5.95], and delivery in either private hospitals [OR:1.54; CI:1.22 - 1.95] or residential homes [OR:1.50; CI:1.05 - 2.14], compared to government hospitals, were significantly more likely to have offsprings with abnormal head sizes. Community-oriented public health education, targeting prospective mothers with multiple pregnancies, teenage girls, and women with little or no formal education on the potential risk of delivery outside public hospitals, may curtail the burden of abnormal head size of their offspring and reduce the pressure on the already overstretched rehabilitation services in resource-poor countries

Incidence of head and neck birth defects in Iran: a cross-sectional study from southwest region

The aims of the study were to assess the prevalence of birth defects [BDs] in Ahwaz, Iran amongst the live births born between 1 Oct 2006 and 31 Oct 2007. There were a total of 4176 live births in Ahwaz during this study period. The interview included the Father and mother's date of birth, Number of gravidity, paternal smoking, maternal delivery age and the occupation of the mother and father. Out of 4176 subjects, 1.43 per 1000 live births presented with multiple anomalies. The overall occurrence of malformation among working mothers were 1.67 and 7.42 for working fathers. Parents in the occupational group 'Commerce' had the highest rates [3.83 per 1000 live births]. The frequencies of BDs in smokers were 3.5 [per 1000 live births]. The gravidity profiles showed that all three types were very close to each other. The occurrence rate of BDs increased in the maternal delivery age of 18-35 years [5.74 per 1000 live birth]. The prevalence of BDs in Southwest of Iran is comparable to those in other countries. Some of the birth defects are not diagnosed at birth and may occur later in the life

Anesthetic characteristics and airway evaluation of patients with weill-marchesani syndrome

Weill-Marchesani syndrome [WMS] is a genetic connective tissue disorder associated with fibrous tissue hyperplasia. Weill-Marchesani syndrome is characterized by short stature, broad head and other facial abnormalities such as hypoplastic maxilla and distinctive ocular abnormalities. Joint stiffness is one of the features of this syndrome. We report 5 cases with classical features of WMS who were subjected to different ophthalmic procedures. To the best of our knowledge, this is the first series on the anesthetic management of this rare syndrome. We observe that patients with WMS can present for cataract, glaucoma as well as retinal surgery. Special consideration should be given to difficult intubation, cardiac abnormalities and patient positioning

Partial duplication of head--a rare congenital anomaly.

Duplication of notochord results in rare congenital anomalies like double headed monsters, with or without trunk/limb duplication, depending upon the extent of notochordal abnormality. Here we describe the morphological abnormalities in a case of partial duplication of cranial structures with fusion of the two. Autopsy findings suggest that the bifurcation of the neural tube took place around 4th to 6th week of gestation. There are only few reports in English literature describing the autopsy findings of such an anomaly, which is termed as Diprosopus triophthalmus in the modern literature.

Isolated cervical osteochondroma manifesting as spasmodic torricollis: a case report

We present a 30-year-old man with cervical spine osteochondroma who manifested as spasmodic torticollis. The patient was treated medically for three years without any improvement. Eventually patient's medical records was reviewed and a structural disparity was detected in plain radiography. Further study confirmed a bone tumor in the left pedicle of third cervical vertebra. Upon surgical excision of the mass, the C3 spinal root was released and main bulk of the tumor was resected. Pathologic findings was in favor of osteochondroma and clinical symptoms was fully abated after surgery. Cervical spine tumors such as osteochondroma is an uncommon cause of spasmodic torticollis, however, always should be kept in mind as one of the etiologies