Defectos bucodentales en personas con síndrome de Down: una prioridad en salud bucal / Oral-dental defects in people with Down's syndrome: a priority in oral health

RESUMEN El síndrome de Down con frecuencia se acompaña de defectos bucodentales que comprometen la función masticatoria y fonatoria del paciente. Las acciones de prevención y promoción de salud bucal, el diagnóstico precoz y seguimiento de estas enfermedades, contribuyen al logro del verdadero enfoque interdisciplinario que demandan estos pacientes, para lograr una plena inclusión social. Se realizó una búsqueda bibliográfica sobre el tema, con el objetivo de estructurar los referentes teóricos relacionados con los principales defectos bucodentales -congénitos y adquiridos- que afectan a la población con síndrome de Down, para lograr la prevención de estos defectos y el incremento de la calidad de vida de los pacientes. Los defectos bucodentales congénitos más frecuentes encontrados en la trisomía 21, fueron la microdoncia, la macroglosia y la erupción dental tardía. La enfermedad periodontal fue el defecto adquirido de mayor presentación, al que se asocian diversos factores de riesgo, muchos modificables. Las intervenciones tempranas en salud bucal pueden incrementar la calidad de vida de los niños y adultos que padecen este trastorno, ayudándolos a lograr un pleno desarrollo como seres humanos (AU).

ABSTRACT Down's syndrome is frequently accompanied by oral-dental defects compromising the masticatory and phonatory function of the patients. Oral health promotion and prevention actions, precocious diagnosis and follow-up of these diseases contribute to achieving the true interdisciplinary approach these patients demand to reach their full social inclusion. A bibliographic search on the theme was carried out, with the objective of structuring the theoretical referents related to the main oral-dental defects -congenital and acquired-, affecting the population with Down's syndrome to reach these defects prevention and increasing these patients' life quality. The congenital oral-dental defects more commonly found in trisomy 21 were microdontia, macroglossia, and delayed tooth eruption. Periodontal disease was more frequently found acquired defect, to which several risk factors are associated, many of them modifiable. Early interventions in oral health may improve the life quality of these children and adults, helping them to achieve a full development as human beings (AU).

Épulis congénito gigante: importancia del diagnóstico prenatal / Congenital giant cell epulis: Importance of the prenatal diagnosis

No disponible

Heterotopic Gastrointestinal Cyst of the Oral Cavity Radiology-Pathology Correlation.

Heterotopic gastrointestinal cyst of the oral cavity is a rare congenital lesion that may arise from ectopic undifferentiated endodermal cells. Imaging, particularly MRI, is useful for surgical planning. On MRI, the cysts typically demonstrate high signal on T2-weighted sequences and variable signal on T1-weighted sequences, which can resemble other conditions, such as dermoids. On histology, the appearance of these lesions can be variable, and may include stratified squamous, simple and ciliated columnar, as well as foveolar and intestinal-type epithelia, often surrounding by smooth muscle. Complete surgical excision is the treatment of choice.

Bilateral congenital mucous extravasation phenomenon: A rare case with literature review.

The purpose of this paper is to discuss a rare case of bilateral congenital mucous extravasation phenomenon on buccal mucosa near commissure of mouth. The lesions were noted at birth, subsequently enlarged to interfere with feeding. The lesion frequently ruptured, regressed, and again enlarged to interfere with normal function. The lesions were surgically removed under general anesthesia at 11 months of age and the diagnosis was confirmed by histopathological examination. Postoperative follow-up after 14 months showed no recurrence.

Pediatric Benign Soft Tissue Oral and Maxillofacial Pathology.

Despite the many types of oral pathologic lesions found in infants and children, the most commonly encountered are benign soft tissue lesions. The clinical features, diagnostic criteria, and treatment algorithms of pathologies in the age group from birth to 18 years of age are summarized based on their prevalence in each given age distribution. Treatment modalities include both medical and surgical management.

Oral Congenital Melanocytic Nevus: A Rare Case Report and Review of the Literature.

Melanocytic nevi are congenital or acquired benign proliferations of cells of melanocytic origin. Oral congenital melanocytic nevi are rare, and only a few cases have been reported in the literature. The purpose of this study is to present the clinical, histological and immunohistochemical features of an oral congenital melanocytic nevus in a 16-year-old female with an 11-year follow-up and to review the pertinent literature. The reported case is the fifth well-documented case report of oral congenital melanocytic nevus in the English literature and the first with a long period of follow-up, thereby making it an important contribution to the knowledge regarding this uncommon oral mucosa lesion.

Teeth erupted from the buccal mucosa: simple odontogenic choristoma or accessory teeth?

The eruption of developed teeth from the buccal mucosa is a rare phenomenon in the head and neck region. Such phenomena are possibly choristomas, tumorlike masses of histologically normal tissue occurring in an abnormal position. However, the accurate classification of this abnormality remains debatable. This report describes a case of a congenital, maldevelopmental, and noncystic lesion in a 4-year-old girl without other anomalies. To the authors' knowledge, this is the first case of multiple supernumerary teeth forming in the buccal and zygomatic regions.

Floor-of-mouth dermoid cysts: report of 3 variants and a suggested change in terminology.

PURPOSE: Dermoid cyst is a frequently used descriptive term, but its definition changes according to the clinician (eg, dermatologists, neurologists, gynecologists, oral and maxillofacial surgeons, and plastic surgeons). It is sometimes used synonymously with teratoma. In oral and maxillofacial surgery, it is applied to describe congenital floor-of-mouth cysts of 3 histologic types: epidermoid, dermoid, and teratoid. This terminology is confusing and has led to some ambiguity in the literature. The purpose of this report is to document 3 cases illustrating the utility of a more specific term, congenital germline fusion cyst of the floor of the mouth. PATIENTS AND METHODS: Patients who presented with floor-of-mouth swelling were evaluated by history, clinical examination, and imaging studies (magnetic resonance imaging and computed tomography). RESULTS: Three cases (1 epidermoid variant, 1 dermoid variant, and 1 teratoid variant) are documented to illustrate the new terminology. CONCLUSION: Congenital germline fusion cyst is more reflective of the embryologic origins of the lesion than dermoid cyst and is inclusive of all 3 histologic variants.

Hairy polyp of the oropharynx in association with a first branchial arch sinus.

OBJECTIVES: Hairy polyps are rare, congenital malformations of the oropharynx and nasopharynx. To date, approximately 145 cases have been reported. However, the histogenesis of these lesions remains unclear. CASE REPORT: We report the case of a 2-day-old neonate presenting with a hairy polyp attached to the left palate, who re-presented aged 16 months with a discharging first branchial arch sinus. CONCLUSION: We propose this case as supporting evidence for the theory that hairy polyps are a malformation of the first branchial arch system.

Conhecendo o HTLV e suas implicações no atendimento odontológico / Getting to know HTLV and their implications during dental care

Os vírus T Linfotrópico humano representam um grupo de retrovírus que possuem tropismo por linfócitos T e são transmitidos pelas vias parenteral, vertical e sexual. Foram identificados os tipos 1, 2, 3 e 4 sendo que o vírus T Linfotrópico humano -1 tem sido o mais associado ao desenvolvimento de doenças nos pacientes infectados. A estimativa global é de que 15 a 20 milhões de pessoas estejam infectadas pelo vírus T Linfotrópico humano -1. No Brasil o vírus é endêmico, apresentando o maior número absoluto de casos no mundo e sendo incluído na triagem hematológica realizada nos hemocentros. As principais doenças associadas ao vírus T Linfotrópico humano -1 são a leucemia/linfoma de células T do adulto e a paraparesia espástica tropical. A única manifestação bucal associada ao vírus T Linfotrópico humano é o linfoma de células T do adulto, sendo que alguns autores apontam para a possibilidade de pacientes com paraparesia espástica tropical também apresentarem a síndrome de Sjõgren relacionada à infecção pelo vírus. O fato de o vírus T Linfotrópico humano ser um vírus contagioso e com capacidade oncogênica requer a atenção do cirurgião-dentista tanto no manejo odontológico quanto no diagnóstico de possíveis doenças associadas.

The human T-lymphotropic viruses represent a group of retroviruses that possess tropism for T lymphocytes and are transmitted parenterally, vertically and sexually. Four types have been identified, 1, 2, 3 and 4. Type 1 has been most frequently associated with disease in infected individuals. It is estimated that 15 to 20 million people are affected by human T-lymphotropic virus type 1. In Brazil, the virus is endemic, presenting the greatest absolute number of cases in the world and included in blood bank screening tests. The main diseases associated with human T-lymphotropic virus type 1 are adult T-cell leukemia/lymphoma and tropical spastic paraparesis. The only oral manifestation associated with human T-lymphotropic virus is adult T-cell lymphoma. Some authors believe it is possible for some patients with tropical spastic paraparesis to also present virus-related Sjõgren?s syndrome. Since human T-lymphotropic virus is contagious and potentially oncogenic, it demands attention from the dental surgeon not only for dental management but also for diagnosing associated diseases.

Congenital eruption cyst.

An eruption cyst is an epithelial cyst of the oral mucosa. We report a case of a congenital eruption cyst successfully managed by removal of fluid from the cyst followed by close monitoring of the lesion until its remission once the teeth had erupted.

Prenatal diagnosis of oral cyst caused by foregut duplication.

Prenatal diagnosis of oral cystic lesions is rare but is reported more frequently. The diagnosis of sublingual cyst is important because of the potential for airway obstruction. A rare case of a foregut duplication cyst associated with unilateral sclerocorneal microphthalmia is reported. The differential diagnosis and the limitations of the prenatal ultrasound and the postnatal MRI are discussed.

Otolaryngologic markers for the early diagnosis of Turner syndrome.

OBJECTIVE: To identify and characterize otolaryngologic markers for the early diagnosis of Turner syndrome (TS). STUDY DESIGN: Prospective cohort survey. SETTING: Clinical Center of the National Institutes of Health (NIH). PATIENTS: Ninety-one females, 7-61 years old (average=28.7 y), enrolled in a multidisciplinary study of karyotype-phenotype correlations in TS. MAIN OUTCOME MEASURES: Age at diagnosis, X chromosome karyotype, history of chronic or recurrent otitis media (OM), sensorineural hearing loss (SNHL), palate dysmorphism, pinna deformity, pterygium colli, low posterior hairline, low-set ears, and micrognathia. RESULTS: Sixty-nine (76%) patients had a history of chronic or recurrent OM, 62 (68%) had a dysmorphic palate, 57 (63%) had SNHL, and 90 (99%) had one or more of these findings. 83 (91%; average age at diagnosis=9.4 y) had one or more external craniofacial signs: pinna abnormalities, pterygium colli, low-set ears, micrognathia or a low posterior hairline. Eight patients (average age at diagnosis=13.2 y) had no external craniofacial signs, although seven (88%) of these eight patients had a history of chronic or recurrent OM, dysmorphic palate or SNHL. The age at diagnosis was not significantly different between groups with or without external craniofacial signs (P=0.126). CONCLUSIONS: PATIENTS with mild or incompletely penetrant TS phenotypes often present with otitis media, hearing loss, or both before the diagnosis of TS is established. Palatal dysmorphism, including ogival morphology, is another otolaryngologic marker for TS. Prompt recognition of these manifestations of TS could hasten its diagnosis and appropriate medical care.

Congenital infiltrating lipomatosis.

The congenital infiltrating lipomatosis (LCI) represents a distinct, clinical and pathological aspect of fat soft-tissue neoplasia; very few cases of which have been described in literature. In 1983, Slavin and Cols described the main characteristics of LCI as:(1) non-encapsulated tumours containing mature adipocites, (2) fat infiltration in muscles and adjacent soft parts, (3) absence of malignant characteristics, (4) absence of lipoblasts, (5) presence of fibrous elements, increased number of vessels and nerves and (6) adjacent bone hypertrophy. In this article, we describe a congenital infiltrating lipomatosis of the face in a child and discuss the diagnoses and the treatment of this disease.

Divided café-au-lait macule of the mouth.

We describe a 4-year-old, otherwise healthy boy with a congenital history of a perioral and labial segmental café-au-lait macule, who was noted to have unilateral localized gingival hyperpigmentation that aligned with the café-au-lait macule. This case is highly illustrative of the embryologic timing of the genetic event locally, which leads to café-au-lait type hyperpigmentation. Because the facial features and the ectoderm overlying the facial muscles develop around the third to fourth week of gestation, the distribution of this café-au-lait macule suggests development at the same time.

Congenital oral cysts in neonates: report of two cases.

Oral cysts in the neonatal period are very uncommon and may cause morbidity and mortality if not treated expeditiously. We report 2 cases of neonatal oral cysts that were managed successfully.