Histiocitosis de células de Langerhans con afectación de la órbita en un lactante: reporte de un caso / Langerhans cell histiocytosis with orbital involvement in an infant: Case report

La histiocitosis de células de Langerhans es una enfermedad poco frecuente, caracterizada por la proliferación clonal de células dendríticas mieloides CD1a positivas, asociada a un componente inflamatorio significativo. El compromiso óseo es común; en niños, las lesiones líticas más frecuentes son en la cúpula craneal y son raras las lesiones en la órbita. Se presenta a un lactante de 18 meses que consultó por edema periorbitario y proptosis del ojo derecho de dos meses de evolución. Al momento del examen físico, no se encontraron otras alteraciones. Se realizó una tomografía y una resonancia magnética, que mostraron una masa tumoral en el seno maxilar de crecimiento expansivo y erosión del techo de la órbita. La biopsia confirmó la proliferación CD1a positiva; recibió tratamiento con prednisona y vinblastina, con evolución favorable. Un tratamiento precoz puede evitar secuelas significativas en los pacientes cuando son sensibles al tratamiento. Es una entidad poco frecuente que requiere un alto índice de sospecha y un manejo multidisciplinario.

The Langerhans cell histiocytosis is a rare disease characterized by the clonal proliferation of CD1a + myeloid dendritic cells associated with a significant inflammatory component. The localized form of the disease is called eosinophilic granuloma. Bone involvement is common; in children, lytic lesions are most frequently found in the cranial dome being rare in the orbit. We present an 18-month-old infant who consulted due to periorbital edema and proptosis of the right eye, with two months of evolution. The computed tomography and the magnetic resonance imaging showed a maxillary sinus tumor mass of expansive growth and erosion of the roof of the orbit. The biopsy confirmed CD1a+ proliferation; it was treated with prednisone and vinblastine with favorable evolution. It is a rare entity that requires a high index of suspicion and multidisciplinary management. Early diagnosis and treatment leads to a favorable prognosis for the patient.

Keratocystic odontogenic tumor with impacted maxillary third molar involving the right maxillary antrum:An unusual case report.

The odontogenic keratocyst (OKC), first described by Phillipsen in 1956, has metamorphosized as a keratocystic odontogenic tumor (KCOT) as reported in WHO classification of head and neck tumors in 2005. KCOT is a benign intraosseous neoplasm of the jaw and its occurrence in maxilla is unusual and its appearance in maxillary antrum along with maxillary impacted third molar is very uncommon. This article reports a case of KCOT associated with impacted maxillary third molar in right maxillary antrum and describes its rare site of occurrence.

Electromyographic activity of masticatory muscles in women with osteoporosis

The purpose of this study was to analyze the electromyographic (EMG) activity and the maximal molar bite force in women diagnosed with osteoporosis in the maxillary and mandibular regions, considering the habits and conditions that lead to development of generalized skeletal bone loss, including on face bones, can disturb the functional harmony of the stomatognathic system. Twenty-seven women with mandibular and maxillary osteoporosis and 27 healthy controls volunteered to participate in the study. A 5-channel electromyographer was used. Muscle activity was evaluated by means of EMG recordings of the masticatory musculature (masseter and temporalis muscles, bilaterally) during the following clinical conditions: rest (5 s); right and left lateral excursions (5 s); protrusion (5 s); maximal dental clenching on Parafilm™ (4 s) and maximal voluntary contraction (4 s). This latter clinical condition was used as the normalization factor of the sample data. It was observed that individuals with osteoporosis presented greater EMG activity when maintaining mandible posture conditions and less activity during dental clenching and when obtaining maximal molar bite force. It may be concluded that facial osteoporosis can interfere on the patterns of masticatory muscle activation and maximal bite force of the stomatognathic system.

Este estudo teve como objetivo analisar a atividade eletromiográfica e a força de mordida molar máxima de indivíduos diagnosticados com osteoporose na região maxilar e mandibular, visto que os hábitos e condições que provocam o desenvolvimento de uma perda óssea generalizada no esqueleto, inclusive no esqueleto facial, podem causar distúrbios na harmonia funcional do sistema mastigatório. Vinte e sete indivíduos portadores de osteoporose na mandíbula e maxila e 27 voluntários sem a doença participaram deste estudo. Um eletromiógrafo de 5 canais foi utilizado. A avaliação da atividade muscular foi realizada por meio de registros eletromiográficos da musculatura mastigatória (músculos masseter e temporal, bilateralmente) durante as seguintes condições clínicas: Repouso (5 s); Lateralidades direita e esquerda (5 s); Protrusão (5 s); Apertamento dental máximo com parafilme (4 s) e a contração voluntária máxima (4 s), sendo que esta condição clínica foi utilizada como fator de normalização dos dados da amostra. Verificou-se que os indivíduos com osteoporose apresentaram maior atividade eletromiográfica durante a manutenção das condições posturais da mandíbula e menor atividade durante o apertamento dental e na obtenção da força de mordida molar máxima. Conclui-se que a osteoporose na região dos ossos da face pode interferir nos padrões de ativação da musculatura mastigatória e na força de mordida máxima do sistema estomatognático.

Dentigerous cyst associated with an ectopic third molar in the maxillary sinus: a rare entity.

Ectopic eruption of teeth into a region other than the oral cavity is rare although there have been reports of teeth in the nasal septum, mandibular condyle, coronoid process, palate, chin and maxillary sinus. Occasionally, a tooth may erupt in the maxillary sinus and present with local sinonasal symptoms attributed to chronic sinusitis. We present a case of an ectopic maxillary third molar tooth that caused chronic purulent sinusitis in relation to the right maxillary sinus.

Dentigerous cyst associated with multiple mesiodens: a case report.

Dentigerous cyst is a developmental odontogenic cyst, which apparently develops by accumulation of fluid between reduced enamel epithelium and the tooth crown of an unerupted tooth. When observed with erupted and complete dentition the diagnosis is a surprise; as about 95% of dentigerous cysts involve the permanent dentition and only 5% are associated with supernumerary teeth. The usual age of clinical presentation of dentigerous cyst due to supernumerary tooth is during the first four decades. Mesiodens is a supernumerary tooth situated between the maxillary central incisors. More frequently the mesiodens occurs unilaterally, but it may also be bilateral, while three or more supernumerary teeth in the median region of the palate are more rarely found. We report a rare case of dentigerous cyst in association with multiple mesiodens in a 14-year-old female patient.

Unusual presentation of inverted impacted premolars as a result of dentigerous cyst: a case report.

A case of dentigerous cyst associated with upper right lateral incisor that had resulted in displacement of the canine, inverted impaction of both the premolars and dilaceration of apical 1/3 of central incisor is reported here.

Oro-nasal fistula of infective origin and alveolar necrosis in a 13 year old boy.

An unusual case of oronasal fistulla of infective origin in a patient with a previous history of aplastic anemia and cancrum oris is discussed here in this report. As the general condition of the patient was not favouring the surgical closure, an obturator was fabricated and inserted to relieve the problems of regurgitation of food and fluids into the nasal cavity.

Inverted central incisor--a case report.

An unusual case of inverted central incisor in a nine year old girl is reported here. The case was diagnosed as an infected dentigerous cyst. The unerupted central incisor was placed inverted almost near the ethmoid bone. The cyst was enucleated successfully.