Sella turcica 3t magnetic resonance imaging in the diagnosis of cushing's disease in children: two case reports / Ressonância magnética 3t de sela túrcica no diagnóstico de doença de cushing em crianças: relato de dois casos

ABSTRACT Objective: To present two clinical cases of pediatric Cushing disease caused by adrenocorticotropic hormone secreting pituitary adenomas, which were diagnosed by magnetic resonance imaging using 3 Tesla technology. Case description: Two cases of Cushing disease in 9-year-old children are reported. Both children presented pituitary adenomas that were smaller than 5 mm at their largest diameter, and which were not seen by standard 1.5 Tesla resonance. One of the patients was submitted to bilateral and simultaneous catheterization of the inferior petrosal sinus, but the result was undetermined. In both cases, the pituitary adenoma was detected by 3 Tesla magnetic resonance imaging. Both patients underwent transsphenoidal surgery and were cured. Comments: Cushing disease presents high morbidity. Therefore, early diagnosis and prompt treatment are essential. It is usually caused by adenomas that are smaller than 5 mm in diameter. Surgery is the first line of treatment, and effective methods of locating the adenoma are necessary for greater therapeutic success. This report suggests that the 3 Tesla magnetic resonance imaging is more sensitive, and thus able to detect pituitary microadenomas (largest diameter <10 mm). This exam may be indicated as a low-morbidity diagnostic tool for finding pituitary microadenomas in Cushing disease that are not visualized by 1.5 Tesla magnetic resonance imaging.

RESUMO Objetivo: Apresentar dois casos clínicos de Doença de Cushing infantil decorrentes de adenoma hipofisário secretor de hormônio adrenocorticotrófico, cujo diagnóstico foi realizado por meio da ressonância magnética pela tecnologia 3 Tesla. Descrição do caso: São relatados dois casos de Doença de Cushing em crianças aos nove anos. Ambas apresentavam adenomas menores que 5 mm em seu maior diâmetro que não foram visualizados por meio de ressonância magnética de sela turca utilizando tecnologia 1,5 Tesla. Uma das pacientes foi submetida ao cateterismo bilateral e simultâneo do seio petroso inferior, porém com resultado indeterminado. Nas duas, o adenoma hipofisário foi visualizado mediante ressonância magnética utilizando tecnologia 3 Tesla. Ambas foram submetidas à cirurgia transesfenoidal e evoluíram para cura. Comentários: A Doença de Cushing apresenta alta morbidade, necessitando de diagnóstico e tratamento precoces, e geralmente é causada por adenomas com diâmetro inferior a 5 mm. O tratamento é cirúrgico, sendo preciso utilizar métodos eficazes de localização do adenoma para maior sucesso terapêutico. Esses relatos sugerem que a ressonância magnética 3 Tesla tem mais sensibilidade na detecção de microadenomas hipofisários (maior diâmetro <10 mm), podendo-se indicar esse exame como uma ferramenta diagnóstica de baixa morbidade na localização de microadenomas hipofisários na Doença de Cushing não visualizados pela ressonância magnética 1,5 Tesla.

Variaciones anatómicas de la silla turca en radiografías laterales de cráneo. Hospital "Faustino Pérez", 2017-2018 / Anatomic variants of sella turcica in lateral crania radiographies. Hospital "Faustino Pérez¨, 2017-2018

RESUMEN Introducción: diversos autores reflejan que la morfología de la silla turca constituye un factor predisponente para algunas enfermedades. Por ejemplo, se considera que existe correlación entre la morfología de esta estructura anatómica y varias patologías; como el síndrome de la silla turca vacía, síndrome de Williams, paladar hendido, entre otras. Objetivo: describir las variaciones anatómicas de la silla turca que se observan en las radiografías laterales de cráneo del Hospital Universitario "Faustino Pérez" de la ciudad de Matanzas, en el período de enero del 2017 a enero del 2018. Materiales y métodos: el universo fue 140 radiografías laterales de cráneo, de estas 85 pertenecieron al sexo femenino y 55 al masculino. Las variables estudiadas fueron edad, sexo y variaciones anatómicas de la silla turca. Se emplearon métodos teóricos y empíricos. Resultados: se observó un predominio de la variación anatómica de la silla turca en forma de U, en ambos sexos. Seguido de la forma de J, predominando la variación en forma de U en el grupo etario de 41 a 50 años y la forma de J en los pacientes mayores de 60 años. Conclusiones: es imprescindible el conocimiento de la anatomía normal de la silla turca y de sus variaciones anatómicas, tanto para las especialidades quirúrgicas como para las no quirúrgicas. Un análisis exhaustivo de la morfología de esta estructura es necesario para establecer parámetros que excluyan determinadas patologías.

ABSTRACT Introduction: several authors declare that sella turcica morphology is a predisposing factor to several diseases. For example, it is considered that there is a correlation between the morphology of this anatomical structure and several pathologies like empty sella turcica syndrome, Williams syndrome, cleft palate and others. Objective: to describe the anatomical variants of sella turcica observed in side cranial radiographies of the University Hospital ¨Faustino Perez¨ of Matanzas, in the period January 2017-January 2018. Materials and methods: the universe was 140 side cranial radiography: 85 belonged to female patients and 55 to male patients. The studied variables were age, sex and sella turcica anatomical variables. Theoretic and empirical methods were used. Results: it was observed a predominance of the U-shaped sella turcica anatomical variant in both sexes, followed by the J-shaped one. The U-shaped form predominated in the 41-50-years-old age-group and the J-shaped form in patients elder than 60 years. Conclusions: it is essential to know sella turcica normal anatomy and its anatomical variables, both for the surgical specialties and for the non-surgical ones. It is necessary the exhaustive analysis of this structure to establish parameters excluding several pathologies.

Acromegalia y silla turca vacía: una asociación infrecuente: presentación de un caso / Acromegaly and empty sella syndrome: an infrequent association: presentation of a case

La acromegalia, originada por un exceso de producción de Hormona de crecimiento (Gh), se caracteriza por crecimiento somático exagerado, alto riesgo cardio-metabólico, así como reducción de la expectativa de vida. Tiene una incidencia de 3-4 casos por millón de habitantes. El diagnóstico se retrasa hasta 10 años aumentando la morbi-mortalidad. Las alternativas terapéuticas incluyen medicamentos y cirugía, que van encaminados a reducir los efectos de masa tumoral, normalizar los parámetros bioquímicos y resolver las manifestaciones clínicas. En casos muy infrecuentes, el tumor hipofisario que la origina se asocia a silla turca vacía.

Acromegaly, caused by an excess production of growth hormone (Gh), it is characterized by exaggerated somatic growth, high cardio-metabolic risk, as well as reduction of life expectancy. It has an incidence of 3-4 cases per million population. The diagnosis is delayed up to 10 years increasing morbidity and mortality. The therapeutic alternatives include medications and surgery, which are aimed at reduce the effects of tumor mass, normalize biochemical parameters and resolve clinical manifestations. In very infrequent cases, the pituitary tumor that originates it is associated with empty sella syndrome. Key words: Acromegaly, Empty sella syndrome, Pituitary tumor.

Tumores hipofisarios, casuística de Anatomía Patológica / Pituitary tumors: anatomopathological reports

This study examines a description of pituitary tumors considering an anatomopathological casuistic. The study of the tumors of the Central Nervous System (CNS) include the pituitary gland, located in the sella turcica. The pathology of the sellar region is represented by the adenomas, tumors of slow development with or without endocrine secretion, that usually involve the population of young adults. The aim of this report, was to describe the casuistic of the " J. Fernandez Hospital" between the years 2000 through 2017. A retrospective review was performed and 234 samples of the sellar region were processed. Mean age was 42 years with a range of 17 to 77 years. Sex distribution was 57% women and 43 % men. Of these, 77% of the cases resulted adenomas. The conclusions obtained in the study are detailed.

Prolactinomas may have unusual presentations resulting from massive extrasellar tumor extension / Prolactinomas podem ter apresentações incomuns resultantes de maciça extensão tumoral extrasselar

ABSTRACT The purpose of this case series is to report eight patients with giant prolactinomas emphasizing presentations and a treatment complication. The study group included six men and two women. The median age was 29 years (18–54 years); median serum prolactin level was 4,562 ng/ml (1,543–18,690 ng/ml); three patients (37.5%) had panhypopituitarism; median tumor diameter was 50 mm (41–60 mm). Five patients (62.5%) had visual field defects and three had improvement during treatment; six patients (75%) reached prolactin normalization, with a median time of 10.5 months (7–84 months) and median dose of 2.0 mg/week (1.0 to 3.0 mg/week). One patient presented as a true incidentaloma. One patient presented a cerebrospinal fluid leakage during medical treatment and refused surgery, however this resolved with conservative measures. This case series illustrate a rare subtype of macroprolactinomas, the importance of considering unusual presentations at the diagnosis, the effectiveness of pharmacological treatment and its possible complications.

RESUMO O objetivo desta série de casos é relatar oito pacientes com prolactinomas gigantes enfatizando as formas de apresentação e uma complicação do tratamento. O estudo incluiu seis homens e duas mulheres. A mediana de idade foi 29 anos (18–54); e dos níveis de prolactina foi 4.562 ng/ml (1.543–18.690); três pacientes (37,5%) apresentaram pan-hipopituitarismo; a mediana do máximo diâmetro tumoral foi 50 mm (41–60 mm). Cinco pacientes (62,5%) apresentaram alterações no campo visual e três tiveram melhora durante o tratamento; seis pacientes (75%) alcançaram normalização da prolactina em 10,5 meses (7–84) com dose mediana de cabergolina de 2,0 mg / semana (1,0 a 3,0). Um paciente se apresentou como um verdadeiro incidentaloma. Um paciente apresentou uma fistula liquórica durante o tratamento medicamentoso e recusou correção cirúrgica. No entanto a fistula foi resolvida com medidas conservadoras. Esta série de casos ilustra um subtipo raro de macroprolactinomas, a importância de considerar apresentações incomuns no diagnóstico, a eficácia do tratamento farmacológico e suas possíveis complicações.

Frequency of ponticulus posticus, sella turcica bridge and clinoid enlargement in cleft lip and palate peruvian patients: a comparative study with non-cleft patients / Frecuencia de ponticulus posticus, puente selar y alargamiento clinoideo en pacientes peruanos con labio y paladar fisurado: un estudio comparativo con pacientes no-fisurados

The aims of this study were to determine the frequency of the Ponticulus posticus (PP) in the C1 vertebra and the Sella Turcica Bridge (STB) and Clinoid enlargement (CLEN) variants in two samples of Peruvian cleft lip and palate patients (CLP), determine if there are significant differences between sexes and compare the results with two samples of non-cleft controls. The digital images of the lateral cephalometric radiography of 163 and 150 CLP patients were utilized to determine the frequency of PP and, STB-CLEN respectively. The controls were composed of 1056 and 417 radiography of non-cleft patients. The chi-square statistic was utilized to determine if there were significant differences between genres for the CLP patients, and between the CLP and control samples. The confidence level was set at p<0.05. The frequency of PP (partial and complete) in CLP patients was 11.04% (18 cases) and 6.13% (10 cases) respectively; both of them were more frequent in males and there were no significant differences between sexes and between the CLP and control groups (p>0.05). The frequency of STB and CLEN in CLP patients was 6% (9 cases) and 8.7% (13 cases) respectively; both of them were more frequent in males and there were no significant differences between sexes and between the CLP and control samples for the STB (p>0.05). The frequency of CLEN was significantly lower (p<0.05) in the cleft group and in the female cleft group when compared to the control group. The frequencies of the Ponticulus Posticus, Sella Turcica Bridge and Clinoid Enlargement were similar to the control group and should not be considered as Cleft Lip and Palate associated anomalies; the frequency of the Clinoid Enlargement was significantly lower in cleft patients and this could be confirmed through studies centered on the Clinoid apophysis.

Los objetivos del estudio fueron determinar la frecuencia del Ponticulus Posticus (PP) y Puente selar - Alargamiento clinoideo (PS­AC) en dos muestras de pacientes Peruanos con fisura labiopalatina, junto con determinar si hay diferencias significativas entre sexos y comparar los resultados con dos muestras de controles sin fisura labiopalatina. Imágenes de radiografías cefalométricas laterales fueron utilizadas para determinar la frecuencia de PP (n= 163) y PS­AC (n=150). Los controles estuvieron conformados por 1056 y 417 radiografías de pacientes sin fisura labiopalatina. La prueba chi-cuadrado fue utilizada para determinar si existen diferencias significativas entre los sexos de los pacientes con fisura labiopalatina, y también entre las muestras de fisura labiopalatina y los controles. El nivel de confianza se determinó en p<0,05. En pacientes con fisura labiopalatina, la frecuencia de PP (parcial y completo) fue de 11,04% y 6,13% respectivamente; fue más frecuente en hombres y no se encontraron diferencias significativas entre sexos y entre la comparación entre muestras (p>0,05). La frecuencia de PS y AC fue de 6% y 8,7%, respectivamente; ambos fueron más frecuentes en hombres y no se encontraron diferencias significativas entre sexos; no se encontró diferencias significativas entre las muestras de fisura labiopalatina y los controles en el caso del PS. La frecuencia de AC fue significativamente menor en el grupo de fisura labiopalatina y en las mujeres con fisura labiopalatina respecto a la población sin fisura labiopalatina (p<0,05). Las frecuencias de PP y PS fueron similares a las del grupo control y no deberían ser consideradas como anomalías asociadas a la fisura labiopalatina; la frecuencia del AC fue significativamente menor en pacientes con fisura labiopalatina, y tampoco debería considerarse como una anomalía asociada; este hallazgo podría ser confirmado en futuros estudios centrados en las apófisis clinoides.

Persistence of intrasellar trigeminal artery and simultaneous pituitary adenoma: description of two cases and their importance for the differential diagnosis of sellar lesions / Persistência da artéria trigeminal intrasselar e simultâneo adenoma de hipófise: descrição de dois casos e importância para o diagnóstico diferencial das lesões selares

Persistent trigeminal artery (PTA) is the most frequent embryonic communication between the carotid and vertebrobasilar systems. However, hormonal changes or the association of PTA with other sellar lesions, such as pituitary adenomas, are extremely rare. The aim of the present study was to report two patients with intrasellar PTA and simultaneous pituitary adenoma in order to emphasize the importance of differential diagnoses for sellar lesions. Case 1. A female patient, 41 years old, was admitted with a history of chronic headache (> 20 years). Pituitary magnetic resonance imaging (MRI) showed a rounded lesion in the left portion of the pituitary gland suggestive of adenoma (most likely clinically non-functioning adenoma). In addition to this lesion, the MRI demonstrated ecstasy of the right internal carotid artery and imaging suggestive of an intrasellar artery that was subsequently confirmed by an angio-MRI of the cerebral vessels as PTA. Case 2. A female patient, 42 years old, was admitted with a history of amenorrhea and galactorrhea in 1994. Laboratorial investigation revealed hyperprolactinemia. Pituitary MRI showed a small hyposignal area in the anterior portion of pituitary gland suggestive of a microadenoma initiated by a dopaminergic agonist. Upon follow-up, aside from the first lesion, the MRI showed a well delineated rounded lesion inside the pituitary gland, similar to a vessel. Angio-MRI confirmed a left primitive PTA. Failure to recognize these anomalous vessels within the sella might lead to serious complications during transsphenoidal surgery. Therefore, although their occurrence is uncommon, a working knowledge of vascular lesions in the sella turcica or pituitary gland is important for the differential diagnosis of pituitary lesions, especially pituitary adenomas.

Persistência da artéria trigeminal (PAT) é a comunicação embrionária mais frequente entre os sistemas carotídeo e vertebrobasilar. No entanto, alterações hormonais ou associação de PAT com outras lesões selares, como adenomas hipofisários, são extremamente raros. O objetivo do presente estudo foi relatar dois pacientes com PAT intrasselar e concomitante adenoma hipofisário e enfatizar a importância para o diagnóstico diferencial de lesões selares. Caso 1. Paciente do sexo feminino, 41 anos, admitida com história de cefaleia crônica (> 20 anos). Ressonância magnética (RM) de hipófise mostrou imagem arredondada na porção esquerda da glândula sugestiva de adenoma (provavelmente adenoma clinicamente não funcionante). Adicionalmente, a RM demonstrou ectasia da artéria carótida interna direita e imagem sugestiva de artéria intrasselar, posteriormente confirmada por angio-RM dos vasos cerebrais como PAT. Caso 2. Paciente do sexo feminino, 42 anos, admitida com história de amenorreia e galactorreia em 1994. A investigação laboratorial revelou hiperprolactinemia. RM de hipófise mostrou pequena área de hipossinal na porção anterior da glândula sugestiva de microadenoma, sendo iniciado agonista dopaminérgico. Na evolução, além da primeira lesão, a RM mostrou uma imagem arredondada bem delimitada dentro da glândula pituitária semelhante a vaso sanguíneo. Angio-RM confirmou PAT primitiva esquerda. A falta de reconhecimento de tais vasos anômalos dentro da sela túrcica pode levar a sérias complicações durante a cirurgia transesfenoidal. Portanto, apesar de sua ocorrência não ser comum, o conhecimento de lesões vasculares dentro da sela túrcica ou glândula hipofisária é importante para o diagnóstico diferencial de lesões da hipófise, especialmente com adenomas hipofisários.

Dental and skeletal components of Class II open bite treatment with a modified Thurow appliance

INTRODUCTION: Due to the lack of studies that distinguish between dentoalveolar and basal changes caused by the Thurow appliance, this clinical study, carried out by the School of Dentistry - State University of São Paulo/Araraquara, aimed at assessing the dental and skeletal changes induced by modified Thurow appliance. METHODS: The sample included an experimental group comprising 13 subjects aged between 7 and 10 years old, with Class II malocclusion and anterior open bite, and a control group comprising 22 subjects similar in age, sex and mandibular plane angle. Maxillary/mandibular, horizontal/vertical, dental/skeletal movements (ANS, PNS, U1, U6, Co, Go, Pog, L1, L6) were assessed, based on 14 landmarks, 8 angles (S-N-ANS, SNA, PPA, S-N-Pog, SNB, MPA, PP/MPA, ANB) and 3 linear measures (N-Me, ANS-Me, S-Go). RESULTS: Treatment caused significantly greater angle decrease between the palatal and the mandibular plane of the experimental group, primarily due to an increase in the palatal plane angle. ANB, SNA and S-N-ANS angles significantly decreased more in patients from the experimental group. PNS was superiorly remodeled. Lower face height (ANS-Me) decreased in the experimental group and increased in the control group. CONCLUSIONS: The modified Thurow appliance controlled vertical and horizontal displacements of the maxilla, rotated the maxilla and improved open bite malocclusion, decreasing lower facial height. .

INTRODUÇÃO: devido ao número reduzido de estudos que distinguem entre as mudanças dentoalveolares e ósseas produzidas pelo aparelho de Thurow, esse estudo clínico, conduzido pelo departamento de Ortodontia da Faculdade de Odontologia de Araraquara, foi proposto para avaliar as mudanças dentárias e esqueléticas induzidas pelo aparelho de Thurow modificado. MÉTODOS: a amostra incluiu um grupo experimental de 13 indivíduos, entre 7 e 10 anos de idade, com má oclusão Classe II e mordida aberta anterior, e um grupo controle de 22 indivíduos, com idades, plano mandibular e má oclusão similares. Com base em 14 pontos cefalométricos, 8 ângulos (S-N-ANS, SNA, PPA, S-N-Pog, SNB, MPA, PP/MPA, ANB) e 3 medidas lineares (N-Me, ANS-Me, S-Go), foram avaliados movimentos maxilares e mandibulares horizontais e verticais dentários e esqueléticos (ANS, PNS, U1, U6, Co, Go, Pog, L1, L6). RESULTADOS: o tratamento produziu diminuição significativamente maior no ângulo entre o plano palatino e o plano mandibular do grupo controle, devido ao aumento do ângulo do plano palatino. Os ângulos A-N-B, S-N-A e S-N-ANS diminuíram significativamente mais no grupo tratado. A PNS sofreu remodelação superior. A altura facial inferior (ANS-Me) diminuiu no grupo tratado, ao passo que aumentou no grupo controle. CONCLUSÕES: o aparelho controlou deslocamento vertical e horizontal da maxila, rotacionou a maxila para melhorar a mordida aberta e diminuiu a altura facial inferior. .

Pure Intrasellar Meningioma Located Under the Pituitary Gland: Case Report

Most intrasellar meningiomas are located in the subdiaphragmatic and supraglandular region because they originate from the diaphragma sellae. Subglandular meningiomas located under the pituitary gland are extremely rare. Intrasellar meningiomas in the subdiaphragmatic and subglandular region probably originate from the dura in the sellar floor. We report a case of a subglandular meningioma along with a review of the literature.

Metástasis selar y al seno cavernoso, de un carcinoma de células de Merkel: reporte de un caso / Sellar and cavernous sinus metastasis from a Merkel cell carcinoma: case report and literature review

El carcinoma de células de Merkel es un tumor maligno y muy raro de la piel. Este tumor emerge de las células epiteliales que presentan una diferenciación neuroendocrina. Ocurre más comúnmente en los adultos masculinos mayores. Las metástasis intracraneales de un carcinoma de células de Merkel son aun más raras. En este articulo, se presentara un caso de un paciente masculino de 70 años con antecedente de carcinoma de células de Merkel quien debuto con una ptosis que progresivamente presento una parálisis completa del tercer par craneal. En los estudios realizados al paciente, se encontró una lesión infiltrante al seno cavernoso y a la silla turca que resulto ser una metástasis por un carcinoma de células de Merkel. En esta publicación, se discutirá el caso clínico y se revisará la literatura al respecto de estas lesiones raras del sistema nervioso central.

Merkel cell carcinoma is a rare and malignant tumor of the skin. This lesion arises from epithelial cells that have undergone neuroendocrine differentiation. They are more commonly found in older male adults. Intracranial metastases from Markel cell carcinoma are even rarer. We will present a case of a 70 year old male with known history of Merkel cell carcinoma that suddenly presented with a right eye ptosis and progressively presented complete third nerve palsy. The patient was studied and was found to have an infiltrating lesion of the cavernous sinus and the sellar region. This tumor resulted in a metastasis from a Merkel Cell Carcinoma. In this article we will discuss the clinical case of this patient and we will review the literature concerning this rare lesion of the Central nervous system.

Skeletal alterations associated with the use of bonded rapid maxillary expansion appliance

Bonded maxillary expansion appliances have been suggested to control increases in the vertical dimension of the face after rapid maxillary expansion (RME). However, there is still no consensus in the literature about its real skeletal effects. The purpose of this prospective study was to evaluate, longitudinally, the vertical and sagittal cephalometric alterations after RME performed with bonded maxillary expansion appliance. The sample consisted of 26 children, with a mean age of 8.7 years (range: 6.9-10.9 years), with posterior skeletal crossbite and indication for RME. After maxillary expansion, the bonded appliance was used as a fixed retention for 3.4 months, being replaced by a removable retention subsequently. The cephalometric study was performed onto lateral radiographs, taken before treatment was started, and again 6.3 months after removing the bonded appliance. Intra-group comparison was made using paired t test. The results showed that there were no significant sagittal skeletal changes at the end of treatment. There was a small vertical skeletal increase in five of the eleven evaluated cephalometric measures. The maxilla displaced downward, but it did not modify the facial growth patterns or the direction of the mandible growth. Under the specific conditions of this research, it may be concluded that RME with acrylic bonded maxillary expansion appliance did promote signifciant vertical or sagittal cephalometric alterations. The vertical changes found with the use of the bonded appliance were small and probably transitory, similar to those occurred with the use of banded expansion appliances.

Os aparelhos disjuntores com cobertura oclusal de acrílico têm sido sugeridos para controlar o aumento da dimensão vertical da face após a expansão rápida da maxila (ERM). Entretanto, ainda não há consenso na literatura sobre seu real efeito esquelético. O objetivo desse estudo prospectivo foi avaliar longitudinalmente as alterações esqueléticas verticais e sagitais após a ERM realizada com o aparelho disjuntor com cobertura oclusal. A amostra consistiu de 26 crianças, com idade média de 8,7 anos (variação: 6.9-10,9 anos), apresentando mordida cruzada posterior esquelética e indicação para ERM. Após a expansão maxilar, o aparelho foi utilizado como contenção fixa por 3,4 meses, sendo posteriormente substituído por uma contenção removível. O estudo cefalométrico foi realizado em telerradiografias laterais tomadas antes do início do tratamento e novamente 6,3 meses após a remoção do disjuntor. A comparação intragrupo foi feita utilizando-se o teste t pareado. Os resultados mostraram que não houve alterações esqueléticas sagitais significantes ao fim do tratamento. Houve um pequeno aumento em cinco das onze medidas cefalométricas verticais analisadas. A maxila se moveu inferiormente, porém não modificou o padrão de crescimento facial, a inclinação ou direção de crescimento mandibular. Considerando-se as condições específicas deste trabalho, pode-se concluir que a ERM realizada com o aparelho disjuntor com cobertura oclusal de acrílico não promoveu alterações esqueléticas verticais ou sagitais prejudiciais. As alterações verticais encontradas com o uso do aparelho colado foram pequenas e provavelmente transitórias, similar ao que ocorre com o uso dos aparelhos expansores bandados.

Masseter muscle thickness in different skeletal morphology: An ultrasonographic study.

Background: The thickness of the masseter muscle during relaxation and contraction states was measured by ultrasonography. Subjects were classified according to their sagittal skeletal relationships. The association between muscle thickness and facial morphology was studied. Context: Masseter muscle thickness influences the skeletal patterns. Aim: To measure and compare the thickness of the masseter muscle in individuals with skeletal class I occlusion and skeletal class II malocclusions and to correlate its relationship with craniofacial morphology. Settings and Design: The study was conducted in a hospital setup and was designed to study the thickness of the masseter muscle in different skeletal morphologies. Materials and Methods: Seventy two individuals between the ages of 18 and 25 years were divided into Group I, Group IIA and Group IIB according to their skeletal relationships. Masseter muscle thickness was measured by ultrasonography. Eight linear and six angular cephalometric measurements were assessed. Statistical Analysis Used: Analysis of variance and Pearson's correlation analysis. Results: There was a statistically significant difference in muscle thickness between subjects of different skeletal patterns. Significant positive correlation between masseter muscle thickness and posterior total face height, jarabak ratio, ramus height, mandibular length and significant negative correlations with mandibular plane angle, gonial angle and PP-MP angle were observed. Conclusion: This study indicates the strong association between the masseter muscle and skeletal morphology.

Análise da expressão dos genes PROP1 e CTNNB1 em craniofaringiomas adamantinomatosos com e sem mutação somática no CTNNB1 / Analysis of PROP1 and CTNNB1 expression genes in adamantinomatous craniopharyngiomas with and without CTNNB1 somatic mutation

Os craniofaringiomas são os tumores mais frequentes da região hipotálamohipofisária na faixa etária pediátrica. Apesar de serem histologicamente benignos, sua tendência infiltrativa e seu comportamento agressivo resultam em significante morbimortalidade. Histologicamente podem ser divididos em dois subtipos: adamantinomatosos e papilíferos. A patogênese dos craniofaringiomas é pouco compreendida. Mutações no gene CTNNB1, que codifica a proteína beta-catenina, são a única alteração molecular conhecida até o momento implicada na tumorigênese dos craniofaringiomas adamantinomatosos. Tais mutações afetam o sítio de degradação da beta-catenina, que passa a se acumular no citoplasma e no núcleo, ativando excessivamente a via de sinalização WNT, através da ligação aos fatores de transcrição da família LEF/TCF, levando a tumorigênese. Recentemente foi descoberto um novo mecanismo de determinação da linhagem celular hipofisária regulado pela beta-catenina, através do qual ela interage diretamente com o PROP1 para determinar a diferenciação celular hipofisária. De acordo com esse modelo, o complexo protéico PROP1/beta- catenina atua simultaneamente como repressor do HESX1 e ativador do PIT1, dependendo dos co-fatores associados. Pacientes com mutações germinativas inativadoras no PROP1 desenvolvem hipopituitarismo e podem apresentar aumento hipofisário com imagens de ressonância nuclear magnética (RNM) da região selar muitas vezes semelhantes àquelas dos craniofaringiomas, com hiperssinal em T1. Por outro lado, camundongos com expressão persistente do Prop1 exibem defeitos na regulação da proliferação celular hipofisária, incluindo cistos da bolsa de Rathke, hiperplasia adenomatosa e tumores, sugerindo que mutações com ganho de função no PROP1 também poderiam contribuir para a patogênese de tumores hipofisários em seres humanos. A semelhança entre as imagens de RNM dos pacientes com craniofaringiomas e daqueles com aumento hipofisário devido a mutações...

Craniopharyngiomas are the the commonest tumors to involve the hypothalamo-pituitary regions in childhood population. Histologically they are benign, and can be divided in two primary subtypes: the adamantinomatous and the papillary. Although histologically benign, their infiltrative tendency and aggressive behavior can result in great morbidity. The pathogenesis of craniopharyngiomas is poorly understood. To date, beta-catenin gene (CTNNB1) mutations have been identified only in the adamantinomatous subtype. These mutations affect the degradation target box of beta-catenin that accumulates in the cytoplasm and the nucleus increasing the transcriptional activity of WNT pathway through interaction with the transcription factors of LEF/TCF family, leading to tumorigenesis. Recently, an interaction between beta-catenin and PROP1 was described as a new mecanism for beta-catenindependent regulation of pituitary cell-lineage determination. According to this novel model, the PROP1/beta-catenin proteic complex would act as a binary switch to simultaneously repress the transcription factor HESX1 and to activate expression of transcription factor PIT1, depending on the associated cofactors. Patients with loss-of-function mutations in PROP1 present combined pituitary hormonal deficiency generally associated with pituitary enlargement and the magnetic resonance imaging (MRI) of the sellar region in these patients sometimes resembles that of the craniopharyngiomas, with T1 hyperintense signal. On the other hand, transgenic mice with persistent Prop1 expression exhibit defects consistent with misregulation of pituitary cell proliferation, including adenomatous hyperplasia with formation of Rathke's cleft cysts and tumors suggesting that misregulation of PROP1 expression in human could contribute to pathogenesis of pituitary tumors. The similarity between the MRI images of craniopharyngiomas patients and that of patients with loss-of-function mutations in...

Ossification of the sella turcica and clinoid ligments: case report, morphological study and literature review

The morphology of the Sella Turcica is important both as a reference in cephalometric studies and in the pituitary gland, internal carotid artery and cavernous sinus surgical boarding. The present report describes a case of partial ossification of the sella turcica walls in a dry skull of 40-year-old Brazilian individual. We analyze the morphometric characteristics of the region.

La morfología de la silla turca tiene importancia tanto como referencia en los estudios cefalomé trieos como en los abordajes quirúrgicos de la glándula hipófisis, carótida interna y seno cavernoso. En el presente reporte se describe un caso de osificación parcial de las paredes de la silla turca, en un cráneo seco de un individuo brasilero de 40 años. Se analizan las características morfométricas de la región.

Adenoma ectópico hipofisiario: reporte de un caso y revisión de la literatura: [revisión] / Ectopic hypophysial adenoma: case report and literature review: [revision]

Los adenomas son los tumores hipofisiarios más frecuentes y ocasionalmente se pueden ubicar fuera de la silla turca, ya sea por extensión desde ésta o más rara vez por ubicación ectópica. Se presenta el caso de una paciente de 56 años, en la que en un TAC de cerebro solicitado en el contexto de un síndrome vertiginoso, se encuentra una lesión en región esfenoidal que se extiende al clivus, frente a la cual se plantean como posibilidades diagnósticas: cordoma, carcinoma nasofaríngeo, tumor derivado de una glándula salival menor y un tumor metastático. La lesión es abordada y extraída endoscópicamente, comprobándose finalmente que correspondía a un adenoma ectópico hipofisiario.

Adenomas are the most frequent hypophyseal tumors, and they can be occasionally located outside the sella turcica, either by growth from it or, less frequently by ectopic location. The case of a 56year old female is presented, in which a brain TC prescribed in the context of a vertiginous syndrome revealed a lesion located in the sphenoid region extending to the clivus. Several diagnoses were discussed: chordoma, nasopharyngeal carcinoma, minor salivary gland derived tumor, and metastatic tumor. The lesión was surgically approached and excised, and it was finally demonstrated to be an ectopic hypophyseal adenoma.

Sellar lesion: not always a pituitary adenoma.

Inflammatory lesions of the hypophysis account for 0.5% of all symptomatic diseases of the pituitary, which include lymphocytic hypophysitis, granulomatous hypophysitis with or without specific etiology and pituitary abscess. Sellar tuberculoma is a rare type of granulomatous hypophysitis. We document a case of a postmenopausal lady who presented with galactorrhea, headache and blurring of vision. Based on preliminary investigations, a clinical diagnosis of pituitary adenoma was made and the pituitary gland was surgically excised. Histopathological examination showed caseating granulomas, along with normal areas of preserved pituitary gland and a final diagnosis of tuberculous hypophysitis was made. This case is being documented due to the extremely rare involvement of the pituitary gland by granulomatous lesions such as tuberculosis and to emphasize the role of intraoperative consultation to obviate the need for radical surgery in such lesions.

Neurosurgical approaches for tumours of sella area: series of 142 tumours

The aim of this retrospective study of 142 patients, who were admitted for tumours of the sellar area [TSA], was to analyse the clinical, neuroimaging, histological features and neurosurgical approaches employed to treat these lesions. One-hundred and forty-two TSA were operated by the senior author between 1990 and 2004 in 72 women and 70 men. Among them there were 36 patients less than 15 years of age. Common clinical presentation included headache, vision impairment and endocrinological symptoms. Blindness, uni or bilateral was observed in 30 cases [21%] and diabetes insipidus in 20 cases [14%]. Imaging features showed tumour diameter at presentation greater than 5 cm in 49 tumours [34.5%]. Histological examination revealed a predominance of craniopharyngiomas in 68 patients [47.8%] and meningiomas in 39 patients [27.4%]. Neurosurgical approaches were chosen according to the size, extent, and components of the tumour. The most practised approach was subfrontal in 77 tumours [54.2%] and pterional or fronto pterional in 55 tumours [38.7%]. Radical tumour removal was achieved in 41 patients [28.8%]: 14 cases [35.9%] of meningiomas, 13 cases [52%] of invasive adenomas, 11 cases [16.1%] of craniopharyngiomas and 3 cases [75%] of epidermoid cysts. Twenty-four patients underwent a second surgical procedure for recurrence or residual tumour. Among them, 6 patients with cystic craniopharyngiomas had undergone radioactive yttrium [6 cases] or bleomycin injection [2 cases]. Mortality rate was 11% [15 patients]; mostly due to brain swelling, brain oedema or meningitis. There is not a standardised neurosurgical approach for TSA but this should result from a careful study of the neuroimaging features. When to stop the operation was mandated by the operative findings and the concern for respect of the optic pathways, pituitary stalk and vessels of this area

Metastatic prolactinoma: case report with immunohistochemical assessment for p53 and Ki-67 antigens

Pituitary carcinomas are rare neoplasms characterized by craniospinal and/or systemic metastases originated from the pituitary. Their histopathology is frequently indistinguishable from that of benign adenomas. The development of markers that better reflect their behavior is desirable. We present the case of a 47 year-old man with a prolactin-secreting macroadenoma who was submitted to surgeries, cranial radiation therapy, and bromocriptine treatment, but evolved to a fatal outcome after the disclosure of intracranial metastases. Tumor samples underwent p53 and Ki-67 immunohistochemical assessment. p53 was absent in all samples, a rare finding among pituitary carcinomas. Ki-67 proliferative index was 2.80% in the original tumor, 4.40% in the relapse, and 4.45% in the metastasis. The figure in the relapse is higher than the expected for a noninvasive adenoma. In conclusion, p53 staining is not positive in all pituitary carcinomas. A high Ki-67 proliferative index in a pituitary adenoma might indicate a more aggressive behavior.

Carcinomas pituitários são neoplasias raras caracterizadas pela presença de metástases cranio-espinhais e/ou sistêmicas originadas da hipófise. Sua histopatologia é freqüentemente indistinguível daquela dos adenomas benignos. O desenvolvimento de marcadores que melhor reflitam o seu comportamento é desejável. Apresentamos o caso de um homem de 47 anos com um macroadenoma secretor de prolactina que foi submetido a procedimentos cirúrgicos, radioterapia e tratamento com bromocriptina, mas que evoluiu para o óbito após o descobrimento de metástases intracranianas. Amostras do tumor foram submetidas à análise imuno-histoquímica para os antígenos p53 e Ki-67. A coloração para p53 foi negativa em todas as amostras, um achado raro entre os carcinomas pituitários. O índice proliferativo Ki-67 foi 2,80% no tumor original, 4,40% na recidiva e 4,45% na metástase. O valor obtido na recidiva é maior que o esperado para um adenoma não-invasor. Concluindo, a coloração para p53 não é positiva em todos os carcinomas pituitários. Um índice proliferativo Ki-67 alto em um adenoma pituitário poderia indicar um comportamento mais agressivo.