Clinical and radiological evaluation of maxillofacial and otorhinolaryngological manifestations of Hansen's disease.

To characterize maxillofacial, otorhinolaryngological and oral manifestations of Hansen's disease (HD), we conducted a cross-sectional study in 21 current patients attending the Unidade Básica de Saúde de Jardim América, Espírito Santo, Brazil and 16 former patients resident at Pedro Fontes Hospital using data from computed tomography imaging, rhinoscopy, and oroscopy. Maxillofacial characteristics were compared with 37 controls. Differences in bone alterations across the three groups were determined mainly by severe resorption/atrophy being more frequent in former HD patients, with severe resorption/atrophy of the anterior alveolar process of maxilla in 50.0% (8/16) of former patients, 28.6% (6/21) of current patients and 10.8% (4/37) of controls and of nasal bones and aperture in 31.3% (5/16) of former patients compared with 0/21 current patients and two controls. There were no substantial differences in otorhinolaryngological and oroscopic findings between the two patient groups. HD patients had more tooth loss than the age-matched control group. Maxillofacial, otorhinolaryngological and oroscopic finding scores were strongly correlated only in current HD patients. Correlation between otorhinolaryngological and maxillofacial scores suggests that protocols for HD patient assessment and follow-up could include otorhinolaryngological evaluation, with radiological imaging where necessary, subject to replication of our findings in a larger study.

Clinical and radiological assessment of rhinomaxillary syndrome in Hansen's disease.

Background More than four million people today live with Hansen's disease, and 200,000 new cases are diagnosed every year. Lifetime effects of Hansen's disease manifest as changes to bones of the face, hands and feet, resulting in physical impairment, secondary complications and facial changes that can be detrimental to quality of life, particularly among the elderly. Aims This study aimed to perform a detailed characterization of rhinomaxillary syndrome and its clinical manifestations in older persons treated in the past for Hansen's disease. Methods This was a cross-sectional study to characterize rhinomaxillary syndrome among older persons (age 60+ years) resident at Pedro Fontes Hospital, Cariacica, Espírito Santo, Brazil. Computed tomography images were examined with three-dimensional reconstructions to assess alterations to maxillofacial bones according to criteria for radiological rhinomaxillary syndrome. Participants were examined to assess facial alterations according to criteria for clinical rhinomaxillary syndrome. Results Rhinomaxillary syndrome was investigated in 16 participants (ten females and six males), median age 70 (range 60-89) years, age at diagnosis 20 (6-43) years and time since diagnosis 46 (26-70) years. Four participants fully met radiological rhinomaxillary syndrome criteria, four partially. All participants with full radiological rhinomaxillary syndrome presented with facial changes which met criteria for clinical rhinomaxillary syndrome, including "saddle nose" (loss of nasal dorsal height and shortened length of nose, due to cartilaginous and/or bone collapse), concave middle third of the face with sunken nose, maxillary retrognathia and inverted upper lip. Limitations Clinical histories were incomplete for some participants because records were lost at the hospital over time. Conclusion Until Hansen's disease is eliminated from endemic countries, persons affected will continue to present with rhinomaxillofacial alterations caused by Mycobacterium leprae infection. Clinical protocols for assessment and long-term care need to include otorhinolaryngological evaluation, mainly to prevent secondary complications. When rhinomaxillofacial bone changes are suspected, this evaluation should be supported by computed tomography imaging, if available.

Skeletal involvement in Gaucher disease: extent of bone disease, splenic volume, and quality of life.

OBJECTIVE: To investigate the correlations among the extent of bone involvement, splenic volume, and quality of life in patients with Gaucher disease. MATERIALS AND METHODS: This was a descriptive, prospective cross-sectional study of 18 patients with Gaucher disease who underwent 3-T magnetic resonance imaging of both femurs and the lumbar spine. Semiquantitative analyses were performed on the basis of the bone marrow burden (BMB) score. We looked for linear relationships among the variables splenic volume, quality of life score, and BMB score. RESULTS: We identified a linear relationship between the BMB scores and splenic volume. The quality of life score showed no statistically significant relationship with splenic volume or the BMB score. CONCLUSION: The linear relationship between the BMB score and the splenic volume indicates that the extent of bone disease is greater in individuals with splenomegaly. No correlation was found between the BMB and quality of life scores, illustrating the insidious and silent progression of Gaucher disease.

OBJETIVO: Investigar a correlação entre a extensão do envolvimento ósseo, o volume esplênico e a qualidade de vida em pacientes com doença de Gaucher. MATERIAIS E MÉTODOS: Estudo descritivo, prospectivo e transversal de 18 pacientes com doença de Gaucher submetidos a ressonância magnética de 3-T de ambos os fêmures e da coluna lombar. Análise semiquantitativa foi feita utilizando o escore bone marrow burden (BMB). Correlação linear foi estudada para as variáveis volume esplênico, qualidade de vida e escore BMB. RESULTADOS: Uma correlação linear entre os escores BMB e volume esplênico foi demonstrada. Em relação ao índice de qualidade de vida, não foi observada correlação estatisticamente significante nem com o volume esplênico e nem com o escore BMB. CONCLUSÃO: Nosso estudo demonstrou uma correlação linear entre o escore BMB e o volume esplênico, correspondendo a maior extensão de doença óssea em indivíduos com maior esplenomegalia. Nenhuma correlação foi encontrada entre o escore BMB e a qualidade de vida, indicando a natureza insidiosa e a progressão silenciosa da doença de Gaucher.

Skeletal involvement in Gaucher disease: extent of bone disease, splenic volume, and quality of life / Envolvimento ósseo na doença de Gaucher: extensão do acometimento ósseo, volume esplênico e qualidade de vida

Abstract Objective: To investigate the correlations among the extent of bone involvement, splenic volume, and quality of life in patients with Gaucher disease. Materials and Methods: This was a descriptive, prospective cross-sectional study of 18 patients with Gaucher disease who underwent 3-T magnetic resonance imaging of both femurs and the lumbar spine. Semiquantitative analyses were performed on the basis of the bone marrow burden (BMB) score. We looked for linear relationships among the variables splenic volume, quality of life score, and BMB score. Results: We identified a linear relationship between the BMB scores and splenic volume. The quality of life score showed no statistically significant relationship with splenic volume or the BMB score. Conclusion: The linear relationship between the BMB score and the splenic volume indicates that the extent of bone disease is greater in individuals with splenomegaly. No correlation was found between the BMB and quality of life scores, illustrating the insidious and silent progression of Gaucher disease.

Resumo Objetivo: Investigar a correlação entre a extensão do envolvimento ósseo, o volume esplênico e a qualidade de vida em pacientes com doença de Gaucher. Materiais e Métodos: Estudo descritivo, prospectivo e transversal de 18 pacientes com doença de Gaucher submetidos a ressonância magnética de 3-T de ambos os fêmures e da coluna lombar. Análise semiquantitativa foi feita utilizando o escore bone marrow burden (BMB). Correlação linear foi estudada para as variáveis volume esplênico, qualidade de vida e escore BMB. Resultados: Uma correlação linear entre os escores BMB e volume esplênico foi demonstrada. Em relação ao índice de qualidade de vida, não foi observada correlação estatisticamente significante nem com o volume esplênico e nem com o escore BMB. Conclusão: Nosso estudo demonstrou uma correlação linear entre o escore BMB e o volume esplênico, correspondendo a maior extensão de doença óssea em indivíduos com maior esplenomegalia. Nenhuma correlação foi encontrada entre o escore BMB e a qualidade de vida, indicando a natureza insidiosa e a progressão silenciosa da doença de Gaucher.

Three-Dimensional Zero Echo Time Magnetic Resonance Imaging Versus 3-Dimensional Computed Tomography for Glenoid Bone Assessment.

PURPOSE: To evaluate the 3-dimensional (3D) zero echo time (ZTE) magnetic resonance imaging (MRI) technique and compare it with 3D computed tomography (CT) for the assessment of the glenoid bone. METHODS: ZTE MRI using multiple resolutions and multislice CT were performed in 6 shoulder specimens before and after creation of glenoid defects and in 10 glenohumeral instability patients. Two musculoskeletal radiologists independently generated 3D volume-rendered images of the glenoid en face. Post-processing times and glenoid widths were measured. Inter-modality and inter-rater agreement was assessed. RESULTS: Intraclass correlation coefficients (ICCs) for inter-modality assessment showed almost perfect agreement for both readers, ranging from 0.949 to 0.991 for the ex vivo study and from 0.955 to 0.987 for the in vivo patients. Excellent interobserver agreement was found for both the ex vivo (ICCs ≥ 0.98) and in vivo (ICCs ≥ 0.92) studies. For the ex vivo study, Bland-Altman analyses for CT versus MRI showed a mean difference of 0.6 to 1 mm at 1.0-mm3 MRI resolution, 0.3 to 0.6 mm at 0.8-mm3 MRI resolution, and 0.3 to 0.6 mm at 0.6-mm3 MRI resolution for both readers. For the in vivo study, Bland-Altman analyses for CT versus MRI showed a mean difference of 0.6 to 0.8 mm at 1.0-mm3 MRI resolution, 0.5 to 0.6 mm at 0.8-mm3 MRI resolution, and 0.4 to 0.8 mm at 0.7-mm3 MRI resolution for both readers. Mean post-processing times to generate 3D images of the glenoid ranged from 32 to 46 seconds for CT and from 33 to 64 seconds for ZTE MRI. CONCLUSIONS: Three-dimensional ZTE MRI can potentially be considered as a technique to determine glenoid width and can be readily incorporated into the clinical workflow. LEVEL OF EVIDENCE: Level II, development of diagnostic criteria (consecutive patients with consistently applied reference standard and blinding).

Case Report: Nasal Myiasis in an Elderly Patient with Atrophic Rhinitis and Facial Sequelae of Leprosy.

We describe a case of nasal myiasis in an 89-year-old Brazilian patient affected by leprosy with severe nasal sequelae. An initial treatment comprising sinusectomy combined with nasal endoscopy removed more than 300 larvae, supplemented by systemic treatment using oral and topical ivermectin and levofloxacin. Infestation recurred after 2 months, was treated similarly, and resolved completely. The case could be attributed to severe nasal leprosy sequelae, with a lack of sneezing reflex, painless ulceration, atrophic rhinitis (ozena), and inability to clean the nose properly due to hand and nose impairment. This case illustrates the importance of long-term medical follow-up of patients with leprosy sequelae.

Imaging paracoccidioidomycosis: A pictorial review from head to toe.

Paracoccidioidomycosis is an infectious disease characterized primarily by pulmonary involvement and potential dissemination to other organs, mainly mucosa and skin; however, it can affect any organ in the body. Although difficult to diagnose purely based on imaging, imaging is important for diagnosis, follow-up, and assessment of disease-related complications. We provide a comprehensive review of the most notable imaging findings of paracoccidioidomycosis.

Discordance between clinical and imaging criteria: assessment by magnetic resonance imaging of the foot of patients with rheumatoid arthritis.

Evaluation of the correlation between disease activity and joint involvement was assessed by MRI in the clinically dominant foot of patients with RA. We conducted a cross-sectional descriptive study of 55 patients with RA, who were subjected to clinical evaluation and MRI assessment. Imaging of the clinically dominant foot was performed, and both the Disease Activity Score 28 (DAS28-CRP) and the Health Assessment Questionnaire-Disability Index (HAQ-DI) were measured. The majority of patients classified as in clinical remission presented some degree of inflammatory activity upon MRI evaluation. Statistical analysis demonstrated no correlation between MRI findings and clinical scores. There is evidence of disease activity on MRI of the clinically dominant foot even in patients classified as in clinical remission according to the DAS28-CRP criteria.

Magnetic resonance imaging and BMB score in the evaluation of bone involvement in Gaucher's disease patients.

OBJECTIVE: To evaluate by magnetic resonance imaging changes in bone marrow of patients undergoing treatment for type I Gaucher's disease. MATERIALS AND METHODS: Descriptive, cross-sectional study of Gaucher's disease patients submitted to 3 T magnetic resonance imaging of femurs and lumbar spine. The images were blindly reviewed and the findings were classified according to the semiquantitative bone marrow burden (BMB) scoring system. RESULTS: All of the seven evaluated patients (three men and four women) presented signs of bone marrow infiltration. Osteonecrosis of the femoral head was found in three patients, Erlenmeyer flask deformity in five, and no patient had vertebral body collapse. The mean BMB score was 11, ranging from 9 to 14. CONCLUSION: Magnetic resonance imaging is currently the method of choice for assessing bone involvement in Gaucher's disease in adults due to its high sensitivity to detect both focal and diffuse bone marrow changes, and the BMB score is a simplified method for semiquantitative analysis, without depending on advanced sequences or sophisticated hardware, allowing for the classification of the disease extent and assisting in the treatment monitoring.

OBJETIVO: Avaliar, por meio de ressonância magnética, alterações da medular óssea de pacientes em tratamento para doença de Gaucher tipo I. MATERIAIS E MÉTODOS: Estudo descritivo e transversal de pacientes com doença de Gaucher tipo I, com realização de ressonância magnética de 3 T da coluna lombar e fêmures, analisada por meio do escore semiquantitativo denominado bone marrow burden (BMB), sem conhecimento das informações clínicas. RESULTADOS: Dos sete pacientes avaliados (três homens e quatro mulheres), todos apresentaram sinais de infiltração da medula óssea. Osteonecrose da cabeça femoral foi encontrada em três pacientes, deformidade em frasco de Erlenmeyer em cinco e nenhum paciente apresentou colapso de corpo vertebral. A pontuação média do escore BMB total foi 11, variando de 9 a 14. CONCLUSÃO: A ressonância magnética é, atualmente, o método de escolha em adultos para avaliar o envolvimento ósseo na doença de Gaucher, em virtude da sua elevada sensibilidade na detecção de alterações tanto focais quanto difusas da medula óssea, sendo o escore BMB um método simplificado de análise semiquantitativa, sem depender de sequências avançadas ou de hardware sofisticado, permitindo classificar a extensão do acometimento da doença e ajudando no monitoramento do tratamento.

Magnetic resonance imaging and BMB score in the evaluation of bone involvement in Gaucher’s disease patients / Ressonância magnética e o escore BMB na avaliação do acometimento ósseo em pacientes com doença de Gaucher

AbstractObjective:To evaluate by magnetic resonance imaging changes in bone marrow of patients undergoing treatment for type I Gaucher’s disease.Materials and Methods:Descriptive, cross-sectional study of Gaucher’s disease patients submitted to 3 T magnetic resonance imaging of femurs and lumbar spine. The images were blindly reviewed and the findings were classified according to the semiquantitative bone marrow burden (BMB) scoring system.Results:All of the seven evaluated patients (three men and four women) presented signs of bone marrow infiltration. Osteonecrosis of the femoral head was found in three patients, Erlenmeyer flask deformity in five, and no patient had vertebral body collapse. The mean BMB score was 11, ranging from 9 to 14.Conclusion:Magnetic resonance imaging is currently the method of choice for assessing bone involvement in Gaucher’s disease in adults due to its high sensitivity to detect both focal and diffuse bone marrow changes, and the BMB score is a simplified method for semiquantitative analysis, without depending on advanced sequences or sophisticated hardware, allowing for the classification of the disease extent and assisting in the treatment monitoring.

ResumoObjetivo:Avaliar, por meio de ressonância magnética, alterações da medular óssea de pacientes em tratamento para doença de Gaucher tipo I.Materiais e Métodos:Estudo descritivo e transversal de pacientes com doença de Gaucher tipo I, com realização de ressonância magnética de 3 T da coluna lombar e fêmures, analisada por meio do escore semiquantitativo denominado bone marrow burden(BMB), sem conhecimento das informações clínicas.Resultados:Dos sete pacientes avaliados (três homens e quatro mulheres), todos apresentaram sinais de infiltração da medula óssea. Osteonecrose da cabeça femoral foi encontrada em três pacientes, deformidade em frasco de Erlenmeyer em cinco e nenhum paciente apresentou colapso de corpo vertebral. A pontuação média do escore BMB total foi 11, variando de 9 a 14.Conclusão:A ressonância magnética é, atualmente, o método de escolha em adultos para avaliar o envolvimento ósseo na doença de Gaucher, em virtude da sua elevada sensibilidade na detecção de alterações tanto focais quanto difusas da medula óssea, sendo o escore BMB um método simplificado de análise semiquantitativa, sem depender de sequências avançadas ou de hardware sofisticado, permitindo classificar a extensão do acometimento da doença e ajudando no monitoramento do tratamento.

Multisystemic Langerhans cell histiocytosis with advanced lung involvement.

Langerhans cell histiocytosis is a rare disease of unknown cause, characterized by the proliferation of histiocytic cells (Langerhans cells), that can sometimes be especially difficult to diagnose due to its wide clinical spectrum, ranging from a single lesion to a multisystemic disorder. Appropriate disease staging is fundamental, since treatment depends upon the severity of the disease, and imaging methods play a fundamental role not only in diagnosing and assessing the extent of Langerhans cell histiocytosis, as well as guiding the appropriate treatment for the patient and their monitoring.

Avaliação morfométrica do tendão de Aquiles por ultra-sonografia / Morphometric evaluation of Achilles tendon by ultrasound

OBJETIVO: Determinar os valores normais dos diâmetros ântero-posterior e transversal do tendão de Aquiles na nossa população e correlacioná-los com sexo, faixa etária, cor da pele, grupo sanguíneo ABO e índice de massa corporal. MATERIAIS E MÉTODOS: Foi feita análise ultra-sonográfica de 100 tendões de Aquiles de 50 voluntários sadios, visando à mensuração dos diâmetros ântero-posterior e transversal desses tendões. Todos os exames foram realizados pelo mesmo examinador, em aparelho de ultra-sonografia com transdutor linear com freqüência de 10 MHz. RESULTADOS: Dos 50 voluntários estudados, 25 eram do sexo masculino e 25, do sexo feminino, com a faixa etária variando de 20 a 52 anos (média de 33,9 anos). O valor médio do diâmetro transversal do tendão de Aquiles foi de 13,3 ± 1,0 mm para o sexo feminino e 14,4 ± 1,4 mm para o sexo masculino; em relação ao diâmetro ântero-posterior, foi de 5,4 ± 0,5 mm para o sexo feminino e 5,6 ± 0,6 mm para o sexo masculino. Os diâmetros do tendão de Aquiles foram significativamente menores no sexo feminino (p < 0,05). Não houve diferença estatisticamente significativa entre os diâmetros ântero-posterior e transversal em relação a faixa etária, grupo sanguíneo e cor da pele. O grupo com índice de massa corporal de sobrepeso apresentou diâmetro transversal do tendão de Aquiles significativamente maior que do grupo com índice de massa corporal normal. CONCLUSÃO: Os valores médios encontrados na nossa casuística foram discordantes em relação à maioria dos estudos da literatura, demonstrando ser de grande importância a padronização e o emprego de tabelas próprias da nossa população na prática clínica diária.

OBJECTIVE: The aim of this study was to determine the normal values of the Achilles tendon thickness in the anteroposterior dimension and width in the transverse dimension in our population, correlating them with gender, age ranges, race, ABO blood group and body mass index. MATERIALS AND METHODS: Ultrasonographic examination was employed to evaluate 100 Achilles tendons of 50 healthy volunteers in order to measure the tendons thickness in the anteroposterior dimension and width in the transverse dimension. All examinations were performed by the same sonographer, using an ultrasound equipment with a 10 MHz linear transducer. RESULTS: Among the 50 volunteers studied, 25 were men and 25 were women, ranging from 20 to 52 years (average 33.9 years). The tendons average width was 13.3 ± 1.0 mm in the female group and 14.4 ± 1.4 mm in the male group; the average thickness was 5.4 ± 0.5 mm in the female group and 5.6 ± 0.6 mm in the male group. These measurements were significantly lower in the female group (p < 0.05). There was no significant correlation between the tendon measurements and the age ranges, ABO blood groups and races. The tendon width was significantly higher in the overweight body mass index group than in the normal body mass index group. CONCLUSION: Mean values found in our study differ significantly from the majority of other studies in the literature, demonstrating the importance of creating our own standards employing tables based on our population in daily clinical practice.

Linfoma adrenal primário bilateral com envolvimento do sistema nervoso central: relato de caso / Bilateral primary adrenal lymphoma with central nervous system involvement: a case report

Os autores demonstram os principais achados do linfoma adrenal primário nas tomografias computadorizadas (TC) de abdome e crânio e na ressonância magnética (RM) do crânio, apresentando um caso raro de um paciente do sexo masculino, 72 anos de idade, com progressiva deterioração do estado mental, evoluindo, num período de dois meses, com perda da memória recente, desorientação e, finalmente, franco quadro demencial. O paciente foi submetido a investigação por métodos de imagem com TC de crânio (sem administração venosa de contraste iodado), tórax e abdome (sem e com administração venosa de contraste iodado), e RM de crânio (antes e após a administração venosa de gadolínio). Na TC de crânio observaram-se áreas nodulares levemente hiperdensas, adjacentes aos ventrículos laterais. A TC de tórax não mostrou alterações relevantes. Na TC do abdome foram demonstrados nódulos sólidos em ambas as adrenais. A RM de crânio evidenciou impregnação difusa e nodular do epêndima do IV ventrículo, III ventrículo, cornos anteriores e temporais, átrios e corpo dos ventrículos laterais. O diagnóstico definitivo foi realizado através do estudo imuno-histoquímico da peça após biópsia da adrenal. Os autores concluíram, após revisão atualizada da literatura, que a TC e a RM são métodos essenciais na detecção e melhor avaliação de linfomas adrenais primários, principalmente quando associados a envolvimento do sistema nervoso central, e a RM mostra-se mais sensível para a detecção de lesões extracerebrais nos espaços epidural e subdural, principalmente após a administração venosa de gadolínio.

Seqüestro broncopulmonar intralobar: relato de caso / Intralobar pulmonary sequestration: a case report

Neste trabalho é descrito um caso de uma paciente de 49 anos de idade que apresentava infecções pulmonares de repetição. Na telerradiografia de tórax observou-se massa no segmento basal posterior do pulmão direito, que na angiotomografia e nas reconstruções em 3D notou-se a presença de um suprimento sanguíneo proveniente da artéria aorta descendente. No exame da peça cirúrgica ratificou-se a presença de seqüestro pulmonar intralobar com cavidade preenchida por muco.

We report the case of a 49-year-old patient with repeated lung infections. Chest x-rays showed a mass in the posterior basal segment of the right lung. Angiotomography and 3D reconstructions showed a blood supply coming from the descending aorta. The analysis of the surgical specimen confirmed the occurrence of intralobar pulmonary sequestration with a cavitation filled with mucus.