Síndrome del incisivo central maxilar medio único en neonato / Solitary median maxillary central incisor syndrome in neonates / Síndrome do incisivo central maxilar médio solitário em neonatos

Introducción: el síndrome del incisivo central maxilar medio único (SMMCI) es un trastorno de etiología desconocida, con base genética heterogénea, que se caracteriza por la erupción de un único incisivo central en el maxilar y que se puede relacionar con multitud de patologías y síndromes, entre los que destacan las alteraciones de la línea media, obstrucción nasal congénita, disfunción hipofisaria, talla baja y holoprosencefalia. Caso clínico: neonato mujer con síndrome dismórfico no filiado y obstrucción nasal congénita, que es diagnosticada de SMMCI tras consultar en repetidas ocasiones por cuadros de dificultad respiratoria y problemas para alimentarse. Conclusiones: el conocimiento de este raro síndrome es fundamental para la realización de un diagnóstico precoz por parte del equipo pediátrico y obstétrico, ya que un diagnóstico temprano es posible, mejorando la evaluación prenatal ecográfica, así como el adecuado manejo posnatal multidisciplinar posterior de nuestros pacientes.

Introduction: the Solitary Median Maxillary Central Incisor Syndrome (SMMCI) is a disorder of unknown etiology, with a heterogeneous genetic basis, characterized by the eruption of a single central incisor in the maxilla and that can be linked to various pathologies and syndromes, among which the alterations of the midline, congenital nasal obstruction, pituitary dysfunction, short stature and holoprosencephaly stand out. Clinical case: female newborns with unknown dysmorphic syndrome and congenital nasal obstruction, diagnosed with SMMCI after repeated consultations due to respiratory distress and feeding problems. Conclusions: understanding this rare syndrome is essential for an early diagnosis to be carried out by the pediatric and obstetric team, since it will improve the ultrasound prenatal assessment, as well as the adequate subsequent multidisciplinary postnatal patient management procedures.

Introdução: a síndrome do incisivo central maxilar médio solitário (SICMMS) é uma desordem de etiologia desconhecida, com base genética heterogênea, caracterizada pela erupção de um único incisivo central na maxila e que pode estar relacionada a uma infinidade de patologias e síndromes. onde se destacam alterações da linha média, obstrução nasal congênita, disfunção hipofisária, baixa estatura e holoprosencefalia. Caso clínico: recém-nascida com síndrome dismórfica de origem desconhecida e obstrução nasal congênita, diagnosticada com SICMSS após várias consultas por desconforto respiratório e problemas de alimentação. Conclusões: o conhecimento desta rara síndrome é essencial para que a equipe pediátrica e obstétrica possa fazer um diagnóstico precoce, pois ele pode melhorar a avaliação ultrassonográfica pré-natal, bem como o adequado manejo pós-natal multidisciplinar pós-natal dos pacientes.

Primer reporte nacional de técnica de puestow modificada para el tratamiento de la pancreatitis aguda recidivante en pediatría / First national report of modified puestow technique for the treatment of recurrent acute pancreatitis in pediatrics / Primeiro relatório nacional de técnica puestow modificada para o tratamento de pancreatite aguda recorrente em pediatria

Se comunica el primer reporte nacional del tratamiento de pancreatitis aguda recidivante mediante derivación Wirsung-yeyunal en pediatría. Se trata de un paciente con múltiples ingresos hospitalarios por episodios de pancreatitis, con complicaciones evolutivas de pseudoquistes pancreáticos, estenosis y litiasis del conducto de Wirsung. Se realiza derivación Wirsung-yeyunal por vía convencional con buena evolución posterior.

The first national report of the treatment of recurrent acute pancreatitis by means of Wirsung-jejunal diversion in pediatrics is communicated. This is a patient with multiple hospital admissions for episodes of pancreatitis, with evolutionary complications of pancreatic pseudocysts, stenosis, and Wirsung duct lithiasis. Wirsung-jejunal bypass was performed by conventional route with good subsequent evolution.

O primeiro relato nacional do tratamento de pancreatite aguda recorrente por derivação Wirsung-jejunal em pediatria é relatado. Trata-se de um paciente com múltiplas internações hospitalares por episódios de pancreatite, com complicações progressivas de pseudocistos pancreáticos, estenose e cálculos do ducto de Wirsung. A derivação Wirsung-jejunal foi realizada por via convencional com boa evolução posterior.

Evaluation of three-dimensional high-resolution magnetic resonance imaging in the diagnosis of intracranial atherosclerotic stenosis / Evaluación de resonancia magnética tridimensional de alta resolución en el diagnóstico de la estenosis aterosclerótica intracraneal

SUMMARY: Intracranial artery stenosis (ICAS) was one of the main causes of ischemic stroke onset and recurrence. About 30 % of strokes were caused by intracranial artery stenosis. Intracranial artery stenosis had a high incidence in China and faced a high risk of recurrence for a long time. It affected patient safety and quality of life seriously. At the same time, it caused a heavy financial burden for the patient´s family. Therefore, early detection and accuracy of intracranial artery stenosis evaluation were extremely important. High-resolution magnetic resonance imaging (HR-MRI) had been widely used in clinical examinations, making up for the shortcomings of traditional vascular imaging methods that could only show the degree of luminal stenosis, making it possible to perform lumens, tube wall and plaque features of atherosclerotic intracranial arteries at the same time. There were still some controversies about the credibility of this technique in assessing the intracranial artery lumen stenosis. This article reviewed the application efficacy of HR-MRI technology in evaluating the degree of intracranial atherosclerotic stenosis.

RESUMEN: La estenosis de arterias intracraneales (ICAS) es una de las principales causas del ictus isquémico, como así también de su recurrencia. Alrededor del 30 % de los ataques cerebrovasculares son causados por estenosis de la arteria intracraneal. La estenosis de arterias intracraneales tiene una alta incidencia en China y enfrenta un alto riesgo de recurrencia, afectando gravemente la seguridad y la calidad de vida de los pacientes. Al mismo tiempo, supone una importante carga financiera para la familia de los pacientes. Por lo tanto, la detección temprana y la precisión de la evaluación de la estenosis de arterias intracraneales es extremadamente importante. La resonancia magnética de alta resolución (HR-MRI, por sus siglas en inglés) es utilizada ampliamente en los exámenes clínicos, compensando las deficiencias de los métodos tradicionales de imágenes vasculares que solo pueden mostrar el grado de estenosis luminal, haciendo posible el estudio de las características del lumen, pared vascular y la placa ateroesclerótica, de las arterias intracraneales afectadas, al mismo tiempo. Aún existen algunas controversias sobre la credibilidad de esta técnica en la evaluación de la estenosis del lumen de arterias intracraneales. En este artículo se revisó la eficacia de la aplicación de la tecnología HR-MRI para evaluar el grado de estenosis aterosclerótica intracraneal.

Estenosis congénita aislada del conducto auditivo interno como causa de hipoacusia neurosensorial unilateral y paresia del nervio facial / Congenital internal auditory canal stenosis presenting with unilateral sensorineural hearing loss and facial nerve dysfunction

RESUMEN La estenosis del conducto auditivo interno con aplasia/hipoplasia del nervio cocleovestibular es una patología muy infrecuente. Suele ser unilateral y puede acompañarse de aplasia/hipoplasia del nervio facial y otras malformaciones del oído interno. Se presentan aquí dos casos clínicos de pacientes pediátricos con estenosis del conducto auditivo interno unilateral con compromiso del séptimo y octavo par craneal ipsilateral. Se describen las historias y evaluaciones clínicas, hallazgos audiovestibulares, hallazgos imagenológicos, tratamientos indicados y sus resultados.

ABSTRACT Congenital internal auditory canal stenosis associated with aplasia/hypoplasia of the cochleovestibular nerve is a very infrequent pathology. It is usually unilateral and may be accompanied by aplasia/hypoplasia of the facial nerve and other malformations of the inner ear. We hereby present two clinical cases of pediatric patients with congenital internal auditory canal stenosis, with involvement of the seventh and eighth ipsilateral cranial nerve. The medical histories and clinical evaluations, audiovestibular findings, imaging findings, treatments and their results are described.

Sclerosing mediastinitis causing unilateral pulmonary edema due to left atrial and pulmonary venous compression. a case report and literature review

Abstract Sclerosing mediastinitis (SM), previously named chronic fibrosing mediastinitis, is an inflammatory process that in its end-stage results to sclerosis around the mediastinal structures. SM is quite rare and has been correlated with inflammatory and autoimmune diseases, as well as malignancy. SM may either present in a mild form, with minor symptoms and a benign course or in a more aggressive form with severe pulmonary hypertension and subsequent higher morbidity and mortality. The diagnosis of SM may be difficult and quite challenging, as symptoms depend on the mediastinal structure that is mainly involved; quite often the superior vena cava. However, practically any mediastinal structure may be involved by the fibrotic process, such as the central airways, as well as the pulmonary arteries and veins, leading to obstruction or total occlusion. The latter may be impossible to undergo proper surgical excision of the lesion, and is considered to be a real challenge to the surgeon. We herein report a case of SM that presented with arterial and venous compression. The imaging appearance was that of unilateral pulmonary edema, associated with lung collapse. The case is supplemented by a non-systematic review of the relevant literature.

Usefulness of preoperative venography in patients with cardiac implantable electronic devices submitted to lead replacement or device upgrade procedures / Utilidade da Venografia Pré-Operatória em Procedimentos de Troca de Cabos-Eletrodos ou Mudança do Modo de Estimulação

Abstract Background: Venous obstructions are common in patients with transvenous cardiac implantable electronic devices, but they rarely cause immediate clinical problems. The main consequence of these lesions is the difficulty in obtaining venous access for additional leads implantation. Objectives: We aimed to assess the prevalence and predictor factors of venous lesions in patients referred to lead reoperations, and to define the role of preoperative venography in the planning of these procedures. Methods: From April 2013 to July 2016, contrast venography was performed in 100 patients referred to device upgrade, revision and lead extraction. Venous lesions were classified as non-significant (< 50%), moderate stenosis (51-70%), severe stenosis (71-99%) or occlusion (100%). Collateral circulation was classified as absent, discrete, moderate or accentuated. The surgical strategy was defined according to the result of the preoperative venography. Univariate analysis was used to investigate predictor factors related to the occurrence of these lesions, with 5% of significance level. Results: Moderate venous stenosis was observed in 23%, severe in 13% and occlusions in 11%. There were no significant differences in relation to the device side or the venous segment. The usefulness of the preoperative venography to define the operative tactic was proven, and in 99% of the cases, the established surgical strategy could be performed according to plan. Conclusions: The prevalence of venous obstruction is high in CIED recipients referred to reoperations. Venography is highly indicated as a preoperative examination for allowing the adequate surgical planning of procedures involving previous transvenous leads.

Resumo Fundamento: Obstruções venosas são frequentes em portadores de dispositivos cardíacos eletrônicos implantáveis (DCEI) endocárdicos, mas raramente causam problemas clínicos imediatos. A principal consequência destas lesões é a dificuldade para obtenção de via de acesso para o implante de novos cabos-eletrodos. Objetivos: Determinar a prevalência de lesões venosas em candidatos a reoperações envolvendo o manuseio de cabos-eletrodos, e definir o papel da venografia pré-operatória no planejamento desses procedimentos. Métodos: De abril de 2013 a julho de 2016, 100 pacientes com indicação de troca de cabos-eletrodos, ou mudança no modo de estimulação, realizaram venografia com subtração digital no período pré-operatório. As lesões venosas foram classificadas em: não significativas (< 50%), moderadas (51-70%), graves (71-99%) ou oclusivas (100%), e a circulação colateral, em ausente, discreta, moderada ou acentuada. A estratégia cirúrgica foi definida a partir do resultado deste exame. Empregou-se análise univariada para a pesquisa de fatores de risco relacionados à ocorrência dessas lesões, com nível de significância de 5%. Resultados: Obstruções venosas moderadas foram observadas em 23%, graves em 13% e oclusões em 11% dos pacientes estudados, não sendo identificadas diferenças significativas em sua distribuição em relação ao lado do implante, ou do segmento venoso. A utilidade do exame para definição da tática operatória foi comprovada, sendo que em 99% dos casos, a estratégia cirúrgica estabelecida pode ser executada. Conclusões: A prevalência de obstruções venosas é elevada em portadores de DCEI que serão submetidos a reoperações. A venografia é altamente indicada como exame pré-operatório para o adequado planejamento cirúrgico de procedimentos envolvendo cabos-eletrodos transvenosos previamente implantados.

Vasculitis primaria del sistema nervioso central: presentación de caso clínico / Primary vasculitis of the central nervous system: presentation of clinical case / Vasculite primária do sistema nervoso central: apresentação do caso clínico

La vasculitis Primaria del Sistema Nervioso Central (VPSNC) se refiere a un grupo de enfermedades que resultan de la inflamación y destrucción de los vasos sanguíneos de la medula espinal, encéfalo y meninges, tanto en el sector venoso como arterial. La presentación es heterogénea y poco sistematizable. El diagnóstico se establece con un cuadro clínico compatible, una angiografía que evidencie vasculitis y/o biopsia del parénquima encefálico o meninges. Las alteraciones en los estudios de imagen son constantes pero inespecíficas para el diagnóstico y se acompañan habitualmente de alteraciones en el líquido cefalorraquídeo (LCR) y electroencefalograma (EEG) Presentamos un paciente con probable VPSNC basados en un cuadro clínico compatible, hallazgos imagenológicos sugestivos, junto con alteraciones en LCR y EEG. Se realizó tratamiento en base a corticoides e inmunosupresores con mala respuesta y evolución.

The primary central nervous system vasculitis (VPSNC) refers to a group of diseases that result from inflammation and destruction of the blood vessels of the spinal cord, brain and meninges, both in the venous and arterial sector. The presentation is heterogeneous and unsystematized. The diagnosis is made based on compatible symptoms, supported by an angiography showing evidence of vasculitis and/or biopsy of the brain parenchyma or meninges. Alterations in imaging studies are consistent but nonspecific for diagnostic and are usually accompanied by alterations in the electroencephalogram (EEG) and cerebrospinal fluid (CSF). We present a clinical case of probable VPSNC based on clinical presentation and findings on imagenological studies suggestive vasculitis, along with alterations in CSF and EEG. Treatment was based on Corticosteroids and immunosuppressive agents with poor response and evolution.

Vasculite Primária do Sistema Nervoso Central (VPSNC) refere-se a um grupo de doenças que resultam de inflamação e destruição dos vasos sanguíneos na medula espinal, o sector venosa arterial cerebral e meninges, ambos. A apresentação é heterogênea e não muito sistematizável. O diagnóstico é estabelecido com um quadro clínico compatível, uma angiografia que evidencia vasculite e / oubiópsia do parênquima cerebral ou meninges. Alterações nos estudos de imagemsão constantes, mas não específica para o diagnóstico e são normalmente acompanhadas por alterações no líquido cefalorraquidiano (LCR) e eletroencefalograma (EEG) descrevem um paciente com VPSNC provável com base em um quadro clínico, achados de imagem sugestivos compatíveis, em conjunto com alterações no CSF e EEG. O tratamento foi realizado com base em corticosteróides e imunos supressores compouca resposta e evolução.

Investigation on the nasal airflow characteristics of anterior nasal cavity stenosis

We used a computational fluid dynamics (CFD) model to study the inspiratory airflow profiles of patients with anterior nasal cavity stenosis who underwent curative surgery, by comparing pre- and postoperative airflow characteristics. Twenty patients with severe anterior nasal cavity stenosis, including one case of bilateral stenosis, underwent computed tomography (CT) scans for CFD modelling. The pre- and postoperative airflow characteristics of the nasal cavity were simulated and analyzed. The narrowest area of the nasal cavity in all 20 patients was located within the nasal valve area, and the mean cross-sectional area increased from 0.39 cm2 preoperative to 0.78 cm2 postoperative (P<0.01). Meanwhile, the mean airflow velocity in the nasal valve area decreased from 6.19 m/s to 2.88 m/s (P<0.01). Surgical restoration of the nasal symmetry in the bilateral nasal cavity reduced nasal resistance in the narrow sides from 0.24 Pa.s/mL to 0.11 Pa.s/mL (P<0.01). Numerical simulation of the nasal cavity in patients with anterior nasal cavity stenosis revealed structural changes and the resultant patterns of nasal airflow. Surgery achieved balanced bilateral nasal ventilation and decreased nasal resistance in the narrow region of the nasal cavity. The correction of nasal valve stenosis is not only indispensable for reducing nasal resistance, but also the key to obtain satisfactory curative effect.

Tratamiento quirúrgico de la estenosis lumbar degenerativa del adulto mayor según la imagenología / Traitement chirurgical de la sténose lombaire dégénérative chez les personnes âgées, d'après les résultats d'imagerie / Surgical treatment of degenerative lumbar stenosis in elderly patients based on imaging findings

Introducción: la estenosis lumbar degenerativa en pacientes adultos mayores constituye un grave problema de salud actual. Objetivo: demostrar la validez del tratamiento quirúrgico de la estenosis lumbar degenerativa del adulto mayor cuando la decisión del tipo de técnica a realizar se determina por los hallazgos imagenológicos por rayos X y resonancia magnética. Métodos: se estudiaron 102 pacientes mayores de 65 años, atendidos en el Centro de investigaciones en Longevidad, Envejecimiento y Salud, entre el 1 de enero de 2002 y el 31 de diciembre de 2008, ambos incluidos. Las variables estudiadas fueron: edad, sexo, localización, principales condiciones comorbidas, clasificación por rayos X y por resonancia magnética. La decisión de la técnica quirúrgica empleada estuvo en relación con los hallazgos imagenológicos, se empleó el índice de Oswestry y la escala visual analógica de dolor como instrumentos evaluadores. Las técnicas quirúrgicas fueron: liberación simple, recalibraje y laminectomía, instrumentación y fusión. Resultados: hubo predominio del sexo masculino, entre 65 y 70 años de edad con localización en dos niveles vertebrales y condiciones comorbidas cardiovasculares, el mayor número de casos se encontró en los grados 3 y 4 de Kellgren y IV y V de Pfirrmann. El índice de Oswestry y la escala visual analógica del dolor mostraron significativa mejoría a los dos años y se obtuvo el 88 por ciento de buenos resultados. Conclusiones: determinar la técnica quirúrgica a emplear según los hallazgos imagenológicos, permite obtener buenos resultados en altos porcentajes de pacientes intervenidos. La relación imagenología/técnica quirúrgica, es adecuada(AU)

Introduction: degenerative lumbar stenosis in elderly patients is a serious health problem. Objective: demonstrate the validity of the surgical treatment of degenerative lumbar stenosis in elderly patients when decision on what technique to apply is based on X-ray and magnetic resonance imaging findings. Methods: a study was conducted of 102 patients aged over 65 cared for at the Center for Research on Longevity, Aging and Health from 1 January 2002 to 31 December 2008. The variables considered were age, sex, location, main comorbid conditions, and classification based on X-ray and magnetic resonance imaging. Decision on the surgical technique to apply was based on imaging findings. The Oswestry index and the Pain Visual Analog Scale were used as evaluation instruments. The surgical techniques applied were simple release, recalibration and laminectomy, instrumentation and fusion. Results: there was a predominance of male patients in the 65-70 age group with location at two vertebral levels and cardiovascular comorbid conditions. The largest number of cases were classed as degrees 3 and 4 on Kellgren's scale, and IV and V on Pfirrmann's. The Oswestry index and the Pain Visual Analog Scale showed significant improvement at two years, and 88 percent of the results obtained were good. Conclusions: determination of the surgical technique to apply based on imaging findings makes it possible to obtain good results in a high percentage of the patients undergoing surgery. There is an appropriate relationship between imaging findings and surgical technique(AU)

Introduction: de nos jours, la sténose lombaire dégénérative chez des patients âgés constitue un problème grave de santé. Objectif: le but de cette étude es de démontrer la validité du traitement chirurgical de la sténose lombaire dégénérative chez les personnes âgées lorsque la technique à utiliser est décidée par imagerie (rayons-X et résonance magnétique). Méthodes: cent-deux patients âgés de 65 ans ont été étudiés et traités au Centre de recherches de la longévité, le vieillissement et la santé entre le 1e janvier 2002 et le 31 décembre 2008. Les variables étudiées ont compris l'âge, le sexe, la localisation, les principales conditions de comorbidité, et la classification par rayons-X et résonance magnétique. Le choix de la technique chirurgicale à utiliser a été en rapport avec les résultats d'imagerie, et les instruments d'évaluation ont compris l'indice d'Oswestry et l'échelle visuelle analogique de la douleur. Les techniques chirurgicales ont compris la libération simple, le recalibrage et la laminectomie, l'instrumentation et la fusion. Résultats: les patients du sexe masculin, âgés de 65 - 70 ans, avec une sténose localisée à deux niveaux vertébraux, une comorbidité cardiovasculaire, en grades 3 et 4 de Kellgren, et IV et V de Pfirmann en majorité, ont été les plus souvent touchés. L'indice d'Oswestry et l'échelle visuelle analogique de la douleur ont montré une amélioration significative en deux ans, et la plupart de cas a eu de bons résultats (88 pourcent). Conclusions: le choix de la technique chirurgicale selon les résultats d'imagerie permet d'atteindre de bons résultats dans un grand nombre de patients opérés. Le rapport entre l'imagerie et la technique chirurgicale est effectivement approprié(AU)

Diagnosis of Proximal Biliary Strictures: Role of serum bilirubin, alkaline phosphatase and abdominal ultrasound

To determine the role of serum bilirubin, alkaline phosphatase and abdominal ultrasound in the diagnosis of proximal biliary strictures. Cross sectional Place and Duration: Surgical Unit IV, Civil Hospital Karachi, from Jan 2007 to August 2008. A total of 407 patients with obstruction of the biliary tree on ultrasound and raised serum levels of alkaline phosphatase had ERCP performed on them. Serum bilirubin and alkaline phosphatase levels were recorded prior to the procedure. A total of 75 patients with strictures on ERCP were included in the study. Jaundice and abdominal pain were present in 68 [90.6%] and 41 [54.6%] of the total 75 patients respectively. A statistically significant difference was present in the mean bilirubin levels of different stricture groups [p=.024]]. Receiver Operator Curve analysis of serum bilirubin level of 19.2gm/dl showed a sensitivity of 43% and specificity of 77% with area under curve= 0.6. Abdominal ultrasound was found to be 31.8% sensitive and 79.2% specific in predicting the presence of proximal biliary strictures. Serum levels of total bilirubin and alkaline phosphatase and abdominal ultrasound have minor role in the diagnosis of proximal biliary strictures

Correlation of lipid profile and carotid artery plaque as detected by Doppler ultrasound in ischaemic stroke patients--A hospital-based study.

Stroke is one of the leading causes of morbidity and mortality all over the world. Carotid plaque formation and intima media thickness can be a predictor of ischaemic stroke. In this regard studies from our country, are few and far between. This is a small hospital-based study to look in to this matter. We have assessed the intima media thickness of the common carotid as well as the internal and external carotid arteries by the ultrasound method. The lipid profiles were estimated and correlated with the intima media thickness. Results indicate that in the common as well as in the internal and external carotid arteries, the intima media thickness is a good predictor of ischaemic stroke. This thickness is also well correlated with the lipid levels in blood. Hence this non-invasive method can be used successfully to identify the high risk patients, prone to develop stroke.

Estenose bilateral de seio venoso transverso associado a papiledema: relato de caso / Bilateral transverse venous sinus stenosis associated with papilledema: case report

Relatar um caso de estenose bilateral de seio venoso transverso cursando com papiledema e discutir evolução clínica, alterações fundoscópicas, diagnóstico diferencial com pseudotumor cerebral e descrever nova proposta terapêutica (por meio do uso de "stent"). RELATO DE CASO: Paciente de 39 anos, do sexo feminino, portador de estenose bilateral de seio transverso associada a papiledema, apresentando baixa de acuidade visual, restrição de campos visuais e cefaléia. O diagnóstico foi confirmado por meio de angiografia cerebral. Instituiu-se terapêutica intervencionista, por meio do implante de "stent" via angiográfica cerebral, com remissão dos sinais e sintomas aos exames oftalmológico e neurológico. DISCUSSÃO: Neste trabalho descrevemos os achados radiológicos e oftalmológicos da estenose bilateral do seio transverso e apresentamos nova terapêutica por meio de implante de "stent" via angiografia cerebral, com melhora documentada dos níveis pressóricos intracranianos, dos achados campimétricos visuais e dados do exame oftalmológico após o procedimento. Não há relato na literatura internacional de estenose bilateral do seio venoso associado a papiledema. Por se tratar do primeiro relato na literatura, os achados descritos sugerem fortemente que esta associação possa ser parte de uma nova entidade nosológica.