Apport de l'imagerie dans les brèches ostéoméningées de l'étage antérieur de la base du crâne / Role of imaging in the osteo-dural defects of the anterior base of the skull

Décrire les caractéristiques radiologiques de la brèche ostéo-méningée (BOM) de l'étage antérieur de la base du crâne. Méthodes: Etude rétrospective, descriptive, monocentrique, colligeant les patients hospitalisés pour prise en charge d'une brèche ostéoméningée (BOM) et explorés par le couple TDM/IRM, du 1er janvier 2012 au 31 Décembre 2021. Résultats: Ont été retenu 23 patients avec un âge moyen de 48 ans et un sex ratio de 0,3. Des antécédents de chirurgie nasosinusienne ou de neurochirurgie ont été notés chez deux patients (9 %). Treize patients (57 %) étaient victimes de traumatisme crânien. Une rhinoliquorrhée était retrouvée dans 21 cas (persistante dans trois cas (12%) et intermittente dans 18 cas (76%)). La découverte de la BOM a été faite suite à une méningite dans quatre cas. Le dosage de la bêta trace a confirmé la présence de LCR chez deux patients. La TDM a confirmé le diagnostic chez 12 patients dont deux patients qui ne présentaient pas de rhinoliquorrhée à l'examen clinique. Elle a aussi permis d'établir le bilan topographique dans ces cas. Elle a suspecté une BOM dans 9 cas et était négative dans deux cas. L'IRM a confirmé le diagnostic dans tous les cas et a permis de: localiser le siège de la fuite de LCR dans les brèches multiples, confirmer le diagnostic deméningoencéphalocèle (trois cas) et objectiver des signes indirects d'hypertension intra-crânienne (HTIC) (quatre cas). Conclusion: L'imagerie en coupes permet de localiser la BOM mais nécessite souvent, une approche par étapes et son interprétation exige une connaissance approfondie de l'anatomie normale et des variantes de la base du crâne. Mots-clés: Liquorrhée, Brèche Ostéoméningée, Chirurgie, TDM, IRM

Anomalías congénitas nasales: Estudio retrospectivo y revisión de la literatura / Congenital Anomalies of the Nose: retrospective study and literature review

INTRODUCTION. Congenital malformations occur in 1 in 5,000 to 40,000 live births. They present as a morphological abnormality of the nasal pyramid or as a picture of moderate to severe nasal obstruction. OBJECTIVE. Evaluate the etiology, associated comorbidities, management and mortality of congenital malformations of the nose in neonates and infants. MATERIALS AND METHODS. Cross-sectional retrospective descriptive study, 105 medical records were reviewed, of which 26 corresponded to patients with congenital nasal malformations, at the Carlos Andrade Marín Specialty Hospital in Quito - Ecuador, between January 2009 and May 2022; the tabulation and analysis of data was carried out in the Excel program. The patients were classified according to Losee et al. in 4 types: hypoplasia, hyperplasia, clefts and tumors RESULTS. Nasal anomalies occurred in males in 73.07%, all presented nasal obstruction, the diagnosis was made by flexible nasofibroscopy; in 42.3% of the cases, the evaluation was complemented with computed tomography. The most frequent congenital pathology was stenosis - choanal atresia with 53.8%, followed by craniofacial clefts with 15.39%. In 42.3% of the cases there was an association with genetic syndromes, neurological, ocular and intestinal pathology. 69.23% of the patients received clinical treatment with nasal lavages, nasal corticosteroids and positive pressure by cannula, while 30.77% were resolved surgically, being: 2 unilateral choanal atresia, 1 middle fossa stenosis, 4 cleft lip and palate and 1 encephalocele. Mortality was 7.69% CONCLUSION. Hypoplasias and clefts are the predominant pathologies, flexible nasal nasofibroscopy is the diagnostic test of choice. The clinical treatment was successful in the initial management in 69.23% of cases. Surgery was performed for the repair of facial clefts; being the hypoplasias or masses managed by endoscopic approach.

INTRODUCCIÓN. Las malformaciones congénitas se presentan en 1 de cada 5.000 a 40.000 nacidos vivos. Se presentan como una anomalía morfológica de la pirámide nasal o como un cuadro de obstrucción nasal moderada a severo. OBJETIVO. Evaluar la etiología, comorbilidades asociadas, manejo y mortalidad de las malformaciones congénitas de nariz en neonatos y lactantes. MATERIALES Y MÉTODOS. Estudio descriptivo retrospectivo transversal, se revisaron 105 historias clínicas de las cuales 26 correspondieron a pacientes con malformaciones congénitas nasales, en el Hospital de Especialidades Carlos Andrade Marín de Quito - Ecuador, entre enero de 2009 a mayo de 2022; la tabulación y análisis de datos se realizó en el programa Excel. Los pacientes fueron clasificados según Losee et al. en 4 tipos: hipoplasia, hiperplasia, hendiduras y tumores. RESULTADOS. Las anomalías nasales se presentaron en el sexo masculino en el 73.07%, el motivo de consulta fue la obstrucción nasal, el diagnóstico se realizó mediante nasofibroscopía flexible; en el 42,3% de los casos se complementó la evaluación con tomografía computarizada. La patología congénita más frecuente fue la estenosis - atresia de coana con un 53,8%, seguida de hendiduras craneofaciales con un 15.39%. En el 42.3% de los casos existió asociación con síndromes genéticos, patología neurológica, ocular e intestinal. El 69,23% de los pacientes recibió tratamiento clínico con lavados nasales, corticoides por vía nasal y presión positiva por cánula, mientras que el 30,77% se resolvió quirúrgicamente, siendo: 2 atresia unilateral de coana, 1 estenosis de fosa media, 4 hendiduras labio palatinas y 1 encefalocele. La mortalidad fue del 7,69%. CONCLUSIÓN: Las hipoplasias y las hendiduras son las patologías que predominaron, la nasofibroscopia flexible nasal es el examen diagnóstico de elección. El tratamiento clínico fue exitoso en el manejo inicial en el 69,23% de casos. La cirugía se realizó para la reparación de hendiduras faciales; siendo las hipoplasias o masas manejadas por abordaje endoscópico.

Anomalías congénitas frecuentes en Uruguay entre 2011 y 2014: importancia de un registro para la evaluación de las necesidades de salud / Frequent congenital anomalies in Uruguay in 2011-2014; importance of a medical record for health assessment / Anomalias congênitas frequentes no Uruguai entre 2011-2014, importância de um registro para avaliação das necessidades de saúde

Introducción: las anomalías congénitas (AC) son un problema de salud pública con impacto en la infancia, la mortalidad infantil (MI) y la discapacidad. En Uruguay, así como en otros países desarrollados, las AC y la prematuridad son las principales causas de MI. Objetivos: el objetivo de este trabajo fue analizar las anomalías más frecuentes en el país entre 2011 y 2014, y evaluar los factores de riesgo. Material y método: los datos se obtuvieron del Registro Nacional de Defectos Congénitos y Enfermedades Raras y Estadísticas Vitales del Ministerio de Salud Pública. Resultados: se determinó que prácticamente la mitad de los casos fueron: cardiopatías congénitas, síndrome de Down y defectos del tubo neural (anencefalia, encefalocele y mielomeningocele), representando 0,42% (812/191.820) y 2,85% (38/1334) de los nacidos vivos (NV) y de los óbitos fetales respectivamente. Las prevalencias por 10.000 NV y OF fueron: 38,52 y 149,93 para cardiopatías congénitas; 3,6 y 7,5 para síndrome de Down; 2,1 y 127,4 para defectos del tubo neural. La edad materna avanzada fue el principal factor de riesgo de síndrome de Down. Los factores de riesgo observados en las tres anomalías seleccionadas fueron: prematurez, depresión neonatal y bajo peso al nacer. Conclusiones: las anomalías congénitas en general y las seleccionadas en este trabajo en particular, constituyen una causa relevante de morbimortalidad en el período neonatal e infantil, plausibles de prevención. El diagnóstico temprano es fundamental para planificar servicios de salud especializados. Los resultados aquí presentados se pueden utilizar como línea de base para medir el impacto de las acciones de salud a nivel nacional.

Introduction: congenital anomalies (CA) are a public health problem with an impact on childhood, infant mortality (IM) and disability. In Uruguay, as well as in other developed countries, CA and prematurity are the main causes of IM. Objectives: analyze the most frequent anomalies in the country in 2011-2014 and evaluate risk factors. Material and methods: the data were obtained from the National Record of Congenital Defects and Rare Diseases and health statistics from the Ministry of Public Health. Results: it was determined that practically half of the cases were: congenital heart disease, Down syndrome and Neural Tube Defects (anencephaly, encephalocele and myelomeningocele), accounting for 0.42% (812/191,820) and 2.85% (38/1334) of live births and stillbirths respectively. The prevalence per 10,000 NV and stillbirths were: 38.52 and 149.93 for Congenital Heart Disease; 3.6 and 7.5 for Down Syndrome; 2.1 and 127.4 for neural tube defects. Advanced maternal age was the main risk factor for Down syndrome. The risk factors observed in the three selected anomalies were: prematurity, neonatal depression and low birth weight. Conclusions: congenital anomalies in general, and those selected in this study in particular, are a relevant cause of morbidity and mortality in newborns and infants, likely to be prevented. Early diagnosis is essential for planning specialized health services. The results presented in the present paper can be used as a baseline to measure the impact of health actions at national level.

Introdução: as anomalias congênitas (AC) são um problema de saúde pública com impacto na infância, na mortalidade infantil (MI) e na deficiência. No Uruguai, assim como em outros países desenvolvidos as AC e prematuridade são as principais causas de MI. Objetivos: analisar as anomalias mais frequentes no país, entre 2011-2014 e avaliar os fatores de risco. Material e métodos: os dados foram obtidos do Registro Nacional de Defeitos Congênitos e Doenças Raras e estatísticas vitais do Ministério da Saúde Pública. Resultados: determinou-se que praticamente a metade dos casos eram: cardiopatias congênitas, Síndrome de Down e Defeitos do tubo neural (anencefalia, encefalocele e mielomeningocele), representando 0,42% (812/191.820) e 2,85% (38/1334) de nascidos vivos-NV e natimortos, respectivamente. As prevalências por 10.000 NV e natimortos foram: 38,52 e 149,93 para Cardiopatia Congênita; 3,6 e 7,5 para Síndrome de Down; 2,1 e 127,4 para defeitos do tubo neural. A idade materna avançada foi o principal fator de risco para a síndrome de Down. Os fatores de risco observados nas três anomalias selecionadas foram: prematuridade, depressão neonatal e baixo peso ao nascer. Conclusões: as anomalias congênitas em geral e as selecionadas neste estudo em particular, constituem causa relevante de morbimortalidade no período neonatal e infantil, possível de ser prevenida. O diagnóstico precoce é fundamental para o planejamento de serviços de saúde especializados. Os resultados aqui apresentados podem ser usados como base para medir o impacto das ações de saúde realizadas a nível nacional.

Encefalocele en paciente con síndrome de Hurler: descripción y revisión de alternativas terapéuticas / Encephalocele in a patient with Hurler syndrome: description and therapeutic alternatives review

Introducción: el síndrome de Hurler es la forma más grave de la mucopolisacaridosis I. El depósito de heparán y dermatán sulfato en las meninges favorece el desarrollo de hidrocefalia y, a su vez, de meningoencefaloceles. Caso clínico: se describe el caso de una paciente de 23 años con este síndrome y un encefalocele nasosinusal intervenido mediante cirugía endoscópica nasosinusal y posterior refuerzo mediante un colgajo pericraneal. Discusión: el abordaje endoscópico de los meningoencefaloceles nasales ha crecido notablemente en los últimos años debido a una tasa de éxito elevada tanto para la eliminación de dicha lesión, como para el adecuado control de la fístula de líquido cefalorraquídeo consecuente. Conclusiones: actualmente, la cirugía endoscópica nasosinusal es una herramienta muy útil para el tratamiento de patologías de base de cráneo y reduce la morbilidad causada por el abordaje transcraneal.

Introduction: Hurler syndrome is the most severe version of mucopolysaccharidosis I. The storage of dermatan and heparin sulfate in meninges allows the development of hydrocephalus and meningoencephaloceles. Case report: We report a 23-year-old female with this syndrome and a sinonasal encephalocele operated by endoscopic sinonasal surgery and subsequent pericranial flap as support. Discussion: Endoscopic sinonasal surgery has grown in last years in relation with treatment of sinonasal meningoencephaloceles due to a high rate of success removing the lesion and closing the subsequent cerebrospinal fluid fistula. Conclusion: Currently, the endoscopic sinonasal surgery has become a useful tool in the management of skull base pathologies, and reduces the morbidity due to a transcranial approach.

Encefalocele occipital gigante sin complicaciones neonatales inmediatas / Giant occipital encephalocele without immediate neonatal complications

RESUMEN El encefalocele se produce por un defecto del tubo neural debido a una insuficiencia de la superficie ectodérmica de separarse del neuroectodermo. Este tipo de defecto incluye una etiología multifactorial. Se presentó el caso de una paciente de 43 años, raza indígena, con historia obstétrica de 14 embarazos (13 partos y 1 aborto espontáneo). La paciente acudió con dolor al servicio de urgencia del Centro de atención integral materno-infantil de Ixchiguán, San Marcos, Guatemala (fue atendida como parte de la colaboración médica cubana). En la cesárea se obtuvo un recién nacido con encefalocele occipital, sin complicaciones neonatales inmediatas. El pronóstico para los pacientes con este tipo de malformación congénita es variable, pues depende, en primer lugar, de la localización y el tamaño, del tipo de tejido cerebral herniado, y por otro lado, del número, tipo y gravedad de las malformaciones asociadas.

ABSTRACT Encephalocele occurs from a neural tube defect due to a failure of the ectodermal surface to separate from the neuroectoderm. This type of defect includes a multifactorial etiology. We present a 43-year-old female indigenous patient, with an obstetric history of 14 pregnancies (13 deliveries and 1 spontaneous abortion). She came with pain to the emergency service of the Comprehensive Maternal and Child Care Center of Ixchiguán, San Marcos, Guatemala and was treated as part of the Cuban medical collaboration. A neonate with occipital encephalocele, without immediate neonatal complications, was born by caesarean section. Patient prognosis with this type of congenital malformation is variable, since it depends, firstly, on its location and size, type of herniated brain tissue, and on the other hand, on the number, type and severity of the associated malformations.

Caso Clínico: Meningohidroencefalocele Gigante / Case Report: Giant Meningohydroencephalocele

INTRODUCCIÓN: Los encefaloceles son una anomalía congénita rara, que afecta a 1 de cada 5 000 nacidos vivos. Un meningohidroencefalocele es la herniación de meninges, ventrículos y parte del encéfalo a través de un defecto congénito en el cráneo. El pronóstico de los recién nacidos con encefalocele depende de varios factores. La corta edad y las anomalías congénitas asociadas en estos pacientes plantean desafíos en el diagnóstico y manejo. CASO CLÍNICO: Recién nacido masculino, nacido a las 37 semanas de gestación, por cesárea de emergencia por parto en fase latente, presentado una masa de gran tamaño, sobre la región parieto-occipital, de bordes lobulados, irregulares, cubierta en parte por cuero cabelludo, y en otras zonas de un tono rojo violáceo. El neonato fue poco reactivo, con tono disminuido, llanto ausente. EVOLUCIÓN: Tras valoración del caso por especialistas, debido al importante defecto de la calota craneal y las anomalías estructurales cerebrales, el paciente no fue candidato para tratamiento quirúrgico. Neonato fue dado de alta con expectativa de vida reducida, para recibir cuidados en el hogar. CONCLUSIÓN: El meningohidroencefalocele gigante es un defecto del tubo neural poco frecuente. El manejo de las encefaloceles puede ser complicado y debe ser individualizado y dependerá de las estructuras anatómicas comprometidas. Si bien en este caso el pronóstico fue malo, siempre es necesario el trabajo multidisciplinario para un adecuado manejo.

BACKGROUND: Encephaloceles are a rare congenital anomaly, affecting 1 in 5 000 live births.A meningohydroencephalocele is the herniation of the meninges, ventricles, and part of the brain through a congenital defect in the skull. The prognosis of newborns depends on several factors. The young age and the associated congenital anomalies in these patients contribute to the diagnostic and treatment challenge. CASE REPORT: Male newborn, born at 37 weeks of gestation, by emergency C-section due to latent labor, presenting a large mass over the parieto-occipital region, with lobed, irregular edges, partially covered by the scalp, and in other areas a purplish red tone. The newborn was no very reactive, had decreased tone, absent crying. EVOLUTION: After specialist's evaluation, due to the significant defect in the cranial shell and the structural brain abnormalities, the patient was not a candidate for surgical treatment. The newborn was discharged with a reduced life expectancy, to receive care at home. CONCLUSION: giant meningohydroencephalocele is a rare neural tube defect. The management of encephaloceles can be complicated and must be individualized, and will depend on the anatomical structures involved. Although in this case the prognosis was bad, multidisciplinary work is always necessary for proper management.

The effect of hematoma puncture drainage before decompressive craniectomy on the prognosis of hypertensive intracerebral hemorrhage with cerebral hernia at a high altitude / 中华创伤杂志(英文版)

PURPOSE@#Rapid decompressive craniectomy (DC) was the most effective method for the treatment of hypertensive intracerebral hemorrhage (HICH) with cerebral hernia, but the mortality and disability rate is still high. We suspected that hematoma puncture drainage (PD) + DC may improve the therapeutic effect and thus compared the combined surgery with DC alone.@*METHODS@#From December 2013 to July 2019, patients with HICH from Linzhi, Tibet and Honghe, Yunnan Province were retrospectively analyzed. The selection criteria were as follows: (1) altitude ≥1500 m; (2) HICH patients with cerebral hernia; (3) Glascow coma scale score of 4-8 and time from onset to admission ≤3 h; (4) good liver and kidney function; and (5) complete case data. The included patients were divided into DC group and PD + DC group. The patients were followed up for 6 months. The outcome was assessed by Glasgow outcome scale (GOS) score, Kaplan-Meier survival curve and correlation between time from admission to operation and prognosis. A good outcome was defined as independent (GOS score, 4-5) and poor outcome defined as dependent (GOS score, 3-1). All data analyses were performed using SPSS 19, and comparison between two groups was conducted using separate t-tests or Chi-square tests.@*RESULTS@#A total of 65 patients was included. The age ranged 34-90 years (mean, 63.00 ± 14.04 years). Among them, 31 patients had the operation of PD + DC, whereas 34 patients underwent DC. The two groups had no significant difference in the basic characteristics. After 6 months of follow-up, in the PD + DC group there were 8 death, 4 vegetative state, 4 severe disability (GOS score 1-3, poor outcome 51.6 %); 8 moderate disability, and 7 good recovery (GOS score 4-5, good outcome 48.4 %); while in the DC group the result was 15 death, 6 vegetative state, 5 severe disability (poor outcome 76.5 %), 4 moderate disability and 4 good recovery (good outcome 23.5 %). The GOS score and good outcome were significantly less in DC group than in PD + DC group (Z = -1.993, p = 0.046; χ@*CONCLUSION@#PD + DC treatment can improve the good outcomes better than DC treatment for HICH with cerebral hernia at a high altitude.

Genetic testing and prenatal diagnosis for a Chinese pedigree affected with Meckel-Gruber syndrome / 中华医学遗传学杂志

OBJECTIVE@#To carry out genetic testing and prenatal diagnosis for a Chinese couple whom had conceived two fetuses featuring multiple malformations including polycystic kidney, polydactyly and encephalocele.@*METHODS@#Following elective abortion, the fetus from the second pregnancy was subjected to whole exome sequencing. Suspected pathogenic variants were verified by Sanger sequencing of the fetus and its parents.@*RESULTS@#The fetus was found to harbor compound heterozygous variants of the CEP290 gene, namely c.2743G>T (p.E915X) and c.2587-2A>T, which were respectively inherited from its father and mother. The same variants were not detected among 100 healthy controls nor reported previously. Bioinformatic analysis suggested both variants to be deleterious. The fetus was diagnosed with Meckel-Gruber syndrome. Prenatal diagnosis for the couple during their next pregnancy suggested that the fetus did not carry the above pathogenic variants.@*CONCLUSION@#The compound heterozygous variants of the CEP290 gene probably underlay the pathogenesis of Meckel-Gruber syndrome in the second fetus. Above finding has provided a basis for genetic counseling and prenatal diagnosis for the couple, and also enriched the mutational spectrum of the CEP290 gene.

Adult Nasoethmoidal Encephalocele Corrected by Supraorbitary Approach: Case Report and Literature Review

Encephalocele is a protrusion of the central nervous system elements through a defect in the dura mater and in the cranium. The prevalence of encephalocele ranges from 0.08 to 0.5 per 1,000 births. The posterior encephaloceles are more common in North America and Europe, while frontal defect is frequently found in Asia. The present paper describes a 26-year-old male patient presenting with cerebrospinal fluid leak and meningitis symptoms. He was diagnosed with congenital nasoethmoidal encephalocele and treated surgically using a supraorbital approach without complications.

Large Vertex Epidural Hematoma: Case Report and Review of Surgical Approaches

Vertex epidural hematomas (VEHs) are a special clinical entity due to their clinical presentation, vascular etiology and options of surgical approach. The clinical suspicion involves recognizing the mechanism of the injury and the correct visualization of the hematoma in computed tomography (CT) coronal sequences. In the present article, we describe a case of a very large (146 mL) VEH with central brain herniation, and provide a technical note on the surgical planning and treatment. A 34-year-old male patient was admitted to the hospital after an injury on the left superior parietal region. The Glasgow coma scale score was 6, and the left pupil of the patient was dilated. The CT scan showed a large epidural hematoma on the vertex between the coronal e lambdoid sutures, and a fracture over the sagittal suture. During the surgery, multiple burr holes were made laterally to the sagittal suture, and after inspection and no visualization of bleeding in the superior sagittal sinus (SSS), we performed a standard biparietal craniotomy. The patient was discharged three days after the surgery without any deficits. Currently, with the improvement in imaging modalities,more cases of VEH are being identified. Identifying the etiology prior to the craniotomy is challenging in severe cases. Tears in the SSS can bleed profusely, and they demand strategies during the craniotomy.With multiple burr holes parallel to the sagittal suture, we can visualize whether there is bleeding in the SSS and design a craniotomy with or without a central osseous bridge to anchor the dura. Neurosurgeons must be prepared to plan a surgical strategy in cases of large VEHs. Due to its rare frequency and bleeding risks, VEHs are considered challenging.

Dermoid cyst with cerebellar meningoencephalocele at different locations accompanied by posterior fossa abnormalities: case report

ABSTRACT CONTEXT: Dermoid cysts are well-defined cysts containing sebaceous glands and dermal structures. In the literature, dermoid cysts and associated closure defects have been described in the same locations. CASE REPORT: In this case, a dermoid cyst was found at the base of the mouth with a coexisting closure defect in the occipital calvarium. Additional abnormalities were also observed, including posterior myeloschisis, right cerebellar dysgenesis, vermian hypogenesis and posterior fusion of the second and third vertebrae. The finding of a dermoid cyst located at the base of the mouth is discussed here, with additional imaging findings. CONCLUSION: Dermoid cysts in the head and neck region may be accompanied by posterior fossa abnormalities.

Tomografía computada y resonancia magnética de variantes normales/congénitas de apariencia quística y presentación frecuente en el encéfalo / CT and MRI of Normal/Congenital Variables with Cystic Appearance and Frequent Presence in the Brain

La presentación de una imagen de apariencia quística durante el estudio del encéfalo constituye un hallazgo incidental cada vez más frecuente, pudiendo encontrarse en el espacio extra o intraaxial. Las mismas pueden ser de naturaleza congénita o adquirida, benigna o maligna, ocupantes de espacio con desplazamiento de la línea media o simplemente presentarse sin efecto compresivo alguno. De localización supra o infratentorial, esas imágenes constituyen un desafío diagnóstico, siendo imprescindible su reconocimiento para no solicitar estudios o tratamientos innecesarios. Valoraremos las imágenes de apariencia quística más frecuentes empleando tomografía computada o imágenes de resonancia magnética.

Presence of a cystic formation in brain examination is frequently an incidental finding. They can be intra or extra-axial in location, congenital or acquired, benign or malignant with or without mass effect. Intracranial cysts can be a diagnostic challenge and we should know them to avoid unnecessary exams or treatment. We will analyze the most common cystic formations seen in computed tomography and magnetic resonance.

Primary Amebic Meningoencephalitis: A Case Report

Primary amebic encephalitis (PAM) is a devastating central nervous system infection caused by Naegleria fowleri, a free-living amoeba, which can survive in soil and warm fresh water. Here, a 43-year-old healthy male was exposed to warm freshwater 5 days before the symptom onset. He rapidly developed severe cerebral edema before the diagnosis of PAM and was treated with intravenous conventional amphotericin B while died of terminal cerebral hernia finally. Comparing the patients with PAM who has similar clinical symptoms to those with other common types of meningoencephalitis, this infection is probably curable if treated early and aggressively. PAM should be considered in the differential diagnosis of purulent meningoencephalitis, especially in patients with recent freshwater-related activities during the hot season.

Tumores nasales congénitos de la linea media / Congenital nasal tumors of the midline in ENT

Las masas nasales congénitas de la línea media se presentan con una frecuencia muy baja ­1/20.000 a 1/40.000 nacidos vivos­. Se trata de hallazgos asintomáticos en el recién nacido y son resultado de anomalías congénitas del desarrollo embrionario, que suelen aparecer como masas en la línea media nasal en un punto cualquiera entre glabela y columela. Estas tumoraciones presentan un riesgo elevado de extensión al sistema nervioso central, lo que es especialmente importante tener en cuenta para prevenir consecuencias tales como la fístula de líquido cefalorraquídeo y/o la aparición de meningitis recidivante. Existen gran cantidad de tumores nasales de la línea media que aparecen en el recién nacido o en el lactante y que constituyen diagnósticos diferenciales de las lesiones congénitas antes descriptas. Describiremos brevemente los más frecuentes según nuestra experiencia. AU

Congenital nasal masses of the midline are very rare ­ 1/20,000 to 1/40,000 live births ­. Nasal tumors are asymptomatic findings in the neonate and are caused by congenital abnormalities during fetal development, usually appearing at the nasal midline between the glabella and columella. These tumors are associated with a high risk of extension to the central nervous system; therefore, it is especially important to prevent the development of a cerebrospinal fluid fistula and/or recurrent meningitis. There is a large number of nasal tumors of the midline in neonates or infants in the differential diagnosis of the above-described congenital lesion. Here we briefly describe the most common nasal tumors seen at our department. AU

Post-operative temporal lobe encephalocele

@#This 24-year-old woman presented to ENT outpatients with an enlarging swelling in the right external auditory canal. A radical mastoidectomy for chronic suppurative otitis media with cholesteatoma had previously been undertaken at another institution. On clinical examination there was an otologic mass that was tender on probing. High resolution imaging of the temporal bones and a subsequent MRI brain confirmed the mass was a temporal lobe encephalocele. A temporal lobe encephalocele is where a segment of the temporal lobe invaginates through a defect in the tegmen tympani. The brain is separated from the middle ear and mastoid process by an exceptionally thin layer of bone – the tegmen tympani. Damage to the tegmen compromises the barrier with the brain and may occur for a number of reasons. This includes congenital, traumatic, post-infectious, malignant invasion, post-radiation therapy and post-surgical causes.1 When this occurs the brain may extrude through the defect resulting in a temporal lobe encephalocele. A bony defect alone, whatever the cause, is insufficient to always result in an encephalocele. Even with dehiscence of the tegmen the dura is capable of supporting the brain issue without herniation. Only when the integrity of the dura is compromised does an encephalocele occur.2 This may be due to the underlying disease process (such as cholesteatoma causing an intracranial abscess) or both purposeful (opening dura to drain an adjacent intracranial abscess) /non-purposeful surgical intervention. Mainstream microsurgical techniques however have lowered the incidence of dural violation.3 Historically, infection was a major cause, but with the ready availability of antibiotics and prompt management, the key contemporary cause is iatrogenic, following mastoid surgery. However, the overall incidence is uncommon following otologic surgery. In a review of 25 years of middle ear/mastoid encephalocele cases 77% were identified to be iatrogenic in origin.4 This patient presented with the finding of a mass observed in the external auditory canal. Less common findings at attendance include tympanic perforation, cholesteatoma, otorrhoea and meningitis.4 The key to diagnosis hinges on cross-sectional imaging: combined imaging with CT to assess the osseous structures and MRI for soft tissue review. The high-resolution CT (HRCT) of the temporal bones illustrates a large defect in the right tegmen tympani with a large soft tissue lesion occupying the post-surgical mastoid cavity abutting the tympanic membrane. (Figures 1A, B) The defect of 15mm in the tegmen was more than double the average of 7.2mm reported elsewhere.4 The MRI confirms the defect in the tegmen with the protrusion of a knuckle of the right temporal lobe and its overlying meninges through the defect into the mastoid cavity. The dumb-bell appearance is typical with the narrower neck at the site of the tegmental dehiscence. The extruded brain occupies the post-operative middle ear cavity. (Figures 2 A, B and C) The defect size and volume of herniated brain can be accurately assessed, both of which may be key determinates of the type of surgical procedure. Revision mastoidectomy with repair of the tegmen defect and dural integrity using a combined intracranial-mastoid approach is planned as a joint case with neurosurgical colleagues.

Modified two flap palatoplasty in asymptomatic transsphenoidal encephalocele: a case report

About one-third of patients with transsphenoidal basal encephaloceles have associated congenital anomalies, including cleft palate. Moreover, they are often plagued by symptomatic exacerbations in the form of upper respiratory obstructions, cerebrospinal fluid leaks, meningitis, etc., with few patients being asymptomatic. We herein present a rare asymptomatic case of transsphenoidal basal encephalocele in an 18-month-old child with cleft palate and highlight a modified version of two-flap palatoplasty.

Transpalatine Route for the Correction of Transsphenoidal Encephalocele in a Child: A Case Report and Literature Review

Introduction Transsphenoidal encephalocele is a rare neural tube defect characterized by the herniation of meninges and eventually of parts of the brain through a bony defect in the sphenoid bone. The clinical presentation is variable, and surgical treatment is controversial. Case Report This report describes the case of an 8-month-old female child diagnosed with transsphenoidal encephalocele. The child presented with obstruction of the upper airways and was fed via a nasogastric tube but did not present changes in the hypothalamic-pituitary-axis. The patient underwent surgery with the transsphenoidal­transpalatine route, with an excellent outcome and without fistulas or infections. Conclusions Although transsphenoidal encephalocele is a rare congenital anomaly, the transsphenoidal­transpalatine route for the correction of this type of encephalocele is a safe option and produces a favorable outcome in pediatric patients.

Introdução A encefalocele transesfenoidal é um defeito do tubo neural raro, caracterizado por herniação de meninges e eventualmente partes do cérebro através de uma falha óssea no osso esfenoide. A apresentação clínica é variável e o tratamento cirúrgico é controverso. Relato de Caso No presente trabalho descrevemos o caso de uma criança de 8 meses, sexo feminino, com diagnóstico de encefalocele transesfenoidal. Apresentava-se com obstrução de vias aéreas superiores, alimentando-se por sonda nasoenteral e sem alterações do eixo hipotálamo-hipofisário. Tratada com cirurgia via transesfenoidal transpalatal, evoluiu com excelente resultado, sem fístulas e sem infecções. Conclusões Embora a encefalocele transesfonoidal seja uma anomalia congênita rara, a via transpalatina-transesfenoidal para correção deste tipo de encefalocele é uma opção segura e produz um resultado favorável no paciente pediátrico.

Surgical correction of grade III hypertelorism

Orbital hypertelorism is an increased distance between the bony orbits and can be caused by frontonasal malformations, craniofacial clefts, frontoethmoidal encephaloceles, glial tumors or dermoid cysts of the root of the nose, and various syndromic or chromosomal disorders. We report a series of 7 cases of hypertelorism that were treated in our hospital. The underlying causes in our series were craniofacial clefts 0 to 14 (4 cases), craniofacial clefts 1 to 12 (1 case), and frontonasal encephalocele (2 cases), all congenital. Surgical techniques used to correct the deformity were box osteotomy and medial wall osteotomy with or without calvarial and rib grafts. A few of our cases were reoperations with specific challenges.

Contralateral Superior Cerebellar Artery Syndrome: A Consequence of Brain Herniation

Vascular compromise is a well-known consequence of brain herniation syndromes. Transtentorial brain herniation most often involves posterior cerebral arteries. However, isolated involvement of contralateral superior cerebellar artery (SCA) during unilateral impending brain herniation is reported only once and we present another case of this exceedingly rare entity. A 24-year-old man was referred to us with impending herniation due to a multiloculated hydrocephalus, and during the course of illness, he developed an isolated SCA ischemia in the opposite side of the most dilated entrapped horn. In the current article we discuss the probable pathophysiologic mechanisms of this phenomenon, as well as recommending more inclusive brain studies in cases suspected of Kernohan-Woltman notch phenomenon in unilateral brain herniation. The rationale for this commentary is that contralateral SCA transient ischemia or infarct might be the underdiagnosed underlying pathomechanism of ipsilateral hemiparesis occurring in many cases of this somehow vague phenomenon.