Opciones quirúrgicas para la cobertura de defectos del cierre del tubo neural. Serie de casos / Surgical options for coverage of neural tube closure defects. Case series

Introducción. Los defectos del tubo neural (DNT) son la segunda causa más prevalente de malformaciones congénitas, solo detrás de las cardiopatías congénitas. Los DTN abiertos comprenden el 80% de estas malformaciones. El mielomeningocele es el defecto abierto más común del tubo neural. La mayoría de los defectos son lo suficientemente pequeños como para soportar un cierre por primera o por segunda intención, pero el cierre primario puede no ser posible en aproximadamente 25% de los casos, lo cual es evidente en defectos grandes. Pacientes y métodos. El trabajo a continuación tiene como objetivo mostrar la experiencia en el manejo quirúrgico posnatal a través de una serie de caso con 3 pacientes con diagnóstico de defectos de cierre del tubo neural en distintas localizaciones anatómicas sometidos a cirugía en forma conjunta entre el Servicio de Neurocirugía y Cirugía Plástica del Parque de la Salud en la ciudad de Posadas Misiones. Resultados. En nuestra serie los colgajos realizados para la cobertura de mielomeningoceles fueron eficaces, evolucionando en primera instancia con complicaciones menores. La resolución del caso de encefalocele occipital con un colgajo miocutáneo de trapecio diferido resultó ser una opción válida y confiable. Conclusión. El uso de colgajos regionales para la reconstrucción de este tipo de malformaciones congénitas es una opción válida y reproducible, con bajo porcentaje de complicaciones.

Introduction. Neural tube defects (NTD) are the second most prevalent cause of congenital malformations, only behind congenital heart defects. Open NTDs comprise 80% of these malformations. Myelomeningocele is the most common open neural tube defect. Most defects are small enough to support a first or second intention closure, but primary closure may not be possible in approximately 25% of cases, being evident in large defects. Patients and methods. The following work aims to show the experience in postnatal surgical management through a case series with 3 patients diagnosed with neural tube closure defects in different anatomical locations undergoing joint surgery between the Neurosurgery and Plastic Surgery Service of Parque de la Salud in the city of Posadas, Misiones. Results. In our series, the flaps made to cover myelomeningoceles were effective, evolving in the first instance with minor complications. Resolution of the occipital encephalocele case with a delayed trapezius myocutaneous flap proved to be a valid and reliable option. Conclusion. The use of regional flaps for the reconstruction of this type of congenital malformations is a valid and reproducible option, with a low percentage of complications.

Sphenoidal meningoencephalocele associated with CSF fistula and arteriovenous malformation Spetzler-Martin V: A case report / Meningoencefalocele esfenoidal asociado a fístula de líquido cefalorraquídeo y malformación arteriovenosa Spetzler-Martin V: reporte de caso

Cerebral Arteriovenous malformations (AVMs) are presumed congenital anomalies of the blood vessels, which can increase intracranial pressure by uncertain mechanisms. We report the rare case of a 55-year-old male patient who complained about CSF rhinorrhea. Persisting CSF leakage prompted CT, which evidenced a bone defect in the right middle cranial fossa with protruding brain tissue. The diagnosis of a sphenoidal meningoencephalocele was made. Neuroimaging evidenced an AVM Spetzler Martin V. The lesion was targeted via an endonasal approach with resection of the herniated brain tissue and closure of the bony and dural defects. The postoperative course was uneventful without recurrence of the CSF fistula. Documentation of these cases is essential to come up with standardized therapeutical protocols and follow-up. Nevertheless, conservative management of the AVM and surgical repair of the bone defects is an appropriate approach in the first instance, depending on the morphology and characterization of the AVM (AU)

Las malformaciones arteriovenosas (MAV) son consideradas anomalías congénitas de los vasos sanguíneos; estas pueden aumentar la presión intracraneal a través de mecanismos inciertos. Reportamos el caso de un hombre de 55 años con presencia de rinolicuorrea de larga data. Se realizó la toma de una TC de cráneo, evidenciando un defecto óseo en la fosa craneal media derecha con protrusión de tejido cerebral, diagnosticando un meningoencefalocele esfenoidal. Las imágenes complementarias evidenciaron una MAV Spetzler-Martin V. La lesión fue tratada con un abordaje endonasal resecando el tejido cerebral herniado con cierre de los defectos dural y óseo. En el postoperatorio no hubo recurrencia de rinolicuorrea. La documentación de estos casos es esencial para generar protocolos estandarizados de tratamiento y seguimiento. En nuestra experiencia el manejo conservador de la MAV y el reparo quirúrgico de los defectos es un abordaje adecuado, teniendo en cuenta la morfología y caracterización de la MAV (AU)

Sphenoidal meningoencephalocele associated with CSF fistula and arteriovenous malformation Spetzler-Martin V: A case report.

Cerebral Arteriovenous malformations (AVMs) are presumed congenital anomalies of the blood vessels, which can increase intracranial pressure by uncertain mechanisms. We report the rare case of a 55-year-old male patient who complained about CSF rhinorrhea. Persisting CSF leakage prompted CT, which evidenced a bone defect in the right middle cranial fossa with protruding brain tissue. The diagnosis of a sphenoidal meningoencephalocele was made. Neuroimaging evidenced an AVM Spetzler Martin V. The lesion was targeted via an endonasal approach with resection of the herniated brain tissue and closure of the bony and dural defects. The postoperative course was uneventful without recurrence of the CSF fistula. Documentation of these cases is essential to come up with standardized therapeutical protocols and follow-up. Nevertheless, conservative management of the AVM and surgical repair of the bone defects is an appropriate approach in the first instance, depending on the morphology and characterization of the AVM.

Oropharyngeal neuroglial choristoma with extension into the parapharyngeal space.

Oropharyngeal neuroglial choristomas are exceptionally rare head and neck tumours that contain mature neuroglial tissue. We report a case of neuroglial choristoma centred in the oropharynx and extending into the parapharyngeal space that, to the best of our knowledge, is the only case whose appearance simulated brain cortical gyri with an apparent differentiation between white and grey matter. We illustrate and detail the magnetic imaging findings of oropharyngeal choristoma that might help to establish a correct preoperative diagnosis.

Coristoma neuroglial orofaríngeo con extensión al espacio parafaríngeo / Oropharyngeal neuroglial choristoma with extension into the parapharyngeal space

Los coristomas neurogliales orofaríngeos son tumores de cabeza y cuello excepcionalmente raros que contienen tejido neuroglial maduro. Presentamos un caso de coristoma neuroglial centrado en la orofaringe y con extensión al espacio parafaríngeo que, hasta donde sabemos, es el único caso cuya apariencia simulaba circunvoluciones corticales cerebrales con una aparente diferenciación entre sustancia blanca y gris. Ilustramos y detallamos los hallazgos de imágenes por resonancia magnética del coristoma orofaríngeo que podrían ayudar a establecer un diagnóstico preoperatorio correcto.(AU)

Oropharyngeal neuroglial choristomas are exceptionally rare head and neck tumours that contain mature neuroglial tissue. We report a case of neuroglial choristoma centred in the oropharynx and extending into the parapharyngeal space that, to the best of our knowledge, is the only case whose appearance simulated brain cortical gyri with an apparent differentiation between white and grey matter. We illustrate and detail the magnetic imaging findings of oropharyngeal choristoma that might help to establish a correct preoperative diagnosis.(AU)

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.

Individual variation of tentorial notch morphometry in a series of neurocritical patients / Variación individual de la morfometría de la hendidura tentorial en una serie de pacientes neurocríticos

Background: Cadaveric studies on humans have shown anatomical variabilities in the morphometric characteristics of the tentorial notch. These anatomical variations could influence the worsening of neurocritical patients. Objectives: 1) To investigate the morphometric characteristics of the tentorial notch in neurocritical patients using computed tomography (CT); 2) To investigate the correlation between tentorial notch measurements by CT and by magnetic resonance imaging (MRI); and 3) To analyze the individual variability of the tentorial notch anatomy seen in neurocritical patients. Methods: Prospective series of neurocritical patients was examined. An imaging protocol for measurements was designed for CT and MRI. The level of the agreement of the measurements from CT and MR images was established. According to the measurements found, patients were divided into different types of tentorial notch. Results: We studied 34 neurocritical patients by CT and MRI. Measurements of the tentorial notch via CT and MRI showed significant agreement: concordance correlation coefficient of 0.96 for notch length and 0.85 for maximum width of tentorial notch. Classification of tentorial notch measurements according to the criteria established by Adler and Milhorat, we found the following: 15 patients (58%) corresponded to a "short" subtype; 7 (21%) to "small"; 3 (9%) to "narrow"; 2 (6%) to "wide"; 2 (6%) to "large"; 1 (3%) to "long"; and 4 (12%) to "typical". Conclusions: The anatomical variability of the tentorial notch could be detected in vivo by means of CT scan and MRI. Good agreement between the measurements made using these two imaging methods was found.

Antecedentes: Estudios cadavéricos en humanos han mostrado variabilidad anatómica en las características morfométricas de la hendidura tentorial (HT). Estas variaciones anatómicas podrían influir en el neurodeterioro agudo de los pacientes neurocríticos. Objetivos: 1) Investigar las características morfométricas de la HT en pacientes neurocríticos mediante tomografía computarizada (TC); 2) Investigar la correlación de las mediciones de la HT realizadas por TC y resonancia magnética (RM); 3) Analizar la variabilidad individual de la anatomía de la HT observada en pacientes neurocríticos. Métodos: Se examinó una serie prospectiva de pacientes neurocríticos. Se diseñó un protocolo de imágenes para mediciones por TC y RM. Se estableció la concordancia de las mediciones realizadas mediante TC y RM. Según las mediciones encontradas, los pacientes se dividieron en diferentes tipos de HT. Resultados: Estudiamos 34 pacientes neurocríticos por TC y RM. Las mediciones de la HT por TC y RM mostraron una concordancia significativa: coeficiente de correlación de concordancia de 0,96 para la longitud de la HT y 0,85 para el ancho máximo de la HT. Clasificando las medidas de la HT de acuerdo con los criterios establecidos por Adler y Milhorat, encontramos: 15 pacientes (58%) correspondieron al subtipo "corto", 7 (21%) al "pequeño", 3 (9%) al "estrecho" ", 2 (6%) a "ancho ", 2 (6%) al "grande ", 1 (3%) al "largo" y 4 (12%) al "típico". Conclusiones: Se pudo detectar variabilidad anatómica de la HT in vivo, mediante TC y RM. Se encontró una buena concordancia en las medidas obtenidas con ambos métodos imagenológicos.

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.

Oropharyngeal neuroglial choristoma with extension into the parapharyngeal space. / Coristoma neuroglial orofaríngeo con extensión al espacio parafaríngeo.

Oropharyngeal neuroglial choristomas are exceptionally rare head and neck tumours that contain mature neuroglial tissue. We report a case of neuroglial choristoma centred in the oropharynx and extending into the parapharyngeal space that, to the best of our knowledge, is the only case whose appearance simulated brain cortical gyri with an apparent differentiation between white and grey matter. We illustrate and detail the magnetic imaging findings of oropharyngeal choristoma that might help to establish a correct preoperative diagnosis.

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.

Transcranial cerebellar herniation following craniotomy: Case report and literature review.

To report a case of post-surgical encephalocele through craniotomy burr holes following the resection of a meningioma of the posterior fossa. A 49-year-old female presented in the emergency room with cephalea. The MRI showed a meningioma of the convexity of the posterior fossa. A resection was performed and the bone flap replaced. The patient recovered uneventfully and was discharged. After 30 days the patient consulted referring cephalea, vomiting and imbalance. Brain MRI revealed a trans-cranial cerebellar herniation through the craniotomy burr holes. An urgent surgery was performed to repair the encephalocele. Post-surgical brain MRI was performed and did not show complications. Post-surgical encephalocele is an uncommon complication after the resection of a posterior fossa lesion. To avoid this complication, it is recommended thorough dural and bony closure, particularly in the posterior fossa surgeries and in high-risk patients.

Frontoethmoidal encephalocele. Report of a case.

Encephaloceles are uncommon in western countries and most cases are located in the occipital bone. Frontal encephaloceles may involve the ethmoid bone, nasal bones and/or the orbits. Surgical repair is complex and usually requires a multidisciplinary approach. The goal of the surgery is to reconstruct the normal anatomy, to achieve a good cosmetic repair and to avoid a cerebrospinal fluid leak. We present a case of a patient with a large congenital frontoethmoidal encephalocele. Autologous calvarian bone grafts were used to repair of encephalocele defect and for the reconstruction of the frontonasal area. The defect closure and the cosmetic result were satisfactory, and the only complication detected was the infection of a previously performed ventriculoperitoneal shunt. A description of the technique and a review of the literature are presented.

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.

Manejo del excedente cutáneo en encefalocele frontonasal gigante / Management of skin facial excess in giant frontonasal encephalocele

Introducción: el Encefalocele frontonasal es poco frecuente y ocurre por una falla en el cierre del tubo neural durante el desarrollo embrionario. Se asocia a otras anomalías del Sistema Nervioso Central. El scanner y la resonancia nuclear magnética permiten decidir la estrategia de resolución según los defectos óseos, la lesión externa y la deformidad de las unidades faciales. Debe manejarse con equipo multidisciplinario para lograr mejores resultados.En la literatura se describe el tratamiento quirúrgico de los encefaloceles anteriores, pero no se menciona la reducción del excedente cutáneo. En la mayoría de los casos el abordaje bicoronal permite el manejo del encefalocele y la piel, pero el problema surge cuando la superficie de la lesión es mayor. Material y método: Reporte de un paciente con encefalocele anterior gigante tratado en nuestro centro. Descripción del caso: Recién nacido de término sin diagnóstico antenatal de encefalocele frontonasal gigante derivado desde región. Se realiza manejo multidisciplinario logrando cierre de defecto neural y manejo del excedente cutáneo considerando las subunidades estéticas faciales para lograr buenos resultados estéticos. Conclusiones: El encefalocele nasofrontal gigante es poco frecuente y requiere un manejo multidisciplinario. La cirugía consiste en la resección de la masa herniada, cierre del defecto y manejo cutáneo. Este caso muestra cómo manejar un colgajo residual considerando las subunidades faciales.

Introduction: The Nasofrontal Encephalocele is rare. It is triggered by a failure in the neural tube closure during the embryonic development. This is associated with other anomalies of the Central Nervous System. The Scanner and the Nuclear Magnetic Resonance allow deciding the best surgical resolution strategy, according to the bone defects, external injury and deformity of the facial subunits. In order to achieve optimal results, a multidisciplinary team is required. In the existing literature, surgical treatment of the Anterior Encephalocele is described. However, the skin facial excess in cases of giant tumors is not described. In most patients, bicoronal incision allows handling both the encephalocele and tissue excess. The problem arises when the injury surface is too wide to be treated by abicoronal incision. Materials and methods: Report of a clinical case of a patient diagnosed with a giant anterior encephalocele, who was treated in our center. Clinical picture and performed surgical treatment are described, emphasizing he skin excess reduction, considering the subunits for the repair. Conclusion: The Giant Nasofrontal Encephaocele is a rare defect that requires a multidisciplinary treatment. This surgery consists of resection of the herniated mass and closure of the defect with or without a cranial vault reconstruction. This case sets an example of how to handle residual flap tissue in patients presenting a frontal facial tumors, considering the facial subunits as a reference for the repair that allowed a good aesthetic results.

Zika y defecto del tubo neural

Presentamos el primer caso confirmado de neonato con microcefalia relacionado a Zika en Panamá, quien además presentaba lesión a nivel del occipucio compatible con encefalocele.

We report the first confirmed case of newborn with microcephaly related with Zika in Panama, who also had lesions to the occiput compatible with encephalocele.

Encefalocele frontonasal: reporte de un caso con diagnóstico prenatal / Frontonasal encephalocele: report of a case with prenatal diagnosis

El encefalocele es un defecto congénito mayor que se caracteriza por la herniación del tejido nervioso y las meninges a través de un defecto de la bóveda craneal. Esto se debe a alteraciones del cierre del tubo neural ocurridas en la cuarta semana de gestación. El diagnóstico prenatal es posible realizarlo a través de ecografía 2D y 3D. Se presenta el caso de un neonato con encefalocele anterior, entidad poco frecuente dentro de los defectos del cierre del tubo neural. Se realizó el diagnóstico presuntivo prenatal por ecografía 2D y 3D, y se confirmó al nacimiento por los hallazgos clínicos y escanografía(AU)

The encephalocele is a major birth defect characterized by herniation of neural tissue and meninges through a defect in the skull, due to alterations of neural tube closure occurring in fourth week of pregnancy. Prenatal diagnosis is possible through 2D and 3D ultrasound. We report a case of anterior encephalocele, a rare disease among the defects of neural tube closure, with diagnosis by 3D and 2D ultrasonography, which was confirmed at birth by clinical findings and scan(AU)

Reparación endoscópica de encefalocele esfenoidal, secundario a persistencia del canal de Sternberg / Endoscopic repair of sphenoid encephalocele, secondary to persistence of Sternberg's canal

Las fístulas de líquido cefalorraquídeo (FLCR) nasales se producen por la comunicación entre el espacio subaracnoídeo y el tracto aerodigestivo. Pueden ser adquiridas (secundarias a trauma o iatrogenia) o espontáneas. Éstas últimas pueden ser de origen congénito, tumoral o idiopáticas. El canal lateral craneofaríngeo o de Sternberg se produce por la falta de fusión de los puntos de osificación del seno esfenoidal durante el período embrionario. Esta región, ubicada posterolateral a la pared inferior del seno esfenoidal, queda cubierta solamente por tejido conectivo, siendo la zona de menor resistencia de la base de cráneo. La persistencia de este canal puede causar FLCR, especialmente cuando se asocia a elevación de la presión intracraneana y extensa neumatización. La cirugía endoscópica nasal es la técnica de elección para la resolución de estos casos. En el presente artículo se realiza una revisión de la técnica quirúrgica endoscópica nasal para la resolución quirúrgica de encefaloceles secundarios a persistencia del canal de Sternberg, en base a dos casos clínicos que fueron sometidos a cirugía endoscópica nasal.

Nasal cerebrospinal fluid leaks are caused by a nasal communication between the subarachnoid space and the aerodigestive tract. They may be acquired (secondary to trauma or iatrogenic) or spontaneous. The latter can be congenital, tumoral or idiopathic. The lateral craniopharyngeal canal or Sternberg canal is caused by the lack of fusion of the points of ossification of the sphenoid sinus during the embryonic period. This region, located posterolateral to the bottom wall of the sphenoid sinus, is covered only by connective tissue, being the area of least resistance of the skull base. The persistence of this canal can cause cerebrospinal fluid leaks, especially when associated with elevated intracranial pressure and extensive pneumatization. Nasal endoscopic surgery is the technique of choice for solving these cases. This article is a review of the endoscopic surgical technique of encephaloceles secondary to persistence of Sternberg canal, based on two clinical cases.

Supra- and infra-torcular double occipital encephalocele.

An encephalocele is a protrusion of the brain and/or meninges through a defect in the skull that is closed or covered with skin. Occipital encephaloceles are the most frequent type in North America and Western Europe, where about 85% of encephaloceles take this form. To the best of our knowledge, there are only three other reported cases of double occipital encephaloceles in the literature. The current study reports a double and both supra- and infra-torcular occipital encephalocele in a neonate and discusses the importance of preoperative neuroimaging studies to optimize the outcome. The patient was a 1-day-old male child who was identified by prenatal ultrasound to have two occipital encephaloceles. The patient underwent a closure of the occipital encephalocele on the second postnatal day. The infant tolerated the procedure well and was extubated on the first postoperative day. The child continues to do well during follow-up.

Síndrome de Meckel con onfalocele y labio fisurado / Meckel syndrome with omphalocele and cleft lip

El síndrome de Meckel es un desorden autosómico recesivo, raro y letal. Se caracteriza por múltiples malformaciones, entre estas, la triada de encefalocele occipital, riñones poliquísticos y polidactilia. Se presenta un caso con múltiples malformaciones incluyendo las menos frecuentes como el onfalocele, la hipospadia y el labio fisurado.

The Meckel Gruber syndrome is a lethal, rare and autosomal disorder. It is characterized by multiple malformations, among these the triad of occipital encephalocoele, large polycystic kidneys and post-axial polydactyly. A case with multiple malformations, including the less frequent as omphalocele, hypospadias and cleft lip is presented.

Reconstrucción de encefalocele frontoetmoidal con injerto de acetábulo / Reconstruction of encephalocele frontoethmoidal using graft acetabular: Case Report

El encefalocele frontoetmoidal es un defecto congénito del tubo neural caracterizado por una herniación parcial del cerebro y las meninges, a través de un defecto craneal anterior que genera deformidad facial. Se presenta el reporte de un niño de 10 años con un defecto facial en línea media que correspondió a un encefalocele frontoetmoidal. Se realizó corrección quirúrgica acompañado de colocación de injerto de acetábulo, obteniéndose una mejoría estética y anatómica favorable. Este parece ser el primer caso en la literatura en el que se utiliza el injerto de acetábulo para la reconstrucción de un encefalocele frontoetmoidal.

Frontoethmoidal encephalocele is a congenital neural tube malformation characterized by a herniation of the brain and the meninges through an anterior cranial defect, which generates facial deformity. This document presents a report of a 10-year-old boy with a midline facial defect corresponding to an encephalocele frontoethmoidal. Surgical correction was performed with the acetabulum graft placement, obtaining an aesthetic improvement and anatomically favorable. This seems to be the first case in the literature in which the acetabulumgraft is used to reconstruct a frontoethmoidal encephalocele.