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.

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.

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.

Meckel-Gruber syndrome: a rare and lethal foetal anomaly

Meckel-Gruber syndrome (MGS) is a rare and lethal autosomal recessive disorder characterized by occipital encephalocele, postaxial polydactyly and bilateral dysplastic cystic kidneys. It can be associated with many other congenital malformations. The incidence of Meckel-Gruber syndrome ranges between 1 in 13,000 to 4,00,000 live births. Antenatal ultrasound examination establishes the diagnosis by identifying at least two of the major features. A case is presented that describes a baby with ambiguous genitalia, who presented with the triad of Meckel-Gruber syndrome. The baby died shortly after birth.

Case report of anaesthetic management of neonate with large occipital encephalocele

In the present case report, endotracheal intubation in a case of large occipital encephalocele presented for surgical excision was performed. As the management of airway even in a neonate is different and complex as compared to airway of old child and that of adult. It was challenging because of the difficulty in securing airway because of prone position in occipital encephalocele. The major concerns during the operation were to avoid premature rupture of the encephalocoele and to manage a possible difficult airway due to restricted neck movement and inability to achieve optimal position for intubation of the trachea. Continuous monitoring of patient was done during the surgery.

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.

Advances in diagnosis and treatment of syndrome of trephined and paradoxical herniation after decompressive craniectomy / 中华创伤杂志

The efficacy of decompressive craniectomy (DC) in reducing traumatic brain injury mortality has been affirmed,but there are also many serious complications.Syndrome of the trephined (ST) and paradoxical herniation (PH) are rare complications.ST is characterized by a series of neurological deterioration due to skin flaps subsidence from weeks to months after DC.These neurological impairments are closely related to the subsequent repair of skull defects.PH shows progressive decrease in consciousness on the basis of ST,changes in pupils on the side of skull defects,low touch pressure at the defect window,and obvious midline shift and brain stem compression on CT.ST and PH have common inducements in pathophysiology,including cerebrospinal fluid dynamics,atmospheric pressure,cerebral blood flow and brain material metabolism.There is no consensus on the diagnosis of ST and PH,and early cranioplasty is suggested in terms of treatment.This article reviews the clinical manifestations,pathophysiological changes,diagnosis and treatment of ST and PH after DC operation,so as to provide references for clinicians to further understand ST and PH.

Encephalocele presenting as lower lid swelling: A rare case report

Encephalocele is a rare congenital abnormality characterized by abnormal protrusion of brain and meninges through an opening in the skull. We report an 8-year-old girl who presented with a swelling in the right lower lid for the last 6 years. In her infancy, she had undergone surgery for a very small swelling located in the right nasolacrimal area. On further clinicoradiological evaluation, anterior encephalocele was diagnosed. This case highlights the uncommon site of anterior encephalocele; misdiagnosis and mismanagement of which could result in dreaded complications such as meningitis and cerebrospinal fluid leaking fistula formation.

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.

False Computed Tomography Findings in Bilateral Choanal Atresia

Abstract Introduction Choanal atresia (CA) is a challenging surgical problem defined as a failure in the development of communication between the nasal cavity and nasopharynx. Objective The objective of this study is to describe computed tomography (CT) findings in cases with bilateral choanal atresia. Methods The study involved performing axial and coronal non-contrast CT scanning with 2-3 mm sections on14 neonates that had bilateral CA. We used fiberoptic nasal endoscopy to confirm the diagnosis. We evaluated coronal CT to study the skull base area in such neonates. Results This study included 14 neonates with bilateral CA; with mean age of 7 3.5 days. Mixed atretic plates were found in 12 (85.7%) cases while two (14.3%) had pure bony atresia. Isolated CA was detected in 9 cases (64.3%) and 5 (35.7%) cases had associated anomalies. Coronal CT showed soft tissue density in the nasal cavity that appeared to extend through an apparent defect in the nasal roof (cribriform plate), falsely diagnosed by radiologists as associated encephalocele. At the time of surgical repair, all patients showed thick tenacious mucous secretions in both nasal cavities and revealed no encephalocele. Nasal roof remained intact in all cases. Conclusion The thick secretion of bilateral CA could give a false encephalocele appearance on the CT. It is highly recommended to perform proper suction of the nasal cavity of suspected CA cases just before CT scanning.

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)

Surgical treatment of central herniation in severely-head injured patients / 中华创伤杂志

Objective To determine the characteristics of treatment and diagnosis,surgical timing and surgical methods in severely head-injured patients with central herniation.Methods Twenty patients with central herniation caused by contusions and lacerations of the bilateral frontal lobes hospitalized from July 2010 to December 2012 were retrospectively reviewed.There were 11 males and 9 females,at mean age of 42 years (range,18-70 years).Injury was caused by traffic accidents in 15 patients,falls in 3 and fighting events in 2.Eight patients were treated immediately on admission and twelve patients underwent emergency operation.All the operations involved simultaneous bilateral craniectomy for decompression,including bilateral decompressive craniectomy in 6 patients and unilateral decompressive craniectomy in 14 patients.Glasgow Outcome Scale (GOS) and Montreal Cognitive Assessment were used to evaluated outcome evaluation and cognitive impairment respectively.Complications were recorded.Results All patients were followed up for 6-12 months (mean,8 months).According to GOS,good recovery was presented in 10 patients,moderate disability occurred in 6,severe disability in 2,vegetative state in 1,and death in 1.Eleven patients suffered severe mental disorders especially personality change and disturbance of intelligence,and restored after 12 months.Five patients were complicated by epilepsy and two hydrocephalus.Conclusions For central herniation in patients with severe head injury,an emergent surgery is necessary if there exist conscious disturbance and pupil aggravations,hematoma enlargement and significant displacement of midline structure.Timely bilateral balance decompressive craniectomy is effective to reduce the mortality and disability and improve quality of life.

Endoscopic Endonasal Repair of Congenital Basal Encephalocele: Report of 3 Cases / 대한이비인후과학회지

Encephalocele is a malformation characterized by protrusions of the brain and meninges through openings in the cranial bones. They are managed surgically and approached in various ways, mainly on the basis of its location. Basal encephaloceles have been traditionally approached intracranially via bicoronal approach in the pediatric population. We report three cases of congenital basal encephaloceles treated by endoscopic endonasal approach in our institution. In 2 patients, the skull base defect was successfully corrected whereas in one case, recurrence was observed. No major complications were encountered. Although the endoscopic endonasal repair approach of basal encephaloceles in the pediatric population is associated with potential technical difficulties, it is a viable and minimally invasive alternative to the traditional craniotomy.

Spontaneous middle fossa encephalocele

@#A 48-year old man presented with a unilateral right hearing loss of four months’ duration. A right middle ear effusion was noted on physical examination. Endoscopic examination of the nasopharynx was unremarkable. Due to the duration of the symptoms, myringotomy with ventilation tube insertion was offered as a treatment option. Upon myringotomy, clear pulsatile liquid flowed out of the incision. More than 5 cc of liquid was collected which continued to flow out despite active suctioning. Due to the realization that the liquid most likely represented cerebrospinal fluid, insertion of a ventilation tube was not performed. The ear canal was packed with sterile cotton, and the patient was given a short course of acetazolamide to decrease CSF production. Upon further questioning, the patient did not have any prior head trauma. The patient then underwent both computerized tomographic (CT) imaging and magnetic resonance imaging (MRI) of the temporal bone to look specifically for evidence of a dehiscence in the middle fossa plate (tegmen) or posterior fossa plate, as well as the presence of a meningoencephalocele.