Challenges in the Management of Symptomatic Fallopian Canal Meningoceles: A Multicenter Case Series and Literature Review.

OBJECTIVE: To describe the presentations, the diagnosis, our treatment approaches, and the outcomes for 11 patients with fallopian canal meningocele (FCM). STUDY DESIGN MULTICENTER: Retrospective case series. SETTING: Tertiary referral centers. PATIENTS: Patients (N = 11) with radiographically or intraoperatively identified, symptomatic FCM. INTERVENTIONS: Surgical repair of cerebrospinal fluid (CSF) leak and meningocele versus observation. MAIN OUTCOME MEASURES: Presentation (including symptoms, radiographic imaging, and comorbidities), management (including surgical approach, technique for packing, use of lumbar drain), clinical outcomes (control of CSF leak, meningitis, facial nerve function), and revision surgery. RESULTS: Patients presented with spontaneous CSF leak (n = 7), conductive (N = 11) and sensorineural hearing loss (n = 3), nonpositional intermittent vertigo (n = 3), headaches (n = 4), and recurrent meningitis (n = 1). Risk factors in our series included obesity (n = 4), Chiari 1 malformation (n = 1), and head trauma (n = 2). Noncontrast computed tomography of the temporal bone and magnetic resonance imaging were positive for FCM in 10 patients. Eight patients were managed surgically via a transmastoid approach (n = 4), combined transmastoid and middle fossa (N = 3), or middle fossa alone (n = 1); three were managed conservatively with observation. Postoperative complications included worsened facial nerve palsy (n = 1), recurrent meningitis (n = 1), and persistent CSF leak that necessitated revision (n = 1). CONCLUSIONS: Facial nerve meningoceles are rare with variable presentation, often including CSF otorrhea. Management can be challenging and guided by symptomatology and comorbidities. Risk factors for FCM include obesity and head trauma, and Chiari 1 malformation may present with nonspecific otologic symptoms, in some cases, meningitis and facial palsy. Layered surgical repair leads to high rates of success; however, this may be complicated by worsening facial palsy.

Carolyn's window approach for spontaneous frontal sinus meningoencephalocele.

Spontaneous meningoencephaloceles (MECs) are sparsely documented in the literature. Those occurring in the frontal sinus are an exceedingly rare entity. MECs are commonly associated with cerebrospinal fluid (CSF) rhinorrhoea. CSF rhinorrhoea is frequently misdiagnosed, causing delays in diagnosis and management. The subsequently increased risk of bacterial meningitis can be life-threatening to patients. We report the case of a woman in her late 70s with a spontaneous frontal sinus MEC, presenting with a 6-month history of CSF rhinorrhoea. The patient was successfully treated using the novel Carolyn's window approach endoscopically; 9-month follow-up revealed no skull-base breach. Our case emphasises the importance of considering MEC as a differential diagnosis for clear rhinorrhoea and demonstrates successful repair through a novel surgical approach.

Posterior median surgical approach to anterior sacral meningocele complicated by rectothecal fistula.

In this case report, we aimed to describe the clinical presentation, surgical approach, and follow-up of a patient with rare anterior meningocele associated with rectothecal fistula. An 17-year-old female patient was admitted to the emergency department with meningitis. On further examinations, an anterior sacral meningocele accompanied by rectothecal fistula was detected. Appropriate antibiotic treatment was arranged and surgical plan was made with the pediatric surgery clinic. The patient underwent meningocele repair via posterior approach and colostomy operation. The patient did not experience any neurological issues after the surgery. The colostomy was reversed 3 months later, and third-month follow-up MRI showed complete regression of the meningocele sac with no neurological complications. Anterior meningocele accompanied by a rectothecal fistula is a rare and complicated case. Only seven cases of coexisting ASM and RTF have been reported in literature. Although both anterior and posterior approaches have been used for the treatment of ASM, the choice of treatment is essentially based on the patient's clinical and imaging findings.

Posterior Surgical Ligation and Cyst Decompression -via Needle Puncture- of a Large Anterior Sacral Pelvic Meningocele Through Posterior Sacral Laminectomy.

The first documented description of an anterior sacral meningocele was Bryant's in 1823. Anterior sacral meningocele patients have constipation as a universal symptom; urinary incontinence is also common. All the symptoms are directly related to the pressure from a pelvic mass on adjacent structures. When the patient stands, a headache often develops because the spinal fluid pressure decreases as the meningocele sac fills. Finally, a scimitar-shaped sacrum on a neuroradiological anteroposterior plain assessment is pathognomonic. The coccyx may be absent, and the lower sacral laminae may be absent or incomplete. The surgical options for this rare clinical condition are still matter of debate.Anterior sacral meningocele is a pathology that lacks a current classification and neurosurgical therapeutic standards, even though a similar dynamic has been shown by the related traumatic pseudomeningocele. Anterior approaches (retro- and transperitoneal meningocele neck occlusion with internal cerebrospinal fluid (CSF) cyst drainage) and posterior approaches (posterior sacral laminectomy, dural sac ligation, and CSF cyst drainage) are the available surgical strategies.We now report the case of an adult patient for whom a posterior approach was suggested and performed and report her postoperative surgical follow-up. The surgical rationale is also discussed.

Bilateral Optic Nerve Sheath Meningoceles in an Asymptomatic Patient.

This case report discusses a diagnosis of bilateral optic nerve sheath meningoceles in a man aged 82 years with stable visual acuity and asymptomatic bilateral choroidal folds.

Meningoencephalocele and supernumerary ectopic limb arising from the occipital bone in a Jacob sheep lamb.

This report describes the clinical, computed tomography, and magnetic resonance imaging findings for a Jacob sheep lamb diagnosed with meningoencephalocele and supernumerary ectopic limb. Key clinical message: This case demonstrates the utility of tomographic imaging in diagnosing congenital malformations in sheep and can be used to assess the extent of the lesion. This may help to determine any viable treatment, or, as in the case presented here, determine that the extent of the lesion precludes surgical intervention.

Méningoencéphalocèle et membre ectopique surnuméraire provenant de l'os occipital chez un agneau Jacob. Ce rapport décrit les résultats cliniques, de tomodensitométrie et d'imagerie par résonance magnétique d'un agneau de Jacob chez qui on a diagnostiqué une méningoencéphalocèle et un membre ectopique surnuméraire.Message clinique clé :Ce cas démontre l'utilité de l'imagerie tomographique dans le diagnostic des malformations congénitales chez le mouton et peut être utilisée pour évaluer l'étendue de la lésion. Cela peut aider à déterminer tout traitement viable ou, comme dans le cas présenté ici, à déterminer que l'étendue de la lésion exclut une intervention chirurgicale.(Traduit par Dr Serge Messier).

An Endoscopic Endonasal Attempt at Pediatric Meningoencephalocele Repair.

Meningoencephalocele is an abnormal skull base protrusion of fluid, brain tissue, and meninges that can lead to nasal obstruction, meningitis, and Cerebrospinal Fluid (CSF) rhinorrhea. This condition can be managed operatively through an open craniotomy or a less invasive endoscopic approach. Here, we report a case of an 18-month-old female who presented with a meningoencephalocele that was part of the Sakoda complex, a rare neurosurgical phenomenon consisting of meningoencephalocele, agenesis of the corpus callosum, and cleft lip/palate. The patient was initially treated with the endoscopic transsphenoidal approach with subsequent open craniotomy.

[A Surgical Case of Currarino Syndrome with Syringomyelia].

Currarino syndrome is characterized by a triad of anorectal malformations, sacral defects, and presacral masses. Although it is not extremely rare, this report presents a surgical case of Currarino syndrome with syringomyelia and discusses related literature. The patient is a girl, aged 2 years and 2 months, who presented with marked constipation, chronic cystitis, and lower limb weakness. After examining the patient through magnetic resonance imaging, we diagnosed her with rectal compression due to meningocele and syringomyelia. The base of the meningocele was detached, and the spinal cord was untethered. One week after surgery, her lower limb weakness and constipation improved. Following up on symptoms and performing imaging is essential to determine a treatment plan for Currarino syndrome. (Received 28 February, 2023; Accepted 22 March, 2023; Published 1 August, 2023).

Intrasacral meningoceles: Clinical presentation, surgical management, and postoperative outcome: The Giannina Gaslini Hospital's experience.

INTRODUCTION: Intrasacral meningoceles are cysts associated with herniating arachnoid with no nerve root within due to an area of weakness of the dura mater. They are thought to be congenital, but they are usually not symptomatic until adulthood. Surgical treatment is generally indicated in the presence of symptoms. METHODS: We selected cases belonging to the IB category of Nabors et al.'s classification who underwent surgery between 2008 and 2021 at Giannina Gaslini Hospital. Exclusion criteria were prior history of trauma, infections, or operations. Patients' clinical details, associated conditions, surgical techniques, peri- and postoperative complications, and outcomes were collected retrospectively from clinical charts. We compared our series to literature: keywords "intrasacral meningocele" were used on the search engine MEDLINE - Pubmed. RESULTS: We identified 23 cases: 5 of the 14 symptomatic patients had a complete resolution, and 5 had a substantial clinical improvement after surgery. Cyst recurrence and major postoperative complication occurred in none. Among 59 articles considered for evaluation, 50 were excluded and remaining 9 articles underwent full-text analysis. DISCUSSION AND CONCLUSION: The pathogenesis of instrasacral meningoceles is still not completely understood and the spectrum of symptoms is wide. A posterior surgical approach with sacral laminectomy is preferred, although in selected cases it is possible to perform a supplemental anterior approach (sometimes endoscopic). In our surgical series, the largest one published in the literature, a good clinical outcome was achieved in most patients with no cyst's recurrence, pointing out the importance of surgical interruption of communication between cyst and subdural space.

A clinical clue toward recognition of split cord malformation type-I resembling meningocele in neonates.

This is a letter to share the clinical experience we had with neonates born with a specific feature of spina bifida. The lesion is like a meningocele containing skim amount of fluid, with a tiny dimple around the center of the cutaneous lesion which causes inward retraction of the dysplastic coverage. This feature is in favor of accompanied split cord malformation (SCM) type I, and the central nidus is in continuous with the dural sleeve around the bony septum of SCM. By recognition of this clinical clue, surgical repair can be planned to perform with appropriate provision, particularly foreseeing the amount of intraoperative bleeding and duration of anesthesia in the newborn patients.

Combined Endoscopic Endonasal and Transpalatal Repair of Congenital Anterior Cranial Fossa Agenesis.

BACKGROUND: Congenital basal meningoceles and encephaloceles are rare pathologies that may present in isolation or with characteristic-associated clinical features. Rarely, children with congenital midline defects may present with massive encephaloceles secondary to anterior cranial fossa agenesis. Traditionally, transcranial approaches with frontal craniotomies were used to reduce the herniated contents and repair the skull base defect. However, high rates of morbidity and mortality associated with craniotomies have favored the development and adoption of less-invasive techniques. OBJECTIVE: To present a novel technique for combined endoscopic endonasal and transpalatal repair of a giant basal meningocele through an extensive sphenoethmoidal skull base defect. METHODS: A representative case of congenital anterior cranial fossa agenesis with a giant meningocele was selected. Clinical and radiological presentations were reviewed, and the intraoperative surgical technique was documented and recorded. RESULTS: A surgical video highlighting each surgical step was included to complement the description of the technique. The surgical outcome from the selected case is also presented. CONCLUSION: This report describes a combined endoscopic endonasal and transpalatal approach to repair an extensive anterior skull base defect with herniation of intracranial content. This technique capitalizes on the advantages of each approach to address this complex pathology.

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)

A topographical analysis of encephalocele locations: generation of a standardised atlas and cluster analysis.

OBJECTIVE: Encephaloceles are considered to result from defects in the developing skull through which meninges, and potentially brain tissue, herniate. The pathological mechanism underlying this process is incompletely understood. We aimed to describe the location of encephaloceles through the generation of a group atlas to determine whether they occur at random sites or clusters within distinct anatomical regions. METHODS: Patients diagnosed with cranial encephaloceles or meningoceles were identified from a prospectively maintained database between 1984 and 2021. Images were transformed to atlas space using non-linear registration. The bone defect, encephalocele and herniated brain contents were manually segmented allowing for a 3-dimensional heat map of encephalocele locations to be generated. The centroids of the bone defects were clustered utilising a K-mean clustering machine learning algorithm in which the elbow method was used to identify the optimal number of clusters. RESULTS: Of the 124 patients identified, 55 had volumetric imaging in the form of MRI (48/55) or CT (7/55) that could be used for atlas generation. Median encephalocele volume was 14,704 (IQR 3655-86,746) mm3 and the median surface area of the skull defect was 679 (IQR 374-765) mm2. Brain herniation into the encephalocele was found in 45% (25/55) with a median volume of 7433 (IQR 3123-14,237) mm3. Application of the elbow method revealed 3 discrete clusters: (1) anterior skull base (22%; 12/55), (2) parieto-occipital junction (45%; 25/55) and (3) peri-torcular (33%; 18/55). Cluster analysis revealed no correlation between the location of the encephalocele with gender (χ2 (2, n = 91) = 3.86, p = 0.15). Compared to expected population frequencies, encephaloceles were relatively more common in Black, Asian and Other compared to White ethnicities. A falcine sinus was identified in 51% (28/55) of cases. Falcine sinuses were more common (χ2 (2, n = 55) = 6.09, p = 0.05) whilst brain herniation was less common (χ2 (2, n = 55) = .16.24, p < 0.0003) in the parieto-occipital location. CONCLUSION: This analysis revealed three predominant clusters for the location of encephaloceles, with the parieto-occipital junction being the most common. The stereotypic location of encephaloceles into anatomically distinct clusters and the coexistence of distinct venous malformations at certain sites suggests that their location is not random and raises the possibility of distinct pathogenic mechanisms unique to each of these regions.

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.

Analysis of the Causes and Experience in the Diagnosis and Treatment of Meningocele Caused by Sternberg's Canal of the Sphenoid Sinus: Two Case Reports and a Review of the Literature.

OBJECTIVE: The present study aimed to improve the diagnosis and treatment outcome of cerebrospinal fluid (CSF) rhinorrhea caused by patent meningoencephalocele of Sternberg's canal of the sphenoid sinus by analyzing the clinical data and imaging features of two rare cases of this disease and by reviewing the relevant literature for possible etiology, diagnoses, and treatments. METHODS: Together with the relevant literature, we retrospectively studied the clinical and imaging data of two patients (mother and child) with CSF rhinorrhea caused by patent meningoencephalocele of Sternberg's canal of the sphenoid sinus, analyzed their diagnostic and treatment procedures, and proposed a potential, feasible treatment method. RESULTS: On the 2nd day after surgery, the expansive sponge and iodoform gauze in the nasal cavity were removed in both patients, and the lumbar subarachnoid drainage was removed 3 days after the operation, as no nasal discharge was observed. One week after the operation, head magnetic resonance imaging (MRI) showed that the abnormal tissue in the sphenoid sinus had disappeared, and no accumulation of the CSF was observed. Both patients were discharged after 2 weeks. At the time of discharge, both patients were without nasal drip, fever, headache, and other discomforts, and they had grade 5 muscle strength in their extremities, with normal muscle tension. CONCLUSION: CSF rhinorrhea is usually caused by secondary factors. Spontaneous CSF rhinorrhea caused by encephalocele of the skull base due to congenital dysplasia of the skull base is very rare and easily misdiagnosed. The presence of brain tissue or CSF signal in the sphenoid sinus on preoperative MR images is an important imaging feature of the disease. Conditional cisternography can be used to further detect CSF leaks. Endoscopic transnasal transsphenoidal repair of CSF leaks combined with short-term postoperative lumbar subarachnoid drainage is an effective treatment method. According to previous literature, the possible causes of meningoencephalocele with patent Sternberg's canal of the sphenoid sinus include abnormal development of the sphenoid sinus or the craniopharyngeal canal and bone defects of the skull base. There are no related reports on patent meningoencephalocele caused by Sternberg's canal in direct blood relatives, such as mother-son; therefore, the possibility of this disease having a genetic origin should be considered in future studies on its pathophysiological mechanisms.

The Need for Emphasis on Intracranial Hypertension Management in Spontaneous Skull Base Meningoencephaloceles.

OBJECTIVE: To provide an analysis of patients with spontaneous skull base meningoencephaloceles (MECs) to determine whether definitive surgical treatment requires management of elevated intracranial pressure (ICP). METHODS: Data of 10 subjects with spontaneous MECs were collected and retrospectively evaluated. Measurement of ICP, prior interventions, treatment with acetazolamide, and characteristics of long-term elevated ICP, among others, were analyzed. Our own indications for cerebrospinal fluid (CSF) diversion and use of postoperative external lumbar drain were analyzed as well. RESULTS: The sphenoid region was the most common location of MECs. CSF leak was diagnosed in all subjects. The most common graphical signs of elevated ICP were empty sella and arachnoid pits, both of which were present in 90% of cases. Lumbar puncture with opening pressure measurement was performed in 7 patients. Ventriculoperitoneal shunt insertion was indicated in 4 cases before skull base repair and in 2 cases after skull base repair. Two postoperative CSF leaks were managed with external lumbar drain and subsequent shunt installation. CONCLUSIONS: Spontaneous MECs are often associated with CSF leak. ICP assessment should be a standard of care to ensure successful operative repair of MECs. Insertion of a CSF diversion device must be considered where direct or indirect signs of intracranial hypertension are present.

Bone fusion in transcele reconstruction of frontoethmoidal meningoencephalocele.

OBJECTIVE: In surgical correction of frontoethmoidal encephalocele with transcranial approach, advanced facilities are required. While with extracranial approach, though deemed as a safe option in area with limited facilities, procedure was associated with cerebrospinal fluid (CSF) leakage. In this case series, we evaluate the results of transcele reconstruction of frontoethmoidal encephalocele, our approach to reduce the incidence of CSF leaks by focusing on the closure of layers by its embryological derivatives, by its bone fusion. METHODS: A case series of 14 patients with various types of frontoethmoidal encephalocele who underwent surgery for defect closure using transcele approach between June 2015 and December 2018 was carried out. Surgery was done by a single surgeon in the Department of Neurosurgery of Cipto Mangunkusumo Hospital in Jakarta, Indonesia. We collected the data of intraoperative blood loss and any signs of infection and CSF leak during the patients' one-year follow up. Bone fusion in the defect was evaluated from 3D rendering of head CT scan that was performed before and in 1 year after surgery. RESULTS: The median percentage of intraoperative blood loss was 5.9% (0.5-18.7%). All 3D rendering of head CT post-surgery during 1 year follow up showed bone fusion and no patient experienced CSF leaks or CNS infections. CONCLUSIONS: This study showed that using transcele approach in frontoethmoidal reconstruction could give good bone fusion with minimal blood loss and no CSF leaks. We assumed that closure of the layers by its embryological derivative played an important part in bone fusion and in reducing the incidence of CSF leaks, although this finding has to be validated with large-scale studies.