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.

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.

[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).

Amniotic band syndrome with CNS involvement: a pediatric neurosurgeon's dilemma-a case series and literature review.

BACKGROUND: Amniotic band syndrome (ABS) is a rare congenital disease characterized by a broad spectrum of congenital anomalies resulting from the strangulated developing organ(s) by the detached fibrous amniotic band. The prevalence of CNS involvement in ABS is rare, but the mortality rate in these cases is high, while morbidity among the surviving patients is inevitable. CASE REPORT: Three-month-old male, 9-month-old female, and newborn female babies were presented with head lump(s), severe facial cleft, syndactyly, and finger amputation. The patient's head imaging confirmed meningoencephalocele as the cause of the head lump in 2 patients; meanwhile, a porencephalic cyst was identified as the origin of head lumps in the other patient. VP shunt placement surgery was performed as the initial management in 2 patients, while one patient directly underwent meningoencephalocele resection surgery. Craniofacial and limb reconstructions were planned as the follow-up management in all cases. Unfortunately, one patient died of complications from suspected aspiration, while another never returned for follow-up treatment. CONCLUSION: Here, we report 3 ABS cases with CNS involvement. Despite the severe disfigurement and disability, the inexistence of fatal malformation might lead to long-term survival. The treatment of malformation(s) that might predispose to another fatal condition and surgery(-ies) to improve functional outcomes and patient's social acceptability should be prioritized in managing the surviving ABS patients.

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.

Allowing Standing and Assisted Steps in a Patient With Renshaw Type IV Sacral Agenesis: A Case Report of a Novel Surgical Approach.

CASE: A 6-year-old boy with a Renshaw type 4 sacral agenesis presented paraplegia and rigid, "Buddha-like" lower-limb contractures, including severe knee pterygia, which made crawling and sitting difficult. Staged surgical treatment involved bilateral knee disarticulation, soft tissue surgery, and bifocal femoral osteotomies for lower-limb reorientation. At 18 months postoperatively and after prosthetic fitting, the patient can stand and take steps with assistance. CONCLUSION: This effective surgical strategy achieves standing in a troublesome orthopaedic congenital condition. The intervention should be tailored to specific orthopaedic disorders and the wishes of patients and families, aiming to improve function.

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.

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.

Local conceptions of the role of folate in neural tube defects in Zambia.

OBJECTIVE: In sub-Saharan Africa, neural tube defects (NTDs) are the second most common birth defect, occurring eight times more frequently than in the US. The objective of this study was to assess baseline Zambian caregiver understanding of folate and NTDs and the effectiveness of an NTD prevention educational program. METHODS: This prospective survey-based study included Zambian caregivers of children born with NTDs who completed pre- and post-educational program surveys between January 2020 and January 2021. The verbal survey was administered in English or local Zambian dialects. The 1-hour educational program administered by local Zambian research nurses sought to facilitate understanding of the direct relationship between prenatal folate supplementation and NTDs. RESULTS: Sixty-one eligible caregivers with a median age of 20 (IQR 24-29) years completed the survey. Participants were predominantly from regions outside of Lusaka Province (68%, 41/60) rather than the capital city, Lusaka (32%, 19/60). Most had received prenatal care (91%, 57/61), and 80% (47/59) reported folate use in pregnancy. Of the mothers who took folate during pregnancy, 24% (11/45) reported use within the first 4 weeks after conception, while 76% (34/45) started thereafter. Myelomeningocele was the most common NTD (74%, 32/43), followed by meningocele (14%, 6/43). Prior to the educational program, 52% (29/56) of caregivers reported that NTDs were caused by a vitamin deficiency, which improved to 98% (55/56) after the program (p < 0.001). Furthermore, only 54% (33/61) of caregivers believed that folate should be taken before conception on the baseline survey evaluation, which improved to 95% (58/61, p < 0.001) after the program. All survey participants (58/58) found the educational session helpful. CONCLUSIONS: This study found that a high proportion of Zambian caregivers had received prenatal care and even had taken folate during pregnancy, but none had taken it prior to pregnancy. An educational program effectively improved understanding about the role and timing of perinatal folate administration in NTD prevention. This result also emphasizes the need for folate fortification and folate education for not only mothers but also primary care providers.

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.

Nasoseptal Flap for Skull Base Reconstruction in a Three-Year-Old Child With Nasofrontal Meningoencephalocele.

Congenital anterior skull base defects with meningoencephaloceles causing nasal obstruction and cerebrospinal fluid (CSF) rhinorrhea are rare clinical entities. Traditionally, skull base defects have been repaired via a bifrontal craniotomy. With the introduction of pediatric endoscopic instrumentation, more of these lesions are accessible via an intranasal endoscopic approach, even in the infant population. However, due to the rarity of pediatric meningoencephaloceles, there is a lack of data demonstrating the successful adaptation of endoscopic skull base techniques to the pediatric population. In this report, we present a case of a pediatric frontonasal meningoencephalocele with an anterior skull base defect in a 3-year-old child that was successfully addressed transnasally following 4 failed transcranial approaches. The case highlights the importance of a thorough preoperative evaluation of the surgical approach as well as interdisciplinary management of these patients at a young age. Congenital anterior skull base defects with meningoencephaloceles and CSF leaks are rare clinical entities. Hence, an interdisciplinary approach is vital including experienced pediatricians, otolaryngologists, and neurosurgeons to evaluate the ideal surgical method on an individual basis. The transnasal endoscopic technique has been shown to be minimally invasive, efficient, and safe to apply even to the infant population which could positively be demonstrated in this case.

Double spinal cord tethering and congenital kyphosis in a 4-year-old boy.

PURPOSE: Congenital kyphosis (CK) is an uncommon condition that develops due to a defect of formation or segmentation of one or more vertebrae during the first weeks of embryonic life and can be frequently associated with abnormalities of the spinal cord. Meningocele manqué (MM) is a rare congenital malformation consisting of intradural fibrous bands tethering the spinal cord, oftentimes in combination with a split cord malformation. The aim of this manuscript is to describe combined surgical management of a rare case of CK associated with double spinal cord tethering. STUDY DESIGN: Case report. METHODS: We report the case of a 4-year-old boy presenting with a combination of congenital kyphosis due to L2 fully segmented posterior hemivertebra and double spinal cord tethering, at the level of the filum terminale and at L1-L2 due to an intradural ventral fibrous band. RESULTS: Spinal cord detethering and L2 hemivertebra resection with kyphosis correction were achieved with a single stage procedure. Segmental kyphosis was corrected from 45° to 11° post-operatively. Apart from a transitory sensory impairment, no immediate or delayed complications were noted and, at 2 years follow-up, correction and spinal alignment were stable. CONCLUSION: To the best of our knowledge, this is the first case to report the simultaneous occurrence of congenital kyphosis and double spinal cord tethering due to MM and tethering of the filum terminale. Our case report shows that surgical correction of the deformity and spinal cord detethering can be safely and effectively achieved during the same surgical procedure.

Case report: Meningoencephalocele and recurrent bacterial meningitis in chronic idiopathic intracranial hypertension.

BACKGROUND: Meningoencephalocele is a rare malformation caused by congenital and acquired lesions. The association between recurrent bacterial meningitis and meningoencephaloceles with cerebrospinal fluid (CSF) leak is reported in the literature. We report a rare case of meningoencephalocele secondary to chronic idiopathic intracranial hypertension as a result of hospitalization repeatedly for meningitis due to the lack of CSF leak. CASE PRESENTATION: This study presents a case of a patient with a decade of recurrent meningitis. With clinical symptoms and imaging examination with chronic idiopathic intracranial hypertension, this patient was diagnosed with meningoencephalocele. With the treatment of acetazolamide to decrease CSF product, the patient had no recurrence of meningitis over the 6-months follow-up period. CONCLUSION: In patients with recurrent intracranial infections but no history of immunodeficiency, cranial trauma, or neurosurgery, the possibility of meningitis should be considered appropriately, even in the absence of CSF otorrhea or rhinorrhea.

A Clinical Case of Delayed Posttraumatic Frontal Sinus Cerebrospinal Fluid Leakage Management Via External Surgical Approach.

ABSTRACT: Cerebrospinal fluid rhinorrhea is frequently encountered after a fracture of the skull base. Individual fractures of the posterior wall of the frontal sinus after brain injury are uncommon. The authors present a case of a 33-year-old man with a distant history of skull base injury after a traffic accident, 12 years ago. He presented with intermittent rhinorrhea and reported 2 episodes of bacterial meningitis the last 7 years since the injury. Cerebrospinal fluid leakage was confirmed with ß2-transferrin testing. Computed tomography and magnetic resonance imaging revealed a small defect of the posterior and superior wall of the left frontal sinus and an ipsilateral meningoencephalocele.Finally, there was a communication between intracranial space and frontal sinus that caused meningitis.A coronal incision and frontal craniotomy with preservation of anterior pericranium was performed (frontal sinus cranialization using osteoplastic flap). In order to determine the precise margins of the frontal sinus and allow an accurate anterior table bone osteotomy and complete exposure of the sinus, the authors used a coronal view of skull X-ray. Scissors are then used to cut along the margins of the sinus. An "R" is scratched into the right side of template to record orientation. The template was sterilized and brought onto the surgical field and then placed over the left sinusAn external surgical approach (anterior table bone osteotomy) with the modern technique of osteoplastic flap access was performed. An elevator was used to separate the dura from the posterior table along the entire margin of the defect. Meningoplasty (cauterization of the meningoencephalocele) and closure of the osseous defect was followed. The authors repaired the defect of the posterior wall of the left frontal sinus using a combination of underlay and overlay technique. Femoral fascia from right thigh and fibrin glue were placed in order to cover the leak of the posterior wall of the left frontal sinus, respectively.Patients who present with a short or/ and distant history of traumatic brain injury should be evaluated for complication of a cerebrospinal fluid leak.

Induction of a NOTCH3 Lehman syndrome mutation in osteocytes causes osteopenia in male C57BL/6J mice.

Lateral Meningocele or Lehman Syndrome (LMS) is associated with NOTCH3 mutations causing deletions of the PEST domain and a gain-of-NOTCH3 function. We demonstrated that Notch3em1Ecan mice harboring Notch3 mutations analogous to those found in LMS are osteopenic because of enhanced bone resorption. To determine the contribution of specific cell lineages to the phenotype, we created a conditional-by-inversion (Notch3COIN) model termed Notch3em2Ecan in which Cre recombination generates a Notch3INV allele expressing a NOTCH3 mutant lacking the PEST domain. Germ line Notch3COIN inversion caused osteopenia and phenocopied the Notch3em1Ecan mutant, validating the model. To induce the mutation in osteocytes, smooth muscle and endothelial cells, Notch3COIN mice were bred with mice expressing Cre from the Dmp1, Sm22a and Cdh5 promoters, respectively, creating experimental mice harboring Notch3INV alleles in Cre-expressing cells and control littermates harboring Notch3COIN alleles. Notch3COIN inversion in osteocytes led to femoral and vertebral cancellous bone osteopenia, whereas Notch3COIN inversion in mural Sm22a or endothelial Cdh5-expressing cells did not result in a skeletal phenotype. In conclusion, introduction of the LMS mutation in osteocytes but not in vascular cells causes osteopenia and phenocopies Notch3em1Ecan global mutant mice.

Cerebrospinal fluid hypotension following fall in a child: Case report.

CSF hypotension arises in the context of a leak of CSF which causes negative intracranial pressure. Sacral fractures result from high-energy trauma which are frequently underdiagnosed. A ten-year-old boy presented with hip pain, after a fall. He mobilized both lower limbs, reported no leg pain, irradiation nor lack of sphincter control. The neurological examination was normal. When asked to stand, he began biparietal headache, nausea and vomiting, which improved laying down. CT scan showed an occult intrasacral meningocele; the MRI revealed collections of CSF along the spine, a S3 fracture with potential laceration of the meningocele and opening of a CSF fistula. Our diagnosis was the CSF hypotension, secondary to the fistula opening. The diagnosis was challenging. The child first presented with symptoms of CSF hypotension without evident cause. The discovery of the meningocele led us to hypothesize the opening of a fistula, a rare diagnosis, later confirmed by MRI.