Prenatal Diagnosis and Outcomes of Cervical Meningocele and Myelomeningocele.

Prenatal diagnosis of myelomeningocele remains challenging for obstetricians, ultrasonographers, and radiologists, although the increased maternal serum alpha-fetoprotein level aids in the confirmative diagnosis. Fetal cervical myelomeningocele and meningocele are very rare and unique types of myelomeningocele. Prenatal diagnosis of cervical myelomeningocele and meningocele should include the differential diagnosis and association of many intracranial and spino-skeletal pathogenetic variants and genetic diseases, including subependymal nodular heterotopia and Klippel-Feil syndrome. In this report, a comprehensive review of fetal cervical myelomeningocele with its prenatal diagnosis and long-term outcomes is presented.

Adult-onset Sacral Meningocele Causing a Specific Headache Triggered by Compression or Adoption of a Sitting or Supine Posture.

We report a rare case of adult-onset sacral meningocele where compression triggered a specific headache. A 46-year-old woman presented with a headache, which worsened when she was in a sitting or supine position. A subcutaneous mass was observed on her left buttock, the compression of which also induced headache. No neurological deficits were evident. Lumbar and sacral magnetic resonance imaging demonstrated a meningocele in the left dorsal buttock, connecting to the sacral cerebrospinal fluid (CSF) space, and spinal computed tomography revealed sacral dysplasia. Initial meningocele resection improved the patient's headache, but the cyst recurred 2 years later. Following repeated surgery to reinforce the meningocele orifice, the headache was relieved and has been absent for more than 6 years. The headache was due to intracranial pressure fluctuations due to CSF influx into and drainage from the meningocele. Meningocele development in adulthood can be owing to a spinal bone defect and pressure load on the spinal dura. Surgical resection can improve symptoms resulting from meningocele, and reinforcement of the orifice using an artificial surgical membrane effectively prevents recurrence.

Endoscopic Transnasal Management of Meningoceles and Encephaloceles in Children: A Systematic Review.

OBJECTIVE: To review surgical techniques used in the endoscopic transnasal repair of pediatric basal meningoencephaloceles and compare perioperative outcomes in children <2 and ≥2 years old. DATA SOURCES: MEDLINE, EMBASE, and CENTRAL. REVIEW METHODS: Data sources were searched from inception to August 22, 2022, using search terms relevant to endoscopic transnasal meningoencephalocele repair in children. Reviews and Meta-analyses were excluded. Primary outcomes were the incidence of intraoperative and postoperative complications, including cerebrospinal fluid leak, recurrence, and reintervention. Quality assessments were performed using Newcastle-Ottawa Scale, ROBIN-I, and NIH. RESULTS: Overall, 217 patients across 61 studies were identified. The median age at surgery was 4 years (0-18 years). Fifty percent were female; 31% were <2 years. Most defects were meningoencephaloceles (56%), located transethmoidal (80%), and of congenital origin (83%). Seventy-five percent of repairs were multilayered. Children ≥2 years underwent multilayer repairs more frequently than those <2 years (P = 0.004). Children <2 years more frequently experienced postoperative cerebrospinal fluid leaks (P = 0.02), meningoencephalocele recurrence (P < 0.0001), and surgical reintervention (P = 0.005). Following multilayer repair, children <2 years were more likely to experience recurrence (P = 0.0001) and reintervention (P = 0.006). CONCLUSION: Younger children with basal meningoencephaloceles appear to be at greater risk of postoperative complications following endoscopic endonasal repair, although the quality of available evidence is weakened by incomplete reporting. In the absence of preoperative cerebrospinal fluid leak or meningitis, it may be preferable to delay surgery as access is more conducive to successful repair in older children.

Spontaneous sphenoid sinus meningocele with associated amenorrhea and headache: illustrative case.

BACKGROUND: Developmental meningoceles of the sphenoid sinus are uncommon. When encountered, they are often associated with cerebrospinal fluid (CSF) rhinorrhea. OBSERVATIONS: The authors present the case of a 27-year-old female with a large meningocele eroding through the sella turcica and sphenoid sinus into the nasopharynx. The patient presented with intractable headaches and amenorrhea without CSF rhinorrhea. LESSONS: The patient underwent an endoscopic endonasal transsphenoidal reduction of the meningocele with reelevation of the pituitary gland and skull base reconstruction with abdominal fat graft and nasoseptal flap.

Pharmacoeconomic Evaluation of Costs of Myelomeningocele and Meningocele Treatment and Screening.

Background: The prevention of myelomeningocele (MMC) and meningocele (MC) is a public health concern. A systematic review on economic factors associated with MMC and MC can help the policy makers to evaluate the cost-effectiveness of screening and treatment. To our knowledge, this is the first systematic review to provide up-to date pharmacoeconomic evidence of all economic studies present in literature on different aspects of MMC and MC. Methods: We searched in the National Health Service Economic Evaluation Database (NHSEED), PubMed, Cost-effectiveness Analysis Registry (CEA Registry), Centre for Reviews and Dissemination (CRD), Health Technology Assessment Database (HTAD), Cochrane Library, and Econlit. The PRISMA guidelines were followed in the search and evaluation of literature. Only articles in English not limited by the year of publication that fulfilled the eligibility criteria were included in this systematic review. Results: Nineteen papers were included in the study. The studies were very heterogeneous and reported a comparison of the costs between prenatal versus postnatal repair, the cost of fetoscopic approach versus open surgery, the cost of ventriculoperitoneal shunting (VPS) versus endoscopic third ventriculostomy (ETV), and ETV with choroid plexus cauterization (ETV/CPC), the cost of hospitalization, and the cost of diagnosis for MMC. Conclusion: The results of this study can help in implementing new policies in different countries to assist MC and MMC patients with the cost of treatment and screening.

Anesthesia management for thoracoscopic resection of a huge intrathoracic meningocele: a case report.

BACKGROUND: Diagnosed intrathoracic meningocele is an uncommon complication of neurofibromatosis type 1. We report an anesthesia management for a rare case undergoing thoracoscopic resection of a huge intrathoracic meningocele. CASE PRESENTATION: A 51-year-old woman was scheduled for thoracoscopic meningectomy under general anesthesia. We monitored intrathecal pressure during anesthesia to prevent a decrease in intrathecal pressure. During surgery, the intrathecal pressure occasionally increased by around 5 cmH2O immediately after the insertion of the drainage tube and occasionally decreased by up to 10 cmH2O during the careful slow aspiration of the cerebrospinal fluid (CSF). The pressure rapidly recovered after the interruption of the procedures. She was discharged on postoperative day 4 without major complications. CONCLUSIONS: The CSF pressure was fluctuated by procedures during thoracoscopic resection of a huge meningocele. A CSF pressure monitoring was useful to detect the sudden change of CSF pressure immediately, which can cause intracranial hemorrhage.

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.

Pathogenesis and treatment of a giant occipital bone defect with meningoencephalocele in an NF1 child: case report and review of the literature.

Autosomal dominantly inherited neurofibromatosis type I (NF1) is a systemic disorder caused by a mutation of a gene on chromosome 17q11.2 and characterized by multiple café-au-lait spots, lentiginous macules, Lisch nodules of the iris, and tumors of the nervous system. Bony manifestations such as scoliosis, dysplasia of the greater sphenoidal wing, tibial pseudoarthrosis, short stature, and macrocephaly have been reported in approximately 50% of patients. However, calvarial bone defects are rare. After screening 324 articles, 23 cases (12 adult and 11 pediatric patients) of occipital bone defects in NF1 patients were selected. All patients had a single/multiple bone defect over the lambdoid suture. Adjacent benign plexiform neurofibromas were observed in 14 patients (60.8%, 7 adults and 7 children); one adult patient was diagnosed with neurofibrosarcoma. Meningoencephalocele over the occipital defect was noted in 8 cases (34.78%, all adults). Cranioplasty was performed in only 17.39% of patients. Histologic examination was performed in 7 of the 15 patients with associated neurofibromas/neurofibrosarcomas. Biopsy of the bone margins surrounding the defect was performed in only one case. Pathologic examination of the herniated parieto-occipital or cerebellar tissue was not performed in any of the patients studied. We report the case of a 9-year-old girl with NF1 and a significant occipital bone defect and performed a systematic review of the relevant literature to highlight the challenges in treating this condition and to investigate the underlying mechanisms contributing to bone defects or dysplasia in NF1.

Dandy-Walker syndrome associated with a giant occipital meningocele: A case report and a literature review.

Background: Dandy-Walker malformation or syndrome is the most common posterior fossa malformation. It is commonly associated with other congenital anomalies such as cardiac defects; however, association with a giant occipital meningocele is extremely rare, as only around 34 cases have been described. Case description: We report a case of a 2-month-old female infant who presented with a midline, gigantic mass in the back of the head. It was first discovered on a prenatal ultrasound. The mass measured about 15 × 5 cm, extending to the midback, not changing in size with crying, not attached to the back, and with a positive transillumination test. The diagnosis was confirmed after doing a brain computed tomography, which revealed hypoplasia of the vermis with an enlarged posterior fossa as well as cystic dilation of both ventricles with herniation through a bone defect. Conclusion: Our case highlights a rare association between giant occipital meningocele and Dandy-Walker syndrome that is rarely discussed or reported in the medical literature. By reporting this extremely rare case of Dandy-Walker syndrome associated with a giant occipital meningocele, we hope to contribute to the creation of a database for future research so that a management protocol can be established for use by clinicians and neurosurgeons for better management of the condition.

Seizures as the Initial Manifestation of Idiopathic Intracranial Hypertension Spectrum Disorder.

Idiopathic intracranial hypertension (IIH) is a syndrome of isolated elevated intracranial pressure of unknown aetiology. The IIH spectrum has evolved over the past decade making the diagnosis and management more challenging. The neurological examination in IIH is typically normal except for papilloedema and possible cranial nerve 6 palsy. Recent publications have highlighted skull base thinning and remodelling in patients with chronic IIH. Resulting skull base defects can cause meningo-encephalocoeles, which are potential epileptogenic foci. We describe the clinical and radiological characteristics of five IIH patients with seizures and meningo-encephalocoeles as the presenting manifestations of IIH spectrum disorder.

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.

Anterolateral Thoracic Myelomeningocele With Split Cord Malformation.

We present a case of a two-year-old male with a history of congenital scoliosis and anterolateral thoracic meningocele. He was able to walk and run, but his parents reported left leg weakness and a frequent cough. The patient had normal neurological examination findings. Magnetic resonance imaging (MRI) of the spine without contrast showed left convex upper thoracic congenital scoliosis and rightward anterolateral meningocele inferiorly to T3, with the spinal cord tethered at this location. Neurosurgical cord detethering and repair of the meningocele were performed simultaneously with scoliosis repair by orthopedics. During the dissection of the meningocele, the bulging neural tissue was found to be a split cord ending in a blind stump. The split cord was determined to be nonfunctional via Prass probe (Medtronic, Minneapolis, MN) stimulation and was subsequently dissected. Detethering of the spinal cord was followed by repair of the dural outpouching and dural closure. The patient was stable post-surgery, but long-term results remain to be seen.

A large growing occipital meningocele with Dandy-Walker syndrome: A case report and review of the literature.

Background: Dandy-Walker syndrome (DWS) is a well-known developmental anomaly. An occipital meningocele (OMC) is recognized as a malformation that is relatively often associated with DWS, but the association of DWS with OMC has been reported in approximately 40 cases. We present herein a rare clinical course of DWS with OMC, in which the sac was small at birth and became progressively larger. Case Description: A 5-day-old baby boy was referred to our hospital due to OMC. He was born at 33 gestational weeks due to premature rupture of the membranes. He was diagnosed as having DWS associated with OMC. The OMC was covered with skin and its maximum diameter at birth was 3 cm. Magnetic resonance imaging showed an occipital bone defect and continuity of the fourth ventricle, posterior fossa cyst, and OMC sac. The aqueduct was patent, and no hydrocephalus was found. The OMC sac increased progressively with moderate hydrocephalus and reached 7 cm at the age of 54 days when his weight was 2508 g. A cystoperitoneal shunt and repair were performed after sinus venography by contrast computed tomography (CT). At the age of 1 year and 8 months, he had moderate developmental disabilities. Conclusion: In most cases reported, the OMC was relatively small, and large and giant sizes were reported in only six cases. Almost all cases remained the same size as at birth and underwent surgical intervention as early as possible. It was possible to understand the relationship between the occipital bone defect and abnormal running of sinuses such as the superior sagittal sinus, torcular Herophili, and transverse sinus preoperatively from the CT venography (CTV) image. CTV may be an effective and important method for safely performing repair and shunt.

Fallopian Canal Meningocele Causing Cerebrospinal Fluid Rhinorrhoea.

Fallopian canal meningocele is an extremely rare cause of cerebrospinal fluid rhinorrhoea. Also, due to complex anatomical relations and a lack of experience, its management remains a challenge. Here we report a case focusing on its clinical course, radiological features, and management.

Superinfected and Ruptured Occipital Meningocele: Case Report.

Meningocele is a rare congenital malformation of the central nervous system resulting from a herniation of the meninges containing cerebrospinal fluid through a bony defect in the skull. It is part of neural tube closure anomalies. The overall incidence of meningocele is 0.8 to 3 per 10 000 live births; it varies based on geographical location and race, with a predominance of occipital localization. Among diagnostic methods, computed tomography (CT) and magnetic resonance imaging (MRI) remain crucial examinations. Prenatal diagnosis of this malformation primarily relies on ultrasound and screening through maternal serum alpha-fetoprotein (AFP) levels. They typically manifest very early at birth with a highly suggestive radioclinical presentation. Surgical treatment usually allows for a definitive cure. Superinfection and rupture of the meningocele are exceptional, resulting from delayed diagnosis and management. In several developed countries, prevention through genetic counseling and the intake of folic acid during the periconceptional period, along with accurate prenatal diagnosis and the legalization of therapeutic abortion, has led to a decrease in the prevalence of meningoceles. In less developed countries, where there is a delay in diagnosis in exceptional cases, superinfection and rupture may occur. We present the case of an 8-month-old infant with a complicated occipital meningocele with superinfection and rupture.

State of the Art Bowel Management for Pediatric Colorectal Problems: Spinal Anomalies.

BACKGROUND: Patients with spinal abnormalities often struggle with fecal and/or urinary incontinence (up to 87 and 92%, respectively) and require a collaborative approach to bowel management in conjunction. METHODS: To define existing approaches and propose state-of-the-art bowel management, a literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published July 2013 and July 2023. RESULTS: Patients with spinal anomalies have impaired innervation of the rectum and anal canal, decreasing the success rate from laxatives and rectal enemas. Thus, transanal irrigations and antegrade flushes are widely utilized in this group of patients. Based on spinal MRI, the potential for bowel control in these children depends on age, type, and lesion level. On referral for bowel management, a contrast study is performed to assess colonic motility and evacuation of stool, followed by a series of abdominal X-rays to define colonic emptying and adjust the regimen. The options for management include laxatives, rectal enemas, transanal irrigations, antegrade flushes, and the creation of a stoma. Approximately 22-71% of patients achieve social continence dependent on the type and level of the lesion. CONCLUSION: Patients with spinal anomalies require a thorough assessment for continence potential and stool burden prior to initiation of bowel management. The optimal treatment option is defined according to the patient's age, anatomy, and mobility. The likelihood of independent bowel regimen administration should be discussed with the patients and their caregivers.

Síndrome de Currarino incompleto. Presentación de un caso

El síndrome de Currarino es una enfermedad hereditaria y de baja incidencia, compuesta por una tríada: estenosis anal, malformación sacro coccígea y masa presacra. Puede cursar desapercibido hasta la adultez y generar subdiagnósticos. Se describe un paciente de 75 años, masculino, piel negra, de procedencia urbana y con antecedentes de hipertensión arterial, quien acudió al hospital por presentar hematuria, dolor en fosa lumbar izquierda y estreñimiento. Se realizaron estudios imagenológicos, como ultrasonido, tomografía de abdomen y resonancia magnética lumbosacra, los cuales condujeron al diagnóstico de tumor renal, síndrome de Currarino incompleto (dado por dos elementos de la triada: malformación sacro coccígea y masa presacra) asociado a otra enfermedad malformativa raquimedular, médula anclada. Son pocos los casos reportados en el mundo (casi 300), por lo que se considera una entidad rara, pero de fácil diagnóstico debido al advenimiento de las nuevas tecnologías en el campo de la imagenología.

Currarino syndrome is a hereditary disease with a low incidence, composed of a triad: anal stenosis, sacrococcygeal malformation and presacral mass. It can go unnoticed until adulthood and generate subdiagnoses. A 75-years-old male, black-skinned, urban origin patient with a history of arterial hypertension is described, who attended the hospital presenting hematuria, pain in the left lumbar fossa, and constipation. Radiological studies such as ultrasound, abdominal tomography and lumbosacral magnetic resonance were performed, which led to the diagnosis of a renal tumor, incomplete Currarino syndrome (given by two elements of the triad: sacrococcygeal malformation and presacral mass) associated with another spinal cord malformation disease, tethered cord. There are few cases reported in the world (almost 300), so it is considered a rare entity, but easy to diagnose due to new imaging technologies.

A rare finding of bilateral facial canal meningoceles involving the tympanic segment in suspected idiopathic intracranial hypertension.

Meningoceles are a common radiological feature found in cases of idiopathic intracranial hypertension (IIH). Rarely, they can affect the facial canal within the petrous temporal bone, leading to symptoms such as facial nerve palsy, hearing loss or meningitis. This is the first case report that describes bilateral facial canal meningoceles involving the tympanic segment of the canal. Prominent Meckel's caves were also seen on MRI, a feature commonly associated with IIH.

Pedunculated Chiari 3 malformation with proatlas defect.

Chiari III malformation is considered to be a rare congenital abnormality in human with very high mortality rates. Seventy percent of Chiari III is found to be associated with C1 arch defect as reported by Cakirer (Clin Imaging 27:1-4, 2003). The herniation of posterior fossa elements or dysplastic neural tissue is a must to stamp it as Chiari 3 malformation. The malformation is a result of the abnormal development of craniovertebral junction (CVJ). The CVJ developed from the occipital somites and the first spinal sclerotome. The major role in the development of the CVJ is played by the fourth occipital somite, which is otherwise known as "proatlas." The Chiari III anomalies are due to a result of proatlas defect, which results from failures of segmentation, failures of fusion of different components of each bone, or hypoplasia and ankylosis. We are presenting a case of a 1-year 4-month-old female child who presented with pedunculated swelling at the suboccipital region. The swelling was cystic and with pulsation. On evaluation, we found Chiari III anomaly with C1 posterior arch deficiency (proatlas defect). He was surgically managed. The outcome of the patient was good. Despite literature concluding Chiari 3 malformation with an unfavorable outcome, however, meticulous management and good pre- and postoperative care, physical therapy, and follow-up are necessary for good outcome.