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Abstract: Presacral tumours are rare developmental tumours associated with midline closure defects. A fifteen-month-old girl presented with pain in the lower abdomen and dysuria. After examination, the child underwent investigations and was diagnosed as a presacral mass with intergluteal extension and caudal mass in the subcutaneous tissue of the left gluteal region. Complete excision of mass along with coccygectomy was done. Histopathology showed presacral as a keratinous cyst and gluteal as a dermoid cyst. Later on, the child also developed a left facial dermoid cyst which was also excised., (Copyright © 2024 Copyright: © 2024 African Journal of Paediatric Surgery.)

Congenital opticmeningoceles were recently described as intraorbital optic nerve cystic lesions, in which neural tissue is found to be part of the lining of the lesion. We describe the case of an eleven-month-old patient with a right opticmeningocele, followed through seven years. He presented with proptosis and hypotropia since birth and imaging exams disclosed an intraconal cystic lesion. A needle puncture revealed a fluid characteristic of cerebrospinal fluid. Surgical drainage performed through a lid crease approach resulted in recurrence of the proptosis. Surgical excision of the capsule finally was definitive, and pathology revealed an epithelial-lined capsule. Eight months after the capsule excision, a strabismus surgery was performed to improve the right hypotropia. Although visual prognosis of opticmeningoceles is poor, a good cosmetic result can be obtained with a multidisciplinary approach involving orbit and strabismus surgeons. The complete capsule excision is believed to be the preferable surgical option for permanently treating the proptosis once needle puncture and cyst drainage are associated with recurrence. The orbital access for approaching the lesion depends on the location of the cyst and should provide wide exposure to avoid new surgical procedures in young patients.

Background: Neurofibromatosis type 1 is a genetic disease that affects multiple organs and systems, leading to various clinical manifestations. In Neurofibromatosis type 1, rare intrathoracic meningoceles often occur alongside bone dysplasia. These meningoceles contain cerebrospinal fluid and can be mistakenly diagnosed as 'pleural effusion'., Case Presentation: In this case report, we mistakenly identified 'cerebrospinal fluid' as 'pleural effusion' and proceeded with drainage. This error posed significant risks to the patient and holds valuable implications for the future diagnosis and treatment of similar patients., Conclusions: In patients with Neurofibromatosis type 1 complicated by spinal deformity, there is a high incidence of intrathoracic meningoceles. Treatment strategies may differ based on the specific features of the lesions, and collaboration among multiple disciplines can significantly improve patient outcomes., (© 2024. The Author(s).)

We highlight an under-recognized epileptic pathology in a 56-year-old left-handed female with progressive right facial numbness and weekly focal seizures characterized by episodic aphasia. She was found to have a left frontoparietal intradiploic meningoencephalocele (IDME). Her only epilepsy risk factor was minor head trauma 10 years prior to presentation. She underwent craniotomy for encephalocele resection and mesh cranioplasty without residual neurological deficits and excellent seizure outcome: at 3-year follow-up, she was still seizure-free since surgery, except for an isolated breakthrough seizure at 7 postoperative months when she discontinued her preoperative regimen of Lacosamide monotherapy. Traumatic IDME is a rare condition and rarely presents with seizures. Symptoms may arise up to decades following minor head trauma and are progressive in nature. The likely definitive treatment is cranioplasty and dural repair with or without resecting the protruding parenchyma., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Diastematomyelia is the asymmetric or symmetric lateral duplication of the spinal cord into two hemicords. Pang divided it into three types: type-1, type-2 and composite split cord malformation (SCM). Composite SCMs are uncommon and are defined by the coexistence of multiple types of split cord with normal cord in between. When partially bridged, they are called a horseshoe cord. We report a case of a young woman who presented with backache and was diagnosed with composite SCM with horseshoe cord and type-2 SCM with intervening normal cord. In our case, 3D-SPACE due to its superior topographical evaluation, allowed us to visualise and characterise the thin meningocoele manque bands and detect horseshoe cords, asymmetric cords and demarcate the precise extent of syrinx, which were suboptimally imaged on isolated T2 and T1WI sequences. If left untreated during surgery, bands can be the potential cause for persistent backache., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: Cerebrospinal fluid (CSF) leaks and meningoencephaloceles originating in the lateral recess of the sphenoid sinus can be challenging. The traditional transpterygoid approach through the pterygopalatine fossa (PPF) is time consuming and places important structures at risk, which can lead to significant morbidity. We report a multi-institutional experience using a simplified, endoscopic modified transpterygoid approach (MTPA), which spares the PPF contents in the management of lateral sphenoid sinus meningoencephaloceles and CSF leaks., Study Design: Multi-Institutional, Retrospective Case Series., Methods: Patients with lateral sphenoid recess CSF leaks and meningoencephaloceles between 2014 and 2020 who underwent the MTPA at two academic medical centers were identified. Repair techniques and outcomes were evaluated., Results: Thirty-three patients underwent the MTPA for management. Skull base reconstruction was performed using a free mucosal graft (24/33, 72.7%), nasoseptal flap (4/33, 12.1%), bone grafts (3/33, 9.1%), and abdominal fat grafts (2/33, 6.1%). Lumbar drains and perioperative intracranial pressure measurements were routinely employed. Postoperative complications were uncommon and included three patients (9.7%) with temporary V2 anesthesia, one patient (3.2%) with prolonged V2 anesthesia, and one patient (3.2%) with subjective dry eye, all of which resolved at 9 months postoperatively. There were no recurrent CSF leaks resulting in a 100% success rate. Average follow-up was 13 months., Conclusion: The MTPA reduces morbidity and greatly simplifies access to the lateral sphenoid sinus for the management of CSF leaks and meningoencephaloceles, without compromising exposure. This technique avoids the need for extensive PPF dissection and should be considered for the management of benign lesions involving the lateral sphenoid sinus., Level of Evidence: 4 Laryngoscope, 131:2224-2230, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc..)

Background: Aspiration pneumonitis is an inflammatory disease of the lungs which is difficult to diagnose accurately. Large-volume aspiration of oropharyngeal or gastric contents is essential for the development of aspiration pneumonitis. The role of cerebrospinal fluid (CSF) rhinorrhea is often underestimated as a rare etiological factor for aspiration in the diagnosis process of aspiration pneumonitis., Case Presentation: We present a case of a patient with 4 weeks of right-sided watery rhinorrhea accompanied by intermittent postnasal drip and dry cough as the main symptoms. Combined with clinical symptoms, imaging examination of the sinuses, and laboratory examination of nasal secretions, she was initially diagnosed as spontaneous sphenoid sinus meningoencephalocele with CSF rhinorrhea, and intraoperative endoscopic findings and postoperative pathology also confirmed this diagnosis. Her chest computed tomography showed multiple flocculent ground glass density shadows in both lungs on admission. The patient underwent endoscopic resection of meningoencephalocele and repair of skull base defect after she was ruled out of viral pneumonitis. Symptoms of rhinorrhea and dry cough disappeared, and pneumonitis was improved 1 week after surgery and cured 2 months after surgery. Persistent CSF rhinorrhea caused by spontaneous sphenoid sinus meningoencephalocele was eventually found to be a major etiology for aspiration pneumonitis although the absence of typical symptoms and well-defined risk factors for aspiration, such as dysphagia, impaired cough reflex and reflux diseases., Conclusions: We report a rare case of aspiration pneumonitis caused by spontaneous sphenoid sinus meningoencephalocele with CSF rhinorrhea, which can bring more attention and understanding to the uncommon etiology for aspiration, so as to make more accurate diagnosis of the disease and early surgical treatment., (© 2021. The Author(s).)

Background: Naso-ethmoidal meningoencephalocele is usually a congenital anomaly consisting of a protrusion of cerebral tissue and meninges into the ethmoidal labyrinth. The condition is a rare cause of structural epilepsy in dogs. We report the clinical presentation, surgical intervention, postoperative complications and outcome in a dog with drug resistant epilepsy secondary to a meningoencephalocele., Case Presentation: A 3.3-year-old male neutered Tamaskan Dog was referred for assessment of epileptic seizures secondary to a previously diagnosed left-sided naso-ethmoidal meningoencephalocele. The dog was drug resistant to medical management with phenobarbital, potassium bromide and levetiracetam. Surgical intervention was performed by a transfrontal craniotomy with resection of the meningoencephalocele and closure of the dural defect. Twenty-four hours after surgery the dog demonstrated progressive cervical hyperaesthesia caused by tension pneumocephalus and pneumorrhachis. Replacement of the fascial graft resulted in immediate resolution of the dog's neurological signs. Within 5 months after surgery the dog progressively developed sneezing and haemorrhagic nasal discharge, caused by sinonasal aspergillosis. Systemic medical management with oral itraconazole (7 mg/kg orally q12h) was well-tolerated and resulted in resolution of the clinical signs. The itraconazole was tapered with no relapsing upper airway signs. The dog's frequency of epileptic seizures was not affected by surgical resection of the meningoencephalocele. No treatment adjustments of the anti-epileptic medication have been necessary during the follow-up period of 15 months., Conclusions: Surgical resection of the meningoencephalocele did not affect the seizure frequency of the dog. Further research on prognostic factors associated with surgical treatment of meningoencephaloceles in dogs is necessary. Careful monitoring for postsurgical complications allows prompt initiation of appropriate treatment.

Congenital optic nerve cystic-like malformations associated with normally developed globes are extremely rare. We describe 3 children who presented since birth with proptosis, and eye motility limitation. MRI showed in all cases that the intraorbital segment of the optic nerves was malformed with large cystic-like lesions in the intraconal segment of the orbit. In all cases, biopsies of the wall of the lesions were positive for glial fibrillary acidic protein. Since this protein is a neurobiomarker that exists only in astrocytes in the central nervous system, nonmyelinating Schwann cells of peripheral nerves, and enteric glial cells, we believe that these lesions represent true opticmeningoceles., (Copyright © 2020 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

An otherwise healthy two-month-old female C57BL/6J mouse presented with a left-sided head tilt. Differential diagnoses included idiopathic necrotizing arteritis, bacterial otitis media/interna ( Pasteurella pneumotropica , Pseudomonas aeruginosa , Streptococcus sp. , Mycoplasma pulmonis and Burkholderia gladioli ), encephalitis, an abscess, neoplasia, a congenital malformation and an accidental or iatrogenic head trauma. Magnetic resonance imaging (MRI) revealed a large space-occupying right olfactory lobe intra-axial lesion with severe secondary left-sided subfalcine herniation. Following imaging, the animal was euthanized due to poor prognosis. Histopathologic examination revealed a unilateral, full-thickness bone defect at the base of the cribriform plate and nasal conchae dysplasia, resulting in the herniation of the olfactory bulb into the nasal cavity. There was also a left midline-shift of the frontal cortex and moderate catarrhal sinusitis in the left mandibular sinus. The MRI and histopathologic changes are consistent with a congenital malformation of the nasal cavity and frontal aspect of the skull known as an ethmoidal meningoencephalocele. Encephaloceles are rare abnormalities caused by herniation of contents of the brain through a defect in the skull which occur due to disruption of the neural tube closure at the level anterior neuropore or secondary to trauma, surgical complications, cleft palate or increased intracranial pressure. The etiology is incompletely understood but hypotheses include genetics, vitamin deficiency, teratogens, infectious agents and environmental factors. Ethmoidal encephaloceles have been reported in multiple species including humans but have not been reported previously in mice. There are multiple models for spontaneous and induced craniofacial malformation in mice, but none described for ethmoidal encephaloceles.

Objective: Nasal meningoencephalocele (encephalocele or cephalocele) is a rare condition with congenital, traumatic, or spontaneous origins. We investigated the clinicopathological characteristics of nasal encephaloceles to improve pathologists' and clinicians' understanding of this disease., Methods: Sixteen patients with nasal encephaloceles were enrolled in this retrospective study investigating the condition's clinical and morphological features., Results: Patients' average age was 37.8 (±20.8) years. The ratio of men to women was 2.2:1, patients' mean age was 47.4 (±11.8) years, and 10/16 patients had spontaneous encephaloceles. All patients with traumatic and spontaneous encephaloceles presented with cerebrospinal fluid leak. In 9/16 patients, the skull defect site occurred on the lateral wall of the sphenoid sinus. Both congenital patients experienced nasal obstruction. Histopathology, herniated tissues were brain and/or meningeal tissue, and the brain tissue was almost mature glial tissue., Conclusion: Nasal meningoencephalocele is a rare condition that can be challenging to diagnose. In patients with recurrent clear nasal discharge or in children with a unilateral nasal mass, a high index of suspicion for encephalocele is essential. In this study, spontaneous cases were most common in adults, and the lateral wall of the sphenoid sinus was the most common location., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Study Design: Retrospective case series., Objective: Anterior sacral meningocele (ASM) is a rare disorder. We reviewed 11 cases of congenital ASM and classified them into three types based on the anatomy and relationship between the cyst and sacral nerve roots., Summary of Background Data: The cohort with ASM is relatively large; the classification is novel and has not been previously reported., Methods: Eleven consecutive patients with ASM who underwent surgery between February 2014 and January 2019 were retrospectively analyzed. They included four males and seven females. The dorsal transsacral approach was adopted in all cases. The follow-up time was at least 3 months., Results: We attempted to classify ASM into three types. Of the 11 cases, six were caudal type, two were paraneural type, and three were nerve-root type. The meningocele was ligated after exploring no nerve involvement, in Type I and II. For Type III, the herniating sac and involved nerve roots were ligated when the nerve roots were indicated as nonfunctional on neurophysiological monitoring; otherwise, the sacral nerve roots were protected and imbricated on the residual sac like a hand-in-glove, and sutured to reconstruct the nerves sleeve. Eight cases were accompanied by tethered cord syndrome (TCS); spinal cord detethering was done with one-stage operation. Ten patients' presenting symptoms improved at 3 to 6 months' follow-up; notably, constipation significantly improved. Only one case accompanied by an epidermoid cyst had a second laparoscopic surgery by a general surgeon., Conclusion: Aim of surgical treatment is to obliterate the communication between the subarachnoid space and herniated sac, detether the spinal cord, and resect the congenital tumor. The new classification helps to recognize the relationship between the meningocele and sacral nerve roots, and subsequently adopt different surgical strategies. We consider the dorsal transsacral approach relatively feasible, safe, and with lower complication., Level of Evidence: 4.

A case of a 31-day-old infant patient with a Tessier 0-14 deformity related to multiple midline deformities is presented. Although Transnasal endoscopic surgery is the mainstay for the treatment of anterior and middle skull base meningoceles, there are complex cases in which a combined and multidisciplinary approach is necessary. The surgical decisions and techniques are described. To date, this is the first patient reported with Tessier 0-14 deformity treated with a combined endoscopic and external surgical approach., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Since the widespread introduction of the conjugate pneumococcal vaccine and subsequent decline of invasive pneumococcal disease in children, a significant proportion of children with pneumococcal meningitis are identified as having an underlying immunodeficiency or predisposing anatomic abnormality. We present 3 cases of recurrent pneumococcal meningitis where detailed neuro-imaging revealed subtle anterior base of skull defects not detected on initial investigations.

Background: Meningocele manqué (MM) is characterized by tethering of the spinal cord, nerve roots, or filum terminale by fibrous attachments formed by atrophic or incomplete meningoceles. Patients with MM can present with symptoms of tethered cord syndrome (TCS)., Case Description: We present the case of an MM discovered incidentally during microsurgical resection of filum terminale for occult TCS. The MM was not visible on the preoperative magnetic resonance imaging (MRI) studies. After L2-L3 laminotomy, an extradural mass was appreciated adherent to the spinal dura, which was found to be an MM. The nerve roots of the cauda equina were found to protrude through the dura, consistent with tethered cauda equina. After microsurgical resection of the filum terminale, the tethered cauda nerve roots were stimulated, and redundant electromyographic signals were detected at both the left- and the right-sided muscles of the lower extremities. Microsurgical repair of the MM was performed, assuming that the patient's symptoms of TCS would also be explained by the MM and that her symptoms would likely have been incompletely addressed by filum terminale release alone. A review of the preoperative 3-dimensional constructive interference in steady state MRI sequence revealed pathological features at the L2-L3 level suspicious for our intraoperative findings of an MM., Conclusions: Surgeons planning filum terminale release for occult TCS should always be aware of the possibility of associated pathological features of tethering that might be overlooked. In retrospect, the detection of MM was enabled by the high-resolution 3-dimensional constructive interference in steady state MRI sequence. This finding advocates for the use of high-resolution MRI for patients undergoing evaluation for occult TCS to detect pathological features that might otherwise be undetected., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Introduction: Cranial nerve lesions can be secondary to a space-occupying lesion of the skull base compressing adjacent nerves., Case Report: We report the case of an 84-year-old man, who presented with rapid and concomitant onset of dysphagia and ipsilateral recurrent laryngeal nerve paralysis, suggesting an isolated lesion of the vagus nerve. MRI revealed a diagnosis of previously unknown clival meningocele., Discussion: Unilateral vagus nerve paralysis constitutes an exceptional mode of presentation of meningocele. Only a few isolated cases of clival meningocele have been reported to date, with no cranial nerve repercussions. The symptomatic management adopted in this case allowed rapid improvement of the patient's disorders., (Copyright © 2019. Published by Elsevier Masson SAS.)

Intraorbital meningoencephalocele is a rare manifestation of neurofibromatosis type 1 (NF1) caused by secondary changes in sphenoid dysplasia, and it seriously affects patients' vision and facial appearance. We retrospectively analyzed the clinical data of 23 patients with NF1 and intraorbital meningoencephalocele, summarized the surgical strategies employed, and evaluated their clinical efficacy in order to better understand its management in clinical practice, establish a reasonable surgical strategy, and assess prognosis. Before surgery, 22 patients had unilateral pulsatile exophthalmos, 18 patients had significant visual impairment, and 13 patients had ptosis associated with an orbital plexiform neurofibroma (PNF). All 23 patients underwent microsurgical craniotomy with skull base reconstruction using a soft titanium mesh. One month after surgery, the degree of exophthalmos in the 22 (95.65%) patients was significantly reduced compared with before surgery (P < 0.001), and ocular pulsation had subsided. The visual acuity did not decrease significantly (P = 0.298) compared with before surgery. Eleven (47.83%) patients received phase-II eyelid PNF resection and/or oculoplastic surgery, and the degree of ptosis was significantly reduced (P < 0.001). There was no recurrence of pulsatile exophthalmos, displacement of titanium mesh, decreased visual acuity, or increased degree of ptosis noted during follow-up. The best strategy is to reconstruct the skull base under microscopy to relieve pulsating exophthalmos and preserve existing visual function. In cases of ptosis caused by an eyelid PNF, surgical resection should be performed as soon as possible to remove the tumor, and/or oculoplastic surgery should be performed to improve the cosmetic outcome.

BACKGROUND Arthrogryposis multiplex congenita is a multifactorial syndromic or non-syndromic group of conditions consisting of multiple congenital contractures of the body, of unknown etiology. It is associated with a heterogenous group of disorders that include but are not limited to processes such as myopathic and neuropathic. Neural tube defect is a neuropathic disorder that incorporates myelomeningocele that might be either isolated or within a spectrum of multiple diseases. CASE REPORT This is a case report of a 28-day-old male born with lower limb arthrogryposis with myelomeningocele and Chiari II malformation in a Mediterranean population. CONCLUSIONS Lower extremity arthrogryposis with myelomeningocele and Chiari II malformation is a prenatal diagnosis that requires high clinical suspicion, early multidisciplinary intervention, and genetic counselling. As long as new approaches are being explored in the management of such cases, babies born now with neural tube defects can expect better quality of life.

Objective: To recount experience with cerebrospinal fluid otorrhoea and temporal bone meningoencephalocele repair in a tertiary care hospital., Method: A retrospective review was conducted of 16 cerebrospinal fluid otorrhoea and meningoencephalic herniation patients managed surgically from 1991 to 2016., Results: Aetiology was: congenital (n = 3), post-traumatic (n = 2), spontaneous (n = 1) or post-mastoidectomy (n = 10). Surgical repair was undertaken by combined middle cranial fossa and transmastoid approach in 3 patients, transmastoid approach in 2, oval window plugging in 1, and subtotal petrosectomy with middle-ear obliteration in 10. All patients had successful long-term outcomes, except one, who experienced recurrence after primary stage oval window plugging, but has been recurrence-free after second-stage subtotal petrosectomy with middle-ear obliteration., Conclusion: Dural injury or exposure in mastoidectomy may lead to cerebrospinal fluid otorrhoea or meningoencephalic herniation years later. Congenital, spontaneous and traumatic temporal bone defects may present similarly. Middle cranial fossa dural repair, transmastoid multilayer closure and subtotal petrosectomy with middle-ear obliteration were successful procedures. Subtotal petrosectomy with middle-ear obliteration offers advantages over middle cranial fossa dural repair alone; soft tissue closure is more robust and is preferred in situations where hearing preservation is not a priority.

Background: An epidermoid cyst is a congenital benign tumor and is extremely rare at the presacral region. Only a few cases have been reported in the literature., Case Description: We herein report a case of a 40-year-old woman with a giant presacral epidermoid cyst mimicking an anterior sacral meningocele who presented with recurrent abortions. This increases risk to the mother and fetus. Magnetic resonance imaging is the investigation of choice., Conclusions: The diagnosis and surgical management of this case are discussed., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Marfan syndrome is a genetic disorder that results in the weakening of connective tissues. Dural ectasia has been defined as a feature of Marfan syndrome and is present in up to 92% of patients. Rarely, dural ectasia can erode through the sacrum expanding into an anterior sacral meningocele., Case Description: Information for this case report was gathered from patient notes and imaging from the patient chart. This is a case of a 46-year-old woman who presented with urinary incontinence, early satiety, and back pain in the setting of a known anterior sacral meningocele. Before operative management, the anterior sacral meningocele ruptured with the patient presenting signs and symptoms of intracranial hypotension. Conservative management did not alleviate the pain. She was ultimately managed with posterior sacroplasty followed by anterior sacral meningocele resection and placement of a lumboperitoneal shunt. The patient did not have reaccumulation of the meningocele or recurrent symptoms at the latest follow-up., Conclusions: The progression of dural ectasia in Marfan syndrome to an anterior sacral meningocele is uncommon. It is important to identify the characteristics associated with an expanding dural ectasia as this patient's symptoms progressed over time and the meningocele grew large. Given its rarity, there are no guidelines in place regarding size at which repair of an anterior sacral meningocele should occur prophylactically. It is important to review these cases in order continue to learn about progression, management, and outcomes of patients with an anterior sacral meningocele., (Copyright © 2018 Elsevier Inc. All rights reserved.)

A 2-year-old male neutered mixed breed dog with a body weight of 30 kg was presented for evaluation of a soft subcutaneous mass on the dorsal midline at the level of the caudal thoracic spine. A further clinical sign was intermittent pain on palpation of the area of the subcutaneous mass. The owner also described a prolonged phase of urination with repeated interruption and re-initiation of voiding. The findings of the neurological examination were consistent with a lesion localization between the 3rd thoracic and 3rd lumbar spinal cord segments. Magnetic resonance imaging revealed a spina bifida with a lipomeningocele and diplomyelia (split cord malformation type I) at the level of thoracic vertebra 11 and 12 and secondary syringomyelia above the aforementioned defects in the caudal thoracic spinal cord. Surgical resection of the lipomeningocele via a hemilaminectomy was performed. After initial deterioration of the neurological status postsurgery with paraplegia and absent deep pain sensation the dog improved within 2 weeks to non-ambulatory paraparesis with voluntary urination. Six weeks postoperatively the dog was ambulatory, according to the owner. Two years after surgery the owner recorded that the dog showed a normal gait, a normal urination and no pain. Histopathological diagnosis of the biopsied material revealed a lipomeningocele which confirmed the radiological diagnosis., Competing Interests: The authors declare no conflicts of interest related to this report., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: The finding of pseudomeningocele from cervical myelogram is widely accepted as a pathognomonic sign for diagnosing root avulsion in brachial plexus injury. In general, motor power in this setting should be absent. However, in clinical practice, we observed that motor power in some patients was still preserved. The objective of this study is to evaluate the accuracy of pseudomeningocele from cervical myelogram for predicting root avulsion in brachial plexus injury., Methods: We retrospectively reviewed 201 patients with brachial plexus injury from 2007-2011. Four patients were excluded due to open wound injury. Motor power of the key muscle of each nerve root was evaluated by skilled hand surgeons. All cervical myelogram was interpreted by radiologists. Sensitivities, specificities, positive predictive values and negative predictive values were calculated with 95% confidence interval., Results: Thirty and 29% of pseudomeningocele occurred at C7 and C8 level, respectively. The sensitivity of pseudomeningocele of each root from C5 to T1 was low (range, 10-62%). The specificity was high only at C5 (91%) and T1 (96.2%). Over 20% of patients with pseudomeningocele at C6-8 levels had some motor function. The initial muscle power of these patients was M1 or M2 and 70% of them recovered to at least M3 at the final follow-up., Conclusions: Pseudomeningocele is not an absolute sign for diagnosing of root avulsion in brachial plexus injury due to its high false positive rate when compared with preoperative motor function of each root. Careful examination of the key muscle of each root is extremely crucial to prevent unnecessary operation on that cervical nerve root.

Introduction Orbital meningoencephalocele formation is primarily a result of congenital defects in the pediatric population and trauma of the anterior cranial fossa in adults. We present a unique case of nontraumatic nasal and orbital meningoencephaloceles presenting as bilateral proptosis with exotropia secondary to chronic hydrocephalus. Clinical presentation A 20-year-old male with a history of tuberous sclerosis, X-linked intellectual disability, and epilepsy presented to the emergency department with two days of nausea, emesis, seizures, and two months of progressive proptosis. Initial radiographs of the skull showed a "copper beaten" appearance, indicating chronically elevated intracranial pressure. Computed tomography imaging of the head demonstrated bilateral defects in the cribriform plate and anterior cranial fossa. Magnetic resonance imaging of the brain revealed triventricular hydrocephalus with meningoencephalocele extension into the nasal cavity and frontal horn herniation into the extraconal space of the orbits. The hydrocephalus was managed with ventriculoperitoneal shunt placement with rapid and complete resolution of the proptosis. Conclusion No reports have described bilateral proptosis as the presenting finding of orbital and nasal meningoencephaloceles in the absence of trauma or congenital defect. This case study demonstrates the management of meningoencephalocele formation secondary to chronic hydrocephalus.

Context: Caudal regression syndrome is a rare disorder, not well described in the literature., Findings: Authors treated two patients with congenital absence of thoracolumbar vertebrae and lower limbs paraplegia. Patients had hypoplasia of the lower trunk and extremities with motion between upper and lower torso. Imaging showed caudal spine agenesis, but cleft sacrum was present. Due to severe kyphotic deformity and spinal instability, deformity correction and posterior fusion was performed at the age 6 and 8. Finally, fusion was achieved in one case and stable but non-fusion kyphotic posture was observed in second., Conclusion: surgery in caudal regression syndrome is challenging and bears high risk of complications.

Background: Pseudomeningoceles most commonly occur due to prior trauma or surgery and are often located in the posterior paraspinous tissues. Here, we report a case of an intraosseous pseudomeningocele that mimicked an intra-osseous T2 hyperintense lesion in the L1 vertebral body., Case Description: A 64-year-old male presented with back, left lateral thigh and left knee pain lasting several months. He had no prior history of trauma or surgery. Radiographs of the lumbar spine showed mild levoscoliotic curvature of the lumbar spine, Baastrup's changes between the spinous processes, multilevel degenerative disc disease and facet arthropathy. Magnetic resonance imaging (MRI) of the lumbar spine performed without intravenous contrast showed severe spinal canal stenosis from L1-L2 to L3-L4 and moderate spinal canal stenosis at L4-L5. MRI also showed a 2.5-cm T2 hyperintense lesion involving the posterior aspect of the L1 vertebral body, with questionable contiguity with cerebrospinal fluid. Computed tomography (CT) myelogram was performed instead of biopsy. CT myelogram showed contiguity of the lesion with the intrathecal contrast and a rent in the posterior longitudinal ligament and anterior dura consistent with an intraosseous pseudomeningocele. The patient opted for non-operative management of the pseudomeningocele and his lumbar stenosis due to medical comorbidities., Conclusions: This case illustrates a rare case of an intra-osseous pseudomeningocele and highlights the importance of CT myelogram for diagnosis.

Purpose: To report a case of anterior sacral meningocele with intralesional bleeding secondary to sacrococcygeal trauma. Likewise, there is a discussion about the physiopathology and the surgical approach to these types of lesions., Methods: A 43-year-old man diagnosed with Marfan syndrome suffered sacrococcygeal trauma. He was admitted to the emergency room due to symptoms of headache, nausea, and lower limb subjective weakness. CT and MRI showed a large retroperitoneal mass with hemorrhagic content close to the sacrum. Likewise, the MRI showed an image compatible with subarachnoid hemorrhage in the thoracic spinal area, cerebral convexity, and the basal cisterns. The patient went into surgery for an anterior abdominal approach in the midline to reduce the content of the lesion, and subsequently, in the same act, a posterior approach was done with an S1-S2 laminectomy and obliteration of the pedicle. Postoperative MRI 5 months later showed resolution of the ASM., Results: Anterior sacral meningocele is characterized by herniation of the dura mater and the arachnoid mater outside the spinal canal through a defect of the sacrum. We add the risk of bleeding after trauma-never seen in the literature-as one of the possible inherent complications of this lesion., Conclusions: This report highlights a complication never seen in the literature of a relatively rare condition. In our case, the combined approach was effective for both clinical control and lesion regression.

Background: Cerebrospinal fluid (CSF) leaks are a dreaded complication after surgery for tethered cord and are associated with significant patient morbidity. Although many strategies for managing postoperative CSF leaks exist, this problem is still daunting, especially in very young patients. In this study, we compared different management techniques for CSF leaks or significant pseudomeningocele in patients with tethered cord syndrome (TCS)., Methods: We analyzed a cohort of children who underwent surgery for TCS from January 2011 to March 2016 (n = 260) and postoperatively experienced either a CSF leak or significant pseudomeningocele. A subset of patients presented with CSF leak (n = 25). We analyzed patient age, sex, presentation, leak appearance, management, and outcome. The different techniques of management were compared for efficacy and morbidity., Results: The diseases associated with leak formation included lipomyelomeningocele (n = 16), myelocystocele (n = 4), and myelomeningocele (n = 5). Three children also had hydrocephalus. Management techniques included cystoperitoneal shunt (CPS) (n = 15), primary resuturing with local rotation flap of muscle (n = 3), external ventricular drain placement (n = 1), ventriculoperitoneal shunt (n = 3), external ventricular drainage (n = 1), and a combination of techniques (rotation flap with external drain; n = 1). Five patients who underwent primary wound revision experienced a leak and required a secondary intervention, but none of the patients who underwent CPS had any complications., Conclusions: In carefully selected cases, CPS performed early after CSF leakage is highly successful with low morbidity. The primary closure can be attempted for low-pressure leaks without an associated pseudomeningocele., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: Type 1 neurofibromatosis, also called "Recklinghausen's disease" is among the most frequent autosomal dominant genetic disorders, with an incidence of 1:3500 births. It mainly affects the skin and peripheral nervous system. However, in its less frequent manifestations, are tumors such as meningocele and skeletal dysplasias leading to severe clinical presentation., Case Report: We report the case of a 55-year-old patient with type 1 neurofibromatosis and dyspnea due to a large left thoracic meningocele combined with a significant kyphoscoliosis, causing a severe restrictive ventilatory defect, complicated by chronic respiratory failure and pulmonary hypertension. Symptomatic treatment with non-invasive ventilation permitted an improvement of the clinical situation., Conclusions: Our observation shows the complexity of the therapeutic support of the neurofibromatosis of type 1. The contribution of non-invasive ventilation was illustrated by the arterial blood gas and clinical improvements as well as improved quality of life, with an acceptable level of inconvenience to the patient., (Copyright © 2018 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Background: Anterior sacral meningoceles are rare, and usually occur with other malformations of the posterior lower spine. While these are more frequently reported in pediatric cohorts, we report a case in an elderly woman., Case Presentation: We report on a 71 year-old woman with a recently diagnosed colorectal adenocarcinoma who presented with a severe bacterial meningitis. The cerebrospinal fluid cell count revealed a pleocytosis of 80,000 cells/μl and a severe disturbance of the blood-brain-barrier. Fusobacterium nucleatum was cultured as the causing pathogen. A lumbar MRI showed, in addition to contrast-enhancing meninges as sign of inflammation, a presacral mass. In the next step, the mass was diagnosed as an anterior sacral meningocele connected to the gut. An adequate antibiotic was used to treat the leptomeningitis. The connection between gut and meningocele was closed surgically and the patient recovered well and underwent further treatment of her colorectal adenocarcinoma., Conclusion: We report on a case of meningitis with an anterior sacral meningocele that was connected to the gut in a patient with a infiltrative colorectal adenocarcinoma. Anatomic variants have to be considered as rare causes of meningitis with typical intestinal germs.

A 10-year-old boy with bilateral colobomatous cavitary disc anomalies presented with a 3-month history of vision loss in his right eye. MRI of the head and orbit revealed bilateral tubular cystic enlargement of the optic nerve/optic sheath complex with thickening of the optic nerves without inflammation or neoplasm, suggestive of bilateral optic nerve meningocele. An optic nerve sheath fenestration was performed OD, and he experienced an improvement and stabilization of vision in his right eye during a 1-year follow-up period. The authors recommend that surgical decompression, particularly optic nerve sheath fenestration, should be considered in cases with progressive vision loss due to optic nerve meningocele.

Temporal bone encephalomeningoceles are uncommon clinical entities that can be challenging to diagnose and treat. Common presenting complaints include symptoms of aural fullness, hearing loss, clear otorrhea and meningitis. Common etiologies are chronic middle ear disease and trauma. A significant proportion of cases are spontaneous, which occurs in relation to benign intracranial hypertension. Normal pressure hydrocephalus associated with temporal bone encepahomeningoceles has not been previously described. The authors present an unusual case of spontaneous right sided temporal bone encephalomeningocele associated with symptomatic normal pressure hydrocephalus. Surgical repair of the defect was successful. The diagnostic and management approach to this unique case are discussed., (Copyright © 2017 Elsevier Inc. All rights reserved.)

The authors report a case of a 70-year-old man who presented with a long history of left pulsatile proptosis, irritation, and a pressure sensation behind the eye. Initial computed tomography brain demonstrated the presence of a suspected ethmoid sinus mucocele. Endoscopic sinus surgery was performed to drain the mucocele. On cautious opening of the nasal mucosa, cerebrospinal fluid was encountered requiring subsequent repair with local mucosal flap and fat graft. On further magnetic resonance imaging, the lesion was confirmed to be giant pseudomeningocele arising from the sphenoid sinus, with extensive invasion and erosion of the middle cranial fossa including the left lateral orbital wall. Here, the authors report, to the best of their knowledge, the first case of spontaneous giant pseudomeningocele in the middle cranial fossa causing pulsatile proptosis. In examining this case, the authors will also discuss the pathogenesis, diagnosis, and management of giant pseudomeningoceles, especially in the situation of a misdiagnosis.

Introduction: Frontoethmoidal meningoencephalocele (FMEC) is a rare congenital anomaly characterized by herniation of brain tissue and meninges through a defect in the cranium and associated with facial dysmorphism. Treatment modalities include extra cranial, transcranial, or combined craniofacial approaches. The combined approach is considered the best treatment choice., Methods: Twelve patients with FMEC aging from 6 months to 4 years were treated by single-stage combined craniofacial approach between July 2011 and July 2015. They were followed up for evaluation of outcome and detection of complications., Results: Seven patients (58.3%) were males and 5 patients (41.7%) were females. Eight patients (66.7%) were less than 2 years while 4 patients (33.3%) were between 2 and 4 years. The main presentations were external mass, telecanthus and hypertelorbitism, radiologically, frontobasal bone defect and herniated dural sac with brain tissue were detected in all patients. Excision of the mass with dural repair, craniofacial reconstruction, and medial canthopexy were done for all patients. Orbital translocation was done for 8 patients (75%), nasal reconstruction for 7 patients (58.3%), while dacryocystorhinostomy in 3 patients (25%). Venticuloperitoneal shunt was done before correction of FMEC in one patient (8.3%). The follow-up period ranged from 6 to 48 months with mean 29.2 months. The esthetic results were satisfactory in 9 patients (75%). Ugly facial scars were recorded in 3 patients (25%)., Conclusion: Early surgical management for FMEC is advisable to avoid deleterious effects on facial growth. Meticulous perioperative care is important for successful surgery. The authors recommend combined craniofacial approach to achieve good outcome and decrease the incidence of complications.

Introduction: Skull base meningoencephaloceles are rare congenital malformations. The two cases of transalar transsphenoidal malformation reported here differ from the classical transsphenoidal meningoencephalocele., Case Reports: Case 1 was a three-and-a-half-year-old boy and case 2 was a 36-year-old man. Both cases presented with clinical features of recurrent meningitis. Surgical management of case 1 was performed via an intradural infratemporal fossa craniotomy with reoperation 2years later. In the second case, surgery was initially performed via an endonasal approach and then via pterional craniotomy. Reoperation via Sekhar's transpetrosal approach was also a failure. Only closure of the trigeminal-pontine angle via a suboccipital retrosigmoid approach allowed repair of the defect., Discussion: Surgical access to the pterygopalatine fossa is complex due to its anatomical position and its anatomical relations with nerves and vessels. An endoscopic approach appears to be a valuable alternative to classical craniotomy., Conclusion: In the two cases reported here, neurosurgery allowed lasting closure of the defect., (Copyright © 2016. Published by Elsevier Masson SAS.)

Meningohydroencephalocele is a herniation of meninges, cerebrospinal fluid, brain parenchyma and a part of the ventricular system through a bony defect in the skull. This bone defect may be congenital, spontaneous or traumatic in origin. The lesions are mostly congenital, discovered generally after birth or in very young infants. We report the first historical case of the entity in this location in a 29-year-old man and discuss the pathogenesis, surgical management and social considerations of this type of neural tube defect in our country., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

The clinical case is presented on a patient with an extensive sacral dysraphism, a history of myelomeningocele surgical repair in her childhood, as well as tethered cord syndrome. The patient was also diagnosed with pseudomeningocele and a cerebrospinal fluid cutaneous fístula. A surgical approach was used, with encouraging results being obtained in the clinical outcome of the patient. A review of the literature was performed to support the surgical decision in this case., (Copyright © 2015 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.)