Extradural spinal cyst in a pediatric patient: A case report.

Background: Spinal extradural arachnoid cysts comprise <1% of all spinal lesions and are rare findings in pediatric patients. The pathogenesis of spinal extradural arachnoid cysts is not well known but is thought to most commonly be due to congenital dural defects. Other origins include trauma, inflammation, or infection, such as arachnoiditis. Spinal magnetic resonance imaging is the gold standard for diagnosis, showing a fluid-filled space dorsal to the spinal cord with signal intensity akin to cerebrospinal fluid (CSF) and often the site of dural defect with CSF leak. While most spinal extradural arachnoid cysts are asymptomatic, large cysts can compress the spinal cord or nerve roots, leading to myelopathy, radiculopathy, or focal pain symptoms. In such cases, surgical management is indicated. Case Description: Here, we present a case of a 15-year-old female who presented with lower back pain radiating to her bilateral posterior thighs and knees, with imaging indicating a thoracolumbar spinal extradural arachnoid cyst. After failed conservative treatment, surgical intervention in the form of laminectomy, fenestration of the arachnoid cyst, and repair of the dural defect was required, resolving the patient's symptoms with no recurrence of the cyst. Conclusion: Complete resolution of pain in our patient following surgical management of spinal arachnoid cyst suggests that treatment of the arachnoid cyst can be achieved through minimal exposure to the site of the CSF leak to fenestrate the cyst and repair the leak.

A Subdural Hygroma Necessitating a Subdural-Peritoneal Shunt in a Pediatric Patient Following Total Cranial Vault Remodeling Surgery.

Sagittal synostosis is a common non-syndromic synostosis treated with open or endoscopic cranial vault remodeling. Early intervention is recommended to avoid restricted brain growth, increased intracranial pressure, and resultant developmental delay. Common complications such as failure or reconstruction, cerebrospinal fluid leak, blood loss, and stroke are well-reported in the literature. Here, we present a rare case of the development of a subdural hygroma following cranial vault remodeling in a seven-month-old male, necessitating the insertion of a subdural-peritoneal shunt.

Pediatric abusive head trauma: ThinkFirst national injury prevention foundation.

BACKGROUND: Pediatric abusive head trauma (AHT) represents 80% of nonaccidental trauma deaths, remaining a lead cause of death among infants and young children. Furthermore, neurosurgical intervention can ameliorate damage from secondary injury, but we are currently unable to alter the impact of the primary injury. Thus, prevention through increased public awareness is imperative. This study identifies injuries and predictors of outcomes in pediatric AHT and highlights the importance of partnering with our community through ThinkFirst, a national injury prevention foundation, to educate parents and caregivers about prevention. METHODS: This single-institution retrospective review identifies injuries and predictors of outcomes in pediatric AHT and highlights the importance of partnering with our community to raise awareness and educate parents and caregivers about prevention. RESULTS: The number of pediatric AHT cases continues to steadily increase over time (P < 0.001), and over 70% of these patients are <1 year of age (P < 0.001). Patients suffering AHT have a mortality rate of nearly 10%. In addition to morbidity and mortality, the economic burden of caring for abused children is high as they often require high levels of care, long hospital stays, and extensive rehabilitation. Furthermore, Medicaid pays for nearly 80% of these patients. CONCLUSION: The population of patients with AHT is unique, and one that will benefit from continued efforts at increased multidisciplinary and public awareness. Prevention of AHT through awareness is critical. Through partnering with ThinkFirst, a national injury prevention foundation, we aim to educate parents and caregivers about prevention.

Pediatric Temporal Lobe Meningioma With Meningioangiomatosis Mimicking Invasive Meningioma.

Meningiomas combined with meningioangiomatosis (MA-M) present similarly to more invasive lesions because of their appearance on neuroimaging. These lesions are especially rare in pediatric patients and suggestive imaging can help identify them for differential diagnosis. An 11-year-old male child who presented with diplopia and a headache was found to have an edematous invasive appearing temporal lobe mass on magnetic resonance imaging. Despite the lesion's appearance, it was completely resected and found to be a benign MA-M upon histopathologic examination. The present case demonstrated a rare meningioma with meningioangiomatosis that appeared to be a higher grade or invasive lesion upon initial imaging in a pediatric patient. A review of the literature was performed on patients who presented similarly. Despite the rarity of this condition in children, neuroimaging should be carefully examined prior to surgical resection of similar masses in preparation for highly vascular tissue, and post-operative course can be better anticipated when MA-M is considered during differential diagnosis.

Paraspinal plexiform schwannoma of unknown nerve origin: A case report.

INTRODUCTION AND IMPORTANCE: Schwannomas are benign, slow-growing nerve sheath tumors of neoplastic Schwann cells. They are the most common peripheral nerve tumors in adults and are typically discovered incidentally due to their asymptomatic presentation. Despite the fact that most schwannomas are unassociated with a syndrome, their etiology is thought to be related to alterations or loss of the neurofibromatosis type two tumor suppressor gene. CASE PRESENTATION: We present the case of a fifteen-year-old female who presented with a recurrent lower back/upper buttocks 9 cm mass with imaging suspicious for schwannoma. Needle biopsy revealed an S100 positive cellular schwannoma with patchy Ki-67. During surgical dissection down to the sacrum, no nerve of origin was identified. CLINICAL DISCUSSION: Schwannomas have no pathognomonic findings on MRI and may occur at any location that Schwann cells are present; therefore, confirming a diagnosis relies on histopathology. Plexiform schwannomas are defined by a "network-like" intraneural growth pattern and are exceedingly rare in paediatric populations. A location distinct from the spinal canal is also very rare as schwannomas typically originate from the head and neck region. CONCLUSION: Paediatric plexiform schwannomas have been rarely reported. Surgical planning relies on multiple factors such as tumor size, tumor location, pathologic features and symptomatic burden. The distinctive features of this case including an unknown nerve origin and a location outside the spinal canal provide a unique opportunity to discuss the diagnosis and management of paraspinal schwannomas and the impact on operative planning when a nerve of origin is not identified.

Anesthetic Management of Awake Craniotomy for Resection of the Language and Motor Cortex Vascular Malformations.

BACKGROUND: Although the safety and feasibility of awake craniotomy are well established for epilepsy and brain tumor surgery, its application for resection of vascular lesions, including arteriovenous malformations (AVMs) and cavernomas, is still limited. Apart from the usual challenges of awake craniotomy, vascular lesions pose several additional problems. Our goal is to determine the safety and practicality of awake craniotomy in patients with cerebral vascular malformations located near the eloquent areas, using a refined anesthetic protocol. METHODS: A retrospective case series was performed on 7 patients who underwent awake craniotomy for resection of AVMs or cavernomas located in the eloquent language and motor areas. Our protocol consisted of achieving deep sedation, without a definitive airway, using a combination of propofol, dexmedetomidine, and remifentanil/fentanyl during scalp block placement and surgical exposure, then transitioning to a wakeful state during the resection. RESULTS: Six patients had intracranial AVMs, and 1 patient had a cavernoma. Six patients had complete resection; however, 1 patient underwent repeat awake craniotomy for residual AVM nidus. The patients tolerated the resection under continuous awake neurologic and neurophysiologic testing without significant perioperative complications or the need to convert to general anesthesia with a definitive airway. CONCLUSIONS: Awake craniotomy for excision of intracranial vascular malformations located near the eloquent areas, in carefully selected patients, can facilitate resection by allowing close neuromonitoring and direct functional assessment. A balanced combination of sedative and analgesic medications can provide both adequate sedation and rapid wakeup, facilitating the necessary patient interaction and tolerance of the procedure.

Saksenaea infection masquerading as a brain tumor in an immunocompetent child.

Saksenaea species are a rare cause of mucormycosis, the majority associated with cutaneous and subcutaneous infections resulting from trauma in both immunocompromised and immunocompetent individuals. Unlike other causative agents of mucormycosis, cerebral infections are exceptionally rare. We describe the first case of isolated cerebral infection by Saksenaea in a 4-year-old previously healthy male child who presented with headaches. He had no past medical history other than an episode of febrile seizures. In addition to raising the awareness of an unusual presentation of infection by Saksenaea, this case highlights the importance of pathologic examination for the prompt diagnosis of mucormycosis as well as the specific fungal identification for treatment as Saksenaea spp. may be more susceptible to posaconazole and less susceptible to amphotericin B compared to more common causes of mucormycosis.

Benign Giant Cell Lesion of C1 Lateral Mass: A Case Report and Literature Review.

Primary osseous tumors of the spinal column account for approximately 1% of the total number of spinal tumors found in the pediatric patient population. The authors present a case of a C1 benign giant cell lesion that was incidentally found in a 15-year-old patient. A transoral biopsy was performed followed by treatment with denosumab, with definitive management in the form of transoral tumor resection with subsequent occiput-cervical three posterior instrumented fusion. The patient tolerated all of the procedures well, as there were no post-operative complications, discharged home neurologically intact and was eager to return to school when assessed during a follow-up visit in clinic. Osteolytic lesions affecting the cervical spine are rare in the pediatric population. It is of utmost importance to have sufficient background knowledge in order to formulate a differential diagnosis, as well as an understanding of principles underlying surgical techniques required to prevent occipital-cervical instability in this patient population. The information presented will guide surgical decision-making by identifying the patient population that would benefit from neurosurgical interventions to stabilize the atlantoaxial junction, in the context of rare osteolytic conditions affecting the cervical spine.

Pial-Dural Intracranial Arteriovenous Fistula with Flow-Associated Aneurysmal Rupture-Case Report with Review of Literature and Proposal on the Mechanism of Hemorrhage and Treatment Options.

BACKGROUND: Vascular anomalies in the form of dural arteriovenous fistulas (DAVFs), arteriovenous malformations, and aneurysms are well described in the literature. Pial arteriovenous fistulas (PAVFs) are described to a lesser extent in the literature. When these anomalies are combined, diagnosis and treatment become complex. CASE DESCRIPTION: A 55-year-old man presented with PAVF/DAVF with a ruptured flow-related aneurysm in the distal left posterior inferior cerebellar artery, which required surgical clipping and disconnection. We performed a PubMed search of all identifiable cases of PAVFs in adults. We then identified which cases had components of DAVFs and flow-related aneurysms. We identified 51 PAVF cases and identified which patients presented with hemorrhage and the treatment modalities of all cases. Of 51 cases identified, 4 were a DAVF/FRA, and 5 were a PAVF/DAVF. One case of PAVF/DAVF/FRA was identified. CONCLUSIONS: The exact mechanism of PAVF/DAVF formation is unknown. Hemorrhage predilection of PAVF/DAVF is more difficult to predict than a sole fistula, likely secondary to the complex flow dynamics. To our knowledge, this literature review is the largest review to date regarding PAVFs in adult patients and can provide insight into decision making when evaluating treatment options.

Abdominal pseudocyst as a complication of ventriculoperitoneal shunt placement: Review of the literature and a proposed algorithm for treatment using 4 illustrative cases.

BACKGROUND: Ventriculoperitoneal (VP) shunt placement is one of the most commonly performed procedures in neurosurgery. One rare complication is the formation of an abdominal pseudocyst, which can cause shunt malfunction. CASE DESCRIPTIONS: We present four unique cases of abdominal pseudocyst formation. Our first patient initially presented with a right upper quadrant pseudocyst. Shunt was externalized and the distal end was revised with placement of catheter on the opposite side. He developed another pseudocyst within 5 months of shunt revision and developed another shunt failure. Our second patient had a history of shunt revisions and a known pseudocyst, presented with small bowel obstruction, and underwent laparotomy for the lysis of adhesions with improvement in his symptoms. After multiple readmissions for the same problem, it was thought that the pseudocyst was causing gastric outlet obstruction and his VP shunt was converted into a ventriculopleural shunt followed by percutaneous drainage of his pseudocyst. Our third patient developed hydrocephalus secondary to cryptococcal meningitis. He developed abdominal pain secondary to an abdominal pseudocyst, which was drained percutaneously with relief of symptoms. The fourth patient had a history of multiple shunt revisions and a previous percutaneous pseudocyst drainage that recurred with cellulitis and abscess secondary to hardware infection. CONCLUSION: Abdominal pseudocysts are a rare but important complication of VP shunt placement. Treatment depends on etiology, patient presentation, and clinical manifestations. Techniques for revision include distal repositioning of peritoneal catheter, revision of catheter into pleural space or right atrium, or removal of the shunt completely.

Chondromyxoid fibroma of the sacrum: A case report and literature review.

BACKGROUND: Chondromyxoid fibroma (CMF) is an extremely rare, benign cartilaginous tumor that makes up <0.5% of all bone tumors, typically presenting in the second or third decade of life. CMF of the sacrum is exceedingly rare, with only seven documented cases reported in the neurosurgical literature. CASE DESCRIPTION: We report a case of a 35-year-old female with a 3 month history of lower back pain after sustaining a fall on her sacrum/coccyx presenting with a progressive complaint of localized lower back pain, occasional urinary retention without incontinence, gluteal hypesthesia, and pressure below the gluteal crease. Imaging demonstrated a large, expansile enhancing soft-tissue lesion involving the sacrum, distal to the S2-3 disc space. The tumor was removed with partial sacrectomy for open en bloc resection with partial nerve sparing. The patient was found at 1.5-year follow-up with the improvement of symptoms, no recurrence, and no residual neurologic dysfunction. CONCLUSION: Sacral CMF is a rare clinical entity that may mirror more aggressive sacral pathology, including chordoma, in both clinical presentation and imaging characteristics. A review of the available literature regarding diagnosis, surgical management options, and prognosis for sacral CMF is provided.

Histoplasmosis with Deep CNS Involvement: Case Presentation with Discussion and Literature Review.

Central nervous system (CNS) histoplasmosis is rare and difficult to diagnose because it is often overlooked or mistaken for other pathologies due to its nonspecific symptoms. A 32-year-old Hispanic man with advanced acquired immunodeficiency virus presented with altered mental status and reported confusion for the past 3 months. He had a Glasgow Coma Scale of 12, repetitive nonfluent speech, and a disconjugate gaze with a right gaze preference. Lung computed tomography (CT) findings indicated a pulmonary histoplasmosis infection. Magnetic resonance imaging of the brain revealed a ring-enhancing lesion in the left caudate nucleus. A CT-guided left retroperitoneal node biopsy was performed and indicated a benign inflammatory process with organisms compatible with fungal yeast. Treatment with amphotericin B followed by itraconazole was initiated in spite of negative cerebrospinal fluid (CSF) cultures and proved effective in mitigating associated CNS lesions and resolving neurologic deficits. The patient was discharged 3 weeks later in stable condition. Six weeks later, his left basal ganglia mass decreased. Early recognition of symptoms and proper steps is key in improving outcomes of CNS histoplasmosis. Aggressive medical management is possible in the treatment of intracranial deep mass lesions, and disseminated histoplasmosis with CNS involvement can be appropriately diagnosed and treated, despite negative CSF and serology studies.

Supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature.

INTRODUCTION: Supratentorial primitive neuroectodermal tumors predominantly occur in children, and are rare in the adult population. Less than 100 cases of supratentorial primitive neuroectodermal tumor have been reported in adults internationally. Our case study reports this rare incident. CASE PRESENTATION: A 22-year-old Hispanic man presented with headaches, blurry vision, diplopia, intermittent vomiting, and grossly decreased vision. A magnetic resonance image showed a left posterior parietal heterogeneously enhancing mass measuring 4.2cm × 7.2cm × 7.0cm. After craniotomy for resection and decompression, the mass was histologically revealed to be a supratentorial primitive neuroectodermal tumor. Standardized immunohistochemical studies for this mass were carried out. CONCLUSION: We have concluded that immunohistochemical and genetic workup should be included in the standardized pathological workup for primitive neuroectodermal tumors in order to provide more prognostic information. Based on our current literature review, we propose an immunohistochemical panel.