Spinal dural arteriovenous fistula: a comprehensive review of the history, classification systems, management, and prognosis.

Spinal dural arteriovenous fistulas account for the majority of spinal vascular malformations. They are typically located in the thoracolumbar region and are diagnosed in the middle-aged and elderly populations. Although spinal dural arteriovenous fistulas have been postulated to be acquired, their exact development remains uncertain. Typically, the arteriovenous shunt is situated close to the spinal nerve root, inside the dura mater, where the blood from the radiculomeningeal artery and radicular vein intermix. Throughout history, there have been multiple classification systems of spinal arteriovenous shunts since 1967. Those were mainly based on the evolution of diagnostic studies as well as the treatment of these lesions. Such classification systems have undergone significant changes over the years. Unlike intracranial dural arteriovenous fistula, spinal dural arteriovenous fistula is progressive in nature. The neurological manifestations, due to venous congestion, tend to be insidious as well as non-specific. These include sensory deficits, such as paresthesia, bilateral and/or unilateral radicular pain affecting the lower limbs, and gait disturbances. Spinal dural arteriovenous fistulas can be suspected on magnetic resonance imaging/magnetic resonance angiography and confirmed by digital subtraction angiography (DSA). The management includes surgery, endovascular therapy, and in selected cases, radiotherapy. The treatment goal of spinal dural arteriovenous fistula is to halt the progression of the disease. The prognosis depends on both the duration of symptoms as well as the clinical condition prior to therapy. The present article comprehensively reviews the pathophysiology, changes in classification systems, natural history, clinical manifestations, radiological features, management, and prognosis.

Intracranial dural arteriovenous fistula: a comprehensive review of the history, management, and future prospective.

Dural arteriovenous fistulas (DAVF) are abnormal acquired intracranial vascular malformations consisting of pathological connections located within the dura between the pial arteries and the veno vasora, comprising the walls of the dural sinuses, bridging veins, or transosseous emissary veins. Dural arteriovenous fistulas are distinguished from arteriovenous malformations by their arterial supply from the vessels that perfuse the dura mater and lack of a parenchymal nidus. They are most commonly situated at the transverse and cavernous sinuses. The mechanism of development behind dural arteriovenous fistula can be explained by the molecular and anatomical factors. Multiple classification systems have been proposed throughout history including; Djindjian and Merland, Cognard, and Borden classification systems. The aggressiveness of the clinical course in intracranial dural arteriovenous fistula can be predicted through the angiographic patterns of venous drainage, more specifically, the presence of cortical venous drainage, the presence of venous ectasia, and the aggressiveness of clinical presentation. Intracranial dural arteriovenous fistulas might be discovered incidentally. However, if symptomatic, the clinical presentation ranges from mild neurological deficits to severe, lethal intracranial hemorrhage. Angiography is the imaging of choice to investigate, diagnose, and plan treatment for intracranial dural arteriovenous fistula. The management algorithm of intracranial dural arteriovenous fistula can be broadly divided into conservative, surgical, endovascular, and/or radiosurgical options. With the advent of endovascular therapies, surgery has fallen out of favor for managing intracranial dural arteriovenous fistulas. In the present article, the pathophysiology, classifications, natural history, clinical manifestations, radiological features, management, and complications are comprehensively reviewed.

The Early and Late Postoperative Complications of Pediatric Neuromuscular Scoliosis at King Abdulaziz Medical City, Riyadh, Saudi Arabia: A Case Series.

Background  Neuromuscular Scoliosis (NMS) is defined as "a coronal plane spinal curvature of 10 degrees or more, measured by the Cobb method, in the setting of muscle imbalance secondary to an underlying neuropathic or myopathic disease". Patients who have the disease usually manifest with diminished balance, asymmetrical seating, abnormal gait, and decreased pulmonary function, which are related to the change in spine posture. Surgery benefits patients with NMS in terms of stopping disease advancement and improving quality of life, but is known to be associated with certain complications in this population. The aim of this study is to identify the most common complication in NMS patients after surgical correction. Methods This study is a chart review-based retrospective case series that has covered patients' data going from 2015 to 2019. The study focused on patients who underwent scoliosis correction surgery of both genders and mainly of a single ethnicity, with the inclusion of patients aged 9 to 18 years old. Under consecutive sampling, the study has met a sample size of 14 patients.  Results Most of the study subjects nine (64%) were female. The age median was 13 years (2.25). The highest documented intraoperative complication was blood loss in 11 (79%) patients. The most prevalent early postoperative complication was urinary tract infection in two (14%) patients. No late postoperative complications were documented in the study. Conclusion  The study concluded that blood loss was the most common intraoperative complication. Pulmonary problems were one of the least reported complications. Possible reasons for these findings and prevention methods should be the focus of future studies.

Pediatric craniocervical fusion: predictors of surgical outcomes, risk of recurrence, and re-operation.

PURPOSE: Craniocervical junction abnormalities include a wide variety of disorders and can be classified into congenital or acquired. This study aimed to review the surgical outcome of pediatric patients who underwent craniocervical and/or atlantoaxial fusion. METHODS: This is a retrospective cohort study including all pediatric patients (≤ 18 years) who underwent craniocervical and/or atlantoaxial fusion between 2009 and 2019 at quaternary medical city. RESULTS: A total of 25 patients met our criteria and were included in the study. The mean age was 9 years (range: 1-17 years). There was a slight female preponderance (N = 13; 52%). Most patients (N = 16; 64%) had non-trauamatic/chronic causes of craniocervical instability. Most patients presented with neck pain and/or stiffness (N = 14; 56%). Successful fusion of the craniocervical junction was achieved in most patients (N = 21; 84%). Intraoperative complications were encountered in 12% (N = 3) of the patients. Early postoperative complications were observed in five patients (20%). Five patients (20%) experienced long-term complications. Revision was needed in two patients (8%). Older age was significantly associated with higher fusion success rates (p = 0.003). The need for revision surgery rates was significantly higher among younger age group (3.75 ± 2, p = 0.01). CONCLUSIONS: The study demonstrates the surgical outcome of craniocervical and/or atlantoaxial fusion in pediatric patients. Successful fusion of the craniocervical junction was achieved in most patients. Significant association was found between older age and successful fusion, and between younger age and need for revision surgery.

CLOVES syndrome and cervical arteriovenous fistula: a unique association managed by combined microsurgical and endovascular therapy.

CLOVES syndrome is a condition characterized by congenital lipomatous overgrowth, vascular malformations, epidermal nevi and skeletal anomalies. The association of cervical arteriovenous fistula with CLOVES syndrome is extremely rare. To the best of our knowledge, only two cases were reported in the literature. We hereby report an additional case and review the literature on this condition. A 12-year-old girl, known to have CLOVES syndrome, presented to the emergency department with a 2-week history of bilateral, progressive lower extremity and left arm weakness. Radiological imaging demonstrated a C3-C6 left extradural lesion, containing multiple enhancing vessels, causing marked compression on the spinal cord. Urgent spinal decompression was performed, with C3-C6 instrumentation, followed by a two-stage embolization of the fistula. Cervical arteriovenous fistula can be rarely identified in patients with CLOVES syndrome. High index of suspicion is required to promptly evaluate patients with CLOVES syndrome presenting with new-onset neurological deficits.

Traumatic thoracolumbar fracture-dislocation in an infant: surgical management using posterior thoracolumbar instrumentation.

Spinal fracture-dislocation in the infantile population is a rare phenomenon, and its surgical management remains poorly discussed in the literature. This article reports a case of traumatic fracture-dislocation in an infant by outlining the surgical management and extensively reviewing the literature. An 8-month-old girl was involved in a motor vehicle accident and was ejected from the car through the windshield. Radiological imaging demonstrated a complete spinal cord injury at the level of T10 and a three-column fracture of T12-L1, with an evidence of kyphosis measuring 47° at the fracture site. Posterior thoracolumbar instrumentation, using the posterior cervical fixation set, was successfully performed. In experienced neurosurgical centers, posterior thoracolumbar instrumentation can be safely performed in infants with traumatic thoracolumbar fracture-dislocation. This allows for the correction of the kyphotic deformity, facilitation of the rehabilitation course and improvement in the health-related quality of life.

Combined Type 1 and 2 split cord malformations, kyphoscoliosis, tethered cord, and a lipoma.

BACKGROUND: Split cord malformations (SCMs) are uncommon congenital anomalies. They can be divided into Type 1 (bony septum and two separate dural sheaths) and Type 2 (fibrous septum and a single dural sheath).[1,2,4] Rarely,SCM can be associated with orthopedic anomalies (mostly seen in older children), such as scoliosis, followed by kyphosis, and/or both.[1-3] Conversely, patients with congenital scoliosis have 40% risk of congenital underlying neural deformities, with 16.3% attributed to SCM.[1,4] Those with thoracic or lumbosacral SCM may have congenital tethering lesions, predominantly consisting of intradural lipomas.[3,4] Surgery is optimally performed in two stages: first, removal of the spinal septum and untethering of the cord, and second, correction of the kyphosis, scoliosis, and/or both.[1-5]. CASE DESCRIPTION: In this video, a 44-year-old female initially presented after having progressively developed kyphoscoliosis since childhood. Three months before presentation, she had developed increased left lower extremity pain with hypoesthesia followed 1 month later by the onset of the left lower extremity monoplegia (0/5). The computed tomography and magnetic resonance imaging studies revealed a SCM at the L3 level with bony and membranous septum, accompanied by an intradural lipoma, and tethered cord. She underwent a two- stage procedure; first, removal of the bony and membranous septum, resection of the intradural lipoma, and untethering of the cord; second, she had correction of the kyphoscoliosis. Postoperatively, although the pain and sensory deficits improved, the left lower extremity monoplegia remained. CONCLUSION: SCM is a rare cause of spinal deformity. If left untreated, the associated neurological deficits may progress. Treatment should include a two-staged approach; first, the bony and membranous septum should be removed followed by lipoma resection and untethering the cord with adequate cord decompression, while second, a fusion may be performed to address attendant kyphoscoliosis. KEYWORDS: Intradural lipoma, Kyphoscoliosis, Split cord malformation, Tethered cord.

Hemorrhagic presentations of cerebellar pilocytic astrocytomas in children resulting in death: report of 2 cases.

Acute hemorrhagic presentation in pilocytic astrocytomas (PAs) has become increasingly recognized. This type of presentation poses a clinically emergent situation in those hemorrhages arising in PAs of the cerebellum, the most frequent site, because of the limited capacity of the posterior fossa to compensate for mass effect, predisposing to rapid neurological deterioration. As examples, we describe two cases of fatal hemorrhagic cerebellar PAs: one of a child with a slowly growing stereotypical WHO Grade I PA with a 1-year period of symptomatology that preceded a rapid clinical deterioration, and another of an asymptomatic child having a PA variant, presenting with progressive obtundation following a presumed Valsalva event. These two scenarios parallel previous reports in the literature of either a setting of progressive expression of cerebellar dysfunction and transient episodes of raised intracranial pressure (ICP), or abrupt onset of features of increased ICP in a previously well child. The literature is further reviewed for a current understanding of the factors that predispose, initiate and propagate bleeding, with specific reference to the role of vascular endothelial growth factor and other angiogenic agents in the genesis and stability of the vasculature in PAs. In this context, we propose that obliterative vascular mural hyalinization with associated altered flow dynamics and microaneurysm formation was the pathogenesis of the hemorrhage in our first case. In the second case, large tumor size, increased growth rate, looseness of the background myxoid matrix, and thinness of the tumor blood vessels with calcospherite deposition predisposed to vascular leakage and bleeding concurrent with sudden increases in intravascular hydrostatic pressure. In that cerebellar PAs are common, this report underscores the importance of considering in the differential diagnosis the possibility of a spontaneous hemorrhage in a posterior fossa PA in a child presenting with a sudden neurological ictus and raised ICP.

Low-grade ependymoma with late metastasis: autopsy case study and literature review.

INTRODUCTION: Ependymoma metastasis occurs usually along with local recurrence within 7 years after the initial diagnosis. Later spinal metastasis without local recurrence after the surgical resection has been rarely reported in patients with low-grade ependymomas but not with high-grade ependymomas. Here, we present a case with autopsy revealing late extensive supratentorial metastasis of a fourth ventricle classic WHO grade II ependymoma with no local recurrence or spinal metastasis. METHODS: A 4-year-old boy underwent a gross total resection (GTR) of the fourth ventricle ependymoma and postoperative radiation therapy. Follow-up MRI showed no recurrence for the next 7 years, but a half year later, extra-axial tumors in the left cerebellopontine angle and right frontal lobe were observed. GTR of the left cerebellopontine angle ependymoma was performed, followed by additional radiation therapy. RESULTS: He was stable for the following 2 years before MRI revealed growth of the right frontal tumor and new lesions. GTR of the right frontal tumor demonstrated similar pathologic features of ependymoma. Despite chemotherapy, follow-up MRIs exhibited increasing numbers and sizes of supratentorial tumors but no infratentorial or spinal tumors. He died 15 years after the initial diagnosis. Postmortem brain examination confirmed the supratentorial subarachnoid dissemination with multifocal metastases of classic ependymomas but no recurrence at the infratentorial sites. CONCLUSION: Our case study and literature review suggest that low-grade ependymomas under the current WHO classification have the risk of late metastasis. Therefore, long-term follow-up of the whole neuroaxis is more important for the patients with low-grade ependymomas even in the absence of local recurrence.