Intracranial arachnoid cysts.

PURPOSE: The purpose of this review article is to outline the natural history, pathogenesis, anatomic considerations and surgical decision-making in caring for patients with intracranial arachnoid cysts. METHODS: A review of the literature for intracranial arachnoid cysts was performed using Embase, PubMed, and Web of Science databases, including review of the bibliographies of eligible articles and the author's own experience. RESULTS: Among those reviewed, 59 relevant original articles were included as well as illustrative cases from the authors own experience. CONCLUSIONS: Arachnoid cysts are congenital lesions characterized by split arachnoid membrane, thick collagen in the cyst wall, absent traversing trabecular processes within the cyst, and hyperplastic arachnoid cells in the cyst wall. The underlying etiology is not entirely known, and they occur in greater proportion in males and in greater incidence with various genetic conditions including Down syndrome, mucopolysaccharidosis, schizencephaly, neurofibromatosis, autosomal dominant polycystic kidney disease (ADPKD), acrocallosal syndrome, and Aicardi syndrome. Most intracranial arachnoid cysts are incidentally found and occur in the middle cranial fossa, with the remaining occurring in the cerebellopontine angle, suprasellar cistern, quadrigeminal cistern, convexity, and posterior fossa/cisterna magna. The current article outlines the natural history, prevalence, demographic factors, and treatment decisions in managing patients with intracranial arachnoid cysts.

Spinal intradural arachnoid cyst as a complication of insertion of an interspinous device.

Spinal intradural arachnoid cysts (SACs) account for approximately 10% of total arachnoid cysts, and iatrogenic attribution is rare. A 50-year-old male presented with low back pain and severe radiating pain in his right leg along the S1 dermatome. He had an interspinous device (ISD) inserted 10 years earlier. Plain radiographs showed breakage and angular movement of the ISD at L4/5. Magnetic resonance imaging (MRI) of the lumbosacral spine disclosed multiple intradural cystic tumors in the entire lumbar region. He underwent removal of the ISD, laminectomy, and resection of the intradural cyst. The pathologic finding of the cystic tumor was compatible with arachnoid cyst. Antibodies to parasites, including cysticercosis, were negative in both the serum and cerebrospinal fluid (CSF). The ventral surface of the implant was clearly broken.

Ventral Intrameningeal Cyst Treatment and Management: Technical Note.

BACKGROUND: Intrameningeal cysts are rare lesions without definitive etiologies that can involve the dura or arachnoid mater. Spinal arachnoid cysts have been described, and several different etiologies have been hypothesized. This includes one-way valve mechanisms, traumatic herniation of arachnoid through the dura, and abnormal arachnoid membrane proliferation. To the authors' knowledge, no such descriptions exist regarding purely dural-based cystic lesions; however, the authors hypothesize similar mechanisms may be involved. Most notably, a traumatic injury to the dura leading to a one-way valve mechanism may allow for egress of cerebrospinal fluid between the dural layers, splitting them open. This progressive enlargement can lead to displacement of neural elements and subsequent neurological compromise. METHODS: We describe a 17-year-old girl who presented with progressive neck and back pain, left upper-extremity numbness, bilateral lower-extremity weakness, paresthesias, and numbness without obvious etiology despite an extensive neurologic investigation. She had undergone conservative management options including multiple medications, physical and chiropractic therapy, and epidural steroid injections. Computed tomography myelography revealed a cerebrospinal fluid leak into the lumbar epidural space for which surgical exploration was performed. Despite utilizing fluoroscopy and intrathecal fluorescein, no leak source was identified. Fluid collection was found contained within the dural layers rather than the epidural space. RESULTS: An intracystic blood patch was performed with near-complete resolution of the lesion by 6-week follow-up and near-complete return of neurologic function. CONCLUSIONS: Ventral panspinal cysts are an exceedingly rare cause of radiculopathy and myelopathy that can be resolved by an intracystic blood patch.

Endoscopic Laser Fenestration to Treat a Bobble-Head Doll Syndrome Caused by Suprasellar Cyst.

The bobble-head doll syndrome (BHDS) is a rare acquired head movement disorder characterized by up and down or side-to-side movement, most commonly seen in the first decade of life. The syndrome occurs more often in lesions causing third ventricle dilatation such as suprasellar or third ventricle cyst, but it is also found in other pathologies associated with hydrocephalus like shunt dysfunctions, trapped fourth ventricle, congenital aqueductal stenosis, Dandy-Walker syndrome, and cerebellar malformations. The pathophysiology of this head movement has different origins theories; one states that this stereotyped movements empties the cyst and move the dome away from the foramina of Monro, which relieves the symptoms of hydrocephalus; the other suggests that the extrapyramidal tracts (rubrotegmentospinal and reticulospinal) are stimulated by the compression of dorsomedial nucleus of the thalamus by the cyst, whose tracts innervate the neck muscles resulting in the bobbling head movements. This video (Video 1) presents a clinical case of BHDS caused by suprasellar cyst in a 10- year-old boy treated by endoscopic procedure. A ventricular-cyst-cisternostomy was performed resulting in complete improvement of the head movements and uneventful recovery. Postoperative images demonstrate decreasing of the cyst lesion and resolution of the hydrocephalus.

Posttraumatic leptomeningeal cyst capsule as a cost-free autograft for its repair: case illustrated technical reports.

The surgical treatment of traumatic leptomeningeal cyst (LMC) is repair of the dural defect with or without cranioplasty. The dural substitutes used are either autografts (which may not be enough) or artificial grafts (which are foreign-body implantations and which also may be too expensive in a low-resource practice). In this report from a developing country, we present the surgical description of the use of the cyst capsule as a cost-free autologous graft in the surgical repair of the dural defects of two cases of traumatic leptomeningeal cyst.

Compresión medular secundaria a quiste aracnoideo intradural de etiología traumática / Spinal cord compression secondary to traumatic intradural arachnoid cyst

Los quistes aracnoideos espinales son entidades raras y casi siempre tienen una etiología congénita o espontánea. Los casos secundarios son muy poco frecuentes y muchas veces constituyen una causa de deterioro neurológico tras varios meses o años de un traumatismo espinal. Presentamos el caso de un varón diagnosticado de quiste aracnoideo espinal de etiología traumática, agrupamos los casos descritos hasta el momento en la literatura y revisamos las distintas opciones quirúrgicas relacionándolas con las teorías fisiopatológicas que tratan de explicar la génesis de estas lesiones

Spinal arachnoid cysts are rare entities, most of which have a congenital or spontaneous etiology. Secondary cases are infrequent and can constitute a cause of neurological deterioration several months or years after spinal trauma. We present the case of a male patient with traumatic spinal arachnoid cyst, together with a review of the cases published to date. Finally, we explain the different surgical options for this pathology relating them to the physiopathological theories that try to explain the origin of these lesions

Quistes aracnoideos espinales y neurocirugía / Spinal arachnoid cyst and neurosurgery

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Post-Traumatic Diploic Leptomeningeal Cyst with Bilateral Posterior Cranial Fossa Epidural Hygroma: A Management Dilemma?

BACKGROUND: Post-traumatic diploic leptomeningeal cyst with bilateral posterior cranial fossa epidural hygroma is a rare complication after calvarial fracture. Very few cases have been reported to date; hence, there are no specific guidelines for the management of these cases. CASE DESCRIPTION: A 4-year-old boy was brought to the emergency department after suffering from head trauma caused by a fall from a rooftop where he was treated conservatively at a local hospital. Later, he developed swelling in the occipital region and was brought to the department of neurosurgery where he was operated on. After the first surgery, recurrence of swelling was seen after a postoperative period of 2 months, and computed tomography scan reported persistent epidural hygroma with extension into the subcutaneous space. The second surgery was performed, and 12-month follow-up did not show any recurrence of swelling in the patient. CONCLUSIONS: Post-traumatic leptomeningeal cyst is commonly associated with occipital injury. The causes are as follows: the dura is very loosely attached to the intracranial lamina in young children, injury to the skull results in potential epidural space, and any tear or breach in the meninges leads to accumulation of cerebrospinal fluid in these spaces giving rise to cystic swelling. Watertight duroplasty with trials of duro-periosteal hitching has been described in the management of these cases.

Pediatric Skull Fracture Characteristics Associated with the Development of Leptomeningeal Cysts in Young Children after Trauma: A Single Institution's Experience.

BACKGROUND: Currently, the pathogenesis of leptomeningeal cysts, also known as growing skull fractures, is still debated. The purpose of this study was to examine the specific skull fracture characteristics that are associated with the development of growing skull fractures and describe the authors' institutional experience managing this rare entity. METHODS: A retrospective cohort study was performed that included all patients younger than 5 years presenting to a single institution with skull fractures from 2003 to 2017. Patient demographics, cause of injury, skull fracture characteristics (e.g., amount of diastasis, linear versus comminuted fracture), concomitant neurologic injuries, and management outcomes were recorded. Potential factors contributing to the development of a growing skull fracture and neurologic injuries associated with growing skull fractures were evaluated using univariate logistic regression. RESULTS: A total of 905 patients met the authors' inclusion criteria. Of these, six (0.66 percent) were diagnosed with a growing skull fracture. Growing skull fractures were more likely to be comminuted (83.3 percent versus 40.7 percent; p = 0.082) and to present with diastasis on imaging (100 percent versus 26.1 percent; p < 0.001; mean amount of diastasis, 7.1 mm versus 3.1 mm; p < 0.001). Univariate logistic regression analysis confirmed the role of a comminuted fracture pattern (OR, 7.572) and the degree of diastasis (OR, 2.081 per mm diastasis) as significant risk factors for the development of growing skull fractures. CONCLUSIONS: The authors' analysis revealed that fracture comminution and diastasis width are associated with the development of growing skull fractures. The authors recommend dural integrity assessment, close follow-up, and early management in young children who present with these skull fracture characteristics. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Growing Skull Fracture of Temporal Bone in Adults: A Case Report and Literature Review.

Growing skull fracture (GSF) is an uncommon post-traumatic complication, which accounts for approximately 0.05% to 1% of all skull fractures. Delayed diagnosis of GSF in adulthood is rare and often involved with a variety of neurological symptoms. Here, we reported an adult patient, with an interval of 17 years from initial head trauma to first diagnosis of GSF. The patient complained of short periods of fainting and bilateral visual hallucinations, with a hard palpable bulge around his right occipitomastoid suture region. Computed tomographic imaging demonstrated an arachnoid cyst extending into right mastoid cavity. Consequently, the delayed diagnosis of GSF was confirmed, and the patient was managed with duroplasty and cranioplasty. At the 8-month follow-up, the patient showed an uneventful postoperative recovery. A comprehensive literature review was also conducted, and a total of 70 GSF cases were identified and summarized. According to the literature review, patients with GSF generally have a history of head trauma in their childhood, and delayed diagnosis is a common situation. Diagnosis of GSF should include complete retrospective medical history, physical, and imaging examinations. Once the diagnosis is confirmed, cranioplasty accompanied with duroplasty might be the most effective way to relieve symptoms and prevent further damage.

Percutaneous Treatment of Meckel Cave Arachnoid Cyst: Case Report, Surgical Strategy and Literature Review

Arachnoid cysts are benign intracranial lesions. They are usually located in the middle fossa, but can be found in other locations. We present a case of symptomatic Meckel cave (MC) arachnoid cyst - a very rare location - and a treatment strategy not elsewhere described before for this condition. A 54-year-old female with trigeminal neuralgia with previous history of radiofrequency rhizotomy treatment 6 years before admission had been experiencing pain recurrence with progression, which required successive increases in carbamazepine dosage. Magnetic Resonance Imaging (MRI) showed dilatation of the right MC with extension to the petrous apex. The lesion was compatible with arachnoid cyst, and due to the worsening of the clinical condition, surgical treatment was chosen. Percutaneous puncture of the cyst through the foramen ovale with injection of intracystic fibrin sealant was performed. The patient woke up from anesthesia with pain improvement and was discharged asymptomatic the next day. After 12 months of follow-up, she remained pain-free. In the literature review, we found only eight cases reported as MC arachnoid cyst. These are likely to progress and become symptomatic owing to their communication with the subarachnoid space and a unidirectional valve mechanism. Pain improvement with this technique is probably secondary to the interruption of these mechanisms.

Iatrogenic Lumbar Arachnoid Cyst Fenestration for Tethered Cord: 2-Dimensional Operative Video.

Iatrogenic lumbar arachnoid cysts are a rare occurrence after intradural surgery.1,2 To our knowledge, there is only 1 other previous case reported in the literature contributing to a symptomatic tethered cord.3 We present a surgical video (Video 1) demonstrating the history, preoperative workup, and operative technique for exploration and fenestration of a symptomatic iatrogenic lumbar arachnoid cyst with a tethered cord. The patient is a 57-year-old female with a history of a closed neural tube defect with a lipoma that was resected over 20 years before her evaluation. She was lost to follow-up and did not retain any previous medical documentation. She complained of a 2-month history of progressive bilateral lower extremity weakness, leg pain, and worsening of chronic urinary incontinence. Imaging demonstrated a cystic lesion compressing her conus and cauda equina at the L3-4 level. The risks, benefits, and alternatives were discussed, and the patient elected to proceed with surgery. Intraoperative findings demonstrated a complex multiloculated arachnoid cyst with tethering of the conus. The cysts were dissected from the conus and fenestrated, allowing spontaneous flow of cerebrospinal fluid and conus pulsations. Biopsy of the cyst wall confirmed the diagnosis of arachnoid cyst without neoplastic tissue. Due to the presence of multiple loculations, primary pial closure could not be performed. Thus, a circumferential detethering of the conus was performed to ensure complete decompression of the neural elements. Postoperatively, the patient demonstrated improvement in her bilateral lower extremity pain and weakness. Magnetic resonance imaging was scheduled during routine follow-up at 3 months.

Suprasellar Cyst Presenting With Bobble-Head Doll Syndrome.

BACKGROUND: Bobble-head doll syndrome is a rare neurological syndrome presenting with repetitive anteroposterior head movements. It is usually associated with expansile cystic lesions in the third ventricular region. CASE DESCRIPTION: An 8-year-old boy presented with involuntary bobbling head movements. Magnetic resonance imaging of the brain revealed an extensive suprasellar cyst resulting in obstructive hydrocephalus. Endoscopic ventriculo-cysto-cisternostomy resulted in improved clinical outcome. CONCLUSIONS: Endoscopic ventriculo-cysto-cisternostomy is an effective, less-invasive technique in the treatment of suprasellar cysts that results in resolution of the bobbling head movements.

Management of quadrigeminal arachnoid cyst associated with obstructive hydrocephalus: report on stereotactic ventricular - cystic stenting.

Quadrigeminal arachnoid cysts (QAC) are usually accompanied by a symptomatic obstructive hydrocephalus. Several endoscopic and surgical treatments exist; however, the critical location of these cysts further complicates treatment and usually more than one procedure is required. In this report, a 31 year old female with QAC and associated obstructive hydrocephalus was successfully treated with stereotactic placement of a permanent ventricular - cystic stent (intraventricualr - cystic catheterization) in single - session. Intraventricular - cystic stenting provides a long lasting communication between these two compartments, allowing persistent "physiologic" solution to this challenging condition.

Spinal Arachnoiditis Ossificans: Report of Quadruple-Triggered Case.

BACKGROUND: Arachnoiditis ossificans (AO) is a rare condition often associated with previous spine surgery. Here we describe a unique case of a patient affected by ankylosing spondylitis (AS), presenting with progressive neurologic deterioration due to AO. We also review the literature on evaluation and management of patients suffering from AO. CASE DESCRIPTION: The 65-year-old patient had a history of previous spinal trauma and related thoracolumbar surgery. Magnetic resonance imaging revealed multiloculated intradural/extramedullary cysts on the posterior surface of the spinal cord at Th9-L1, with clustered nerve roots. Computed tomography, with 3-dimensional reconstruction, demonstrated a likely ossification of both the dura and arachnoid from Th9 to S1. Microsurgical debridement of scar tissue from previous surgery, drilling of posterior ossified plaques at Th11-Th12-L1, and marsupialization and drainage of arachnoid cysts at Th11-Th12 were performed. CONCLUSIONS: We submit that AS, spinal trauma, epidural hematoma, and related surgery may be synergistic and independent factors in the etiopathogenesis of AO. This should be considered in patients with AS and/or a history of spinal surgery who present neurologic worsening.

Quistes aracnoideos e higroma subdural / Arachnoid cysts and subdural hygroma

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Less common manifestations in TSC.

Tuberous sclerosis complex (TSC) is due to pathogenic variants in TSC1 or TSC2 genes resulting in hyperactivation of the mTOR pathway. Many organ systems can be affected, such as brain, skin, eye, heart, bone, kidney, or lung. Typical lesions of TSC usually are those included as major criteria, including angiofibromas, hypomelanotic macules, tubers, subependymal nodules, angiomyolipomas, cardiac rhabdomyomas, and lymphangioleiomyomatosis. However, there are many other manifestations less frequent and/or less well known, many of them not included as clinical diagnostic criteria that are part of the clinical spectrum of TSC. The focus of this review will be on these less common and less well-known manifestations of TSC. Among the rare manifestations, we will discuss some clinical findings including arteriopathy, arachnoid cysts, lymphatic involvement, chordomas, gynecological, endocrine, and gastrointestinal findings. Among the manifestations that are very frequent but much less well known, we find the sclerotic bone lesions. Although they are very frequent in TSC they have been largely overlooked and not considered diagnostic criteria, mainly because they are asymptomatic. However, it is important to know their typical characteristics to avoid misdiagnosing them as metastasis.

Rare Case of Convexity Meningioma Growing into Arachnoid Cyst.

BACKGROUND: Meningioma growing into an arachnoid cyst is an extremely rare event. Only 3 cases are reported in the literature. In 2 of them, an operative procedure in or near the arachnoid cyst preceded tumor growth. CASE DESCRIPTION: We report a case of a patient requiring marsupialization of an arachnoid cyst of the middle cranial fossa. On follow-up, 3 years postoperatively he showed no signs of recurrence or tumor growth. One year later, the fourth year after surgery on the cyst, he presented with large tumor growth into the former cyst's cavity. Pathologic workup after resection revealed an atypical meningioma (World Health Organization grade II). CONCLUSIONS: We discuss the possible pathogenesis in light of the scarce published literature, as well as the differential diagnosis of this rapidly growing tumor.

Clinical and radiological features of spinal extradural arachnoid cysts: Valve-like mechanism involving the nerve root fiber as a possible cause of cyst expansion.

BACKGROUND: Although a valve-like mechanism has been proposed for expansion of spinal extradural arachnoid cysts (SEACs), the detailed mechanism remains unclear. Moreover, closure of the communication site is essential during surgery, but the method to identify the communication site remains unclear. The aim of this study was to determine the detailed mechanism of expanding SEACs through retrospective analysis of SEAC cases undergoing surgery and to elucidate the characteristics of the communication sites. METHODS: The authors retrospectively evaluated 12 patients with SEACs who underwent surgery between 2000 and 2014 and analyzed their perioperative findings. RESULTS: Dural defects were detected in 11 out of 12 patients, and a valve-like mechanism was observed in 7 patients, wherein a nerve root fiber moved back and forth through the dural defect along with the flow of cerebrospinal fluid (CSF) between the intradural space and the extradural arachnoid cysts. The dural defect was located at the thoracolumbar junction in 7 patients, below the distal end of the bridging ossification in 2, at the level of vertebral wedge deformity in 2, and at the level of disc herniation in 1. CONCLUSIONS: A valve-like mechanism was observed in 7 of the 12 patients, which suggests that it could serve as a mechanism of SEAC formation. The communication sites were variously located at the end of ossification in patients with diffuse idiopathic skeletal hyperostosis (DISH), wedge deformity of the vertebral body, or disc herniation, indicating the contribution of mechanical stress to SEAC formation.

Development of Neuromyelitis Optica Spectrum Disorder and Spinal Arachnoid Cysts in a Patient With Intractable Epilepsy.

Neuromyelitis optica is an inflammatory, demyelinating disease of the central nervous system that is characterized by severe relapsing attacks of optic neuritis and transverse myelitis. The current case describes a 29-year-old man with intractable epilepsy and diplegic spastic cerebral palsy who was given the diagnosis of neuromyelitis optica spectrum disorder after presenting with weakness, incontinence, and decreased visual acuity. His symptoms recurred 21 months after initial presentation. Magnetic resonance imaging of his spine revealed arachnoid cysts with regional mass effects. Differentiation of arachnoid cysts from a demyelinating process may be difficult in the early stages of the disease. Close monitoring of patients with neuromyelitis optica spectrum disorder is important, especially in patients with recurrent or refractory symptoms.