Intracranial Arachnoiditis and Hydrocephalus.

A 62-year-old man presented a diffuse and predominantly cisternal acute nonaneurysmal subarachnoid hemorrhage associated with hydrocephalus. An external ventricular drain was placed, followed by clinical deterioration after its removal. At this point, a heavily T2-weighted high-resolution sequence of a brain magnetic resonance imaging showed acute hydrocephalus recrudescence and multiple arachnoid adhesions in the supravermian and interpeduncular cisterns, creating a loculated/cystic appearance. The diagnosis of intracranial arachnoiditis was made. Intracranial arachnoiditis results from meningeal inflammation. Fibrosis and adhesions at the subarachnoid spaces may follow, restricting cerebrospinal fluid circulation, particularly at the cranial base. Hydrocephalus probably resulted from the combination of subarachnoid hemorrhage and extensive scaring at the basal cisterns, precluding transdural and transvenous cerebrospinal fluid efflux. Heavily T2-weighted high-resolution magnetic resonance imaging sequences allow an exquisite depiction of arachnoiditis, displaying obstructive cisternal membranes, and contribute to better etiologic assessment and management of hydrocephalus.

Giant intradural extramedullary spinal ependymoma, a rare arachnoiditis-mimicking condition: case report and literature review.

BACKGROUND AND IMPORTANCE: Ependymomas are tumours arising from the ependymal cells lining the ventricles and the central canal of the spinal cord. They represent the most common intramedullary spinal cord tumour in adults and are very rarely encountered in an extramedullary location. Only 40 cases of intradural extramedullary (IDEM) ependymomas have been reported, all of which were diagnosed pre-operatively as IDEM ependymomas on contrast-enhanced MRI. CLINICAL PRESENTATION: We report a 23-year old male presenting with rapidly worsening signs and symptoms of spinal cord disease. A spinal MRI demonstrated a posterior multi-cystic dilatation extended between T1 and T12. Post-contrast sequences showed peri-medullar leptomeningeal enhancement and the diagnosis of spinal arachnoiditis was made. The patient underwent surgery and the spinal cord appeared circumferentially wrapped by an irregular soft tissue. The tissue was sub-totally removed and the pathological diagnosis was ependymoma WHO grade II. The patient experienced an excellent neurological recovery and no further treatments were administered. A small residue is now stable at 2.5 years follow-up. CONCLUSIONS: Giant IDEM ependymomas are rare entities and pre-operative diagnosis can be challenging in some cases. Surgery represents the main treatment option being resolutive in most cases.

Magendie's foramen debridement and catheterisation for the treatment of syringomyelia due to diffuse craniocervical junction arachnoiditis. A case report and technical note.

A 36 year old woman was referred to our department for symptomatic lumbar spinal arachnoiditis following an epidural anaesthesia for childbirth. She did not had other known causative factor and she was free of any neurological symptoms before. She rapidly developed lower limbs impairment by compressing intradural lumbar collections and arachnoiditis requiring surgical decompression and subsequently internal cerebrospinal fluid shunting for acute hydrocephalus. Three years and the half later, she developed a severe tetraparesis due to a massive syrinx consecutive to the fourth ventricle outlets obstruction cause by the ongoing diffuse craniocervical junction arachnoiditis. Our aim was to treat all the problems in one step. An open fourth ventriculostomy of the Magendie's foramen with catheter insertion from the fourth ventricle down to the upper cervical subarachnoid space improve both the patient status and imagery.

MRI characteristics of syringomyelia associated with foramen magnum arachnoiditis: differentiation from Chiari malformation.

BACKGROUND: It is important to distinguish foramen magnum arachnoiditis (FMA) from Chiari malformation (CM) before surgery because the operative strategies for these diseases differ. In the current study, we compared pretreatment magnetic resonance imaging (MRI) of FMA with CM and investigated the MRI findings useful to differentiate between these diseases. METHODS: We retrospectively reviewed patients with FMA or CM aged ≥ 18 years who underwent surgeries at our institution between 2007 and 2019. The morphologies of the syrinx, neural elements, and posterior cranial fossa were preoperatively evaluated with MRI. We used the receiver operating characteristic (ROC) curve for the fourth ventricle-to-syrinx distance (FVSD). RESULTS: Ten patients with FMAs and 179 with CMs were included. FVSD in the FMA group was significantly shorter than that in the CM group (7.5 mm [IQR, 2.8-10 mm] in FMA vs. 29.9 mm [IQR, 16.3-52.9 mm] in CM, p < 0.0001). The other MRI findings that showed the height, size, and length of the syrinx; size of the foramen magnum; degree of cerebellar tonsillar descent; shape of the cerebellar tonsil; and dorsal subarachnoid space at the foramen magnum differed significantly between the two groups. The ROC curve analysis showed that patients whose FVSD was less than 11 mm could be diagnosed with FMA with a specificity of 90% and sensitivity of 96%. CONCLUSIONS: A more cranial syrinx development (FVSD < 11 mm) appears to be the characteristic MRI finding in FMA.

Spinal Syrinx Due to Lipiodol-Induced Arachnoiditis.

We present a case of a progressive symptomatic intramedullary cyst, diagnosed decades after Lipiodol injection. Lipiodol was originally administered intrathecally for the radiologic diagnosis of spinal masses. A link between the lesion and the history of Lipiodol injection was never suspected. Surgical exploration revealed a membrane above the lesion, separating the intradural space in a cranial and caudal compartment. On the level of the cyst, we identified glassy pearls containing a fatty liquid, compatible with Lipiodol deposits. We hypothesize that the syrinx is secondary to the impact of cerebrospinal fluid pulsations on the reactive membrane and that this membrane originated from an arachnoiditis caused by Lipiodol deposits. Lipiodol was indeed abandoned after it was found to cause arachnoiditis and neurologic sequelae. Despite the cessation of its usage, the causal role of Lipiodol in arachnoiditis and spinal cyst formation should still be considered, as symptoms may arise many years after Lipiodol administration.

Reintervention rate of arachnolysis versus shunting for nonforaminal syringomyelia.

OBJECTIVE: Surgical treatment for nonforaminal syringomyelia related to spinal arachnoiditis is still controversial. The authors sought to assess respective outcomes and rates of reintervention for shunting and spinal cord untethering (arachnolysis) in spinal arachnoiditis with syringomyelia. METHODS: This retrospective cohort study was conducted at a single reference center for syringomyelia. Patients undergoing arachnolysis and/or shunting interventions for nonforaminal syringomyelia were screened. RESULTS: The study included 75 patients undergoing 130 interventions. Arachnolysis without shunting was performed in 48 patients, while 27 patients underwent shunting. The mean follow-up between the first surgery and the last outpatient visit was 65.0 months (range 12-379 months, median 53 months). At the last follow-up, the modified McCormick score was improved or stabilized in 83.4% of patients after arachnolysis versus 66.7% after shunting. Thirty-one (41.3%) patients underwent reintervention during follow-up, with a mean delay of 33.2 months. The rate of reintervention was 29.2% in the arachnolysis group versus 63.0% in the shunting group (chi-square = 8.1, p = 0.007). However, this difference was largely driven by the extension of the arachnoiditis: in patients with focal arachnoiditis (≤ 2 spinal segments), the reintervention rate was 21.6% for arachnolysis versus 57.1% for shunting; in patients with extensive arachnoiditis, it was 54.5% versus 65.0%, respectively. Survival analysis assessing the time to the first reintervention demonstrated a better outcome in both the arachnolysis (p = 0.03) and the focal arachnoiditis (p = 0.04) groups. CONCLUSIONS: Arachnolysis led to fewer reinterventions than shunting in patients with nonforaminal syringomyelia. There was a high risk of reintervention for patients with extensive arachnopathies, irrespective of the surgical technique.

Arachnoiditis Ossificans of the Lumbar Spine: A Rare Cause of Progressive Cauda Equina Syndrome.

Arachnoiditis ossificans of the spine is a rare entity defined as an ossification of the leptomeninges resulting in neurologic decline. We describe the case of a 42-year-old woman, without any obvious predisposing factor, who presented with a progressive cauda equina syndrome. The imaging findings on magnetic resonance imaging were confusing by showing an atypical intraspinal lesion extending from L1 to S1. The computed tomography scan was more specific by showing suggestive images of a huge arachnoiditis ossificans of the lumbar spine. The patient underwent a large lumbar laminectomy with an incomplete resection of the ossified arachnoid. The histologic study confirmed the bony nature of the lesion. This illustrative case highlights the importance of helical computed tomography scan with multiplanar reconstruction for the diagnosis of arachnoiditis ossificans.

Hemifacial Spasm Secondary to Arachnoiditis Due to Neurocysticercosis: Clinical Image.

Subarachnoid neurocysticercosis (NCC) is a form of NCC with cysticerci located in the subarachnoid space. This form of NCC can cause general and focal neurologic symptoms, and sometimes requires surgical intervention as a treatment. In this report, we present a rare case of hemifacial spasm secondary to arachnoiditis because of an NCC cyst in the cerebellopontine angle. The cysticercus was removed and the facial nerve was liberated via a retrosigmoidal approach. At 8-month follow-up, the patient reported no recurrence of symptoms. To our knowledge, this is the first case reported to surgically manage hemifacial spasm secondary to NCC arachnoiditis.

Recurrent arachnoid cysts secondary to spinal adhesive arachnoiditis successfully treated with a ventriculoperitoneal shunt.

Spinal adhesive arachnoiditis (SAA) with cyst formation secondary to infectious meningitis is a rare clinical entity. These cysts can compress the spinal cord and cause neurologic decline. We present a case of a patient who underwent resection for an intradural schwannoma which was complicated post-operatively by bacterial meningitis and development of several recurrent thoracic arachnoid cysts. After two separate thoracic decompressions with lysis of intradural adhesions, a permanent ventriculoperitoneal shunt (VPS) was eventually placed with complete recovery of his symptoms. Our review of the literature showed that CSF shunts have previously been successfully used to treat spinal fluid collections. Although there are many factors to consider when treating these patients, CSF diversion may be beneficial for similar SAA presentations in order to simultaneously treat and prevent recurrence of symptoms.

Endoscopic-Assisted Spinal Arachnoiditis Adhesiolysis and Placement of a Spinal Cysto-Subarachnoid Shunt.

BACKGROUND: Spinal arachnoiditis and associated arachnoid adhesions can cause debilitating neurological symptoms due to nerve root and spinal cord compression or tethering. Adhesiolysis using a microscopic approach has traditionally been used for this condition. This procedure has been further refined in recent years with the use of flexible endoscopes. CASE DESCRIPTION: We report the case of a patient with progressive thoracic myelopathy secondary to arachnoid adhesions associated with arachnoiditis. A minimally invasive technique of adhesiolysis and placement of a cysto-arachnoid shunt was performed with the assistance of a flexible endoscope. CONCLUSIONS: The present case report has highlighted the advantages of using a flexible endoscope to treat spinal arachnoiditis adhesiolysis and placement of a cysto-arachnoid shunt, including a smaller skin incision and extended visualization.

A rare cause of progressive neuropathy: Arachnoiditis ossificans.

Arachnoiditis ossificans (AO) is a rare type of chronic arachnoiditis characterized by the presence of calcification or ossification of the spinal arachnoid which is usually associated with progressive neurological deficits. It is usually followed by prior history of trauma, surgery, infection, or myelography. Magnetic resonance imaging and computed tomography are the characteristics that are helpful in the diagnosis of most cases. Prognosis and treatment depends on the site and clinical presentation of the patients. We present a case of a young female who presented with a long-standing history of neurological symptoms and a intradural lesion mimicking a tumor.

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.

Surgical treatment of a thoracic ventral intradural arachnoid cyst associated with syringomyelia: case report.

The pathogenesis of thoracic ventral intradural spinal arachnoid cyst (ISAC) is unknown due to its extremely low incidence. In addition, its surgical treatment is complicated because of the ventral location, large craniocaudal extension, and frequent coexistence of syringomyelia. The optimal surgical strategy for thoracic ventral ISAC remains unclear and continues to be a matter of debate. In this report, the authors describe an extremely rare case presenting with a compressive thoracic ventral ISAC associated with syringomyelia that was successfully treated with a simple cyst-pleural shunt. The patient's medical history revealed bacterial spinal meningitis along with an extensive spinal epidural abscess, suggesting the incidence of extensive adhesive arachnoiditis (AA) to be a plausible cause for this pathology. Thoracic ventral ISAC reportedly occurs secondary to AA and is commonly associated with syringomyelia. Placement of a cyst-pleural shunt is an effective, safe, and uncomplicated surgical strategy, which can provide sufficient cyst drainage regardless of the coexistence of AA, and thus should be considered as primary surgical treatment. Syrinx drainage could be reserved for a later attempt in case the cyst-pleural shunt fails to reduce the extent of syringomyelia.

Postarachnoiditis Anterior Spinal Arachnoid Cyst Formation with Compressive Myelopathy: Report of 2 Cases.

BACKGROUND: Spinal cystic arachnoiditis is a rare complication of a subarachnoid haemorrhage or infectious meningitis. The inflammatory process leads to fibrosis, adhesions, and in severe cases cyst formation. Large arachnoid cysts are an uncommon cause of compressive myelopathy. The majority are located posterior of the spinal cord at the thoracic level. Anterior cyst formation is exceptional, especially at the cervical region. CASE DESCRIPTION: We present 2 cases of progressive myelopathy secondary to anterior arachnoid cyst formation. In a 54-year-old female a large anterior symptomatic thoracic cyst arose 4 years after rupture of a posterior inferior cerebellar artery aneurysm. The other 59-year-old-patient, however, developed an anterior cervical cyst only weeks after a varicella meningoencephalitis. Both female patients were treated with a decompressive laminectomy and wide fenestration of the cysts. Partial recovery was obtained in 1 patient, but there was no improvement in the other case. CONCLUSIONS: Spinal cystic arachnoiditis with anterior cyst formation is an extremely rare complication of subarachnoid haemorrhage and infectious meningitis but can cause severe neurologic deficits. Clinicians should be aware of this rare complication. Due to the risk of irreversible spinal cord injury, rapid surgical intervention is recommended in most cases.

Spinal adhesive arachnoiditis following the rupture of an Adamkiewicz aneurysm: Literature review and a case illustration.

BACKGROUND: The rupture of an isolated spinal aneurysm is an exceptional occurrence. It might be responsible for a spinal subarachnoid haemorrhage (SSAH) that in rare cases can be complicated by arachnoiditis. Among the former the adhesive type is the most severe leading to the formation of a cyst and/or a syrinx. PATIENTS AND METHODS: The literature review was performed via a PubMed search using the following keywords. Adhesive arachnoiditis; spinal subarachnoid haemorrhage; spinal arachnoiditis; spinal arachnoid cyst; arachnoid cyst .Thesearch yielded 24 articles. Given the fact that only a few studies had been reported on the subject, we decided to include all studies regarding adhesive arachnoiditis after SAH leading to a descriptive literature review. Furthermore, a case illustration of a 57 year old man harbouring this type of rare pathology is described. RESULTS: Twenty-four case reports were found regarding spinal adhesive arachnoiditis (SAA) following SSAH. Posterior cerebral circulation bleeding (66.7%) most often occurred followed by spinal (9.1%) and anterior cerebral circulation (9.1%) respectively. The mean time between the haemorrhage and the SAA onset was 10 months. A higher predominance of symptomatic thoracic SAA was found. Including the present case, 80% of patients had a laminectomy, 72% had a micro adhesiolysis, and 56% a shunt placement. Cervical and upper thoracic involvement appeared to have a better outcome. CONCLUSION: Although most authors suggested surgical treatment, the long-term outcome remains unclear. Early stage diagnosis and management of this rare and disabling pathology may lead to a better outcome. Larger co-operative studies remain essential to obtain a better understanding of such a rare and complex disease.

Treatment of Syringomyelia in Patients with Arachnoiditis at the Craniocervical Junction.

OBJECTIVE: Craniocervical junction arachnoiditis (CCJA) is an uncommon cause of syringomyelia. The pathophysiology of syrinx formation is uncertain, and the appropriate management unclear. A series of cases is reported to demonstrate variations in etiology, uniformity of functional cerebrospinal fluid obstruction at the foramen magnum, and results of surgical intervention. METHODS: We retrospectively analyzed the clinical and radiologic features of a consecutive series of patients treated for syringomyelia related to CCJA. RESULTS: Eight patients (5 male, 28-66 years old) were treated from 2000 to 2016. Magnetic resonance imaging demonstrated cervicothoracic syringomyelia in all cases, with the rostral extension of the syrinx suggesting communication with the fourth ventricle in all but one case. There was reduction of foramen magnum cerebrospinal fluid space in all cases, cerebellar ectopia in 5 cases, and fourth ventricular entrapment in 3 cases. Treatment consisted of posterior fossa decompression with either a GoreTex or pericranial patch graft. Six patients had a fourth-ventricle spinal subarachnoid shunt. Two patients had titanium mesh cranioplasty. The immediate postoperative period was associated with reduction in syrinx cavity size and improvement in neurologic symptoms in all cases. At follow-up 10-60 months postoperatively, 3 patients exhibited recurrence of the syrinx and underwent successful reoperation at the craniocervical junction. One patient with persistence of the inferior component of the syrinx was treated with a syrinx-spinal subarachnoid shunt. CONCLUSIONS: Most syrinx cavities associated with CCJA communicate with the fourth ventricle. Posterior fossa decompression and fourth ventricle to spinal subarachnoid space shunting appears a reasonable treatment for this form of syringomyelia.

[Treatment of syringomyelia associated with adhesive arachnoiditis]. / Lechenie siringomielii, assotsiirovannoi s adgezivnym arakhnoiditom.

The prevalence of syringomyelia (SM) caused by adhesive arachnoiditis (AA) is 2 to 4 cases per 100000 population. Surgical treatment of this pathology usually includes implantation of shunts into the cyst cavity or opening and drainage of the cavity. In this case, SM continues to progress in 72-100% of patients. Unsatisfactory outcomes of this surgical approach necessitate searching for other treatment options. PURPOSE: To define the optimal amount of surgery for SM associated with AA and the criteria for assessment of surgery outcomes. MATERIAL AND METHODS: The authors treated 47 SM patients in the period from 2010 to 2015. Of these, 34 (72.3%) patients underwent surgery; a total of 40 operations were performed. The patients' age ranged from 18 to 64 years (mean, 43.5 years). Tethering of the spinal cord was eliminated in 25 patients; 9 patients underwent cyst shunting. RESULTS: Among operated patients, 5 patients had grade 1 arachnopathy, 13 patients had grade 2 arachnopathy, 12 patients had grade 3 arachnopathy, and 4 patients had grade 4 arachnopathy. The minimal postoperative follow-up period was 11 months. After shunting, the condition improved in 8 of 9 patients; in 7 patients, the condition returned to the baseline level within the first postoperative year; in 6 (66.7%) of these patients, the disease continued to progress. After surgical release of spinal cord tethering, satisfactory long-term results were achieved in 13 (86.6%) patients with grade 1-2 arachnopathy. In 3 (50%) patients with grade 3 arachnopathy, the condition was stabilized. Among patients with grade 4 arachnopathy, progression of the disease was stopped in 1 patient; the condition worsened in 2 (50%) patients. Among all the operated patients, complications developed in 7 patients. There were no lethal outcomes. CONCLUSIONS: In grade 1-2 arachnopathy, progression of SM after release of spinal cord tethering occurs only in 13.4% of patients. Therefore, release of spinal cord tethering is recommended for these patients. In grade 3-4 arachnopathy, the rate of relapse after this surgery is more than 80%. Therefore, given the simplicity and a lower risk of complications of cyst shunting, this procedure is advisable for these patients.

Shunt tube placement for amelioration of cerebrospinal fluid flow obstruction caused by spinal cord subarachnoid fibrosis in dogs.

OBJECTIVE: To describe a novel technique for ameliorating cerebrospinal fluid flow obstruction secondary to pia-arachnoid fibrosis in dogs and report outcome. STUDY DESIGN: Descriptive report and retrospective case series. ANIMALS: Dogs with cerebrospinal fluid (CSF) flow obstruction (n = 7). METHODS: Medical records were searched for dogs that had a subarachnoid-subarachnoid shunt placed for treatment of CSF flow obstruction. Data collected included age, sex, breed, clinical signs and duration of signs prior to examination, neurologic status and localization prior to surgery, pre-surgical diagnostics, surgical technique, histopathology, postoperative neurologic examination, time to discharge from hospital, and outcome. RESULTS: All dogs were diagnosed at surgery with a fibrotic adhesion between the arachnoid and pia mater. A subarachnoid shunting tube was implanted to allow CSF flow across the lesion site. Five dogs showed improvement of clinical signs, 3 of which showed complete recovery and 2 of which showed improvement without resolution of all clinical signs. Two dogs showed no change at 7 and 24 months postoperatively. CONCLUSION: Bridging a region of pia-arachnoid fibrosis with a tube placed in the subarachnoid space can ameliorate or prevent progression of associated clinical signs.