Surgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis.

BACKGROUND AND PURPOSE: Spinal cerebrospinal fluid (CSF) leaks may cause a myriad of symptoms, most common being orthostatic headache. In addition, ventral spinal CSF leaks are a possible etiology of superficial siderosis (SS), a rare condition characterized by hemosiderin deposits in the central nervous system (CNS). The classical presentation of SS involves ataxia, bilateral hearing loss, and myelopathy. Unfortunately, treatment options are scarce. This study was undertaken to evaluate whether microsurgical closure of CSF leaks can prevent further clinical deterioration or improve symptoms of SS. METHODS: This cohort study was conducted using data from a prospectively maintained database in two large spontaneous intracranial hypotension (SIH) referral centers in Germany and Switzerland of patients who meet the modified International Classification of Headache Disorders, 3rd edition criteria for SIH. Patients with spinal CSF leaks were screened for the presence of idiopathic infratentorial symmetric SS of the CNS. RESULTS: Twelve patients were included. The median latency between the onset of orthostatic headaches and symptoms attributed to SS was 9.5 years. After surgical closure of the underlying spinal CSF leak, symptoms attributed to SS improved in seven patients and remained stable in three. Patients who presented within 1 year after the onset of SS symptoms improved, but those who presented in 8-12 years did not improve. We could show a significant association between patients with spinal longitudinal extrathecal collections and SS. CONCLUSIONS: Long-standing untreated ventral spinal CSF leaks can lead to SS of the CNS, and microsurgical sealing of spinal CSF leaks might stop progression and improve symptoms in patients with SS in a time-dependent manner.

Postoperative Spinal Cerebrospinal Fluid-Venous Fistulas Associated With Dural Tears in Patients With Intracranial Hypotension or Superficial Siderosis-A Digital Subtraction Myelography Study.

BACKGROUND: Postoperative spinal cerebrospinal fluid (CSF) leaks are common but rarely cause extensive CSF collections that require specialized imaging to detect the site of the dural breach. OBJECTIVE: To investigate the use of digital subtraction myelography (DSM) for patients with extensive extradural CSF collections after spine surgery. METHODS: A retrospective review was performed to identify a consecutive group of patients with extensive postoperative spinal CSF leaks who underwent DSM. RESULTS: Twenty-one patients (9 men and 12 women) were identified. The mean age was 46.7 years (range, 17-75 years). The mean duration of the postoperative CSF leak was 3.3 years (range, 3 months to 21 years). MRI showed superficial siderosis in 6 patients. DSM showed the exact location of the CSF leak in 19 (90%) of the 21 patients. These 19 patients all underwent surgery to repair the CSF leak, and the location of the CSF leak could be confirmed intraoperatively in all 19 patients. In 4 (19%) of the 21 patients, DSM also showed a CSF-venous fistula at the same location as the postoperative dural tear. CONCLUSION: In this study, DSM had a 90% detection rate of visualizing the exact site of the dural breach in patients with extensive postoperative spinal CSF leaks. The coexistence of a CSF-venous fistula in addition to the primary dural tear was present in about one-fifth of patients. The presence of a CSF-venous fistula should be considered if CSF leak symptoms persist in spite of successful repair of a durotomy.

Clinical profile, demographic distribution, and outcomes of ocular siderosis: Electronic medical record-driven big data analytics from an eye care network in India.

Purpose: To describe the demographics, clinical profile, and outcomes of ocular siderosis in patients presenting to a multi-tier ophthalmology hospital network in India. Methods: This cross-sectional and hospital-based study included 3,082,727 new patients who presented between August 2010 and December 2021. Patients with a clinical diagnosis of ocular siderosis in at least one eye were included. Results: Overall, 58 eyes of 57 patients (0.002%) were diagnosed with ocular siderosis. The majority were men (96.49%) and had unilateral (98.25%) affliction. The most common age group at presentation was during the third decade of life with 24 patients (42.11%). A clear history of ocular trauma was documented in 47 patients (81.03%). Major clinical signs included corneal pigment deposition in nearly half of the eyes (27/58 eyes, 46.55%), corneal scar (20/58 eyes, 34.48%), cataract (22/58 eyes, 37.93%) and retinal detachment (11/58 eyes, 18.96%). The intraocular foreign body (IOFB) was anatomically localized in a majority of the eyes (i.e., 45/58 eyes, 77.59%). The most common location of the IOFB was in the posterior segment (22/58 eyes, 37.93%). The eyes that underwent a vitreoretinal surgery with removal of IOFB had a slightly better BCVA (1.0 ± 1.01) when compared to eyes with non-removal of IOFB (1.58 ± 1.00). Conclusion: Ocular siderosis is a rare sight-threatening entity, with half of the affected eyes exhibiting severe visual impairment. Majority of the eyes in ocular siderosis will have a detectable IOFB. Surgical removal of IOFB may lead to a better visual gain when compared to non-removal.

Safety and effectiveness of spinal dural defect repair in the management of superficial siderosis: A systematic review and patient-level analysis.

OBJECTIVE: Superficial siderosis (SS) is a disabling neurodegenerative condition that may be caused by spinal dural defects. Surgical repair is increasingly performed, however clinical outcomes remain unclear. METHODS: A systematic search of PubMed, MEDLINE, and EMBASE was conducted (inception to February 2020). Studies reporting cases of (i) superficial siderosis, (ii) spinal dural defect, (iii) and surgical closure of the defect were included. Demographic characteristics, clinical presentation, operative technique and clinical outcome were extracted for patient-level analysis. RESULTS: A total of 26 publications were included, which reported 38 patients with a median age of 58 years, and a male predominance (78.9 %). Ataxia (85.7 %) and hearing loss (80.0 %) were the most common presenting symptoms. The causative dural defect was most commonly ventral in location (91.7 %) and most commonly identified by CT myelography (48.6 %). Operative technique was highly variable and included primary suture, fibrin glue, dural substitute, or tissue (fat or muscle) graft. Clinical improvement was reported in 21 %, with stabilisation of symptoms in the majority (66 %) and clinical deterioration in 13.2 %. Surgical complications were observed in 7.9 %. CONCLUSION: In patients with superficial siderosis and spinal dural defect, operative closure leads to improvement or stabilisation of symptoms in the vast majority (87%) of patients.

Infratentorial Superficial Siderosis and Spontaneous Intracranial Hypotension.

OBJECTIVE: Spontaneous spinal cerebrospinal fluid (CSF) leaks cause intracranial hypotension (SIH) and also may cause infratentorial superficial siderosis (iSS) but the rate of development among different CSF leak types and outcome of treatment are not known. We determined the time interval from SIH onset to iSS and the outcome of treatment. METHODS: A total of 1,589 patients with SIH underwent neuroimaging and iSS was detected in 57 (23 men and 34 women, mean age = 41.3 years [3.6%]). We examined the type of underlying CSF leak by various imaging modalities. Percutaneous and surgical procedures were used to treat the CSF leaks. RESULTS: The iSS was detected in 46 (10.3%) of 447 patients with ventral CSF leaks, in 2 (3.9%) of 51 patients with dural ectasia, in 5 (2.6%) of 194 patients with CSF-venous fistulas, in 4 (0.9%) of 457 patients with simple meningeal diverticula, and in none of the 101 patients with lateral CSF leaks or the 339 patients with leaks of indeterminate origin (p < 0.001). The estimated median latency period from SIH onset to iSS was 126 months. Ventral CSF leaks could not be eliminated with percutaneous procedures in any patient and surgical repair was associated with low risk (<5%) and resulted in resolution of the CSF leak in all patients in whom the exact site of the CSF leak could be determined. Other types of CSF leak were treated with percutaneous or surgical procedures. INTERPRETATION: The iSS can develop in most types of spinal CSF leak, including CSF-venous fistulas, but mainly in chronic ventral CSF leaks, which require surgical repair. ANN NEUROL 2023;93:64-75.

[A case of ocular siderosis caused by iron ore].

A patient complained of vision loss of his left eye which was crushed by iron ore for 11 months. The cornea of the injured eye was thin and swollen, and a large amount of rust-like material was observed to be deposited. An intraocular foreign body was found by orbital CT. During vitrectomy, a piece of metal sheet was found near the ora serrate, and the intraocular structure was severely damaged, and characterized by vitreous brown turbidity, a white optic disc, occlusion of blood vessels in the fundus, and peripheral retinal atrophy with degeneration. The patient was diagnosed as ocular siderosis in the left eye.

Targeted detection and repair of a spinal dural defect associated with successful biochemical resolution of subarachnoid bleeding in classical infratentorial superficial siderosis.

BACKGROUND AND IMPORTANCE  : Classical infratentorial superficial siderosis (iSS) is characterised by repeated insidious bleeding into the subarachnoid space, leading to haemosiderin deposition within the subpial layers of the brainstem, cerebellum and spinal cord, sometimes with supratentorial involvement. Although nearly always associated with a dural defect (usually from previous trauma or neurosurgery) there is little evidence to support definitive investigation and management strategies. Here, we present a novel investigation strategy to identify a dural defect and subsequent successful surgical repair with biochemical resolution of subarachnoid bleeding. CLINICAL PRESENTATION: A 55-year-old gentleman presented with a 15-year progressive history of sensorineural deafness, followed by a slowly worsening gait ataxia. He had previously sustained cranio-spinal trauma. On examination there were features of myelopathy and ataxia. MRI demonstrated classical iSS, affecting cerebellum and cerebral cortices, with a cervicothoracic epidural CSF collection. Lumbar puncture (LP) revealed elevated ferritin 413 ng/mL and red cell count of 4160. Reverse CT myelography, a novel technique involving contrast injection into the collection, delineated a dural defect at the T9/T10 level that was not present on conventional myelography. Following surgical repair, repeat LP twelve months later demonstrated biochemical improvement (ferritin 18 ng/mL, red cells < 1). There was no further neurological deterioration in symptoms during eighteen months follow-up. CONCLUSION: We show the value of a rational targeted investigation pathway in identifying a surgically reparable dural defect underlying classical iSS. We also provide proof of concept that surgical repair can facilitate biochemical resolution of subarachnoid bleeding and might prevent progression of neurological disability.

Quantitative clinical and radiological recovery in post-operative patients with superficial siderosis by an iron chelator.

BACKGROUND: Superficial siderosis is a rare neurodegenerative disease caused by hemosiderin deposition on the brain surface. Although the efficacy of the iron chelator-deferiprone-in superficial siderosis has recently been documented, a comparative study of patients who underwent surgical ablation of their bleeding source and subsequently received treatment with or without deferiprone has not yet been conducted. METHODS: Fifteen postoperative patients with superficial siderosis were recruited, and seven patients were administered deferiprone (combination therapy group). Quantitative changes in the hypointense signals on T2*-weighted magnetic resonance images were acquired; additionally, cerebellar ataxia was assessed (International Cooperative Ataxia Rating Scale score and Scale for the Assessment and Rating of Ataxia). Audiometry was performed and the results were compared with those of patients who did not receive deferiprone (surgical treatment group; controls). RESULTS: Significant improvements in signal contrast ratios were noted in the lateral orbitofrontal gyrus, superior temporal lobe, insular lobe, brainstem, lingual gyrus, and cerebellar lobe in the combination therapy group. The scores of patients in the combination therapy group on the cerebellar ataxia scales significantly improved. The degree of signal improvement in the cerebellar lobe correlated with the improvement of cerebellar ataxia scores. Early deferiprone administration after disease onset and long-term administration were correlated with greater signal improvements on magnetic resonance imaging. No adverse effects were observed in the clinical or laboratory parameters. CONCLUSIONS: Deferiprone administration significantly improved radiological and clinical outcomes in patients with postoperative superficial siderosis. Earlier and longer courses of deferiprone could result in better patient prognosis.

Superficial siderosis associated with an iatrogenic posterior fossa dural leak identified on CT cisternography.

Superficial siderosis refers to hemosiderin deposition along the pial surface of the brain and spinal cord. It results from chronic and repetitive low-grade bleeding into the subarachnoid space. Dural tears are a common cause of superficial siderosis. Although such tears typically occur in the spine, dural tears can also occur in the posterior fossa. In many cases, posterior fossa dural tears are iatrogenic, and patients may present with neuroimaging evidence of postoperative pseudomeningoceles. We present a case of superficial siderosis caused by a persistent posterior fossa dural leak. The patient presented with superficial siderosis 30 years after a Chiari I malformation repair. A pinhole-sized dural tear was identified preoperatively using computed tomography cisternography. The dural defect was successfully repaired. An additional small tear that was not seen on imaging was also identified at surgery and successfully repaired.

Outcomes of Cochlear Implantation in Patients with Superficial Siderosis: A Systematic Review and Narrative Synthesis.

This study aimed to establish outcomes following cochlear implantation (CI) in patients with superficial siderosis (SS). MEDLINE, Embase, Web of Science, Cochrane, and ClinicalTrials.gov databases were searched for this systematic review. No limits were placed on the language or the year of publication. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Of a total of 46 studies, 19 studies met the inclusion criteria reporting outcomes in 38 patients. Of the 44 implants, 23 implants (52.27%) had good hearing outcomes at the last follow-up, 9 (20.45%) were initially beneficial for the patient, but then the performance deteriorated (4 of which were re-implanted), and 12 (27.27%) were not beneficial for the patient. All studies were classified as grade 4 studies using the Oxford Centre for Evidence-Based Medicine (OCEBM) grading system, being retrospective in nature and consisting of case reports and noncontrolled case series with a small number of patients. Of the 44 implants, 32 (72.73%) showed improved hearing outcomes following CI for SS at some point in time, of which 23 (52.2%) implants showed sustained benefit at the last follow-up (average follow-up time was 21.97 months). It is difficult to predict the longevity of benefit owing to the progressive nature of the disease or the patients in whom it may be beneficial, as the preoperative investigations inadequately predict benefit. Preimplantation and postimplantation counseling with the patient and their family regarding the potential limited benefit and eventual parallel decline with the neurological disease are crucial, along with thorough clinical investigations.

Role of Transnasal Endoscopic Surgery in the Treatment of Superficial Siderosis of Central Nervous System Secondary to Clivus Arachnoidocele: Report of Successful Case and Literature Review.

BACKGROUND: Superficial siderosis (SS) of the central nervous system is a disease characterized by deposition of hemosiderin in the leptomeninges (arachnoid and pia mater) due to chronic intradural bleeding. One of the etiologic mechanisms proposed is a dural breach secondary to trauma with a consequent arachnoidocele in contact with an exuberant venous plexus. We describe a unique case of clival arachnoidocele treated by an endoscopic endonasal approach and closure of the defect with fat and nasoseptal flap. CASE DESCRIPTION: A 35-year-old man with a history of severe head trauma 20 years ago presented with hearing deficit and a mild motor ataxia impairing gait. Magnetic resonance imaging disclosed hemosiderin deposition throughout the brain cortical layer and in the cerebellum, affecting the dentate nucleus as well. A computed tomography revealed an osteolytic formation in the clivus, involving the inner bone table and bone marrow. The patient was then submitted to an endoscopic endonasal transclival approach to close the defect. CONCLUSIONS: SS may be a result of several etiologies generating repetitive meningeal bleeding. Our patient had the diagnosis of posttraumatic clival arachnoidocele and SS probably related to trauma with some dural injury. An endoscopic endonasal approach with tear reconstruction is feasible and successful to address clival arachnoidoceles and, in this case, to avoid progression of the SS.

Superficial Siderosis Associated with Craniopharyngioma.

BACKGROUND: Superficial siderosis (SS) associated with craniopharyngioma is an extremely rare occurrence. To our knowledge, only 3 cases have been reported in literature. Two of the cases occurred following resection of the tumor. Similar to the present case, 1 of the cases was discovered before surgical intervention, "pure tumor-related" SS. The clinical presentation, diagnosis, management, and outcome are discussed in this article. CASE DESCRIPTION: A 50-year-old man presented with an 11-month history of left-sided tinnitus, hearing impairment, and a 2-month history of visual disturbance. Cerebellar ataxia and gait disturbance were found on examination. Brain magnetic resonance imaging findings were consistent with SS. Cerebrospinal fluid (CSF) analysis before surgery revealed xanthochromia confirming an existing chronic sustainable subarachnoid hemorrhage. He underwent a tumor resection in endoscopic endonasal approach followed by stereotactic radiosurgery. On follow-up, the visual symptoms resulting from craniopharyngioma subsided, whereas hearing impairment, cerebellar ataxia, and gait disturbance associated with SS did not progress but were unchanged despite subsequent evidence of watery-clear CSF. CONCLUSIONS: In the present case, SS could be associated with craniopharyngioma on the basis of CSF findings and clinical symptoms. Detection and early treatment aimed at eliminating the bleeding source offer the best chance of halting the disease process.

Updating superficial siderosis of the central nervous system: bleeding of a dorsal osteophyte into the subarachnoid space from a perforating artery.

Superficial siderosis of the central nervous system (SSCNS) is an uncommon and often unrecognized disorder that results from recurrent and persistent bleeding into the subarachnoid space. Currently, there is no effective treatment for SSCNS. The identification and surgical resolution of the cause of bleeding remains the most reliable method of treatment, but the cause of bleeding is often not apparent. The identified sources of recurrent bleeding have typically included neoplasms, vascular malformations, brachial plexus or nerve root injury or avulsion, and previous head and spinal surgery. An association between recurrent bleeding in the CNS and dural abnormalities in the spine has recently been suggested. Dural tears have been identified in relation to a protruding disc or osteophyte. Also in these patients, the exact mechanism of bleeding remains unknown because of a lack of objective surgical data, even in patients who undergo neurosurgical procedures.The present case concerns a 48-year-old man who presented with longstanding symptoms of mild hearing loss and mild gait ataxia. A diagnosis of SSCNS was made in light of the patient's history and the findings on physical examination, imaging, and laboratory testing. MRI and CT detected a small calcific osteophyte in the anterior epidural space of T8-9. The patient underwent surgical removal of the bone spur and dural tear repair. During the surgery, the authors detected a perforating artery, which was on the osteophyte, that was bleeding into the subarachnoid space. This case shows a possible mechanism of chronic bleeding from an osteophyte into the subarachnoid space. In the literature currently available, a perforating artery on an osteophyte bleeding into the subarachnoid space has never been described in SSCNS.

Superficial Siderosis of Central Nervous System as Primary Clinical Manifestation Secondary to Intradural Thoracic Disk Herniation.

BACKGROUND: Superficial siderosis of the central nervous system is a rare neurologic disorder characterized by the superficial deposition of hemosiderin in the subpial layer resulting in iron-related progressive neurodegeneration. CASE DESCRIPTION: In this report, we present a case of superficial siderosis of the central nervous system secondary to an intradural thoracic disk herniation causing a cerebrospinal fluid (CSF) leak. CONCLUSIONS: The patient was successfully treated with T6-T8 transpedicular partial corpectomy, as well as diskectomy with decompression followed by watertight closure of the CSF leak. Intraoperative watertight closure of the CSF leak was achieved.

Spinal endoscopy combined with selective CT myelography for dural closure of the spinal dural defect with superficial siderosis: technical note.

The authors describe a new procedure to detect the tiny dural hole in patients with superficial siderosis (SS) and CSF leakage using a coronary angioscope system for spinal endoscopy and selective CT myelography using a spinal drainage tube. Under fluoroscopy, surgeons inserted the coronary angioscope into the spinal subarachnoid space, similar to the procedure of spinal drainage, and slowly advanced it to the cervical spine. The angioscope clearly showed the small dural hole and injured arachnoid membrane. One week later, the spinal drainage tube was inserted, and the tip of the drainage tube was located just below the level of the dural defect found by the spinal endoscopic examination. This selective CT myelography clarifies the location of the dural defect. During surgery, the small dural hole could be easily located, and it was securely sutured. It is sometimes difficult to detect the actual location of the small dural hole even with thin-slice MRI or dynamic CT myelography in patients with SS. The use of a coronary angioscope for the spinal endoscopy combined with selective CT myelography may provide an effective examination to assess dural closure of the spinal dural defect with SS in cases without obvious dural defects on conventional imaging.

A case of superficial siderosis ameliorated after closure of dural deficit detected by MRI-CISS (constructive interference in steady state) imaging.

A 64-year-old male developed headache, dizziness, and difficulty hearing, two years after an operation for chronic subdural hematoma due to head injury. These symptoms gradually worsened over the following 15 years. As he showed bloody cerebrospinal fluid (CSF) and marginal hypointensity on the surface of the brain and spinal cord on T2/T2*-weighted MRI, he was diagnosed with superficial siderosis (SS), although the source of the bleeding was unclear and anti-hemorrhagic drugs were ineffective. When he was admitted to our hospital, neurological examination disclosed horizontal gaze-evoked nystagmus, severe bilateral hearing loss, scanning speech, and limb and truncal ataxia. CISS (constructive interference in steady state) MRI detected a dural defect at the Th2-3 level on the anterior side of the spinal canal. On operation, a 2 mm × 6 mm size dural defect with blood clots was found at the Th2-3 level. After closure of the dural defect, bloody CSF became transparent, and his persistent headache, dizziness, and hearing impairment improved. Brain and whole spine MRI, especially CISS imaging, should be considered for detecting the source of bleeding in intractable cases of SS.

Superficial siderosis associated with peripheral autonomic failure and tetraventricular hydrocephalus: a case report.

We describe the case of a man whose initial clinical presentation included sensorineural hearing loss and orthostatic hypotension. The patient was diagnosed with superficial siderosis associated with peripheral autonomic failure and tetraventricular hydrocephalus.

Detection of Endogenous Iron Reduction during Hepatocarcinogenesis at Susceptibility-Weighted MR Imaging: Value for Characterization of Hepatocellular Carcinoma and Dysplastic Nodule in Cirrhotic Liver.

OBJECTIVE: To investigate the value of susceptibility-weighted imaging (SWI) for characterization of hepatocellular carcinoma (HCC) and dysplastic nodule (DN). MATERIALS AND METHODS: Sixty-eight cirrhotic patients with 89 hepatocellular nodules underwent SWI. The radiological features of hepatocellular nodules on SWI were classified into three types: type A (iso- or hypointensity, and background liver siderosis), type B (hyperintensity, and background liver siderosis), or type C (hyperintensity, and no background liver siderosis). Intranodular and background liver iron content was quantified and correlated with SWI pattern. Prussian blue staining was performed to quantify intranodular and background liver iron content. RESULTS: Type A pattern (n = 12) contained 11 (91.7%) DNs and 1 (8.3%) HCC, Type B pattern (n = 66) comprised 1 (1.5%) DN and 65 (98.5%) HCCs (including 12 DN-HCCs and 53 overt HCCs), and type C pattern (n = 11) was exclusively seen in HCCs. The iron scores of DN-HCCs and overt HCCs were significantly lower than those of background livers [(0.091±0.30) VS (2.18±0.87), P = 0.000; (0.11±0.41) VS (2.16±0.97), P = 0.000; respectively]. There was no significant difference between iron scores of DNs and those of background livers [(1.92±0.29) VS (2.17±039), P = 0.191]. For lesion-based and patient-based analysis of HCCs (DN-HCCs and overt HCCs), type B pattern showed a sensitivity, specificity, accuracy, positive predicative value (PPV), and negative predicative value (NPV) of 84.4% and 84.4%, 91.7% and 75%, 85.4% and 83.8%, 98.5% and 98.2%, 47.8% and 23.1%, respectively. CONCLUSION: SWI can provide valuable information for characterization of HCC and DN based on endogenous iron reduction during hepatocarcinogenesis.