Pancreatic iron in pediatric transfusion-dependent beta-thalassemia patients: A longitudinal MRI study.

BACKGROUND: In pediatric transfusion-dependent thalassemia (TDT) patients, we evaluated the prevalence, pattern, and clinical associations of pancreatic siderosis and the changes in pancreatic iron levels and their association with baseline and changes in total body iron balance. PROCEDURE: We considered 86 pediatric TDT patients consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. Iron overload (IO) was quantified by R2* magnetic resonance imaging (MRI). RESULTS: Sixty-three (73%) patients had pancreatic IO (R2* > 38 Hz). Global pancreas R2* values were significantly correlated with mean serum ferritin levels, MRI liver iron concentration (LIC) values, and global heart R2* values. Global pancreas R2* values were significantly higher in patients with altered versus normal glucose metabolism. Thirty-one patients also performed the follow-up MRI at 18 ± 3 months. Higher pancreatic R2* values were detected at the follow-up, but the difference versus the baseline MRI was not significant. The 20% of patients with baseline pancreatic IO showed no pancreatic IO at the follow-up. The 46% of patients without baseline pancreatic IO developed pancreatic siderosis. The changes in global pancreas R2* between the two MRIs were not correlated with baseline serum ferritin levels, baseline, final, and changes in MRI LIC values, or baseline pancreatic iron levels. CONCLUSIONS: In children with TDT, pancreatic siderosis is a frequent finding associated with hepatic siderosis and represents a risk factor for myocardial siderosis and alterations of glucose metabolism. Iron removal from the pancreas is exceptionally challenging and independent from hepatic iron status.

Axillary Cutaneous Hemosiderosis in a Patient With Hyperhidrosis, After Intravenous Iron Infusion.

ABSTRACT: Hemosiderosis consists of an iron deposit in tissues, which does not cause organic damage to them. However, in the case of the skin, being an organ exposed to sight, siderosis can produce a brownish coloration that is aesthetically discomforting for the individual. Most cutaneous sideroses are because of venous insufficiency with hemorrhagic extravasation. There is also a group secondary to iron extravasation in the injection site of the transfusion. However, there are very few cases in which an intravenous injection of an iron preparation has produced diffuse siderosis in extensive areas of the skin. We present the case of a 31-year-old woman with hyperhidrosis, who was transfused as a result of postpartum hemorrhage and, shortly after receiving an intravenous iron infusion, developed extensive hyperpigmentation in both axillae.

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.

Progressive loss of hearing and balance in superficial siderosis due to occult spinal dural defects.

PURPOSE: Superficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension. METHODS: Retrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur. RESULTS: The diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve. CONCLUSIONS: In patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.

Siderosis idiopática del sistema nervioso central y asociación con atrofia óptica e infarto cerebral. Caso clínico / Superficial siderosis of the central nervous system. Report of one case

Superficial siderosis of the central nervous system is produced by the deposit of hemosiderin within the subpial layers of the central nervous system and central parts of the cranial nerves, leading to progressive degeneration. We report a 55-year-old male who consulted for hearing loss and long-standing progressive decrease in visual acuity, associated with sudden onset of left hemiparesis. A brain CAT scan showed subacute ischemic lesions in the territory of the right posterior cerebral artery (thalamus and right subcortical temporal regions), old ischemic lesions in the right subcortical occipital regions and cerebellar atrophy. A magnetic resonance confirmed the lesions and the presence of superficial diffuse siderosis. A cerebrospinal fluid analysis showed slight xanthochromia, 26 leukocytes/mm3, glucose 51 mg/dL and proteins 1.23 g/L. He was managed with aspirin in low doses and statins. His motor function improved and was discharged two weeks after admission.

Cochlear Implantation Outcomes in Patients With Retrocochlear Pathology: A Systematic Review and Pooled Analysis.

OBJECTIVE: To review the current literature regarding cochlear implantation in patients with retrocochlear pathologies and extract speech perception scores between 6 months and 1 year after surgery. DATABASES REVIEWED: PubMed/MEDLINE, Embase and Cochrane CENTRAL via Ovid, CINAHL Complete via Ebsco, and Web of Science. METHODS: The review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Search strategies included keywords and subject headings to maximize retrieval and reflect cochlear implants and retrocochlear pathologies. Patients with previously resected vestibular schwannoma (VS) were excluded. RESULTS: There were 2,524 abstracts screened against inclusion criteria, and 53 studies were included, with individual data available for 171 adult patients. Pathologies included were either observed or irradiated VS (previously operated tumors were excluded) (n = 99, 57.9%), superficial siderosis (n = 39, 22.8%), neurosarcoidosis (n = 11, 6.4%), and previous central nervous system or skull base radiation (n = 22, 12.9%). Mean (standard deviation) postoperative consonant-nucleus-consonant (CNC) word scores were 45.4% (24.2) for observed VS, 44.4% (20.8) for irradiated VS, 43.6% (21.0) for superficial siderosis, 89.5% (3.0) for neurosarcoidosis, and 30.0% (30.2) in patients with previous central nervous system or skull base irradiation. Irradiated compared with observed VS had similar postoperative CNC word scores (effect size, 0.06; p = 0.71). Age, sex, maximal tumor dimension, and neurofibromatosis type 2 status did not significantly impact cochlear implant performance in patients with VS. Eighty-two percent of patients with reported device usage were daily users, and overall, 82% of cases benefitted from cochlear implantation. CONCLUSION: Cochlear implantation in patients with concomitant retrocochlear pathology generally results in improved speech discrimination scores sustained over time.

Superficial siderosis in long-standing pilocytic astrocytoma.

BACKGROUND: Pilocytic astrocytoma (PA) rarely spreads along neuraxis, and association with superficial siderosis (SS) and chronic signs of intracranial hypertension is exceptional. CASE REPORT: A 48-year-old woman presented with slow onset hearing loss in the past year. Clinical examination revealed dysarthria, positive Romberg test, and severe optic neuropathy. Cerebrospinal fluid (CSF) analysis showed numerous red blood cells, increased proteins and LDH, and high opening pressure. Brain and spine MRI demonstrated extensive superficial siderosis, bone remodeling of the skull base and spine, and diffuse nodular leptomeningeal enhancement. Histological examination of a nodule in the dorsal spine evidenced PA. CONCLUSION: We report a case of PA associated with dural remodeling and SS. The mechanism of SS is unclear but might be related to meningeal tumor infiltration and altered CSF composition and resorption.

Deep Brain Stimulation in a Patient with Parkinson's Disease and Cortical Superficial Siderosis.

Cortical superficial siderosis (cSS) is a rare condition that is regarded as a potential magnetic resonance marker of cerebral amyloid angiopathy (CAA). We describe the case of a 68-year-old man with cSS and Parkinson's disease (PD) who subsequently exhibited incidental microhemorrhages, which were only detected on magnetic resonance imaging (MRI), at one week after deep brain stimulation (DBS) surgery. cSS is now considered to be a significant risk factor for CAA and future bleeding. Therefore, because DBS surgery is invasive and may increase the risk of intracerebral hemorrhage, the procedure should be performed carefully when managing patients with PD and CAA.

Superficial siderosis of the central nervous system associated with ventral dural defects: bleeding from the epidural venous plexus.

OBJECTIVE: Superficial siderosis of the central nervous system is a rare intractable disease induced by chronic subarachnoid hemorrhage. Neurological deficits, such as cerebellar ataxia and hearing difficulties, gradually progress if left undiagnosed. Hemosiderin deposition is irreversible because standard medical treatment has not yet been established. Interventions at the source of bleeding may be the key to a preferable outcome of treatment for chronic subarachnoid hemorrhage; however, the source is not clear in many cases. METHODS: Among the consecutive cases diagnosed with a spontaneous cerebrospinal fluid (CSF) leak, cases of superficial siderosis associated with a CSF leak due to a ventral dural defect were retrospectively analyzed. RESULTS: Among 77 cases of a CSF leak, 7 cases (9%) of superficial siderosis were identified (median age of 59 years, male, 4 cases). Defects were diagnosed on 1-mm sliced fast imaging employing steady-state acquisition MRI (n = 5), conventional myelographic CT (n = 1), or dynamic myelographic CT (n = 1) at high thoracic levels (T1-T4). All defects were repaired by direct neurosurgery. During surgery, continuous bleeding from the epidural veins of the internal vertebral venous plexus was identified as the source of subarachnoid hemorrhage. Epidural CSF pulsations through the defect prevented clot formation by the epidural veins. Dural repair stopped free communication between the subarachnoid and epidural spaces, leading to the disappearance of chronic subarachnoid hemorrhage. CONCLUSION: Bleeding from the epidural venous plexus may be the cause of superficial siderosis associated with ventral dural defects. Neurosurgical repair may stop the progression of this condition.

Superficial siderosis of the central nervous system in a patient with asymptomatic sacral paraganglioma as source of chronic bleeding. / Siderosis superficial del sistema nervioso central en una paciente con paraganglioma sacro asintomático como fuente de sangrado crónico.

INTRODUCTION: Superficial siderosis of the central nervous system is an infrequent pathology secondary to chronic bleeding into the cerebrospinal fluid. Spinal tumors are infrequent cause of superficial siderosis being ependymoma the most common etiology. CASE REPORT: We report the case of a woman with sensorineural hearing loss and cerebellar ataxia, diagnosed of superficial siderosis on brain MRI. She had no previous history of axial back pain or radicular leg pain or bowel or bladder incontinence. On spine MRI an intradural lesion was found at the S1 level. No signs of intratumoral hemorrhage were observed on MRI gradient-echo images. At surgery, an intradural soft mass with signs of chronic bleeding was completely resected. Based on microscopic examination and immunohistochemistry of the specimen, a diagnosis of paraganglioma World Health Organization grade I was made. CONCLUSIONS: Since the only proven treatment able to prevent further deterioration from superficial siderosis is to stop chronic bleeding into subarachnoid space, is of paramount importance to establish an early diagnosis of the source of bleeding. Cases of unexplained superficial siderosis of central nervous system should include routine spinal MRI to rule out bleeding of spinal tumor even in asymptomatic patients. Due to severity of potential deterioration caused by superficial siderosis, any tumoral lesion observed on spinal MRI even without documented sings of bleeding should be considered for resection.

TITLE: Siderosis superficial del sistema nervioso central en una paciente con paraganglioma sacro asintomático como fuente de sangrado crónico.Introducción. La siderosis superficial del sistema nervioso central es una patología poco frecuente secundaria al sangrado crónico en el líquido cefalorraquídeo. Los tumores medulares son causa poco habitual de siderosis superficial, y el ependimoma es la etiología más común. Caso clínico. Mujer con pérdida auditiva neurosensorial y ataxia cerebelosa, diagnosticada de siderosis superficial en la resonancia magnética cerebral. No tenía antecedentes de dolor raquídeo axial, dolor radicular ni incontinencia esfinteriana. En la resonancia magnética de la columna se encontró una lesión intradural en S1. No se observaron signos de hemorragia intratumoral en las secuencias de resonancia magnética en eco de gradiente. En la cirugía, se apreció una masa blanda intradural con signos de sangrado crónico que se resecó. Basado en el examen microscópico e inmunohistoquímico de la muestra, se alcanzó el diagnóstico de paraganglioma de grado I de la Organización Mundial de la Salud. Conclusiones. Dado que el único tratamiento probado capaz de prevenir un mayor deterioro por la siderosis superficial es detener el sangrado crónico en el espacio subaracnoideo, es importante establecer un diagnóstico temprano de la fuente de sangrado. Los casos no justificados de siderosis superficial del sistema nervioso central deben incluir una resonancia magnética de la columna rutinaria para descartar el sangrado de un tumor medular, incluso en pacientes asintomáticos. Debido a la gravedad del deterioro potencial causado por la siderosis superficial, cualquier lesión tumoral observada en una resonancia magnética del raquis, incluso sin presentar signos de sangrado, debería ser objeto de indicación quirúrgica.

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.

[Benign Outcome of Cochlear Implantation in a Patient with Superficial Siderosis].

We report the case of a 38-year-old man with gait disorder and hearing loss. The patient had developed gait disorder due to a cervical meningioma since 4 year-old disappeared for 15 years after the surgical removal of the meningioma. However, at the age of 21 year-old, the gait disorder reappeared and worsened progressively. Sensorineural hearing loss (SNHL) and epileptic seizures developed during the disease course, and he was diagnosed with superficial siderosis (SS). When he was 37 years old, he experienced sudden-onset of right-side hearing impairment and was considered a candidate for cochlear implantation (CI) at the otorhinolaryngology clinic of the nearby University Hospital. He underwent CI in November 2014. Eight months after the operation, his right side hearing improved although ataxia, hearing loss, and pyramidal sign persisted. At the long term follow-up of 29 months after CI, his hearing remained at the improved level. Thus, CI may be an effective long-term treatment for SNHL in patients with SS and could prevent the progression of his hearing loss. (Received February 1, 2017; Accepted August 23, 2017; Published January 1, 2018).

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.

Superficial Siderosis Associated with Pineal Cavernous Malformation.

BACKGROUND: Cavernous malformations in the pineal region are rare and difficult to anticipate from preoperative evaluation in patients with pineal apoplexy. We herein report the first case of a pineal cavernous malformation with superficial siderosis. Radiological findings were helpful in identifying the presence of the cavernous malformation. CASE DESCRIPTION: A 47-year-old female presented with a 4-month history of progressive headache, nausea, and dizziness. She complained of double vision and exhibited upward gaze palsy and papilledema on fundoscopy. Radiological examination revealed subacute hemorrhage in the pineal region and enlarged lateral ventricles. Furthermore, T2-star-weighted gradient-echo magnetic resonance imaging demonstrated a linear hypointensity along the pial surface of the cerebral cortex, brainstem, and cerebellum, indicating hemosiderin deposition consistent with superficial siderosis. Suspecting the presence of a cavernous malformation based on the radiological findings of superficial siderosis, we performed total mass resection. The postoperative course was uneventful and her preoperative symptoms resolved completely. CONCLUSION: Radiological findings of superficial siderosis on T2-star-weighted gradient-echo imaging are useful to making a diagnosis of cavernous malformation in cases of pineal apoplexy. They are also important for making the treatment decision to perform total mass resection, which is the best curative method for pineal cavernous malformations.

Aneurysmal wall imaging in a case of cortical superficial siderosis and multiple unruptured aneurysms.

We report a case of interhemispheric and bifrontal cortical superficial siderosis in association with two intracranial aneurysms. The patient had no clinical history suggestive of aneurysm rupture, no feature of amyloid angiopathy or other apparent etiology for cortical siderosis. We performed high resolution brain MRI with dark blood T1 sequences before and after IV contrast injection. An anterior communicating aneurysm showed partial wall enhancement on the posterior wall whereas a left posterior communicating aneurysm did not. In the light of recent reports of the association of wall enhancement with unstable aneurysms, we considered wall enhancement to be a marker of inflammation and remodeling of the aneurysm wall, resulting in chronic hemorrhagic suffusion in the subarachnoid spaces. To our knowledge, this is the first report offering proof for a possible link between apparently unruptured aneurysms and cortical siderosis.