Retained Intraocular Iron Foreign Body Leading to Late-Onset Siderotic Glaucoma: A Challenging Case Report.

BACKGROUND A retained ferrous intraocular foreign body (IOFB), introduced via penetrating ocular trauma, may result in ocular siderosis and visual loss that may occur after days or years. If diagnosis is delayed, therapy may also be delayed, resulting in a poor outcome. The present report presents the case of a 58-year-old man with a retained iron IOFB and late-onset siderotic glaucoma 1 month after the initial trauma. CASE REPORT A 58-year-old man presented with redness and eye pain in the right eye for 1 month after ocular trauma. His visual acuity was very good, with no sign of eye strain. High intraocular pressure had been detected for several weeks, but the B-scan ultrasound and fundus examination were normal and the reason for the high intraocular pressure was unknown. He was later transferred to our senior hospital. The diagnosis of IOFB was confirmed by computed tomography (CT) scan and ultrasound biomicroscopy (UBM). The patient was successfully managed by vitrectomy. CONCLUSIONS This report highlights that a retained IOFB can be challenging to diagnose and that cases associated with siderotic glaucoma require multiple investigations. Early detection of the IOFB using the right tools is vital to reduce the risk of siderotic glaucoma. Although the fundus examination was normal after ocular trauma, the use of CT scan and UBM assisted in finding the IFOB and the patient was successfully treated by vitrectomy.

Cerebellar Superficial Siderosis in Cerebral Amyloid Angiopathy on 1.5T T2*-Weighted Imaging.

BACKGROUND: Cerebellar superficial siderosis (SS) has been recently reported to be present in about 10% of both hereditary and sporadic cerebral amyloid angiopathy (CAA) on 3T MRI using primarily susceptibility-weighted imaging. OBJECTIVES: Our aim was to assess cerebellar SS in sporadic CAA patients using 1.5T T2*-weighted MRI and to evaluate possible underlying mechanisms. METHOD: We retrospectively screened MRI scans of sporadic probable CAA patients initially presenting with intracerebral hemorrhage-, acute subarachnoid hemorrhage- or cortical SS-related symptoms between September 2009 and January 2022 registered in our stroke database. Patients with familial CAA were excluded. On 1.5T T2*-weighted MRI, cerebellar SS (including kappa statistics for interobserver agreement) was assessed together with typical CAA hemorrhagic features and with the presence of supratentorial macrobleed and cortical SS adjacent to the tentorium cerebelli (TC) and TC hemosiderosis. RESULTS: We screened 151 patients and finally included 111 CAA patients (median age 77) with cerebellar SS observed in 6 (5%) patients. Cerebellar SS presence was associated with a higher number of supratentorial macrobleeds (median n = 3 vs. n = 1, p = 0.0012), presence of supratentorial macrobleed adjacent to the TC (p = 0.002), and TC hemosiderosis (p = 0.005). CONCLUSIONS: Cerebellar SS in CAA patients can be identified on 1.5T T2*-weighted imaging. Associated MRI characteristics suggest contamination from supratentorial macrobleeds.

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.

Cerebral Superficial Siderosis : Etiology, Neuroradiological Features and Clinical Findings.

Superficial siderosis (SS) of the central nervous system constitutes linear hemosiderin deposits in the leptomeninges and the superficial layers of the cerebrum and the spinal cord. Infratentorial (i) SS is likely due to recurrent or continuous slight bleeding into the subarachnoid space. It is assumed that spinal dural pathologies often resulting in cerebrospinal fluid (CSF) leakage is the most important etiological group which causes iSS and detailed neuroradiological assessment of the spinal compartment is necessary. Further etiologies are neurosurgical interventions, trauma and arteriovenous malformations. Typical neurological manifestations of this classical type of iSS are slowly progressive sensorineural hearing impairment and cerebellar symptoms, such as ataxia, kinetic tremor, nystagmus and dysarthria. Beside iSS, a different type of SS restricted to the supratentorial compartment can be differentiated, i.e. cortical (c) SS, especially in older people often due to cerebral amyloid angiopathy (CAA). Clinical presentation of cSS includes transient focal neurological episodes or "amyloid spells". In addition, spontaneous and amyloid beta immunotherapy-associated CAA-related inflammation may cause cSS, which is included in the hemorrhagic subgroup of amyloid-related imaging abnormalities (ARIA). Because a definitive diagnosis requires a brain biopsy, knowledge of neuroimaging features and clinical findings in CAA-related inflammation is essential. This review provides neuroradiological hallmarks of the two groups of SS and give an overview of neurological symptoms and differential diagnostic considerations.

[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.

[Pulmonary siderosis in a welder, diagnosis on unusual pneumopathy]. / Pneumopathie atypique chez un soudeur : sidérose pulmonaire ?

INTRODUCTION: Pulmonary siderosis or welder's lung is an occupational lung disease that is usually observed after chronic exposure to iron dust. OBSERVATION: A 55-years-old welder visited hospital with dyspnea linked to occupational exposure. Pulmonary function studies revealed lung function abnormalities with decerase of FEV1 and TPC. Based on the chest Tomography CT results, he was diagnosed with obliterans bronchiolitis. A chest biopsy was performed and the specimen is for a pulmonary siderosis aspect. DISCUSSION: This case of pulmonary siderosis is an unusual one by symptoms, CT images and short latency of exposure. An intense inhalation of iron particles could explain this case and inflammatory process and it highlights need of histological analysis of chest biopsy.

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.

[Cerebral amyloid angiopathy-related transient focal neurological episodes with diffusion-weighted imaging hyperintense lesions in subcortical white matter and cortical superficial siderosis].

A 72-year-old man presented with two episodes of migratory left-sided paresthesia lasting 10 min. At the first episode, diffusion-weighted imaging hyperintense lesions (DWIHLs) were seen in the right parietal lobe, suggesting an initial diagnosis of acute ischemic stroke, for which we administered antiplatelet therapy for secondary prevention. Four months later, he again developed transient migratory left-sided paresthesia. Gradient-echo T2*-weighted imaging at this time showed disseminated cortical superficial siderosis (cSS) and strictly cerebral microbleeds around the DWIHLs in the right parietal lobe. These findings led to a diagnosis of cerebral amyloid angiopathy and its related findings, including transient focal neurological episodes (TFNE) and DWIHLs, and antiplatelet medication was stopped. In clinical settings, although it is challenging to distinguish TFNE of hemorrhagic origin from cerebral ischemic symptoms, including transient ischemic attacks, this case suggests that even when elderly patients with transient neurological symptoms present with cortical DWIHLs, paramagnetic-sensitive MRI should be performed to check for cSS around the DWIHLs.

Different etiologies of superficial siderosis / Diferentes etiologias de siderose superficial

Superficial Siderosis (SS) is an uncommon condition caused by hemosiderin deposition into the subarachnoid space. SS is characterized by cerebellar ataxia, progressive sensorineural hearing loss and pyramidal signs, but is often an unrecognized disorder. Magnetic Resonance Imaging (MRI) is the diagnostic procedure of choice due its high sensitivity to hemosiderin deposits in addition to being a non-invasive exam. This paper aims to describe a case of SS and to perform a literature review about SS etiologies, neuroimaging features and clinical characteristics. A 65-year-old man came to a neurology outpatient clinic with seizures and cerebellar ataxia with a history of car accident and severe traumatic brain injury 45 years ago. MRI SWAN showed a hyposignal in the cisterns of the base and on the cerebellar surface and T1-weighted images left hippocampal sclerosis.

A Siderose Superficial (SS) é uma condição rara causada por depósitos de hemossiderina no espaço subaracnóideo. SS é caracterizada por ataxia cerebelar, perda neurosensorial auditiva progressiva e sinais piramidais, mas é frequentemente uma desordem de difícil diagnóstico. A Ressonância Magnética (RM) é o exame de escolha para o diagnóstico devido a sua alta sensibilidade aos depósitos de hemossiderina, além de ser um exame não invasivo. Este artigo tem como objetivo descrever um caso de SS e realizar uma revisão da literatura sobre as etiologias da SS, suas características na neuroimagem e suas características clínicas. Um homem de 65 anos de idade procurou o ambulatório de neurologia com convulsões e ataxia cerebelar. Ele informou histórico de acidente automobilístico e lesão cerebral traumática grave há 45 anos. A RNM SWAN mostrou hipossinal nas cisternas da base e na superfície cerebelar e as imagens em T1 evidenciaram a presença de esclerose hipocampal esquerda.

Unilateral Ocular Siderosis Bulbi Due to Missed Metallic Intraocular Foreign Body Masquerading as Anisocoria of Neurological Origin: A Case Report.

BACKGROUND Ocular siderosis is an uncommon cause of vision loss due to a retained ferrous intraocular foreign bodies (IOFB) that cause iron deposition in ocular tissues. The most common manifestations are cataract formation, diffuse pigmentary changes of the retinal pigment epithelium, iris heterochromia, dilated pupils, secondary glaucoma, iritis, and cystoid macular edema. CASE REPORT We report a case of 38-year-old man who presented with a left dilated pupil and visual field defect. Neurological examination results were normal. Brain magnetic resonance imaging revealed a gross artifact at the site of the left globe. The visual field test showed a peripheral arcuate nasal visual field defect in the left eye. Ophthalmic examination revealed peripheral pigmentary changes and a black elongated and elevated lesion located very anterior and inferior-temporal and attached to the retina with fibrous tissue. A computed tomography scan revealed a 1×1-mm-round hyperdense IOFB in the left vitreous cavity. The diagnosis of siderosis bulbi secondary to a missed IOFB was established. The patient underwent a pars plana vitrectomy for removal of the IOFB. Two weeks later, rhegmatogenous retinal detachment developed, and repair with silicon oil injection was done. One year after the last operation, the best corrected visual acuity in the left eye was 6/120, with normal intraocular pressure and an attached posterior pole. CONCLUSIONS This case highlights the importance of investigating for a retained IOFB in cases of unilateral retinitis pigmentosa changes.

Retained iron intraocular foreign body with late onset siderotic glaucoma: a clinical challenge.

Siderotic glaucoma is a rare and challenging clinical scenario caused by a retained iron intraocular foreign body (IOFB), which results in irreversible vision loss. Presented here is a case of secondary open-angle glaucoma in a 36-year-old man that was refractory to maximal medical therapy. A meticulous history and careful clinical examination revealed that he had a penetrating trauma 15 years ago, a self-sealed corneal entry wound, open angles, a retained encapsulated metallic IOFB and siderosis bulbi. Removal of the foreign body not only controlled intraocular pressure, but also improved vision.

Isolated cognitive dysfunction in the presence of superficial siderosis after meningioma resection at the cervicothoracic junction.

Superficial siderosis is a rare disorder characterised by the deposition of haemosiderin on the surface of the central nervous system. Cognitive dysfunction has sporadically been reported in relation with superficial siderosis. We present a 61-year-old man with cognitive dysfunction in the presence of the typical radiological image of temporal and cerebellar superficial siderosis, most likely due to pseudomeningocoele 14 years after resection of a meningioma at the cervicothoracic junction. Xantochromia was present on cerebrospinal fluid investigation and a source of bleeding was seen during surgical exploration. Despite surgical treatment of the suspected bleeding source, the patient deteriorated and neuropsychological examination 1 year after surgery showed progression of cognitive dysfunction to dementia. It is likely that in the absence of other typical symptoms such as cerebellar ataxia and hearing loss, the cognitive dysfunction was not related to the superficial siderosis.

Hemochromatosis and Hepatocellular Carcinoma Secondary to Immunoglobulin G4-Related Disease with Hepatopathy: A Case Report.

Immunoglobulin G4-related disease (IgG4-RD) is a recently characterized illness in which lymphocytes and plasma cells infiltrate various anatomical sites. IgG4-hepatopathy, a manifestation of IgG4-RD, is a broader term covering various patterns of liver injury. The clinical course, including the malignant potential of IgG4-RD, remains unclear. Here we report the first case of secondary hemochromatosis and hepatocellular carcinoma (HCC) developing from IgG4-hepatopathy. A 67-year-old man was admitted to our hospital for treatment of deteriorating glucose tolerance. Blood test results showed hypergammaglobulinemia, especially IgG4. He was readmitted 2 months later with dyspnea due to lung disease and pleural effusion, and elevated transaminase levels. He underwent liver and lung biopsies. IgG4-RD was diagnosed and he was treated with steroid therapy, which improved serum IgG4 levels and imaging abnormalities. A follow-up computed tomography (CT) scan conducted 38 months later revealed a tumor (diameter, 50 mm) in liver segments 7 and 8. The resected specimen revealed HCC and abundant siderosis in the background liver, indicating a diagnosis of hemochromatosis. IgG4-positive cells were scarce, probably because of corticosteroid therapy. In the present case, IgG4-RD was well controlled with prednisolone (PSL) and an immunosuppressive agent, and chronic hepatitis was not severe, even though the patient subsequently developed HCC. However, extensive siderosis consistent with hemochromatosis was unexpectedly noted. These findings suggest that secondary hemochromatosis and HCC developed during IgG4-RD with hepatopathy. We believe this case sheds light on IgG4-RD.

Siderotic glaucoma without detectable intraocular foreign body in a pseudophakic eye: a case report.

BACKGROUND: Ocular siderosis is induced by a retained intraocular foreign body (IOFB) containing iron and can present as siderotic glaucoma. We report a rare case of histopathologically proven siderotic glaucoma in a middle-aged blacksmith with a preceding history of ocular trauma but no radiologically detectable IOFB. CASE PRESENTATION: A 42-year-old blacksmith presented with an elevation of intraocular pressure (IOP) in left eye showing iris heterochromia and brownish deposits throughout the trabecular meshwork (TM). Preoperative ophthalmic examination did not reveal any retained IOFBs. Electroretinography showed the classic changes of retinal degeneration in ocular siderosis. Histopathologic staining of the TM verified the presence of iron deposits. CONCLUSION: This case underlines the importance of the close monitoring of patients with a history of ocular trauma and highlights the necessity of electroretinography, histopathologic study, and detailed ophthalmic examination in the diagnosis of siderotic glaucoma, even if there is no definite radiologically detectable IOFB.

Superficial Siderosis of the Central Nervous System Caused by Glioneuronal Tumor: A Case Report and Literature Review.

Superficial siderosis is a rare disease resulting from the deposited hemosiderin owing to repeated subarachnoid hemorrhage. It has been reported that hemosiderin deposits on the brain surface and brain parenchyma causes nerve disorder, resulting in progressive and irreversible hearing loss, cerebellar ataxia and pyramidal disorder. The brain tumor is one of the cause of superficial siderosis. A 16-year-old female present a nearby hospital with complaints of absence seizure. A magnetic resonance imaging (MRI) revealed a heterogeneously enhanced mass at the right temporal lobe. Susceptibility-weighted imaging revealed diffuse and extensive superficial siderosis on the brain surface. The tumor was gross totally removed and diagnosed as glioneuronal tumor. The patient had been well, although susceptibility-weighted imaging performed one year after the surgery showed superficial siderosis remained. Early diagnosis and prevention of bleeding sources are recommended to prevent symptom progression associated with superficial siderosis. Susceptibility-weighted imaging is considered useful for early detection of superficial siderosis.

Optical coherence tomographic findings of ocular siderosis following intraocular foreign body removal: A case report.

RATIONALE: Ocular siderosis is arrested by the removal of intraocular foreing body (IOFB). The progression of ocular siderosis is very rare and few reports demonstrate the optical coherence tomographic (OCT) findings. PATIENT CONCERNS: A 55-year-old Asian man presented to our clinic with the chief complaint of decreased vision in his left eye for 5 months. On slit lamp examination of the left eye, the corneal stroma had a rust-colored hue, and the retina was not visible due to vitreous opacity. An orbital computed tomography was ordered considering the possibility of left IOFB, which confirmed the presence of a vitreous IOFB. On the next day, he had a continuous curvilinear capsulorrhexis with phacoemulsification and intraocular lens implantation, pars plana vitrectomy, and removal of IOFB in the left eye. Six years later, he revisited our clinic. On slit lamp examination, the corneal haziness had worsened, and the iris showed heterochromia resembling the spokes of a wheel in the left eye. DIAGNOSIS: Ocular siderosis. INTERVENTION: Anterior and posterior segment OCT was performed. OUTCOMES: The anterior segment OCT showed linear hyperreflectivity on the anterior corneal stroma just beneath the Bowman's layer. The posterior segment OCT showed inner retinal degeneration observed at the parafoveal area. LESSONS: Ocular siderosis progression can happen after the removal of IOFB. The swept source OCT might be useful to assess the cornea and retina in ocular siderosis patient with corneal haziness.

Ocular siderosis resulting from a retained intralenticular metallic foreign body.

Intraocular foreign bodies are a potential factor threatening with loss of vision. The development of cataract and symptoms of ocular siderosis are the most common signs of ferrous metal entering the eye. We present a case of a 45-year-old man who reported to the hospital for planned cataract surgery. He denied the possibility of any past eye injury. Despite this, apart from the cataract, X-ray and CT scans confirmed the presence of an intralenticular foreign body and symptoms of ocular siderosis. Cataract surgery was successfully performed using phacoemulsification, and the metallic foreign body was removed. Intraocular foreign body symptoms may be overlooked by patients and even physicians and may occur with considerable delay. Hence, in patients with indirect symptoms of penetrating eye injury, the presence of an intraocular foreign body should not be ruled out, even if the patient denies this possibility.

Successful Treatment of Iatrogenic Cutaneous Siderosis with Pigment Lasers: A Retrospective Study in 15 Consecutive Patients.

Intravenous ferric carboxymaltose is increasingly used to treat iron deficiency. However, a common side-effect is paravenous extravasation of iron preparations, resulting in cutaneous siderosis. Quality-switched (QS) lasers and, recently, picosecond (PS) lasers have been used to treat these hyperpigmentations with variable success. The optimal treatment protocol remains unclear. The aims of this study were to assess the response of cutaneous siderosis to treatment with pigment lasers and to determine the optimal wavelength, number of treatment sessions and pulse duration. Fifteen patients with cutaneous siderosis on the arms were included. The effectiveness of laser treatment was evaluated using a 5-point standard Physician Global Assessment (PGA) grading system. Differences in continuous variables between distinct groups of patients were assessed with a Mann-Whitney U test. In all 15 patients clearance of at least 50% was obtained. In 12 patients, at least 75% of pigment was removed. In conclusion, pigment lasers are an effective and safe method to treat cutaneous siderosis.

A case of post-splenectomy transfusion-dependent homozygous beta-thalassemia major complicated with myocardial siderosis and osteoporosis and usage of iron-chelating therapy with deferiprone in pregnancy.

Beta-thalassemia major is a subtype component of hemoglobinopathies; autosomal recessive disorders complicated with anemia that affect at least 50,000 babies each year. It contributes to problems in reproductive entities such as infertility due to iron deposition in the endocrine organs, which leads to malfunction of the hypothalamus-pituitary axis. Due to this, there have been very few pregnancies discovered and reported with this type of condition as they usually required an ovulation-induction agent with assisted reproductive technique to achieved pregnancy. We report a successful spontaneous pregnancy in a woman with beta-thalassemia major who underwent splenectomy with lifelong transfusion-dependence complicated with myocardial siderosis and osteoporosis. The close monitoring and regular blood transfusion are a core of successful support to this type of pregnancy. The unintentional consumption of Fosamax, hydroxyurea and deferiprone (Ferriprox) up till 20 weeks of gestation did not show any adverse effects on fetal well-being. As expected, this pregnancy ended with the preterm delivery via cesarean section due to intrauterine growth restriction with oligohydramnios, and currently, this child is thriving. We concluded that pregnancy is not a contraindication in beta-thalassemia major; complex individual care is needed to achieve a safe outcome for the mother.