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.

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.

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.

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.

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.

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 of the Central Nervous System Due to Recurrent Surgeries of the Thoracic Spine: A Rare Case.

BACKGROUND: Superficial siderosis (SS) of the central nervous system is a rare condition caused by hemosiderin deposition in the subpial layers of the brain and spinal cord. Surgical complications are the primary factor for occurrence of secondary SS. We present a case of SS with an identified bleeding origin in the thoracic spine. CASE DESCRIPTION: A 58-year-old female patient experienced 9 months of continuous progressive dizziness, difficulty with mobilization, drop attacks, and lack of hearing. The patient also had an extensive history of thoracic spinal surgeries. She came to the hospital with gait imbalance. Gradient echo (GE) magnetic resonance imaging (MRI) confirmed hemosiderin deposition along the cerebellar folia and vermis. GE sequences are preferable in this diagnosis, because of higher sensitivity, and for detecting characteristic T2 hypointensity. The dural defect was repaired with an artificial dural patch in thoracal operation area. Clinical findings, imaging studies, intraoperative findings, and literature information are presented. CONCLUSIONS: Performance of an open neurosurgical procedure to repair a dural defect in the presence of MRI confirmed that superficial siderosis is an optimal method and a crucial step to ensure the safe resolution of the condition and to break the circle of emergency admissions of a patient with a dural defect and a history of multiple spinal surgeries.

The evolution of cerebrovascular changes in Köhlmeier-Degos disease: An 11-year follow-up case report.

Köhlmeier-Degos disease is rare idiopathic vasculopathy, the exact pathogenesis of which remains unclear. Here, we review pertinent literatutre and present a case of a Köhlmeier-Degos disease with central nervous system involvement followed-up over 11 years with various neuroimaging modalities. Evolution of neurovascular and neuropathological changes over an extended time period has not been previously described.

[Intraocular ferritin production due to an iron-containing intraocular foreign body]. / Synthèse intraoculaire de ferritine suite à un corps étranger intraoculaire ferrique.

INTRODUCTION: Ocular siderosis is a rare but severe complication of open globe trauma with intraocular retention of a metallic foreign body. CASE STUDY: We report a case of recurrent uveitis in a 37-year-old patient. The ophthalmic examination revealed poor vision in the left eye, lid edema, limbal scleromalacia, hyphema and severe ocular hypertension. Orbital CT showed the presence of a radio-opaque IOFB between the crystalline lens and vitreous body. An aqueous humor sample was obtained for iron and ferritin levels. The results came back 100 and 2000 times higher, respectively, than the serum reference values. DISCUSSION: The very high iron content is the result of a sustained release from the metallic INFB and is responsible for ocular siderosis in our patient. The extremely high ferritin level would be the result of in situ synthesis by the various cells of the ocular structures in order to preserve the components of the eye. Measurement of these two levels would improve the diagnosis, prognosis and treatment of metallic IOFBs.

An autopsy case of superficial siderosis of the central nervous system accompanied by anterior sacral polycystic meningocele in neurofibromatosis type 1.

A 74-year-old female patient, who was diagnosed with neurofibromatosis type 1 (NF1) at the age of 40, was admitted with complaints of flickering vision and gait disturbance for the last 2 years. On admission, neurological examination revealed mild bilateral hearing loss and ataxia in the limb and trunk. Laboratory tests revealed anti-hepatitis C virus (HCV) antibody positivity and elevated HCV RNA by real-time polymerase chain reaction. The cerebrospinal fluid examination revealed a slightly yellowish appearance with elevated total protein levels. Gradient echo T2*-weighted brain magnetic resonance imaging (MRI) demonstrated a rim of hypointense lesions surrounding the surface of the cerebellum, brainstem, frontal and temporal lobes, and thalamus, which was considered as hemosiderin depositions. From these MRI findings, she was diagnosed as having superficial siderosis of the central nervous system. Cerebral angiography revealed an aneurysm-like dilatation at the bifurcation of the right internal carotid-posterior communicating artery. (99m)Tc-ethyl cysteinate dimer single-photon emission computed tomography revealed hypoperfusion in the bilateral frontal and temporal lobes. Pelvic plain X-ray, pelvic computed tomography, and lumbosacral MRI revealed a sacral defect and an anterior sacral polycystic meningocele communicating with the spinal subarachnoid space. The patient's symptoms gradually worsened, and she died of septic shock because of pyelonephritis at the age of 77. An autopsy was performed; on pathological examination, we did not observe any findings associated with rupture of the aneurysm-like dilatation in the bifurcation of the right internal carotid-posterior communicating artery and cerebral amyloid angiopathy. Because duropathies-a new neurological disease concept-have been implicated as a cause of bleeding in the superficial siderosis, the anterior sacral polycystic meningocele, a type of duropathies, was presumed to be the most probable bleeding source of the superficial siderosis in this patient. Bleeding from the meningocele might result from the vulnerability of vessel walls in NF1.