Dysthyroid optic neuropathy (DON)

Dysthyroid Optic Neuropathy (DON) affects a small percentage of patients with Graves disease, but, when it occurs, it can cause significant and permanent loss of vision. DON is treatable if recognized early. Systemic steroids can be effective, but may cause side affects. Orbital injection of steroids may play a role in selected patients. Orbital radiation has a more permanent effect and has gained wide acceptance as a relatively non-invasive method of reversing DON. Surgery to decompress crowded orbits has been used for years and continues to be a viable approach for those patients with optic neuropathy, especially when there is significant proptosis. Optic nerve decompression can also be achieved through a transethmoidal approach.     

Clinical features of dysthyroid optic neuropathy: a European Group on Graves' Orbitopathy (EUGOGO) survey

BACKGROUND: This study was performed to determine clinical features of dysthyroid optic neuropathy (DON) across Europe. METHODS: Forty seven patients with DON presented to seven European centres during one year. Local protocols for thyroid status, ophthalmic examination and further investigation were used. Each eye was classified as having definite, equivocal, or no DON. RESULTS: Graves' hyperthyroidism occurred in the majority; 20% had received radioiodine. Of 94 eyes, 55 had definite and 17 equivocal DON. Median Clinical Activity Score was 4/7 but 25% scored 3 or less, indicating severe inflammation was not essential. Best corrected visual acuity was 6/9 (Snellen) or worse in 75% of DON eyes. Colour vision was reduced in 33 eyes, of which all but one had DON. Half of the DON eyes had normal optic disc appearance. In DON eyes proptosis was > 21 mm (significant) in 66% and visual fields abnormal in 71%. Orbital imaging showed apical muscle crowding in 88% of DON patients. Optic nerve stretch and fat prolapse were infrequently reported. CONCLUSION: Patients with DON may not have severe proptosis and orbital inflammation. Optic disc swelling, impaired colour vision and radiological evidence of apical optic nerve compression are the most useful clinical features in this series.     

甲状腺功能障碍性视神经病变的影像学研究进展

甲状腺功能障碍性视神经病变（ DON）是甲状腺相关眼病（ TAO）引起视神经功能损害的严重并发症,主要由眶内肿胀的眼外肌压迫眶尖视神经所致。早期诊治对疾病的疗效和预后至关重要。眼眶影像学检查在DON的诊断和评估上发挥着重要的作用,可以显示眼眶组织解剖结构,评估疾病分期及疗效,还能帮助识别倾向于发展为DON的患者。本文回顾了DON的病因、临床特征和影像学,着重对DON的影像学研究进行综述,包括CT、MRI和多普勒超声研究。     

Chronic orbital inflammatory disease and optic neuropathy associated with long-term intranasal cocaine abuse: 2 cases and literature review

Orbital inflammatory disease and secondary optic neuropathy is a rare but devastating complication of long-term intranasal cocaine abuse. We describe 2 patients with a history of intranasal cocaine consumption who presented with subacute onset of unilateral vision loss from optic neuropathy and limitation of abduction in the affected eye. Magnetic resonance imaging findings included an orbital mass in combination with absent nasal septum and partial destruction of the paranasal sinuses. Biopsies and histopathologic examination of the nasal cavity and the orbital mass revealed chronic inflammation. Both patients were treated with oral corticosteroids, ocular movements completely normalized but no improvement of visual acuity was noted. Intranasal cocaine abuse can cause orbital complications from chronic sinonasal inflammatory disease and these patients are at risk to develop optic neuropathy. Optic neuropathy may be caused by compression, infiltration, or ischaemia.     

Two cases of drug-induced orbital inflammatory disease

Orbital inflammatory disease (OID) includes all inflammatory processes affecting the orbit. Although several aetiologies are recognised, a cause may not be elucidated. We describe 2 cases in which drugs (hyaluronidase and zoledronic acid) were the cause of OID. In patients with a clinical picture of OID simulating an orbital cellulitis, the recent drug history should be considered as a possible aetiology, and treatment with steroids with or without a biopsy should be considered after an infection has been excluded.     

Clinical manifestations and treatment outcome of optic neuropathy in thyroid-related orbitopathy.

BACKGROUND AND OBJECTIVE: To investigate the incidence and outcome of optic neuropathy associated with thyroid-related orbitopathy. PATIENTS AND METHODS: All patients diagnosed as having optic neuropathy associated with thyroid-related orbitopathy who underwent treatment for optic neuropathy between January 1, 1999, and March 1, 2003, were reviewed. Demographic and clinical data were extracted from the oculoplastic registry (electronic medical record). RESULTS: Optic neuropathy occurred in 20 of 595 patients with thyroid-related orbitopathy (3.4%). All patients received systemic steroids, 7 patients received orbital steroid injections, and 2 patients were treated with orbital radiotherapy. Orbital decompression was performed in all 26 orbits. Visual acuity improved from a mean of 20/150 to 20/30 (P < .001). Color vision improved from a mean of 5.2/14 to 11/14 (P = .001). The afferent pupillary defect disappeared shortly after treatment for all but 1 case, and improved in all cases. Exophthalmos decreased from 26.4 +/- 2.5 to 21.5 +/- 2.1 mm (P< .001; 95% confidence interval, 3.8 to 5.7). CONCLUSIONS: Optic neuropathy manifests rarely in patients with thyroid-related orbitopathy. Monitoring visual acuity or afferent pupillary response may be a reliable way of assessing and monitoring optic neuropathy. In this study, patients had improved visual acuity and optic nerve function after a combination of medical and surgical treatment.     

Diagnosis and management of orbital pseudotumor

Orbital pseudotumor, also known as idiopathic orbital inflammatory syndrome (IOIS), may have protean clinical manifestations. Some presentations of IOIS may mimic common conditions such as orbital cellulitis and optic neuritis. IOIS should be considered a diagnosis of exclusion, with evaluation directed toward eliminating other causes of orbital disease. Orbital magnetic resonance imaging is the single most important diagnostic test, but serologic studies are necessary to exclude a systemic cause. Biopsy is usually not performed at presentation, as the risk of producing damage to vital structures within the orbit outweighs the benefits. Patients with multiple recurrences, or those unresponsive to therapy, should have biopsy samples taken. Corticosteroids are the mainstay of therapy and are administered for several months to ensure remission. Radiotherapy may be used in patients who fail to respond to steroids or who have a rapidly progressive course. For those patients who are refractory to both corticosteroids and radiotherapy, anecdotal reports have suggested the use of chemotherapeutic agents such as cyclophosphamide, methotrexate, and cyclosporine.     

Unusual cause of bilateral optic disc swelling: POEMS syndrome

BACKGROUND: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) is a rare disorder associated with plasma cell disorder. Optic disc swelling can be present, and macular oedema has been reported once before. PATIENTS AND METHODS: Three patients with POEMS syndrome were investigated. Lumbar puncture and neuro-ophthalmic examination were performed on all patients. RESULTS: Visual symptoms included decreased vision, photopsia and blurred vision. Optic disc swelling was present in all three patients. Visual acuity was decreased in only one patient (0.2 in right eye, 0.7 in left eye) due to macular oedema, and another patient with normal visual acuity showed also a mild macular oedema on fluorescein angiography. In these two patients, elevated intracranial pressure was found (30 and 39 cm H(2)O, respectively). The macular oedema disappeared under steroids and acetazolamide therapy with visual improvement, but optic disc swelling remained. CONCLUSION: Optic disc swelling is frequent in POEMS and macular oedema may be more frequently present than previously thought. Interestingly macular oedema was detected in the two patients with increased intracranial pressure.     

Retrobulbar pressures measured during surgical decompression of the orbit.

AIMS/BACKGROUND: In Graves' ophthalmopathy the increase in volume of intraocular muscles and fat will cause elevated intraorbital pressure. In order to investigate the pressure levels involved, intraorbital pressure, or retrobulbar pressure (RBP) was measured continuously in orbits of patients with Graves' ophthalmopathy during surgical decompression. METHODS: Retrobulbar pressure was measured before and during surgical decompression using an intraorbitally applied pressure transducer. RESULTS: In eight patients with dysthyroid optic neuropathy (DON) RBPs between 17 and 40 mm Hg were recorded. At the end of the surgical procedure the mean RBP was reduced from 28.7 mm Hg to 18.7 mm Hg, the decrease ranging from 8 to 12 mm Hg, which showed a high correlation with the starting pressures (p < 0.001). In two cases without DON, pressures were 11 and 9 mm Hg. Forces exerted by spatula manipulation usually resulted in a RBP level of more than 70 mm Hg. CONCLUSIONS: This study shows that RBPs are markedly elevated in Graves' ophthalmopathy and that surgical decompression can result in a significant reduction in the intraorbital pressure. Optic nerve dysfunction in Graves' ophthalmopathy may not be caused exclusively by the direct pressure of swollen extraocular muscles upon the optic nerve, but also by a raised RBP. It is hypothesised that the damage inflicted upon the optic nerve can be caused in consequence by RBP induced incarceration of the nerve, compressed by surrounding periosteal lined orbital fat bulging posteriorly into the entrance of the optic canal.     

Clinical profile and visual outcomes after treatment in patients with dysthyroid optic neuropathy

Purpose

To report the clinical data and visual outcomes after treatment of patients with dysthyroid optic neuropathy (DON).

Methods

We retrospectively reviewed the medical records and orbital images of 40 patients (65 eyes) with DON and analyzed the visual outcomes after treatment with intravenous steroids pulse therapy, radiotherapy and orbital decompression.

Results

The study included 21 men and 19 women, with 10 (25%) being diabetic patients. Visual field test results revealed defects in 88.7% of DON eyes; afferent pupillary defects in 63.2%; reduced color vision in 78.5%; and abnormal visual evoked potentials in 84%. Orbital imaging showed moderate to severe apical crowding in 95% of the orbits and intracranial fat prolapse in 24.2%. Median best corrected visual acuity improved from 0.4 to 1.0 after one year of treatment (p < 0.001). We noted more improvement in vision with the use of decompressive surgery than with non-surgical methods (p < 0.05). Recurrences occurred in 7 patients who had not received orbital radiotherapy.

Conclusions

Visual field defects and apical crowding seen on orbital imaging were the most sensitive indicators for the detection of DON. Treatment with intravenous steroids pulse therapy, radiotherapy and orbital decompression effectively improved visual outcomes in cases of DON.     

Klinische und neuroradiologische Diagnostik bei Orbitatumoren

Exophthalmus is the leading sign of space-occupying lesions of the orbit. Patients may further present with lid swelling, impaired ocular motility and optic neuropathy including a relative afferent pupillary defect, compressive optic disc edema or optic atrophy. Orbital tumors can be classified into various categories depending on the etiology, as lymphoproliferative lesions (in particular non-Hodgkin's lymphoma as the most common malignant orbital tumor of adulthood), optic nerve and meningeal lesions, lacrimal gland lesions, secondary orbital tumors which extend to the orbit from neighboring structures and metastases. Slightly less common are vasculogenic and cystic lesions including cavernous hemangioma as the most common benign orbital tumor of adulthood and dermoid cysts as the most common benign orbital tumor of childhood. Rhabdomyosarcoma is the most common malignant orbital tumor of childhood but has a low total incidence. Orbital tumors might not only cause symptoms like pain, diplopia and loss of visual acuity but may also lead to esthetically disfiguring changes. Particular attention should be paid to underlying systemic diseases and generalized tumor diseases. This article illustrates the approach to a detailed clinical and neuroradiological assessment which is mandatory for the care of orbital tumor patients.     

8. Orbital Cellulitis

Orbital cellulitis secondary to sinusitis has been the most common cause of proptosis in children. A case report is presented which emphasizes that the signs and laboratory findings in orbital Cellulitis may be confusing, especially when modified by prior antibiotics. When orbital cellulitis is a possibility, adequate antibiotic therapy should be instituted while diagnostic studies are being performed. CT scanning is a new and useful modality for the evaluation of these patients, but cautious and experienced interpretation is essential. In patients who do not respond promptly to appropriate medical therapy, surgical intervention is indicated for drainage and biopsy.     

Incidence of postblepharoplasty orbital hemorrhage and associated visual loss

PURPOSE: Orbital hemorrhage, especially when associated with visual loss, is a significant complication of cosmetic eyelid surgery. We investigated the incidence of this complication among cases handled by members of the American Society of Ophthalmic Plastic and Reconstructive Surgery. We also reviewed the length of time between surgery and the onset of bleeding and sought any factors that may contribute to these cases. METHODS: A 2-page questionnaire was sent to all American Society of Ophthalmic Plastic and Reconstructive Surgery members. Responses were tabulated as to total numbers of cases performed, the incidence of postoperative orbital hemorrhage, treatments, and incidence of permanent visual loss. Comorbidities were also reviewed. RESULTS: Two hundred thirty-seven responses were received from American Society of Ophthalmic Plastic and Reconstructive Surgery members, collectively representing 269,433 cases. Among these cases, there were 149 orbital hemorrhages, 48 cases associated with temporary visual loss, and 12 cases associated with permanent visual loss. The majority of these cases occurred within the first 3 hours of surgery, and the risk decreased significantly after 24 hours after surgery. Common comorbidities were hypertension, perioperative aspirin use, postoperative vomiting, and increased physical activity. Treatment commonly consisted of wound drainage with cauterization of active bleeders, lateral canthotomy, intravenous steroids, and intraocular pressure-lowering drugs. Orbital decompression was infrequently used; anterior chamber paracentesis was not used. This study is limited by recall biases regarding the number of cases performed by each responding surgeon and the number of relevant cases of orbital hemorrhage. CONCLUSIONS: The incidence of orbital hemorrhage associated with cosmetic eyelid surgery is 0.055% (1:2,000), and orbital hemorrhage with permanent visual loss is 0.0045% (1:22,000) [corrected] Development of orbital hemorrhage is most common within the first 24 hours after surgery, especially within the first 0 to 3 hours, but can occur as late as several days after surgery.     

Small Versus Coronal Incision Orbital Decompression In Graves’ Orbitopathy

Ideally the planning of decompression surgery should be adequate to the severity of the orbitopathy, its possible “lipogenic” or “myopathic” variants, the patient’s specific orbital osteology and possible previous surgeries. Due to surgeon’s experience and local traditions, however, a standardized rather than a tailored approach is often offered to the patient. An inferior fornix incision can be used for infero medial bony decompression and/or for removing fat from the medial and lateral inferior orbital quadrants. Through the same route a lateral osteotomy can also be performed although an upper skin crease incision offers a wider access to the lateral orbital wall. As an alternative the swinging eyelid technique, offering an adequate access to the bony orbit and to the orbital fat compartments is a versatile technique that can virtually be used as a standard approach for the greatest majority of patients needing decompression surgery. Orbital decompression by coronal incision is an invasive technique and for this not to be used as a standard approach to orbital decompression. Nevertheless, it is not to be abandoned as it can be an additional tool in surgeons’ hands when dealing with patients who can better benefit out of a particular, tailored rather than a standardised approach. Many are the circumstances in which this may happen. Major complications associated with the coronal approach have been mainly described in small series, where only a few patients per year were operated. In this respect it is therefore unavoidable to emphasize that each technique has its own learning curve and it may be difficult to differentiate the effects of each technique from the experience of the surgeon.     

Optic Neuritis: An Update. II. Optic Neuritis and Multiple Sclerosis

Demyelinating optic neuritis (DON) is an acute inflammatory demyelinating disorder affecting the optic nerve. The risk of having a subsequent clinical episode elsewhere in the central nervous system leading to a diagnosis of clinically definite multiple sclerosis (CDMS) varies in different parts of the world. In countries with a high prevalence of MS, most patients with DON will eventually develop CDMS. The presence of oligoclonal bands in cerebrospinal fluid and asymptomatic lesions on magnetic resonance imaging (MRI) of the brain at presentation are strong predictors of progression to CDMS. The Optic Neuritis Treatment Trial reported that DON patients treated with intravenous methylprednisolone had a delay in progression to CDMS, but only up to two years. Interferon-β and glatiramer actetate have now been shown to reduce the risk of developing CDMS after two to three years when compared with placebo. The BENEFIT study has demonstrated that delaying interferon-β1b therapy in patients presenting with a clinically isolated syndrome (CIS) such as optic neuritis leads to a slightly higher risk for sustained disability progression compared with patients who started interferon-β1b after a CIS. Controversy remains if these small benefits of reducing conversion to CDMS from CIS and slowing disability with interferon-β treatment outweigh the adverse effects of the medication and its costs as the overall disability progression off treatment is mild, at least in the short-term, and particularly in patients in whom optic neuritis is the initial event.     

Optic Neuritis: An Update Typical and Atypical Optic Neuritis

Optic neuritis (ON) is an acute inflammatory disorder affecting the optic nerve. In those parts of the world where Multiple Sclerosis (MS) is common most cases will have typical demyelinating ON (DON) which will recover spontaneously, but the patients will be at risk of going on to be diagnosed with clinically definite MS after a second episode in another part of the central nervous system. Other patients (perhaps the majority where MS is rare) will have “atypical” ON, not associated with MS, and will require corticosteroids to induce recovery and often continued immunosuppression to maintain remission. This paper discusses recent advances in knowledge concerning the presentation and treatment of both typical DON and atypical ON, including algorithms to help identify the variety of ON a patient is presenting with and how to treat them acutely.     

Optic nerve glioma: an update

Optic nerve glioma is the most common optic nerve tumour. However, it has an unpredictable natural history. The treatment of optic nerve gliomas has changed considerably over the past few years. Chemotherapy and radiation therapy can now stabilize and in some cases improve the vision of patients with optic nerve gliomas. The treatment of optic nerve glioma requires a multi-disciplinary approach where all treatment options may have to be implemented in a highly individualized manner. The aim of this review article is to present current diagnostic and treatment protocols for optic nerve glioma.     

Juvenile uveitis

Chronic anterior uveitis in children suffering from juvenile rheumatoid arthritis may develop very early, when the patient is as young as two or three years old; it affects twice as many girls as boys. The arthritis is often localized in one joint (pauciarticular or oligoarticular form); Still's syndrome is very rare. Local treatment with cycloplegics and steroids has to be combined with systemic immunosuppressive medication with cyclophosphamide. Chronic intermediate uveitis (pars planitis) usually affects teenagers in good health. Peripheral fundus changes can be detected most easily by biomicroscopy with the three-mirror contact lens. Cystoid macular edema is frequent and, together with vitreous opacities, is responsible for a deterioration in central vision. In most cases local treatment with subconjunctival injection of depot steroids is sufficient. Immunosuppressive treatment or surgical intervention is only indicated in severe cases where there is a risk of complete loss of vision.     

Hemangioblastoma of the optic nerve

Optic nerve hemangioblastoma is a rare tumor that is usually unilateral and most commonly occurs in the context of von Hippel-Lindau disease. Differential diagnosis is based on clinical history and imaging. Magnetic resonance imaging with gadolinium enhancement is the most useful imaging modality as it can reveal flow voids and an absence of dural attachment, differentiating optic nerve hemangioblastoma from other more commonly encountered optic nerve tumors. Optic nerve hemangioblastoma are usually well-circumscribed vascular lesions composed of stromal cells and vascular endothelial cells. These lesions are diagnosed at a mean age of 37 years and can be asymptomatic, but over time, patients may develop reduction in vision, proptosis, and pain. Surgical excision is well described via orbital, transsphenoidal, or transcranial approaches. Given the risks associated with surgery, a stepwise conservative approach is advocated by most clinicians in the absence of severe symptoms. Although uncommon, this optic nerve tumor should be considered in young patients presenting with pain, proptosis, and optic nerve pallor, with or without a history of von Hippel-Lindau disease.     

Pseudotumor and lymphoid tumor: distinct clinicopathologic entities

Although in the past some have considered lymphoid tumor to be a form of pseudotumor, clinical, radiologic and pathologic features show them to be distinct entities. Acute pseudotumor presents with abrupt, painful onset, and CT scan shows a diffuse process with no distinct mass to biopsy. Chronic or recurrent pseudotumor leads to a mass of fibrosis apparent on CT scan, which should be biopsied. Lymphoid tumor presents without pain, and orbital CT scan shows a homogenous mass that should be biopsied. Acute pseudotumor usually responds to steroids, but patients with chronic or recurrent pseudotumor may require low-dose radiation or immunosuppressive therapy. Lymphoid tumors that are classified as reactive lymphoid hyperplasia initially may be treated with steroids, followed by low-dose radiation if steroids are ineffective. Orbital lymphomas are treated with higher dose radiation and, perhaps, chemotherapy, if systemic disease is present. Reports of representative cases of acute and chronic pseudotumor and lymphoid tumor illustrate the distinguishing clinical, radiologic and pathologic characteristics of each type of tumor, and recommendations for management of each type are outlined.