Dysthyroid Optic Neuropathy Research Articles

Complete recovery of visual acuity as the main goal of treatment in patients with dysthyroid optic neuropathy.

To evaluate the effectiveness of methylprednisolone (MP) and surgical treatment in achieving complete reversal of dysthyroid optic neuropathy (DON) and predictive factors of this therapy. The group consisted of 10 patients (18 eyes) with DON. The diagnosis of DON was based on at least two criteria from the following: (i) deterioration of visual acuity (VA< 1.0), (ii) loss of colour vision, (iii) optic disc swelling, and/or (iv) signs of DON in magnetic resonance imaging (presence of apical crowding and/or optic nerve stretching). A complete recovery of DON was defined as the normalisation of VA (VA = 1.0), normal colour vision, and reversal of optic disc swelling. A significant improvement was defined as improvement of VA of at least 0.2. The consecutive steps of treatment of DON consisted of: (i) first-line treatment - intravenous MP pulse therapy (3 × 1 g); (ii) second-line treatment - endoscopic intranasal orbital decompression of medial wall; (iii) additional treatment - additional MP therapy and/or surgical decompression. A significant improvement in VA could be achieved in the majority of patients; a complete recovery was noted in 22.2%, 33.3%, and 66.7% of eyes after first-line, second-line, and additional treatment, respectively. Positive predictive factors were: younger age (p = 0.049), shorter duration of DON (p = 0.035), and a higher Graves' orbitopathy clinical activity score (p = 0.035). By using combination therapy (intravenous MP pulse therapy and surgical decompression), a complete recovery can be achieved in the majority of patients with DON.

Risk Factors for Graves' Orbitopathy; the Australian Thyroid-Associated Orbitopathy Research (ATOR) Study.

Previous association studies suggest the development of Graves' orbitopathy (GO) is variably influenced by environmental risk factors. To determine the risk factors and predict odds for developing GO in Graves' hyperthyroidism (GH). Case-control study. Multi-centre Australian Thyroid-associated Orbitopathy Research group consisting of tertiary endocrinology and ophthalmology outpatients and related private practices. A total of 1042 participants with GH were designated as cases if they had GO (n = 604) and controls if they did not have GO (n = 438). Primary outcome was GO risk factors and secondary outcome was dysthyroid optic neuropathy (DON) with the effects of risk factors measured by odds ratio (OR) using multiple logistic regression, adjusted for known risk factors and exploratory variables. The odds of GO increased by 17% for each decade increase in the age of onset of GH (OR 1.17, confidence interval (CI): 1.06-1.29; P = .002) and by 7% for each year increase in the duration of GH (OR 1.07, CI: 1.05-1.10; P < .001). Smoking increased the odds for GO by 2.22 for current smoker and 2.07 for exsmoker (P < .001), compared with never smoking. The odds of GO are 86% less in Graves' patients using antithyroid medication than those not (OR 0.14, CI: 0.06-0.34; P < .001). Predictors for DON were older age, oculomotility restriction, strabismus, reduced palpebral aperture, and active GO. This study identified increase age of onset, duration of GH, and smoking as risk factors for GO. Usage of antithyroid medication was negatively related to GO. Older patients with restricted ocular motility, strabismus, and active GO are at higher risk of DON and may benefit from early medical intervention.

An update on thyroid-associated ophthalmopathy in children and adolescents.

Risk of developing thyroid-associated ophthalmopathy (TAO) in children and adolescents is similar or may be even slightly higher than in adults. The aim of this article is to review and summarize current knowledge regarding diagnostic and therapeutic measures in pediatric TAO. MEDLINE and EMBASE papers were searched using the terms 'pediatric Graves' ophthalmopathy' 'pediatric Graves' orbitopathy', 'thyroid-associated ophthalmopathy in childhood and adolescence' from the year 1970 to December 2015. TAO usually accompanies hyperthyreosis in Graves' disease, but may also occur in patients with hypothyreosis due to chronic lymphocytic thyroiditis (Hashimoto's disease) or in euthyroid patients. Current information regarding epidemiology, pathogenesis, symptoms and treatment of TAO in children and adolescents is presented. The course of the disease is usually mild, eye disturbances often regress after restoring euthyroidism and a 'wait and see' policy is appropriate in the majority of patients. In rare cases, sight-threatening complications [dysthyroid optic neuropathy (DON) or corneal breakdown] may develop and immediate surgical intervention might become necessary. Close cooperation between pediatric endocrinologists and opthalmologists is extremely important to ensure best care and quality of life in patients with thyroid gland dysfunction. Further investigations on pathogenesis and course of TAO in children and adolescents should be performed for better management of this disease in this group of patients.

Clinical and immunological changes in patients with active moderate-to-severe Graves' orbitopathy treated with very low-dose rituximab.

Glucocorticoids represent the therapy of choice for active and moderate-to-severe Graves' orbitopathy (GO). In some patients, rituximab, a monoclonal antibody against the cluster of differentiation (CD) 20 receptor of B-lymphocytes, can serve as a second-line or an alternative treatment. The effect of very low-dose of rituximab on the clinical activity of GO and corresponding clinical or laboratory changes is reported. Changes of Clinical Activity Score (CAS) for GO, proptosis, levels of thyroid-stimulating hormone receptor antibodies, and depletion of CD19+ and CD20+ B-lymphocytes were determined in ten patients (two men and eight women) with active moderate-to-severe GO treated with a single 100-mg dose of rituximab. Correlations between differences of clinical and laboratory parameters were performed. A significant decrease of CAS was found during subsequent examinations compared to the baseline values. A significant depletion of CD19+ and CD20+ B-lymphocytes was detected after rituximab administration. Differences between follow-up and baseline levels of CD20+ positively correlated with differences in CAS after six (p < 0.05) and 12 months (p < 0.01). Differences in CD19+ levels correlated with differences in CAS after 12 months (p < 0.05) of the treatment. Two patients developed dysthyroid optic neuropathy (DON) requiring orbital decompression. No other rituximab side effects were reported during the whole study duration. A single very low-dose of rituximab appears to be very well tolerated and effective enough to reduce clinical activity in active moderate-to-severe GO patients without impending DON.

Outcomes following orbital decompression surgery for dysthyroid optic neuropathy associated with Graves’ ophthalmopathy.

Purpose: To investigate the outcomes of orbital decompression surgery for dysthyroid optic neuropathy associated with severe Graves’ ophthalmopathy. Design: Ten years (2000-2010) retrospective case series.Methods: Thirty-eight orbits (with dysthyroid optic neuropathy) of 119 surgical orbital decompressions. Patients with dysthyroid optic neuropathy associated with Graves’ ophthalmopathy, who underwent orbital decompression surgery at Sydney Eye Hospital (Sydney, Australia), were investigated for outcome measures.Results: Thirty-five orbits were eligible for data analysis. Orbital decompression surgery improved visual acuity in 29 orbits and maintained visual acuity in four orbits. In patients with dysthyroid optic neuropathy, there was a statistically significant mean improvement in visual acuity of 2.8 lines postoperatively (standard deviation = 3.2; 95% confidence interval 3.9 to 1.7, p-value &lt; 0.05). There were no statistically significant differences invisual acuity amongst different combinations of orbital walls being decompressed, with the majority of orbits had the medial orbital wall decompressed. This may reflect the small number of decompressions performed in each subgroup. Orbital decompression surgery reduced proptosis by a mean of 3.2 mm (standard deviation = 2.9; 95% confidence interval -4.32 to -2.07; p-value &lt; 0.05). Medial and lateral orbital walls decompression resulted in the greatest mean reduction in proptosis. There were no severe visual impairment cases postoperatively (VA worse than 6/60). There were two patients with new onset diplopia postoperatively. There were three orbits with bleeding and one orbit with CSF leakage, all without major sequelae postoperatively.Conclusion: Regardless of surgical access, orbital decompression surgery is effective and safe in the management of dysthyroid optic neuropathy and in reducing proptosis in patients with Graves’ ophthalmopathy.

Graves’ orbitopathy in pediatrics

Graves’ orbitopathy (GO) is rare in pediatric patients, however is the most common extrathyroid manifestation of Graves’ disease (GD), being present in 30-67% of patients. GO is an autoimmune inflammatory disorder involving orbital connective and fatty tissues as well as the extraocular muscles. In children, GO is less common and less severe than in adults. The most common symptoms are upper eyelid retraction, conjunctival injection, and proptosis and periorbital edema. Severe complications include dysthyroid optic neuropathy, corneal ulceration and eyeball subluxation. The diagnosis is established by clinical, laboratory and imaging findings. There are no management guidelines for GO in children but adult recommendations include the assessment of clinical activity and its severity, to implement the best treatment. Supportive therapies are intended to relieve symptoms and prevent corneal damage in mild cases. Tobacco exposure should also be avoided. The first line of treatment is systemic administration of corticosteroids in active and severe cases. Other options as somatostatin use and retrobulbar radiation have not been used in children, therefore their use is not recommended.

Epidemiology of Oculoplastic and Reconstructive Surgeries Performed by a Single Specialist With 15 Years' Experience at a Tertiary Center.

To evaluate the prevalence of oculoplastic surgeries performed in a tertiary hospital in South Korea and to highlight the proper choice of surgery according to disease. Retrospective chart review was conducted over a 12-month period (March 2013 to February 2014) on all of the patients who underwent oculoplastic and reconstructive surgery by a single oculoplastic specialist with 15 years' experience. A total of 656 patients were enrolled in the current study. Of them, 355 patients (54.1%) had eyelid surgery, 151 patients (23.0%) had orbital surgery, and 150 patients (22.8%) had lacrimal surgery. Orbital surgery was more commonly performed in men (122 male patients versus 29 female patients, P = 0.00 by Pearson chi-squared test) and was also more common in younger patients than in other surgery groups (56.3 years in eyelid surgery versus 39.3 years in orbital surgery versus 46.9 years in lacrimal surgery, P = 0.001 by Pearson chi-squared test). Blepharoplasty was the most common eyelid surgery, contributing 24.2% of total eyelid surgeries, followed by lid mass excision (68 patients, 19.1%), Hotz operation (59 patients, 16.6%), and levator advancement (47 patients, 13.2%). Among orbital surgeries, reconstruction of orbital wall fractures was the most common, and was performed in 89 patients (58.9%) of total orbital surgeries, followed by orbital tumor debulking surgery (25 patients, 16.5%) and orbital decompression for dysthyroid optic neuropathy (19 patients, 12.5%). Endoscopic endonasal dacryocystorhinostomy (DCR) was the most common lacrimal surgery (84 patients), occurring in 56% of total lacrimal surgeries, followed by Monoka tube intubation (16 patients, 10.6%) for congenital nasolacrimal duct obstruction and conjunctivo-DCR using Jones tube (12 patients, 8%). Eyelid-related conditions, especially cosmetic blepharoplasty, were among the most commonly encountered surgeries in the Oculoplastics Department of a tertiary care hospital in South Korea. Blowout fracture repair and endoscopic DCR were the most common in orbital and lacrimal surgery. The authors hope that the current survey will contribute to the surgical training of ophthalmology residents and, on a larger scale, health care policies.

Endoscopical Orbital Fat Decompression with Medial Orbital Wall Decompression for Dysthyroid Optic Neuropathy

ABSTRACTObjective: To describe a novel approach to orbital decompression for dysthyroid optic neuropathy (DON).Methods: An augmented endoscopic transethmoid medial orbital wall decompression (ETMOWD) was performed on 43 consecutive patients (72 eyes) with DON in order to access the orbital apex adequately. Concurrently, endoscopic transethmoid fat decompression (ETFD) using a novel aspiration/cutting instrument to remove orbital fat was performed for further reduction of proptosis. All patients were followed up periodically for at least 6 months. Outcomes such as improvement of visual acuity (VA), color vision, degree of proptosis reduction as well as the incidence of surgical induced diplopia were analyzed at the final review.Results: Sixty-nine out of 72 eyes (95.8%) had a statistically significant improvement in VA from −0.65 ± 0.30 to −0.25 ± 0.22 postoperatively, with a mean improvement of 0.55 ± 0.17 (p < 0.001). Thirty-four out of 45 eyes had an improvement in color vision (p < 0.001). The range of proptosis reduction was 4 to 9 mm (mean 6.2 ± 1.2 mm). Postoperative symmetry was achieved to within 2 mm using an exophthalmometer in 39 of 43 patients (90.7%). Five patients developed diplopia in the postoperative phase, but had complete resolution within 3 months. Two patients had further deterioration in their diplopia following surgery.Conclusion: Our technique of a combined ETFD with ETMOWD appears to be effective in managing patients with DON, with minimal morbidities and a low incidence of postoperative diplopia.

Surgical Treatment of Dysthyroid Optic Neuropathy: Long-Term Visual Outcomes with Comparison of 2-Wall versus 3-Wall Orbital Decompression

ABSTRACTPurpose: To compare the long-term visual outcomes after 2-wall (medial-lateral) versus 3-wall (medial-lateral-inferior) orbital decompression combined with fat removal in patients with dysthyroid optic neuropathy (DON).Methods: Records of 68 eyes of 42 patients were retrospectively reviewed. Two-and 3-wall decompression was performed in 41 and 27 eyes, respectively. Transcaruncular approach was used for medial wall decompression. Lateral canthotomy combined with upper eyelid crease incision was used for lateral wall removal, and combined with transconjunctival lower eyelid incision if floor decompression performed. Outcome measures were visual acuity (VA), color vision, Hertel measures, visual field mean deviation (MD) and pattern standard deviation (PSD)Result: The mean follow-up time was 39.3 months (range, 12–72 months). All indicators of visual function significantly improved after 2-and 3-wall decompression. The improvement in VA and color vision was similar between groups. (logMAR VA: 2-wall: 0.52 ± 0.68 versus 3-wall: 0.71 ± 0.86, p = 0.335); (color vision on Ishihara plates: 2-wall: 10.1 ± 8.1 versus 3-wall: 11.6 ± 7.8, p = 0.447). The improvement in MD and PSD were higher after 3-wall decompression (MD: 2-wall: 10.0 ± 5.5 versus 3-wall: 14.3 ± 7.5 dB, p = 0.020); (PSD: 2-wall: 3.5 ± 1.9 versus 3-wall: 4.8 ± 3.0 dB, p = 0.045). Proptosis reduction was higher after 3-wall decompression (2-wall: 5.1 ± 1.3 versus 3-wall: 7.2 ± 1.9 mm, p = 0.0001). New onset diplopia was seen 20% and 28.5% of cases in 2-and 3-wall decompression, respectively. No adnexal/orbital complications were seen in 2-wall group, however orbital hematoma (1 case) and persisting eyelid edema (1 case) were encountered in 3-wall group.Conclusion: Both 2-and 3-wall orbital decompressions are safe and effective for management of visual dysfunction in DON. Although 3-wall decompression provide better improvement in the parameters of visual field analysis and Hertel measures, new onset diplopia, adnexal/orbital complications are more common with this technique.

Thyroid-stimulating immunoglobulins indicate the onset of dysthyroid optic neuropathy.

Recognition of dysthyroid optic neuropathy (DON) requires sensitive diagnostic tools. Clinical assessment may fail to reliably evaluate the acuteness of DON especially if signs for inflammation are missing. Aim of this cross-sectional study was to assess the relationship between thyroid-stimulating immunoglobulins (TSI) and onset of DON. At a multidisciplinary orbital center, serum TSI levels were measured in 180 consecutive patients with thyroid eye disease (TED) and 302 healthy controls with a FDA-cleared cell-based bioassay using a chimeric TSH receptor and a CRE-dependent luciferase. Thirty of 180 (16.7 %) patients with TED had DON of recent onset or a past history of DON (post-DON). Optic disk swelling was present and visual-evoked potentials were pathologic in all eyes with DON of recent onset, but in one of 13 (7.7 %) with post-DON, only (p = 0.005). 19/20 (96 %) patients with DON of recent onset were TSI-positive. TSI was associated with DON of recent onset (OR: 20.96; 95 % CI 1.064-412.85, p = 0.045). All controls were TSI negative. TSI correlated with the clinical activity score (R = 0.70, p < 0.001) and higher TSI-levels were noted in active vs. inactive TED (485.1 ± 132.3 vs. 277.7 ± 143.7 %, cut-off < 140 %; p < 0.001). Six of seven (85.7 %) patients with inactive TED with recent onset DON versus one of four (25 %) with active post-DON were TSI-positive (p = 0.006). A discriminatory cut-point of 377 SRR % for TSI was determined based on a ROC analysis (sensitivity: 0.95, specificity: 0.8). Serum TSI levels identify patients with DON of recent onset requiring urgent therapy.

Prospectively recorded and MedDRA-coded safety data of intravenous methylprednisolone therapy in Graves' orbitopathy.

Safety of intravenous (IV) steroid pulses in patients with Graves' orbitopathy (GO) is still controversial while steroid dose and treatment application have not been finalized. Frequency, severity and characterization of adverse events (AE) were prospectively analyzed. Academic referral orbital center with a joint thyroid-eye clinic. Eighty consecutive and unselected patients with active and severe GO. During an established treatment with IV methylprednisolone (cumulative dose 4.5 g) occurring AE were prospectively coded according to the standardized and recognized medical dictionary for regulatory activities (MedDRA). Outcome and severity of AE were documented. AEs judged as at least possibly related to drug treatment were graded as side effect (SE). AEs matching a seriousness criteria as defined by the ICH guideline E6 (good clinical practice) were graded as serious. A total of 38.75% (31/80) of the treated GO patients reported at least one AE while 18 patients (22.5%) reported at least one SE. All SE were within the safety profile of IV methylprednisolone; 31/32 SE (96.87%) were mild-moderate and reversible and only 1/80 patient (1.25%) stopped steroid treatment due to exacerbation of her depression. Most AE were accessory symptoms of the underlying disease and a few only were directly related to IV steroids. Most AEs (90.6%) were graded as mild. Only six patients (7.5%) were hospitalized, three of them due to a dysthyroid optic neuropathy. Prospective and standardized evaluation with MedDRA and the ICH guideline demonstrated the good pharmacological tolerance and low morbidity of this moderate steroid regimen.

Optic Neuropathy in 2 Thyroidectomized Patients with Moderate to Severe Graves Ophthalmopathy Following L-Thyroxine Withdrawal Prior to Radioiodine Treatment for Thyroid Carcinoma

ABSTRACT Objective: To report on 2 patients with moderate to severe Graves ophthalmopathy (GO) who developed dysthyroid optic neuropathy following levothyroxine (LT4) withdrawal in preparation for ...

An algorithmic approach in the diagnosis and management of thyroid eye disease

Thyroid eye disease (TED) or Graves ophthalmopathy is commonly associated with thyroid dysfunction and often causes a debilitating effect on the individual. Its presentations can result in misdiagnosis and often treatment is delayed in the initial acute inflammatory stages of the disease. The pathophysiology of this disease remains a complex process. The clinical examination, the activity and severity can help us decide the stage of the disease to help us formulate a management plan. The treatment strategy for TED is multi-pronged and consists of different modalities such as medical therapy, functional and cosmetic surgery and radiotherapy. Medical therapy ranges from supportive ocular surface protection and lubrication to potent immunosuppressive agents. Surgery, too can be vision-saving in severe dysthyroid optic neuropathy or exclusively cosmetic in cases of burnt-out orbitopathy. This review, discusses the examination, investigations of TED but essentially presents a step-by-step question based approach in the staging, diagnosis and management of thyroid eye disease.

Thyroid eye disease: a Southeast Asian experience

AimTo study the demographics, comorbidities, clinical manifestations and treatment methods of thyroid eye disease (TED) in Singapore.MethodsIn this retrospective case series, we analysed the case records of all patients with...

Is high dose intravenous methylprednisolone pulse therapy in patients with Graves' orbitopathy safe?

High dose intravenous glucocorticoid pulse (i.v. GCS) therapy is a proven approach in patients with active, moderate to severe Graves' orbitopathy (GO) and dysthyroid optic neuropathy (DON). In moderate to severe GO, the European Group on Graves' Orbitopathy (EUGOGO) recommends a 12-week course of intravenous methylprednisolone (i.v. MP) pulse therapy with a cumulative dose of 4.5 g. The response rate of i.v. GCS treatment is significantly higher than oral glucocorticoid (oral GCS) therapy and is associated with fewer adverse events. However, a major concern was raised because of reports of fatal side effects which may be associated with this therapy, especially when single and cumulative doses of methylprednisolone (MP) are higher than recommended. The prevalence and severity of adverse effects during treatment have not been fully described. The aim of this review was to summarise the frequency of major adverse effects of i.v. GCS compared to oral GCS and attempt to propose some practical suggestions as to how to monitor and prevent the development of side effects.

Survey of Common Practices Among Oculofacial Surgeons in the Asia-Pacific Region: Graves’ Orbitopathy

Aim: A web-based anonymous survey was performed to evaluate practice preferences in the management of Graves’ orbitopathy amongst oculofacial surgeons in the Asia-Pacific region.Methods: A questionnaire with contentious topics in Graves’ orbitopathy was sent out via email to oculofacial surgeons in 14 countries within Asia-Pacific between May to December 2012.Results: A response rate of 25.2% to 34.6% was achieved (32–44 of 127 participants). 61.0% of respondents encountered Graves’ orbitopathy commonly in their practice. The more common causes of vision loss in Graves’ orbitopathy included dysthyroid optic neuropathy (67.5%) and exposure keratopathy (15.0%). The clinical activity score was the most popular grading system for assessing Graves’ orbitopathy. The preferred non-surgical therapeutic approaches included intravenous pulsed methylprednisolone (79.5%), oral steroids (56.4%), orbital radiation (12.8%), steroid-sparing immunosuppressants (10.3%) and watchful observation (7.7%). Thyroid orbital decompression was uncommonly or rarely performed by respondents. Orbital surgical decompression was most commonly performed via the two-wall technique (73.5%) and most respondents performed fat decompression (69.4%). Post-operatively, the most common complications include under correction (45.5%) and diplopia (42.4%).Conclusion: We report the results of the first survey on the management of Graves’ orbitopathy amongst oculofacial surgeons in Asia-Pacific. Our respondents practice preferences reflected the Graves’ orbitopathy management consensus statement by the European group on Graves’ orbitopathy in 2008.

Thyroid eye disease: therapy in the active phase.

The management of active thyroid eye disease (TED) can be a challenging therapeutic dilemma. The pathogenic complexity, disease heterogeneity, clinical unpredictability, and ocular morbidity associated with TED necessitate a team approach. A literature search ending on December 31, 2013, was performed using PubMed (http://www.ncbi.nlm.nih.gov/pubmed) with the following search terms: Graves' disease, hyperthyroidism, hypothyroidism, Graves' orbitopathy, Graves' ophthalmopathy, thyroid eye disease, thyroidectomy, antithyroid medications, radioactive iodine, orbital decompression, orbital radiotherapy (ORT), proptosis, and optic neuropathy. The search included manuscripts in English only. Additional articles and textbooks were retrieved from the reference list of articles that were obtained from the original PubMed literature search. Corticosteroids, ORT, and orbital decompression have been the mainstay treatment modalities for active TED for more than 50 years. Few randomized controlled studies have systematically evaluated these treatment strategies, and of those trials that have been executed, they are difficult to compare and contrast because of inconsistencies in study design and outcome measures. Newer immunosuppressive and immunomodulating agents are being investigated with anecdotal evidence of improved efficacy compared with traditional treatments. All patients with TED must be assessed for disease activity and severity to determine the best course of action. Risk factor modification begins with smoking cessation and attaining euthyroid status. The first-line treatment for moderate-to-severe TED or dysthyroid optic neuropathy is systemic corticosteroids; but often a multimodality approach with the addition of ORT or orbital decompression may be required. The development of novel therapeutic agents against specific immunological targets will improve upon the current treatment armamentarium available to clinicians and patients with TED. Uniformly accepted, scientifically reliable and clinically valid outcome measures integrated into well-designed clinical trials are needed to advance the management of TED to a more evidence-based approach.

Orbital Volumetry in Graves' Orbitopathy: Muscle and Fat Involvement in relation to Dysthyroid Optic Neuropathy

Purpose. We wanted to investigate the relative significance of fat and muscle enlargement in the development of dysthyroid optic neuropathy (DON) in Graves' orbitopathy (GO). Methods. Preoperative coronal CT scans of 13 patients with and without DON who subsequently underwent orbital decompression were retrospectively analyzed. Thirteen patients imaged for unilateral orbital fractures served as controls. Results. The retrobulbar muscle volume was 2.1 ± 0.5 cm3 (mean ± SD) in controls, 4.3 ± 1.5 cm3 in GO without DON, and 4.7 ± 1.7 cm3 in GO with DON. The retrobulbar fat volume was 5.4 ± 1.6 cm3 in controls, 8.7 ± 8.0 cm3 in GO without DON, and 9.4 ± 3.1 cm3 in GO with DON. The muscle and fat volumes were higher in patients with GO than in controls (P < 0.001), but the volumes in orbits with and without DON were not significantly different. The volume of the optic nerve were similar in the 3 groups. The number of apical, coronal 2 mm thick slices with no fat was 2.9 ± 0.9 in normal orbits, it was 4.1 ± 1.0 in GO orbits without DON and 5.3 ± 0.8 in GO orbits with DON (P = 0.007). Conclusion. Apical muscle enlargement may be more important than orbital fat enlargement in the development of DON. However, the fact that apical crowding and muscle enlargement also occur in orbits without DON suggests that other factors also play a role in the development of DON.

Therapeutic Outcomes of High-Dose Intravenous Steroids in the Treatment of Dysthyroid Optic Neuropathy

While pulsed intravenous methylprednisolone (iv-MP) has been shown to be effective and well tolerated in moderate to severe Graves' orbitopathy (GO), limited data are available on dysthyroid optic neuropathy (DON). The objective of this retrospective study was to investigate the efficacy of iv-MP in the treatment of DON and to seek parameters predictive of response. Twenty-four DON patients (40 eyes) treated with iv-MP from 2007 to 2012 were included in the study. Concurrent neurological or ophthalmologic diseases or signs of corneal exposure were considered as exclusion criteria. Iv-MP was administered daily for three consecutive days and repeated the following week. At six months, eyes not requiring surgery to preserve visual function were considered as responsive to treatment. Visual acuity, color sensitivity, visual field, and optic discs were analyzed at two and four weeks, and at 3, 6, and 12 months after treatment. Activity of GO was graded using a clinical activity score (CAS). Visual and clinical characteristics of the eyes responsive to iv-MP were studied by comparison to those of nonresponsive eyes. At six months, 17 of 40 (42.5%) eyes had complete visual recovery and were spared from surgical decompression. At two weeks, visual acuity, color sensitivity, and visual field improved significantly in almost all eyes, but GO inactivated (CAS<4) only in the eyes that permanently responded to iv-MP (p<0.01). The CAS at two weeks was a good predictor of response (cutoff ≥4; 66.7% sensitivity, 76.9% specificity). Optic disc swelling at diagnosis was highly predictive for unresponsiveness to iv-MP (34% sensitivity, 100% specificity). At baseline, high CAS (cutoff >5; 40.2% sensitivity, 94.1% specificity) and severely altered visual field mean defect (cutoff ≤6.31 dB; 73.9% sensitivity, 58.8% specificity) were associated with unresponsiveness to steroids. No major side effects were observed. High-dose iv-MP was effective in permanently restoring visual function in about 40% of the eyes treated. When successful, it generally induced inactivation of the orbital disease within two weeks and normalization of visual function within one month. The presence of optic disc swelling at diagnosis and persistent active disease at two weeks were good predictors of unresponsiveness to steroids.

Intraocular Pressure Lowering Effect of Orbital Decompression is Related to Increased Venous Outflow in Graves Orbitopathy

Purpose: To investigate the effects of combined orbital bone and fat decompression on intraocular pressure (IOP) and superior ophthalmic vein blood flow velocity (SOV-BFV), and their association with the clinical features of Graves orbitopathy (GO).Methods: During the 2002–2008 period, 72 eyes of 36 GO cases demonstrating moderate to severe orbitopathy were evaluated according to their clinical features as: cases with or without dysthyroid optic neuropathy (DON), and underwent orbital decompression. A control group comprised 40 eyes of 20 healthy subjects. In both groups, a full ophthalmic examination including IOP and Hertel measurements was performed, and SOV-BFV was analyzed with color Doppler imaging. Examinations were repeated after orbital decompression in GO patients.Results: All the cases demonstrated clinical features of inactive disease. Among the patients 24 of 72 eyes (33.3%) showed clinical features of DON. After surgery, the mean decrease in Hertel values was 6.2 ± 1.8 mm (p = 0.001). The mean decrease in IOP was 3.0 ± 1.7 mmHg (from 17.3 ± 2.7 to 14.3 ± 2.0 mmHg) after orbital decompression where the post-operative values were comparable with the control group (12.9 ± 1.4 mmHg, p = 0.36). The mean increase in SOV-BFV achieved with decompression was 1.2 ± 0.6 cm/s (from 4.8 ± 1.7 to 6.0 ± 1.8 cm/s) and post-operative SOV-BFV values were also comparable with the control group (6.6 ± 1.3 cm/s, p = 0.26). The increase in SOV-BFV in cases with DON did not differ from cases without DON (p = 0.32), however, post-operative SOV-BFV of cases with DON was stil lower than cases without DON (p = 0.035).Conclusions: Combined orbital bone and fat decompression significantly reduced the IOP levels and increased the SOV-BFV in GO. This could be the confirmative finding of prediction that elevated IOP in GO is associated with increased episcleral venous pressure. The post-operative changes in IOP and SOV-BFV show differences regarding the clinical features of disease.