Restrictive Myopathy Research Articles

Clinical Profile of Thyroid Eye Disease and Factors Predictive of Disease Severity in Chennai District of Tamil Nadu

Abstract Background: Thyroid eye disease (TED) is a possibly sightthreatening ocular disease generally occurring in the patients diagnosed with hyperthyroidism. TED, also known as Graves orbitopathy, is typically self-limiting auto-immune disease linked with a dysthyroid status. Based on the ocular symptoms and signs, the current grading systems of TED are the VISA classification and the EUGOGO classification; each parameter of VISA score is separately graded and scored. Worsening in any of the VISA parameters is graded as an active disease in the geographic region of South India; there are no studies on clinical profiles of TED; hence, we planned to do this study to analyse the demographic profile and clinical manifestations of TED in patients. Material and Methodology: This study was done as an observational study for a period of 12 months. All patients with systemic thyroid disorder are included in the study. If lid retraction is seen, the presence of laboratory evidence of thyroid dysfunction, exophthalmos, optic nerve dysfunction, or extra ocular muscle involvement will be considered as TED. In case of no lid retraction in a patient with laboratory evidence of thyroid dysfunction, the presence of exophthalmos, optic nerve involvement, or restrictive myopathy will be considered as TED. Patients with diagnosis of TED were further evaluated. TED was graded according to VISA and EUGOGO classification. Results: This study was done as an observational study in 50 patients diagnosed with TED. In our study population, 32 (64%) were hyperthyroid, 11 (22%) were hypothyroidism, and the rest 7 (14%) were euthyroid. The most common presenting complaints were dryness of eye in 46% of patients, followed by blurred vision in 38% of patients. We evaluated severity of disease using EUGOGO score, and 42 patients had mild disease, while six had moderate disease and two patients had severe disease. Based on VISA score, in our study population, only two patients had active disease and the rest 48 had inactive disease. Conclusion: Thyroid status has a direct and positive correlation with active stage of disease and severity. Hyperthyroidism and active disease were risk factors for severe and sight-threatening disease, emphasising the need for close follow-up and prompt detection and management of severe and sight-threatening disease, especially in newly diagnosed hyperthyroid patients. It is important to diagnose TED at the earliest to prevent vision-threatening complications. Early identification and management will help in reducing the severity of disease.

Orbital radiotherapy with combined intravenous methylprednisolone and mycophenolate mofetil in moderate-to-severe thyroid eye disease

PurposeTo report the clinical outcomes of orbital radiotherapy (ORT) with combined intravenous methylprednisolone (IVMP) and mycophenolate mofetil (MMF) in thyroid eye disease (TED) patients with restrictive myopathy. DesignProspective comparative case series of patients managed at The Chinese University of Hong Kong from 2015 to 2021. MethodsThe primary outcome was the change in Gorman diplopia score at 52 weeks. Secondary outcomes were the changes in extraocular muscle motility (EOMy), area of extraocular muscle (EOM) on MRI, exophthalmos, and marginal reflex distance (MRD) 1 and MRD2 at 52 weeks. ResultsA total of 40 (29 females) TED patients, medium age 56 years, were treated with the double regimen: combined IVMP and MMF (17) or triple regimen: IVMP, MMF, and ORT (23). The triple group has a higher age (P = 0.028). Sex, smoking history, thyroid, and pre-treatment orbital status were comparable between the 2 groups. EOMy improved more after the triple regimen(P = 0.026). Nine patients from the triple group showed a significant reduction in the size of the inferior rectus (both eyes: P < 0.05), while five from the double group showed no significant reduction of any EOM on MRI. The improvement of CAS, diplopia score, exophthalmos, MRD1, and MRD2 were similar. No patient reported any severe or organ-threatening side effects from IVMP, MMF, or ORT requiring discontinuation of treatment. ConclusionThe triple regimen demonstrates better structural and functional outcomes in TED-related restrictive myopathy. Future randomized clinical trials are warranted to clarify the adjuvant role of ORT in this specific indication of TED management.

Yes-Associated Protein Mediates the Transition from Inflammation to Fibrosis in Graves' Orbitopathy.

Background: In Graves' orbitopathy (GO), localized orbital inflammation within the fixed orbit often leads to a fibrotic phenotype resulting in restrictive myopathy or refractory proptosis. However, the molecular pathways related to the transition from inflammation to fibrosis in GO are less understood. Yes-associated protein (YAP) and its homolog, transcriptional coactivator with PDZ-binding motif (TAZ; a Hippo pathway effector), are critical mechanosensors of mechanical stimuli and activate signaling cascades for cell proliferation, differentiation, and transformation. In this study, we aimed to examine the role of YAP in both inflammatory and fibrotic GO pathogenesis. Methods: Based on RNA sequencing performed on freshly obtained orbital adipose tissue from patients with GO and healthy individuals, Gene Ontology analysis and gene set-enrichment analysis were performed to analyze gene-expression differences between GO and normal orbital tissues. The role of YAP in GO-related inflammation and fibrosis was studied in primary cultured orbital fibroblasts. The effects of interleukin-1β (IL-1β)-induced inflammation and transforming growth factor-beta (TGF-β)-induced fibrosis on YAP expression were evaluated using real-time polymerase chain reaction and Western blotting analyses. The effects of YAP on inflammatory and fibrotic responses were also examined by YAP silencing or treatment with pharmacological YAP inhibitors. Results: RNA sequencing revealed enhanced YAP expression in GO orbital tissues. Gene Ontology analysis indicated that "response to mechanical stimulus"-related genes were overexpressed in GO orbital tissues, along with those enriched for the "adipose proliferation," "inflammatory responses," and "hormone stimulus responses" terms. IL-1β did not enhance YAP expression, and YAP silencing decreased IL-1β-induced IL-6 expression while increasing prostaglandin-endoperoxide synthase 2 expression, leading to paradoxical pro-inflammatory effects. Conversely, TGF-β enhanced YAP expression, and YAP silencing and pharmacological YAP inhibitor (cerivastatin, verteporfin, TED-347, and CA3) treatment significantly reduced TGF-β-induced myofibroblast differentiation and collagen formation. Conclusion: YAP, a mechanotransducer responding to mechanical stimuli, was strongly expressed in GO orbital tissues, and YAP was induced by TGF-β in orbital fibroblasts. Our study establishes YAP as a novel mediator of GO pathobiology, potentially mediating the transition from early inflammation to chronic fibrosis in GO. The finding that YAP inhibition suppressed TGF-β-induced fibrotic response suggests YAP as a therapeutic target against the fibrotic mechanism of GO.

Neuroimaging Findings in Acquired Esotropia Patients at Siriraj Hospital from 2009–2020

Objective: To analyze the neuroimaging findings in patients with acquired esotropia at Siriraj Hospital from 2009 to 2020. Materials and Methods: A retrospective review of the medical records of all patients aged above 6 months old who presented with acquired esotropia was done. All the patients had an angle of deviation 10 prism diopters (PD), refraction &lt; +3.0 diopters (D), and had neuroimaging performed. The patients with restrictive myopathy and extraocular neurological abnormalities were excluded from the study. Results: In total, 177 subjects aged between 8 months old to 81 years old (mean age 32.35 ± 24.40 years old) were included in the study. The patients’ demographic data, ocular findings, and neuroimaging findings were collected. The mean angle of deviation near and at distance were 26.88 ± 18.90 PD and 29.81 ± 17.25 PD, respectively. Of the 177 cases, 113 cases (63.8%) had no neuroimaging abnormalities, while 64 cases (36.2%) had abnormal neuroimaging findings. In those 64 cases with abnormal findings, there were 16 intracranial tumors, 6 inflammations, 4 vascular lesions, 7 degenerations, and 31 other lesions. Sixth nerve palsy was found to be significantly different between the positive and negative neuroimaging group from both the uni- and multivariate analysis. Conclusion: Although more than half of acquired esotropia patients had no abnormalities in neuroimaging, but 14.7% of those with abnormal neuroimaging findings had underlying serious conditions, which consisted of intracranial tumors, inflammation, and vascular lesions. Special attention should be concentrated to patients with sixth nerve palsy as it was the significant factor found to be associated with positive neuroimaging findings in acquired esotropia.

Restrictive medial rectus myositis in orbital sarcoidosis

Sarcoidosis is a systemic multiorgan granulomatous disease of unknown etiology in middle age group women. Orbital sarcoidosis is uncommon and the most common orbital manifestation is dacryoadenitis (70%). Extraocular muscle involvement is rare; if present, the superior rectus/levator complex and lateral rectus are most commonly involved. We report a 57-year-old male, a known case of biopsy-proven sarcoidosis, with medial rectus myopathy and lacrimal dacryoadenitis in the left eye, and angle closure with raised intraocular pressure (IOP) in both eyes. Imaging by computed tomography (CT) also confirmed the above findings. The forced duction test was positive confirming restrictive myopathy of the left medial rectus muscle. Orbital sarcoidosis has a highly diverse presentation; medial rectus involvement is a rare occurrence.

Early response to intravenous methylprednisolone therapy for restrictive myopathy in patients with thyroid eye disease.

To report the therapeutic efficacy of intravenous methylprednisolone (IVMP) in patients with restrictive myopathy caused by thyroid eye disease (TED). The present prospective uncontrolled study comprised 28 patients with TED and restrictive myopathy who presented with diplopia that had developed within 6months before their visit. All patients were treated with IVMP for 12weeks. Deviation angle, limitation of extraocular muscle (EOM) movement, binocular single vision score, Hess score, clinical activity score (CAS), modified NOSPECS score, exophthalmometric value, and the size of EOMs on computed tomography were evaluated. The patients were divided into two groups: those whose deviation angle had decreased or remained unchanged 6months after treatment (group 1; n = 17) and those whose deviation angle had increased in that time (group 2; n = 11). The mean CAS of the whole cohort significantly decreased from baseline to 1month and 3months after treatment (P = 0.03 and P = 0.02, respectively). The mean deviation angle significantly increased from baseline to 1, 3, and 6months (P = 0.01, P < 0.01, and P < 0.01, respectively). The deviation angle decreased in 10 (36%), remained constant in seven (25%), and increased in 11 (39%) of the 28 patients. When groups 1 and 2 were compared, no single variable was identified as a cause of deviation angle deterioration (P > 0.05). When treating patients with TED who have restrictive myopathy, physicians should be aware that some patients show worsening of the strabismus angle despite inflammation control with IVMP therapy. Uncontrolled fibrosis can result in motility deterioration.

Rare clinical phenotype of filaminopathy presenting as restrictive cardiomyopathy and myopathy in childhood

BackgroundFLNC is one of the few genes associated with all types of cardiomyopathies, but it also underlies neuromuscular phenotype. The combination of concomitant neuromuscular and cardiac involvement is not often observed in filaminopathies and the impact of this on the disease prognosis has hitherto not been analyzed.ResultsHere we provide a detailed clinical, genetic, and structural prediction analysis of distinct FLNC-associated phenotypes based on twelve pediatric cases. They include early-onset restrictive cardiomyopathy (RCM) in association with congenital myopathy. In all patients the initial diagnosis was established during the first year of life and in five out of twelve (41.7%) patients the first symptoms were observed at birth. RCM was present in all patients, often in combination with septal defects. No ventricular arrhythmias were noted in any of the patients presented here. Myopathy was confirmed by neurological examination, electromyography, and morphological studies. Arthrogryposes was diagnosed in six patients and remained clinically meaningful with increasing age in three of them. One patient underwent successful heart transplantation at the age of 18 years and two patients are currently included in the waiting list for heart transplantation. Two died due to congestive heart failure. One patient had ICD instally as primary prevention of SCD. In ten out of twelve patients the disease was associated with missense variants and only in two cases loss of function variants were detected. In half of the described cases, an amino acid substitution A1186V, altering the structure of IgFLNc10, was found.ConclusionsThe present description of twelve cases of early-onset restrictive cardiomyopathy with congenital myopathy and FLNC mutation, underlines a distinct unique phenotype that can be suggested as a separate clinical form of filaminopathies. Amino acid substitution A1186V, which was observed in half of the cases, defines a mutational hotspot for the reported combination of myopathy and cardiomyopathy. Several independent molecular mechanisms of FLNC mutations linked to filamin structure and function can explain the broad spectrum of FLNC-associated phenotypes. Early disease presentation and unfavorable prognosis of heart failure demanding heart transplantation make awareness of this clinical form of filaminopathy of great clinical importance.

Correlation of clinical parameters and objective assessment tools in active thyroid eye disease – Prospective analytical study

Primary objective of this study was to identify the disease in active phase, clinically assess it and start medical management. Secondary objective was to analyze whether the objective tools like B-scan and serum levels of IL-6 and HS-CRP aid in the diagnosis of activity in thyroid eye disease. : The aim wasto analyze the correlation of clinical parameters and significance of objective assessment tools in active thyroid eye disease. Clinically diagnosed cases of TED with lid signs, soft tissue changes, restrictive myopathy and bilateral axial proptosis were included in our study. Patients in chronic stable phase were excluded from study. This was a prospective analytical study, conducted from June 2010 till June 2012. Sample size was thirty patients in the age group of 20 to 60 years. Complete ophthalmology workup and objective disease assessment tools including B scan (OTI 1000 with 7.5-10 MHz) was used in our study. All patients underwent CT scan orbit-axial and coronal view. After assessing the severity and stage of the disease, each patient was managed accordingly using different modalities. : Identifying the disease activity early and aggressive management with systemic steroids in moderately active and severe stage has decreased the morbidity associated with the disease. A- scan with orbital B-scan helps in diagnosing the activity in thyroid eye disease. This procedure is very economical with relatively short examination time and no risk of radiation .Follow up of the patients can also be performed easily. IL-6 and HS-CRP was statistically significantly elevated in patients with severe disease when compared to control group. Hence can help in assessing disease activity in patients with severe disease.

Intraocular Pressure and Glaucoma in Thyroid Eye Disease.

Current literature lacks a summary of knowledge on intraocular pressure (IOP) elevation and glaucoma in thyroid eye disease (TED). This review aims to augment existing literature by providing such a summary. It qualitatively evaluates current knowledge on the pathogenesis, prevalence, and management of concomitant ocular hypertension or glaucoma in TED patients. In this narrative review, relevant publications were identified through a computerized database search. Search results were screened for relevance. Correspondence, Editorials, and Letters to the Editors were excluded. References cited within the identified articles were used to further augment the search. Information extracted for qualitative analysis included epidemiologic data, methods of IOP assessment, management protocols, and response to treatment. Studies in current literature are relatively heterogenous, differing in country of origin, TED cohort sizes, and IOP measurement techniques. Further studies are required to elucidate the true epidemiologic relationship between TED and ocular hypertension or glaucoma. Proposed models of IOP elevation include elevation of episcleral venous pressure, mucopolysaccharide deposition within the trabecular meshwork, restrictive myopathy, steroid-induced glaucoma, and secondary glaucoma. IOP-reducing effects of TED treatment options are discussed. While raised IOP in TED is a common phenomenon, the diagnosis of glaucoma in TED requires a high index of suspicion before any intervention.

28131 Hashimoto thyroiditis associated with unilateral Graves ophthalmopathy

Thyroid-associated orbitopathy (TAO) is caused by T-cell activation of retro-ocular fibroblasts and autoantibodies against thyroid-stimulating hormone receptor (TSHR) leading to accumulation of glycosaminoglycans and inflammation. The condition can present with soft tissue inflammation, proptosis, restrictive myopathy, and optic neuropathy. Although typically associated with Graves disease, TAO is occasionally seen in Hashimoto thyroiditis. We report a case of TAO presenting with unilateral periorbital edema in a patient with Hashimoto disease. A 63-year-old female with hypothyroidism was referred to our dermatology clinic by the ophthalmology service with a 2-year history of persistent and progressive right-sided nontraumatic periorbital swelling, associated with blurry vision, pain upon eye movement, and facial paresthesia. The patient was treated with high-dose systemic corticosteroids and radiotherapy for suspected Graves ophthalmopathy with partial response. Physical examination was remarkable for right-sided erythema and edema of the upper and lower eyelids. Skin biopsy revealed a superficial and deep lymphocytic infiltration with mucin. An orbital CT scan revealed right-sided hypertrophy of the medial rectus muscle and proptosis. Laboratory findings demonstrated elevated thyroglobulin, thyroid peroxidase, and TSHR antibodies. TSH and free T4 levels were normal. Clinicopathologic correlation supported a diagnosis of TAO in the setting of Hashimoto disease. TAO affects 2-5% of patients with Hashimoto thyroiditis. As seen in our patient, asymmetrical ophthalmopathy may be present, and in fact, these symptoms may precede endocrine manifestations. Prompt recognition and management of this condition may spare permanent neurological damage, including vision loss.

Prognostic factors of restrictive myopathy in thyroid eye disease

To investigate the prognostic factors of extraocular muscle restriction in patients with thyroid eye disease (TED), 65 patients with TED and restrictive myopathy were evaluated. Demographics, clinical activity score (CAS), smoking status, thyroid disease status, thyroid hormone status, thyroid autoantibody status, orbital computed tomography (CT) scan at initial presentation, and treatment regimens were assessed. The movements of the most severely affected extraocular muscles were categorized into five grades. The patients were divided into the improved and the not-improved group based on the improvement in the limitation of the extraocular muscle excursion (LOM) throughout the follow-up, and the groups were compared using clinical factors. The mean LOM significantly improved from 2.3 ± 1.1 to 1.7 ± 1.2 after 1 year of follow-up. The excursion of the most restricted muscle improved in 32 patients but not in 33 patients during the follow-up. The initial concentration of the thyroid-stimulating antibody (TSAb) was significantly lower in the improved (229.3 ± 114.1) than in the not-improved group (345.0 ± 178.6) (P = 0.02) Age, sex, smoking status, CAS, thyroid status, and muscle thickness on the CT scan did not significantly differ in the groups. This study showed that the initial concentration of TSAb is a factor affecting the recovery of restrictive myopathy.

Bilateral lacrimal gland enlargement in a patient with thyroid orbitopathy

: Thyroid Orbitopathy is a condition associated with thyroid dysfunction. In addition to exophthalmos, eyelid retraction, eyelid oedema, restrictive extra-ocular myopathy, lacrimal gland enlargement can also be observed in these patients. However thyroid orbitopathy is not usually been considered in the differential diagnosis of cases of isolated lacrimal gland enlargement. An elderly male patient at our institution presented with bilateral lacrimal gland enlargement as the clinical or radiologic sign and was later diagnosed as thyroid orbitopathy. Computerized tomography and/or magnetic resonance imaging of the orbits were obtained and demonstrated isolated lacrimal gland enlargement with enlargement of lateral rectus and inferior rectus. A case of bilateral lacrimal gland enlargement found in a patient with thyroid orbitopathy was noted. Patient was investigated and kept on follow up.: This case represents, enlargement of lacrimal gland as a presenting sign of thyroid orbitopathy. Further clinical and radiological studies are essential for a better understanding of the timing of lacrimal gland involvement. In such patients presenting with lacrimal gland enlargement, thyroid orbitopathy should be strongly considere d in the differential diagnosis in addition to causes such as of dacryo-adenitis and neoplasms.

P89-F Neurophysiological findings in 11 cases of dropped head syndrome

Background Dropped head syndrome (DHS) is a rare disorder characterized by weakness of the cervical paraspinal muscles with inability to extend the neck. It can be a part of several generalized neuromuscular processes or secondary to local conditions. Also, a restricted myopathy, “isolated neck extensor myopathy” (INEM), has been described. Material and methods 11 cases of DHS were referred to our Neurophysiology department in the period 2015–2018 for evaluation, with suspected neuromuscular etiology. ENG/EMG, repetitive nerve stimulation, sfEMG or all of them were performed according to the clinical suspicion and findings. Results In 4 patients, neurophysiological studies were compatible with neuromuscular junction disorder (one of them with overlapping myopathic features): findings showed clearly positive repetitive nerve stimulation in facial and spinal nerves. sfEMG mean consecutive difference (MCD) was above 80 μs, with some blocking, in these cases. In 6 patients, EMG proved myopathic changes: mild spontaneous activity in all of them (positive waves, mainly), and short, small motor unit action potentials in 5. Of particular interest, in the myopathic group, sfEMG showed increased jitter in few pairs of fiber, but normal mean jitter and without blocking. In 2 cases, the evaluation could not be concluded. The final diagnosis in 4 cases was myasthenia gravis, and myopathy in 7 (inflammatory myopathy or isolated neck extensor myopathy). All patients improved with specific treatment. Conclusions In our series, miastenia gravis and myopathy (INEM or inflammatory) were the main etiologies. Neurophysiological studies revealed distinctive features in both groups, with high diagnostic yield.

Clinical signs of thyroid eye disease

Thyroid Eye Disease (TED) or Thyroid Ophthalmopathy or Grave’s Ophthalmopathy is the single most common cause of bilateral proptosis in adulthood. It is associated with orbital involvement in 25-50% of patients and 5-10% of patients may develop serious complications like compressive optic neuropathy improved motility and inflammation. The characteristic clinical signs may include a combination of eyelid signs, lid lag, globe lag, proptosis, restrictive extraocular myopathy and optic neuropathy. Werner’s classification is helpful in summarizing the clinical classification of Grave’s Ophthalmopathy. Various diseases such as myasthenia gravis, orbital myositis, CPEO etc mimic thyroid eye disease and its important to know how they can be differentiated. Finally blood investigations and imaging studies are very helpful in endorsing the clinical signs and help one arrive at the diagnosis. Since various medical and surgical management is amenable to us, establishing the correct diagnosis of TED is extremely important. This disease may have sight threatening complications, if not diagnosed on time, therefore, it is important to know the clinical signs of TED. The management is conservative most of the times and a better understanding of the immunological pathogenesis may propel the medical management of the disease in a different direction. Keywords: Proptosis, Rrestrictive myopathy, Thyroid ophthalmopathy.

Strabismus, Strabismus Surgery, and Reoperation Rate in the United States: Analysis from the IRIS Registry

To describe the rates of strabismus, strabismus surgery, and strabismus surgery reoperations among all age groups in the United States. Retrospective analysis of electronic health record data. Patients from the IRIS (Intelligent Research in Sight) Registry. Description of the types and rates of strabismus and strabismus surgery from 2013 to 2016 with subgroups by age, sex, race/ethnicity, and region of the United States. The 1-year reoperation rate for strabismus surgery performed during 2013-2015 for all age groups. A total of 30 827 185 unique patients were identified; 846 477 (2.75%) had a diagnosis of strabismus: 3.02% of male patients and 2.55% of female patients (difference= 0.47%, 95% confidence interval [CI], 0.46-0.48, P < 0.0001). Strabismus surgery was performed in 40 780 (0.13%) unique patients during the 4 years. The rate of surgery ranged from 1.99% for children birth to 5 years of age to 0.05% for adults 40 years of age and older. Horizontal surgical codes were reported 38 813 times, vertical surgery codes were reported 9304 times, and superior oblique codes were reported 711 times. Adjustable sutures were used for 3027 patients (7.42%). Cases with a code for repeat eye muscle surgery or restrictive myopathy were reported for 6098 patients (14.9%). Esotropia accounted for 30.06% and exotropia in 21.77% of diagnoses reported for surgery. The rate of undergoing a reoperation within 1 year of a strabismus surgery was 6.72%, lowest for the group 6 to 9 years of age (3.95%) and increasing with age (P < 0.001) to 11.5% for patients 65 years of age and older. Strabismus is an uncommonly reported diagnosis in ophthalmologic practice. Overall, approximately 1 in 750 patients in the IRIS Registry received strabismus surgery (1 in 20 with a strabismus diagnosis) during a 4-year period. Reoperations during the first year after surgery were performed for 1 in 15 patients, increasing with age at surgery. "Big" data from clinical data registries represent real-world care that can be used to develop benchmarks for clinical outcomes and to identify areas for practice improvement and training program design.

Deep lateral wall orbital decompression following strabismus surgery in patients with Type II ophthalmic Graves’ disease

ABSTRACTPurpose: Surgical management of ophthalmic Graves’ disease traditionally involves, in order, orbital decompression, followed by strabismus surgery and eyelid surgery. Nunery et al. previously described two distinct sub-types of patients with ophthalmic Graves’ disease; Type I patients exhibit no restrictive myopathy (no diplopia) as opposed to Type II patients who do exhibit restrictive myopathy (diplopia) and are far more likely to develop new-onset worsening diplopia following medial wall and floor decompression. Strabismus surgery involving extra-ocular muscle recession has, in turn, been shown to potentially worsen proptosis. Our experience with Type II patients who have already undergone medial wall and floor decompression and strabismus surgery found, when additional decompression is necessary, deep lateral wall decompression (DLWD) appears to have a low rate of post-operative primary-gaze diplopia.Methods: A case series of four Type II ophthalmic Graves’ disease patients, all of whom had already undergone decompression and strabismus surgery, and went on to develop worsening proptosis or optic nerve compression necessitating further decompression thereafter. In all cases, patients were treated with DLWD. Institutional Review Board approval was granted by the University of Kansas.Results: None of the four patients treated with this approach developed recurrent primary-gaze diplopia or required strabismus surgery following DLWD.Conclusions: While we still prefer to perform medial wall and floor decompression as the initial treatment for ophthalmic Graves’ disease, for proptosis following consecutive strabismus surgery, DLWD appears to be effective with a low rate of recurrent primary-gaze diplopia.

Thyroid-associated Ophthalmopathy.

Thyroid-associated ophthalmopathy is the most frequent extrathyroidal involvement of Graves’ disease but it sometimes occurs in euthyroid or hypothyroid patients. Thyroid-associated ophthalmopathy is an autoimmune disorder, but its pathogenesis is not completely understood. Autoimmunity against putative antigens shared by the thyroid and the orbit plays a role in the pathogenesis of disease. There is an increased volume of extraocular muscles, orbital connective and adipose tissues. Clinical findings of thyroid-associated ophthalmopathy are soft tissue involvement, eyelid retraction, proptosis, compressive optic neuropathy, and restrictive myopathy. To assess the activity of the ophthalmopathy and response to treatment, clinical activity score, which includes manifestations reflecting inflammatory changes, can be used. Supportive approaches can control symptoms and signs in mild cases. In severe active disease, systemic steroid and/or orbital radiotherapy are the main treatments. In inactive disease with proptosis, orbital decompression can be preferred. Miscellaneous treatments such as immunosuppressive drugs, somatostatin analogs, plasmapheresis, intravenous immunoglobulins and anticytokine therapies have been used in patients who are resistant to conventional treatments. Rehabilitative surgeries are often needed after treatment.

Preoperative clinical features of reactivated of Graves' orbitopathy after orbital decompression.

PurposeTo investigate the incidence and preoperative clinical features of reactivated Graves' orbitopathy (GO) after orbital decompression.MethodsThis study included patients with GO who underwent orbital decompression for disfiguring proptosis and not compressive optic neuropathy and received postoperative follow-up care for more than 12 months. Patients who experienced active inflammatory signs within 6 months of decompression were excluded from analysis. The demographic characteristics, ophthalmic manifestations, and biochemical parameters of the patients were analyzed for association with reactivation of GO by logistic regression analysis.ResultsOut of the 92 patients included in this study, seven (7.6%) experienced reactivation of GO after orbital decompression. The mean time interval between surgery and reactivation of GO was 36.3±14.3 weeks. Univariate logistic regression analysis identified age, existing smoking habits, and modified NOSPECS and Gorman scores as significant factors for the reactivation of GO. The results of multivariate logistic regression analysis revealed that smoking and modified NOSPECS and Gorman scores were associated with the reactivation of GO.ConclusionsQuitting smoking is important for the prevention of reactivation of GO after orbital decompression. Patients with severe symptoms, especially those with restrictive myopathy, should be carefully monitored for reactivation of GO after orbital decompression.

Changes in pupillary distance after fat versus bony orbital decompression in Graves’ orbitopathy

ObjectiveTo report changes in pupillary distance (PD) after orbital decompression in patients with Graves’ orbitopathy (GO). DesignRetrospective comparative case series. ParticipantsA total of 59 cases who underwent the same type of orbital decompression on both eyes. MethodsThe medical records of 111 patients who underwent orbital decompression were reviewed retrospectively. Fifty-nine patients without restrictive myopathy who underwent the same procedure on both eyes were included. Patients were divided into the following 3 groups by procedure: only fat decompression (group 1, n = 18), fat and medial wall decompression (group 2, n = 11), and fat, medial, and inferior wall decompression (group 3, n = 30). Preoperative and postoperative PD and proptosis were measured. ResultsPD shortened by 2.9 mm in group 1 (p < 0.001), 2.4 mm in group 2 (p < 0.001), and 3.5 mm in group 3 (p < 0.001). Proptosis improved by 3.5 mm in group 1 (p < 0.001), 3.5 mm in group 2 (p < 0.001), and 4.6 mm in group 3 (p < 0.001). PD changes were not significantly different between the groups (p = 0.328). The change in PD was significantly correlated with the mean amount of proptosis reduction (r = 0.374, p = 0.003). ConclusionsPD shortened after orbital decompression in patients with fat proliferative GO. The change in PD was not related to the type of orbital decompression, but rather to the amount of proptosis reduction. Postoperative changes in PD should be considered when performing orbital decompression.

Botulinum toxin injection for restrictive myopathy of thyroid-associated orbitopathy: success rate and predictive factors

To evaluate the rate of and predictive factors for successful treatment of restrictive myopathy in thyroid-associated orbitopathy (TAO) using botulinum toxin injection. Twenty patients with restrictive myopathy of TAO were enrolled in the study. Abnormal thyroid function test results were not a prerequisite for inclusion. In each extraocular muscle 25 units of botulinum toxinA were injected. The success rate, calculated at 2years or last follow-up before surgery, was defined as proportion of the cases with esotropia of <10(Δ), vertical deviation of <5(Δ), and no diplopia in primary position and downgaze for at least 1year. Both univariate analysis and multivariate logistic regressions were performed to identify the factors associated with success. The procedure was successful in 11 cases (55%): in 8 patients with predominantly esotropia, 1 patient with predominantly hypotropia, and 2 patients of mixed type. Four factors were significantly associated with the success: type of deviation (P=0.007), lower amounts of hypotropia (P=0.001) and esotropia (P=0.05), and lower degree of extorsion (P=0.01). In the multivariate logistic regression, only lower amount of hypotropia was significantly associated with the success (P=0.09, OR=1.36). Botulinum toxin injection can be an effective alternative for the treatment of the restrictive myopathy in TAO. The best candidates for injection of the toxin are patients with esotropia, smaller angle of horizontal and vertical deviations, and lower degree of extorsion.