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Purpose To report a single-center experience with non-thyroid causes of extraocular muscle enlargement (EME), describing the association between clinical-radiological findings at presentation and the final histopathological diagnosis. Methods Retrospective consecutive case series of 59 patients with single or multiple EME on orbital imaging, in the absence of an etiological diagnosis at the time of presentation. All patients were submitted to orbital muscle biopsy in order to achieve a final etiological diagnosis. Patients with a confirmed diagnosis of thyroid-associated orbitopathy and vascular causes of EME which were angiographically and clinically diagnosed were excluded. Orbital ultrasound and radiologic evaluation (CT and/or MRI) were performed before surgery in all cases. Main outcomes measured included initial clinical-radiological findings and final histopathological features of EME. Results A diagnosis of lymphoma was confirmed in 13 cases (22%). Sixteen cases (27%) were diagnosed as orbital inflammatory disease including nonspecific idiopathic orbital inflammatory disease in 9 cases, IgG4-related disease in 4 cases, and sclerosing idiopathic orbital inflammatory disease in 3 cases. In 11 patients (18%), a diagnosis of metastatic tumor was made, whereas sarcoidosis, vascular malformations, Erdheim-Chester, and necrobiotic xanthogranuloma were diagnosed in 8 eyes (13.5%). Three patients (5%) with single muscle enlargement developed Graves disease 10 months later. Four patients (6.7%) were diagnosed with granulomatosis with polyangiitis. In 2 cases (3.3%), the diagnosis was unknown, with inconclusive biopsy results. Differential patterns for inflammatory/vascular, lymphomatous and metastatic EME were identified based on age and gender distribution and clinical-radiological characteristics at presentation. Conclusions Initial clinical and radiological features may orientate the differential diagnosis of non-thyroid EME.

Thyroid associated orbitopathy (TAO) is a common diagnosis encountered by ophthalmologists and oculoplastic surgeons. TAO has a varying clinical presentation that can include upper eyelid retraction, restrictive strabismus, proptosis, exposure keratopathy, and optic neuropathy. In this review, we discuss the most recent literature on and the current understanding of the pathophysiology of TAO. We also review available and potential future treatment options for the management of TAO.

Presented are 2 cases of acute corneal hydrops in keratoconus with Graves’ orbitopathy (GO). Two patients (Case 1: female, 54 years old; Case 2: male, 33 years old) with coexisting keratoconus and GO demonstrated typical findings of acute corneal hydrops (ACH) in 1 eye during the active stage of orbitopathy. There was no history of trauma. The ACH healed with scarring after management with a therapeutic contact lens and medical treatment in each patient within 3 months and 5 months, respectively. The development of ACH in keratoconus patients has previously been reported to be associated with male gender, ethnicity, age, eye rubbing, trauma, rapidly progressive disease, atopy, and vernal conjunctivitis. GO involves ocular surface inflammation and fluctuation of intraocular pressure. Active GO can be a risk factor for ACH in keratoconus patients.

Purpose: To report the different uncommon pathogenesis of three cases of severe vertical restrictive strabismus associated with progressive unilateral proptosis with similar clinical features. Methods: Case series of three patients who presented to the Orbit Outpatient Service of Policlinico Gemelli with a history of left progressive unilateral proptosis, slowly worsening vertical strabismus and the left eye fixed in downward position. A thorough hematologic work up was performed. All patients underwent complete abdomen ultrasonography, orbital contrast enhanced magnetic resonance imaging, forced duction test under general anesthesia, and orbital biopsy. Results: Patients were 30, 60, and 46 years old respectively. MRI showed left inferior rectus enlargement in two cases and superior rectus enlargement in one case, with contrast enhanced combined muscle belly and tendon enlargement in all cases. Patients underwent forced duction test, muscle weakening (in two cases), and muscle biopsy with histopathologic examination. The superior rectus appeared infiltrated by an undifferentiated high-grade pleomorphic sarcoma, whereas the two inferior recti were positive for idiopathic orbital inflammatory disease with fibrosis areas and neuromuscular choristoma, respectively. Conclusion: Although proptosis and acquired vertical restrictive strabismus are most commonly associated with thyroid associated orbitopathy (TAO), they can also be a manifestation of many other conditions and the differential diagnosis can be particularly challenging. The three reported cases presented indeed with similar clinical features but had three distinct underlying orbital etiologies, two of which were extremely uncommon.

Thyroid associated orbitopathy (TAO) occurs in patients with various diseases of the thyroid gland. The levels of episcleral venous pressure (EVP), intraocular pressure and intraorbital pressure are inter- related. There are no precise data on the change of EVP in patients with TAO. Purpose. To evaluate EVP in patients with compensated and sub-compensated TAO forms. Methods. Data of 41 eyes of 22 patients were enrolled into the study. The main index to be evaluated was the EVP. Results. EVP level was significantly higher in complete venous compression in the lower- temporal quadrant in patients with sub-compensated TAO stage (p = 0.013). Conclusion. The degree of venous outflow obstruction and the EVP level of are interrelated. Thus, the level of EVP can be used as an additional factor in assessing the severity of the disease course and the treatment efficacy.

Purpose To describe the effect of tocilizumab in two patients with thyroid associated orbitopathy (TAO). Observations We present two patients with TAO who could not tolerate corticosteroids and had a reduction in clinical and laboratory markers of inflammatory activity with subsequent tocilizumab therapy. Conclusions and importance The IL-6 receptor antibody tocilizumab is a promising candidate for the treatment of TAO because it selectively targets a key inflammatory mediator and has a favorable side effect profile. Our report demonstrates that tocilizumab can achieve further reduction in inflammatory activity after treatment with corticosteroids. Importantly, we and others have observed a decrease in the level of thyroid stimulating immunoglobulin (TSI) with tocilizumab treatment. This suggests an upstream effect in the inflammatory cascade. Although the impact of tocilizumab on long-term outcome is unknown at this time, we believe that early disruption of the inflammatory process may prevent late complications and decrease the need for rehabilitative surgery.

Sang Beom HanDepartment of Ophthalmology, Kangwon National University Hospital, Kangwon National University Graduate School of Medicine, Chuncheon, KoreaI read with interest the article entitled “Subconjunctival orbital fat prolapse and thyroid associated orbitopathy: a clinical association” by Chatzistefanou et al.1 The case-series study was undoubtedly well designed and conducted, in which the authors successfully revealed that subconjunctival orbital fat prolapse may occasionally be a predominant clinical manifestation of thyroid-associated orbitopathy (TAO) and suggested that the presence of subconjunctival orbital fat prolapse can alert the diagnosis of thyroid orbitopathy.However, I would like to point out that the mechanism underlying the association between the two conditions is still unclear. Subconjunctival fat prolapse is usually caused by forward herniation of intraconal fat tissue due to dehiscence of tenon capsule precipitated by aging process or trauma.2 In TAO, swelling and inflammation of orbital fat can lead to separation of the orbital septum from the capsulopalpebral fascia, which can cause fat prolapse into the orbit.3 Previous studies showed that intraorbital inflammation in TAO can lead to apical crowding in orbit and intracranial fat prolapse.4,5 Therefore, as the authors postulated, it would be plausible that elevated intraorbital pressure and increase in orbital fat in TAO can also allow anterior herniation of intraorbital fat.Authors’ replyKlio I Chatzistefanou,1 Christianna Samara,2 Ioannis Asproudis,3 Dimitrios Brouzas,1 Marilita M Moschos,1 Elisabeth Tsianta,1George Piaditis41First Department of Ophthalmology, National and Kapodistrian University of Athens Medical School, Athens, Greece; 2Department of Radiology and Endocrinology, 3Department of Ophthalmology, University of Ioannina, Ioannina, Greece; 4Athens General Hospital “G. Gennimatas”, Athens, GreeceWe appreciate Dr Han’s comments and interest in our article. Intracranial herniation of intraorbital fat through the superior orbital fissure has been reported at an incidence of 19% in patients with thyroid ophthalmopathy in one study,1 and its prevalence may vary between 24% and 82% in different studies1–3 among patients with dysthyroid ophthalmopathy.We reviewed again data from the patients’ charts for symptoms and signs of compressive optic neuropathy upon presentation with subconjunctival fat prolapse.4 There were no afferent pupillary defects or optic nerve head swelling or atrophy noted on fundoscopy in any of the patients reported in this series. Clinical suspicion for possible compressive optic neuropathy, prompting paraclinical investigation with visual field testing, had been raised for patient #2 who had a slight decrease in visual acuity to 0.9 in the right eye. Visual field testing by automated perimetry was within normal limits. Visual acuity improved to 1.0+ in the involved eye with a change in astigmatic correction.View original paper by Chatzistefanou et al

AIM: To determine the relevance of the objective parameters addressing the altered biomechanical properties of cornea for glaucoma monitoring in patients with mild or moderate thyroid associated orbitopathy (TAO), and in healthy individuals. METHODS: Twenty-five patients with TAO (group 1) and 25 healthy adults (group 2) were included to the study. Both groups were of a similar age and the ratio women:man. For each patient, the following parameters of both eyes were measured with ocular response analyzer (ORA): corneal hysteresis (CH), corneal resistance factor (CRF), Goldmann correlated intraocular pressure (IOPg) and corneal compensated intraocular pressure (IOPcc). In both groups participating in our study, all measurements were performed within minutes to reduce the diurnal effects. RESULTS: The mean age in group 1 was 56±11y and 76% were women, 24% were men. The mean age in group 2 was 64±11y and 68% were women, 32% were men. CH correlated negatively with IOPg in group 1 (r2=0.10, P0.05) and also no significant correlation in group 2 (r2=0.04, P>0.05). CRF mean value in group 2 (11.51±1.72 mm Hg) was higher than in group 1 (10.85±1.45 mm Hg) (P

Purpose To descirbe a series of patients in which Thyroid Associated Orbitopathy (TAO) occurred after periocular surgery. Methods A retrospective case review of patients who developed TAO in close temporal association with periocular surgical interventions and presented at the orbital clinic from 1997 to 2004. History of previous thyroid abnormality and the lack of TAO signs and symptoms before surgery were reviewed and analyzed. Results Nine patients that developed TAO in association with periocular surgery were identified. All were women with an average age of 59.3years. (range: 45-75 years). The patients divided into two groups. Group 1 consisted of four patients who had previously been diagnosed with Graves' hyperthyroidism (GH). They ranged in age from 48 to 75 years (average: 58.8 years). The diagnosis of GH had been made an average of 50.5 months (range: 12-96 months) before presentation with TAO. Group 2 consisted of five patients who had no previous history of thyroid abnormality. They ranged in age from 45 to 74 years (average: 60.2 years). No patients had any signs or symptoms of TAO before their recent presentation. Conclusions Periocular surgery may lead to local inflammatory events that may contribute to the instigation of TAO in predisposed individuals.