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In patients with thyroid-associated ophthalmopathy, responses to treatment are rare and usually minor. Teprotumumab, an antibody to the insulin-like growth factor I receptor, led to significant responses in 69% of patients and to decreased proptosis. Medical therapies for moderate-to-severe thyroid-associated ophthalmopathy (Graves’ orbitopathy) that have proved to be effective and safe in adequately powered, prospective, placebo-controlled trials are lacking. This unmet need is due to the incompletely understood pathogenesis of the disease. 1 Current treatments are inconsistently beneficial and often associated with side effects, and their modification of the ultimate disease outcome is uncertain. 1 – 3 Previous clinical trials, which were rarely placebo-controlled, suggest that high-dose glucocorticoids, alone 3 – 5 or with radiotherapy, 6 , 7 can reduce inflammation-related signs and symptoms in patients with active ophthalmopathy. However, glucocorticoids and orbital radiotherapy minimally affect proptosis and can cause dose-limiting adverse . . .

Thyroid-stimulating hormone (TSH) receptor antibody (TSH-R-Ab or TRAb) testing plays a pivotal role in arriving at the aetiological diagnosis in patients with thyrotoxicosis. A positive test establishes the diagnosis of Graves’ disease (GD) while a negative result in conjunction with imaging studies supports other possible aetiologies. In patients with GD, TRAb levels at diagnosis and at the time of withdrawal of antithyroid drugs can identify patients who are unlikely to achieve remission and guide clinical management decisions. We provide an algorithm that incorporates TRAb in the decision-making process for the management of thyrotoxicosis. The utility of TRAb in predicting the risk of fetal and neonatal thyroid dysfunction is established and widely accepted in guidelines. TRAb may also help in the diagnosis of Graves’ orbitopathy, especially in euthyroid or hypothyroid patients and its role in guiding its management is evolving as a useful adjunct to the clinical parameters used in making therapeutic decisions.Anti-thyroid peroxidase antibodies (TPOAb) and anti-thyroglobulin antibodies (TgAb) indicate thyroid autoimmunity. The most common use of TPOAb is to identify patients at a higher risk of progression to treatment-requiring hypothyroidism. They also aid the diagnosis of immune thyroiditis and Hashimoto’s encephalopathy. Thyroglobulin measurement is used to help guide differentiated thyroid cancer treatment. TgAb is used as an accompanying test with thyroglobulin measurement as its presence can interfere with the thyroglobulin assay. A negative TgAb result reduces the likelihood of, but does not exclude, interference with thyroglobulin assay.

Purpose To evaluate the changes in Bell’s phenomenon after inferior and medial orbital wall decompression in patients with thyroid-associated ophthalmopathy (TAO). Methods This prospective interventional study included patients with moderate to severe non-active TAO, who underwent inferior and medial wall orbital decompression. Bell’s phenomenon and adjusted Bell’s phenomenon were evaluated at baseline and six months after surgery. Bell’s phenomenon was assessed by holding the upper lid open during forced eyelid closure. Afterwards, the upward excursion, the distance between lower eyelid margin and lower corneal limbus, was measured. Adjusted Bell’s phenomenon distance is defined as the difference between margin reflex distance two (MRD2) and Bell’s phenomenon distance. Results Thirty TAO patients, including 15 males (50%), with a mean age of 43.6 ± 11.6 years, were enrolled. The distance of Bell’s phenomenon significantly decreased after surgery by an average of 3.25 ± 1.57 mm ( P  < 0.001). In addition, the difference between pre- and post-operative distance of adjusted Bell’s phenomenon was − 1.58 ± 2.13 ( P  < 0.001) corresponding to the worsening in the adjusted Bell’s phenomenon. Conclusion The result of our study demonstrated that Bell’s phenomenon decreases significantly after inferior and medial wall decompression, which could be considered a complication of this form of orbital wall decompression in TAO.

PurposeTo evaluate the long-term clinical efficacy of orbital fat decompression in treating proptosis in Graves' ophthalmopathy (GO).MethodsRetrospective review of 1604 eyes of 845 patients with symmetric (1518 eyes) and asymmetric (86 eyes) proptosis who received orbital fat decompression between 2003 and 2014. Changes in Hertel values were evaluated at baseline, 6 months postoperatively and yearly thereafter. Recurrence of proptosis, diplopia and other complications that required additional surgeries were documented and analysed. The surgical outcome was defined as complete success if there was proptosis reduction with no recurrence and improved or no diplopia. Partial success was considered if there was proptosis reduction without recurrence yet persistent or new-onset diplopia. Failure was considered if there was recurrence of proptosis regardless of diplopia. The patient’s quality of life was also evaluated as a long-term outcome.ResultsAfter follow-up for 37.9±24.4 months, 1365 eyes (85.1%) achieved complete success, 219 eyes (13.7%) achieved partial success and 20 eyes (1.2%) had failure. Newly onset diplopia and secondary decompression occurrence rate remained low at 3.3% and 0.6%, respectively. The total proptosis reduction was 4.1±1.3 mm, which was consistent all through the intermediate and long-term (5–10 years) follow-up. The amount of orbital fat removal (4.5±1.1 mL) played a significant role in the long-term Hertel change. Importantly, the overall quality of life increased significantly for GO patients after undergoing orbital fat decompression.ConclusionsOrbital fat decompression has a long-term efficacy in correcting disfiguring proptosis with a low complication rate and without the need of secondary decompression procedures. This was also associated with a significant improvement in quality of life.

Purpose To investigate the use of fascia lata (FL) grafts for inferior rectus muscle (IRM) tendon elongation in patients with large vertical squint angles with Graves’ orbitopathy (GO). Methods In this retrospective study, we included a consecutive series of 20 eyes of 13 patients with GO who underwent IRM tendon elongation with FL. Orthoptic and ophthalmologic examinations including measurement of the head posture, the extent of deviation in primary position (PP), elevation, motility, and binocular diplopia at the tangent of Harms were conducted preoperatively and after a mean postoperative time of 10.8 (5.0–35.0) months in all patients. Results The mean total repositioning distance was 9.3 ± 3.6 (3.5–16.0) mm. Postoperatively, we found a significant increase in elevation (5.4 ± 2.4 vs. 2.7 ± 2.4 mm preoperatively, p  = 0.011). A significant reduction in vertical squint angle (2.8 ± 3.7 vs. 20.2 ± 18.8 Δ preoperatively, p  = 0.004), chin elevation (2.3 ± 3.7 vs. 12.9 ± 6.3° preoperatively, p  < 0.001), extorsion in PP (0.1 ± 3.8 vs. 8.4 ± 7.8° preoperatively, p  = 0.002), and in elevation (1.8 ± 4.8 vs. 11.1 ± 10.9° preoperatively, p  = 0.004) occurred postoperatively. A mean dose–effect relation of 2.6 ± 2.9 Δ/mm was calculated. Postoperatively, the lower eyelid retraction was significantly increased (1.5 ± 1.4 vs. 0.4 ± 0.5 mm preoperatively, p  = 0.005). Conclusion IRM tendon elongation with FL is a feasible and effective procedure without relevant risk for surgery-related complications.

Pretibial myxedema, more generally thyroid dermopathy, results from mucopolysaccharide accumulation in the dermis, typically between the knee and dorsal foot. Thyroid dermopathy presents in Graves disease, but can occur in Hashimoto thyroiditis, primary hypothyroidism, and euthyroid patients. Treatment of thyroid eye disease with teprotumumab is established in the literature, with few case reports also showing improvement in pretibial myxedema. Reported is a 76-year-old man with thyroid eye disease and pretibial myxedema treated with teprotumumab; improvement was demonstrated in both conditions. He developed \"muffled\" hearing as an adverse effect, a complication not widely published in the dermatology literature. At 18 months post-treatment, his symptoms are stable without recurrence, but hypoacusis persists. Given the long-term efficacy and side-effects, dermatologists should recognize the potential benefits and risks of using teprotumumab for thyroid dermopathy. A baseline audiogram may be considered prior to therapy. Additionally, longitudinal data is needed to document the benefits and risks of this novel therapy.

Background Lacrimal gland (LG) involvement in patients with Graves ophthalmopathy (GO) has been considered as a potential cause of the associated GO symptoms and different studies demonstrated the LG involvement in patients with GO than healthy controls. The aim of this study was to evaluate LG involvement, through measurement of its herniation, using a magnetic resonance imaging (MRI) index, in patients with different GO activities. Methods Thirty-two consecutive Caucasian patients affected by GO were enrolled and grouped in group A (16 with inactive GO, CAS < 3) and B (16 with active GO, CAS ≥ 3) according to their GO activity. All patients underwent clinical-endocrinological assessment, a complete ocular evaluation, and orbital MRI examination. Results No difference was found between the hormonal parameters, thyroid ultrasound-derived parameters, and thyroid-stimulating hormone (TSH) receptor (TSH-R) antibodies (TRAb) levels in group B and those in group A. The LG herniation (LGH) measurement evaluated by MRI was significantly higher in group B for both right (10.1 (7.3–17) vs. 7 (0–3.4) mm; p = 0.004) and left (8.5 (6.6–13) vs. 5.8 (0–12) mm; p = 0.026) eye than group A. A positive correlation was found between TRAb and LGH herniation (Rho 0.462, p = 0.009). Conclusions Measurement of LGH seems to be a good marker of the disease and GO activity. Key Points • Lacrimal gland herniation is a simple index related to disease activity • Lacrimal gland herniation is correlated to TRAb levels • Lacrimal gland evaluation could be useful to differentiate active from inactive Graves ophthalmopathy in an early stage of disease

Purpose To analyse risk factors for the development of superior limbic keratoconjunctivitis (SLK) in thyroid eye disease (TED). Methods This prospective, observational study included 638 eyes/sides from 319 patients with TED. The eyes were classified into two groups, based on the presence and absence of SLK. Multivariate logistic regression analysis was performed to evaluate potential risk factors, including sex, patient age, past treatment history (steroid, orbital radiotherapy and radioiodine therapy), smoking, clinical activity score, margin reflex distance (MRD)-1 and -2, Graefe sign/lid lag, Hertel exophthalmometric results, Schirmer’s test results, tear break-up time (TBUT) and tear meniscus height (TMH). Results SLK was found in 198 eyes (31.0%) from 121 patients. Young age (OR, 0.977; P  = 0.006), smoker (OR, 1.785; P  = 0.009), presence of Graefe sign (OR, 2.912; P  < 0.001), absence of lid lag (OR, 0.485; P  = 0.031), high Hertel exophthalmometric values (OR, 1.125; P  = 0.002), shorter Schirmer’s test results (OR, 0.962; P  < 0.001), shorter TBUT (OR, 0.815; P  = 0.002) and high upper TMH (OR, 1.003; P  = 0.013) were associated with the development of SLK. A high MRD-1 measurement value also tended to be associated with a risk of SLK, with an OR of 1.187 ( P  = 0.056). Conclusion The present study proposed several risk factors in relation to the development of SLK in TED.

Objective To evaluate the ability of quantitative MRI parameters for predicting dysthyroid optic neuropathy (DON). Methods We retrospectively collected and analyzed the clinical features and 3.0 T MRI data of 59 patients with Graves orbitopathy (GO), with ( n  = 26) and without DON ( n  = 33). We compared MRI quantitative parameters, including the modified muscle index (mMI), proptosis, volume of intra-orbital fat, mean apparent diffusion coefficient value, and T2 value of the optic nerve among patients with and without DON. A logistic regression analysis was performed to identify the risk factors associated with DON. Moreover, we performed a receiver operating characteristic curve analysis and decision curve analysis to evaluate the diagnostic performance of the identified parameters for DON. Results We studied 118 orbits (43 and 75 with and without DON, respectively). The mMI and mean T2 value of the optic nerve were significantly greater in orbits with DON ( p  < 0.001). A greater mMI at 21 mm (odds ratio (OR), 1.039; 95% confidence interval (CI): 1.019, 1.058) and higher mean T2 value of the optic nerve (OR, 1.035; 95% CI: 1.017, 1.054) were associated with a higher risk of DON. A model combining the mMI at 21 mm and mean T2 values for the optic nerve effectively predicted DON in patients with GO, with a sensitivity and specificity of 95.3% and 76%, respectively. Conclusion A quantitative MRI parameter combining the mMI at 21 mm and mean T2 value of the optic nerve can be an effective imaging marker for identifying DON. Key Points • Patients with GO and DON had greater mMI than those without DON. • Optic nerves in patients with DON demonstrated an increased T2 value. • The quantitative MRI parameter combining the mMI at 21 mm and mean T2 value of the optic nerve is the most effective method for diagnosing DON.

IntroductionDiagnosing ocular myasthenia gravis (MG) can be challenging because serum antibodies are often not detected. We aimed to explore whether determining extraocular muscle (EOM) weakness using orthoptic measures, including an adapted Hess chart examination, can aid in diagnosing MG.MethodsWe conducted a prospective study among patients with acetylcholine receptor antibody positive MG (20 recently diagnosed, 19 chronic) and 14 seronegative MG patients. We compared orthoptic measures to 19 healthy and 18 disease controls with Graves orbitopathy, chronic progressive external ophthalmoplegia or oculopharyngeal muscular dystrophy. Maximal eye duction angles were measured using a synoptophore. Gaze deviations between eyes were measured using standard Hess chart examination with addition of 1 min persistent gaze to assess MG-associated fatiguability. Receiver operating characteristics curve analysis was performed.ResultsFor duction angles, the area under the curve (AUC) was 0.73 comparing MG to healthy, and 0.69 comparing to patient controls. For the outer field of the Hess chart, the AUC was 0.89 comparing to healthy and 0.54 to patient controls. For drift, the AUC was 0.93 comparing to healthy and 0.93 to patient controls. The sensitivity and specificity of the presence of drift was 81% and 100%.DiscussionOrthoptic measurements can be used to diagnose MG by quantifying EOM weakness and fatiguability. Drift during persistent gaze on a Hess chart is specific for MG and could be used for diagnostic purposes. The Hess chart examination is widely available, inexpensive and fast. Moreover, orthoptic measurements may be a clinically relevant outcome measure for clinical trials.