Clinical phenotypes of euthyroid, hyperthyroid, and hypothyroid thyroid-associated ophthalmopathy.

OBJECTIVE: To compare the demographic and clinical phenotypes of thyroid-associated ophthalmopathy (TAO) with euthyroidism (Eu-TAO), hyperthyroidism (Hr-TAO), and hypothyroidism (Ho-TAO). METHODS: We enrolled 2158 TAO patients in this retrospective study and assessed their demographics, clinical manifestations, activity, and severity. RESULTS: Among the enrolled patients, 526 (24.37%) had Eu-TAO, 1544 (71.55%) had Hr-TAO, and 88 (4.08%) had Ho-TAO. Compared to Hr-TAO (2.02) and Ho-TAO (2.52) patients, Eu-TAO (1.57) patients had the lowest female-to-male ratio (p = 0.026). The mean ages of Eu-TAO, Hr-TAO, and Ho-TAO patients were 43.11 ± 12.05, 42.23 ± 13.63, and 47.39 ± 13.28 years, respectively (p = 0.001). Patients with Eu-TAO had more unilateral involvement (50% vs. 14.38% vs. 21.59%, p < 0.001) than Hr-TAO or Ho-TAO patients. Clinically active TAO patients presented 8.56% in euthyroid vs. 13.86% in hyperthyroid vs. 11.36% in hypothyroid (p = 0.006). Regarding the severity of the European Group on Graves' Orbitopathy (EUGOGO) classification among euthyroid, hyperthyroid, and hypothyroid patients, mild TAO was present in 67.68, 54.27, and 72.72% of participants, moderate-to-severe TAO in 31.18, 42.49, and 26.14%, and sight-threatening TAO in 1.14, 3.24, and 1.14%, respectively. Eu-TAO was positively correlated with unilateral involvement (OR = 5.671, p < 0.001) and age (OR = 1.013, p = 0.003) and negatively correlated with the female-to-male ratio (OR = 0.656, p < 0.001) and TAO severity (OR = 0.742, p < 0.01). CONCLUSIONS: Eu-TAO patients are older and less likely to be female, and show more unilateral and milder clinical phenotypes than hyper/hypothyroid TAO patients.

[Clinical Analysis of 2 170 Cases of Thyroid-Associated Ophthalmopathy Involving Extraocular Muscles].

OBJECTIVE: To explore the clinical features of thyroid-associated ophthalmopathy (TAO) with extraocular muscle involvement. METHODS: The data of 2170 TAO patients who were seen at the Orbital Disease Clinic, West China Hospital, Sichuan University from September, 2009 to January, 2020 were collected retrospectively. The extraocular muscle involvement of these patients was confirmed by CT or MRI. Their general condition, medical history, clinical manifestations and imaging features were analyzed retrospectively. RESULTS: Among the 2170 TAO patients, 932 were male and 1238 were female. The mean (± SD) age of all the patients was (46.95±13.06) years, ranging between 6 and 85. 1684 patients (77.60%) suffered from hyperthyroidism, 13 patients (0.59%) had thyroid cancer, 80 patients (3.69%) had hypothyroidism, and 393 patients (18.11%) had normal thyroid function. Proptosis (55.25%) and diplopia (33.09%) were the main reasons for their visits to the clinic, and restricted eye movements (83.46%) was the most common sign. 122 patients with a mean age of (53.24±13.07) years did not show any eyelid sign and had only extraocular muscle involvement. The 2170 TAO patients had a total of 3799 eyes of extrocular muscle involvement, with 541 patients experiencing monocular involvement and 1629 patients, binocular involvement; 1204 eyes (31.69%) had a single extrocular muscle involved and 2595 eyes (68.31%) had multiple extrocular muscles involved. Inferior rectus was the most commonly involved muscle, followed by superior rectus, medial rectus, and lateral rectus in descending order of involvement frequency. Of the 1014 patients who underwent enhanced MRI, 71.99% were shown to be in the active phase. 69.03% of the 775 patients identified as being in inactive phase according to their clinical activity score (CAS) were shown to be in the active phase according to their MRI results. CONCLUSION: TAO patients with extraocular muscle involvement have their own specific clinical manifestations. CT and MRI can both be used to assist in the diagnosis of extraocular muscle involvement. MRI can be used to assess the pathological stage of extraocular muscles and is more sensitive than CAS.

Clinical features and clinical course of thyroid-associated ophthalmopathy: a case series of 3620 Chinese cases.

OBJECTIVES: To determine the clinical features and course of thyroid-associated ophthalmopathy (TAO) in a large sample of Chinese patients. DESIGN AND METHODS: We retrospectively identified a cohort of consecutive patients diagnosed with TAO at the West China Hospital from October 1, 2009 to October 1, 2019. We analysed clinical data from 3620 patients, including demographic data, clinical manifestations, ophthalmology examinations, and prognosis. RESULTS: TAO most frequently occurred with hyperthyroidism, with most patients developing TAO after thyroid disease (TD). The TAO phenotype was asymmetric in 375 (50.7%) euthyroid patients, 25 (27.8%) hypothyroid patients, and 314 (12.1%) hyperthyroid patients (p < 0.0001). The most frequent symptom was lid lag and the most commonly involved extraocular muscle was the inferior rectus. Severity assessment (NOSPECS score) and clinical activity assessment (Clinical Activity Scores, CAS) differed significantly between male and female patients (P < 0.000). The majority (88.8%) of patients had clinically inactive TAO, and only 3.2% of cases were sight-threatening. Regarding the clinical process, 75.5% of patients had an active phase time less than 12 months and 2.1% showed complete remission. CONCLUSIONS: TAO most commonly develops in females and is closely related to hyperthyroidism. Euthyroid TAO often has an asymmetric clinical phenotype. CAS combined with magnetic resonance imaging can improve the detection of TAO. NOSPECS scores should be slightly refined regarding the criteria for corneal involvement. Clinical management of TAO should be individualized according to CAS or NOSPECS assessments and a multidisciplinary approach is paramount. A minority of patients showed complete remission.

Peribulbar injection of glucocorticoids for thyroid-associated ophthalmopathy and factors affecting therapeutic effectiveness: A retrospective cohort study of 386 cases.

Thyroid-associated ophthalmopathy (TAO) is common in Graves' disease. However, to date, no standard treatment has been established for TAO. The present study aimed to assess peribulbar injection of corticosteroids for TAO treatment as well as factors affecting therapeutic effectiveness. A retrospective cohort study was performed at West China Hospital, Sichuan University (Chengdu, China). Patients with TAO were administered peribulbar injection of triamcinolone acetonide and dexamethasone monthly. Ocular signs after each injection were assessed until the end of treatment. All patients were followed up for at least six months. Best corrected visual acuity, proptosis values, eye motility assessed by the Hess chart, as well as eyelid width and downward movement were determined. In addition, clinical data were retrospectively reviewed to explore factors affecting therapeutic effectiveness by logistic regression analysis. In the present study, 386 patients with TAO (515 eyes) were evaluated; 71.37% of cases of eyelid swelling were relieved and upper eyelid retraction was improved in 47.58% of affected patients. Eye movement disorders, diplopia and strabismus were all alleviated to varying degrees, with few adverse reactions. Logistic regression analysis demonstrated that therapeutic effectiveness was relatively lower in males [odds ratio (OR)=0.32, P=0.001] and patients with thyroid dysfunction (OR=0.41, P=0.002), and that non-smokers had a higher odds of substantial improvement (OR=4.62, P=0.008). The duration of TAO was not significantly associated with the clinical outcome. Patients with reduced disease severity and elevated clinical activity score exhibited higher effectiveness (all P<0.05). In conclusion, peribulbar injection of corticosteroids is effective in treating mild to moderate TAO, with the therapeutic response affected by gender, smoking and disease severity.

Multiple recurrent myxofibrosarcoma of the orbit: case report and review of the literature.

BACKGROUND: Although myxofibrosarcoma (MFS) is the most common mesenchymal tumor, occurrence in the orbit is extremely rare. A characteristic clinical feature of MFS is its propensity for local recurrence (LR). Still, none of published literature has described the entire clinical course of multiple recurrences of MFS in the orbit. Here we present an unusual case in which a patient with multiple recurrences of MFS in the orbit followed-up for 5 years. We describe its clinical-pathological correlation, treatment, and prognosis. CASE PRESENTATION: A 70-year-old woman sought treatment for a year history of right upper eyelid swelling in May 2014. Since then, she underwent three surgical procedures in the right orbit in the same region (in 2014, 2016, and 2017). The pathology analysis of the three surgical tissue samples was consistent with myxofibrosarcoma, characterized by spindle-shaped cells and variably myxoid stroma. In the 2 years follow-up after the third surgery, there was no evidence of tumor recurrence. CONCLUSION: Myxofibrosarcoma of the orbit is extremely rare. Since few reports are available in the literature, the diagnosis and management of the orbit myxofibrosarcoma is still a challenge to ophthalmologists. Histopathology is highly valuable in the diagnosis. As a clinical characteristic, the lesions have a high risk of local recurrence; therefore, aggressive resection and careful postoperative follow-up are paramount.

Paediatric orbital alveolar soft part sarcoma recurrence during long-term follow-up: a report of 3 cases and a review of the literature.

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a clinically and morphologically distinct malignant soft tissue tumour. It occurs mostly in the lower extremities in adults. The purpose of our study was to describe the related clinicopathologic factors, treatment and prognosis of recurrent orbital ASPS in children. CASE PRESENTATION: Three children aged from 1 to 12 years presented with unilateral proptosis, restricted ocular motility and impaired visual acuity of the affected eye. Periodic acid Schiff (PAS) -positive diastase-resistant crystalline granules were found in all cases. Immunostaining of TFE-3, INL1 and Ki67 was positive in the tumour cells of two patients. The time until local recurrence of primary tumor in patient 2 and patient 3, who only underwent tumour resection, was shorter than that of patient 1, who underwent tumour excision and postoperative radiotherapy. The recurrent masses were successfully treated with complete tumour excision followed by adjuvant radiotherapy. Patient 1 presented metastasis at 11 years after radiotherapy. CONCLUSIONS: Orbital ASPS in children is easily misdiagnosed due to its rare occurrence and atypical clinical findings. Early diagnosis with multidisciplinary, complete surgical resection combined with adjuvant radiotherapy is essential for achieving long-term disease-free survival in orbital ASPS patients.