Purpose: To describe the clinical course in a heterogeneous series of subjects with thyroid eye disease (TED) treated with teprotumumab. Methods: Cross-sectional cohort study including patients with clinical diagnosis of TED who was treated with teprotumumab. The entire cohort was analyzed together and subsequently in clinical subgroups based on stage and grade of disease. Primary outcome measure was change in proptosis ≥2 mm. Secondary outcome measures included change in clinical activity score (CAS), ductions, strabismic deviation, MRD1, and MRD2. Bivariate and multivariate statistics were performed. Results: The study included 21 patients. Mean ± SD age was 61.5 ± 12.6 years and 71.4% were female. Reduction in proptosis ≥2 mm was achieved in 71.4% of the sample. Stage and grade were not significant predictors of outcome. Treatment with teprotumumab resulted in a 2.5 ± 1.8 mm reduction of proptosis (P < 0.001), 2.2 ± 1.4 reduction in CAS (P < 0.001), and 16.9 ± 19.3 degree improvement in extraocular motility (P < 0.001). There were no significant differences for change in CAS, proptosis, ductions, or MRD2 between different grades and stages of disease. Total strabismus and MRD1 improvement were greater in the active stage of disease (P < 0.05). Three cases of dysthyroid optic neuropathy, refractory to methylprednisolone therapy improved after initiation of teprotumumab. Conclusions: Treatment of TED with teprotumumab in a heterogeneous patient population is associated with improvement in proptosis, extraocular motility, and CAS. Patients beyond those defined in the clinical trials, including those affected by stable stage, milder grade, and vision-threatening TED may benefit from this therapy. There are, however, limits on the overall efficacy of this medication in the management of certain physical characteristics in TED including eyelid position and strabismus.

The systematic classification of vascular disease as proposed and refined by the International Society for the Study of Vascular Anomalies (ISSVA) divides vascular pathology first into tumors and malformations. Malformations are described as simple and complex, where simple malformations contain a single vascular system and complex malformations comprised of multiple vascular systems. Arteriovenous malformations are considered in terms of inflow characteristics which are primarily responsible for the key management challenges. Management utilizing endovascular embolization and/or surgical resection is often employed; however, recurrence can occur, particularly in diffuse cases. There may be an increasing role for systemic antiangiogenic therapy in such cases. Lymphaticovenous malformations are divided into the principle components on the lymphatic and venous sides for clarity of discussion. Lymphatic malformations are described morphologically as macrocystic and microcystic, and physiologically in terms of the processes responsible for growth. In both cases, surgical options are challenging and local therapeutics intended to close large luminal spaces in the case of macrocystic and to slow biological signaling for growth in microcystic. Venous malformations are described physiologically in terms of flow and distensibility, as volume plays a critical role in the limited space of the orbital cavity. Combined embolic-surgical approaches can be effective for management. More complicated, combined lesions can be managed by dividing the lesion into principal components and treating each appropriately.

Purpose: In response to the coronavirus (COVID-19) pandemic, teprotumumab production was temporarily halted with resources diverted toward vaccine production. Many patients who initiated treatment with teprotumumab for thyroid eye disease were forced to deviate from the standard protocol. This study investigates the response of teprotumumab when patients receive fewer than the standard 8-dose regimen. Methods: This observational cross-sectional cohort study included patients from 15 institutions with active or minimal to no clinical activity thyroid eye disease treated with the standard teprotumumab infusion protocol. Patients were included if they had completed at least 1 teprotumumab infusion and had not yet completed all 8 planned infusions. Data were collected before teprotumumab initiation, within 3 weeks of last dose before interruption, and at the visit before teprotumumab reinitiation. The primary outcome measure was reduction in proptosis more than 2 mm. Secondary outcome measures included change in clinical activity score (CAS), extraocular motility restriction, margin reflex distance-1 (MRD1), and reported adverse events. Results: The study included 74 patients. Mean age was 57.8 years, and 77% were female. There were 62 active and 12 minimal to no clinical activity patients. Patients completed an average of 4.2 teprotumumab infusions before interruption. A significant mean reduction in proptosis (–2.9 mm in active and –2.8 mm in minimal to no clinical activity patients, P < 0.01) was noted and maintained during interruption. For active patients, a 3.4-point reduction in CAS (P < 0.01) and reduction in ocular motility restriction (P < 0.01) were maintained during interruption. Conclusions: Patients partially treated with teprotumumab achieve significant reduction in proptosis, CAS, and extraocular muscle restriction and maintain these improvements through the period of interruption.

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Purpose: To report the effect of frontalis linkage without intraoperative eyelid elevation for the management of myopathic ptosis. Methods: Retrospective analysis of 21 (42 eyelids) myopathic patients with bilateral ptosis who were operated between 1999 and 2017. All patients had orbicularis weakness and poor or absent Bell’s phenomenon. Surgery consisted of using an autogenous fascia sling to link the tarsal plate to the frontalis muscle without any degree of intraoperative eyelid elevation. The main outcome measures were margin reflex distance, brow height and degree of brow excursion and degree of lagophthalmos, and exposure keratitis. Results: After surgery, there were significant changes (p <0.0001) in both margin reflex distance and brow position. Mean margin reflex distance increased to 1.4 mm ± 1.34 DP and with full frontalis contraction, it reached 3.0 mm ± 1.73 DP, while mean brow position decreased 1.6 mm ± 1.59 SD, p < 0.0001. Postoperative lagophthalmos was not detected in 31 (74%) eyes. In the remaining 11 eyes (26%), lagophthalmos ranged from 1.2 to 5.2 mm (mean = 1.7 mm ± 0.74 DP). Mild inferior superficial keratitis was detected in 14 eyes (33.3%) of 7 patients only 3 of which had lagophthalmos. One patient needed additional surgery to correct unilateral eyelid retraction. Overall, 81.81% of the patients were pleased with the procedure. Conclusions: Myopathic ptosis can be alleviated with a minimal amount of lagophthalmos by just linking the tarsal plate to the frontalis muscle without lifting the eyelid margin intraoperatively.

Purpose: To evaluate the effect of orbital decompression on the upper eyelid contour. Methods: A paired cross-sectional analysis of the upper eyelid contour was performed for 103 eyes of 66 patients who underwent orbital decompression. A control group of 26 normal subjects was also included. The eyelid contour of all participants were measured with Bézier lines adjusted to the eyelid contour and 9 midpupil eyelid margin (MPD) distances from a horizontal line bisecting the pupil. One central, corresponding to the margin reflex distance (MRD 1), and 8 equally distributed medially and laterally at 20% of the interval between the lines. Patients were classified as with flare if the height of the most lateral MPD relative to the MRD 1 was above the upper limit of the controls. Results: Preoperatively 63 of the 103 contours were classified as flare + (F+). After decompression MRD1 showed a mean decrease of 0.4 mm and the location of the contour shifted 0.8 mm medially. These changes were not correlated with proptosis reduction. Orbital decompression decreased the lateral curvature of the contours especially for the F+ lids. In 40% of the F+ eyelids the flare sign disappeared after decompression. Conclusions: Orbital decompression affects the lateral eyelid contour and diminishes the amount of lateral eyelid retraction surgery necessary to correct the flare sign. In 40% of the patients, the eyelid contour is normalized with proptosis reduction only.

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