Teprotumumab in thyroid eye disease.

Thyroid eye disease (TED) is an inflammatory condition involving the periocular and orbital soft tissues, affecting most commonly patients with hyperthyroid disorders. Traditional treatments used for the active phase of the disease range from conservative lubrication for mild symptoms to systemic immunomodulating drugs for moderate-to-severe symptoms. Teprotumumab (Tepezza) is a monoclonal antibody with an inhibitory effect on insulin-like growth factor 1 and is the first Food and Drug Administration (FDA) approved targeted medical therapy for reducing the inflammatory signs and symptoms associated with TED. Two large multicenter, randomized, double-masked, placebo-controlled trials have confirmed the efficacy and safety of teprotumumab in patients with active, moderate-to-severe TED. Recent reports and publications have also demonstrated the efficacy of teprotumumab in a wider range of patients. In this review, we summarize the clinical features and pathophysiology of TED, disease course, and traditional management methods. We further detail the development of teprotumumab, the founding studies that brought it to its FDA approval, adverse events profile, and ongoing as well as future investigations.

Effect of Tranexamic Acid on Intra- and Postoperative Bleeding in Eyelid Surgery: A Prospective, Randomized, Multicenter, Double-Masked, Control Trial.

PURPOSE: Pilot studies suggest that waiting 15 minutes after a subcutaneous tranexamic acid injection is associated with decreased intraoperative bleeding and postoperative ecchymosis in eyelid surgery. The outcomes of commencing eyelid surgery immediately after injection without a waiting period remain unexplored. METHODS: This prospective, randomized, multicenter, double-masked, controlled study examined bilateral symmetric upper and/or lower lid blepharoplasty or ptosis repair. Patients received tranexamic acid in 1 eyelid and control in the contralateral eyelid. The surgeon recorded the side with more intraoperative bleeding. Two masked graders evaluated periocular ecchymosis at postoperative day 0 and postoperative week 1 (POW 1) with a 5-point scale. At POW 1, patients reported subjective grading of bruising as increased on 1 side or similar on both sides. Results were analyzed with Wilcoxon signed-rank and sign tests. RESULTS: Of 130 patients, there was less eyelid ecchymosis on the tranexamic side at postoperative day 0 ( p = 0.001) and POW 1 ( p < 0.001). By surgery type, the 69 levator advancement surgeries had significantly less ecchymosis at postoperative day 0 ( p < 0.001) and POW 1 ( p = 0.001), while upper eyelid blepharoplasty, combined upper and lower lid blepharoplasty, and conjunctivomullerectomy trended toward significance. Of 68 patients reporting a POW 1 grading, 69% reported less bruising on the tranexamic side ( p < 0.001). Intraoperative bleeding was not significantly different between sides ( p = 0.930). CONCLUSIONS: Without a postinjection waiting period, subcutaneous tranexamic acid for eyelid surgery significantly decreased postoperative ecchymosis on postoperative day 0 and POW 1 but did not affect intraoperative bleeding. Subcutaneous tranexamic acid was not associated with any complications.

Perceptions and Understanding of Transgender Patient Care: A Survey of Practicing Facial Plastic Surgeons.

Transgender individuals face significant health disparities including deficiencies in physician education, knowledge, and comfort with care. The objective of this study was to determine the perceptions, practice patterns, and familiarity of facial plastic surgeons with transgender health care. An anonymous questionnaire was sent to facial plastic surgeons within the American Academy of Facial Plastic and Reconstructive Surgery list-serve. Primary datapoints included participant characteristics, transgender-related experience, and educational goals. Of the 66 facial plastic surgeons surveyed, 49% had treated 1 to 10 transgender patients during their career, and 70% were actively treating at least 1 transgender patient. The number of patients treated and surgeries performed was significantly associated with self-perceived competence, comfort counseling on gender-affirming surgeries, discussing gender identity, asking preferred pronouns, and a desire to learn more about transgender care. Most participants (61%) obtained transgender care training through real-world experience, with only 18% receiving formal training in residency or fellowship. In total, 50% of respondents believe transgender care training among facial plastic surgeons is inadequate and 60% support its incorporation into residency/fellowship curricula. Increased awareness is needed to address the disparities experienced by transgender patients. Many facial plastic surgeons desire to learn more and support incorporating transgender care into training. Understanding the current state of transgender care can assist the facial plastic community in promoting education that strengthens physicians' ability to deliver competent care that addresses the inequities faced by this diverse group.

Outcomes of Patients With Thyroid Eye Disease Partially Treated With Teprotumumab.

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.

Orbital Ependymoma.

Orbital ependymomas are rare in the orbit and usually occur secondary to extracerebral extension of an intraventricular ependymoma. The authors present a rare case of orbital ependymoma in a 74-year-old female. The patient was initially diagnosed with intraventricular ependymoma at the age of 13 years that required multiple repeat craniotomies for tumor recurrence. She then developed progressive tumor growth with extension into the bilateral frontal lobes and orbit. The orbital involvement produced binocular diplopia, epiphora, and globe distortion with compressive optic neuropathy. To the authors knowledge, this is the first such report in the English language ophthalmic literature.

Metastatic Breast Cancer Presenting as Progressive Ophthalmoplegia 30 Years After Initial Cancer Diagnosis.

ABSTRACT: A 60 year-old woman presented with painless progressive ophthalmoplegia of the right eye. She had a history of left-sided breast carcinoma 30 years ago that was managed with mastectomy only, with appropriate serial follow-up investigations. On examination, her visual acuity was 20/400 in the right eye and 20/20 in the left. She had a right relative afferent pupillary defect. Ocular examination was significant for 2 mm of ptosis, complete ophthalmoplegia, and 2+ chemosis in the right eye. The left eye was normal. MRI of the brain and orbits showed bilateral retrobulbar infiltrative disease in the right eye greater than that in the left eye. Right orbitotomy and biopsy confirmed an infiltrative signet ring cell/histiocytoid carcinoma consistent with metastatic lobular breast carcinoma. Given that recurrence of breast cancer is most common during the second year after the initial disease and rarely reported beyond 20 years after the initial diagnosis, our patient's delayed recurrence 30 years after the treatment of initial disease is unusual and rare. However, it highlights the importance of including metastatic cancer in the differential diagnosis for ophthalmoplegia.

Outcomes of optic nerve sheath fenestration from superomedial eyelid crease approach.

PURPOSE: The purpose of this study is to report the safety and outcomes of optic nerve sheath fenestration (ONSF) performed via superomedial eyelid crease approach. METHODS: A retrospective chart review was performed on patients undergoing superomedial eyelid crease ONSF at a single institution between 2014-2019. Data obtained included preoperative visual acuity (VA), mean deviation (MD) on visual field (VF), papilledema grade, intraoperative time, estimated blood loss, intraoperative complications and optic nerve sheath biopsy results. Postoperative month 6 (POM6) data collected included VA, MD, papilledema grade, and lid contour. Outcome measures included overall change in VA, MD, and papilledema grade at POM6. Statistical analysis was performed using STATA version 16 statistical software. Data analysis was done comparing all patients pre- and postoperatively. Additional subgroup analysis was performed on patients undergoing ONSF for IIH vs other indications, and on the contralateral unoperated eye. RESULTS: A total of 31 eyes of 24 patients were identified, four of whom were excluded due to being lost to follow-up. At POM6, improvement was seen in VA (p<0.001) across all groups. There was improvement in POM6 median papilledema grade and MD, but these did not reach statistical significance. Additional subgroup analysis on patients with IIH showed statistically significant improvement in POM 6 VA (p=0.009), papilledema (p=0.009) and MD (p<0.001), as well as VA improvement in the contralateral unoperated eye (p<0.001). CONCLUSIONS: Superomedial eyelid crease approach for ONSF is a safe and effective surgery in IIH and other conditions of excess pressure within the optic nerve sheath.

Is Telemedicine our cup of tea? A nationwide cross-sectional survey regarding doctors' experience and perceptions.

OBJECTIVES: To evaluate the experience and perceptions regarding Telemedicine and the perceived barriers among medical doctors. METHODS: This cross-sectional survey was carried out by enrolling practicing doctors of Pakistan with experience of ≥6 months by sending a validated and piloted questionnaire through email. Data collection was done from 10th October to 9th November 2020 after taking ethical approval from the concerned authorities. Data was analysed using SPSS v. 19.0. RESULTS: Two-hundred-forty responses were received with a response rate of 63%. Female participants (62.8%) were in majority and most of the participants were working in urban (88.5%) or semi-urban (9%) locality in either teaching (35.9%) or tertiary care hospitals (34.6%). Seventy-three percent of the doctors didn't receive formal training with more than half of the doctors reporting non-availability of infrastructure and specific hardware. A large number of the participants were concerned regarding the non-availability of regulatory bodies, evaluations and accreditations of the service providers, the risks of malpractice, missed-diagnosis, prescription errors and medico-legal issues. The availability of specific infrastructure was statistically related to the hospital setup, locality and the specialty of the participants. Lack of technological literacy and infrastructure were considered the main constraints for the public in using telemedicine. CONCLUSION: Evidence of effectiveness of telemedicine across different fields is inconsistent and lacks technical, legal, cultural and ethical considerations. Inadequate training, low level of technological literacy and lack of infrastructure are the main barriers in implementing tele-health. High-quality evidence based studies are required for practical and long-term policies.

Orbital Compartment Syndrome After High-speed Air-Gasoline Blast Injury.

Orbital compartment syndrome is an ophthalmologic emergency that requires timely surgical intervention. The authors present a rare case of orbital compartment syndrome in a 30-year-old male injured by forceful entry of air-gasoline mixture into the orbit, secondary to inadvertent firing of the piston from running mechanical diagnostics on an automobile internal combustion engine. Orbital CT revealed extensive orbital emphysema with both pre- and postseptal involvement and diffuse chemical cellulitis. Serial exams revealed rapid deterioration of vision with elevated intraocular pressure and development of eyelid, corneal, and orbital edema; a relative afferent pupillary defect and optic nerve hypoperfusion. He was started on intravenous steroids and underwent an emergent lateral canthotomy with cantholysis, which temporarily reduced the intraocular pressure. However, a second rapid increase in soft tissue swelling resulted in another episode of ocular hypertension and compressive optic neuropathy, requiring emergent orbital bony decompression, which was followed by decreased intraocular and orbital pressure. The patient later developed progressive corneal opacification indicating delayed chemical injury. This was managed with a 10-day course of aggressive topical and systemic antiinflammatory agents with significant improvement in visual acuity. At last follow up, the vision was 20/30 and the corneal and eyelid edema had cleared.

Transient vision loss after optic nerve sheath fenestration.

Optic nerve sheath fenestration (ONSF) is a well-known, relatively safe, and effective surgical treatment for visual loss related to papilledema. Visual loss following ONSF is uncommon but can occur from direct surgical trauma, ischemic optic neuropathy, orbital or intrasheath hemorrhage, or arterial occlusion. Transient severe (e.g., light perception (LP) or no light perception (NLP) visual loss) after ONSF is uncommon but has been reported. We describe a case of LP vision following uncomplicated ONSF with orbital imaging demonstrating significant postoperative inflammation and slow recovery after intravenous steroid treatment. We discuss the various mechanisms for this phenomenon after ONSF. To our knowledge, this is the first clinico-radiologic description with follow up and recovery of this postoperative complication of ONSF to be reported in the English language ophthalmic literature.

Vitamin D and Selenium in a Thyroid Eye Disease Population in Texas.

There is growing evidence of thyroid eye disease association with nutritional deficiencies including selenium and vitamin D. We conducted a retrospective chart review of all patients with clinical diagnosis of TED seen at our clinic from 2016 to 2017. Thirty-five patients met inclusion criteria and had serum 25-hydroxyvitamin D levels available, and 19 had selenium levels available. 7/35 (20%) patients had vitamin D deficiency, and 11 (31%) had vitamin D insufficiency, but none had selenium deficiency. Although both selenium and vitamin D supplementation have been recommended for TED, further investigation is necessary to justify supplementation for patients with TED.

Surgical site infection surveillance following total knee arthroplasty: Tertiary care hospital experience.

INTRODUCTION: Surgical Site Infection (SSI) after knee arthroplasty is a major cause of morbidity and mortality that increases the hospital stay, financial burden and mental anguish of the patient. Infection Control Unit at Aga Khan University Hospital (AKUH) incorporated total knee arthroplasty in its surgical care surveillance program and started collecting data in June 2012. The purpose of this study is to review Surgical Site Infection (SSI) rates in patients undergoing primary total knee replacement (TKR) surgery. PATIENTS AND METHODOLOGY: All patients from June 2012 to December 2013 undergoing knee arthroplasty at our hospital were included. Data was acquired from the hospital SSI database for knee arthroplasty surgery. Data was collected by SSI nurses for inpatients a well as post-discharge monitoring in clinics till 90 days post-op follow-up. The work has been reported in line with the PROCESS criteria. RESULTS: During this time period a total of 164 patients had primary TKR at AKUH. Out of these, 85 patients (52%) had bilateral TKR while 79 (48%) had unilateral TKR. The overall SSI was in 2 patients (1.2%). CONCLUSION: Identifying SSIs is multidimensional. Since our 2 infected cases after TKR occurred after discharge, this highlights the importance of post-discharge surveillance and not limiting the surveillance for inpatients only. Furthermore, the SSI program may be effective in controlling postoperative wound infections.