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.

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.

Orbital Inflammatory Syndrome Secondary to Flea Bite.

A 34-year-old previously healthy Hispanic male presented to the emergency room complaining of progressive left upper eyelid swelling and pain for more than 2 weeks. He was previously diagnosed and treated for a "pink eye" but failed to improve. He reported a previous "bug bite" around the left lateral canthus a few weeks prior to admission. Computer tomography orbit with contrast showed left exophthalmos, an enhancing left lacrimal gland and orbital inflammatory signs suggestive of possible intraorbital abscess. Intravenous antibiotics did not improve his symptoms. Surgical debridement showed no abscess but inflamed soft tissues and lacrimal gland. Intravenous steroids failed to improve his symptoms. On postoperative day 3, the patient reported that an insect had "jumped" out from his left orbit. Identification of the specimen proved to be a mature flea. Biopsy of the lacrimal gland showed degranulation of eosinophils and foreign body material consistent with probable insect leg parts.

Idiopathic Orbital Inflammation Associated With Relapsing Polychondritis.

The authors describe a patient with recurrent idiopathic orbital inflammatory disease as an unusual presentation of relapsing polychondritis. There are very few reported cases in the literature of relapsing polychondritis associated with idiopathic orbital inflammation. Clinicians should be aware of the orbital and ophthalmic presentations of relapsing polychondritis.

Optic Neuropathy Due to Chronic Lymphocytic Leukemia Proven With Optic Nerve Sheath Biopsy.

Central nervous system involvement from chronic lymphocytic leukemia (CLL) occurs infrequently, and manifestations include cognitive and cerebellar dysfunction and cranial nerve palsies. We report a 45-year-old man with CLL believed to be in clinical remission, who presented with vision loss and bilateral optic disc edema. His optic neuropathy due to CLL was proven by optic nerve sheath biopsy, and he experienced visual recovery after treatment with ibrutinib and intrathecal methotrexate.

Anatomical and visual results of transconjunctival sutureless vitrectomy using subconjunctival anesthesia performed on select patients taking anticoagulant and antiplatelet agents.

PURPOSE: The purpose of this study was to report the observed outcomes of anticoagulated patients undergoing transconjunctival sutureless vitrectomies using subconjunctival anesthesia without preoperative cessation of anticoagulation and antiplatelet (AC/AP) agents. METHODS: A retrospective chart review was performed on patients taking AC/AP agents who were undergoing transconjunctival sutureless vitrectomies with subconjunctival anesthesia between January 2007 and June 2009. Intra- and postoperative complications (such as massive hemorrhage), anatomical results, satisfactory analgesia (informed by patients and recorded by surgeon), anatomical results, and visual acuity were documented. RESULTS: Sixty-three surgeries were performed on 56 eyes of 52 patients. Thirty-two patients were taking aspirin and clopidogrel, 18 were taking warfarin, 5 were taking clopidogrel, 3 were taking aspirin and warfarin, 2 were taking acetylsalicylic acid and dipyridamole, and 1 was taking warfarin and clopidogrel. All patients had satisfactory analgesia. No intraoperative complications occurred. Seven eyes (13%) had postoperative vitreous hemorrhage. Overall, 79% of patients had improved postoperative visual acuity, 16% of patients had unchanged visual acuity, and 5% of patients had worse visual acuity at last follow-up visit. CONCLUSION: Medical disease requiring systemic AC/AP therapy is often present in patients with vitreoretinal disease. Transconjunctival sutureless vitrectomies using subconjunctival anesthesia may be a safe and effective surgical option in select patients in whom cessation of AC/AP and/or delayed ophthalmic intervention is imprudent.

Interval to treatment of sexually transmitted infections in adolescent females.

STUDY OBJECTIVE: To describe (1) the treatment interval for adolescent females with Chlamydia trachomatis (CT), Neisseria gonorrhoeae (GC), or Trichomonas vaginalis (TV); (2) the proportion treated in < or =7 days; and (3) factors influencing the treatment interval. DESIGN AND PARTICIPANTS: Charts of sexually active females from an urban teen health center who participated in a larger study and were positive for CT, GC or TV (N = 58) were retrospectively reviewed for dates of treatment, and compared to demographic and symptom data. The treatment interval was defined as days from visit to treatment. CT and/or GC were analyzed together (CT/GC) because presumptive treatment covered both infections, and the diagnostic test (nucleic acid amplification) differed from that of TV (wet mount or culture). RESULTS: The median treatment interval was 0 days for TV, 5 days for CT/GC, and 3 days for any STI. Overall, 39 (69%) were treated within 7 days of their visit. Those with TV were more likely than those with CT/GC to receive treatment at their initial visit (58% vs. 6%). Genitourinary symptoms increased the odds of treatment in < or =7 days. The treatment interval was significantly shorter for subjects who had their prescriptions phoned to a pharmacy than for those who returned to clinic for treatment (median 2.5 vs. 8 days). CONCLUSIONS: Where presumptive treatment was uncommon, providers were more likely to prescribe same-day therapy to symptomatic patients or those with TV on wet mount. Additional strategies are needed to improve the proportion of adolescent females treated in < or =7 days.