Manhattan Eye, Ear and Throat Hospital (MEETH) is a specialty hospital in New York City that was founded in 1869 and is currently located on the Upper East Side of Manhattan at 210 East 64th Street (between 2nd and 3rd Avenues). After 131 years as an independent entity, in 2000 MEETH affiliated with Lenox Hill Hospital, a 652-bed acute care hospital, established in New York City in 1857 and located at 77th Street (between Park and Lexington Avenues) in Manhattan. MEETH is recognized in medical circles for its long history of contributions in developing the fields of ophthalmology, otolaryngology and plastic surgery. MEETH provides thousands of patients a year with treatment in its ambulatory surgery facilities.
PURPOSE:To characterize and compare the clinical features, timing, and duration of thyroid eye disease relapse after teprotumumab in patients treated during the active versus stable phases of the disease. METHODS:A retrospective case series was conducted of 26 adult patients who experienced a return of thyroid eye disease symptoms after completing teprotumumab therapy between July 2021 and July 2024 at 2 academic centers. Patients were stratified by thyroid eye disease phase at treatment initiation (active vs. stable). Data included clinical history, treatment details, and relapse characteristics. Relapse was defined by worsening of proptosis or extraocular motility deficits after completion of treatment. Statistical comparisons were made using t tests and Fisher's exact test. RESULTS:Of 26 patients, 20 were treated during the active phase and 6 during the stable phase. Active phase patients relapsed sooner (mean 8.2 vs. 12.3 months) and experienced significantly longer relapse duration (mean 11.1 vs. 4.2 months, p = 0.000035). In the active phase group, relapse was associated with progression of clinical signs beyond pretreatment levels, including 1 case of new-onset optic neuropathy. In contrast, stable phase patients uniformly returned to pretreatment clinical baselines without further progression. Both groups had similar rates of adverse events. CONCLUSIONS:Thyroid eye disease relapse after teprotumumab differs by disease phase at initiation. Active phase patients experience earlier, more prolonged, and more severe relapses, suggesting teprotumumab acts as a disease modulator. Stable phase patients exhibit shorter, nonprogressive symptom return, which we would term "rebound"-suggesting a distinct mechanism. These findings underscore the need for phase-specific counseling, monitoring, and further investigation into potentially disparate mechanisms of action of teprotumumab in the acute and stable phases.
Nodular fasciitis (NF) is a benign, rapidly growing myofibroblastic proliferation that can closely mimic sarcoma clinically, radiographically, and histologically, posing a significant diagnostic challenge. We report a case of an 11-year-old boy presenting with a painless but progressively enlarging periorbital mass initially suspected to be a dermoid cyst. Although his ultrasound was reassuring, MRI showed an enhancing lesion concerning for neoplasm, prompting surgical excision. Histopathologic evaluation revealed spindle-cell proliferation within a myxoid stroma consistent with NF. However, the USP6 fluorescence in situ hybridization analysis was negative. Further genomic analysis identified a rare USP6–PAFAH1B1 fusion. This case highlights a diagnostically challenging subset of NF in which conventional USP6 fluorescence in situ hybridization testing may be negative, necessitating next-generation sequencing for a definitive diagnosis. Increased awareness of NF and its clinical, histologic, genetic, and radiographic presentation is essential to avoid misdiagnosis and to guide appropriate management.
Purpose:We aim to study a case of pathologic myopia with visible light OCT. Design:Case report. Subjects:We recruit 1 patient with pathologic myopia presenting with an acute lacquer crack with submacular hemorrhage (SMH) in the right eye and a chronic lacquer crack in the left eye. Methods:We acquire visible light OCT images with 1 μm axial resolution. Images are processed to depict spectral centroid shift, and spectral fitting is performed to determine oxygen saturation. Results are compared to clinical imaging. Main Outcome Measures:Visible light OCT images, spectral centroid shift (redshift), oximetry, and spectral fitting. Results:Departures from the moderate retinal pigment epithelium (RPE) redshift, characteristic of a healthy macula, were observed. In the acute phase, we observe a further RPE redshift correlating with SMH. Spectra were mostly consistent with oxyhemoglobin. In the chronic lacquer crack, we demonstrated focal Bruch's membrane (BM) changes in vivo with spectral evidence of an overlying RPE deficit (deficient red shift) and photoreceptor abnormalities. Conclusions:As visible light OCT technology advances, its application toward well-characterized human retinal pathology can clarify its utility. We describe the first case of visible light OCT applied to pathologic myopia, a primary RPE-BM-choriocapillaris interface disease. We confirm that extravascular hemoglobin can be subject to spectral fitting, and we quantify the oxygen saturation of acute SMH. We further show that structural changes in chronic lacquer cracks can be characterized with this new technology. Financial Disclosures:Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
