OBJECTIVE:To review the literature on the benefits and risks of systemic corticosteroids in the setting of orbital cellulitis. METHODS:A literature search was last performed in the PubMed database in August 2025. Articles were limited to original research in the English language that included at least 10 patients and specifically assessed outcomes in patients with orbital cellulitis who received systemic corticosteroids. The initial search yielded 104 articles; 9 articles were reviewed in full text and met all of the criteria for inclusion for this assessment. The panel methodologist assigned a level of evidence rating to each of the studies. RESULTS:One study was rated level I, 4 studies were rated level II, and 4 studies were rated level III. The studies included in this assessment used variable dosing patterns and durations of systemic corticosteroids. Some studies reported shorter lengths of hospitalization when these agents were used, but larger studies failed to replicate this benefit. Although patients who received corticosteroids experienced faster resolution of symptoms in the 2 studies that specifically assessed this metric, most studies reported equal rates of surgical intervention and hospital readmission among patients who did and did not receive it. Adverse events were rare and seemed to be self-limited at the doses used, although many studies did not specifically report these complications. CONCLUSIONS:The available evidence examining the effectiveness of systemic corticosteroids showed a lack of standardization, and many studies did not specifically report the rates of adverse events. Nevertheless, systemic corticosteroids may be safe in the setting of orbital cellulitis and may improve the rate of symptom resolution. The impact of corticosteroids on duration of hospitalization remains unclear. FINANCIAL DISCLOSURE(S):Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

BACKGROUND:Eosinophilic granuloma (EG) is a localized subtype of Langerhans cell histiocytosis (LCH) that involves infiltration of tissues by a clonal proliferation of Langerhans cells. EG is a rare tumor of the orbit and most commonly presents in childhood. This tumor is known to regress after minimal intervention (including biopsy), and management includes curettage and intralesional corticosteroids. The natural history of EG is not well understood, and true spontaneous regression of the lesion without intervention has rarely been described. OBSERVATIONS:Herein, the authors present a unique case of an adult patient with presumed orbitocranial EG with true spontaneous regression over a 4-month period. Biopsy of the residual mass revealed reactive changes and no evidence of neoplasia, consistent with resolved EG. LESSONS:Adult patients with EG may experience regression of the lesion without intervention. https://thejns.org/doi/10.3171/CASE25426.

PURPOSE:A previous study found high rates of proptosis regression after teprotumumab treatment for thyroid eye disease, though having been published relatively early in the teprotumumab era, it had gaps in follow-up frequency and duration. This study extends on the previously reported cohort to better understand teprotumumab's durability. METHODS:Retrospective analysis of all patients who completed all 8 teprotumumab infusions at 1 institution from January 1, 2020 to December 31, 2022. Primary outcomes were proptosis regression at most recent follow-up compared with immediately posttreatment and pretreatment. RESULTS:Of 113 patients, 150 (66.4%) eyes of 83 patients had regression at most recent follow-up (mean 20.4 months posttreatment) compared with immediately posttreatment, averaging 2.8 mm (range 0.5-10.0). Ninety-five eyes of 55 patients (42.0%) regressed by ≥2 mm (mean 3.8, range 2.0-10.0). Mean time to first documentation of regression was 10.2 months (range 2.7-39.0); 67.2% of eyes continued to worsen after initial regression. Compared with pretreatment, 137 eyes of 77 patients (60.6%) had net improvement, averaging 2.7 mm (range 0.5-10.0), while 71 eyes of 43 patients (31.4%) regressed to become worse than pretreatment by 1.8 mm (range 0.5-5.0). Thyroid eye disease flare occurred in 61 (54.0%) patients, defined as a clinical activity score increase ≥2 (n = 6, 5.3%), proptosis regression ≥2 mm (n = 37, 32.7%), or both (n = 18, 15.9%). CONCLUSIONS:This extended follow-up analysis demonstrated proptosis regression after teprotumumab in two-thirds of eyes at long-term follow-up, with one-third worsening beyond pretreatment proptosis. Two-thirds continued to worsen after initial regression, and over half of patients developed a thyroid eye disease flare. Though most patients maintained net improvement, these results suggest that proptosis effects are not durable in most patients after standard teprotumumab dosing.

Histiocytic sarcoma is an extremely rare malignancy typically presenting in extranodal sites, including the gastrointestinal tract, lung, nasal cavity, and soft tissue. Primary presentation in the skin is an even rarer entity, with only one prior case reported in the eyelid skin. Herein, we describe a case of histiocytic sarcoma that presented in the eyelid of an 80-year-old man. The lesion presented as a rapidly growing left lower eyelid lesion over 4 months. Shave biopsy revealed histologic and immunophenotypic findings most consistent with a morphologically low-grade histiocytic sarcoma. Complete excision was performed with an additional 2 mm peripheral and deep margins, which were found to be clear of malignancy. Systemic work-up and whole body imaging showed no evidence of metastases or other associated malignancy. The decision was made to forego radiation and proceed with close observation every 3 months. Two years from presentation, the patient had no evidence of recurrence.

PURPOSE:To evaluate the efficacy of teprotumumab in a subset of thyroid eye disease (TED) patients with prolonged disease duration and high clinical activity score (CAS). METHODS:This is a retrospective study of all TED patients who underwent eight infusions of teprotumumab and had a consistently documented CAS ≥ 4 for at least 2 years prior to teprotumumab initiation at a single institution. Primary outcome measures included proptosis response (difference in median pre- and post-treatment Hertel exophthalmometry), CAS response (difference in median pre- and post‑treatment CAS), and diplopia response (≥1 point improvement in Gorman diplopia score), comparing pre-treatment values to post-treatment measurements at the immediate follow-up visit using Mann-Whitney U testing. . RESULTS:Of 198 patients who initiated teprotumumab treatment from April 1 2020 to March 31 2024, 8 patients met inclusion criteria. The median TED duration, measured from the first clinic visit to the clinic visit closest to initiation of teprotumumab was 42.3 months; the average follow‑up interval was 32.7 months. The median pre-treatment Hertel measurement was 24.25 mm (IQR 4.88) compared to a post-treatment measurement of 21.81 mm (IQR 4.63) (p < 0.001) - a mean proptosis reduction of 2.44 mm (13.6%). The median pre-treatment CAS was 5 (IQR 1) compared to a post-treatment CAS of 1 (IQR 2.25) (p < 0.001). The median pre-treatment diplopia score was 1.5 (IQR 1.25) compared to a post-treatment score of 0.5 (IQR 1.0) (p = 0.04). CONCLUSIONS:This retrospective study suggests that teprotumumab can be an effective treatment for patients with thyroid eye disease who have prolonged disease duration and high clinical activity.

PURPOSE:To review the literature on the outcomes of severe congenital ptosis repair in the pediatric population and to assess the rates of exposure keratopathy and other complications. METHODS:A literature search was last conducted in May 2025 in the PubMed database. Articles were limited to original research in the English language that included detailed postoperative data on at least 25 eyelids with severe congenital ptosis (defined as levator function of 4 mm or less) after any given ptosis repair technique with at least 6 months of postoperative follow-up. The initial search identified 209 citations; 30 articles were reviewed in full text, and 11 articles were selected for inclusion in this assessment. The panel methodologist assigned a level of evidence rating to each of the included studies. RESULTS:One study was rated level I, 1 study was rated level II, and 9 studies were rated level III. The included studies examined frontalis flap, frontalis suspension, and maximal levator resection surgeries. They focused on a wide range of pediatric ages (infancy to 16 years), but the average age range in all cases was squarely in the younger pediatric demographic (ages 1-8 years). The literature demonstrated variable descriptions of success, revision surgery, and exposure keratopathy. Significant overlap in success, exposure, and revision exists for all 3 surgical techniques. Perhaps biased by the greater number of articles defining success, frontalis suspension surgery studies demonstrated the widest range of efficacy and had the additional surgery-specific complications of sling infections, extrusions, and granulomas. CONCLUSIONS:Although the level of evidence and dearth of comparative literature precluded evaluation of clear equivalency data, frontalis flaps, frontalis suspension, and maximal levator resection appear to have overlapping rates of success and rates of exposure. Risks of surgery may influence surgical choice. Frontalis-related techniques were associated with unique risks, including sling-specific inflammation, infection, and exposure; and flap-related lid displacement with supraduction. Long-term, multi-institutional, comparative prospective studies would allow further insight into surgical durability and complication rates. FINANCIAL DISCLOSURE(S):Proprietary or commercial disclosure may be found after the references.

The rise of artificial intelligence has ushered in a wave of interest and research on its potential applications in ophthalmology, including oculoplastic surgery. This review summarizes current efforts to incorporate artificial intelligence in the management of various oculoplastic conditions, including eyelid neoplasms, blepharoptosis, thyroid eye disease, orbital fractures, orbital lesions, nasolacrimal duct obstruction, as well as patient education and counseling.

Purpose To review the literature and identify the health-related quality-of-life (HRQL) outcomes that used a validated instrument in the assessment of upper blepharoplasty, blepharoptosis surgery, or combination surgery. Methods A literature search was last conducted in the PubMed database in January 2025 to identify all studies in the English language investigating HRQL outcomes that used a validated instrument in the assessment of upper blepharoplasty, blepharoptosis surgery, or combination surgery. The literature search yielded 773 citations, and 20 studies met the inclusion criteria. All studies included a validated instrument to assess HRQL. A panel methodologist then assigned a level of evidence rating for each study that met the inclusion criteria. Results Three studies were rated level I, 8 studies were rated level II, and 9 studies were rated level III. The surgeries performed in the studies included upper blepharoplasty, blepharoptosis correction, or both. Some studies also included the addition of brow ptosis repair. Seven studies focused on the effect of the upper eyelid interventions on dry eye symptoms, 5 of which observed no subjective worsening and 1 of which demonstrated symptoms that were transient. The remaining studies focused on aspects of visual function, patient satisfaction, self-consciousness of appearance, benefit in daily life, ocular symptoms, headache symptoms, or a combination thereof and found a benefit of surgery in these various domains. Three studies that examined the long-term HRQL benefit of blepharoptosis correction noted continued improvement at mean follow-up intervals ranging from 1.5 years to 3.6 years after surgery. Conclusions Most HRQL studies examining the effects of upper blepharoplasty, blepharoptosis correction, or both on dry eye symptoms found no significant worsening of subjective metrics. Other studies demonstrated that surgery could provide improved HRQL outcomes that persist for several years. Financial Disclosure(s) Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

PURPOSE:Computed tomography (CT) uses Hounsfield units (HU) for density, which is a standardized unit of measure. This study created a HU reference guide and evaluates the use of HU for orbital lesions and normal structures. METHODS:Retrospective chart review at two academic centers identified patients with biopsy proven orbital lesions and CT imaging. Patients with normal orbital structures were included as a normative database. HU was measured. RESULTS:The study included 219 patients with orbital lesions and 50 control patients with a total of 341 images. Of these, 241 images were orbital lesions, with 137 non-contrast and 104 contrast. There were 50 non-contrast and 50 contrast normal scans. The orbital lesion group represented 29 distinct orbital diagnoses. Major orbital structures were distinguishable by HU, and extraocular muscles were found to be significantly different between muscles. A subset of orbital lesions had significantly different mean HU compared to normal orbital structures. CONCLUSIONS:Analysis of Hounsfield units provides additional depth to orbital imaging that may aid in diagnostic yield and therapeutic intervention for certain lesions. This study summarizes HU data for orbital lesions as well as a normative database of orbital structures.

PurposeTo present a modified evisceration technique with a full-thickness horizontal sclerotomy and assess post-operative motility and long-term outcomes.MethodsThis is a retrospective chart review of patients who underwent evisceration with a single surgeon (TJM). The standard initial steps of evisceration were performed. Before implant placement, a complete radial sclerotomy was extended posteriorly at the 3 and 9 o'clock positions, 2 mm above the optic nerve, longitudinally dividing the medial and lateral rectus insertions. The scleral halves were mobilized, allowing them to "swing" forward and overlap over the spherical implant. In a subgroup of five patients, postoperative motility was assessed by marking the conjunctiva overlying the implant and taking photos in extreme gazes. Operative and non-operative eye measurements were compared in SiliconCoach Digitizer Software.ResultsThirty patients were identified (16 male, 14 female; mean age 56.9, range 4.6-90.1 years). Eleven patients were noted to have phthisis bulbi. Implant sizes were 18 mm (n = 13), 20 mm (n = 14), and 22 mm (n = 2) with a mean of 19.24 mm. Mean horizontal and vertical motility ratios of operated to non-operated eye were 0.61 +/- 0.18 and 0.76 +/- 0.28, respectively, with no statistically significant differences (p = 0.38).ConclusionsEvisceration with complete horizontal full thickness sclerotomy is an effective technique that prevents the sclera from limiting implant size, even in patients with phthisis bulbi. Longitudinal division of the recti insertions also did not affect function or implant stability.

Introduction: Complete surgical excision with clear margins is the standard treatment for cutaneous squamous cell carcinoma (cSCC). Historically, orbital invasion required high-dose radiation, with or without chemotherapy or exenteration. However, if the orbital involvement is limited to perineural spread within a nerve and remains distal to the skull base foramen, a neurectomy offers a globe-sparing option that may more definitively prevent intracranial spread. This study is the first to report outcomes of globe-sparing neurectomy for isolated frontal nerve perineural spread in cSCC. Methods: A retrospective chart review was performed to identify patients with zone 1 (distal to the superior orbital fissure) cSCC who underwent globe-sparing frontal nerve (FN) resection for perineural spread (PNS). Patient demographics, medical history, and treatment details were obtained from electronic medical records. Radiographic measurements of nerve thickness and enhancement were obtained from pre- and post-gadolinium coronal T1-weighted MRI orbit images, with or without fat suppression. Surgical margin and resection lengths were based on pathology report measurements. Results: Five consecutive patients underwent frontal nerve resection via anterior orbitotomy. Four patients with negative proximal margins received subsequent radiation and/or chemotherapy/immunotherapy and remained alive and free of intracranial disease at 18-54 months postoperatively. One patient, despite undergoing extended resection beyond the radiographically involved segment, had a positive margin and subsequently required exenteration. Up to 80% of the frontal nerve length (maximum 44 mm) was safely resected without injury to the vision, globe, or orbit. Conclusion: Frontal nerve excision via orbitotomy, combined with radiation and/or chemotherapy/immunotherapy, prevented intracranial tumor extension in all cases with negative proximal margins and avoided orbital exenteration in 80% of patients. Given the risk of microscopic spread beyond visible radiographic margins, maximal excision should be considered. Globe-sparing neurectomy is a viable, low-morbidity surgical option that can be integrated into multidisciplinary treatment strategies to achieve local disease control in appropriately selected patients.

PURPOSE:Silent sinus syndrome (SSS), known as chronic maxillary atelectasis, is characterized by inward collapse of the maxillary sinus walls with resultant bowing of the maxillary sinus bones due to negative antral pressures. Functional endoscopic sinus surgery (ESS) is an important first step to normalize pressures. The impact of SSS on nasolacrimal duct (NLD) anatomy remains unclear. The study hypothesized that SSS may distort NLD anatomy, since the duct sits within the maxillary bone, and aimed to compare the radiographic changes on the affected and unaffected sides, as well as pre- and post-ESS. METHODS:A retrospective review was conducted on patients with computed tomography (CT) face/orbit with radiographic findings of SSS. A subset who underwent ESS with a post-operative CT was included for secondary analysis. The NLD, sinus, and orbit were measured in a standardized fashion. Primary outcome assessed differences between the affected and unaffected sides, while secondary outcomes evaluated pre- and post-ESS changes. Analysis was performed with a paired-t test. RESULTS:Twenty patients met inclusion criteria, with 12 included in post-ESS analysis. The affected side showed significantly smaller maxillary sinus width and height, and orbit height (p < .001). The distal NLD (lateral) was 0.44 mm larger on the affected side (p = .01), but all other NLD measurements were not significant. No significant changes were observed in NLD, sinus, or orbit measurements after ESS. CONCLUSIONS:SSS did not significantly distort NLD anatomy, nor were there anatomical changes post-ESS, aside from a larger distal NLD on the affected side, possibly due to bowing of the inferior turbinate. Future directions could consider 3-D volumetric analysis of the NLD.

Immunoglobulin G4-related disease (IgG4-RD) comprises a spectrum of inflammatory conditions characterized by lymphoplasmacytic infiltration by IgG4+ plasma cells, resultant tumefactive lesions at various locations in the body, storiform fibrosis, and elevated serum IgG4 levels. IgG4-related ophthalmic disease (IgG4-ROD) can occur in isolation or in conjunction with systemic findings. The most common manifestations of IgG4-ROD are dacryoadenitis and eyelid edema, though patients may also have trigeminal nerve enlargement, extraocular muscle involvement, and other orbital soft tissue lesions and inflammation. Workup is multifactorial and should include full body imaging, serum IgG4 levels, and biopsy, which must include staining for IgG4+ plasma cells and may show hallmark storiform fibrosis and/or obliterative phlebitis. IgG4-ROD can be misdiagnosed due to potential clinical, radiographic, and histopathologic overlap with other orbital inflammatory conditions; however, the presence of infraorbital or supraorbital nerve enlargement on imaging, as well as biopsy with strong levels of IgG4 histochemical staining, can be helpful in distinguishing IgG4-ROD from other orbital inflammatory pathology. Steroids are typically first-line treatment, although given high rates of recurrence, patients may require concurrent or subsequent treatment with steroid-sparing agents, most commonly rituximab.

PURPOSE:The objective was to review the treatment response of advanced conjunctival squamous cell carcinoma (SCC) to systemic immune checkpoint inhibitor (ICI) therapy at a single institution. METHODS:A retrospective review of patients treated at a single institution from 2015 to 2024 was conducted to identify those with advanced conjunctival SCC who had been treated with ICI therapy. Advanced disease included patients with orbital invasion of tumors, unresectable disease, or metastatic disease. Computed tomography imaging and tumor mutational burden data were evaluated for all patients. RESULTS:Five patients with advanced conjunctival SCC were treated with ICIs. All patients had the American Joint Committee on Cancer stage cT3N0M0. All patients had best corrected visual acuity in the affected eye of 20/30 or better at presentation. All patients progressed while on ICIs, with 3 ultimately requiring exenteration at a median time of 6 months from initial diagnosis. One patient had progressive metastatic disease, and one had direct intracranial extension. All patients had low tumor mutational burden. CONCLUSIONS:Unlike prior reports demonstrating complete treatment response while on ICI therapy in patients with advanced conjunctival SCC, the current study demonstrates that 5 of 5 patients had disease progression while on ICI therapy. All patients also had low tumor mutational burden. Tumor mutational burden may be important in predicting disease response to ICI in patients with conjunctival SCC; however, given the small number of patients with conjunctival SCC treated with ICI to date, more data are needed to understand the role of ICIs in conjunctival SCC.

PURPOSE:While previous small studies report positive effects of teprotumumab on thyroid eye disease-associated compressive optic neuropathy (TED-CON), the timing and duration of CON improvement remains unclear. This study analyzes the efficacy, timing, and durability of teprotumumab treatment for TED-CON. METHODS:Retrospective study of all patients who completed eight teprotumumab infusions at one institution from January 1 2020 to December 31 2022. Primary outcome was CON resolution rate after teprotumumab. Secondary outcomes included number of infusions to CON improvement or resolution, factors impacting resolution, and recurrence rates at late follow-up. RESULTS:Of 129 patients who completed teprotumumab, 35 (13.6%) eyes of 21 patients had TED-CON: 4 (11.4%) eyes had disc edema, 15 (42.9%) had dyschromatopsia, and 32 (91.4%) had Humphrey visual field defects. Of 19 patients with CON clinical data after initiating teprotumumab, 100% of eyes had CON improvement after a median of 1.5 infusions (range 1-7), with improvement after only one infusion in 43.8%. CON resolved in 25 (78.1%) eyes of 16 patients after a median of 4 infusions (range 1-8). Despite proptosis regression at most recent follow-up (median 20.4 months) in 11 (55.0%) TED-CON patients averaging 2.9 mm (range 0.5-6.0, SD 1.9), only 3 (15.0%) patients had recurrence of CON. CONCLUSIONS:All patients had CON improvement with teprotumumab, typically over a rapid time course, and the vast majority had CON resolution. Despite proptosis regression, nearly all patients remained CON-free at long-term follow-up. These results suggest that teprotumumab may be an effective treatment for dysthyroid optic neuropathy, especially for patients with mild CON.