PURPOSE:Commonly, dermatochalasis is assessed mainly as a functional condition in which redundant upper-eyelid skin causes demonstrable visual field restriction. This narrative review examines if visual field assessment alone captures the full clinical burden of upper-eyelid dermatochalasis and the benefits of upper-eyelid blepharoplasty. METHODS:A narrative review was undertaken using PubMed/MEDLINE, Google Scholar and citation searching. All primary studies included in the review that reported symptoms, patient-reported outcomes, anatomical severity or postoperative functional outcomes were extracted into structured evidence tables. Reviews, policy documents and referral guidelines were synthesized narratively. RESULTS:Evidence supports improvement in the superior visual field after upper-eyelid blepharoplasty. Other reported benefits include improved contrast sensitivity, headache-related quality of life, eyelid heaviness, tearing and patient-reported visual quality of life, lid-margin position, redundant-skin severity, lateral hooding or aesthetic outcome. No single validated dermatochalasis-specific functional symptom questionnaire is currently available that captures the full burden, and anatomical severity does not consistently predict patient-reported benefit. The current UK National Health Service (NHS) eligibility criteria are commonly centered on visual field loss, eyelid positioning or pupil occlusion. The Danish criteria provide a comparator by combining symptom burden with anatomical findings. CONCLUSIONS:Visual field restriction is an important indication for publicly funded upper-eyelid blepharoplasty, but it is not a complete measure of disease burden. Future referral criteria should consider the addition of a structured dermatochalasis symptom score.
PURPOSE:To review the current literature on imaging modalities and segmentation techniques used to evaluate the orbital fat compartment in thyroid eye disease (TED), with particular emphasis on their role in assessing disease activity and improving diagnostic accuracy. METHODS:A literature review was performed of studies using CT, MRI, ultrasound, and nuclear medicine imaging to evaluate orbital fat involvement in TED. Imaging findings were considered in the context of known histopathological changes, with particular focus on emerging MRI sequences and advances in segmentation methodology. RESULTS:There is currently no established gold-standard imaging modality for assessing changes in orbital fat volume or composition in TED. Fat-suppressed MRI techniques, particularly STIR and Dixon sequences, show promise for detecting inflammatory activity within the orbital fat compartment. Diffusion-weighted imaging (DWI) may enable identification of subclinical disease, while diffusion tensor imaging (DTI) demonstrates potential for quantitative assessment of disease activity. Multiparametric "whole-orbit" imaging approaches incorporating extraocular muscles, lacrimal gland, and orbital fat improve diagnostic performance compared with single-structure assessment. However, accurate anatomical delineation remains challenging, and although automated segmentation and deep-learning-based volumetric analysis offer important future opportunities, these techniques are currently largely confined to research settings. CONCLUSION:Orbital fat pathology represents a key component of TED pathogenesis, yet radiological assessment of the orbital fat compartment remains insufficiently standardized. Fat-suppressed MRI techniques - particularly DWI - show significant promise for improving detection of inflammatory activity and may support future development of multiparametric imaging-based disease‑activity assessment.
Lipoblastomatosis is a rare primitive embryonal adipose tissue tumor seen in the early pediatric age group. It is diffusely infiltrative but typically benign in nature. The standard of care for the management of these tumors is complete surgical extirpation. Incomplete excision is reported to have a higher incidence of recurrence. It is an extremely uncommon tumor in the orbit and only a few cases have been previously reported in the ophthalmic literature. Herein, the authors present the case of a diffuse orbital lipoblastomatosis in a young girl that displayed very aggressive behaviour with relentless recurrence even after a radical surgical approach with adjuvant therapy. Apart from documenting the rarity of this tumor in the orbit, this report also explores the challenges in making a definitive diagnosis and planning the management in cases that are aggressive and recurrent in nature.
We report a 25-year-old male who underwent en bloc resection of a large NF1-associated high-grade malignant peripheral nerve sheath tumor (MPNST) and oculoplastic reconstruction. Following adjuvant chemo-radiotherapy and 3 years of oncologic surveillance, sequential oculoplastic refinements were performed to address radiation-induced periorbital and facial fibrosis, ptosis, and volume loss. These included brow lift, ptosis repair, blepharoplasty, lateral canthal lengthening, and autologous fat grafting. At one-year follow-up after reconstruction: symmetrical palpebral fissures (30 mm OD, 31 mm OS), bilateral MRD1 of 5 mm, no lagophthalmos, FACE-Q scores improved from 25/100 to 85/100, and MRI confirmed 70% fat graft retention with no tumor recurrence. A structured, stepwise approach to immediate functional stabilization, adjuvant therapy, and surveillance, followed by sequential functional and aesthetic refinements, may help guide reconstructive planning in similarly complex cases.
Pneumosinus dilatans (PSD) is a rare expansile disorder characterized by enlargement of an aerated paranasal sinus beyond its normal anatomic boundaries while preserving bony integrity. We report a 26-year-old man with progressive unilateral proptosis presenting 18 years after severe craniofacial trauma complicated by permanent sixth and seventh cranial nerve palsies. Examination demonstrated 5 mm axial left proptosis with paralytic lagophthalmos, lower eyelid retraction, and exposure keratopathy. Computed tomography revealed marked left frontal sinus enlargement (62.7% volumetric increase) with intact bony walls, consistent with frontal PSD. This case demonstrates the importance of objective exophthalmometry and orbital imaging in distinguishing true space-occupying sinus-related proptosis from smaller anterior globe displacement and periocular changes associated with facial nerve palsy alone. Management consisted of orbital decompression combined with upper and lower eyelid rehabilitation. Hertel asymmetry improved from 5 mm to 1 mm with satisfactory functional and cosmetic outcomes. The case emphasizes that careful imaging is essential when evaluating globe prominence in the setting of facial asymmetry.
Giant cell tumors (GCT) are a rare group of fibrohistiocytic neoplasms that may originate from bone or soft tissue. They are typically found in the extremities or trunk and are rarely reported in the head and neck. While they are benign, they may grow rapidly and be locally destructive. To our knowledge, only three case reports of giant cell tumors of soft tissue involving the orbit have been reported in the literature. We report two cases: one case of giant cell tumor associated with Paget's disease of the bone and one of tenosynovial giant cell tumor.
A 12-year-old girl presented with pain, swelling, and decreased vision in the left eye of 1-month duration. She had a significant history of left Wilms' tumor treated with left radical nephrectomy and chemotherapy at three years of age. Ocular examination revealed massive left proptosis, inferior globe dystopia, visual acuity of 20/160, ptosis, conjunctival chemosis, and corneal exposure changes. Orbital magnetic resonance imaging revealed a well-circumscribed T1-hypo and T2-hyperintense tumor in the superotemporal orbit with marked contrast enhancement. An excisional biopsy was performed, and histopathology was consistent with embryonal rhabdomyosarcoma. Genetic counseling was undertaken in view of multiple malignancies. Whole-exome sequencing identified a likely pathogenic null variant in the TRIP13 (c.998_999delCT, p.Ser333Leufs *30) gene, suggesting a diagnosis of mosaic variegated aneuploidy syndrome 3. Systemic surveillance showed no metastatic lesions. Adjuvant chemoradiotherapy was advised. Mosaic variegated aneuploidy syndrome can be associated with multiple malignancies, including Wilms' tumor and orbital rhabdomyosarcoma.
PURPOSE:To characterize nuclear expression of Yes-associated protein 1 (YAP1), a key effector of the Hippo pathway, in lacrimal gland adenoid cystic carcinoma (LG ACC) and explore potential associations with clinicopathologic features and clinical outcomes. METHODS:A retrospective, multi-institutional study of 24 LG ACC specimens was performed. Immunohistochemistry (IHC) was used to assess nuclear YAP1 expression. Staining intensity and percentage expression were combined into a composite NY-SCORE. Exploratory analyses evaluated relationships between YAP1 expression scores, clinicopathologic features, and clinical outcomes. RESULTS:Nuclear YAP1 expression was observed in all 24 LG ACC cases: 6 tumors showed moderate staining (NY-SCORE 5-6), 18 tumors showed high staining (NY-SCORE 7-8), and none were low-scoring. Exploratory analyses did not demonstrate statistically significant associations between YAP1 expression and clinicopathologic features or clinical outcomes. CONCLUSIONS:Nuclear YAP1 expression was consistently present in LG ACC tissue, suggesting that YAP1 nuclear localization is a common feature of this malignancy and supports future investigation of Hippo pathway activation in LG ACC. While YAP1 expression did not distinguish clinicopathologic risk or outcomes in this cohort, its uniform presence provides a foundation for future studies evaluating its biological role and potential therapeutic relevance.
PURPOSE:To evaluate intraocular pressure (IOP) changes after levator aponeurosis advancement (LAA) for acquired aponeurotic ptosis and compare eyes with and without glaucoma-filtering blebs. METHODS:This retrospective study included consecutive patients undergoing LAA at the University of Tokyo Hospital between May 2016 and November 2017. IOP was measured via Goldmann applanation tonometry preoperatively and at 1 and 3 months postoperatively. Eyes were classified by the presence of filtering blebs. Patients with ocular abnormalities other than glaucoma were excluded. A linear mixed-effects model with a random intercept per patient assessed the effects of time, bleb status, and interaction. RESULTS:Sixteen (32%) of 50 eyes from 48 patients had filtering blebs. Baseline IOP did not differ significantly between groups. Adjusted IOP changes at 1 and 3 months were not significantly different between eyes with and without filtering blebs. Within-group analysis showed a significant IOP reduction at 3 months in non-bleb eyes (p = 0.020), with no significant change at 1 month (p = 0.051). Bleb eyes showed no significant change at 1 month (p = 0.170) or 3 months (p = 0.790). CONCLUSIONS:LAA for aponeurotic ptosis did not cause early postoperative IOP increases in eyes with filtering blebs. Postoperative IOP changes were similar between groups.
Nontuberculous mycobacteria may cause indolent postoperative or device-associated infection and can be overlooked in anophthalmic socket inflammation. A 71-year-old woman with a right anophthalmic socket reconstructed with a dermis-fat graft developed progressive inability to retain her prosthesis, followed by purulent discharge. Socket swab was acid-fast smear-positive and subsequently identified as Mycobacterium abscessus species group. Orbital magnetic resonance imaging showed mild curvilinear enhancement along the prosthesis margins without drainable abscess. Management included prolonged susceptibility-guided multidrug antimicrobial therapy in collaboration with the infectious disease team, with staged oculoplastic procedures for concurrent cicatricial contracture. Symptoms improved, a new prosthesis was fitted approximately 3 months after presentation, and the socket remained clinically quiet through approximately 15 months of follow-up. This case highlights atypical mycobacterial infection as a diagnostic consideration in persistent anophthalmic socket inflammation with progressive contracture.
PURPOSE:To evaluate the relationship between adenoid size, systemic immune-inflammation index (SII), and the severity of orbital complications in pediatric acute bacterial rhinosinusitis. METHODS:A retrospective analysis was conducted on 51 pediatric patients hospitalized with orbital complications of acute bacterial rhinosinusitis between 2011 and 2025. Demographic characteristics, length of hospital stay, adenoid-to-nasopharynx (A/N) ratio, computed tomography findings, and laboratory parameters were analyzed. Patients were classified as having preseptal or postseptal complications. RESULTS:The median age was 8 years (range, 1-18), and 58.8% were male. Preseptal cellulitis was observed in 56.9% of patients, while 43.1% had postseptal complications. The A/N ratio was significantly higher in the postseptal group (0.63 vs. 0.55, p = 0.012), as was the length of hospital stay (15 vs. 7 days, p < 0.001). SII values were similar between groups (p = 0.238), but correlated with C-reactive protein levels and hospitalization duration (p < 0.05). The A/N ratio showed moderate discriminative ability (AUC = 0.72; cut-off: 0.60). CONCLUSION:A higher A/N ratio may be associated with more severe orbital complications and prolonged hospitalization in pediatric acute bacterial rhinosinusitis. It may serve as an adjunctive radiologic marker for risk stratification, whereas SII appears to reflect systemic inflammatory burden without predicting complication severity.
Current grading systems for thyroid eye disease (TED) are subjective, highlighting the need for objective assessment tools. This pilot study evaluated whether anterior segment optical coherence tomography angiography (AS-OCTA) can detect characteristic vasculature changes in TED. A prospective comparative case series at Moorfields Eye Hospital included patients with TED and healthy controls. AS-OCTA images were captured using the AngioVue system, and masked-image analysis quantified the vessel density index (VDI) and fractal dimension (FD). Twenty-nine TED patients (58 eyes) and 13 controls (26 eyes) were included. In total, 965 images were analysed (671 TED, 294 controls). Mean VDI was significantly higher in TED patients (33.3%) than in controls (20.7%) (p = 0.01). Highest VDI occurred in the conjunctival/episcleral complex in both groups. TED patients had higher episcleral/scleral VDI than conjunctival epithelium; the inverse was observed in controls. Active TED (CAS = 3-7) exhibited the highest VDI versus inactive TED (CAS = 0-2) and controls. FD was higher in TED, increasing progressively from controls to inactive and active TED. Four patients (6 eyes) underwent decompression surgery with non-significant reductions in VDI and FD. AS-OCTA detects significant differences in vascular parameters between TED and healthy controls, supporting its potential as a non-invasive, quantitative adjunct in TED grading systems.
PURPOSE:To identify clinical and Magnetic Resonance Imaging (MRI) features predictive of response to corticosteroid therapy in patients with inflammatory dacryoadenitis. METHODS:This retrospective clinical comparative study included patients diagnosed with inflammatory dacryoadenitis who received corticosteroid therapy and underwent baseline MRI prior to treatment. Patients were classified as good responders (complete or partial response) or poor responders (no response or refractory disease) according to their clinical outcomes. The primary outcome was response to corticosteroid therapy. RESULTS:Fifty-six patients were included, of whom 44 (79%) were women with a mean age of 42 years (±16). In multivariate analysis, good responders were older than poor responders (aOR = 1.07 [1.01, 1.15]; p = 0.037) and had a shorter duration of symptoms (aOR = 0.97 [0.94, 0.99]; p < 0.001). After adjusting for age and symptom duration, hyperintensity of the lacrimal gland on Diffusion-Weighted Imaging (DWI) was associated with a poorer response to corticosteroids (aOR = 0.19 [0.03, 0.93]; p = 0.040). No differences were observed in diffusion restriction on apparent diffusion coefficient (ADC) maps or in ADC values. A clinico-radiological score incorporating age, symptom duration and lacrimal gland width on baseline MRI showed good predictive performance for treatment response (AUC = 0.84, 95% CI 0.73-0.94). CONCLUSION:Younger age and a longer symptom duration are associated with poorer corticosteroid response in inflammatory dacryoadenitis. Although DWI hyperintensity was linked to treatment failure, the absence of corresponding ADC abnormalities limits its reliability as a predictive marker. Overall, conventional MRI appears to have limited value in predicting therapeutic response.
PURPOSE:The study aimed to investigate the cytotoxic effect of benzalkonium chloride on human lacrimal canalicular epithelial cells under both constant and dynamically decreasing concentrations, which more accurately reflects real ocular surface conditions after eye drop instillation. METHODS:This in vitro study was conducted at the Krasnov Research Institute of Eye Diseases. Primary cell cultures were established from human lacrimal canaliculi tissue and characterized as exhibiting a partial epithelial - mesenchymal transition (EMT) phenotype, confirmed by spindle-shaped morphology and immunocytochemical expression of cytokeratin-19. Cytotoxicity was assessed using live/dead staining and MTT assay following exposure to benzalkonium chloride (BAC) at constant concentrations (2.5-100 µg/mL) and dynamically decreasing concentrations simulating physiological tear dilution. RESULTS:The half-lethal dose (LD50) of benzalkonium chloride at constant concentrations was 21.02 µg/mL (range: 16.45-26.87 µg/mL). When dynamically decreasing concentrations were used (mimicking physiological dilution), the cytotoxic effect was significantly reduced compared to constant concentration exposure at equivalent time points (p < 0.05, two-way ANOVA). Cell viability measurements demonstrated that decreasing benzalkonium chloride concentration over time substantially preserved epithelial cell metabolic activity and membrane integrity. CONCLUSIONS:The dynamically decreasing concentration model, which better reflects in vivo conditions, demonstrates that physiological dilution of BAC in tear fluid significantly reduces its cytotoxic effect on lacrimal canalicular cells undergoing partial EMT. These findings support the hypothesis that dry eye syndrome and impaired tear drainage - conditions that prolong BAC exposure and may promote EMT - increase the risk of secondary lacrimal drainage obstruction in patients receiving long-term BAC-preserved therapy.
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.
