This retrospective case series describes a transorbital endoscopic approach to perform an excision of the frontal nerve branch of the ophthalmic nerve. This was performed for suspected zone 1 perineural spread (PNS) seco...This retrospective case series describes a transorbital endoscopic approach to perform an excision of the frontal nerve branch of the ophthalmic nerve. This was performed for suspected zone 1 perineural spread (PNS) secondary to squamous cell carcinoma. Four patients were identified for inclusion. All were male with a mean age of 76 years (range: 65-81 years). Three patients had histological confirmation of neoplastic nerve infiltration from squamous cell carcinoma. One patient with corneal paraesthesia and radiological frontal nerve enlargement had no histological evidence of PNS. Postoperative complications were encountered in one patient, which included ptosis and upgaze limitation. All patients with histological confirmation of PNS underwent adjuvant radiotherapy and were disease-free at last follow-up (range: 8 months-9 years). The transorbital endoscopic approach to an en bloc stripping of the frontal nerve enables access for biopsy along any segment of the frontal nerve, or for definitive treatment of malignant PNS.
PURPOSE: To quantitatively compare the degrees of surgical freedom achieved by a lateral orbitotomy without bone removal versus a lacrimal keyhole-assisted approach, in which the superolateral orbital rim is shaved witho...PURPOSE: To quantitatively compare the degrees of surgical freedom achieved by a lateral orbitotomy without bone removal versus a lacrimal keyhole-assisted approach, in which the superolateral orbital rim is shaved without full removal. METHODS: Preoperative orbital computed tomography scans from consecutive lateral orbitotomy patients (January 2023-April 2025) were retrospectively analyzed. Using Brainlab software, the authors compared the theoretical volume of surgical freedom and horizontal and vertical attack angles between a simulated orbitotomy without bone removal and the keyhole-assisted technique with incremental rim shaving (25% to 100%). Statistical analysis included paired t tests and linear regression. RESULTS: Twelve patients (mean age: 52.2 years; 58% female) were evaluated. The complete keyhole-assisted approach significantly increased the volume of surgical freedom by 21% (6.2 ± 2.2 mm³ vs. 5.1 ± 2.0 mm³; p = 0.005) and the horizontal and vertical attack angles by 62% (20.4 ± 7.2° vs. 12.6 ± 4.2°; p < 0.001) and 38% (36.4 ± 9.5° vs. 26.4 ± 8.4°; p < 0.001), respectively. Partial rim shaving also yielded significant, progressive gains ( p < 0.05). Linear regression showed that each 1 mm of bone shaved improved the horizontal angle by 16%, the vertical angle by 10%, and the volume of surgical freedom by 6%. The size of the rim prominence strongly correlated with surgical freedom (r = 0.74, p = 0.02). No postoperative cosmetic or functional deficits were observed. CONCLUSIONS: The lacrimal keyhole-assisted orbitotomy significantly enhances surgical freedom for orbital lesions. This minimally invasive technique offers a titratable alternative to marginotomy, allowing tailored bone removal for required maneuverability while preserving orbital rim integrity.
A 53-year-old female with a history of childhood craniofacial fibrous dysplasia presented with several months of right periorbital pain, hyperglobus, and a palpable cheek mass. Imaging suggested malignant transformation...A 53-year-old female with a history of childhood craniofacial fibrous dysplasia presented with several months of right periorbital pain, hyperglobus, and a palpable cheek mass. Imaging suggested malignant transformation of the maxillary tumor, and endoscopic endonasal biopsy confirmed malignant transformation to osteosarcoma. Guanine nucleotide-binding protein, alpha stimulating (GNAS)-activating mutation confirmed the fibrous dysplasia origin. The patient underwent neoadjuvant chemotherapy, right total maxillectomy and free flap reconstruction, postoperative chemotherapy and radiation, and secondary orbital and facial reconstruction for globe malposition and repair of a postoperative sinocutaneous fistula. The patient remains tumor-free at 20-month follow-up. This case highlights the rare malignant transformation of craniofacial fibrous dysplasia, demonstrates the importance of molecular analysis for confirmation of its etiogenesis, and illustrates the challenges associated with reconstruction of the orbit and maxilla following cancer resection.
Ocular manifestations of Waldenström macroglobulinemia (WM) are most frequently related to hyperviscosity-related retinopathy but may also include conjunctival infiltration and vitritis. Orbital and ocular adnexal lympho...Ocular manifestations of Waldenström macroglobulinemia (WM) are most frequently related to hyperviscosity-related retinopathy but may also include conjunctival infiltration and vitritis. Orbital and ocular adnexal lymphomatous deposition, including within the orbit, is exceedingly rare. We report a unique case of a 66-year-old female with orbital infiltration from WM presenting with recurrent episodes of transient vision loss secondary to angiopathic deposition of eosinophilic hyaline paraprotein in the absence of hyperviscosity syndrome. Right orbital biopsy demonstrated adipose tissue with deposition of eosinophilic hyaline material, outlining adipocytes and deposited within walls of blood vessels. The material was strongly PAS positive, weakly metachromatic with crystal violet but was negative for apple-green birefringence on Congo red staining. IgM immunohistochemistry strongly decorated the orbital eosinophilic material, suggesting macroglobulinosis paraprotein deposition and not amyloid. There was an associated patchy infiltrate of low-grade lymphoplasmacytic B-cell lymphoma with kappa light chain restriction demonstrated in the neoplastic plasma cell population, consistent with her known WM.
The authors present a case of eyelid nocardiosis following lower eyelid transconjunctival transposition blepharoplasty with externalized transposition sutures. After incision and drainage of the abscess, microbial cultur...The authors present a case of eyelid nocardiosis following lower eyelid transconjunctival transposition blepharoplasty with externalized transposition sutures. After incision and drainage of the abscess, microbial cultures revealed Nocardia asteroides. Oral trimethoprim-sulfamethoxazole was initiated, leading to resolution of the infection. Nocardia can impact multiple organ systems and cause disseminated disease, most commonly affecting the immunocompromised. However, cutaneous nocardiosis has been described in immunocompetent patients. The species N. asteroides typically spreads hematogenously, rather than through direct cutaneous inoculation. Eyelid surgeons should be aware of the rare possibility of Nocardia infection in patients who develop signs of postoperative eyelid cellulitis or abscess following routine surgery and should recognize the importance of timely, appropriate antibiotic therapy to prevent adverse outcomes.
Neurofibroma can present in a myriad of body tissues, including, very rarely, in the sclera; a unique presentation that is described herein. We describe a case of a 21-year-old man with neurofibromatosis type 1, who pres...Neurofibroma can present in a myriad of body tissues, including, very rarely, in the sclera; a unique presentation that is described herein. We describe a case of a 21-year-old man with neurofibromatosis type 1, who presented with 2 weeks of a blue-gray scleral discoloration overlying a protrusion of the sclera. His presentation was initially concerning for scleritis; however, histopathological analysis revealed an epibulbar neurofibroma. Recognition of rare manifestations of neurofibromatosis is essential to properly diagnose and manage ocular findings in this condition.
Advanced conjunctival squamous cell carcinoma (SCC) can be difficult to treat and may require orbital exenteration-a disfiguring and often psychologically distressing procedure. Recently, immune checkpoint inhibitors, su...Advanced conjunctival squamous cell carcinoma (SCC) can be difficult to treat and may require orbital exenteration-a disfiguring and often psychologically distressing procedure. Recently, immune checkpoint inhibitors, such as cemiplimab and pembrolizumab, have shown promise as alternatives for advanced disease, with an early case series reporting encouraging outcomes. Herein, the authors describe a rare case of primary conjunctival SCC arising during ongoing pembrolizumab therapy for an unrelated malignancy. This unexpected development highlights the variability in tumor responsiveness to immune checkpoint inhibitors and the underlying biological heterogeneity of conjunctival SCC. This case, along with emerging literature, underscores the importance of a more individualized approach to immunotherapy in ocular surface malignancies. It also reinforces the need for reliable predictive biomarkers to guide treatment selection and improve outcomes in conjunctival SCC.
Davila N, Pradeep T, Saffari PS
… +3 more, Evans WI, Wong B, Lee WW
Ophthalmic Plast Reconstr Surg
· 2026 Jul-Aug 01 · PMID 41335095
·
Full text
PURPOSE: To evaluate whether blepharoptosis is associated with an increased risk of incident falls and fractures among adults. METHODS: This retrospective cohort study utilized data from the TriNetX Health Research Netwo...PURPOSE: To evaluate whether blepharoptosis is associated with an increased risk of incident falls and fractures among adults. METHODS: This retrospective cohort study utilized data from the TriNetX Health Research Network (January 2004 to January 2024). Adults aged 18 years or older diagnosed with blepharoptosis were matched 1:1 with controls who underwent an ophthalmologic examination without any abnormal findings. Exclusion criteria included severe ocular pathology, low vision/blindness, or falls/fractures within 6 months before the diagnosis. The primary outcomes were incident falls and fractures within 1 year, assessed using risk ratios (RRs) and Cox regression. RESULTS: A total of 119,101 matched patient pairs were analyzed. At 2 weeks postdiagnosis, patients with blepharoptosis had a significantly increased risk of falls (RR, 1.53; confidence interval [CI]: 1.18-1.98; p < 0.01) and fractures (RR, 2.46; CI: 2.02-3.00; p < 0.01). Fracture risk remained elevated at 3 months and was most pronounced in the skull, facial, and cervical regions (RR, 2.87; 95% CI: 2.32-3.55; p < 0.01). By 1 year, patients who underwent blepharoptosis repair (15.4%) had a significantly lower fall risk compared to those who did not (RR, 0.65; 95% CI: 0.54-0.77; p < 0.01), with multivariable analysis showing an 89% risk reduction (hazard ratio, 0.11; 95% CI: 0.02-0.83; p = 0.03). CONCLUSIONS: Blepharoptosis is associated with an increased risk of falls and fractures, particularly among older adults. Surgical correction mitigates this risk, suggesting a potential role for blepharoptosis repair in fall prevention strategies.
PURPOSE: The identification of novel pathways in the etiopathogenesis of basal cell carcinoma (BCC) holds promise for the development of innovative therapeutic strategies. This study investigates the immunohistochemical...PURPOSE: The identification of novel pathways in the etiopathogenesis of basal cell carcinoma (BCC) holds promise for the development of innovative therapeutic strategies. This study investigates the immunohistochemical expression of carbonic anhydrase IX (CA-IX) in periocular BCC, as well as the levels of CA-IX and key antioxidant enzymes in serum. METHODS: Fifteen patients with periocular BCC and 18 age- and gender-matched individuals undergoing upper eyelid blepharoplasty were prospectively enrolled. Immunohistochemical analysis of CA-IX expression was performed on tumor tissue excised with clear surgical margins of at least 2 mm, as well as on normal eyelid tissues from the control group. Serum levels of superoxide dismutase 1, catalase, glutathione peroxidase 1, and CA-IX were measured in both groups preoperatively and 1 month postoperatively in the BCC group, using the enzyme-linked immunosorbent assay method. RESULTS: In the BCC group, preoperative serum CA-IX levels were significantly higher, while serum glutathione peroxidase 1, catalase, and superoxide dismutase 1 levels were significantly lower compared with the control group ( p = 0.001). CA-IX was immunohistochemically positive in 11 of 15 (73%) BCC patients. One month after surgery, an increase in superoxide dismutase 1 and glutathione peroxidase 1 levels and a decrease in CA-IX levels were observed in BCC patients ( p < 0.05). CONCLUSIONS: This study's findings suggest that CA-IX is strongly expressed in periocular BCC tissues and may also serve as a systemic marker, given its elevated serum levels. The lower levels of antioxidant enzymes may be an indicator of possible systemic oxidative stress in BCC patients.
PURPOSE: To assess the current and future supply and demand for oculoplastic surgeons in the United States and evaluate whether the current number of fellowship positions aligns with projected workforce needs. METHODS: O...PURPOSE: To assess the current and future supply and demand for oculoplastic surgeons in the United States and evaluate whether the current number of fellowship positions aligns with projected workforce needs. METHODS: Ophthalmic Plastic Surgeons (OPS) were identified using the American Academy of Ophthalmology membership directory. Each OPS's age was estimated based on the year of medical school graduation. Three retirement models were used, assuming average retirement ages of 62, 65, and 69. These projections were compared with the number of graduating fellows. United States Census Bureau population forecasts were used to calculate future OPS-to-population ratios for 2033, 2043, and 2053. RESULTS: In 2023, an estimated 735 OPS were practicing, yielding a ratio of 1 OPS per 455,000 people. If 57 fellowships are maintained, the projected ratio in 2033 will be 1 OPS per 449,000 people, assuming an average retirement age of 69. CONCLUSIONS: Maintaining the current number of fellowships will sustain the 2023 OPS-to-population ratio through 2033. A slight oversupply is expected thereafter, helping to offset earlier retirements and to meet the rising demand for ophthalmic plastic surgery in the aging US population.
Superior ophthalmic vein thrombosis (SOVT) has many well-defined infectious, autoimmune, hematologic, and malignant etiologies. The authors present the first known case of SOVT associated with idiopathic cryoglobulinemia...Superior ophthalmic vein thrombosis (SOVT) has many well-defined infectious, autoimmune, hematologic, and malignant etiologies. The authors present the first known case of SOVT associated with idiopathic cryoglobulinemia. A 42-year-old woman with a history of idiopathic cryoglobulinemia presented with severe headache, right eye pain, and diplopia. Imaging revealed retrobulbar inflammation and an engorged superior ophthalmic vein, consistent with SOVT. The patient was treated with heparin and intravenous methylprednisolone, resulting in significant clinical improvement. Cryoglobulinemia is a small to medium vessel vasculitis caused by immune complex precipitates; it is typically associated with conditions such as hepatitis C and hematologic disorders, but idiopathic cases are also recognized. The authors report SOVT as an unusual manifestation of cryoglobulinemia, emphasizing the importance of considering SOVT in the differential diagnosis of patients presenting with cryoglobulinemia and eye pain.
PURPOSE: This study analyzed the long-term glycemic trends in thyroid eye disease patients treated with teprotumumab. METHODS: A retrospective study was performed at a single institution, including thyroid eye disease pa...PURPOSE: This study analyzed the long-term glycemic trends in thyroid eye disease patients treated with teprotumumab. METHODS: A retrospective study was performed at a single institution, including thyroid eye disease patients who started treatment with teprotumumab and had pre- and posttreatment hemoglobin A1c (HbA1c) measurements. Patients were categorized as normoglycemic, prediabetic, or diabetic at baseline. Outcomes included changes in HbA1c from baseline to peak and immediately posttreatment, worsening glycemic control (progression to a higher glycemic category or HbA1c increase ≥0.5%), and recovery to baseline glycemic status. RESULTS: Seventy-one patients were included: 41 (57.7%) normoglycemic, 20 (28.2%) prediabetic, and 10 (14.1%) diabetic (median follow-up 28 months, interquartile range: 18.5-36.0). Median HbA1c increased most in those with diabetes (+1.4%, peak at 4.8 months), followed by prediabetes (+0.5%, peak at 7.7 months) and normoglycemia (+0.3%, peak at 13.2 months). Worsening glycemic control occurred in 90.0% of diabetic, 50.0% of prediabetic, and 44.0% of normoglycemic patients. Initiation or escalation of medical treatment was required by 60.0% of diabetic, 30.0% of prediabetic, and 2.4% of normoglycemic patients. Among patients with worsening glycemic control, those with diabetes had the lowest rate of recovery to baseline (33.3%), relative to prediabetes (75.0%) and normoglycemia (35.3%). CONCLUSIONS: Teprotumumab may be associated with worsening of glycemic control, with the most pronounced and persistent effects in patients with preexisting glycemic disturbances. The varied timing of peak HbA1c and ongoing need for medical treatment highlight the importance of systematic glycemic risk assessment and routine HbA1c monitoring throughout and after treatment to guide clinical management.
PURPOSE: This study aims to investigate the early use of a vertical lid split orbitotomy for the management of orbital compartment syndrome. METHODS: This was a retrospective study of all patients in the authors' center...PURPOSE: This study aims to investigate the early use of a vertical lid split orbitotomy for the management of orbital compartment syndrome. METHODS: This was a retrospective study of all patients in the authors' center who had a vertical lid split procedure for orbital compartment syndrome. Intraocular pressure was measured before and after completion of the procedure as a surrogate for orbital pressure. Information regarding the mechanism of injury as well as the ophthalmic exam was reviewed. RESULTS: Ten patients were included, 3 of which were bilateral, for a total of 13 eyes. The average age of the patients was 46.4 years (19-2 years). Gunshot wounds were the most common mechanism of injury (40%). Five eyes in 4 patients had prior failed canthotomy and cantholysis prior to presentation by an outside provider. The average intraocular pressure prior to intervention was 52.5 mm Hg (39-60 mm Hg). Average postintervention intraocular pressure was 21.7 mm Hg, with all patients achieving adequate reduction of pressure below 30 mm Hg (15-27 mm Hg). This resulted in an average reduction in pressure of 30.8 mm Hg (20-36 mm Hg), or 58.7%. No patients in the study had iatrogenic globe injury. CONCLUSIONS: Orbital compartment syndrome poses a time-sensitive threat to vision. This is the first study of the use of the vertical lid split to decompress the orbit in a clinical setting.
Saffari PS, Davila N, Pradeep T
… +2 more, Wong B, Lee WW
Ophthalmic Plast Reconstr Surg
· 2026 Jul-Aug 01 · PMID 41251628
·
Full text
PURPOSE: The purpose of this study is to evaluate whether the use of glucagon-like peptide-1 receptor agonists (GLP-1RAs) is associated with periorbital changes in patients with type 2 diabetes mellitus (T2DM) or obesity...PURPOSE: The purpose of this study is to evaluate whether the use of glucagon-like peptide-1 receptor agonists (GLP-1RAs) is associated with periorbital changes in patients with type 2 diabetes mellitus (T2DM) or obesity. METHODS: A retrospective cohort study was conducted using the TriNetX Research Network database. Patients with T2DM treated with insulin or other antidiabetic agents and who were GLP-1RA naive served as the T2DM control group. The obesity control group included patients with obesity (body mass index ≥30 kg/m 2 ) who neither had bariatric surgery nor T2DM and were GLP-1RA naive. Study groups included patients with T2DM or obesity treated with GLP-1RAs. Rates of blepharoptosis, brow ptosis, dermatochalasis, ectropion, and entropion were recorded alongside their respective rates of surgical repair as well as the frequency of rhytidectomy and botulinum A toxin use. Outcomes were assessed at 3 and 20 years postdrug approval for the obesity and T2DM study groups, respectively. RESULTS: Patients with T2DM using any GLP-1RA were significantly more likely to develop brow ptosis ( p < 0.001) and receive botulinum toxin A ( p = 0.001) compared to controls. Patients with obesity using a GLP-1RA were significantly more likely to develop brow ptosis ( p = 0.004), dermatochalasis ( p < 0.001), and receive botulinum toxin A ( p < 0.001). Patients with obesity on a GLP-1RA were significantly more likely to undergo brow ptosis repair ( p = 0.001), blepharoptosis repair ( p < 0.001), blepharoplasty ( p < 0.001), and rhytidectomy ( p = 0.011) compared to controls. CONCLUSIONS: GLP-1RA use by patients with obesity or T2DM resulted in significant periorbital changes and subsequent repair. When compared to their respective controls, patients using GLP-1RAs for obesity observed more periorbital changes than those with TD2M, likely secondary to excess adipose tissue accumulation and loss.
Unilateral yellow discoloration of the eye, commonly referred to as scleral icterus, is an uncommon clinical finding; there are only 14 cases of persistent unilateral scleral icterus documented in the literature. Here, t...Unilateral yellow discoloration of the eye, commonly referred to as scleral icterus, is an uncommon clinical finding; there are only 14 cases of persistent unilateral scleral icterus documented in the literature. Here, the authors describe a patient with Gilbert's syndrome who presented with left-sided conjunctival and scleral icterus presumed secondary to poor vascular outflow following surgical and radiation treatment of squamous cell carcinoma of the left ear with parotid and cervical lymph node involvement. The current case describes a unique physical finding, reviews the pathophysiology of conjunctival and scleral icterus, and reviews the current literature on this topic.
PURPOSE: To evaluate the diagnostic performance of 7 composite complete blood count-derived indices-systemic immune-inflammatory response index, systemic immune-inflammation index, systemic inflammation response index, p...PURPOSE: To evaluate the diagnostic performance of 7 composite complete blood count-derived indices-systemic immune-inflammatory response index, systemic immune-inflammation index, systemic inflammation response index, platelet-to-lymphocyte ratio, platelet-to-monocyte ratio, hemoglobin-to-platelet ratio, and hemoglobin-to-lymphocyte ratio-in distinguishing periorbital necrotizing fasciitis (PNF) from orbital cellulitis at initial emergency department presentation. METHODS: This retrospective case-control study reviewed adult patients diagnosed with either PNF or orbital cellulitis. Diagnoses were confirmed via clinical, radiographic, intraoperative, histopathologic, and microbiologic findings. Leukocyte, neutrophil, monocyte, lymphocyte, platelet, and hemoglobin count from initial complete blood count upon presentation to the emergency department at a single academic medical center were used to calculate systemic immune-inflammatory response index, systemic immune-inflammation index, systemic inflammation response index, platelet-to-lymphocyte ratio, platelet-to-monocyte ratio, hemoglobin-to-platelet ratio, and hemoglobin-to-lymphocyte ratio values. Group comparisons were performed using Mann-Whitney nonparametric analysis, and receiver operating characteristic curve analysis was conducted for indices with statistically significant differences. RESULTS: Twenty-two PNF patients (mean age 57.8 years) and 18 orbital cellulitis patients (mean age 47.2 years) were included, with no significant differences in age or gender. Systemic immune-inflammation index, systemic inflammation response index, hemoglobin-to-platelet ratio, and hemoglobin-to-lymphocyte ratio were significantly higher in the PNF group ( p = 0.0341, 0.0108, 0.0127, and 0.0099, respectively) with receiver operating characteristic area under the curve values ranging from 0.6970 to 0.7374. Systemic immune-inflammatory response index, platelet-to-lymphocyte ratio, and platelet-to-monocyte ratio showed no significant differences between groups. CONCLUSIONS: Systemic immune-inflammation index, systemic inflammation response index, hemoglobin-to-platelet ratio, and hemoglobin-to-lymphocyte ratio may serve as inexpensive, readily available biomarkers for differentiating PNF from orbital cellulitis at presentation. When interpreted alongside clinical and radiographic findings, elevated values may prompt earlier recognition of PNF and facilitate rapid and appropriate management to reduce morbidity and preserve vision.
The authors report a case of nonsurgical management of lash ptosis using eyelash lamination. A 36-year-old male presented with persistent foreign body sensation and visual obscuration. Slit lamp examination revealed elon...The authors report a case of nonsurgical management of lash ptosis using eyelash lamination. A 36-year-old male presented with persistent foreign body sensation and visual obscuration. Slit lamp examination revealed elongated, ptotic upper eyelashes that rested on the cornea and extended into the pupillary axis. After thorough consultation, the patient declined surgical correction and instead elected to undergo a semipermanent eyelash lamination procedure, which relies on thioglycolic acid and polyacrylamide. This chemical treatment lifted and reshaped the lashes, which redirected them away from the corneal surface. At the 3-month follow-up visit, the patient reported significant improvement in visual function and ocular comfort. He also reported marked satisfaction with the cosmetic outcome. This case highlights the potential of eyelash lamination as a low-risk, noninvasive, and cosmetically favorable alternative for managing lash ptosis, particularly in patients who are poor surgical candidates or prefer to avoid operative procedures.
PURPOSE: To review and analyze lymphoid tumors involving the lacrimal drainage apparatus (LDA) through retrospective review of an orbital surgeon's (T.J.S.) practice across multiple centers, including incidence, clinical...PURPOSE: To review and analyze lymphoid tumors involving the lacrimal drainage apparatus (LDA) through retrospective review of an orbital surgeon's (T.J.S.) practice across multiple centers, including incidence, clinical presentation, signs, radiological features, management, and mortality. METHOD: Retrospective cohort analysis of ocular and adnexal lymphoproliferative disease (OALD) seen through Queensland orbital services between 1992 and 2024. Inclusion criteria were patients with histologically proven OALD. RESULTS: Twenty-two patients with OALD demonstrating involvement of the LDA were identified. Fifty percent of patients presented with dacryocystitis, 32% with a palpable mass in the medial canthus and 27% with symptomatic epiphora. However, 7 (30%) patients had disease detected incidentally at the time of elective dacryocystorhinostomy surgery. CT findings included variable patterns of bony remodeling, erosion, or destruction depending on histology and nasolacrimal duct obstruction. MRI, when utilized, better defined the extent of lesions; commonly isointense on T1 and T2-weighted imaging and moderately gadolinium enhancing, with positron emission tomography-CT effective at screening for undiagnosed systemic disease. Histological review highlighted 8 cases (38%) of diffuse large B-cell lymphoma, 4 (18%) T-cell lymphoma, 3 (14%) chronic lymphocytic or small lymphocytic lymphoma, and 7 (30%) other variants. Ann Arbor lymphoma classification highlighted 30% (6) of patients with stage 1, with 60% (13) identified at stage 3 or 4. Ten patients have achieved disease-free survival, with 1 patient requiring ongoing treatment. Three patients died due to OALD. CONCLUSION: OALD affecting the LDA, while rare, represents a potentially life-threatening differential for patients with LDA symptoms. Presentations are variable; early detection, timely imaging, and histological identification can meaningfully affect associated mortality.
Costello Syndrome (CS) results from a germline mutation in the HRAS gene. Rhabdomyosarcoma in CS patients has arisen from various sites but not the orbit. The authors present the first case report of orbital rhabdomyosar...Costello Syndrome (CS) results from a germline mutation in the HRAS gene. Rhabdomyosarcoma in CS patients has arisen from various sites but not the orbit. The authors present the first case report of orbital rhabdomyosarcoma in a patient with CS. An 18-month-old male known for CS presented with progressively worsening proptosis and periorbital edema. On presentation, he had proptosis with inferolateral globe displacement, chemosis and injection. An urgent computed tomography scan revealed a left orbital mass extending from the posterior aspect of the globe to the orbital apex, and pathological examination confirmed embryonal rhabdomyosarcoma. Treatment with chemotherapy consisted of vincristine, dactinomycin, and cyclophosphamide, and received 8 cycles of photon beam radiation. Radiological surveillance up to 12 months showed no signs of recurrence or metastatic lesions. Patients with CS are at a high risk of developing rhabdomyosarcoma and clinicians should be vigilant in the context of any clinical orbital signs of disease.