External Compressive Ischemic Orbitopathy: A Rising Clinical Entity.

PURPOSE: Saturday night retinopathy, the term coined by Jayam et al . in 1974, is a rare condition in which external compression of the orbit during a drug and alcohol stupor causes a unilateral orbitopathy with ophthalmoplegia and ischemic retinopathy. This condition has been increasingly reported in the last decade, correlating with an increasing burden of substance use. This condition mirrors a similar entity typically reported in patients following spinal surgery, where a headrest supporting the patient's face compresses the orbit. The current authors combine these 2 entities, entitled external compressive ischemic orbitopathy, and present a comprehensive literature review describing this entity. METHODS: A systematic review was carried out in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. All related publications of vision loss in the setting of orbital compression were reviewed. Data collected included patient demographics, precipitating circumstances of vision loss, presenting ocular symptoms, outcomes, and ancillary imaging. RESULTS: In total 31 articles were selected for inclusion, yielding 46 patients. A total of 10 patients suffered orbitopathy in the setting of a drug stupor, and 36 following prone-positioned surgery. However, 79% of patients presented with visual acuity of light perception or worse. Also, 86% of patients presented with ophthalmoplegia, 92% with proptosis and orbital edema, and 86% with varying degrees of retinal ischemia. When compared with iatrogenic cases, self-induced stuporous cases demonstrated worse presenting visual acuity, ophthalmoplegia, retinal and choroidal filling, and worse final outcomes. CONCLUSION: External compressive ischemic orbitopathy is a severe vision-threatening condition that has been increasingly reported in the last decade.

Diffusion-Weighted Imaging of the Orbit: A Case Series and Systematic Review.

PURPOSE: To describe the findings of diffusion-weighted imaging (DWI) for a series of orbital lesions and provide a systematic review of relevant literature. METHODS: A retrospective review of 20 patients with orbital lesions who underwent MRI with DWI at two academic institutions between 2015 and 2020 was performed. Lesion diagnosis was histopathologically confirmed except a presumed cavernous hemangioma. Echoplanar diffusion-weighted images had been acquired using 2 or 3 b values (b=0 and 1000 or b=0, 500, and 1000) at 1.5T or 3T. Lesions with significant artifacts were excluded. DWI sequences were analyzed by neuro-radiologists blinded to the diagnosis. Mean ADC values of lesions were calculated from a single region of interest. An independent two-tailed t test was used to compare categories of lesions with p < 0.05 considered significant. A systematic review of the literature was performed. RESULTS: Our study included 21 lesions. ADC values were significantly lower for malignant lesions (0.628 ± 0.125 × 10 -3 mm 2 /s) than inflammatory lesions (1.167 ± 0.381 × 10 -3 mm 2 /s) ( p < 0.001). ADC values were significantly lower for orbital lymphoma (mean 0.621 ± 0.147 × 10 -3 mm 2 /s) than idiopathic orbital inflammation (mean 1.188 ± 0.269 × 10 -3 mm 2 /s) with no overlap ( p < 0.001). CONCLUSIONS: Orbital malignancies demonstrated lower ADC values, while inflammatory processes demonstrated higher ADC values, except IgG4-related disease. DWI and ADC values differentiated idiopathic orbital inflammation from orbital lymphoma. This study highlights the role of DWI in evaluating orbital pathology.

A comparison of primary and secondary eye removal after open globe injury: A multi-centre study.

BACKGROUND/OBJECTIVES: Our goal was to compare the characteristics and surgical outcomes of patients who underwent primary eye removal surgery after open globe injury with those who underwent secondary eye removal surgery after open globe repair. SUBJECTS/METHODS: This was a retrospective review of subjects who underwent evisceration or enucleation within 3 months of an open globe injury, at three Level I trauma centres in three U.S. cities between July 2014 and July 2020. RESULTS: 19 patients underwent primary eye removal and 20 underwent secondary eye removal. The most common mechanism of trauma in patients who underwent primary eye removal was gunshot. Compared to the secondary eye removal group, patients who underwent primary eye removal were significantly more likely to be male; have longer hospital stays; be discharged to another care facility rather than home; have facial fractures; suffer intracranial injury; and be unable to consent themselves for surgery. Both groups had a low surgical complication rate with one case of socket contracture in each group. CONCLUSIONS: The standard of care for an open globe injury is prompt repair, but there are occasions when the globe is so damaged that it is deemed unrepairable. We found that globes that required primary eye removal were more often due to gunshot wounds, and that there was greater morbidity associated with these injuries. The authors' preferred surgical approach was evisceration with placement of a silicone sphere; patient outcomes demonstrate that this method was found to be safe, with a low complication and infection rate.

Orbital Index: A Novel Comprehensive Quantitative Tool for Prediction of Delayed Enophthalmos in Orbital Floor Fracture Management.

SUMMARY: Early identification of surgical indication is critical to optimizing outcomes in orbital floor fracture management. However, identifying those at risk for delayed enophthalmos and requiring subsequent repair has remained a challenge. This study aimed to validate the Orbital Index, a prediction tool using computed tomography to stratify risk for delayed enophthalmos and establish a threshold for repair. The Orbital Index stratifies fractures by size, location, and inferior rectus rounding (a surrogate for fascioligamentous sling disruption) on a scale 0 to 6. A 22-year (1998 to 2020) multi-institution analysis of unilateral isolated orbital floor fractures was performed. Index scores were assigned to each scan, unoperated patients invited for blinded Hertel exophthalmometry assessment, and enophthalmos measurements correlated with Index scores. Interobserver scoring reproducibility was assessed with weighted Cohen kappa. Preintervention and postintervention Likert scale surveys were administered to determine whether this tool improved understanding and communication. The Orbital Index demonstrated high fidelity and interobserver reproducibility and identified a score of four or greater as a surgical threshold. Of 1769 computed tomography scans, 395 met criteria and were included for analysis. Eighty of 395 were managed operatively (operative rate, 20.3 percent). Of 315 patients managed nonoperatively, 41 (13.0 percent) agreed to follow-up evaluation and 28 (68.3 percent) were found to have enophthalmos. Unoperated patients with an Orbital Index score of 4 or higher were more likely to have enophthalmos than those with a score of 3 or less ( p = 0.001). The mean weighted Cohen kappa was 0.73, corroborating reproducibility. Communication ( p = 0.0003) and ability to correctly identify surgical need ( p = 0.01) were improved with use of this tool. The Orbital Index is a reproducible tool to stratify risk for enophthalmos in orbital floor fracture management.

An age-related algorithm for management of micro-orbitism from anophthalmia: a systematic review with supplemental case reports.

Management of pediatric anophthalmia and resultant micro-orbitism is challenging. The efficacy and safety of treatment methods vary with age as bony changes grow recalcitrant to implants in those at skeletal maturity and osteotomies become technically challenging following frontal sinus pneumatization. This study aims to review methods for managing micro-orbitism and develop an age-based treatment approach. A systematic literature review was conducted. Data were screened and extracted by two investigators and relevant English-language primary-literature was analyzed. Information on sample-size, number of orbits, intervention, age, complications, and prosthetic retention was obtained. Representative case reports are presented, in addition. Nineteen studies met inclusion: 294 orbits in 266 patients were treated. Two studies reported distraction-osteogenesis. Two studies utilized bone grafting. Osteotomies were performed in 41 patients from three studies. Use of solid implants was detailed in two studies. Three studies described osmotic implant. Four studies described inflatable implants. Other techniques were described by three of the included studies, two of which utilized dermis-fat grafting. All but one study were observational case reports or case series. Across all studies regardless of surgical technique, risk of bias and heterogeneity was high due to attrition bias and selective outcomes-reporting. Selection of therapy should be tailored to skeletal-age to optimize outcomes; those 0-4 yrs are managed with dermis-fat grafts, 5-7 yrs managed with implants, and 8+ yrs managed with osteotomies. For those 8+ yrs with aerated frontal sinuses or insufficient bone stock, we propose onlay camouflage prosthetics which improve projection, increase orbital volume, and avoid risk for frontal sinus injury.

Bilateral periorbital leukemia cutis presenting as suspected cellulitis.

Many conditions present with periorbital edema and erythema, mimicking preseptal cellulitis. We report the unique case of a patient with relapsed monoblastic mutant isocitrate dehydrogenase-2 (IDH2) acute myeloid leukemia (AML) who presented with periorbital edema and erythema, unresponsive to antibiotics. Histopathology from punch biopsy was consistent with leukemia cutis. The patient responded rapidly to the initiation of enasidenib, a novel targeted inhibitor of mutant IDH2 enzymes. Our case highlights the importance of considering leukemia cutis in patients with a history of leukemia presenting with periorbital edema and erythema.

Validated Outcome Measures and Postsurgical Scar Assessment Instruments in Eyelid Surgery: A Systematic Review.

BACKGROUND: Determining which postsurgical scar assessment instruments, if any, cover important eyelid outcome measures can either attest to the strength of one or more instruments or reveal the need for a more comprehensive scale. OBJECTIVE: To systematically review validated outcome measures after eyelid surgery and postsurgical scar assessment tools to see whether any individual or combination of 2 assessment tools encompass all relevant, validated eyelid outcome measures. METHODS: Systematic reviews of validated eyelid outcome measures and postsurgical scar assessment tools were conducted using PubMed/MEDLINE and Ovid. Outcome measure papers that met inclusion criteria were sorted into 8 categories: Patient Subjective, Visual Function, Mechanical Function, Daily Activities, Adverse Effects, Aesthetic Quantitative: Clinical Measurements, Aesthetic Qualitative: Global, and Aesthetic Qualitative: Specific. Outcome measure papers were categorized into tiers of evidence support, and assessment tools were evaluated based on which outcome measures each covered. RESULTS: No one or combination of 2 assessment tools covered all selected eyelid outcome measures. Although measures related to the subjective patient experience were included in several of the assessment scales, none covered measures of visual function or eyelid-specific clinical measurements. CONCLUSION: There is currently no existing postsurgical scar assessment instrument that covers all important eyelid-specific outcome measures.

Epithelial-Myoepithelial Carcinoma of the Lacrimal Sac and Literature Review of the Lacrimal System.

BACKGROUND: Epithelial-myoepithelial carcinomas make up less than 0.1% of head and neck malignancies and are regarded as rare, low-grade malignant neoplasms of the salivary gland. They are thought to arise from intercalated ducts with histopathology showing a classic biphasic morphology of an outer layer of myoepithelial cells and inner layer of epithelial cells. These tumors most commonly occur in the parotid gland; however, rare cases have also been described in the nasal cavity, nasopharynx, subglottis, base of tongue, and the lacrimal gland. OBJECTIVE: To describe the clinical presentation, surgical management, and histopathology of the first reported case of lacrimal sac epithelial-myoepithelial carcinoma. To conduct a literature review of this malignancy, which is present in the lacrimal system. METHODS: Case report (n = 1) and literature review. RESULTS: We report a case of a 72-year-old man presenting with epiphora and a lacrimal sac mass with intranasal extension on imaging and nasal endoscopy. A combined endoscopic endonasal and open approach provided successful definitive treatment for final pathologic diagnosis of epithelial-myoepithelial carcinoma of the lacrimal sac, with orbital reconstruction and lacrimal stenting providing good cosmetic and functional results. CONCLUSIONS: After PubMed database search for any case series or reports of lacrimal system epithelial-myoepithelial carcinomas, we believe this is the first documented case originating from the lacrimal sac. Although the histopathology of this tumor is distinct, unusual location and clinical presentation may pose significant diagnostic difficulties.

Assessment of the Safety and Efficacy of Prolapsed Orbital Fat Resection During Involutional Entropion Repair.

PURPOSE: To assess the recurrence rate of involutional entropion in patients treated with a combined approach including a modified Bick procedure, excision of preseptal orbicularis muscle, and conservative resection of prolapsed orbital fat. METHODS: A retrospective chart review of patients undergoing repair of involutional entropion with the combined procedure including orbital fat resection and a second group with standard entropion repair without orbital fat resection was performed. Only patients with follow-up greater than 6 months were included in the study. RESULTS: Seventy eyelids of 54 patients met all inclusion criteria for the combined procedure group over a 9-year period from 2008 to 2016. Average follow-up was 46.9 months. There was a documented recurrence of entropion in 1 eyelid during the follow-up period (1.4%). The remaining 69 cases had successful subjective and objective results without need for any additional procedures. In the group undergoing entropion repair without fat resection, 22 eyelids of 19 patients had the required follow-up period with a recurrence rate of 4.5% (p > 0.05). CONCLUSIONS: The authors demonstrate good surgical success with a combined approach of a modified Bick procedure, preseptal orbicularis excision, and conservative orbital fat resection. Conservative fat resection during entropion repair was found to be safe, and the combined procedure was found to be effective with a rate of recurrent entropion of 1.4% on extended follow-up.The authors propose that orbital fat prolapse contributes to the mechanics of involutional entropion and that conservative orbital fat resection during surgical repair of entropion can be done safely, resulting in low recurrence rates.

Post-operative epiphora following the transcutaneous medial canthal incision.

PURPOSE: The safety profile of the transcutaneous medial canthal incision for access to the medial orbit is assessed with a focus on the risk of post-operative iatrogenic epiphora. METHODS: A retrospective chart review of patients undergoing medial orbitotomy via the transcutaneous medial canthal incision was performed. Patients with a minimum of 3 months of follow-up were included and post-operative complications were assessed and characterized. RESULTS: One-hundred-fifty patients were included in the study. A total of 4 complications were identified, including one each of the following: nasolacrimal duct obstruction, hypertrophic scar, suture granuloma and soft tissue infection. Only the nasolacrimal duct obstruction required surgical intervention. DISCUSSION: Access to the medial orbit has been achieved through a variety of approaches, each with their own benefits and risk profile. The transcaruncular approach has increased in usage as a means to avoid a visible cutaneous scar and decrease the risk of iatrogenic epiphora, however, there are specific patients who may have relative contraindications to this approach. The current study demonstrates the low risk profile of the transcutaneous medial canthal incision, specifically the minimal risk of iatrogenic damage to the nasolacrimal outflow system. This approach is another useful tool which orbit surgeons should be familiar with to offer as an option to patients requiring medial orbitotomy.

Intracapsular hemorrhage rates in non-fixated nylon sheet orbital implants for orbital fracture management.

PURPOSE: To examine the incidence of intracapsular hemorrhage in orbital fracture repair with non-fixated nylon sheet implants. METHODS: A retrospective chart review of 227 patients presenting from January 2013 to December 2016 for orbital fracture repair with nylon sheet implants. RESULTS: Of the 331 orbital fractures repaired over 4 years, a total of 227 met inclusion criteria. The average implant thickness was 0.38 mm and no implants were fixated. Four total implants (1.8%) were removed due to complications; one each secondary to exploration for ongoing postoperative diplopia, immediate post-operative orbital hemorrhage, a cystic mass anterior to the implant, and pain. There were no cases of intracapsular hemorrhage nor infection for any of the 227 patients over 4 years. CONCLUSIONS: To the authors knowledge, this represents the largest case series to date to assess the rate of intracapsular hemorrhage in non-fixated nylon sheet orbital implants. In the 227 cases reviewed over a 4-year period, there were no cases of intracapsular hemorrhage. This suggests a much lower complication rate than previously reported. PRéCIS: A case series of 227 patients who underwent orbital fracture repair with non-fixated nylon sheet implants.

Single Suture Müeller Muscle Conjunctival Resection (ssMMCR): A Modified Technique for Ptosis Repair.

PURPOSE: To evaluate the efficacy in degree of ptosis correction achieved by single suture Müeller muscle conjunctival resection (ssMMCR) when compared with that of traditional MMCR. METHODS: A retrospective chart analysis of patients who underwent either ssMMCR or traditional MMCR at 2 institutions. Single suture MMCR was performed after using a ptosis clamp to imbricate conjunctiva and Müeller muscle. Margin-to-reflex distance 1 was measured pre- and postoperatively, and the change in margin-to-reflex distance 1 was analyzed for both groups. Patients were monitored in follow up for postoperative complications including lagophthalmos, corneal abrasions, and change in visual acuity. Statistical analysis was performed using the Microsoft Excel and Stata software programs. RESULTS: Twenty-seven and 30 patients underwent single suture and traditional MMCR, respectively. The ssMMCR and MMCR groups were followed postoperatively for approximately 4.2 and 9.7 months, respectively and the average margin-to-reflex distance 1 increased by 2.93 mm and 2.81 mm, respectively. Notably, there was no statistically significant difference in the means identified by t test. Of the 94 eyelid surgeries evaluated, 1 ssMMCR and 3 MMCR eyelids required further surgical revision for persistent ptosis, and 1 ssMMCR developed a transient corneal epithelial abrasion. Surgical revisions were rare with both procedures, and patient satisfaction was high. CONCLUSIONS: Single suture MMCR is an efficient and effective method for ptosis repair. It results in comparable outcomes including elevation in margin-to-reflex distance 1, safety profile, and reoperation rates, when compared with traditional MMCR.

Hairpulling causing vision loss: a case report.

Subperiosteal extension of a subgaleal hematoma (SGH) to the orbit is a reported, but rare complication of trauma. This report details a 13-year-old African-American male who originally presented to the emergency department after trauma with headache and was found on CT imaging to have a contained subgaleal hemorrhage. He presented 2 days later with increased pain and proptosis of the left eye with findings of decreased visual acuity, elevated intraocular pressure, proptosis, and complete external ophthalmoplegia. Repeat imaging revealed enlargement of the SGH with subperiosteal extension into the left orbit. He required an emergent lateral canthotomy with inferior and superior cantholysis, followed by surgical drainage of the subperiosteal and SGH. Hematologic workup for coagulopathy was negative. The authors urge point-of-care providers to consider ophthalmic evaluation for patients with large SGHs where orbital extension and vision loss may occur. Furthermore, SGH causing orbital compartment syndrome may develop in patients who have normal blood work and clotting factors.

Anterior vestibule salvaging technique to limit silicone orbital implant extrusion following evisceration.

Purpose: The anterior vestibule salvaging ('Birdcage') technique may limit orbital implant extrusion following evisceration. Methods: A 10-year retrospective chart review from 2005 to 2015 of individuals who underwent evisceration procedures utilizing the vestibule salvaging technique was performed. Results: A total of 96 patients (61 male; 35 female; average age 64 years; range 17-96 years) underwent evisceration with a technique avoiding anterior scleral relaxing incisions. Three (3.1%) patients had documented extrusion of the silicone implant. Time from evisceration to extrusion ranged from 26 to 372 days. Conclusions: Maintenance of the anterior scleral vestibule during evisceration may decrease extrusion rates after surgery compared with traditional evisceration techniques that utilize anterior relaxing incisions.

Retrospective Analysis of Physician-based Surveys Published in OPRS.

PURPOSE: To review physician-based clinical surveys published in Ophthalmic Plastic and Reconstructive Surgery. METHODS: Complementary Ovid and PubMed searches of Ophthalmic Plastic and Reconstructive Surgery journal content were performed for the term "survey." Results were narrowed to studies that specifically addressed physicians' clinical practices. This search resulted in 162 articles, and after dual-investigator independent screening, 13 surveys met inclusion criteria. RESULTS: Of the 13 surveys published from 2007 to January 2017, 6 were published since 2015, showing an increased trend in survey-based publications. Topics included assessing practice patterns regarding eyelid disorders, thyroid eye disease, optic nerve sheath fenestration, anophthalmic socket, and diagnosing lacrimal disorders. Average response rate was 38.7% (range 17.5-60%), with 201 average number of replies (range 72-310). Nine out of 13 surveys included some form of statistical analysis with the remainder presenting data in percentages. CONCLUSIONS: There has been an increased rate of survey-type publications in Ophthalmic Plastic and Reconstructive Surgery over the past 10 years. The low response rate and frequent lack of statistical analysis raise concerns regarding the validity and usefulness of such studies. The authors believe that survey studies can be improved through better standardization and the use of author guidelines. They have made specific recommendations to improve the impact of survey papers in the future.

Targeting Transforming Growth Factor-ß Signaling in Primary Open-Angle Glaucoma.

Transforming growth factor-ß (TGF-ß) may play a role in the pathogenesis of primary open-angle glaucoma (POAG). Elevated levels of TGF-ß are found in the aqueous humor and in reactive optic nerve astrocytes in patients with glaucoma. In POAG, aqueous humor outflow resistance at the trabecular meshwork (TM) leads to increased intraocular pressure and retinal ganglion cell death. It is hypothesized that TGF-ß increases outflow resistance by altering extracellular matrix homeostasis and cell contractility in the TM through interactions with other proteins and signaling molecules. TGF-ß may also be involved in damage to the optic nerve head. Current available therapies for POAG focus exclusively on lowering intraocular pressure without addressing extracellular matrix homeostasis processes in the TM. The purpose of this review is to discuss possible therapeutic strategies targeting TGF-ß in the treatment of POAG. Herein, we describe the current understanding of the role of TGF-ß in POAG pathophysiology, and examine ways TGF-ß may be targeted at the levels of production, activation, downstream signaling, and homeostatic regulation.

A Case of Orbital Histoplasmosis.

Histoplasma capsulatum var capsulatum is a dimorphic fungus endemic to the Ohio and Mississippi River Valleys of the United States. In this case report, a 33-year-old woman who presented with a right orbital mass causing progressive vision loss, diplopia, and facial swelling is described. Lateral orbitotomy with lateral orbital wall bone flap was performed for excisional biopsy of the lesion. The 1.5 × 1.8 × 2.3 cm cicatricial mass demonstrated a granulomatous lesion with necrosis and positive staining consistent with Histoplasma capsulatum var capsulatum infection. To the authors' knowledge, this is the first case of orbital histoplasmosis to be reported in the United States and the first case worldwide of orbital histoplasmosis due to Histoplasma capsulatum var capsulatum.

Ocular Trauma From Dog Bites: Characterization, Associations, and Treatment Patterns at a Regional Level I Trauma Center Over 11 Years.

PURPOSE: Canine bites frequently result in periocular injury. The authors aimed to further characterize the dog breeds, types of injuries inflicted, and treatment outcomes. METHODS: A retrospective chart review was performed on all dog bites recorded in the University of Washington trauma registry from 2003 to 2013. Cases involving ocular injury were further investigated to identify ocular tissues affected, treatment patterns, and outcomes. RESULTS: A total of 342 dog bite victims were identified, of whom 91 sustained ocular trauma (27%). The mean age of patients with ocular injuries was significantly lower than those without (14.1 ± 1.9 vs. 30.0 ± 1.3 years, p < 0.001). Children bitten by dogs were 4.2 times more likely to sustain ocular injuries than adults (45.2% vs. 10.8%). The most common breed of dog inflicting ocular injury was the pit bull (25%). Forty percent of patients with ocular trauma sustained canalicular lacerations and epiphora was noted in only 3 patients (8%) after repair. Three percent had orbital fractures and 2% sustained ruptured globes. Infections were rare, affecting only 2% of patients. CONCLUSIONS: To our knowledge, this study is the largest to date to report the incidence and characteristics of ocular injuries sustained from dog bites. These injuries were disproportionately more common in children and have a high incidence of canalicular laceration. Though rare, globe injuries and orbital fractures were seen in this population. Importantly, this study establishes that pit bulls are the most frequent breed associated with ocular injuries from dog bites.

The role of transforming growth factor ß in glaucoma and the therapeutic implications.

Glaucoma is a progressive optic neuropathy frequently associated with elevated intraocular pressure, ocular vascular changes and extracellular matrix remodelling at the optic nerve head and in the trabecular meshwork. The pathogenesis is multifactorial and complex, but many recent studies have suggested that transforming growth factor-ß (TGF-ß) plays a major role in the process. Significantly elevated levels of TGF-ß have been identified in the anterior chamber of glaucomatous eyes. TGF-ß has also been shown to directly cause increased intraocular pressure. It is believed that this occurs through complex interaction with the trabecular meshwork, leading to decreased aqueous humour outflow. These processes occur through specific interactions with various proteins and signalling molecules also present in ocular tissues. By understanding the role that TGF-ß plays in the pathogenesis of glaucoma, alternative therapeutic agents can be developed, which target these pathways and improve and assist in the management of disease. This review will cover previous investigative studies and discuss the current understanding of TGF-ß's role in glaucoma and how it may serve as a potential therapeutic target.