Topical Application of Perchlorate: A Toxicity Study in Rats.

PURPOSE: To investigate the ocular safety profile of topical perchlorate as a potential preventive treatment for nasolacrimal obstruction associated with excessive use of radioactive iodine therapy. METHODS: Nine Wistar male rats (18 eyes) were randomly assigned to receive an ocular application (topical eye drop on the OD, 3 times a day for 5 days) consisting of either: 1) sterile saline solution, 2) 30 mg/ml NaClO4 or 3) 30 mg/ml KClO4. The rat eyes were examined daily for corneal cloudiness/clarity, discharge, mucous secretions, conjunctival injection, eyelid erythema, and/or changes in behavior. Seven days after the first dose, the rats were euthanized and OU were harvested, fixed, embedded in paraffin, and stained with H&E and Masson's trichrome using standard techniques. RESULTS: The data collected over the 7 days revealed no behavior changes or ocular complications in any of the 3 study groups. Pathologic analysis of the corneas revealed normal findings on all groups without signs of inflammation, fibrosis, or any other abnormality, and no difference between the treated and control eyes. CONCLUSIONS: The findings of this study suggest that the use of topical perchlorate is safe to use on eyes in high concentrations. The efficacy of this compound in minimizing fibrosis of the nasolacrimal sac and duct warrants further study.

Transcutaneous retrobulbar amphotericin B for rhino-orbital-cerebral mucormycosis: a multi-center retrospective comparative study.

PURPOSE: To assess whether transcutaneous retrobulbar amphotericin B injections (TRAMB) reduce exenteration rate without increasing mortality in rhino-orbital-cerebral mucormycosis (ROCM). METHODS: In this retrospective case-control study, 46 patients (51 eyes) with biopsy-proven ROCM were evaluated at 9 tertiary care institutions from 1998 to 2021. Patients were stratified by radiographic evidence of local orbital versus extensive involvement at presentation. Extensive involvement was defined by MRI or CT evidence of abnormal or loss of contrast enhancement of the orbital apex with or without cavernous sinus, bilateral orbital, or intracranial extension. Cases (+TRAMB) received TRAMB as adjunctive therapy while controls (-TRAMB) did not. Patient survival, globe survival, and vision/motility loss were compared between +TRAMB and -TRAMB groups. A generalized linear mixed effects model including demographic and clinical covariates was used to evaluate the impact of TRAMB on orbital exenteration and disease-specific mortality. RESULTS: Among eyes with local orbital involvement, exenteration was significantly lower in the +TRAMB group (1/8) versus -TRAMB (8/14) (p = 0.04). No significant difference in mortality was observed between the ±TRAMB groups. Among eyes with extensive involvement, there was no significant difference in exenteration or mortality rates between the ±TRAMB groups. Across all eyes, the number of TRAMB injections correlated with a statistically significant decreased rate of exenteration (p = 0.048); there was no correlation with mortality. CONCLUSIONS: Patients with ROCM with local orbital involvement treated with adjunctive TRAMB demonstrated a lower exenteration rate and no increased risk of mortality. For extensive involvement, adjunctive TRAMB does not improve or worsen these outcomes.

Teprotumumab-related Cutaneous Hypersensitivity Reactions.

The authors report 4 cases of cutaneous hypersensitivity reactions developing in the course of teprotumumab treatment for thyroid eye disease. The onset of the cutaneous hypersensitivity reaction was also observed during the treatment course in all cases, between the second and fifth infusions. Teprotumumab-related cutaneous reactions suggest a possible immunogenic component of the monoclonal antibody and highlight the importance of close monitoring during treatment.

Outcomes of Patients With Thyroid Eye Disease Partially Treated With Teprotumumab.

PURPOSE: In response to the coronavirus (COVID-19) pandemic, teprotumumab production was temporarily halted with resources diverted toward vaccine production. Many patients who initiated treatment with teprotumumab for thyroid eye disease were forced to deviate from the standard protocol. This study investigates the response of teprotumumab when patients receive fewer than the standard 8-dose regimen. METHODS: This observational cross-sectional cohort study included patients from 15 institutions with active or minimal to no clinical activity thyroid eye disease treated with the standard teprotumumab infusion protocol. Patients were included if they had completed at least 1 teprotumumab infusion and had not yet completed all 8 planned infusions. Data were collected before teprotumumab initiation, within 3 weeks of last dose before interruption, and at the visit before teprotumumab reinitiation. The primary outcome measure was reduction in proptosis more than 2 mm. Secondary outcome measures included change in clinical activity score (CAS), extraocular motility restriction, margin reflex distance-1 (MRD1), and reported adverse events. RESULTS: The study included 74 patients. Mean age was 57.8 years, and 77% were female. There were 62 active and 12 minimal to no clinical activity patients. Patients completed an average of 4.2 teprotumumab infusions before interruption. A significant mean reduction in proptosis (-2.9 mm in active and -2.8 mm in minimal to no clinical activity patients, P < 0.01) was noted and maintained during interruption. For active patients, a 3.4-point reduction in CAS ( P < 0.01) and reduction in ocular motility restriction ( P < 0.01) were maintained during interruption. CONCLUSIONS: Patients partially treated with teprotumumab achieve significant reduction in proptosis, CAS, and extraocular muscle restriction and maintain these improvements through the period of interruption.

Treatment of Orbital Desmoid-type Fibromatosis With Sorafenib.

Desmoid-type fibromatosis is a rare tumor, particularly in the orbit, with fewer than 10 cases of primary orbital desmoid-type fibromatosis reported in the literature. The authors present a case of an infant who presented with rapid onset of OD proptosis, disc edema, and hyperopic shift who was found to have a retrobulbar desmoid-type fibromatosis. After initial biopsy, due to risk of vision loss with complete excision, the tumor was treated with sorafenib, a tyrosine kinase inhibitor. During the course of treatment with sorafenib, the tumor stabilized and then regressed in size. To the authors' knowledge, this is the first reported case of orbital desmoid-type fibromatosis to be treated with sorafenib.

The effect of visually significant dermatochalasis and blepharoptosis on driving safety.

OBJECTIVE: To evaluate the impact of visually significant blepharoptosis and dermatochalasis on driving safety and performance. Patients with visually significant blepharoptosis often complain of difficulty with driving, but the impact of blepharoptosis on driving has not been evaluated in a real-world setting. METHODS: Retrospective cohort study of 610 consecutive adult patients undergoing bilateral functional blepharoplasty or ptosis repair surgery at a single, University-based practice between 2014 and 2017. This cohort had a median age of 65 and was predominantly female. Pre-operative rates of motor vehicle collisions (MVCs) and moving violations (MVs) extracted from state Department of Transportation (DOT) records were compared with post-operative rates, using each patient as their own control. Poisson models were used for analysis. RESULTS: Subjects were included in the study for a median of 730 days before eyelid surgery (IQR 346 - 730) and 783 days after surgery (IQR 449 - 1176). There were 30 total MVCs before surgery (0.024 per subject, per year) and 48 after surgery (0.036 per subject, per year) (p = 0.08). There were 81 MVs before surgery (0.065 per subject, per year) and 66 after (0.049 per subject, per year) (p = 0.11). The multivariable model comparing MVCs pre- versus post-surgery adjusting for age, ptosis severity, gender, and comorbidities yields a rate ratio of 0.63 (p = 0.05). The multivariable model comparing MVs pre- versus post-surgery demonstrates a rate-ratio of 1.2 (p = 0.20). Older age was associated with lower rates of moving violations (coefficient of -0.03, p < 0.01). None of the other variables included in the final models had a significant association with MVCs or MVs. CONCLUSIONS: In this cohort, visually significant blepharoptosis and dermatochalasis were not associated with rates of MVCs or MVs. Further work is needed to study the impact of these common conditions on driving, for example adjusting MVC and MV rates by miles driven, which may influence decisions about when to operate on ptotic eyelids.

Silicone Scaffold Support Using a Bilayer Dermal Regeneration Matrix Template for Correction of Primary or Recurrent Eyelid Retraction.

PURPOSE: To evaluate the efficacy and safety of a bilayer dermal regenerative matrix for primary or complex/recurrent eyelid retraction. METHODS: Retrospective review of patients undergoing eyelid retraction repair using the bilayer dermal regenerative matrix from 2005 to 2019. Nineteen eyelid surgeries from 15 patients were identified. Collected data included patient demographics, symptoms, preoperative/postoperative lower eyelid position, inferior scleral show, lagophthalmos, etiology of retraction, history of prior retraction surgeries, major/minor complications, and follow-up duration (minimum 6 months). Postoperative measurements were taken at a minimum of 1 week, 3-6 weeks, 2-4 months, and 6 months. RESULTS: Postoperatively, 90% of cases had good improvement of lower eyelid retraction (defined as 1 mm or less below the inferior limbus). Postoperative elevation of the lower eyelid ranged 1-3.5 mm compared with preoperative measurements. When used in the upper eyelid for conjunctival scarring, the implant improved the superior fornix depth. Complications were minimal and included transient conjunctival injection, eyelid edema, and foreign body sensation. No patients requested early removal of the silicone layer due to ocular pain. CONCLUSIONS: The bilayer dermal regeneration matrix template may be considered a reasonable alternative to other spacers to reduce the vertical palpebral fissure and eyelid malposition in primary, complex, or recurrent cases. It also worked well for first-line correction of thyroid retraction, which tend to be more challenging due to globe proptosis. Suboptimal results may have occurred due to active cicatrizing conjunctival disease, or residual negative vector of the cheek and orbit. High cost may be a consideration, and the bilayer dermal regeneration matrix template was not studied directly against hard palate and other spacer materials.

Inverted Papilloma of the Orbit and Nasolacrimal System.

PURPOSE: Periocular inverted papilloma (IP) is a rare, locally aggressive tumor with a propensity for recurrence and malignant transformation. Historically treated via wide excision, this study examines the characteristics and management of periocular IP, comparing those confined to the nasolacrimal system with those invading the orbit. METHODS: An Institutional Review Board-approved, Health Insurance Portability and Accountability Act-compliant retrospective, comparative case series was conducted in patients with IP of the orbit or nasolacrimal system across 15 clinical sites. RESULTS: Of 25 patients, 22 met inclusion criteria with 9 limited to the nasolacrimal system and 13 invading the orbit. Mean age was 60.4 years, 55% were women, all were unilateral. Mean follow-up was 48 months. Rates of smoking, dust and/or aerosol exposure, human papillomavirus (HPV) status, and inflammatory polyps were elevated compared to rates in the general population (45%, 18%, 18%, and 14%, respectively). Bony erosion on computed tomography scans was statistically significantly associated with orbit-invading IP (p = 0.002). Treatment involved all confined IP undergoing surgery alone while 39% of orbit-invading IP also received radiation therapy and/or chemotherapy (p = 0.054). Orbit-invading IP was more likely to be excised with wide margins than IP confined to the nasolacrimal system (85% vs. 22%, p = 0.007). Overall rates of malignancy, recurrence, and patient mortality from IP were found to be 27%, 23%, and 9%, respectively. CONCLUSIONS: IP invading the orbit typically requires aggressive therapy, while IP confined to the nasolacrimal system may be treated more conservatively. Using risk factors, characteristics, and outcomes, a treatment algorithm was created to guide management.

Isolated Ophthalmoplegia Following Filler Injections to the Upper Face.

Ophthalmoplegia following cosmetic facial filler injections is a rare but serious complication. The authors report 2 cases of ophthalmoplegia following filler injection. In the first case, a 54-year-old female presented with acute onset headache, vomiting, and diplopia during malar and temporal injection of hyaluronic acid. In the second case, a 37-year-old female presented with binocular diplopia that developed following injection of an unknown filler to the upper face. Neither of the 2 patients had skin necrosis or ocular abnormalities other than motility deficits. To the authors' knowledge, there have been no other cases of isolated ophthalmoplegia without evidence of other ocular injuries following facial filler.

Capillary Hemangioma Presenting as a Scleral Vascular Lesion in a Child.

A 7-year-old healthy girl presented for an evaluation of a left vascular scleral mass. The lesion appeared spontaneously with no history of trauma, coagulopathy, or topical medication use. It was nontender, enlarging, and did not extend intraocularly. Her OS vision was 20/20, and the remainder of her eye examination was normal. Evaluation of the ocular mass included B-scan ultrasound, ultrasound biomicroscopy, anterior segment optical coherence tomography (OCT), and orbital MRI. The anterior segment OCT demonstrated vessels within the mass with no defined capsule. The orbital MRI confirmed a lesion isolated to the scleral layers of the globe, with low blood flow. The patient had a partial response to oral propranolol. Because the lesion vessels began to extend into her corneal endothelium, there was a concern for malignancy. A biopsy confirmed a benign intrascleral capillary hemangioma. Discontinuation of the propranolol demonstrated stability of the lesion 6 months later.

Müller's Muscle-conjunctival Resection Combined With Tarsectomy for Treatment of Congenital Ptosis.

PURPOSE: To evaluate the efficacy of Müller's muscle-conjunctival resection combined with tarsectomy for the treatment of congenital ptosis. METHODS: A retrospective, noncomparative case series was performed on 38 eyes of 36 patients who underwent Müller's muscle-conjunctival resection combined with tarsectomy for the treatment of congenital ptosis. Age range was 2-25 years (mean: 7.86 years). Follow-up measurements taken up to 4 years after procedure were compared with baseline values. RESULTS: Thirty-six patients presenting with congenital ptosis underwent Müller's muscle-conjunctival resection combined with tarsectomy. All patients had fair-to-good levator function of 5-10 mm. A mean improvement in the margin reflex distance-1 of 2.79 mm (p value < 0.0001) was noted. All cases except one achieved excellent lid height and postoperative symmetry of the eyelids. CONCLUSIONS: Müller's muscle-conjunctival resection combined with tarsectomy is a safe and effective procedure in the treatment of congenital ptosis in patients with moderate-to-good levator function. The surgery is rapid with quick recovery time. No complications were noted.The authors describe a retrospective case analysis of patients who underwent Müller's muscle-conjunctival resection combined with tarsectomy, for the treatment of congenital ptosis with moderate-to-good levator function, demonstrates excellent results.

Traumatic Ruptured Globe From a .22 Cartridge Casing in a 2-Year-Old Girl.

The authors report the case of a 2-year-old girl who presented with an acute ruptured globe secondary to penetration by the casing of a .22 caliber cartridge that was ignited by a car cigarette lighter. Although penetrating injuries are a common mechanism of ocular trauma, open globe induced by cartridge casing represents an unusual and preventable cause. [J Pediatr Ophthalmol Strabismus. 2017;54:e88-e90.].

Adult-Onset Asthma and Periocular Xanthogranulomas Associated with Systemic IgG4-Related Disease.

PURPOSE: The aim of this study was to report a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) associated with systemic IgG4-related disease (IgG4-RD). OBSERVATIONS: A 57-year-old man presented with bilateral periorbital swelling for 1 year. Histopathology of a left orbital biopsy showed fibro vascular connective tissue inundated with foamy, lipid-laden histiocytes and touton giant cells with lymphocytic inflammation. Additional stains revealed CD68 positivity, and S100 negativity. The IgG and IgG4 stained slides showed increased IgG4 positive plasma cells but did not meet the criteria for IgG4-related orbital disease. His IgG4 serology was elevated, and IgG4 staining of his tissue previously diagnosed as autoimmune sclerosing pancreatitis was found to meet the criteria for IgG4-RD. CONCLUSIONS AND IMPORTANCE: AAPOX can be associated with systemic IgG4-RD.

Osseointegrated implants for orbito-facial prostheses: Preoperative planning tips and intraoperative pearls.

PURPOSE: Implant-retained facial prostheses are becoming increasingly sophisticated. We describe our experience with successful implant placement. METHODS: Retrospective case series. Patients with severe unilateral orbital deformity who underwent socket reconstruction with placement of orbital implants were identified. Data on patient age, gender, mechanism of eye, soft tissue, and bone loss, prior reconstructive surgeries and radiation, and orbital imaging were collected and analyzed. RESULTS: Four patients (9 implants) between 2010 and 2014, who had osseointegrated implants placed for orbito-facial prostheses were identified. Three were male, one female. Average age was 59 years (range 34-86). Reason for eye loss was trauma in two patients, exenteration for recurrent rhabdomyosarcoma in one patient, and enucleation for retinoblastoma in one patient. All patients had Vistafix® (Gothenburg, Sweden) osseointegrated titanium implants (4 mm) placed in a 2-stage procedure over a span of 3-6 months with subsequent successful prosthesis fitting. CONCLUSION: Implant-retained orbito-facial prostheses are safe, easy, and reliable. The ideal socket has minimal dead space, robust bone, and soft tissue 4-5 mm in depth. Preoperative planning should consist of: 1) orbit CT; 2) careful clinical exam of the orbital deformity; and, 3) analysis of socket topography. Operative tips for successful implant placement include: 1) 2-3 points of fixation; 2) placement of implants in bone of adequate thickness; and 3) implant placement as a 2-stage rather than 1-stage procedure. There appears to be no difference in outcome in irradiated and non-irradiated sockets in this series, but should be a consideration and discussed with the patient.

Ocular trauma resulting in enucleation: A 12-year experience from a large regional institution.

PURPOSE: To review the frequency and cause of traumatic enucleation at the University of Wisconsin. METHODS: A 12-year retrospective chart review (2000-2012) from the University of Wisconsin Hospital and Clinics of patients who underwent enucleation following ocular trauma with specimens submitted to the University of Wisconsin Eye Pathology Laboratory. RESULTS: A total of 188 eyes enucleated following ocular trauma were identified between 2000 and 2012. One hundred eleven (59%) cases had an identifiable mechanism of injury recorded in the medical record and were included in the final analysis. The overall median patient age was 41 years with 83.8% male. Assault was the most common reason for enucleation (n=30, 27.0%) of which 15 (13.5%) cases were related to gunshot wounds. Other causes included outdoor or recreational activities (n = 20, 18.0%), fall (n = 14, 12.6%), non-motor vehicle accidents (n = 6, 5.5%), motor vehicle accidents (n = 15, 13.5%), work-related injury (n = 15, 13.5%), and sports-related injury (n = 11, 10%). CONCLUSION: Assault is the most common cause of traumatic ocular injury leading to enucleation. Gunshot and stab wounds were responsible for the majority of these cases. Men were much more likely to undergo enucleation due to ocular trauma with the exception being that caused by falls, where the rate was nearly equal between men and women.