Oral Hedgehog Inhibitor, Vismodegib, for Locally Advanced Periorbital and Orbital Basal Cell Carcinoma: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the efficacy and safety of oral vismodegib (Erivedge; Genentech) in the management of locally advanced orbital and periorbital basal cell carcinoma (BCC). METHODS: A literature search was conducted last in September 2023 in the PubMed database for English language original research that evaluated the effect of oral vismodegib on orbital and periorbital BCC. Sixty articles were identified and 16 met the inclusion criteria. RESULTS: Most studies demonstrated high response rates, with up to 100% of patients responding to the medication in individual studies and initial complete regression occurring in up to 88% of patients. Vismodegib treatment resulted in significant reductions in tumor volume, resulting in globe preservation for most patients. However, in 12% of patients, the response was partial. Recurrences also occurred with substantial frequency, even after an initial complete response. As such, up to 79.4% of patients required surgical intervention, and up to 23% of patients still required exenteration. Use of these agents resulted in reductions in tumor volume that may delay or prevent the need for exenteration in some, but not all, patients. Importantly, molecular analysis of tissue excised after vismodegib therapy revealed persistent tumor in all patients, with frequent accumulation of mutations that may confer resistance to further hedgehog inhibitor therapy. Although most adverse events were rated as level I or II, side effects were common, with up to 100% of patients in studies experiencing at least 1 event. Muscle cramps, alopecia, weight loss, fatigue, and dysgeusia were the most common adverse events, and several patients discontinued therapy because of them. Furthermore, 1 patient died of sepsis that may have resulted from the therapy. CONCLUSIONS: Although level I and II evidence are lacking, most studies indicate a benefit from the use of oral vismodegib to treat orbital and periorbital BCC tumor volume. However, patients should be cautioned about the adverse side effects of treatment and the persistence of tumor cells with mutations that may cause long-term resistance. Use of vismodegib as short-term neoadjuvant therapy may be effective in shrinking tumor volume to reduce surgical morbidity while reducing the frequency and severity of side effects. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Intraoperative Image Guidance in Orbital and Lacrimal Surgery: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the efficacy and safety of the use of intraoperative image guidance (IIG) in orbital and lacrimal surgery. METHODS: A literature search of the PubMed database was last conducted in November 2023 for English-language original research that assessed the use of any image guidance system in orbital and lacrimal surgery that included at least 5 patients. The search identified 524 articles; 94 were selected for full-text analysis by the panel. A total of 32 studies met inclusion criteria. The panel methodologist assigned a level II rating to 2 studies and a level III rating to 30 studies. No study met the criteria for level I evidence. RESULTS: Procedures reported on were as follows: fracture repair (n = 14), neoplasm and infiltrate biopsy or excision (n = 6), orbital decompression for Graves ophthalmopathy (n = 3), dacryocystorhinostomy (n = 1), and mixed etiology and procedures (n = 8). Four studies used more than one IIG system. One study that met level II evidence criteria compared the outcomes of orbital fracture repair with IIG (n = 29) and without IIG (n = 29). Borderline better outcomes were reported in the IIG group: 2% versus 10% with diplopia (P = 0.039) and 3% versus 10% with enophthalmos (P = 0.065). The other level II study compared the repair of fractures with navigation (n = 20) and without (n = 20). The group in which navigation was used had a measured mean volume reduction of 3.82 cm3 compared with 3.33 cm3 (P = 0.02), and there was a greater measured reduction in enophthalmos in the navigation group of 0.72 mm (P = 0.001). Although the remaining 30 assessed articles failed to meet level II criteria, all alleged a benefit from IIG. No complications were reported. CONCLUSIONS: A small number of comparative studies suggest that there are improved outcomes when IIG is used in orbital fracture repair, but each study suffers from various limitations. No high-quality comparative studies exist for the management of lacrimal surgery, neoplastic disease, or decompression. Complications attributable to the use of IIG have not been identified, and IIG has not been analyzed for cost savings. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Cutaneous Mucormycosis of the Eyelid Treated With Subcutaneous Liposomal Amphotericin B Injections.

Cutaneous mucormycosis is a rare, opportunistic fungal infection that typically affects immunocompromised hosts. Current treatment consists of systemic antifungal therapy, surgical debridement, and when applicable, restoration of immune function. Despite intervention, the morbidity and mortality of invasive fungal disease remains high. There are few reports of primary or secondary cutaneous mucormycosis involving the ocular adnexa. The authors describe the course of 2 children with cutaneous mucormycosis of the eyelid treated with subcutaneous liposomal amphotericin B (LAmB) injections (3.5 mg/ml) in an off-label application as an adjunct to debridement and systemic antifungal therapy. To the authors' knowledge, these are the first 2 cases of invasive fungal disease involving the eyelid treated with subcutaneous LAmB injections, and the first reported case of disseminated fungal infection with secondary cutaneous involvement of the eyelid.

Eye Trauma in Mixed Martial Arts and Boxing.

PURPOSE: To evaluate and compare eye and face trauma in mixed martial arts (MMA) and boxing. DESIGN: Retrospective cohort study. METHODS: Data from boxing and MMA competitions were extracted from the Nevada Athletic Commission (NAC) between 2000 and 2020. Details of competitions, contestants, outcomes, and injuries were extracted. RESULTS: In total 1539 boxing injuries (from 4313 contests) and 1442 MMA injuries (from 2704 contests) were identified. Boxing had higher eye injury rates compared to MMA ( p < 0.0001), with an odds ratio of 1.268 (95% CI, 1.114-1.444). Eye trauma represented 47.63% of boxing injuries and 25.59% of MMA injuries, with periocular lacerations being the most common eye injury in both. Orbital fractures represented 17.62% of eye injuries in MMA and 3.14% in boxing contests. However, 2%-3% were retinal in both sports, and 3.27% were glaucomatous in boxing. MMA contestants had an odds ratio of 1.823 (95% CI, 1.408-2.359) for requiring physician evaluation following an eye injury compared with boxing. MMA contestants also had a higher rate of face ( p < 0.0001) and body ( p < 0.0001) injuries. For both sports, an increased number of rounds and being the losing fighter were associated with increased odds of eye and face injury. CONCLUSION: Although boxing has a higher rate of eye injuries, MMA eye injuries are more likely to require physician evaluation. MMA contestants also have a higher rate of orbital fractures and face and body trauma. A detailed postfight examination and long-term follow-up of ocular injury in combat sports will be vital in proposing reforms to prevent eye trauma.

Müller Muscle Conjunctival Resection: A Prospective Multicenter Comparison of Eyelid Height at the Immediate, 1-Week, and 3-Month Postoperative Time Points.

PURPOSE: The primary objective was to document change in postoperative marginal reflex distance-1 (MRD1) after Müller muscle conjunctival resection surgery. The secondary objective was to identify predictors of change in postoperative MRD1. METHODS: A multicenter prospective cohort study was performed on patients consecutively recruited for Müller muscle conjunctival resection. MRD1 was measured immediately after Müller muscle conjunctival resection, at the 1-week postoperative visit, and the ≥3-month postoperative visit. MRD1 at the immediate and 1-week time points were compared with MRD1 ≥3 months using descriptive statistics. Predictors of change in MRD1 were analyzed using multivariate regression analysis. RESULTS: A total of 150 patients (226 eyelids) were included. Regarding the immediate to ≥3-month interval, 53.8% of eyelids remained clinically similar (rise or fall ≤0.5 mm), 19.8% rose ≥1 mm, and 26.4% fell ≥1 mm. Regarding the 1-week to ≥3-month interval, 76.5% remained clinically similar, 17.3% rose ≥1 mm, and 6.2% fell ≥1 mm. No variable predicted change in MRD1 over either interval with both clinical and statistical significance. CONCLUSIONS: Immediate postoperative MRD1 is likely to reflect the late result in only 54% of cases. However, 1-week postoperative MRD1 is similar to the late result in 77% of cases and is highly unlikely (6%) to fall by the final visit. No variable significantly impacts change in postoperative MRD1.

Outcomes for expanded polytetrafluoroethylene strip in frontalis suspension surgery.

PURPOSE: This study evaluates surgical outcomes and complication rates of frontalis suspension with expanded polytetrafluoroethylene (ePTFE). METHODS: This retrospective cohort study reviewed all patients undergoing frontalis suspension surgery using ePTFE as the sling material from January 1 2012 to March 3 2020 by a single surgeon at a single academic center. Two different surgical techniques were evaluated in the placement of the sling material. Demographic, clinical, and operative data were extracted. Outcome data including postoperative lid height, reoperation, and complication rate were extracted for the cohort and compared between the two surgical techniques. Descriptive statistics were utilized. RESULTS: Sixty-four eyes from 49 unique patients were included in this study. Forty-three (67.2%) patients had isolated congenital blepharoptosis; 14 (21.9%) had blepharophimosis, ptosis, and epicanthus inversus syndrome (BPES); and 2 (3.1%) had cranial nerve III palsy. Fifty-one (79.7%) patients had no prior blepharoptosis surgery. Lid crease incision and stab incision techniques were utilized for 24 (37.5%) and 40 (62.5%) eyes, respectively. Overall, 21 (32.8%) eyes required reoperation with ePTFE to achieve appropriate eyelid height or contour. Only one patient experienced implant infection, requiring removal of ePTFE sling after a second reoperation. There were no cases of implant exposure or granuloma formation noted during the study period. CONCLUSION: An ePTFE strip soaked in cefazolin prior to utilization in surgery is a viable material for frontalis suspension surgery, with a lower infectious or inflammatory complication rate than previously reported. However, reoperation rate was still relatively high.

Use of Mitomycin C in Dacryocystorhinostomy: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the literature on the adjuvant use of mitomycin C (MMC) during dacryocystorhinostomy (DCR) in adults with primary nasolacrimal duct obstructions (NLDOs) to determine the efficacy in improving functional and anatomic outcomes with an acceptable level of risk. METHODS: A literature search conducted in November 2020 and updated in November 2022 yielded 137 articles. Twenty-four articles met the inclusion criteria and were rated for level of evidence by the panel methodologist. Inclusion criteria required controlled studies on the effect of MMC on outcomes of external, endoscopic endonasal, or diode laser-assisted transcanalicular DCR in adults with primary acquired nasolacrimal obstruction with 6 months minimum follow-up and at least 10 participants. RESULTS: Six of the 24 articles were rated level I evidence, 15 level II , and 3 level III. In primary external DCR, MMC significantly improved functional outcomes in 3 of 9 series. In primary endoscopic endonasal DCR, MMC significantly improved functional outcomes in 1 of 9 series. In revision endoscopic endonasal DCR, MMC significantly improved functional success in 1 of 3 series. The use of MMC did not improve outcomes statistically in any diode laser-assisted transcanalicular DCR studies. Concentrations of MMC ranged from 0.05 to 1 mg/ml, with 0.2 mg/ml used most frequently in 12 series, with duration of application ranging from 2 to 30 minutes. Ostium size was significantly larger in MMC groups than in control groups at 6 months after surgery in 4 of 5 reporting studies. However, these larger ostia did not confer higher functional success rates. Reporting of adverse events related to MMC were rare, with delayed cutaneous wound healing reported in 1 of 750 patients. CONCLUSIONS: Intraoperative use of MMC in external and endoscopic endonasal DCR has been shown to improve functional and anatomic outcomes compared with controls in some series, but there is no agreement on the recommended concentration or application time for MMC in DCR. The data support that MMC use can result in a larger ostium size, decreased granulation tissue formation, and a decreased number of postoperative nasal debridements compared with controls, but this does not translate into improved functional success. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Thermal Pulsation in the Management of Meibomian Gland Dysfunction and Dry Eye: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the literature to determine the efficacy and safety of thermal pulsation technologies in improving signs or symptoms of meibomian gland dysfunction (MGD) and dry eye compared with no therapy or with conventional warm compress therapy or eyelid hygiene. METHODS: A literature search was conducted in the PubMed database in June 2022 and again in March 2023 to identify all studies in the English language on the use of thermal pulsation to treat MGD or dry eye. The search yielded 59 citations, and 11 articles met all of the inclusion criteria. The panel methodologist then assigned a level of evidence rating for each study; 8 studies were rated level I evidence and 3 studies were rated level II evidence. RESULTS: All included studies evaluated a single 12-minute session using the LipiFlow automated thermal pulsation system (TearScience, Inc, or Johnson & Johnson). Improvements were detected in subjective and objective metrics of MGD or dry eye in patients within 1 to 12 months of thermal pulsation treatment compared with nontreatment. Most of the studies (9/11) reported greater efficacy with thermal pulsation than with standard warm compress therapy and eyelid hygiene. Four of these studies showed relevant industry conflicts of interest. Two of the 4 level I studies without direct industry participation concluded that thermal pulsation treatment was not significantly different from conventional hygiene or warm compress therapy control treatments (in symptoms in one of the studies and in objective findings in the second study). No serious adverse events were reported in any of the 11 studies. CONCLUSIONS: According to the current literature, a single thermal pulsation session may improve subjective or objective parameters of MGD and dry eye safely. However, industry support and participation were present in 4 of the 8 level I studies. The durability beyond several months and cost efficacy remain uncertain. Because the inclusion parameters of this assessment captured only the LipiFlow system, the conclusions are limited to that product. High-quality independent studies are needed to assess the long-term benefits of this intervention. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Müller Muscle Conjunctival Resection: A Multicentered Prospective Analysis of Surgical Success.

PURPOSE: This is a multicenter prospective cohort study investigating Müller muscle conjunctival resection success rates based on marginal reflex distance-1 (MRD1) and symmetry criteria. A secondary objective was to identify predictors of success. METHODS: One hundred fifty-two patients with unilateral or bilateral blepharoptosis (229 eyelids) undergoing Müller muscle conjunctival resection were consecutively recruited from 2015 to 2020 at the Université de Montréal and University of California San Francisco. Ptosis was defined as MRD1 ≤ 2.0 mm or MRD1 > 1 mm lower than the contralateral eyelid. Patients were selected for Müller muscle conjunctival resection surgery if they demonstrated significant eyelid elevation following phenylephrine 2.5% testing. MRD1 success (operated eyelid achieving MRD1 ≥ 2.5 mm) and symmetry success (patient achieving an intereyelid MRD1 difference ≤ 1 mm) were evaluated for the patient cohort. Predictors of MRD1 and symmetry success were analyzed using multivariate regression analysis. RESULTS: MRD1 success was achieved in 72.1% (n = 165) of 229 operated eyelids. Symmetry success was achieved in 75.7% (n = 115) of 152 patients. MRD1 before phenylephrine testing was the only statistically significant predictor of MRD1 success (odds ratio [OR] 2.69, p = 0.001). Symmetry following phenylephrine testing was the only variable associated with increased odds of symmetry success (OR 2.71, p = 0.024), and unilateral surgery (OR 0.21, p = 0.004), the only variable associated with reduced odds of symmetry success. CONCLUSIONS: Müller muscle conjunctival resection effectively achieves postoperative MRD1 and symmetry success. MRD1 before phenylephrine testing is the strongest determinant of MRD1 success. Neither a large rise in MRD1 with phenylephrine nor increasing tissue resection length adequately counterbalance the effect of a low MRD1 before phenylephrine. Unilateral surgery and the absence of symmetry following phenylephrine predict greater odds of symmetry failure.

Prevalence and Predictors of Hering's Response in Müller's Muscle-conjunctival Resection.

PURPOSE: Determine the prevalence and predictors of Hering's response following Muller's muscle-conjunctival resection (MMCR). METHODS: Seventy-five consecutive patients undergoing unilateral MMCR were recruited in this prospective, multicenter, cohort study. Margin-reflex distance-1 (MRD1) of both eyelids was recorded preoperatively and postoperatively. One hundred forty-three variables were investigated as potential predictors of a late postoperative (≥3 months) Hering's response using regression analyses. Main outcome measures were Hering's response (≥0.5 mm descent of the unoperated eyelid from baseline), and a clinically relevant Hering's response (descent of the unoperated from baseline to a MRD1 ≤ 2.0 mm, or descent from baseline such that the MRD1 of the unoperated eyelid became >1 mm lower than the operated eyelid). RESULTS: Twenty-four (32.0%) patients had a late postoperative Hering's response, but only 6 (8.0%) responses were clinically relevant. A Hering's response at the immediate (OR 16.24, p = 0.02) and 1-week postoperative (OR 8.94, p = 0.04) timepoints predicted a late postoperative response. However, the presence (OR 7.84, p = 0.07) and amplitude (OR 8.13, p = 0.06) of a preoperative phenylephrine Hering's response did not predict a late postoperative response. Of the 10 patients with a clinically relevant phenylephrine Hering's response, only 1 demonstrated a clinically relevant response late postoperatively. CONCLUSION: Unilateral MMCR induces a clinically relevant Hering's response in 8% of patients. A preoperative phenylephrine Hering's response does not predict a late postoperative Hering's response. Therefore, when unilateral phenylephrine testing unmasks contralateral blepharoptosis, only the side with blepharoptosis at baseline should be operated.

Augmentation of telemedicine post-operative follow-up after oculofacial plastic surgery with a self-guided patient tool.

PURPOSE: This study evaluates a web-based tool designed to augment telemedicine post-operative visits after periocular surgery. METHODS: Adult, English-speaking patients undergoing periocular surgery with telemedicine follow-up were studied prospectively in this interventional case series. Participants submitted visual acuity measurements and photographs via a web-based tool prior to routine telemedicine post-operative visits. An after-visit survey assessed patient perceptions. Surgeons rated photographs and live video for quality and blurriness; external raters also evaluated photographs. Images were analyzed for facial centration, resolution, and algorithmically detected blur. Complications were recorded and graded for severity and relation to telemedicine. RESULTS: Seventy-nine patients were recruited. Surgeons requested an in-person assessment for six patients (7.6%) due to inadequate evaluation by telemedicine. Surgeons rated patient-provided photographs to be of higher quality than live video at the time of the post-operative visit (p < 0.001). Image blur and resolution had moderate and weak correlation with photograph quality, respectively. A photograph blur detection algorithm demonstrated sensitivity of 85.5% and specificity of 75.1%. One patient experienced a wound dehiscence with a possible relationship to inadequate evaluation during telemedicine follow-up. Patients rated the telemedicine experience and their comfort with the structure of the visit highly. CONCLUSIONS: Augmented telemedicine follow-up after oculofacial plastic surgery is associated with high patient satisfaction, rare conversion to clinic evaluation, and few related post-operative complications. Automated detection of image resolution and blur may play a role in screening photographs for subsequent iterations of the web-based tool.

Magnetic Resonance Imaging Prognostic Findings for Visual and Mortality Outcomes in Acute Invasive Fungal Rhinosinusitis.

PURPOSE: To identify initial, preintervention magnetic resonance imaging (MRI) findings that are predictive of visual and mortality outcomes in acute invasive fungal rhinosinusitis (AIFRS). DESIGN: Retrospective cohort study. PARTICIPANTS: Patients with histopathologically or microbiologically confirmed AIFRS cared for at a single, tertiary academic institution between January 2000 and February 2020. METHODS: A retrospective review of MRI scans and clinical records of patients with confirmed diagnosis of AIFRS was performed. For each radiologic characteristic, a modified Poisson regression with robust standard errors was used to estimate the risk ratio for blindness. A multivariate Cox proportional hazards model was used to study AIFRS-specific risk factors associated with mortality. MAIN OUTCOME MEASURE: Identification of initial, preintervention MRI findings associated with visual and mortality outcomes. RESULTS: The study comprised 78 patients (93 orbits, 63 with unilateral disease and 15 with bilateral disease) with AIFRS. The leading causes of immunosuppression were hematologic malignancy (38%) and diabetes mellitus (36%). Mucormycota constituted 56% of infections, and Ascomycota constituted 37%. The overall death rate resulting from infection was 38%. Risk factors for poor visual acuity outcomes on initial MRI included involvement of the orbital apex (relative risk [RR], 2.0; 95% confidence interval [CI], 1.1-3.8; P = 0.026) and cerebral arteries (RR, 1.8; 95% CI, 1.3-2.5; P < 0.001). Increased mortality was associated with involvement of the facial soft tissues (hazard ratio [HR], 4.9; 95% CI, 1.3-18.2; P = 0.017), nasolacrimal drainage apparatus (HR, 5.0; 95% CI, 1.5-16.1; P = 0.008), and intracranial space (HR, 3.5; 95% CI, 1.4-8.6; P = 0.006). Orbital soft tissue involvement was associated with decreased mortality (HR, 0.3; 95% CI, 0.1-0.6; P = 0.001). CONCLUSIONS: Extrasinonasal involvement in AIFRS typically signals advanced infection with the facial soft tissues most commonly affected. The initial, preintervention MRI is prognostic for a poor visual acuity outcome when orbital apex or cerebral arterial involvement, or both, are present. Facial soft tissues, nasolacrimal drainage apparatus, intracranial involvement, or a combination thereof is associated with increased mortality risk, whereas orbital soft tissue involvement is correlated with a reduced risk of mortality.

Orbital Radiation for Thyroid Eye Disease: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the current literature on the safety and efficacy of orbital radiation for the management of thyroid eye disease (TED). METHODS: A literature search was conducted last in February 2021 of the PubMed database to identify all articles published in the English language on original research that assessed the effect of orbital radiation on TED. The search identified 55 articles, and 18 met the inclusion criteria for this assessment. A panel methodologist then assigned a level of evidence rating for each study, and all of them were rated level III. RESULTS: Two large retrospective studies demonstrated the efficacy of radiation treatment, with or without corticosteroid use, in preventing or treating compressive optic neuropathy (CON). Three studies highlighted the role of orbital radiation therapy (RT) to facilitate the tapering of corticosteroids. Several other studies showed a possible role for RT to improve diplopia and soft tissue signs. CONCLUSIONS: Although no level I or level II evidence exists, the best available evidence suggests that orbital radiation, used with or without corticosteroids, is efficacious in preventing CON, improving motility restriction, and decreasing clinical activity in TED. Orbital radiation also may facilitate a corticosteroid taper. Together, these studies show that RT seems to modify the active phase of TED. Short-term risks of orbital radiation are minor, but long-term outcome data are lacking.

Efficacy of Care and Antibiotic Use for Chalazia and Hordeola.

OBJECTIVES: To evaluate whether use of an antibiotic improves the efficacy of care for a chalazion or hordeolum. METHODS: A cross-sectional retrospective review was performed. All patients treated for a newly diagnosed chalazion or hordeolum at the University of California, San Francisco from 2012 to 2018 were identified. Patients were excluded when clinical notes were inaccessible or there was inadequate documentation of treatment modality or outcome. Patient demographics, setting of initial presentation, treatment modalities, antibiotic use, and outcomes were analyzed. RESULTS: A total of 2,712 patients met inclusion criteria. Management with an antibiotic was observed in 36.5% of patients. An antibiotic was 1.53 times (95% confidence interval [CI], 1.06-2.22, P=0.025) more likely to be prescribed in emergency or acute care setting for a chalazion. Older age was associated with a higher risk of receiving an antibiotic for a hordeolum (adjusted RR 1.07 per decade, 95% CI, 1.05-1.11, P<0.001). The addition of an antibiotic to conservative measures for a chalazion (adjusted RR, 0.97, 95% CI, 0.89-1.04, P=0.393) or hordeolum (adjusted RR, 0.99, 95% CI, 0.96-1.02, P=0.489) was not associated with an increased likelihood of treatment success. CONCLUSION: Although frequently prescribed, an antibiotic is unlikely to improve the resolution of a chalazion or hordeolum.

Plexiform Neurofibroma With Activating KRAS Mutation and Segmental Presentation Involving the Unilateral Eyelid.

Plexiform neurofibromas are classically thought to be pathognomonic for neurofibromatosis type 1. However, isolated forms may occur, particularly as a manifestation of segmental neurofibromatosis related to postzygotic mosaicism in the NF1 gene. Most cases occur on the head and neck, trunk, and extremities with very few cases reported in the periorbital area. The authors report a case of plexiform neurofibroma with perineuriomatous features of the right upper eyelid in a patient with no other stigmata of neurofibromatosis. While suggestive of segmental neurofibromatosis, genetic analysis revealed activating KRAS mutation and inactivating mutation in PHF6 with no evidence of NF1 mutation in germline or tumor tissue. Neither KRAS nor PHF6 have been previously reported in association with neurofibroma.

Multidisciplinary Frontotemporal Orbitozygomatic Craniotomy for Spheno-orbital Meningiomas: Ophthalmic and Orbital Outcomes.

PURPOSE: Advances in surgical approaches have improved access for total or near-total resection of spheno-orbital meningiomas (SOM). Herein, the outcomes of multidisciplinary resection and reconstruction of SOM via frontotemporal orbitozygomatic craniotomy at a single institution are evaluated. METHODS: A retrospective chart review was performed of all patients with SOM who underwent joint neurosurgical and oculofacial plastic resection via frontotemporal orbitozygomatic craniotomy between January 1999 and December 2018. Demographic data, clinical presentation, risk factors for meningioma, radiographic imaging, pathology results, postoperative outcomes, and surgical complications were reviewed. The outcome measures assessed included visual acuity, pupillary function, color vision, ocular motility, visual fields, and proptosis. RESULTS: A total of 48 patients were identified having had multidisciplinary frontotemporal orbitozygomatic craniotomy for SOM of which 43 met inclusion criteria. A mean follow-up period of 23.9 ± 20.4 months (range 1-60) was observed. There were 35 patients who underwent primary resection and 8 patients who had prior surgical resection. The main presenting complaints were proptosis (88%), headache (44%), and reduced vision (12%). Gross total resection was achieved in 15 patients (35%) while near-total or subtotal resection was achieved in the remainder. Histologic analysis revealed World Health Organization grade I meningioma in 72% of tumors, grade II in 23%, and grade III in 5%. Mean visual acuity (Logarithm of the Minimum Angle of Resolution) improved from 0.24 ± 0.46 preoperatively to 0.09 ± 0.13 postoperatively (p = 0.03). Surgery improved proptosis, with a reduction in mean Hertel exophthalmometry from 22.37 ± 3.78 mm to 17.91 ± 3.84 mm (p < 0.001), of which 80% had no residual proptosis or developed subsequent recurrence. Exophthalmic index calculated by radiologic evaluation also improved from a mean preoperative value of 1.32 ± 0.19 to 1.12 ± 0.13 at the 6-month interval after surgery (p < 0.001). Before surgery, 19 (45%) patients had a relative afferent pupillary reaction with improvement in 9 (24%) after surgery. Of the 14 (33%) patients with preoperative ocular motility deficit, 7 (16%) had resolution of ocular motility deficit postoperatively. The most common surgical complications were temporalis muscle atrophy with temporal hollowing (14%), wound infection (7%), neurogenic strabismus secondary to trochlear nerve palsy (5%), restrictive strabismus (5%), and aponeurotic blepharoptosis (5%). CONCLUSIONS: Multidisciplinary frontotemporal orbitozygomatic for resection of SOM is a safe and effective means of tumor removal. It can provide improved visual acuity and proptosis metrics, as well as relief of optic neuropathy and ocular motility deficits.

Patient-Specific Implants in Oculofacial Plastic Surgery.

PURPOSE: To investigate how patient-specific implants (PSIs) are being utilized for periocular facial skeletal reconstruction. Specifically, to characterize indications for custom implants, areas of reconstruction, intraoperative variables impacting implant placement, as well as to report on postoperative outcomes. MATERIALS AND METHODS: A retrospective chart review was performed for patients who received a PSI for periocular skeletal reconstruction between 2015 and 2019. Three independent academic centers were included in this study, which encompassed 4 different primary surgeons. Medical records, radiographic imaging, and operative reports were reviewed. RESULTS: Eleven patients, 8 females and 3 males, ages ranging from 15 to 63 years old received PSIs. The average duration of follow up was 16 months ± 6.6 months (range: 9-30 months). The most common underlying etiology for reconstruction was prior trauma (54.5%) followed by benign tumor resection (18.2%). The most frequent area of reconstruction involved the inferior orbital rim and adjacent maxilla (63.6%). Implant materials included porous polyethylene, polyetheretherketone, and titanium. Six implants required intraoperative modification, most commonly accommodate critical neurovascular structures (66.6%) or improve contour (33.3%). Two postoperative complications were noted, both in the form of infection with 1 implant requiring removal. CONCLUSIONS: Reconstruction of complex facial skeletal defects can be achieved by utilizing computer-assisted design software and 3D printing techniques to create PSIs. These implants represent the most customizable option for symmetric restoration of the facial skeleton by not only addressing structural deficits but also volumetric loss. This was particularly apparent in reconstruction of the orbital rim and midface. PSIs were found to be of most benefit in patients with prior trauma or complex skeletal defects after tumor resection.

Acute Invasive Rhino-Orbital Mucormycosis in a Patient With COVID-19-Associated Acute Respiratory Distress Syndrome.

Acute invasive fungal rhinosinusitis is a rare, although highly morbid, infection primarily affecting immunosuppressed individuals. The same population is at particularly high risk of complications and mortality in the setting of SARS-CoV-2 infection and coronavirus disease 2019 (COVID-19)-related acute respiratory distress syndrome. The authors present a case of acute invasive fungal rhino-orbital mucormycosis in a patient with COVID-19 and discuss the prevalence, diagnosis, and treatment of fungal coinfections in COVID-19. Prompt recognition, initiation of therapy, and consideration of the challenges of rapidly evolving COVID-19 therapy guidelines are important for improving patient survival.

The Role of Topical Antibiotic Prophylaxis in Oculofacial Plastic Surgery: A Randomized Controlled Study.

PURPOSE: The usefulness of topical antibiotic prophylaxis for routine oculofacial plastic surgery is not well established. Given concerns such as contact dermatitis, antibiotic resistance, and healthcare costs in conjunction with a low baseline rate of surgical site infections, the investigators sought to determine the frequency of infection with and without the use of topical antibiotic prophylaxis. DESIGN: Randomized, controlled, unmasked clinical trial. PARTICIPANTS: Adult patients undergoing routine periocular surgery without prior history of periocular surgical site infection, need for perioperative oral or parenteral antibiotics, or allergy to all study medications. METHODS: Participants were randomized before surgery to receive either antibiotic or placebo (mineral oil and petrolatum-based) ointment after surgery. Outcomes were measured at the first postoperative visit. The 2-tailed Fisher exact test was used to compare outcomes between groups. MAIN OUTCOME MEASURES: The primary outcome was the incidence of surgical site infections. The secondary outcomes included stratification of infections by patient risk characteristics, incidence of allergic contact dermatitis, and incidence of wound complications. RESULTS: Four hundred one participants were enrolled and randomized, and 13 participants did not proceed with surgery or were lost to follow-up. High-risk features for infection were identified in 24% of the placebo group and 21% of the antibiotic group. Surgical site infections were more common in the placebo group (2.7% vs. 0.0%; P = 0.025). The rate of contact dermatitis was similar (0.5% vs. 0.5%; P = 1.00), as was the rate of wound dehiscence (2.7% vs. 3.5%; P = 0.77). Among the placebo group, the incidence of infections in the low- and high-risk participants was 2.9% and 2.2%, respectively. Infections were treated with oral or topical antibiotics and resolved without complication, except in 1 patient who required 2 subsequent surgeries to address the sequelae. CONCLUSIONS: After routine oculofacial plastic surgery, patients treated with a topical antibiotic ointment showed a lower risk of surgical site infection compared with patients treated with a nonantibiotic ointment.

Dacryops - A review.

Purpose: To review and summarize the epidemiology, clinical presentations, histopathologic characteristics and management of dacryops.Methods: A comprehensive PubMed, Embase and Google Scholar search of all articles written in English and non-English language articles with abstract translated to English on dacryops were reviewed. Data reviewed included epidemiology, etiology, pathogenesis, clinical presentations, histopathologic characteristics and management of dacryops.Results: Lacrimal ductal cysts or dacryops are classified based on locations: palpebral lobe cysts (simple dacryops); orbital lobe cysts; cysts of the accessory lacrimal glands of Krause and Wolfring; and cysts of ectopic (choristomatous) lacrimal glands. The exact etiology of dacryops remains unknown. Dacryops is usually asymptomatic but can present with varied symptoms depending on the type and associated complications. While complete excision of dacryops is generally recommended, marsupialization is also a safe and effective method for the treatment of dacryops in the palpebral lobe of lacrimal gland. Complete surgical excision via orbitotomy is required for deep orbital lesions.Conclusion: Dacryops is an uncommon benign cystic lacrimal gland tissue neoplasm. Dacryops can be classified into four types based on locations. Lesions could be either superficial that can be seen through the fornix or deep into the orbit. Observation is also an option for small or asymptomatic lesions. Surgery remains the mainstay of treatment for symptomatic cases, but approaches are different depending on the location of the lesions.