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.

Infection of a Nylon Foil Orbital Implant Due to Fusarium brachygibbosum and Lomentospora prolificans After Intranasal Methamphetamine Use.

The authors describe a case of nylon foil implant infection caused by Fusarium brachygibbosum , and Lomentospora prolificans following medial orbital wall fracture repair in the setting of postoperative nasal methamphetamine use. A 61-year-old male presented with OS pain and swelling after a physical assault on his face. A CT of maxillofacial bones without contrast showed a moderately comminuted fracture of the medial wall of the left orbit with depression of fracture fragments into the left ethmoid air cells. Six days after repair of the medial wall fracture, the patient returned with a new onset headache, OS pain, and swelling to the left medial canthal area. He reported snorting methamphetamine approximately 48 hours before his current presentation. CT imaging showed fat stranding and soft tissue density in the extraconal space adjacent to the left medial rectus muscle and chronic fracture deformity of lamina papyracea with approximately 4 mm of medial displacement of the fracture fragments. The patient showed little clinical improvement after 48 hours of intravenous antibiotics, which led to the removal of the nylon foil implant by a left orbitotomy. Intraoperative tissue cultures grew coagulase-negative Staphylococcus , F. brachygibbosum , and Lomentospora (Scedosporium) prolificans . The patient was subsequently transitioned to oral clindamycin 600 mg three times daily and voriconazole 200 mg two times daily. To the authors' knowledge, this is the first case report to document an association between snorted methamphetamine and a fungal infection of an orbital implant.

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.

Reconstruction of Large Full-Thickness Lower Eyelid Defects With Remnant Eyelid Rotation, Semicircular Skin and Muscle Flap, and Lateral Tarsoconjunctival Flap.

PURPOSE: To describe and assess the results of reconstruction of large, full-thickness defects of the central or medial lower eyelid using a semicircular skin flap, rotation of the remnant lateral eyelid, and a lateral tarsoconjunctival flap. METHODS: The surgical approach is described, and the authors performed a retrospective chart review of consecutive patients between 2017 and 2023 reconstructed with this technique. Outcomes were assessed for size of eyelid defect, vision, subjective symptoms, facial and palpebral aperture symmetry, eyelid position and closure, corneal examination, surgical complications, and need for subsequent surgical intervention. Postoperative appearance was graded on malposition, distortion, asymmetry, contour deformity, and scarring (MDACS). RESULTS: Charts of 45 patients were identified. The average size of the lower eyelid defect was 18 mm (ranging from 12 to 26 mm). Facial and palpebral aperture symmetry were acceptable and all patients had preserved visual acuity and eyelid position and closure. The MDACS cosmetic score was perfect (0) in 15.6% (7/45) of eyelids, good (1-4) in 80.0% (36/45) of eyelids, and mediocre (5-14) in 4.4% (2/45) of eyelids. Second stage reconstruction was not necessary in 32 (71.1%) cases. There were no serious surgical complications, but minor complications included redness of the eyelid margin and pyogenic granulomas. CONCLUSIONS: Medial rotation of the remnant lower eyelid with a lateral semicircular skin and muscle flap over a lateral tarsoconjunctival flap was very effective in this series. Benefits include scarring within the facial skin tension lines, maintained vision throughout the recovery period, no eyelid retraction, and often single stage reconstruction.

Metastatic Epithelioid Sarcoma of the Eyelid in a 47-Year-Old Man: A Case Report.

Epithelioid sarcoma is a rare soft tissue neoplasm of uncertain differentiation that typically affects the distal extremities. Primary orbital epithelioid sarcoma is rare, and no reports exist characterizing metastases of this tumor to the orbit and ocular adnexa. In this article, the authors describe a rare case of eyelid metastasis in a 47-year-old man with epithelioid sarcoma of the right fibula diagnosed 16 months earlier who was otherwise doing well on the adjuvant tazemetostat therapy. In addition, the authors perform a retrospective review of cases of primary orbital epithelioid sarcoma reported in the literature, of which 4 patients responded favorably to surgical excision whereas 2 others eventually died as a direct result of their disease.

Three-Dimensional Analysis of Tear Trough Volume After Lower Blepharoplasty with Midface Lift.

Background: This study aims to evaluate three-dimensional stereophotogrammetry measured volume changes in the tear trough area after lower blepharoplasty with midface lift with or without orbital fat manipulation. Objective: Among patients undergoing midface lift surgery, volume change by three-dimensional photography in the tear trough region was compared between those having no orbital fat manipulation, fat transposition, or fat resection. Methods: This is a prospective study of 260 eyelids of 130 patients who underwent cosmetic midface surgery. All patients had an external skin muscle flap midface lift. During the procedure, medial inferior orbital fat was resected, repositioned to a preperiosteal dissection pocket, or not manipulated. Forty-six patients (35.4%) had no fat manipulation, 42 patients (32.3%) had fat preservation with repositioning to the preperiosteal pockets on the medial inferior orbital rim, and 42 patients (32.3%) had fat resection without repositioning. Results: All patients had volume gain in tear trough volume. The amount was not statistically significantly different between fat treatment groups. Mean follow-up time was 12 months (range 6-20 months). Conclusions: Midface lifting improved tear trough volume irrespective of how fat was manipulated.

Kimura Disease of the Periorbita and Earlobe in a 15-Year-Old Male: A Case Report and Review of the Literature.

PURPOSE: The authors describe a case of Kimura disease (KD) affecting the lacrimal gland, the ipsilateral periorbita, and the contralateral earlobe. The authors also review the literature for characteristics and outcomes of KD affecting the periorbita or earlobe. METHODS: The authors review retrospectively a single chart and abstract data from the English language literature. RESULTS: A 15-year-old Samoan male presented with a left upper eyelid mass and later the contralateral earlobe and ipsilateral periorbita. Surgical resection was partially effective and normal vision and eye motility were maintained. Histopathology was consistent with KD. Thirty-seven cases of periorbital KD were identified in the literature. The average age of patients with periorbital KD was 31.9 years (standard deviation: 17.8 years), with 81.1% (30/37) of patients being male and 69.4% (25/36) Asian. Over half of periorbital KD patients had lacrimal gland involvement (51.3%; 19/37). Fifteen patients were initially treated with surgery; 3 (20%) had recurrence of the disease. Another 15 patients were initially treated with corticosteroids; 12 (80%) had recurrence of the disease. Ophthalmologic data, when reported, demonstrated that, most patients had no visual (77.8%; 14/18 patients) or motility disturbances (82.4%; 14/17 patients) but most had proptosis 71.4% (10/14). Only 4 cases of earlobe involvement in KD were identified, all in women. CONCLUSIONS: In the literature, orbital KD was rare but often affected the lacrimal gland and caused proptosis, commonly in young adult Asian males. Vision and extraocular motility were usually normal. Earlobe involvement was very rare. This unique case of KD that affected the orbit and contralateral earlobe corroborates the prior literature that KD is prone to recurrence but may not adversely affect vision or extraocular motility.

Oculofacial plastic surgeon distribution by county in the United States, 2021.

PURPOSE: To characterize the number of oculofacial plastic surgeons (OPS) per county in the United States (U.S.). METHODS: The 2021 public databases of the American Society of Ophthalmic Plastic and Reconstructive Surgery and the American Academy of Ophthalmology were used to identify all OPS in the U.S. Surgeon practice location was used to determine per capita physician density by county. RESULTS: A total of 1184 OPS in the U.S. were identified. Three hundred forty-eight counties were served by at least one OPS whereas 2795 counties (89%), and two states, North Dakota and Wyoming, had no OPS. The average ratio of OPS to 100,000 population was 0.3572 (1 per 279,955). Of the counties with at least one OPS, the average was 0.5860 surgeons per 100,000 population (1 per 170,648), ranging from 0.0705 (1 per 1,418,440) to 11.26 (1 per 8,881) per 100,000. The counties with the greatest OPS density were Pitkin County, CO (1 per 8,881), San Juan County, WA (1 per 17,580), and Montour County, PA (1 per 18,231). Counties with the lowest density of those with at least one OPS were Bronx County, NY (1 per 1,418,238), San Bernardino County, Ca (1 per 1,090,037), and Gwinnett County, GA (1 per 936,329). The counties with the most OPS were Los Angeles County, CA (46), New York County, NY (38), and Cook County, IL (25). CONCLUSIONS: Geographic disparities in OPS distribution exist in the U.S. Future investigations of OPS supply according to population and other characteristics for demand may be useful.

Homeopathic Agents or Vitamins in Reducing Ecchymosis after Oculofacial Surgery: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the published literature to determine the efficacy and safety of homeopathic agents or vitamins in reducing ecchymosis after oculofacial surgery or laser surgery. METHODS: A literature search was conducted in the PubMed database initially in December 2019 and updated in March 2020 to identify all studies in the English language literature on the use of homeopathic agents or vitamins in oculofacial procedures, including laser surgery. The search yielded 124 citations, and 11 articles met all inclusion criteria for this assessment. A panel methodologist then assigned a level of evidence rating for each study. Eleven studies met inclusion criteria; 9 were rated level I, and 2 were rated level III. RESULTS: The agents studied in the articles identified included oral or topical Arnica montana (AM), oral Melilotus extract, topical vitamin K oxide, and topical AM combined with Rhododendron tomentosum. Metrics to describe ecchymosis varied. In 7 controlled studies, perioperative AM provided no or negligible benefit versus placebo. In 2 studies, vitamin K cream was equivalent to placebo. One study of oral Melilotus extract had less ecchymosis compared with controls in paranasal and eyelid ecchymosis at postoperative day (POD) 7, but not at PODs 1 and 4. A lone cohort study of combined topical AM and R. tomentosum lacked objective metrics and adequate controls. No serious side effects from administration of homeopathic agents or vitamins were identified. CONCLUSIONS: The current literature does not support the use of AM, vitamin K oxide, R. tomentosum, or Melilotus extract for reducing ecchymosis after oculofacial surgery or pulsed dye laser surgery.

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.

A Prospective Randomized Study of Lateral Brow-Eyelid Complex Volume after Internal Browpexy Using Three-Dimensional Stereophotogrammetry.

Background: A full lateral brow-eyelid complex may help rejuvenate and feminize the face. Objectives: To compare periorbital volume change of patients as measured by three-dimensional (3D) stereophotogrammetry before and after blepharoplasty with or without internal browpexy sutures. Methods: This is a prospective randomized controlled study of 124 eyelids of 62 adult female patients who underwent cosmetic upper eyelid blepharoplasty by a single surgeon. Subjects were randomized to blepharoplasty alone (control) or blepharoplasty with an adjunctive "brassiere suture" internal browpexy (intervention). An observer masked to the intervention measured the brow-eyelid volume using standardized images obtained with a 3D camera, before and after surgery. The minimum follow-up was 6 months postoperatively. Results: Sixty eyelids (30 patients) were treated with upper blepharoplasty alone and 64 eyelids (32 patients) had blepharoplasty plus internal browpexy. The mean volume gain was 0.85 mL in the internal browpexy intervention group and 0.19 mL in the control group. The volume increased in all patients receiving browpexy and this change was greater than after blepharoplasty alone (p < 0.05). The mean follow-up time was 16.8 months (range 6-24 months). Conclusions: Adjunctive internal browpexy during blepharoplasty was associated with increased volume of the lateral brow-eyelid complex.

Carotid-Cavernous Sinus Fistula Masquerading as Thyroid Eye Disease.

A 29-year-old man with a medical and social history notable for smoking presented with progressive orbital congestion, conjunctival injection, and extraocular muscle enlargement consistent with thyroid eye disease (TED). On ophthalmologic examination, tortuous episcleral vessels and blood in Schlemm's canal on gonioscopy clued an alternative diagnosis. Cavernous sinus enhancement on computed tomography also suggested a retro-orbital process. Digital subtraction angiography confirmed a low-flow indirect carotid-cavernous fistula (CCF). He subsequently underwent endovascular embolization treatment. Ocular symptoms resolved by seven weeks, and he remained ocular symptom free at six months. Eye redness and proptosis frequently cause patients to seek medical attention. In the absence of a mass or signs of infection, TED is high on the differential, especially with a smoking history and even with normal thyroid parameters. However, CCF may lurk; the authors describe key diagnostic features and management.

A Digital Microscreen for the Enhanced Appearance of Ocular Prosthetic Motility (an American Ophthalmological Society Thesis).

PURPOSE: This study aims to improve the apparent motility of ocular prosthetic devices using technology. Prevailing ocular prostheses are acrylic shells with a static eye image rendered on the convex surface. A limited range of ocular prosthetic movement and lack of natural saccadic movements commonly causes the appearance of eye misalignment that may be disfiguring. Digital screens and computational systems may obviate current limitations in eye prosthetic motility and help prosthetic wearers feel less self-conscious about their appearance. METHODS: We applied convoluted neural networks (CNNs) to track pupil location in various conditions. These algorithms were coupled to a microscreen digital prosthetic eye (DPE) prototype to assess the ability of the system to capture full ocular ductions and saccadic movements in a miniaturized, portable, and wearable system. RESULTS: The CNNs captured pupil location with high accuracy. Pupil location data were transmitted to a miniature screen ocular prosthetic prototype that displayed a dynamic contralateral eye image. The transmission achieved a full range of ocular ductions and with grossly undetectable latency. Lack of iris and sclera color and detail, as well as constraints in luminosity, dimensionality and image stability limited the real eye appearance. Yet, the digitally rendered eye moved in the same amplitude and velocity as the native, tracked eye. CONCLUSIONS: Real-time image processing using CNNs coupled to microcameras and a miniscreen DPE may offer improvements in amplitude and velocity of apparent prosthetic eye movement. These developments, along with ocular image precision, may offer a next-generation eye prosthesis. NOTE: Publication of this article is sponsored by the American Ophthalmological Society.