Reverse Lid Swinging Approach: A Case Series.

In this report, the authors describe the reverse lid swinging approach, which comprises lateral canthotomy, superior lateral cantholysis to free the upper eyelid, and an incision at the superonasal conjunctival fornix for orbital exposure. Four patients underwent orbitotomy using the reverse lid swinging approach for orbital tumor removal (n = 3) or orbital abscess drainage (n = 1). All 4 operations were uneventful, with no optic nerve dysfunction or extraocular dysmotility over the follow-up period. Satisfactory cosmesis was achieved with no visible external scar in all cases. The reverse lid swinging approach provides easy access to medial orbital lesions as demonstrated in the present case series.

Atypical mycobacterium infection following upper eyelid Müller's muscle-conjunctival resection - case report.

Periorbital non-tuberculous mycobacterium (NTM) infections are uncommon. To the best of our knowledge, NTM infection as a complication following Müller's muscle-conjunctival resection (MMCR) surgery has not been reported before. We report a case of left upper lid M. Chelonae infection following MMCR surgery. A 61-year-old lady presented with left upper lid swelling and nodular mass 4 weeks after bilateral MMCR surgery for aponeurotic ptosis. Past medical and ocular history include systemic lupus erythematosus (SLE), chronic hepatitis B infection, bilateral cataract operation done 14 years ago and right eye Fuch's dystrophy with Descemet stripping automated endothelial keratoplasty done 3 years ago. She was initially treated with topical and oral antibiotics, as well as repeated incision and curettage and intralesional steroid injection with limited improvement. Seven months post-MMCR, repeated biopsy and nodule debulking were performed. Biopsy revealed granulomatous inflammation with mycobacterial infection and PCR identified M. Chelonae. A total of 6 months course of combination systemic antibiotics were given, with good response. Limited blepharoplasty with repeat nodular excision was performed 15 months after the initial MMCR surgery, and biopsy culture and PCR were both negative. No relapse of symptoms was noted and good lid height was maintained at 30 months of follow-up. Management of periorbital NTM infections can be challenging. Clinicians should consider early diagnostic workup with mycobacterial culture and PCR in suspicious cases, followed by prompt initiation of empiric treatment with systemic macrolides. A combination of surgical excision of nodules and prolonged systemic antimicrobial treatment is needed for complete organism eradication.

Giant Fornix Syndrome: How Much Conjunctiva Should Be Resected?

PURPOSE: To characterize clinical profiles of Chinese patients with giant fornix syndrome (GFS), compare surgical outcomes with amount of Müller's muscle-conjunctival resection (MMCR), and elicit risk factors for those who have prolonged recovery after MMCR. METHODS: Retrospective, observational, interventional cohort study on GFS eyes. Two treatment groups were established: limited MMCR-as defined by 8 mm or less resection; maximal MMCR-as defined by 10-12 mm resection. Good responders were defined as eyes exhibiting disease resolution within 3 months after surgery. Primary outcome was disease resolution, secondary outcome was ptosis improvement. RESULTS: Mean age was 81.9 years old (range, 76-89), with 6 (75%) females and 2 (25%) males. All 10 eyes presented with discharge, partial ptosis, and conjunctival injection. In the limited MMCR group, time to symptom resolution was longer at 5.56 months, while maximal MMCR group was 2.02 months (p = 0.004). Limited MMCR group also had lower primary surgical success and required additional surgery compared with maximal MMCR group (p = 0.008). At mean follow up of 34.4 months (range, 11-65 months), all eyes achieved disease resolution, no recurrence, and ptosis improvement. CONCLUSIONS: In the largest series on Chinese eyes with GFS to date, GFS is mainly a disease in elderly females. Maximal MMCR has a higher rate of surgical success with no additional complications. For those who underwent MMCR, additional treatment such as topical steroids and fortified antibiotics do not affect time to recovery. These findings may help ophthalmologists consider maximal MMCR as a definitive surgical treatment in GFS eyes.

Oculoplastic surgical simulation using goat sockets.

PURPOSE: To describe a reproducible and easily available goat socket model for training of various oculoplastic operations, and to evaluate trainees' perception of this training model in terms of their learning progress and satisfaction. METHODS: Goat sockets including orbital rim and eye with eyelids were harvested in form of a split-head model. Ophthalmology residents underwent individual surgical training using the goat socket model, supervised by an oculoplastic attending. Participants completed a questionnaire in form of a 5-point Likert Scale to evaluate their learning progress and satisfaction. OUTCOME MEASURES: Types of oculoplastic operations performed using the goat socket models, and participants' rating of their learning progress and satisfaction were reported. RESULTS: A wide range of oculoplastic operations including both eyelid and orbital operations could be simulated because of similarities of the goat eye model to the human eye anatomy. Fifteen ophthalmic trainees participated in surgical training using the goat eye model. All (100%) participants agreed that surgical simulation using the goat socket model increased their skills in surgical instrumentation and carrying out surgical steps, and their confidence in operating on patients. Most (87%) agreed the model resembled reasonably well compared to surgeries in human, and 93% would recommend training with the model to fellow resident ophthalmologists before operating on human patients. CONCLUSIONS: Oculoplastic surgical training using goat sockets is simple, readily available, and inexpensive. Trainee users showed promising feedback and positive learning progress using the goat socket model.

Transnasal Endoscopic Sclerotherapy for Orbital Apex Lymphatic Malformation.

Orbital lymphatic malformations are benign, slowly progressive vascular malformations. Management of these malformations is challenging due to their infiltrative and diffuse nature. The authors present a case with orbital apex lymphatic malformation treated with transnasal endoscopic sclerotherapy.

Extraocular silicone oil migration to orbit and retrolaminar region: case report and systematic review.

BACKGROUND: Silicone oil insertion is a common modality in vitreoretinal surgeries. The purpose of this study is to conduct a systematic review to summarize the uncommon complication of extraocular silicone oil migration. METHODS: Following the PRISMA guidelines, a systematic review of the literature was performed on January 11, 2020, using PubMed and EMBASE with the following terms: "silicone oil," "eye," and "migrat*." RESULTS: A total of 69 patients-68 patients from 59 articles and one case from our institution-were included in the final analysis. The median age was 54 years (range, 9-92) and 40 patients (57.9%) were men. Orbital migration was reported in 34 patients, and retrolaminar migration (including optic nerve, optic chiasm, suprasellar, subarachnoid space, intraventricular spaces) was reported in 35 patients. Orbital migration group had more aphakics (p = 0.007), implanted glaucoma drainage device (p = 0.005), scleral buckle (p = 0.000), history of trauma-related indications for pars plana vitrectomy (p = 0.000), shorter silicone oil endotamponade time (p = 0.008), more symptomatic (p = 0.000), and requiring surgical intervention (p = 0.000). Retrolaminar migration group had older patients (p = 0.016) and more diabetics (p = 0.041). CONCLUSION: Systematic review sheds light on plausible risk factors on site of silicone oil migration. Majority of orbital cases are symptomatic and require intervention while retrolaminar cases are incidental and can be managed conservatively. Awareness of this complication can help guide clinicians predict which patients would likely need surgical intervention. Graphical abstract.

Medical and sclerosing agents in the treatment of orbital lymphatic malformations: what's new?

PURPOSE OF REVIEW: Currently, there is no ideal management for orbital lymphatic malformations. Significant advances have been made since the discovery of new agents in the treatment. The purpose of this manuscript is to review the recent evidence on new sclerotherapy agents and systemic medications. RECENT FINDINGS: Traditional sclerosants are OK-432, sodium tetradecyl sulphate and ethanol. More recent developments are the use of doxycycline, bleomycin, and pingyangmycin. Sirolimus as a systemic medication has revolutionized the medical management of lymphatic malformations. Other oral drugs such as propranolol and sildenafil are controversial. Future treatment involves targeting lymphangiogenic pathways including inhibition of vascular endothelial growth factors and the phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit. SUMMARY: The development of new agents allows multimodal management either as monotherapy or combined therapy to achieve better outcomes in this difficult to manage disease.

Unilateral Eyelid Swelling as a Sign of Antimelanoma Differentiation-Associated Gene 5 (Anti-MDA5)-Antibody-Positive Dermatomyositis.

The authors describe a 40-year-old woman who presented with unilateral eyelid swelling, and later on developed heliotrope rash, extensive oral and cutaneous ulcers, and muscle weakness. She was diagnosed with dermatomyositis associated with antimelanoma differentiation-associated gene 5 antibody. Even after combination of immunosupressants including a corticosteroid, tacrolimus, and rituximab, her eyelid swelling and oral ulcers progressively worsened. Administration of loading and then monthly intravenous immunoglobulin resulted in clinical remission, suggesting that intravenous immunoglobulin could be a useful remedy in cases refractory to conventional immunosuppressive agents, especially those with antimelanoma differentiation-associated gene 5 antibody-dermatomyositis.

Inferonasal prolapsed orbital fat: report of a case and review of literature.

Prolapsed orbital fat is a common entity in the literature and is mostly located in the superotemporal quadrant. It can be confused with other conjunctival tumors. The authors describe a 56-year-old woman with a rare inferonasal prolapsed orbital fat. Excision of the lesion was performed without recurrence at 6 months.

Burnishing of exposed porous polyethylene orbital implant: is it useful?

OBJECTIVE: To describe and report the surgical outcomes of the burnishing technique for exposed porous polyethylene (PP) orbital implants. METHODS: A retrospective review of consecutive patients who underwent repair of an exposed PP orbital implant at Hong Kong Eye Hospital and Queen Elizabeth Hospital, Hong Kong, between January 2002 and April 2022 was conducted. Exposed PP orbital implants were burnished with an electric drill. The exposed area was then covered by a donor scleral graft followed by conjunctival wound closure. Patients with a shallow lower eyelid fornix would undergo additional fornix deepening procedures that mobilize the conjunctiva and provide adequate coverage of the implant. RESULTS: Six patients who had exposed PP orbital implants following enucleation (n = 4) and evisceration (n = 2) underwent repair. Five of the six patients did not experience any recurrence at an average follow-up of 25 months (range, 7-42 months). One patient who suffered from endophthalmitis experienced orbital implant re-exposure 16 months after the revision was managed by reimplantation of an acrylic implant and wrapping with both donor scleral graft and dermis fat graft. CONCLUSION: In conclusion, we described a burnishing technique for repairing exposed PP orbital implants. Our technique is effective in preventing implant re-exposure and is easy to perform.

Bilateral synchronous conjunctival myxoma and review of the literature.

PURPOSE: The purpose of this study was to report the clinicopathological features and management of the first case of bilateral synchronous conjunctival myxoma. METHODS: This study was a case report and literature review. RESULTS: A 66-year-old Chinese male with past ocular history of uncomplicated bilateral phacoemulsification and intraocular lens (IOLs) 3 years ago prior to presentation presented with bilateral red and swollen conjunctiva for over a year. On examination his corrected distance visual acuity (CDVA) was 25/20 in the right eye 20/20 in the left eye. Slit lamp examination revealed swollen temporal conjunctiva bilaterally which appeared as painless, well-circumscribed, salmon-pink, fleshy patches. The lesion in the right eye was subsequently excised, followed by excision of the lesion in the left eye at 3-week interval. Microscopically, histopathological examination of both excised specimens revealed hypocellular conjunctival mucosa covered by non-dysplastic epithelium, with presence of myxoid degeneration in the subepithelial stroma and immunostaining findings consistent with conjunctival myxoma. At his latest follow-up at 24 months, there were no recurrences of the conjunctival masses and the CDVA was the same as preoperatively.

Doxycycline sclerotherapy in patients with periorbital lymphatic malformation: A case series and literature review.

PURPOSE: To describe and report the outcomes of doxycycline sclerotherapy in patients with periorbital lymphatic malformations(LMs). BASIC PROCEDURES: A retrospective review of consecutive patients diagnosed with periorbital LMs and who received doxycycline sclerotherapy at Hong Kong Eye Hospital and Queen Elizabeth Hospital, Hong Kong between January 2016 and June 2022. Doxycycline was prepared with a concentration of 100 mg diluted in 10 mL water for injection. A 23-gauge needle aiming at the center of the macrocyst was used to aspirate fluid from the lesion; this was then followed by an intralesional injection of 0.5 to 2 ml of doxycycline depending on the size of the cavity. MAIN FINDINGS: A total of eight patients(six females) were included in this study. All of them received doxycycline sclerotherapy for periorbital LMs(five extraconal, three intraconal). The median age for receiving sclerotherapy was 29 years old. Seven patients had macrocystic LMs, and one had mixed macro- and microcystic LM. Two of the LMs had venous components radiologically. The average number of sclerotherapy treatment in one patient was 1.4 ± 0.7times. Seven of the eight patients had excellent response radiologically or clinically. One patient showed a satisfactory response after three cycles of sclerotherapy. No recurrence was experienced at median follow-up of 14 months. None of the patients experienced visual threatening or systemic complication. PRINCIPLE CONCLUSIONS: Our preliminary experience with doxycycline sclerotherapy has shown encouraging results for the treatment of macrocystic or mixed-type periorbital LMs, with a favourable safety profile. Further clinical trials with longer follow-ups are warranted on this topic.

Malignancies in Immunoglobulin G4-related ophthalmic disease.

PURPOSE: Clinical phenotypes in Immunoglobulin G4-related disease (IgG4-RD) according to the patterns of affecting organs have different risks of malignancies. We attempt to determine the association of malignancies with IgG4-related ophthalmic disease (IgG4-ROD). DESIGN: Retrospective cohort study. METHODS: Review of medical records, orbital images and histopathology reports in a territory-wide cohort of biopsy proven IgG4-ROD patients from 2005-2019. FINDINGS: Among 122 patients who had biopsies taken from adnexal lesions including lacrimal glands (n = 108), orbital mass (n = 30), infiltrated orbital fat (n = 10), conjunctiva (n = 2) or extraocular muscles (n = 3), 13% (16/122) developed malignancies over 73 ± 48months' follow-up. There were 9 cases of ocular adnexal lymphoma (OAL) and 7 extra-orbital malignancies. Compared with the general population, the incidence of OAL was significantly higher (standardized incidence ratios, SIRs = 10.0, 95%CI = 4.5-17.6) while that of extra-orbital malignancies was similar. The SIRs was highest within the first year (SIR = 46.7, 95%CI = 18.5-87.6) when 7 OAL were concomitantly diagnosed. Patients who developed OAL or extra-orbital malignancies were older than other patients at IgG4-ROD diagnosis (64.9 ± 7.1, 68.3 ± 8.5 versus 55.2 ± 15.0 years, P < 0.05). Asymmetric lacrimal gland enlargement (78% versus 13%), lack of frontal (0% versus 12%) or infraorbital nerve enlargement (0% versus 36%) were associated with OAL (all P < 0.05). Pre-treatment serum IgG4 level or extra-orbital IgG4-RD involvement was similar among patients with or without malignancies. CONCLUSION: In this biopsy-proven IgG4-ROD cohort, 7% developed OAL which was 10 times higher than the general population. Patients with asymmetric lacrimal gland enlargement or without trigeminal nerves involvement radiologically were associated with OAL.

Ocular Surface Evaluation in Immunoglobulin G4-Related Ophthalmic Disease.

PURPOSE: To evaluate the functional and structural changes of the meibomian glands and ocular surface in immunoglobulin G4-related ophthalmic disease (IgG4-ROD) patients. DESIGN: Cross-sectional, matched case-control comparison study. METHODS: This study included 64 patients with biopsy-proven IgG4-ROD (aged 63.4 ± 12.2 years, 39 male) and 64 sex- and age-matched healthy controls. Patients were managed by hospitals covering the publicly funded ophthalmology service in Hong Kong. Outcome measures included anterior segment examination and keratographic and meibographic imagings. RESULTS: A total of 64 worst-affected eyes of the 64 IgG4-ROD patients were analyzed. Corneal fluorescein staining (P = .0187), lid margin telangiectasia (P = .0360), lid-parallel conjunctival folds (P = .0112), papillae (P = .0393), meibomian gland plugging (P = .0001), meibomian gland expressibility (P = .0001), and meibum quality (P = .0001) were more significant in IgG4-ROD patients compared with healthy controls. Both upper and lower meibomian gland dropouts (P = .001 and .0003), and tear meniscus height (P = .0001) were higher in IgG4-ROD patients. Non-invasive tear break-up time (NITBUT) (P = .0166) and Schirmer test results (P = .0243) were lower in IgG4-ROD patients. Upper (r = 0.336, P = .0140) meibomian gland dropouts and NITBUT (r = -0.293, P = .0497) were positively and negatively correlated with the IgG4-ROD onset age, respectively. The number of extraocular organ involvement was negatively correlated with the Schirmer test(r = -0.341, P = .0167). Lower NITBUT was found in IgG4-ROD eyes with lacrimal gland enlargement than in IgG4-ROD eyes without lacrimal gland enlargement radiologically (P < .0001). CONCLUSIONS: IgG4-ROD patients showed features of both aqueous tear deficiency and evaporative dry eye disease. We recommend ocular surface evaluation to all patients newly diagnosed with IgG4-ROD. Further studies are warranted to clarify the mechanism of IgG4-related dry eye disease.

Treatment outcomes and their determinants of IgG4-related ophthalmic disease: a territory-wide cohort study.

BACKGROUND: Oral corticosteroid remains the first-line treatment of IgG4-related ophthalmic disease, but steroid-dependence is common and serious. Factors associated with steroid dependence and relapse have to be further explored. STUDY POPULATION: A city-wide, biopsy-proven, Chinese cohort. METHODS: Retrospective, masked review of medical records, orbital images and histopathology reports. RESULTS: There were 101 patients with at least 24-month follow-up. Up to 82% (82/101) received oral corticosteroid as first-line treatments, and 7 of them received also concomitant steroid-sparing agents (SSA)/biological agents as primary treatment. There was 61% (50/82) of patients required long-term corticosteroid (alone=23, with SSA=27) after 1.9±0.7 (range 1-5) relapses. When compared with the 21% (17/82) of patients who tapered corticosteroid successfully for 24 months, steroid dependence was associated with elevated baseline serum IgG4 level (94% vs 65%, p<0.01) and Mikulicz syndrome (46% vs 18%, p<0.05). Up to 13% (11/82) of patients tolerated residual disease after tapering off corticosteroid. There was 17% (17/101) of patients did not require any medications after biopsies. They were more likely to have debulking surgeries (71% vs 40%, p<0.05), discrete orbital lesions (65% vs 26%, p<0.05), normal baseline serum IgG4 level (24% vs 6%, p<0.05) and no Mikulicz syndrome (94% vs 61%, p<0.05). CONCLUSION: In this cohort, 60% of patients required long-term maintenance oral corticosteroid. Elevated pretreatment serum IgG4 level and Mikulicz syndrome were associated with steroid dependence. Debulking surgery is an alternative for a subgroup of patients with discrete orbital lesions, normal baseline IgG4 level and no Mikulicz syndrome.

Systemic Involvement in Immunoglobulin G4-Related Ophthalmic Disease.

BACKGROUND: Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) poses clinical challenges due to its heterogeneous ocular and systemic manifestations. We aim to report the systemic involvement and the clinical, serological and radiological associations of a cohort of Chinese patients. METHODS: A territory-wide, biopsy-proven, Chinese cohort. A retrospective, masked chart review of medical records, orbital images, and histopathology reports. RESULTS: A total of 122 (65 male) patients with a follow-up of 81 ± 49 (24 to 84) months were reviewed. Ninety (74%) patients presented bilaterally. Subacute upper eyelid swelling was the commonest presentation (82/122, 67%). During follow-up, 91/122 patients (75%) underwent extra-orbital imaging including computer tomography (692 films), ultrasonography (182 films), magnetic resonance imaging (76 films) and whole body FDG-PET scan (33 films). Eighty-six (95%) of these 91 patients had extra-orbital involvement radiologically (2.7 ± 1.6 regions, range: 0 to 9). Lymph node was the most prevalent (N = 60,66%), followed by salivary gland (N = 51,56%), lung (N = 49,54%), kidney (N = 22, 24%), hepatobiliary tree (N = 18, 20%) and pancreas (N = 17, 19%). Other organs include thyroid, aorta, meninges/brain and skin. Twenty-eight (23%) patients had allergic diseases (19 asthma, 16 allergic rhinitis, and 6 eczemas). Fifty-seven (48%) patients had paranasal sinusitis. Serum eosinophilia was associated with a higher number (3.24 versus 2.52, P = 0.0304) of organ involvement. Patients with deep organ involvement was associated with a higher age of IgG4-ROD onset (70 ± 12 versus 56 ± 13, P < 0.0001). CONCLUSIONS: 95% of the patients who underwent systemic imaging in our cohort had systemic organ involvement. An early physicians' assessment and radiological imaging are recommended after the diagnosis of IgG4-ROD.

Radiological Determinants of Complicated Immunoglobulin G4-Related Ophthalmic Disease: A Territory-Wide Cohort Study.

PURPOSE: To evaluate the presenting radiological features of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and their associations with IgG4-related optic neuropathy (IgG4-RON), and IgG4-related ocular adnexal lymphoma (IgG4-ROL). METHODS: A territory-wide, biopsy-proven, Chinese cohort. Masked review of orbital images, medical records, and histopathology reports. RESULTS: A total of 115 (94%) of the 122 patients in our cohort had preoperative orbital images (computed tomography=105, magnetic resonance imaging=40). Among them, 103/115 (90%) showed enlarged lacrimal glands, and 91 (88%) were bilateral. Nerve enlargement was observed: infraorbital in 31/115 (27%) patients and frontal in 17/115 (15%), 10 and 9 being bilateral, respectively. At least 1 or more extraocular muscle (EOM) enlargement was found in 41/115 (37%) patients, bilaterally in 20. Lateral rectus occurred in 30 (73%) of these 41 EOM patients and inferior rectus in 28 (68%). Two adjacent EOMs (inferior and lateral recti in 11 patients, inferior and medial recti in 7 patients) or multiple EOMs (at least 3) were enlarged in 23/41 (56%) and 13/41 (32%) of the patients, respectively. Intraconal lesions (67% vs 11%, P<0.05), infraorbital (83% vs 23%, P<0.005), or frontal (50% vs 15%, P<0.05) nerve enlargement was significantly associated with IgG4-RON (6 patients) by univariate analyses. Asymmetric lacrimal gland enlargement and discrete orbital mass (both P<0.05) were associated with IgG4-ROL (9 patients) by multivariate analyses. CONCLUSIONS: In this IgG4-ROD cohort, most patients had bilateral enlarged lacrimal glands, and the lateral rectus is the most frequently involved EOM. For the first time, unique radiological patterns associated with the development of IgG4-RON and IgG4-ROL are found.

Ophthalmology in the time of COVID-19: experience from Hong Kong Eye Hospital.

AIM: To review international guidelines and to share our infection control experience during the coronavirus disease 2019 (COVID-19) pandemic at a tertiary eye centre in Hong Kong. METHODS: Infection control guidelines and recommendations from international ophthalmological bodies are reviewed and discussed. The measures at our hospital were drawn up as per international and local health authorities' guidelines and implemented with the collaboration of doctors, nurses and administrative staff. RESULTS: The aims of our infection control measures are to 1) minimize cross-infection within the hospital; 2) protect and support hospital staff; 3) ensure environmental control. To minimize the risk of cross-infection, outpatient attendance and elective surgery have been reduced by 40%, and general anesthesia procedures were reduced by 90%. Patients entering the hospital are screened for fever, travel history, contact and cluster history, and COVID-19 related symptoms. To protect and support hospital staff, we ensure provision of adequate personal protective equipment (PPE) and provide clear guidelines on the level of PPE needed, depending on the clinical situation. Other protective measures include provision of work uniforms, easy access to alcohol-based hand rub, opening new lunch areas, implementation of self-monitoring and self-reporting systems, and communication via online education and updates. Finally, environmental control is achieved by ensuring regular disinfection of the hospital premise, enhancing ventilation, and usage of disposable ophthalmic instruments. CONCLUSION: Our multi-pronged approach to infection control is, so far, successful in minimizing infection risks, while allowing the maintenance of essential ophthalmic services.