Photochemical Crosslinking of Tarsal Collagen as a Treatment for Eyelid Laxity: Evaluation in Ex Vivo Human Tissue.

PURPOSE: Experimental investigation in human eyelids to confirm that exposing excised tarsal plates to ultraviolet-A radiation can induce a stiffening effect through the riboflavin-photosensitized crosslinking of tarsal collagen. METHODS: Thirteen tarsal plates excised from nonfrozen human cadavers were irradiated with ultraviolet-A rays (365 nm wavelength) at an irradiance of 75 mW/cm2 for 3 minutes, equivalent to a radiation fluence of 13.5 J/cm2, in the presence of a riboflavin derivative as a photosensitizer. The tensile stress (strength) and Young's modulus (stiffness) of both nonirradiated and irradiated specimens were measured with the BioTester 5000 in the uniaxial mode at a strain of 10% and analyzed statistically. Individual specimens excised from 2 cadavers were also examined by routine histopathologic protocols to assess the effect of radiation on the Meibomian glands and collagen organization. RESULTS: The irradiation enhanced both stiffness and strength of the human tarsal specimens, the difference between the test samples and controls being statistically significant (p < 0.0002 for n = 13). Histology indicated no damage to tarsal connective tissue or to Meibomian glands, and revealed a more compact packing of the collagen network located around the glands, which may be beneficial. The existence of collagen compaction was also supported by the reduction of samples' thickness after irradiation (p = 0.0645). CONCLUSIONS: The irradiation of tarsal tissue with ultraviolet-A light of tarsus appears to be a safe and effective method for reducing eyelid laxity in human patients.

Blepharospasm Secondary to Deep Brain Stimulation of the Subthalamic Nucleus in Parkinson Disease: Clinical Characteristics and Management Outcomes.

BACKGROUND: Deep brain stimulation of the subthalamic nucleus (STN-DBS) is an effective treatment for patients with motor symptoms of Parkinson disease but can be complicated by disabling blepharospasm and apraxia of eyelid opening (ALO). Currently, there is no clear consensus on optimal management, and addressing these issues is further hindered by systemic morbidity and resistance to treatments. We aim to describe the different phenotypes of these eyelid movement disorders, to report our management approach and patient responses to treatment. METHODS: A retrospective case series of all patients with blepharospasm/ALO secondary to STN-DBS that were treated at a tertiary center between 2011 and 2020. Data collected included date of Parkinson diagnosis, date of DBS surgery, date of development of blepharospasm/ALO symptoms, STN-DBS stimulation settings, and treatment given. Patients' symptoms before and after treatment were measured using the blepharospasm disability index and Jankovic Rating Scale. RESULTS: Five patients were identified with eyelid movement disorders secondary to STN-DBS. All patients had moderate-to-severe symptoms at presentation. Four patients received periocular botulinum toxin injections. Three patients underwent surgery in the form of frontalis suspension or direct brow lift with or without upper lid blepharoplasty. All reported an improvement in symptoms following treatment. CONCLUSIONS: A multimodality, patient-specific approach is required in the treatment of blepharospasm/ALO secondary to STN-DBS. Botulinum toxin injections can be effective, but patients may require surgery if toxin treatment alone becomes ineffective. Tailoring treatment to individual needs can result in a measurable improvement in symptoms.

Thirty Years of Experience With Ocular Adnexal T-Cell Lymphoma.

PURPOSE: To evaluate the disease characteristics and survival of patients with ocular adnexal T-cell lymphoma. METHODS: A retrospective, observational study of patients with a histopathological diagnosis of T-cell lymphoma of the ocular adnexa seen between 1992 and 2022. Demographic data, clinical presentation, imaging, histology, immunohistochemistry, treatment, and outcomes were reviewed. RESULTS: Fifteen patients were included in the study with the mean age at diagnosis of 50 years old (range 7-85). The most common presenting symptoms were ulcerated eyelid skin lesions (40%) followed by eyelid swelling (13.3%), and lacrimal passageways obstruction (13.3%). The anaplastic large cell lymphoma (33%) and primary cutaneous T-cell lymphoma (33%) were the most diagnosed peripheral T-cell lymphoma subtypes, followed by the nasal type natural killer/T-cell lymphoma (27%) and peripheral T-cell lymphoma not otherwise specified (7%). The most prevalent stage of disease progression was stage I and stage IV (Ann Arbor classification) with seven (47%) patients each. Eight (53%) patients succumbed to the disease process of which three (37.5%) deceased in the first six months and six (75%) within the first year of diagnosis. We identified a strong statistical association between stage and disease-related death ( p = 0.003). CONCLUSIONS: Peripheral T-cell lymphoma occurring in the orbit and ocular adnexa is extremely rare. Advanced stage at diagnosis leads to almost certain death from the disease despite aggressive local and systemic treatment. Early diagnosis improves the chances of survival but can be hindered by this condition's ability to simulate benign inflammatory conditions both clinically and histologically.

Granulomatosis with polyangiitis-associated sclerokeratitis in a case of ocular adnexal B-cell lymphoma.

We describe the first reported case of granulomatosis polyangiitis (GPA)-associated sclerokeratitis in a patient with treated ocular adnexal lymphoma (OAL). The patient presented with pain and decreased vision in the left eye over several weeks. Past medical history was significant for recent bilateral relapsing OAL that was treated successfully with radiotherapy. Examination of the eyes revealed sectoral scleritis and peripheral ulcerative keratitis. Magnetic resonance imaging (MRI) of the orbits excluded recurrence of OAL and serum antineutrophil cytoplasmic autoantibody (ANCA) titres confirmed the diagnosis of GPA. Disease was poorly responsive to systemic steroids, azathioprine and rituximab. Ultimately, resolution was achieved with successive subconjunctival dexamethasone and subconjunctival triamcinolone injections. This case highlights the need to consider ocular inflammation in patients with a history of malignant hemopathies.

Long term follow-up of congenital infantile fibrosarcoma of the orbital region.

We present the long-term follow-up of a case of periorbital congenital infantile fibrosarcoma (CIFS) treated with chemotherapy and surgery. The tumor was detected on a routine prenatal ultrasound at 30 weeks of gestation and diagnosed via an orbital biopsy day 9 postnatal age. The patient underwent chemotherapy and surgical debulking within the first 3 months of life and has maintained complete tumor remission for 7 years. The case highlights that early recognition and prompt treatment of periorbital CIFS can lead to complete long-term remission of this uncommon malignancy.

Effect of external eyelid weighting on eyelid and eyebrow position in normal and ptosis patients.

PURPOSE: This study aims to characterize the physiologic response of both eyelid and eyebrow position to increasing downward forces simulated by external weights. METHODS: In this prospective observational study, both normal individuals and patients affected by ptosis were tested. External eyelid weights were placed on one upper eyelid with incrementally increasing weight from 0.2 to 2.4 g. The eyelid carrying the weight was randomly selected for normal subjects and patients with bilateral ptosis, whereas for unilateral ptosis, the ptotic eyelid was utilized. Photographs were obtained at baseline and with increasing weight until MRD1 reached 0 on the weighted side or, until 2.4 g was reached. Eyelid and brow position on the weighted and unweighted sides were digitally measured in millimeter. Primary outcome measures were change in the margin to reflex distance (MRD1) and pupil to brow distance (PTB) with weight on the weighted and unweighted sides for normal and ptosis subjects. RESULTS: The weighted eyelid MRD1 decreased linearly with increasing weight. This was true for normal and ptosis subjects. The unweighted eyelid MRD1 increased linearly with increasing weight. This was also the case for both normal and ptosis subjects. With increasing weight, PTB increased linearly on the weighted side. No significant intergroup differences were noted. CONCLUSIONS: In normal and ptosis subjects, when external weight on the eyelid is incrementally increased, the weighted eyelid MRD1 decreases, the unweighted eyelid MRD1 increases, and both brows elevate in a linear fashion.

The first UK national blepharospasm patient and public involvement day; identifying priorities.

PURPOSE: We describe the planning and outcomes of the first 'Blepharospasm Day' in the UK. Blepharospasm is a distressing condition for patients and carers. Our 'patient and public involvement' event aimed to: cultivate a more informed patient group via active dialogue, help clinicians more effectively prioritise research and to facilitate peer-to-peer support for affected patients and public. DESIGN: A national one-day event was organised by the oculoplastics department at Moorfields Eye Hospital. The event was divided into informative lectures delivered by professionals and a patient panel, during which patients shared their experiences and expectations. METHODS: Data were collected from a variety of sources including: an interactive voting "LiveWall" poster, a pre-event questionnaire; "living with Blepharospasm", transcripts from patient panel discussions; and a feedback questionnaire. RESULTS: The event was well-received with 100% of respondents rating it good or excellent. Four research themes were identified: "aetiology", "alternative treatments", "faster, more accurate diagnosis", and "symptom control". Delegates' self-reported knowledge of blepharospasm increased significantly after the event. Limitations of the BdSI severity-assessment tool were noted with 22% of respondents failing to utilise it appropriately. CONCLUSION: Through our innovative "Blepharospasm Day", patient's priorities for research were identified, delegates understanding of blepharospasm increased and an independent blepharospasm patients-representatives' group was established; a first in the UK. Furthermore, short-fallings identified in the BdSI tool highlight the need for better severity-assessment tools. We demonstrate the benefits of the 'patient and public involvement' approach in the management of complex conditions such as blepharospasm. ABBREVIATIONS: PPI: Patient and public involvement; SLV-PSP: sight loss and vision sector - priority setting partnership; BRC: Biomedical Research Centre; NIHR: National Institute for Health Research; BsDI: Blepharospasm Disability Index.

Long-Term Outcomes of StopLoss Jones Tubes for Epiphora in Patients With Early or Multiple Loss of Lester Jones Tubes.

PURPOSE: Extrusion is the most common cause of failure in conventional Lester Jones tubes (LJTs). StopLoss Jones tubes (SLJTs) with distal flange are designed to reduce this complication. This study compared the survival of SLJTs with their prior LJTs and control patients with LJTs-only. METHODS: Retrospective review of consecutive LJT or SLJT insertion between January 2014 and December 2016. RESULTS: Twenty-eight eyes of 23 patients had 31 SLJTs. Before their SLJTs, these patients had 116 cumulative failed LJTs. Forty-seven eyes of 34 patients had 59 LJTs only. Fifty-two percent of SLJT group and 18% of LJT-only group had complex medial canthal conditions (p = 0.003). Compared to their previous LJTs, the SLJTs were less likely to extrude (3% SLJT vs. 64% prior LJT, p < 0.0001); there was no statistical difference in rates of sinking in (26% SLJT vs. 13% prior LJT, p = 0.0964). The median survival of LJTs in those who went on to have a SLJT was 3.5 months. SLJT insertion significantly lengthened the median survival to 26 months (p < 0.0001), comparable to the LJT-only group (25.5 months, p = 0.45). While extrusion was also the most common complication in the LJT-only group, this occurred only in 20% of eyes; tube failure from sink-in occurred in 14% of eyes. CONCLUSIONS: SLJTs, in patients prone to multiple or early prior LJT losses, can be used to rescue this group and allow them to regain a similar survival curve to the less complicated LJT-only group. Rates of tube extrusion are significantly reduced with the use of SLJT.

Unilateral cilioretinal artery occlusion following cannabis use.

There is a growing trend towards the decriminalization of cannabis and this is associated with its increased use over the past decade. Although a variety of adverse effects are attributed to its use, complications involving the eye are rare. We present the case of cilioretinal artery occlusion in a young man with no previous medical history. We discuss the management offered to him and present a review of the literature on this topic. Given the cilioretinal artery occlusion, medical management achieved an excellent result, with his best-corrected visual acuity improving to 6/12. Cilioretinal artery occlusions in healthy young individuals are extremely rare. The only risk factor identified in this patient was the use of cannabis on one occasion prior to his symptoms. There are no other cases in the literature that associate this complication with cannabis use and therefore it serves to raise awareness among the ophthalmic community.

Immune Reconstitution Inflammatory Syndrome in a Case of Nonspecific Orbital Inflammation.

The immune reconstitution inflammatory syndrome is an exaggerated abnormal immune response, typically seen in HIV-positive patients following restoration of a normal CD4 count as a result of initiation of antiretroviral therapy. It has been described in relation to either occult opportunistic infections or to a paradoxical relapse of a previously successfully treated infection with negative microbiological cultures. The authors report the case of a 60-year-old HIV-positive African male who presented with 2 episodes of orbital inflammation that occurred in conjunction with improvements of CD4 count following Highly Active Antiretroviral Therapy. This phenomenon was underpinned by biopsies obtained following each episode. Interestingly, on both occasions, he responded well to corticosteroid therapy. Although the soft tissues of the orbits are a common area affected by other inflammatory diseases, it is rare for them to be involved in immune reconstitution inflammatory syndrome. To the authors' knowledge, this is the first case report of immune reconstitution inflammatory syndrome affecting the orbits exclusively. The authors believe that it is probably an underdiagnosed condition and may be erroneously labeled as idiopathic in many cases. This case report inspires us to keep an open mind when dealing with patients on antiretroviral therapy.