Scleromalacia perforans as an early manifestation of late-onset rheumatoid arthritis: a case-based review.

Rheumatoid arthritis is a chronic autoimmune disease that can affect different organs beyond the joints. Ocular involvement includes keratoconjunctivitis sicca, peripheral ulcerative keratitis (PUK), episcleritis, scleritis, anterior uveitis, and corneal impairment. The most severe form of scleritis, scleromalacia perforans, is an aggressive ophthalmic manifestation that can potentially lead to blindness, usually occurring in late stages of disease. We report a case of an elderly woman in which this severe ocular manifestation occurred early on disease onset, differing from most of the previously reported cases of scleromalacia perforans. Ocular symptoms started concomitantly with the polyarthritis and other extra-articular manifestations, including rheumatoid nodules and vasculitic skin lesions. Ocular disease progressed due to patient's loss to follow-up, requiring pulse therapy with methylprednisolone. However, despite treatment, right eye enucleation was required due to melting of the corneal patch with uveal exposition. The patient was then treated with rituximab with improvement of systemic disease. The present case reinforces that, although rare, this complication is severe and must be promptly diagnosed and aggressively treated to improve prognosis of ocular and systemic RA.

Anterior segment spectral domain optical coherence tomography imaging of patients with anterior scleritis.

The purpose of the study was to describe the findings seen on anterior segment spectral domain optical coherence tomography (SD-OCT) in patients with anterior scleritis and determine the feasibility of using SD-OCT to image and grade the degree of scleral inflammation and monitor response to treatment. All patients underwent slit lamp examination by a uveitis specialist, and the degree of scleral inflammation was recorded. Spectral domain OCT imaging was then performed of the conjunctiva and scleral tissue using a standardized acquisition protocol. The scans were graded and compared to clinical findings. Twenty-eight patients with anterior scleritis and ten patients without ocular disease were included in the study. Seventeen of the scleritis patients were followed longitudinally. Common findings on SD-OCT in patients with active scleritis included changes in hyporeflectivity within the sclera, nodules, and visible vessels within the sclera. There was significant variation in findings on SD-OCT within each clinical grade of active scleritis. These changes on SD-OCT improved with treatment and clinical improvement. SD-OCT imaging provided various objective measures that could be used in the future to grade inflammatory activity in patients with anterior scleritis. Longitudinal imaging of patients with active scleritis demonstrated that SD-OCT may have great utility in monitoring response to treatment.

Novel technique for late scleromalacia in a ciliary body melanoma due to brachytherapy.

INTRODUCTION: We describe a technique using an sclerocorneal endograft sewed through the cornea to solve late scleromalacia following exoresection and brachytherapy of a ciliary body melanoma. METHODS: Vitrectomy ports were placed. A perforating 400-micron trepan keratoplasty of the cornea was performed allowing the prepared donor graft to be inserted and sutured in place. Intravitreal Perfluorocarbon liquid (PFCL) was used to stabilize the pressure throughout and maintain the graft in place. Once sutured in place, the PFCL was removed and 5000cs of silicone oil introduced. An amniotic membrane was placed surrounding the scleromalacia. The cornea was closed with running sutures. RESULTS: At one month, the anterior segment optical coherence tomography showed successful endograft placement with no aqueous humor leakage nor hypotony. Currently, the conjunctiva is becoming slightly thicker due to light inflammation, and the eye has recovered the whiteness at the scleromalacia. CONCLUSIONS: This internal graft technique provides advantages when dealing with poorly healing radiated tissue and reduces the risk of post-operative aqueous humor leakage. Surgeons may consider this approach in cases with a high risk of poor healing or complications resulting from lack of tissue.

Scleral thinning causes, diagnosis, and management: A narrative review.

INTRODUCTION: Sclera forms the outer fibrous coat of the eye and provides structural integrity for the housing of intraocular contents. Scleral thinning is a serious progressive condition which can lead to perforation and worsening visual functioning. This review aims to summarize the anatomical consideration and causes of scleral thinning, diagnosis, and the various surgical approaches available to treat scleral thinning. MATERIALS AND METHODS: The narrative literature review was conducted by senior Ophthalmologists and researchers. PubMed, EMBASE, Web of Science, Scopus, and Google Scholar databases were searched for relevant literature from infinity till March 2022. Terms of the search referred to 'sclera' or 'scleral thinning' or 'scleral melting', and were combined with 'treatment', or 'management' or 'causes'. Publications were included in this manuscript if they offered information about the nature of these topics. Reference lists of relevant literature was searched. There were no limits on type of article to be included for this review. RESULTS: Scleral thinning arises from diverse congenital, degenerative, immunological, infectious, post-surgical, and traumatic etiologies. It is diagnosed upon slit-lamp examination, indirect ophthalmoscopy, and optical coherence tomography. Conservative pharmacological treatment of scleral thinning may include anti-inflammatory drugs, steroid drops, immunosuppressors, monoclonal antibodies, and surgical treatments including tarsorrhaphy, scleral transplantation, amniotic membrane transplantation, donor corneal graft, conjunctival flaps, tenon's membrane flap, pericardial graft, dermis graft, cadaveric dura mater graft, and other autologous and biological grafts. CONCLUSION: Scleral thinning treatments have developed dramatically in recent decades and the rise of alternative grafts for scleral transplantation procedures or use of conjunctival flaps have taken center stage in surgical management. This review adds a comprehensive summary of the scleral thinning with attention to the positive and negative features of new treatments alongside previous mainstay management strategies.

Tarsoconjunctival flap for scleromalacia related to treatments for conjunctival melanoma.

Purpose: To describe the use of a tarsoconjunctival pedicle flap for the repair of scleral melt secondary to treatment of conjunctival melanoma. Observation: A 67-year-old woman developed progressive scleromalacia after multiple treatments for an American Joint Committee on Cancer cT2d category conjunctival melanoma. Prior to referral, she underwent synchronous topical chemotherapy (interferon, 5-fluorouracil, mitomycin). Then, incomplete tumor regression led to excision with adjuvant cryotherapy. Lastly, systemic metastasis treated with systemic immunotherapy provided durable remission. However, her multiple treatments (e.g., topical chemotherapy, resection, cryotherapy) were associated with progressive nasal bulbar scleromalacia treated by conjunctival advancement and amniotic membrane grafts. Sclera reinforcement was achieved after a tarsoconjunctival flap was affixed to the eye to cover, and thus vascularize the scleral defect. The tarsoconjunctival flap provided 5 years of tectonic support. Conclusions and Importance: Tarsoconjunctival pedicle flaps can provide scleral integrity for a patient with progressive scleral melting.

A Rare Occurrence of Scleromalacia Perforans With Juvenile Rheumatoid Arthritis: A Case Report.

Here, we report a case of a nine-year-old boy with chronic juvenile rheumatoid arthritis (JRA) leading to scleromalacia perforans (SP). We rarely see SP with fixed deformities of rheumatoid arthritis in the hands, but not as a starting point of the disease. He reported eye manifestations associated with JRA on further investigation and inquiry. The right globe was preserved on presentation, and the left was perforated. He lost sight in his left eye when he was treated with antibiotics and visited various physicians in his native region.

The Use of Tutoplast as an Adjunct in Scleral Buckle Procedure in Patients with Extremely Thin Sclera.

Extremely thin sclera often necessitates abortion of scleral buckle procedures. In patients in whom a scleral buckle is desired, previous techniques have included the use of cyanoacrylate glue and continuing with surgery or placing donor tissue over the areas of thin sclera, but this can delay surgery. This was a retrospective review of three patients with thin sclera encountered during scleral buckling procedures. All patients had Tutoplast Pericardial Graft placed over the areas of thin sclera which allowed the scleral buckle to be sutured onto the Tutoplast rather than the thin sclera. Tutoplast Pericardial Graft is a useful adjunct in scleral buckle procedures with extremely thin sclera, and a scleral buckle can be safely placed over it and lead to successful retinal reattachment.

Preserved Corneal Lamellar Grafting Reduces Inflammation and Promotes Wound Healing in a Scleral Defect Rabbit Model.

Purpose: To investigate the effect of preserved corneal lamellar grafting on inflammation and wound healing and to compare its effect with that of preserved scleral grafting in a scleral defect rabbit model. Methods: New Zealand White rabbits were assigned to a corneal lamellar grafting group (n = 5) or a scleral grafting group (n = 5). After lamellar dissection of superotemporal sclera using 6.0-mm trephine, the same sizes of preserved human corneal or scleral grafts were transplanted with 10-0 nylon interrupted sutures. The grafted areas were photodocumented at 3 to 21 days after surgery to evaluate epithelial wound healing index (%), neovascularization and presence of filaments. The existence of CD3+ T cells and CD34+ cells at the grafted areas was analyzed at 21 days. Results: Epithelial wound healing index was significantly higher in the corneal grafting group at 9 days (P < 0.05). Scleral grafts showed copious formation of filaments adherent to the engrafted area from 9 to 14 days, whereas the corneal grafts were free of filaments. The numbers of inflammatory cells were significantly higher in the scleral grafts (P < 0.05), and CD3+ T cells and CD34+ cells were populated within inflammatory cells at graft-recipient junctions in both groups. The mean areas of the estimated perigraft and intragraft neovascularization tended to be higher in scleral grafts. Conclusions: Preserved corneal lamellar grafting enhances epithelial wound healing and alleviates inflammation in a scleral defect rabbit model. Translational Relevance: This work suggests that the preserved corneal graft may be considered as a favorable alternative option for repairing scleral defects.

Delayed Onset Scleromalacia and Conjunctival Bleb Formation Following Intraoperative Mitomycin C Application during Conjunctival Melanoma Excision.

PURPOSE: To present a case of delayed onset scleromalacia and conjunctival bleb formation one year after conjunctival melanoma excision with intraoperative use of mitomycin-C (MMC) and double-freeze-thaw technique. METHODS: Case report. RESULTS: A 69-year-old woman had a conjunctival melanoma excised by the 'no touch technique' with intraoperative application of 0.02% MMC for 3 min on bare sclera, freeze-thaw cryotherapy and amniotic membrane transplant performed elsewhere. Three months later, she presented to us with a red, sore and painful right eye. Examination revealed severe scleritis. She was treated with lubricants and oral steroids for 6 weeks. She settled well with no recurrence of melanoma. At one year, she developed scleromalacia and conjunctival blebs in the inferonasal quadrant of right eye. She remains under closer observation as she is at high risk of perforation. CONCLUSION: Caution should be exercised with intraoperative use of MMC on bare sclera during excision and cryotherapy of conjunctival melanoma. As published in the literature, when using MMC, it is recommended to use the lowest dose topically in the form of eye drops in the postoperative period for the shortest time to avoid any sight-threatening complications.