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.

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.

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.

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.

Transplantation of autologous perichondrium with amniotic membrane for progressive scleral necrosis.

PURPOSE: Scleral necrosis with severe ischemia is refractory to conventional treatment because of avascular progressive necrosis. We assessed the therapeutic efficacy and safety of autologous perichondrium transplantation in patients with progressive scleral necrosis (PSN) and analyzed the clinical effects. METHODS: This study was a prospective, interventional, and noncomparative case series. Reconstructive surgery using autologous perichondrium and amniotic membrane (AM) was performed in patients with PSN who showed progressive ischemic scleral melting with impending perforation state and/or broad avascular area larger than 10 mm in diameter. The primary outcome was restoration of scleral integrity with healthy vascularized epithelium over the graft at six months after surgery. The secondary outcome was complication rate associated with autologous perichondrium graft use. RESULTS: Eighteen eyes of 14 patients underwent reconstructive surgery using autologous perichondrium patch and AM grafts. Observations indicated the graft provided the eyeball with successful structural integrity in 17 out of the 18 cases (94.4%) at six months after surgery. One eye showed a small scleral defect due to wound dehiscence at four month after the surgery. Additional surgery using perichondrium and AM stabilized the eye. The scleral necrosis healed completely after perichondrium and AM transplantation, even in cases with full-thickness scleral defect. The scleral integrity was maintained until the last follow-up session. There were no serious complications of endophthalmitis or graft infection. CONCLUSIONS: Reconstructive surgery using autologous perichondrium and AM is an effective method for restoration of scleral integrity and vascularization of the episclera and conjunctiva in eyes with PSN. Therefore, autologous perichondrium can be considered as an appropriate new biologic tissue for PSN.

Conjunctival Flap with Biodegradable Collagen Matrix Implantation for the Treatment of Scleromalacia after Periocular Surgery.

Purpose: To report a new surgical procedure using biodegradable collagen matrix (Ologen) implantation with conjunctival flap for reconstruction of scleromalacia after periocular surgery. Methods: A total of 26 areas of 25 eyes in 25 patients were included. We divided all eyes according to the preoperative extent of calcified plaque or scleral defect as follows: small, large, and large and severe. Morphologic outcomes were graded from 0 to 7, with higher scores indicating worse cosmetic outcomes. Results: Mean follow-up period was 8.3 ± 5.6 months. No eyes showed recurrence or specific complications related to Ologen such as implant extrusion or allergic reaction. Minor complications such as flap vessel engorgement and flap hypertrophy were easily treated. Mean morphologic outcome scores were 1.8, 2.4, and 5.3 in groups 1, 2, and 3, respectively. Discussion: The combined surgery may be an effective and safe procedure for the treatment of scleromalacia after periocular surgeries.

Scleromalacia perforans: a case report.

BACKGROUND: Scleromalacia perforans is a rare ocular manifestation of rheumatoid arthritis which can potentially lead to blindness and is a late consequence in the course of the disease. It is an unusual finding for it to be present in a patient with joint pain without any rheumatologic progression of disease. CASE PRESENTATION: We describe a rare case of scleromalacia perforans and orbital inflammatory disease in a 40-year-old Pakistani woman with apparently no associated rheumatologic deformity. It is rare in the sense that we usually see scleromalacia perforans with fixed deformities of rheumatoid arthritis in the hands or progressed systemic complications but not as a starting landmark of disease. She presented to us with pronounced eye manifestation which on further inquiry and investigation was found to be associated with rheumatoid arthritis. There was perforation of left globe on presentation and the right one was preserved. She visited various physicians and ophthalmologists and was treated with topical and systemic antibiotics but ended up losing sight in her left eye. CONCLUSION: We conclude that ocular manifestations, however rare they are, should be foreseen, investigated, and treated in patients with suspected arthritis as the complication is grave and sight threatening.

Glaucoma Surgery in Scleromalacia: Using Endoscopic Cyclophotocoagulation where Conventional Filtration Surgery or Angle Procedures are contraindicated.

AIM: To describe the surgical management of glaucoma in a patient with severe scleromalacia, and secondary angle closure. INTRODUCTION: The management of glaucoma with coexisting scleromalacia plus secondary angle closure is challenging as most commonly performed incisional glaucoma surgery as well as minimally invasive glaucoma surgery (MIGS), which targets the drainage angle are all contraindicated. CASE REPORT: Medically refractory glaucoma in a 60-year-old male with a 30-year history of granulomatosis with polyangiitis resulting in extensive severe scleromalacia, cicatricial lower lid retraction with significant conjunctival exposure, and widespread synechial angle closure from chronic anterior uveitis was managed with combined phacoemulsification cataract surgery, and endoscopic cyclophotocoagulation (ECP). Careful postoperative management with intensive immunosuppression was used to successfully prevent complications related to the surgery, which resulted in improved visual acuity, and control of intraocular pressure (IOP). CONCLUSION: The ECP is a minimally invasive procedure that targets inflow of aqueous, and can be safely and successfully used to control IOP in challenging patients with complex secondary glaucoma, where the use of traditional incisional surgery, and other MIGS procedures are all contraindicated. CLINICAL SIGNIFICANCE: The choice of surgical treatment for medically refractory glaucoma needs to be selected based on the circumstances of individual patients, and take into consideration the condition of the sclera, conjunctiva and drainage angle, against the safety and efficacy of possible treatments. HOW TO CITE THIS ARTICLE: Rodrigues IAS, Lindfield D, Stanford MR, Goyal S. Glaucoma Surgery in Scleromalacia: Using Endoscopic Cyclophotocoagulation where Conventional Filtration Surgery or Angle Procedures are contraindicated. J Curr Glaucoma Pract 2017;11(2):73-75.

[Post-traumatic scleromalacia]. / Scléromalacie post traumatique.

We here report the case of a 18-year old patient who was referred for evaluation of pigmented subconjunctival tumor of the left eye mimicking uveal melanoma. The patient reported that the lesion had evolved rapidly during the previous months. Eye exam showed prominent dark brown lesion adjacent to the limbus between 3 and 9 hours, measuring 10 mm x 6 mm (A). Ultrasonographic biomicroscopy revealed cystic structure communicating with the vitreous cavity, suggesting the diagnosis of uveal hernia rather of melanoma. During a more thorough interview, the patient reported that he had undergone transfixing keratoplasty for cloudy patch secondary to blunt trauma from stone's throw dating back 10 years. We assumed that the scleral wall had been damaged, leading to the development of scleromalacia with uveal hernia. The patient had recently had an acute episode of vomiting which could have lead to an increase in lesion size. The patient was followed up for several weeks, during which the lesion was stable. The patient underwent surgery with successful uveal tissue reintegration and sclera closure (B).

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.

Clinical features and visual outcomes of scleritis patients presented to tertiary care eye centers in Saudi Arabia.

AIM: To describe the clinical features, systemic associations, treatment and visual outcomes in Saudi patients with scleritis. METHODS: A retrospective chart review was performed for patients with scleritis presenting to two tertiary care eye hospitals in Riyadh, Saudi Arabia, from 2001 to 2011. Data were collected on the clinical features of scleritis, subtypes of scleritis, associated systemic disease, history of previous ocular surgery and medical therapy, including the use of immunosuppressants. Treatment outcomes were evaluated based on best-corrected visual acuity (BCVA) and response to treatment. RESULTS: Of the 52 patients included in the study, non-necrotizing anterior scleritis was the most common type of scleritis in 22 patients (42.3%), followed by posterior scleritis in 14 patients (26.9%). The majority of cases, 31 patients (59.6%), were idiopathic in nature. Systemic associations were present in 12 patients (23.1%). Infectious scleritis was confirmed in 6 patients (11.5%): 3 with bacterial scleritis after pterygium excision, 2 patients with scleritis related to tuberculosis and 1 patient with scleritis resulting from herpes simplex infection. For the various subtypes of scleritis, BCVA values after treatment and time to remission significantly differed (P<0.05, all cases). Systemic immunosuppressive therapies in addition to steroids were administered to 46.2% of all patients. The T-sign was present on B-scan ultrasonography in 9 (64.3%) of the 14 posterior scleritis patients. CONCLUSION: Non-necrotizing anterior scleritis was the most common subtype of scleritis. Final visual outcome and time to remission differed among the various scleritis subtypes.

Delayed manifestation of bilateral scleral thinning after I-BRITE(®) procedure and review of literature for cosmetic eye-whitening procedures.

PURPOSE: To report a case of delayed-onset bilateral scleral thinning and calcium deposition following a cosmetic ocular-whitening procedure (I-BRITE(®)). METHODS: A 33-year-old male patient with a history of right-sided ptosis repair and left-sided anterior uveitis had previously undergone bilateral I-BRITE treatment for chronic conjunctival hyperemia. Four years after the procedure, the patient was referred to our institution with bilateral scleral thinning and overlying calcific depositions. A literature review was performed through PubMed from 1980 through 2014 using the search terms 'cosmetic', 'ocular', 'conjunctivectomy', 'regional conjunctivectomy', 'I-BRITE', 'eye-whitening', 'scleritis', 'necrotizing scleritis', 'anterior uveitis', 'mitomycin C', '5-fluorouracil', and 'bevacizumab', along with associated cross-referencing from relevant articles. RESULTS: Examination of the patient revealed bilateral necrotizing scleritis within the nasal region of both eyes. Calcified plaques were also present within the areas of scleromalacia, along with epithelial defects demonstrated with fluorescein staining. Although evidence of previous intraocular inflammation was apparent within the left eye, there were no active signs of inflammation evident within either eye on initial presentation. Complication rates reported in the literature include: scleral thinning (1.8%), calcific plaque formation (2.9%), fibrovascular proliferation (13%), diplopia (1.2%), elevation of intraocular pressure (4.2%), and recurrence of conjunctival hyperemia (2.1%). CONCLUSION: Cosmetic ocular whitening procedures have an attendant high complication rate, and have been associated with several adverse postoperative complications, which have in turn generated several reservations regarding the veritable benefit of the procedure. Many postsurgical complications may demonstrate delayed apparition, varying from several months to several years after primary surgical intervention as in the case reported here.

Conjunctival Flap Surgery Using Calcium Plaque Scissors for Calcified Scleromalacia after Cosmetic Conjunctivectomy

PURPOSE: To research the advantage of using calcium plaque scissors in conjunctival flap surgery for calcified scleromalacia after cosmetic conjunctivectomy. METHODS: We analyzed 55 eyes that had undergone conjunctival flap surgery for calcified scleromalacia occurring after cosmetic conjunctivectomy. Surgical blade was used in 30 eyes (Group 1) and calcium plaque scissors in 25 eyes (Group 2). Time after conjunctivectomy, plaque size, operation time and visual acuity before and after the flap surgery were analyzed and compared. Additionally, necessity of additional scleral surgery was evaluated. Optical coherence tomography (OCT) of the sclera was performed both pre- and postoperatively and the results were compared. RESULTS: An average of 3.9 +/- 1.0 years elapsed until conjunctival flap surgery and follow-up time was 5.2 +/- 3.8 months. Post conjunctivectomy time and plaque size were similar in both groups (p = 0.87 and 0.49, respectively). The surgery time in Group 2 was shorter than in Group 1 (17.5 +/- 6.3 minutes and 21.9 +/- 8.5 minutes, respectively, p = 0.20). Uncorrected visual acuity was similar in both groups before and after conjunctival flap surgery (p = 0.53 and p = 0.20, respectively). In Group 1, one sclera transplantation and three Ologen(TM) insertion surgeries were performed as an additional scleral surgery. Based on OCT, calcium plaque scissors were confirmed as a new surgical tool for calcium plaque removal with minimal scleral damage. CONCLUSIONS: Using calcium plaque scissors when performing conjunctival flap surgery for calcium plaque removal that occurred after cosmetic conjunctivectomy reduces the necessity of an additional scleral surgery and surgery time.

Treatment of Scleromalacia with Scleral Autograft

PURPOSE: To report a case of a patient who developed scleromalacia after cosmetic eye whitening conjunctivectomy and treated with scleral and conjunctival autograft. CASE SUMMARY: A 42-year-old male patient who received cosmetic eye whitening conjunctivectomy in both eyes on the nasal side in 2008 developed scleromalacia in the left eye. Calcium deposits and deformed conjunctiva were removed from the left eye. Autogenous sclera and conjunctiva were obtained from the upper side of the left eye and autogenous graft was performed. Topical antibiotics, topical steroid, topical autologous serum, and antibiotic ointment were applied postoperatively. The patient was given oral steroid for 1 month after surgery. During the postoperative 6 months, the grafted autogenous sclera was well maintained and improved cosmetically. CONCLUSIONS: In cases of scleromalacia occurring after cosmetic eye whitening conjunctivectomy, autogenous sclera can be considered as a treatment filler.

The development of scleromalacia after regional conjunctivectomy with the postoperative application of mitomycin C as an adjuvant therapy.

A 40-year-old woman presented with ocular discomfort in both eyes that had persisted for several months. Six months ago, she had undergone a bilateral nasal and temporal conjunctivectomy using a bare scleral technique followed by a postoperative application of 0.02% mitomycin C (MMC) to treat her chronic hyperemic conjunctiva for cosmesis. Slit-lamp examination revealed that the patient had bilateral nasal and temporal scleral thinning, and a calcified plaque on her nasal conjunctiva. There was no episcleral tissue present around the wound area, and it was difficult to detect any normal conjunctival tissue in the adjacent area for covering the lesion. We believe that performing an aggressive conjunctival excision procedure followed with MMC application for cosmetic enhancement may be disastrous in certain cases.