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.

Case report of ocular Kaposi's sarcoma.

BACKGROUND: Kaposi's sarcoma (KS) is generally considered a neoplastic disorder of vascular origin and occurs in patients with acquired immunodeficiency syndrome (AIDS) or who have received immunosuppressive treatments after an organ transplant (Soulier et al., Blood 86(4):1276-80, 1995; Viejo-Borbolla and Schulz, AIDS Rev 5(4):222-9, 2003; Schulz, J Antimicrob Chemother 45(Suppl T3):15-27, 2000; Aversa et al. Crit Rev Oncol Hematol 53(3):253-65, 2005; Mbulaiteye and Engels, Int J Cancer 119(11):2685-91, 2006; Tessari et al., Eur J Dermatol 16(5):553-7, 2006). Several Kaposi's sarcoma case reports involving eyelids and conjunctiva have been published (Bavishi et al., Int J STD AIDS 23(3):221-2, 2012; Baumann et al., Ger J Ophthalmol 4(4):239-45, 1995). CASE PRESENTATION: we report a 13 years old asian male patient rare case of ocular KS that was initiated from the sclera and progressed into the cornea and conjunctiva without an human Immunodeificiency Virus (HIV) or HHV-8 infection after a peripheral blood stem cells transplantation. In this case, anti- vascular endothelial growth factor (VEGF) therapy was attempted to stop the advance of ocular lesions and failed. Eventually, the KS was cured by a limbo-corneal lamellar graft, an amniotic membrane and scleral allograft transplantation plus intraoperative mitomycin C(MMC) after the complete excision of the tumors. CONCLUSION: A compete surgical excision combined with the intraoperative application of MMC, as well as grafts to repair the scleral, conjunctival, and corneal surfaces, could prevent a recurrence of KS.

Treatment and management of scleral disorders.

Scleral inflammatory disorders, with their extensive differential diagnoses and sometimes as controversial treatment options, pose both diagnostic and clinical challenges for physicians. Targeted laboratory investigations derived from careful history taking and physical examination are crucial in the early identification of patients with associated systemic conditions. Prompt discrimination between episcleritis and scleritis is of therapeutic importance, as management, prognosis, and complications differ for these diseases. Although immunomodulatory and new biologic agents have improved the management of scleritis, continued studies are warranted to establish effective treatment guidelines across patient populations, especially in refractory cases.

Recurrent spontaneous scleral rupture in Marfan's syndrome.

The ocular manifestations of Marfan's syndrome (MS) range from ectopia lentis, microspherophakia, myopia, glaucoma and retinal detachment. Spontaneous scleral rupture is a rare complication and recurrent scleral perforation is extremely rare. We report a rare case of a 26-year-old male with MS who had sequential recurrent spontaneous scleral rupture which required surgical repair. He suffered from a similar problem 4 years later in both eyes in a different location, with overlying thin cystic blebs and hypotony maculopathy. Surgical repair with preserved scleral donor patch graft and conjunctival autograft in one eye, and conjunctival advancement in the other eye was performed. This helped stabilise the eyes, and resulted in complete visual recovery in both eyes.

[Management of Radius-Maumenee syndrome : Treatment with deep sclerectomy, viscocanalostomy and collagen matrix implantation]. / Management des Radius-Maumenee-Syndroms : Therapie mit tiefer Sklerektomie, Viskokanalostomie und Kollagenmatriximplantation.

We describe the case of an 18-year-old female patient who presented with vasodilation of the episcleral vessels in both eyes. The dilated vessels were more prominent in the right eye and, furthermore, examination of the fundus oculi showed a glaucomatous excavation of the right optic disc. No underlying eye or systemic disease was found as the cause for the vasodilation, therefore, Radius-Maumenee syndrome (idiopathic dilated episcleral vessels with secondary open angle glaucoma) was diagnosed. Radius-Maumenee syndrome is a diagnosis by exclusion. If no underlying disease can be detected primary therapy of the glaucoma is required. Carotid cavernous fistulas as the most common cause for dilated episcleral vessels and elevated episcleral venous pressure should be ruled out. Due to the progression of the excavation and the pathologically elevated intraocular pressure in the right eye of our patient we decided to perform a deep sclerectomy in combination with viscocanalostomy and implantation of a collagen matrix. The operation and postoperative period were free from complications. Two months after the surgical procedure the right eye showed a normalized intraocular pressure of 7 mmHg but no morphological changes in terms of reduction of the vasodilation. In summary, deep sclerectomy in combination with viscocanalostomy and implantation of a collagen matrix showed good results in the reduction of intraocular eye pressure in Radius-Maumenee syndrome.

Presentation and Management Outcomes of Corneal and Scleral Perforations in Geriatric Nursing Home Residents.

We compared the clinical presentation and treatment outcomes of corneal and scleral perforations in geriatric nursing home residents, geriatric community residents, and non-geriatric population. The medical records of patients who were treated for corneal and scleral perforations at the Prince of Wales Hospital, Hong Kong between January 1, 2004 and May 1, 2013, were reviewed retrospectively. Of 144 cases, 53 (37%) occurred in the geriatric population, of which 16 (11%) lived in nursing homes, and 37 (26%) were community residents. There were 91 (63%) patients in the non-geriatric group. The mean age of the patients in nursing home geriatric group was 86.5 years (87.5% females). The most common etiology of perforation was trauma. Rupture due to fall was more common in geriatric patients (P < 0.001) whereas laceration due to penetrating eye injury was more common in non-geriatric patients (P < 0.001). There were more cases of infection leading to spontaneous perforation in geriatric nursing home group compared to the other groups (P = 0.001). In the geriatric nursing home group, visual acuity at presentation (P < 0.001) and postoperative visual acuity (P = 0.012) was worse compared to the other groups. Our study showed that corneal and scleral perforations in the geriatric nursing home residents carry a poor visual prognosis. The causes and anatomical outcomes of such events in geriatric age group differ from those in the general population. In our study, geriatric patients residing in nursing homes had worse baseline as well as posttreatment visual acuity, compared to community residents.

Ocular Surface Squamous Neoplasia in 200 Patients: A Case-Control Study of Immunosuppression Resulting from Human Immunodeficiency Virus versus Immunocompetency.

PURPOSE: To describe and compare the clinical presentation, treatment outcomes, and histopathologic features of ocular surface squamous neoplasia (OSSN) based on human immunodeficiency virus (HIV) status. DESIGN: Case-control study. PARTICIPANTS: A total of 200 patients with OSSN, of whom 83 (41%) had positive results for HIV and were classified as cases and 117 (59%) had negative results for HIV and were classified as controls. METHODS: Enzyme-linked immunosorbent assay for HIV, conjuntival excision biopsy, extended enucleation, orbital exenteration. MAIN OUTCOME MEASURES: Clinical features, treatment outcomes, and histopathologic characteristics. RESULTS: The mean age at presentation of OSSN in both cases and controls was 40 years (median, 40 years; range, 13-65 years) and in controls was 40 years (median, 38 years; range, 15-80 years). On comparison of cases versus controls with OSSN, HIV-positive individuals had larger (12 vs. 8 mm; P < 0.001) and thicker (3.2 vs. 2.3 mm; P = 0.041) tumors, with a higher incidence of corneal (60% vs. 40%; P = 0.007), scleral (19% vs. 9%; P = 0.044), and orbital (13% vs. 3%; P = 0.019) invasion and a higher need for extended enucleation or exenteration (27% vs. 11%; P < 0.001). The bilateral presentation (11% vs. 4%; P = 0.13), need for lamellar sclerectomy (13% vs. 8%; P = 0.29), and tumor recurrence after primary treatment (30% vs. 20%; P = 0.12) was higher in HIV-positive cases compared with HIV-negative controls. However, these features were not statistically significant. Based on American Joint Committee on Cancer classification, T1 tumor was more common in controls (13% in cases vs. 35% in controls; P = 0.0009), and T4 tumor was more common in cases (13% in cases vs. 4% in controls; P = 0.019). None of the patients demonstrated systemic metastases or died of disease during a mean follow-up period of 10 months (median, 4 months; range, <1-75 months) in cases and 9 months (median, 4 months; range, <1-99 months) in controls. CONCLUSIONS: Ocular surface squamous neoplasia in HIV-positive individuals is aggressive with larger and thicker tumors and with higher incidence of corneal, scleral, and orbital invasion. These patients are associated with poor ocular prognosis with higher need for extended enucleation, exenteration, or both.

Honeybee sting of the sclera: occular features, treatment, outcome and presumed pathogenesis.

Ocular bee sting injury has caused several reactions in the eye but has rarely been reported among local African farmers, and Nigerians in particular. This case seeks to report the first ocular and external eye reactions following a honey bee sting of the eye through the sclera, highlighting the treatment and outcome. Oral interview, clinical examination and external photographs were used to obtain and document findings. Medical treatment was instituted as soon as subject presented. There was complete inflammatory resolution within a week, normal vision and no evidence of stinger migration after four weeks of follow up. The wound site healed with ciliary staphyloma. The role of physical properties, immunological and genetics interplay and the presumed pathogenesis is further discussed. Health education on early presentation and avoidance of harmful traditional eye medications should be promoted among the farming populations in our communities, in order to prevent blinding complications.

On the ocular findings in ochronosis: a systematic review of literature.

BACKGROUND: Ochronosis/Alkaptonuria is a tyrosine metabolism disorder where accumulation of homogentisic acid, in eye, skin, cartilage and several other connective tissues leads to a black pigmentation of the affected tissues. It is autosomal-recessive inherited in men with a frequency of 1-9/1,000,000. While it is clear that pigment deposits lead to joint destruction, renal stone formation and cardiac valvulopathy respectively, the significance of ocular findings is still unclear. We therefore aim to evaluate the frequency and clinical significance of ocular findings in ochronosis and discuss possible therapeutic options. METHODS: Systematic review of literature via Medline and Web of Science. Only case reports in English, German, French, Spanish or Italian documenting detailed ophthalmologic examination were included. RESULTS: Our search revealed 36 case reports including 40 patients. Average age at the onset of ocular signs was 40.6 years. The most frequent sign was symmetric brown sclera pigmentation present in 82.5 percent of the patients. "Oil-drops", brown pigment spots in the limbus are generally considered pathognomonic but were a little less frequent (75 percent). Vermiform pigment deposits at the level of the conjunctiva or increased conjunctival vessel diameter is also frequent. We found an increased incidence of central vein occlusion and elevated intraocular pressure going along with chamber angle hyperpigmentation. Another condition observed twice is rapid progressive astigmatism attributable to corneoscleral pigment accumulation. CONCLUSION: Our observations suggest that ocular findings are of double relevance. First, characteristic ocular findings can anticipate the time of diagnosis and second, ocular findings may complicate to various conditions putting sight at risk. Opthalmologists and general physicians should be aware of both. Therapeutic options include protein restriction, administration of high dose vitamin C or nitisonone. Evidence for all of them is limited.

Injerto-parche de esclera en perforación corneana secundaria a queratitis periférica ulcerativa / Scleral graft patch in corneal perforation due to peripheral ulcerative keratitis

Objetivo: reportar un caso de perforación corneana manejado con injerto-parche de esclera. Diseño: reporte de caso. Discusión: las perforaciones corneanas requieren tratamiento quirúrgico urgente para reducir las complicaciones. Contamos con varias opciones de acuerdo al tamaño de la perforación incluyendo adhesivo tisular, transplante de membrana amniótica y queratoplastia penetrante. El injerto-parche de esclera es otra alternativa útil para el tratamiento de esta condición. Conclusiones: describimos el caso de una perforación corneana secundaria a queratitis ulcerativa periférica (QPU) asociada a Síndrome de Sjögren que fue manejada con un aloinjerto de esclera.

Objective: to report a case in which scleral patch graft was used to treat a corneal perforation. Design: case report. Discussion: corneal perforations require urgent management to reduce subsequent ocular morbidity. Depending on its size and location, treatment options include corneal gluing, amniotic membrane transplantation and corneal transplantation. Scleral patch grafting is another feasible alternative for treating this condition.Conclusions: We described a case of scleral allograft use in the management of corneal perforation due to peripheral ulcerative keratitis associated with Sjögren's syndrome.

Short-term effect of cryotherapy on human scleral tissue by atomic force microscopy.

This study investigated the inflammatory effect of cryotherapy application on collagen matrix network in human infant sclera. Donor scleral tissues taken from three infant patients divided into five groups: control group, sham-treated group, and three cryotreated groups. In the cryotherapy groups, the sclera was treated for 5 s, 10 s, and 20 s with -80°C freezing by a cryosurgical system. The cryotreated reactions were examined using double histological analysis with hematoxylin-eosin and Masson's trichrome, and atomic force microscopy analysis to quantify the diameter and D-banding of collagen fibrils. The infant scleral tissues treated with cryotherapy showed a significantly increased collagen density associated with inflammatory response (p < 0.05), increased fibril diameter (p < 0.005) compared to the scleral tissues in the control group. The results directly suggest that the cryotherapy affects the morphology of scleral collagen.

Large post-traumatic ciliary staphyloma--a simple, safe and successful management.

Surgical management of scleral staphylomas is challenging and is usually associated with poor visual outcome. A middle aged lady developed a large post-traumatic scleral staphyloma following injury with a cow's horn. She sought treatment for poor vision and cosmetic blemish. She was satisfactorily managed by surface diathermy and closure of defect with partial thickness rotational scleral autografts and her corrected visual improvement was 6/12.

Juvenile xanthogranuloma of the corneoscleral limbus: report of two cases.

PURPOSE: To report the clinical and histopathologic findings of limbal juvenile xanthogranuloma (JXG) in two children. METHODS: Case report. RESULTS: Two children (a 9-month old boy and a 4-year old girl) presented with an enlarging yellowish mass at the corneoscleral limbus. The girl had been diagnosed with neurofibromatosis 1 (NF1). Both lesions underwent simple excision. Histopathologic examination revealed foamy histiocytes within an inflammatory infiltrate with the presence of multiple multinucleated giant cells. In both cases intraoperative subconjunctival steroids and postoperative topical steroids were administered during 1 month. CONCLUSIONS: Juvenile xanthogranuloma may rarely present as a mass occurring at the corneoscleral limbus. Therefore it should be considered in the differential diagnosis of any corneoscleral limbal mass lesion, particularly in children. Most are isolated lesions without systemic involvement which are treated by simple excision. But association with NF1 is possible and in that case screening for hematological malignancies is recommended.