35 articles
Adherent leukoma
Adherent leukoma (leukoma adhaerens, leucome adhérent): a dense white corneal scar with iris fused to its back surface after corneal perforation; ICD-10 H17.0, ICD-11 9A77.1; treated by keratoplasty.
Autoimmune keratitis
Autoimmune keratitis (ICD-11 9A75) is immune-mediated corneal inflammation, chiefly peripheral ulcerative keratitis, linked to rheumatoid arthritis, Sjögren syndrome, SLE, and ANCA vasculitis; treated with systemic immunosuppression and surgery for perforation.
Autosomal dominant keratitis
Autosomal dominant keratitis (hereditary keratitis, OMIM 148190) is a rare PAX6-related inflammatory corneal disorder with peripheral stromal opacification, vascularization, foveal hypoplasia, and childhood onset.
Blue sclera
Blue sclera (blue sclerae), ICD-11 LA11.0: a blue, gray, or purplish tint of the eye's white sclera from translucency letting the uvea show through. Causes include osteogenesis imperfecta, Marfan and Ehlers-Danlos syndromes, iron deficiency, and drugs such as minocycline.
Central cloudy dystrophy of François
Central cloudy dystrophy of François (CCDF, MIM 217600) is a rare stromal corneal dystrophy with central polygonal gray opacities, first described in 1955, nonprogressive, asymptomatic, possibly identical to posterior crocodile shagreen.
Congenital corneal opacity
Congenital corneal opacity (CCO) is clouding of the cornea present at birth or within the first 4 weeks of life, also called congenital corneal opacification. Peters anomaly is its most common cause; key genes include PAX6, FOXC1, FOXE3, and B3GALTL.
Congenital hereditary endothelial dystrophy type II
Congenital hereditary endothelial dystrophy type II (CHED2, Maumenee corneal dystrophy, OMIM 217700) is a rare autosomal recessive corneal dystrophy caused by SLC4A11 mutations on 20p13, causing bilateral corneal clouding from birth, treated by keratoplasty.
Congenital stromal corneal dystrophy
Congenital stromal corneal dystrophy (CSCD, congenital hereditary stromal dystrophy, Witschel dystrophy, OMIM 610048) is an extremely rare autosomal dominant DCN (decorin) disorder causing bilateral flaky corneal stromal clouding from birth and moderate to severe vision loss, treated with keratoplasty.
Corneal neovascularization
Corneal neovascularization: abnormal ingrowth of new blood vessels from the limbus into the normally avascular cornea, caused by hypoxia, inflammation, infection, or graft rejection; treated with steroids, anti-VEGF agents, and laser therapy.
Corneal oedema
Corneal oedema (corneal edema) is excess water in the cornea causing swelling and loss of transparency, mainly from endothelial dysfunction (Fuchs dystrophy, bullous keratopathy, cataract surgery). Treated with hypertonic saline or endothelial keratoplasty.
Corneal scars or opacities
Corneal scars or opacities (leukoma; ICD-10 H17, ICD-11 9A77) are loss of corneal transparency from scarring by infectious, inflammatory, congenital, dystrophic, or traumatic causes, treated mainly by keratoplasty.
Corneal staphyloma
Corneal staphyloma (anterior staphyloma) is a rare ectatic, uveal-lined bulging of an opaque cornea, congenital or acquired after corneal perforation, treated by sclerokeratoplasty or penetrating keratoplasty rather than evisceration.
Epithelial basement membrane dystrophy
Epithelial basement membrane dystrophy (EBMD), also called map-dot-fingerprint or Cogan microcystic dystrophy, is a common bilateral anterior corneal dystrophy causing map, dot, and fingerprint lines, reduced vision, and recurrent corneal erosions in about 10% of patients.
Epithelial recurrent erosion dystrophy
Epithelial recurrent erosion dystrophy (ERED, OMIM 122400), also called Dystrophia Smolandiensis or recurrent hereditary corneal erosions, is a rare autosomal dominant COL17A1 corneal dystrophy causing painful corneal erosions from childhood that lessen by the fourth decade.
Gelatinous drop-like corneal dystrophy
Gelatinous drop-like corneal dystrophy (GDLD, OMIM 204870), also called subepithelial amyloidosis of the cornea, is a rare autosomal recessive TACSTD2-related corneal amyloidosis causing childhood-onset vision loss, commonest in Japan, with recurrent deposits after keratoplasty.
Idiopathic scleritis
Idiopathic scleritis (ORPHA:648675, ICD-10 H15.0) is painful inflammation of the sclera without infection or systemic autoimmune disease; the most common scleritis category, treated with NSAIDs, corticosteroids, or methotrexate.
Immune-mediated scleritis
Immune-mediated scleritis (ORPHA:648681, ICD-10 H15.0) is a rare, severe scleral inflammation linked to systemic autoimmune disease, most often rheumatoid arthritis or granulomatosis with polyangiitis, typically granulomatous or necrotizing and treated with NSAIDs, corticosteroids, and immunosuppressives.
Infectious scleritis
Infectious scleritis is microbial infection of the sclera, 5–10% of scleritis cases, usually bacterial (Pseudomonas aeruginosa) after pterygium surgery or trauma; distinct from autoimmune scleritis, needing antimicrobials plus debridement.
Iridocorneal endothelial syndrome
Iridocorneal endothelial (ICE) syndrome, also called Chandler syndrome, progressive iris atrophy, or Cogan-Reese syndrome, is a rare unilateral disorder in which abnormal corneal endothelial cells migrate over the angle and iris, causing secondary glaucoma and corneal edema.
Limbal stem cell deficiency
Limbal stem cell deficiency (LSCD), a rare corneal disorder (1-5/10,000) from loss or dysfunction of limbal stem cells, causing corneal conjunctivalization, neovascularization, and vision loss; treated with limbal transplants such as CLAU, SLET, and CLET.
Lisch epithelial corneal dystrophy
Lisch epithelial corneal dystrophy, also called band-shaped and whorled microcystic dystrophy, is a rare corneal dystrophy with feathery gray opacities that first appear in childhood and slowly progress.
Macular corneal dystrophy
Macular corneal dystrophy is a rare autosomal recessive corneal disease caused by CHST6 gene mutations, in which glycosaminoglycan buildup clouds both corneas and progressively impairs vision.
Meesmann corneal dystrophy
Meesmann corneal dystrophy (MECD, juvenile epithelial corneal dystrophy, Meesmann-Wilke syndrome) is a rare autosomal dominant corneal epithelial disorder from KRT3 or KRT12 mutations, causing tiny intraepithelial microcysts with little vision loss.
Mooren ulcer
Mooren ulcer (ulcere de Mooren), also called rodent ulcer, is a rare idiopathic autoimmune peripheral ulcerative keratitis of the cornea, diagnosed by exclusion, bilateral in about one third of cases, treated with a stepladder immunosuppressive approach.
Posterior polymorphous corneal dystrophy
Posterior polymorphous corneal dystrophy (PPCD, Schlichting dystrophy) is a rare autosomal dominant corneal endothelial dystrophy with vesicular, band-like, and diffuse Descemet membrane opacities, subtypes PPCD1-4 linked to OVOL2, COL8A2, ZEB1, and GRHL2, and variable severity from asymptomatic to glaucoma and corneal edema.
Pre-Descemet corneal dystrophy
Pre-Descemet corneal dystrophy (PDCD), including punctiform and polychromatic PPPCD, is a rare inherited stromal corneal dystrophy with deep stromal opacities anterior to Descemet membrane, asymptomatic, PRDX3-linked, good prognosis.
Recurrent erosion of cornea
Recurrent corneal erosion (RCE, recurrent erosion syndrome): repeated breakdown of the corneal epithelium causing sharp morning eye pain, usually after prior trauma (45–64% of cases) or epithelial basement membrane dystrophy; treated with lubricants, bandage lenses, and surgery.
Reis-Bücklers corneal dystrophy
Reis-Bücklers corneal dystrophy (RBCD, granular corneal dystrophy type 3, CDB1) is a rare autosomal dominant TGFBI corneal dystrophy causing painful recurrent erosions and geographic Bowman layer opacities, distinguished from Thiel-Behnke dystrophy.
Ring dermoid of cornea
Ring dermoid of cornea (ring dermoid syndrome, OMIM 180550, ORPHA:91481) is a rare autosomal dominant condition of bilateral annular limbal dermoids extending onto cornea and conjunctiva, linked to PITX2 mutations, with fewer than 30 reported cases and no extraocular anomalies.
Schnyder corneal dystrophy
Schnyder corneal dystrophy (SCD), formerly Schnyder crystalline corneal dystrophy, is a rare autosomal dominant stromal corneal dystrophy caused by UBIAD1 mutations on 1p36, with cholesterol and phospholipid corneal deposits, crystals in only about half of patients, and hypercholesterolemia association.