Hornhauthaze

Hornhauthaze is a rare ophthalmic condition characterized by diffuse haziness of the cornea, leading to reduced visual acuity and increased light sensitivity. The opacity can be unilateral or bilateral and may progress over months to years. Patients typically report blurry vision, glare, and decreased contrast, with refractive changes that may complicate correction.

Etiology and pathophysiology are not fully understood. Proposed mechanisms include disruption of stromal collagen lamellae, accumulation of extracellular matrix components, or intermittent edema due to epithelial dysfunction. In some instances, a history of ocular trauma, surgery, or inflammatory disease precedes onset. Hereditary cases have been described in rare families, suggesting potential genetic contributions, though no single causative gene is established.

Diagnosis relies on clinical examination and imaging. Slit-lamp biomicroscopy reveals a diffuse, gray-white corneal haze that spans multiple layers of the stroma and possibly the epithelium. Anterior segment optical coherence tomography and corneal pachymetry help assess stromal involvement and thickness. Corneal topography can show irregular astigmatism, while the posterior segment is usually unaffected. Differential diagnosis includes corneal scars from infection or injury, other stromal dystrophies, Fuchs’ endothelial dystrophy with secondary haze, and inflammatory keratitis.

Treatment aims to preserve vision and relieve symptoms. Mild cases may benefit from lubricants and rigid gas-permeable or scleral contact lenses. Superficial haze can be addressed with phototherapeutic keratectomy. In advanced disease, options include deep anterior lamellar keratoplasty or penetrating keratoplasty. Management of any associated inflammation is essential.

Prognosis varies with severity and progression; some patients stabilize after treatment, while others experience continued decline. Hornhauthaze remains exceedingly rare, and evidence guiding standardized care is limited.