Acute hydrops of the cornea occur, manifested by sudden diminution of vision associated with central corneal edema. Predisposition: The progressive corneal thinning in advanced keratoconus can lead to a rupture in Descemet's membrane as well as the overlying endothelium. Acute corneal hydrops (CH) is the development of marked corneal edema due to a tear in Descemet membrane (DM) followed by leakage of aqueous into stroma. Recent evidence suggests that corneal hydrops is strongly associated with mitral valve prolapse. Contact lenses may correct vision satisfactorily in early stages of the disease but up to 20% of patients will eventually need a corneal transplant. If there is significant corneal compromise, a topical antibiotic can be considered to prevent a secondary infection. Although initially it was reported in cases of keratoconus (KC), subsequently it has also been reported in other corneal ectasias including pellucid marginal degeneration (PMD), keratoglobus, Terrien's marginal degeneration (TMD), LASIK-associated keratectasia, keratectasia after radial keratotomy (RK), deep anterior lamellar keratoplasty (DALK), and penetrating keratoplasty (PKP) for KC. Clinical profile and risk factors for keratoplasty and development of hydrops in north Indian patients with keratoconus.
Efficacy and safety of intracameral perfluoropropane (C3F8) tamponade and compression sutures for the management of acute corneal hydrops.

Spontaneous corneal hydrops and perforation in keratoconus and pellucid marginal degeneration. Amniotic membrane transplantation with cauterization for keratoconus complicated by persistent hydrops in mentally retarded patients.
Management of acute corneal hydrops secondary to keratoconus with intracameral injection of sulfur hexafluoride (SF6). Intrastromal fluid drainage with air tamponade: Anterior segment optical coherence tomography guided technique for the management of acute corneal hydrops. Possible predisposing factors for the development of hydrops include the presence of systemic allergies, eye rubbing, Down's syndrome, rapid progression of ectasia, and eccentric cones. Acute hydrops usually clears gradually without treatment but often leaves apical and Descemet membrane scarring.
Anti-glaucoma medications may be used to decrease the hydrodynamic force on the posterior cornea. According to various studies the resolution of corneal edema may occur any time between 5 and 36 week.
Corneal edema can be graded according to its extent; grade 1 within a circle of 3 mm diameter, grade 2 between circles of 3 and 5 mm diameters, and grade 3 larger than a circle of 5 mm diameter.

Investigations are required to determine the size and extent of edema and DM tear which helps in formulating the treatment plan, monitoring the response to treatment, and identifying any complication. It includes topical lubricants, antibiotics (prevent secondary infection), cycloplegics (to reduce pain and photophobia), hypertonic saline eye drops (help draw fluid), anti-glaucoma medications (to lessen the hydrodynamic force on the posterior cornea), and topical steroids or nonsteroidal anti-inflammatory drugs (NSAIDs). Antiglaucoma treatment may have to be given to avoid any rise of intraocular pressure (IOP). In KC the cone usually does not involve the central area, hence VA shows some improvement after healing of the hydrops. For extensive corneal edema and the presence of multiple stromal fluid pockets we have standardized the new technique of intrastromal fluid drainage with air tamponade which is guided by ASOCT. Although intracameral gas injection does not affect the final visual outcome, it reduces the duration of morbidity and also the risk of complications such as corneal neovascularization that may jeopardize the subsequent graft.
Newer treatment modalities such as tissue adhesive, AMG, and compressive sutures may further widen the available options for corneal surgeons but further studies are required to validate these techniques.

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