Background

Corneal edema occurs for many reasons, but it is often a sequela of intraocular surgery. Corneal
edema resulting from cataract extraction is called either pseudophakic bullous keratopathy
(PBK) or aphakic bullous keratopathy (ABK). Knowledge of the structure of the cornea and the
proper functioning of its layers is fundamental to understanding corneal edema.
Pathophysiology

Bullous keratopathy occurs when the corneal endothelium becomes damaged and, as a result, the
cornea swells. The inner cell layer of the cornea, the endothelium, is responsible for maintaining
the cornea in a relatively dehydrated state. As endothelial cells are damaged, the remaining cells
rearrange themselves to cover the posterior corneal surface. The remaining endothelial cells
become irregularly shaped and enlarged, and their pump functions begin to fail.
As the endothelium becomes increasingly unable to act as a pump to deturgesce the cornea, the
stroma begins to swell, especially in the central cornea. As the stroma swells, the cornea thickens
and folds are seen in the Descemet membrane. The edema may fluctuate in response to changing
intraocular pressure with higher pressures leading to more edema. At this point, maintenance of
intraocular pressure at a low level is important. The combination of variable endothelial function
and variable intraocular pressure determines the extent of corneal edema.
Epithelial edema manifests as fluid accumulation between the basal epithelial cells. With
increased fluid accumulation, blisters and then bullae develop. Epithelial edema may result from
anterior movement of aqueous and fluid in the stroma driven by intraocular pressure. With a
small amount of epithelial edema, environmental factors (eg, temperature, humidity) may affect
evaporation of tears with blinking. At night with the eye closed, epithelial edema typically
worsens due to a lack of tear evaporation. This results in symptoms that are generally worse in
the morning hours.
Patients with bullous keratopathy demonstrate decreased visual acuity and can have symptoms of
pain or discomfort. Decreased visual acuity is related to the inability of the stroma to maintain its
deturgescence, which often is followed by epithelial edema. Epithelial edema can be responsible
for great changes in visual acuity due to irregularity in the corneal refractive surface.
Examination with contact lens over refraction may be the best way to confirm the status of the
posterior segment.
Pain associated with bullous keratopathy can be due to swelling of the epithelium with resultant
stretching of corneal nerves or rupture of bullae with exposure of corneal nerve endings to an
often noxious environment. Bullae rupture results in pain, photophobia, and epiphora.
Subsequent epithelial defects predispose the cornea to infection and can contribute to the
development of anterior uveitis.
Epidemiology

Frequency
United States

Prior to implantation of intraocular lenses, in the era of intracapsular cataract extraction and
postoperative aphakia, the rate of ABK was reported to be less than 1% in uncomplicated cases
without vitreous loss. Early results with implantation of anterior chamber intraocular lenses by
Barraquer in the 1950s, while initially promising, ultimately resulted in corneal decompensation
in half of the postoperative eyes. As intraocular lenses have evolved, these rates have steadily
dropped. In the modern era, numerous closed loop anterior chamber intraocular lenses have
consistently resulted in an elevated risk of PBK relative to flexible open loop anterior chamber
and posterior chamber intraocular lenses. Despite improved surgical techniques, PBK remains a
leading indication for penetrating keratoplasty because of the high volume of cataract surgery
performed.
Several studies in the 1980s demonstrated rates of corneal decompensation after uncomplicated
extracapsular cataract extraction with posterior chamber intraocular lens placement to be 0.10.5%. In the setting of vitreous loss, the rate of corneal edema 4 years postoperatively has been
reported to increase to 2.4%.
Mortality/Morbidity
Corneal bullae may cause pain.
Age
Most cataract surgery is performed after age 65 years; thus, this condition is more frequent in
elderly persons.
http://emedicine.medscape.com/article/1194994-overview#showall