ANIRIDIA
Definition
refers to the absence of the iris, usually involving both eyes.
PATHOPHYSIOLOGY
The AN2 region of the short arm of chromosome 11 (11p13) includes the PAX6 gene (named for its PAired boX status), whose gene product helps regulate a cascade of other genetic processes involved in the development of the eye (as well as other non-ocular structures).[3] This PAX6 gene is around 95% similar to the pax gene found in zebrafish, a creature whose ancestors diverged from human evolutionary development around 400 million years ago. Thus the PAX6 gene is highly conserved across evolutionary lineages.
CLINICAL MANIFESTATIONS
The iris may be absent, with only a stump visible on gonioscopy
bacterial ulcers and chronic inflammation of the cornea
Subluxation of the lens due to weak zonules
Poor vision
nystagmus
NURSING MANAGEMENT
Keep the patient's room dimmed at all times
topical antiglaucoma medications may be used
low vision rehabilitation services may be helpful.
Administer antibiotics for infection
give anti-inflammatory drugs for inflammation
MEDICAL MANAGEMENT
Patients with mild keratopathy can be managed with topical preservative-free lubricants
Moderate keratopathy is treated with autologous serum and amniotic membrane transplant.
In patients with severe phase 3 keratopathy, stabilizing the corneal surface involves replenishing limbal stem cells through transplantation.
Cataract extraction improves vision, but the surgeon should be prepared to manage complications in eyes with weak zonular fibers
Specialized intraocular lenses that incorporate an artificial iris diaphragm can be used to correct aniridia at the time of surgery.
NURSING INTERVENTIONS
Give/administer topical preservative-free lubricants
Administer topical glaucomma medications as prescribed.
keep patient away from areas with that are too light
Educate the patient about his or her condition and complications.
HEALTH EDUCATION
Inform and counsel the patient about the importance of surgery
Wearing Sun glasses when exposed to light due to photophobia
Try not to strain your eyes by looking deeply into something that you can't see clearly
Post surgery open your eyes slowly until they are healed completely.