What is the Retina?
About Pediatric Retina
Pediatric retina includes several rare diseases, many involving genetic predisposition, infection, trauma, or prematurity. Certain conditions, like retinopathy of prematurity, can develop in very premature infants while in neonatal nurseries and require multiple screenings.
Find a Pediatric Retina Specialist
Pediatric Retina Conditions
There are different kinds of pediatric retina conditions, including the following:
- Coats disease
- Familial exudative vitreoretinopathy (FEVR)
- Penetrating retinal injuries
- Retinopathy of prematurity (ROP)
- Stargardt disease
- X-linked retinoschisis
Symptoms
If you or your child notices any of the following, see a pediatric ophthalmologist:
- White pupils or an eye that appears white (rather than red) in photographs
- Full or partial vision loss
- Night blindness
- Black floaters within the field of vision
- Sudden, persistent flashing lights
- Any dramatic changes to the appearance of the eye
- Misalignment of the eyes or trouble focusing
- Sensitivity to light
Treatment
Surgical Care
Pediatric surgeries are performed by University of Utah Health physicians at the John A. Moran Eye Center Surgery Unit and at Primary Children’s Hospital, a Level 1 Pediatric Trauma Center. Both operating suites are fully equipped to perform intricate vitreoretinal procedures on premature babies, infants, and children.
The anesthesiologists at Primary Children’s specialize in anesthesia services for the tiniest preterm infant on up, and are skilled in managing anesthesia for the medically complex child during difficult procedures.
Coats Disease
Causes & Symptoms
In Coats disease, the blood vessels in the retina leak into the back of the eye. This leakage causes the retina to swell and can eventually cause partial or complete retinal detachment. The disease can develop gradually and involves both eyes but usually causes symptoms in only one eye.
Retinal detachment from Coats disease can appear like retinoblastoma, so it is important to have careful evaluations with both a pediatric retinal specialist and pediatric ophthalmologist to help ensure an accurate diagnosis and swift treatment.
Treatment
Treatment for Coats disease depends on the stage of the disease. If caught early, a laser can be used to constrict the abnormal blood vessels and stop the leakage of fluid. In more advanced stages, surgery becomes necessary to remove excess fluid. Surgery may also be necessary if partial or complete detachment of the retina has occurred.
Coats disease can recur throughout life, and it is important to schedule regular follow up appointments with a pediatric retina specialist.
Familial Exudative Virtreoretinopathy (FEVR)
Causes & Symptoms
Familial exudative vitreoretinopathy (FEVR) is a genetic condition that affects the blood vessels that support the retina. FEVR is sometimes symptomless, but it can cause a wide variety of eye issues including bleeding, retinal detachment, and excessive myopia that can be helped with glasses.
FEVR is diagnosed by a retinal specialist after a full eye exam that may include a fluorescein angiography test, which shows any leakage from damaged blood vessels.
Treatment
Treatment with laser or surgery may be performed.
Penetrating Retinal Injuries
Causes & Symptoms
A penetrating injury occurs when a foreign object becomes embedded in the eye. Such objects can increase risk of serious infection or can be toxic. Surgery is often necessary, beginning with a vitrectomy, which removes the foreign body as well as any loose blood or particles. Subsequent surgeries may be needed.
Treatment
Trauma often affects multiple tissues and functions in the eye. Because of this, retinal specialists often coordinate care with other specialists, including experts in glaucoma, oculoplastics, cornea, cataract, and pediatric ophthalmology.
Retinopathy of Prematurity (ROP)
Retinopathy of prematurity (ROP) affects very premature babies. In this disease, abnormal blood vessels grow between the retina and the front of the eye. These blood vessels are fragile and can bleed. The exact causes of ROP are unknown, but poor infant growth and genetic factors are suspected factors. Babies born weighing less than 2.75 pounds or before 31 weeks gestation are the most at risk for developing ROP.
Stages of Disease
ROP is classified in five stages of increasing severity.
Stages 1 & 2
In early ROP, which includes stages 1 and 2, development of the retina’s blood vessels is delayed. These stages usually resolve without treatment. However, ongoing monitoring from a pediatric ophthalmologist is important to determine whether glasses or other treatments are needed to ensure healthy visual development.
Stage 3
Early ROP can develop over several weeks into stage 3 ROP, where blood vessels grow off the surface of the retina and into the center of the eye. Laser therapy can often be used to reduce abnormal blood vessels. In cases where the laser cannot be used, medications can be a viable alternative. A retina specialist can provide more information on treatment options.
Stages 4 & 5
Stages 4 and 5 of ROP result in partial or total retinal detachment. In these cases, microsurgery is required to prevent additional detachment of the retina or to reattach it. In most cases, careful monitoring and treatment of earlier stages of ROP prevent it from reaching these advanced stages.
Rehabilitation
It is important that patients with ROP receive rehabilitation with a pediatric ophthalmologist. Children who have recovered from ROP are at a high risk of conditions like strabismus (improper alignment of the eyes) and refractive errors, which can impair visual development.
Learn more about retinopathy of prematurity:
Stargardt Disease
Stargardt disease is an inherited form of macular degeneration that affects children and young adults. It causes deterioration of the cells in the center of the retina that convey information to the brain.
Over time, Stargardt disease causes a blurred, distorted, dark, or empty area in the center of vision. The center of the retina (known as the macula) is responsible for our ability to see color and fine detail when looking directly at an object, and diseases like Stargardt affect the individual’s ability to drive and read.
Treatment
Definitive treatment is not yet available, but it is recommended that patients avoid bright light and vitamin A supplements.
Learn more about Stargardt disease:
X-Linked Retinoschisis (XLR)
Causes
X-linked retinoschisis (XLR) causes a splitting of the retina and can affect the central and side vision. It is an inherited disease and occurs almost exclusively in males. Because XLR causes the layers of the retina split more easily than normal, jarring or eye trauma may cause injury to the retina.
Symptoms
Early symptoms include the following:
- A decline in vision
- Inability of both eyes to focus on an object (strabismus)
- Roving, involuntary eye movement (nystagmus)
These symptoms can occur in infancy or later in childhood.
Treatment
Imaging studies can be helpful in XLR, and occasionally a trial of eye drops or medications may be considered. Surgery may also be recommended.