Children"s Syndromes That May Cause Eye Problems to Develop
Updated May 02, 2014.
Written or reviewed by a board-certified physician. See About.com's Medical Review Board.
When we take our kids to the eye doctor, it is most often because the child failed a school vision screening or they are complaining of blurred vision. Or perhaps it's because you noticed an eye turn. However, just as adults, systemic medical conditions can sometimes affect our children’s vision and eye health. Following are four medical conditions that may create significant eye complications in children.
Marfan’s Syndrome
Marfan’s Syndrome is an inherited genetic condition that affects connective tissue. While the prevalence of the condition is fairly rare (about 1 in 5,000), it affects the connective tissue by making it much more elastic than normal. Children with Marfan’s Syndrome have a range of skeletal, cardiac, and eye health problems. Kids affected by this condition are typically tall and thin and have longer than average arms, legs and fingers. They tend to be very flexible, and despite never having a history of weight loss, they may have stretch marks on their skin. Some kids also have scoliosis, an abnormal curvature of the spine and abnormalities in the breast bone. Cardiac problems associated with Marfan's tend to set the condition apart from other genetic connective tissue disorders.
The most common side effect of Marfan's Syndrome that affects the eyes is a condition called ectopia lentis. The lens of the human eye is held in place by lens zonules, little fibers that suspend the lens over the pupil.
In Marfan’s Syndrome, the lens becomes sublimed - zonules break allowing the lens to float upward. In rare cases, the lens can become completely loose and fall back into the eye. Other eye complications of Marfan’s syndrome are nearsightedness, cataract, strabismus (eye turn), glaucoma or retinal detachment.
Ehlers-Danlos Syndrome
Ehlers-Danlos Syndrome is a group of diseases that affect the collagen in our bodies. Children with Ehlers-Danlos tend to bruise easily. Their joints are very mobile and some may have localized pain. This disease can affect blood vessels. Arteries can sometimes rupture which can result in death.
The eye problems associated with Ehlers-Danlos Syndrome include dermatochalasis (upper eyelids that sag down lower), keratoconus (a corneal dystrophy), microcornea (an abnormally small cornea) glaucoma, retinal detachment and myopia. Because this disease can affect blood vessels, a more serious eye complication is the formation of a carotid-cavernous fistula, an abnormal communication between the arteries and veins inside the head. Kids with this condition may have eye pain, swelling and redness in one or both eyes and some may report hearing a pulsating sound inside their heads. This complication requires immediate hospital admission.
Pseudoxanthoma Elasticum
Pseudoxanthoma Elasticum is a progressive disorder that affects about 1 in 25,000 to 1 in 100,000 people. The condition is more common in females, and affects the skin and cardiovascular system. The condition is characterized by calcium deposits in the elastic fibers of connective tissues. Children with this disease have yellowish spots on their skin and can develop an orange peel appearance. Over time their skin can become excessively wrinkled. The eye complication that appears in children affected by Pseudoxanthoma Elasticum occurs in the retina. The retina takes on a mottled appearance. These patients may also develop a retinal condition called angioid streaks. Angioid streaks are breaks in the membrane that makes up part of the retina. These streaks radiate off the optic nerve and are found in 85% of patients with Pseudoxanthoma Elasticum. Angioid streaks allow new blood vessel growth and potential tissue disruption.
Juvenile Idiopathic Arthritis
Juvenile Idiopathic Arthritis (JRA) is a group of autoimmune conditions affecting the immune system around the joints. The condition can affect children under the age of 16. Although there is a strong genetic component to developing JRA, scientists believe there may be an environmental trigger. Patients with JRA have inflammatory joint problems that include pain, stiffness and swollen joints.
The most serious side effect of JRA on the eyes is uveitis. Uveitis is an inflammatory disorder that can cause acute pain and destruction of the anterior structures of the eye. Some patients have a very slow, smoldering case of uveitis and although the patient is not badly affected by acute symptoms, they are at risk for developing glaucoma, cataracts and a corneal opacification called band keratopathy.
Source: Medical Syndromes That Affect Children’s Vision. Review of Optometry, 15 March, 2014 pp70-75.
Written or reviewed by a board-certified physician. See About.com's Medical Review Board.
When we take our kids to the eye doctor, it is most often because the child failed a school vision screening or they are complaining of blurred vision. Or perhaps it's because you noticed an eye turn. However, just as adults, systemic medical conditions can sometimes affect our children’s vision and eye health. Following are four medical conditions that may create significant eye complications in children.
Marfan’s Syndrome
Marfan’s Syndrome is an inherited genetic condition that affects connective tissue. While the prevalence of the condition is fairly rare (about 1 in 5,000), it affects the connective tissue by making it much more elastic than normal. Children with Marfan’s Syndrome have a range of skeletal, cardiac, and eye health problems. Kids affected by this condition are typically tall and thin and have longer than average arms, legs and fingers. They tend to be very flexible, and despite never having a history of weight loss, they may have stretch marks on their skin. Some kids also have scoliosis, an abnormal curvature of the spine and abnormalities in the breast bone. Cardiac problems associated with Marfan's tend to set the condition apart from other genetic connective tissue disorders.
The most common side effect of Marfan's Syndrome that affects the eyes is a condition called ectopia lentis. The lens of the human eye is held in place by lens zonules, little fibers that suspend the lens over the pupil.
In Marfan’s Syndrome, the lens becomes sublimed - zonules break allowing the lens to float upward. In rare cases, the lens can become completely loose and fall back into the eye. Other eye complications of Marfan’s syndrome are nearsightedness, cataract, strabismus (eye turn), glaucoma or retinal detachment.
Ehlers-Danlos Syndrome
Ehlers-Danlos Syndrome is a group of diseases that affect the collagen in our bodies. Children with Ehlers-Danlos tend to bruise easily. Their joints are very mobile and some may have localized pain. This disease can affect blood vessels. Arteries can sometimes rupture which can result in death.
The eye problems associated with Ehlers-Danlos Syndrome include dermatochalasis (upper eyelids that sag down lower), keratoconus (a corneal dystrophy), microcornea (an abnormally small cornea) glaucoma, retinal detachment and myopia. Because this disease can affect blood vessels, a more serious eye complication is the formation of a carotid-cavernous fistula, an abnormal communication between the arteries and veins inside the head. Kids with this condition may have eye pain, swelling and redness in one or both eyes and some may report hearing a pulsating sound inside their heads. This complication requires immediate hospital admission.
Pseudoxanthoma Elasticum
Pseudoxanthoma Elasticum is a progressive disorder that affects about 1 in 25,000 to 1 in 100,000 people. The condition is more common in females, and affects the skin and cardiovascular system. The condition is characterized by calcium deposits in the elastic fibers of connective tissues. Children with this disease have yellowish spots on their skin and can develop an orange peel appearance. Over time their skin can become excessively wrinkled. The eye complication that appears in children affected by Pseudoxanthoma Elasticum occurs in the retina. The retina takes on a mottled appearance. These patients may also develop a retinal condition called angioid streaks. Angioid streaks are breaks in the membrane that makes up part of the retina. These streaks radiate off the optic nerve and are found in 85% of patients with Pseudoxanthoma Elasticum. Angioid streaks allow new blood vessel growth and potential tissue disruption.
Juvenile Idiopathic Arthritis
Juvenile Idiopathic Arthritis (JRA) is a group of autoimmune conditions affecting the immune system around the joints. The condition can affect children under the age of 16. Although there is a strong genetic component to developing JRA, scientists believe there may be an environmental trigger. Patients with JRA have inflammatory joint problems that include pain, stiffness and swollen joints.
The most serious side effect of JRA on the eyes is uveitis. Uveitis is an inflammatory disorder that can cause acute pain and destruction of the anterior structures of the eye. Some patients have a very slow, smoldering case of uveitis and although the patient is not badly affected by acute symptoms, they are at risk for developing glaucoma, cataracts and a corneal opacification called band keratopathy.
Source: Medical Syndromes That Affect Children’s Vision. Review of Optometry, 15 March, 2014 pp70-75.
Source...