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Blue Sclerae
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Blue Sclerae

My daughter has optic nerve hypoplasia (concurrent with her dx of septo-optic dysplasia). Her optic nerves are extremely underdeveloped, but she does have some sight. For those who don't know, SOD also includes panhypopituitarism, so is treated for hormone deficiencies as well. She also has ventriculomegaly and mild hydrocephalus.

Her sclerae have steadily been getting more bluish in color. This has not been present since birth, but has developed over time. She is on a fairly low dose of corticosteroids, so I don't think this would cause the blue sclerae. Her grandmother has Ehlers-Danlos syndrome, but I don't think I do, so it would unlikely that my daughter would have this syndrome. I've looked at the other syndromes that cause blue sclerae, and none seem to apply. She doesn't have Marfan's, Diamond-Blackfan anemia, Osteogenesis imperfecta, or any of those connective tissue disorders. What could be causing the blue sclerae?
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Hi Megan,   You've obviously done your home-work. For others that might read this posting let me give some basic information to them.

The white part of the eye-the sclera- is normally white and is the outer of the three main layers of the eye 'wall'. Normally it is whitish in color, under the sclera is the 'choroid' the layer that carrys the arteries, veins and other blood vessels of the eye. The choroid has pigment cells in it and is dark blue in color, under the choroid and nearest the center of the eye is the 'retina'. The retina is the sight forming tissue of the eye. The retina is colorless and transparent in health.

When you look at someone's eye you see blood vessels that appear to be on the surface of the sclera. These are in the 'conjunctiva' another transparent, elastic tissue that covers the sclera on the surface that we see when we look at someone's eyes. If the conjunctiva is irritated due to allergy, infection, or trauma the blood vessels dilate and the eye looks 'blood-shot' or 'pink'.

The most common color change of the visible sclera is a yellowing that comes with age. This is really a result of the elastic fibers in the conjunctiva yellowing with time and exposure to ultraviolet light from the sun. A yellow thickening often develops at the 3  and 9 o'clock positions called a pingueculum. (a good reason to protect your eyes from extended bright sunlight with a hat and ultra-violet blocking sunglasses).


Now to your specific question. A blue appearance of the sclera is really due to thinning of the sclera allowing the bluish color of the choroid to show through. In older adults there often develops a localized blue spot on the visible conjunctiva called a 'scleral plaque'. It is not a serious problem and does not require any treatment.  When Ophthalmologists (EyeMDs or MD physicians that do medical and surgical eye care) operate on the back of the eye (especially retinal detachment surgery) there are often areas of blue sclera, sometimes rather large, that represent splits or almost abscent areas of sclera. These are called 'staphylomas'.

Diffuse blue sclera results from thinning of the sclera. This might be present at birth or develop in childhood. Blue sclerae are most importantly associated with types I and II osteogenesis imperfecta. Blue sclera may also be associated with:

Ehler's danlos syndrome
pseudoxanthoma elasticum
Marfan's syndrome

Blue sclera may also be acquired through long-term use of corticosteroids.

There is no specific treatment usually done for blue sclera. Diagnosis and treatment of any underlying disease is very important. If a localized area of thinning is about to rupture sometimes a 'scleral patch graft' can be used to strengthen the area.

With your daughter's complex history and the excellent job her physicians have done making these unusual and often difficult diagnoses, I'm sure she's seen a pediatric ophthalmologist and an endocrinologist. They would be in the best position to answer your question more specific to her case. I suggest you discuss your concern with them.

You sound like a great mom that will make sure your daughter gets the best care possible. Good luck to both of you.

JCH MD


2 Comments
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Thank you for your reply. I have spoken with both her endocrinologist and her ophthalmologist about this issue. While both of them are very good doctors, and very knowledgeable, they have not been able to give me a definitive answer. Since this problem seems to be progressive, I am more inclined to wonder about it than if it were a stable condition. I have also spoken to her geneticist, who has been unable to attribute this symptom to any syndrome. I was just looking for a fresh perspective. I appreciate your time.
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Hello,
I white parts of my eyes are also blue and I have Ehlers Danlos Syndrome. If you have any further questions or need help regarding EDS I suggest you join an EDS support group such as CEDA which is the   Canadian Ehlers Danlos Association. There are members from all over the world there. There is a CEDA message board where you can ask questions and it is one of Yahoo's support groups. Go to ***@****   Since joining I have been able to access all types of medical documentation to take to doctors and help with my care. EDS is not as rare as one thought and can cause many problems such as extreme pain, bruising, joint hypermobility, aneurysm's and eye/ear/nose and throat problems.
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