Vision Problems: What is Congenital Ptosis?

Ptosis is the medical term for a drooping of the upper eyelid. If a baby is born with this kind of drooping, it is known as congenital ptosis, which is the most common abnormality of the eyelid. "All babies have a bit of facial asymmetry, including their eyelids, probably because of how they lay in the uterus," explains Jane Edmond, M.D., a pediatric neuro-ophthalmologist at Texas Children's Hospital and Baylor College of Medicine, and clinical spokesperson for the American Academy of Ophthalmology. "Most of these minor asymmetries correct themselves in the first few months of life. But if we see a significant lid droop at birth and it does not change over time, we know it's congenital ptosis."

Ptosis can occur in one or both eyelids; the droop can partially or even completely block vision in an affected eye. Ptosis often occurs hand-in-hand with astigmatism because the eyelid rests in an unusual position on top of the cornea. If left untreated, ptosis can lead to amblyopia, especially if the lid droops enough to block vision.

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What Causes Congenital Ptosis?

The muscle that enables you to lift your eyelid is called the levator muscle. In congenital ptosis, the levator muscle does not develop properly, so it isn't strong enough to lift the upper lid. Researchers at Boston Children's Hospital are working to identify a genetic cause of congenital ptosis by studying one family in which 150 members were born with the condition. But nobody really understands why this happens: "There is usually no known cause for most cases of congenital ptosis," Dr. Edmond says. "We think it's simply a fluke of nature." If both lids are droopy, however, the reason may be genetic or neurological in nature. Dr. Edmond does point to one unusual specific type of ptosis, called a "Marcus Gunn Jaw wink." In this version of the condition, the eyelid is only slightly drooped, but when a baby chews or sucks on a bottle, her upper lid hops up and down or "winks." "Marcus Gunn Jaw is caused by an unusual miswiring of neurons between the jaw and the eyelid," Dr. Edmond explains. "Fortunately, this is usually fairly mild and doesn't usually require surgery."

Congenital ptosis is often isolated, meaning it is the only birth defect present, but it can also be one piece of a bigger puzzle, especially if it occurs in both eyes. Your baby may need additional testing to rule out a larger issue like eye-movement abnormalities, muscular diseases, eyelid tumors, or neurological disorders.

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What Treatments Are Available?

Unfortunately, congenital ptosis is not usually something that children outgrow, though it's also unlikely to get any worse. "Most cases of ptosis are mild to moderate and don't require any treatment unless the family wants elective surgery to correct the eye's appearance," Dr. Edmond explains. "That procedure is best performed when a child is at least 3 years old, so the eyelid is a bit bigger and easier to work with."

If the droop is severe enough to block vision, your baby may need surgery urgently to enable him to develop normal vision. In most cases, a surgeon will tighten the levator muscle, but if the muscle is extremely weak, the eyelid can be suspended from just under the eyebrow so forehead muscles can do the heavy lifting. If your child's ptosis has caused amblyopia or astigmatism, an ophthalmologist will need to treat those conditions to restore normal vision. This may involve patching the stronger eye to encourage the weaker eye to work or having your child wear glasses to correct his astigmatism. Your child will also need regular eye exams to ensure that their ptosis has resolved and no other vision problems are detected.

Warning Signs to Watch Out For

In congenital ptosis, the upper eyelid creases may appear asymmetrical at birth. Over time, you'll notice a droopiness in the upper lid. Children with ptosis may also tip their head back frequently in an effort to see underneath the drooping lid, or raise their eyebrows a lot to try to lift up their lids.

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