Congenital ptosis is a condition resulting in droopy eyelid(s) at birth, and can occur in one eye or both. The most common type of ptosis is associated with the levator muscle, the main muscle that raises the eyelid. The secondary muscle that raises the lid is the Müeller’s muscle. It is thought that ptosis is caused by a local developmental muscle problem of unknown cause that is present at birth. Some patients may show improvement during the first year or two of life, but generally, the disorder remains throughout life if it is not repaired. Most cases are sporadic; however, we have also seen it occur in families.
Congenital ptosis can also occur as part of syndromes such as Marcus-Gunn jaw winking syndrome and congenital third nerve palsy, or can occur secondary to trauma at birth. Marcus-Gunn jaw winking syndrome is a rare syndrome caused by an abnormal crossing of the nerve fibers from the levator muscle to the third cranial nerve. This results in ptosis and eyelid movement (winking) that moves with mouth movement, crying, eating, and sucking.
Children with congenital ptosis should be referred for an eye evaluation. Since ptosis can cause amblyopia (lazy eye) by either blocking the visual axis or inducing astigmatism, the vision of the eye and the refractive error have to be determined. If the vision is affected, then appropriate amblyopia therapy must be started. This can consist of patching the good eye and/or prescribing glasses. If the eyelid is so droopy that the lid interferes with vision, then early ptosis surgery is indicated. If the vision is not affected, then the child needs to be monitored closely until the ptosis is repaired, generally until the child is 3 to 5 years of age. Amblyopia may still develop as the patient grows if the ptosis is still present. Ptosis can also cause abnormal head positions, such as raising the chin up to see below the eyelid and indicates that the ptosis is significant.
There are several different operations that can repair congenital ptosis. The most important factor in determining which operation is required is how well the levator muscle functions. For a patient with excellent levator function and less than 3 mm of ptosis, surgery to the levator muscle or Müeller's muscle can correct the ptosis. For a patient with moderate or excellent levator function and ptosis greater than 3mm, levator muscle resection is indicated. In a patient with poor levator function, frontalis muscle suspension surgery is indicated. This is an operation whereby certain materials are used to suspend the eyelid to the muscle above the eyebrows.
