Congenital ptosis
 

Poor upper lid function,there is  frontalis overaction Poor upgaze due to superior rectus weakness Poor left skin crease

The left eye has poorly defined skin crease. There may be elevation of the chin to gain good binocular vision.  The forehead may be wrinkled due to frontalis overaction. The upgaze is restricted (due to superior rectus  muscle weakness and is the result of close embryologic development between it and the levator).The lid muscle may fail to relax on downgaze (not obvious in this patient; see figures below) and as result the ptotic eye is higher than the normal eye on down gaze. Other examination: look at the lid(s) for previous surgery and also the forehead for any scars from brow suspension slings if the patient has glasses, check for anisometropia which is commonly seen in such patient.

Questions:

1. What are the features of congenital ptosis?

answer
Main features are:
usually unilateral
present from birth
poor levator function (due to dystrophy of the levator muscle)
failure of levator to relax on down gaze
poor superior rectus function in some due to maldevelopment in the levator muscle/superior rectus complex
fatty infiltration of the levator muscle commonly seen at surgery
 

2. How would you manage a patient with congenital ptosis?

answer
Exclude pseudoptosis such as hypotropia, microphthalmos or enophthalmos
Look for other causes of ptosis such as congenital third nerve or Horner's syndrome
Refract the patients for anisometropia which need treatment to avoid amblyopia
Unless the patient has complete ptosis or the visual axis is obscured, surgery can be performed when the patient is about 2 to 3 years old. The operation of choice depends on the levator function:
Farsanella-Servat procedure for patient with 8 mm or more of levator function
levator resection for those with more than 4 mm of levator function
frontalis suspension for those with 3 mm or less of levator function

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This 13 year-old boy underwent a  left anterior resection (note the scar) for  congenital  ptosis. Note the failure of the left upper lid to relax on down-gaze.  This is a commonfeature in patient with dystrophic levator and often become  exaggerated  following anterior levator resection.