Introduction

Congenital ptosis is caused by maldevelopment of the levator muscle. The condition can be unilateral or bilateral and the severity of the ptosis is dependent on the number of viable striated levator muscle fibers. Congenital ptosis often has a family lineage, although sporadic cases are common.

The most concerning complication of congential ptosis is the development of amblyopia in the growing child. Depending on the degree of ptosis, induced anisometropia and even occlusion can profoundly affect visual maturation. In general, congenital ptosis repair should be delayed until 4–5 years of age. For severe cases in younger patients, manual taping of the ptotic eyelid can be performed to stimulate visual development until the child is old enough for surgery. Patching and optimizing any refractive errors is performed with any signs of amblyopia. The loss of a head tilt in a unilateral case should also prompt patching of the fellow eye as suppression is occurring in the ptotic eye.

In 1979, Beard described a useful grading scheme for the management of congenital ptosis ( Table 14.3 ). During the evaluation, the position of the upper eyelid (MRD1) and levator function with the brow suspended inferiorly are carefully determined. These measurements are a starting point for determining the amount of levator to resect to achieve an adequate eyelid position. Beard’s table provides a useful starting point and, with experience, one should modify these numbers with their own surgeon’s factor. Levator function less than 5 mm may warrant frontalis suspension ( Chapter 15 ), although a supermaximal levator resection may be considered first.

The expectations and complications should be discussed in depth with the parents prior to surgery. It should be explained to the parents that in addition to the functional visual improvements with ptosis correction, the cosmetic improvement in the eyelid position has also shown to improve social development. Indeed, from a functional standpoint, congenital ptosis repair is not considered a cosmetic operation but rather a reconstructive surgery.

Common complications include over- and under-correction and eyelid contour asymmetry. Lagophthalmos is seen in virtually all patients, particularly with large (≥18 mm) levator resections. Children tolerate this corneal exposure particularly well, probably as a combination of a good Bell’s reflex and a healthier tear film compared to adults. It should be explained to the parents that this will likely be seen and may improve with time.