Congenital myogenic dysgenetic ptosis

Congenital myogenic ptosis is a sporadic or more rarely inherited dysgenesis of the levator muscle resulting in reduced levator function and ptosis, either unilateral or bilateral.

The levator function varies from good to poor and similarly the ptosis ranges from mild to severe. When levator function is poor there is also lack of a skin crease and lag of the lid on downgaze since the muscle both contracts and relaxes inadequately. Lagophthalmos may also be present.

The initial priority is to assess the child's visual development and to treat any co-existant strabismus, ametropia and amblyopia. When severe unilateral ptosis covers the visual axis there is a risk of occlusion amblyopia and ptosis repair is required urgently. Similarly, bilateral ptosis with a marked chin-up head posture may require early intervention. If visual development is normal then most surgeons would delay surgery to about 4 years of age when more accurate pre-operative measurements are possible, and yet is prior to school age. The choice between levator resection and brow suspension depends on the levator function.

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