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August L. Stein, Avery H. Weiss, John P. Kelly; Incidence of Deprivation Amblyopia and Systemic Associations in Congenital Ptosis. Invest. Ophthalmol. Vis. Sci. 2012;53(14):6744.
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Recent ophthalmic literature suggests that the incidence of deprivation amblyopia in patients with congenital ptosis is low (4-7%) relative to the overall incidence of amblyopia in this population (20-30%). It has been postulated that the low incidence is secondary to adoption of a chin-up posture and use of the frontalis muscle by affected children. Most studies, however, assessed amblyopia retrospectively without the use of preverbal visual acuity data. The current study aims to quantify the incidence of sensory deprivation amblyopia in congenital ptosis through longitudinal visual acuity data. Additionally, associations between congenital ptosis and systemic disorders were examined.
A chart review of patients diagnosed with congenital ptosis by a single pediatric ophthalmologist from 1993-2011 was performed. Visual acuity data was available on all patients through either Teller Acuity, Allen pictures, or Snellen acuity. These were all converted to logMAR for comparison. Amblyopia was defined as a > 0.3 logMAR difference between eyes or from age corrected normals on two consecutive visits at least 3 months apart. Anisometropia was defined as >1D of myopia, >3D of hyperopia, or >1.5D of astigmatism. Sensory deprivation amblyopia was determined to be present when neither anisometropia nor strabismus could account for the presence of amblyopia (or amblyopia was present prior to 15 months of age). Data collected at each visit included cyclopleigic refraction, lid position and function measurements, alignment, and compensatory mechanisms.
111 eyes of 84 patients were analyzed, with 27 of the patients having bilateral congenital ptosis. The average age of presentation was 13.95 months, with an average follow-up of 53.8 months. The overall incidence of amblyopia was 23%, with an incidence of deprivation amblyopia of 7.14%. The incidence of amblyopia secondary to strabismus and anisometropia was 9.52% and 5.95%, respectively. All but 2 children with an MRD1 of <1.5mm used frontalis, chin-up posture, or both. 34.5% of patients had concurrent systemic abnormalities, with a statistically significant number of these being cases of bilateral ptosis (p=0.005).
Our data demonstrates a low rate of sensory deprivation amblyopia in patients with congenital ptosis, owing primarily to use of the frontalis muscle and/or to adoption of chin-up posture. This is the first study to demonstrate this using longitudinal visual acuities. Due to this, surgical correction of the ptosis can almost always wait until the growth of the fronto-nasal complex is complete. Additionally, there is a high rate of association with systemic conditions, mandating a careful history and physical exam.
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