Blepharophimosis: a recommendation for early surgery in patients with severe ptosis
Article first published online: 28 MAR 2003
Clinical & Experimental Ophthalmology
Volume 31, Issue 2, pages 138–142, April 2003
How to Cite
Beckingsale, P. S., Sullivan, T. J., Wong, V. A. and Oley, C. (2003), Blepharophimosis: a recommendation for early surgery in patients with severe ptosis. Clinical & Experimental Ophthalmology, 31: 138–142. doi: 10.1046/j.1442-9071.2003.00621.x
- Issue published online: 28 MAR 2003
- Article first published online: 28 MAR 2003
- blepharoptosis surgery;
- congenital ptosis
Purpose: To determine the optimal age for surgical correction of blepharophimosis. Associated features and their effects on incidence of amblyopia were also investigated.
Methods: The study was a retrospective case series of 28 patients with blepharophimosis, ptosis and epicanthus inversus syndrome presenting to a tertiary referral eyelid, lacrimal and orbital clinic.
Results: Amblyopia was present in 39% of patients. Patients with coexistent strabismus had a 64% incidence of amblyopia compared to 24% for those without strabismus. Hypermetropia was present in 43% of patients and 7% were myopic. Significant astigmatism was found in 40% of patients, but these factors did not increase the risk of amblyopia. Patients with severe ptosis had lower rates of amblyopia than those with moderate ptosis but had their ptosis corrected at a median age of 2 years compared to 5 years for those with moderate ptosis. There was an 18% incidence of nasolacrimal drainage problems. A good to excellent cosmetic outcome was achieved in 86% of patients. A positive family history was noted in 75% of patients, usually with paternal inheritance.
Conclusions: Patients with blepharophimosis have a high rate of amblyopia. Co-existent strabismus doubles the risk of amblyopia. Ptosis alone causes mild to moderate amblyopia only. Patients with severe ptosis should have their ptosis corrected before 3 years of age, and all other patients should undergo surgery before 5 years of age.