Blepharophimosis Syndrome: Our Experiences

1. Dr Mukesh Sharma
2. Dr Mamta Sharma
3. Dr Seema
4.Dr Neha

S.M.S.Medical College, Jaipur, India

Purpose: Blepharophimosis syndrome also known as Kohn Romano Syndrome is a congenital bilateral autosomal dominant condition characterized by "Abnormal narrowing of the palpebral fissures with drooping of lids, telecanthus and epicanthus inversus" Management of this relatively rare disease entity is essentially surgical and difficult . Timing of eyelid surgery is controversial; it involves weighing the balance of early surgery to prevent deprivation amblyopia and late surgery to allow for more reliable ptosis measurements & proper tissue development .Blepherophimsis syndrome can be managed either by single stage or two stage surgery and there are many options available ; to avoid this management dilemma and to have more predictable results we have derived a fixed surgical protocol out of all available options

Methods: Total of 28 patients were included in period between Jan 2001—Dec 2009 Amongst all available options a fixed two stage surgical protocol was derived and all patients were treated accordingly
First stage- Correction of blepharophimosis, epicanthus ,telecanthus & lateral ectropion(if present) at >4 years of age→ medial canthoplasty(hornblass's c→ u plasty)with shortening of MPL (Trans nasal wiring\non abs. sutures) +lateral canthoplasty
Second stage- Correction of Ptosis (minimum 8 wks after first stage) → Fascia lata sling( Pentagon) + lateral canthoplast y(repeat)

Results : Satisfactory surgical results could be obtained in all the patients

Conclusions: Realistic results needs to be explained to the patients before surgery and a reasonably favorable outcome can be achieved in this difficult entity by employing this 2 stage management regimen