Related Experiment Videos
Orbitoblepharophimosis syndrome: a 16-year perspective
Darina Krastinova1, Michel A Jasinski
1Unité de Chirurgie Cranio-orbito-palpébrale, Hôpital Foch, 40 Rue Worth, BP 36, 92151 Suresnes, France. d.krastinova@hopital-foch.org
Plastic and Reconstructive Surgery
|March 7, 2003
Summary
Orbitoblepharophimosis syndrome is a rare congenital condition affecting the eye socket and eyelids. Surgical treatment involves multiple procedures, with outcomes varying by severity and tissue quality.
Area of Science:
- Ophthalmology
- Medical Genetics
- Plastic Surgery
Background:
- Orbitoblepharophimosis syndrome is a rare congenital malformation of the orbitopalpebral region.
- It is an autosomal-dominant condition characterized by palpebral and orbital phimosis, ptosis, epicanthus inversus with telecanthus, and enophthalmia.
- The syndrome presents in minor, major, and extreme forms and is linked to the 3q21-24 gene locus.
Purpose of the Study:
- To present a series of 50 patients treated for orbitoblepharophimosis syndrome.
- To review the surgical management and outcomes for this rare condition.
Main Methods:
- Surgical treatment typically involves three to four operations.
- Procedures include orbital remodeling (burring and grafting), epicanthus correction, and ptosis repair.
- Patient data from the past 16 years were analyzed.
Main Results:
- The study reviewed 50 cases of orbitoblepharophimosis syndrome.
- Surgical outcomes were observed to be variable.
- Results depended on the specific form of the syndrome and the quality of patient tissues.
Conclusions:
- Orbitoblepharophimosis syndrome requires complex surgical intervention.
- Treatment success is influenced by disease severity and tissue characteristics.
- Further research may elucidate more refined treatment strategies.