BACKGROUND: Blepharophimosis is a rare autosomal-dominant syndrome involving the periorbital region. It is characterized mainly by eyelid ptosis, palpebral phimosis, telecanthus, and epicanthus inversus. METHODS: Ten patients ranging in age from 2 to 22 years who presented with blepharophimosis were retrospectively studied over the past 14 years; they had an average follow-up of 7 years and 5 months. They were treated at the Division of Plastic Surgery of the Faculty of Medicine, University of São Paulo. Eight patients presented with the complete form, including epicanthus, telecanthus, and severe palpebral ptosis, and two presented with the incomplete form. The epicanthi and telecanthi were corrected with Mustardé's technique, and the ptosis was treated by frontalis suspension with a soft, preformed silicone device called the silicone implant suspensor. RESULTS: The results were classified as good, fair, and poor, according to the postoperative position of the upper eyelid in relation to the scleral limbus, medial canthus position, and residual epicanthal fold. The results were assessed as good in seven cases and fair in three. CONCLUSIONS: The silicone eyelid suspensor was effective in improving the patients' difficult condition. It was a better alternative than the use of fascia lata, because it permitted an easier readjustment of the eyelid margin and it offered the possibility of creating a well-defined palpebral fold, which is usually absent in this group. The results were maintained with long-term follow-up. The silicone suspensor was safe and long-term results were good in both children and adults.
BACKGROUND:Blepharophimosis is a rare autosomal-dominant syndrome involving the periorbital region. It is characterized mainly by eyelid ptosis, palpebral phimosis, telecanthus, and epicanthus inversus. METHODS: Ten patients ranging in age from 2 to 22 years who presented with blepharophimosis were retrospectively studied over the past 14 years; they had an average follow-up of 7 years and 5 months. They were treated at the Division of Plastic Surgery of the Faculty of Medicine, University of São Paulo. Eight patients presented with the complete form, including epicanthus, telecanthus, and severe palpebral ptosis, and two presented with the incomplete form. The epicanthi and telecanthi were corrected with Mustardé's technique, and the ptosis was treated by frontalis suspension with a soft, preformed silicone device called the silicone implant suspensor. RESULTS: The results were classified as good, fair, and poor, according to the postoperative position of the upper eyelid in relation to the scleral limbus, medial canthus position, and residual epicanthal fold. The results were assessed as good in seven cases and fair in three. CONCLUSIONS: The silicone eyelid suspensor was effective in improving the patients' difficult condition. It was a better alternative than the use of fascia lata, because it permitted an easier readjustment of the eyelid margin and it offered the possibility of creating a well-defined palpebral fold, which is usually absent in this group. The results were maintained with long-term follow-up. The silicone suspensor was safe and long-term results were good in both children and adults.
Authors: Salil Kumar Mandal; Aparna Mandal; James Christian Fleming; Tara Goecks; Andrew Meador; Brian T Fowler Journal: J Clin Diagn Res Date: 2017-03-01