Management of eyelid anomalies associated with Blepharo-cheilo-dontic syndrome.

Abstract

To describe the previously unreported management of the eyelid anomalies associated with blepharocheilo-dontic syndrome. Blepharo-cheilo-dontic syndrome is a syndrome of congenital cleft lip/palate, oligodontia, euryblepharon, eyelid ectropion, and lagophthalmos. Case report. A 3-month-old male presented with bilateral upper and lower eyelid ectropion with eyelid retraction, marked euryblepharon, severe lagophthalmos, and a right side cleft lip/palate. Lateral tarsal strips, lower eyelid retractor disinsertion, myocutaneous advancement of the cheek and eyelids, and lateral tarsorrhaphy were performed. Correction of the ectropion, eyelid retraction, euryblepharon, and marked improvement in his lagophthalmos were achieved postoperatively. No recurrence of the ectropion or euryblepharon has been noted after 6 months of follow-up. Appropriate reconstructive surgery of the eyelids reduces the morbidity associated with the eyelid anomalies and provides an excellent cosmetic result for patients with blepharo-cheilo-dontic syndrome.