Long-Term Results of the Surgical Management of the Upper Eyelids in “Ablepharon”-Macrostomia Syndrome

Long-Term Results of the Surgical Management of the Upper Eyelids in “Ablepharon”-Macrostomia Syndrome: Purpose:

To describe the late results of the placement of skin graft over conjunctiva-Müller muscle complex in 3 patients with ablepharon-macrostomia syndrome (AMS) and to review the procedures used to manage the upper eyelids in AMS.

Methods:

The authors searched the Pubmed database for all articles that used the term “ablepharon-macrostomia syndrome” in any field. Data collection included description of eyelid changes, age at surgery, status of the cornea before surgery, type of eyelid repair, and final outcome. Two previously reported siblings were reevaluated 10 and 15 years after a single reconstructive operation. A new case from Peru is also described.

Results:

Only 15 patients with AMS have been described in 12 articles. In 60% of the cases, the lids were described as absent. The surgical modalities employed to reconstruct the upper eyelids were quite variable, including local flaps, lid sharing procedures, and even a masquerade flap. At long-term follow-up, all 3 cases who underwent upper eyelid lengthening with full thickness skin grafts placed over Müller muscle had clear corneas with a small amount of lagophthalmos.

Conclusions:

The lids in AMS are not absent and should not be managed with complex reconstructive techniques. Full thickness skin grafts placed over the inner aspect of the palpebral conjunctiva allow permanent eye protection.

The upper eyelids in ablepharon-macrostomia syndrome can be permanently lengthened with full thickness skin grafts over Müller muscle.