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Updated: Jul 20, 2026

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Modified Reverse Hatchet Flap for Ablepharon-Macrostomia Syndrome.

Yash J Vaishnav1,2, Louise A Mawn1,2

  • 1Department of Ophthalmology and Visual Sciences, Vanderbilt University School of Medicine.

Ophthalmic Plastic and Reconstructive Surgery
|July 5, 2024
PubMed
Summary

Ablepharon-macrostomia syndrome, a rare disorder, requires prompt intervention. A novel multiplanar eyelid reconstruction technique using modified flaps and skin grafts offers a promising solution for vision preservation.

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Area of Science:

  • Ophthalmology
  • Medical Genetics
  • Plastic Surgery

Background:

  • Ablepharon-macrostomia syndrome is a rare genetic disorder.
  • It is characterized by TWIST2 mutations and anterior lamellar dysgenesis.
  • Early intervention is crucial to prevent severe ocular complications like exposure keratopathy and vision loss.

Purpose of the Study:

  • To present a novel surgical approach for multiplanar eyelid reconstruction in patients with ablepharon-macrostomia syndrome.
  • To address the challenges posed by anterior lamellar dysgenesis and eyelid abnormalities.

Main Methods:

  • A modified reverse hatchet flap was utilized for reconstruction of one lower eyelid.
  • Division at the eyelid margin and recession of eyelid retractors were performed.
  • Preputial skin grafting was employed for anterior lamellar restoration in the remaining three eyelids.

Main Results:

  • The described surgical technique allowed for comprehensive eyelid reconstruction.
  • This approach addressed both structural eyelid abnormalities and anterior lamellar defects.

Conclusions:

  • This novel multiplanar eyelid reconstruction technique offers a viable solution for ablepharon-macrostomia syndrome.
  • The combined use of modified flaps and skin grafting shows potential for restoring eyelid function and preserving vision.