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Bilateral Tessier #10 Cleft: Repairing Large Colobomas
Jonathan Y Lee, MD, MPH; Katherine E Duncan, MD; S. Tonya Stefko, MD; Jesse A Goldstein, MD; Joseph E Losee, MD
University of Pittsburgh Medical Center
2017-02-14
Presenter: Jonathan Y Lee, MD, MPH
Affidavit:
Vu Nguyen
Director Name: Vu Nguyen
Author Category: Fellow Plastic Surgery
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial
Introduction
The number 10 cleft is one of the most rare craniofacial clefts. It is characterized by a congenital upper eyelid coloboma, eyebrow deformities, and ocular anomalies. Eyelid colobomas can be large, resulting in exposure keratopathy and blindness.
Case Presentation
We report a case of a 2-year-old male who presents with bilateral craniofacial clefts involving the lip, nose, palate, upper eyelids, and eyebrows. The bilateral upper eyelid colobomas were each 50% defects of the eyelid, centered in the middle third. No ocular anomalies (symblepharon, exposure keratopathy, corneal opacification) were found on ophthalmologic exam. Lip and nose repair was performed at 7 months-of-age, followed by palatoplasty at 11 months-of-age. Bilateral repair of the Tessier #10 clefts were performed at 18 months-of-age.
Results
Full-thickness excision of the clefted eyelid/brow tissue was performed bilaterally. On the right, a forehead/brow rotation flap was used to reapproximate the eyebrow. An eyelid rotation flap was also used to reapproximate the lid margin. On the left, a forehead/brow rotation flap allowed realignment of the eyebrow. A series of Z-plasties were used in the eyelid to reapproximate the lid margin and to lengthen the eyelid. Complete eyelid closure bilaterally was achieved intraoperatively, and was maintained at 6-month follow-up with no evidence of ocular pathology.
Conclusions
Large, bilateral upper eyelid colobomas require repair to prevent blindness. Although free tarsomarginal grafts and lid-sharing procedures have been described, we demonstrate that large rotation flaps designed along the cleft margin can provide a reliable reconstruction and minimize donor-site morbidity.
