At 6 months of age, she underwent upper eyelid surgery to protect the cornea. Horizontal skin incisions were made, which allowed the eyelids to correctly appose the globes. Full-thickness grafts were then taken from the thigh to cover the resulting skin defects. Affected patients usually die within the first few days or weeks of life. Surviving patients are at risk of severe exposure keratitis due to ectropion of all 4 eyelids.
Ocular treatment consists of intensive topical eye ointment.
Any signs of keratitis should be treated promptly. During infancy, there is a spontaneous improvement of the skin with reduction in the severity of the ectropion. However, skin-release surgery with autologous skin grafting may be necessary in patients with severe exposure keratitis or cosmetically unacceptable ectropion. Management and follow-up of harlequin siblings. Br J Dermatol.