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Corneal Complications due to Absence of Meibomian Glands and Lacrimal Insufficiency following Plastic Surgery in Congenital Upper-lid Aplasia

Moustafa B., Käsmann-Kellner B., Hille K., Ruprecht K. W.,
Universitätskliniken des Saarlandes, Augenklinik und Poliklinik (Homburg/Saar)

Background: Upper eye lid colobomata are rare congenital malformations. They result from incomplete fusion of the lids in approx. the 8th week of pregnancy. In the very rare case of bilateral complete upper aplasia one of the main problems is the absence of Meibomian glands and the resulting tear film deficiency.
Patient, treatment and development: In 1996 an otherwise healthy mature girl was born after an uneventful pregnancy. She presented bilateral congenital upper lid aplasia, no other malformations were noted. She was treated with excessive ointment up to the third day of life, when a plastic advancement flap (Tenzel) was performed. To try to shape a fornical fold an Illig shell was placed and a temporary lid closure was performed bilaterally. The plastic flap rotated from the temporal skin and the forming of Burrow triangles lead to a sufficient anatomical lid reconstruction. Following the re-opening of the temporary lid closure on the 10th postoperative day lid closure was only achieved when the child cried. Lid closure ability improved over the following weeks and 8 weeks postoperatively the lids were closed when the child slept. Over the following years recurrent corneal erosiones occurred and corneal vascularisation and clouding increased in spite of the very consequent treatment the parents performed. Therefore and in the hope to improve visual impairment lamellar keratoplasty was performed. Unfortunately these had bad outcomes due to the insufficient tear film due to the absence of the Meibomian glands. Vascularisation and corneal clouding slowly increase over time.
Conclusions: In cases of upper lid aplasia immediate plastic surgical intervention is necessary to protect the cornea. However, in spite of good anatomical reconstruction of lid structures the lids will not be able to take up the physiological functions of natural lids, as the Meibomian glands in plastic flaps are missing and the tear film in insufficient. Patients will develop corneal haze and extended corneal vascularisations which are extremely difficult to treat.