Severe congenital lagophthalmos with tarsal aplasia

Congenital elongation of the palpebral fissure associated with large eyelids was named euryblepharon after Desmarres in 1854. In some cases the length of the lower lid margin exceeded that of the upper lid margin by more than 3 mm. The lids are lax and the elongated lid margin of the lower lid is only partially in contact with the globe. In marked cases of euryblepharon there may be an additional minor vertical shortening of the upper lid. However, the abnormalities described by Desmarres did not produce severe kerato-conjunctival xerosis. We speculate that euryblepharon as originally described is merely a mild manifestation of congenital lagophthalmos. More severe forms show additional malformations, leading to pronounced insufficiency of lid closure. We report on four patients with severe congenital lagophthalmos. The appearance differs from that of euryblepharon by the following details: (a) a symmetrical congenital retraction of both the lower and the upper lid margins; (b) a marked vertical shortening of the lids (case 1 was referred for bilateral descemetoceles); (c) tarsal aplasia; and (d) severe kerato-conjunctival xerosis after insufficient surgical treatment, leading to different degrees of disability. The surgical approach involved bilateral shortening of the lid margins and free transplantation of retroauricular skin. Stabilization of the lax lower lid margin was achieved by lyodura strips applied with medium tension and in contact with the lid margin. At 5 years after the first operation a petty horizontal shortening of the lower lids was necessary because of circumscribed trichiasis. The dura strip remained present. The long-term result as evaluated at more than 8 years following the first operation was very satisfactory.