Neubauer H, Severin M, Neubauer L, Kirchhof B
Augenklinik der Universität zu Köln, Germany.
Ger J Ophthalmol. 1996 Jan;5(1):60-5.
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.
1854年，德马尔雷斯（Desmarres）将伴有大眼睑的先天性睑裂延长命名为阔睑裂。在某些情况下，下睑缘长度超过上睑缘长度3毫米以上。眼睑松弛，下睑延长的睑缘仅部分与眼球接触。在明显的阔睑裂病例中，上睑可能还会有轻微的垂直缩短。然而，德马尔雷斯描述的这些异常情况并未导致严重的角膜结膜干燥症。我们推测，最初描述的阔睑裂仅仅是先天性眼睑闭合不全的一种轻度表现。更严重的形式还伴有其他畸形，导致明显的眼睑闭合不全。我们报告了4例严重先天性眼睑闭合不全患者。其外观与阔睑裂的不同之处在于以下细节：（a）上下睑缘均有对称性先天性退缩；（b）眼睑明显垂直缩短（病例1因双侧角膜后弹力层膨出前来就诊）；（c）睑板发育不全；（d）手术治疗不充分后出现严重的角膜结膜干燥症，导致不同程度的功能障碍。手术方法包括双侧睑缘缩短和耳后皮肤游离移植。通过以中等张力应用并与睑缘接触的来多聚甲醛条带实现松弛的下睑缘固定。首次手术后5年，由于局限性倒睫，需要对下睑进行轻微的水平缩短。来多聚甲醛条带仍然存在。首次手术后8年以上评估的长期结果非常令人满意。
