Merriam J C, Stalnecker M C, Merriam G R
Edward S. Harkness Eye Institute, Columbia-Presbyterian Medical Center, New York, New York.
Trans Am Ophthalmol Soc. 1988;86:55-93.
The initial stages in the rehabilitation of a male child with severe microblepharon, corneal opacities, bilateral facial clefts, bilateral complete cleft lip and palate, and unilateral syndactyly are described. Review of the literature suggests that severe microblepharon is associated with other craniofacial anomalies, and often the child is stillborn or retarded. Surviving children have been abandoned because of their appearance. The child described in this case appears to be unique because his intelligence is normal, and, to our knowledge, this is the first reported case of penetrating keratoplasty after reconstruction of functional eyelids. The principal problems after corneal grafting appear to have been chronic partial exposure due to inadequate lid length and a poor Bell's reflex and the persistence of a rim of vascularized fibrous tissue around the corneal graft. Future reconstructive surgery is outlined.
描述了一名患有严重睑裂狭小、角膜混浊、双侧面部裂隙、双侧完全性唇腭裂和单侧并指畸形的男童的康复初期阶段。文献回顾表明,严重睑裂狭小与其他颅面畸形有关,患儿常为死产或发育迟缓。存活的患儿因其外貌而被遗弃。本病例中描述的患儿似乎很独特,因为他智力正常,据我们所知,这是功能性眼睑重建后穿透性角膜移植的首例报道病例。角膜移植后的主要问题似乎是由于睑裂长度不足和贝尔反射不良导致的慢性部分暴露,以及角膜移植周围血管化纤维组织边缘的持续存在。概述了未来的重建手术。