Cruz Antonio Augusto V, Quiroz Doris, Boza Tatiana, Wambier Sarah P F, Akaishi Patricia S
Department of Ophthalmology, Otorhinolaryngology, and Head and Neck Surgery, School of Medicine of Ribeirão Preto, University of Sao Paulo, Ribeirão Preto, Brazil.
Departamento de Oftalmologia, Hospital Nacional Guillermo Almenara Irigoyen, Lima, Peru.
Ophthalmic Plast Reconstr Surg. 2020 Jan/Feb;36(1):21-25. doi: 10.1097/IOP.0000000000001442.
To describe the late results of the placement of skin graft over conjunctiva-Müller muscle complex in 3 patients with ablepharon-macrostomia syndrome (AMS) and to review the procedures used to manage the upper eyelids in AMS.
The authors searched the Pubmed database for all articles that used the term "ablepharon-macrostomia syndrome" in any field. Data collection included description of eyelid changes, age at surgery, status of the cornea before surgery, type of eyelid repair, and final outcome. Two previously reported siblings were reevaluated 10 and 15 years after a single reconstructive operation. A new case from Peru is also described.
Only 15 patients with AMS have been described in 12 articles. In 60% of the cases, the lids were described as absent. The surgical modalities employed to reconstruct the upper eyelids were quite variable, including local flaps, lid sharing procedures, and even a masquerade flap. At long-term follow-up, all 3 cases who underwent upper eyelid lengthening with full thickness skin grafts placed over Müller muscle had clear corneas with a small amount of lagophthalmos.
The lids in AMS are not absent and should not be managed with complex reconstructive techniques. Full thickness skin grafts placed over the inner aspect of the palpebral conjunctiva allow permanent eye protection.The upper eyelids in ablepharon-macrostomia syndrome can be permanently lengthened with full thickness skin grafts over Müller muscle.
描述3例睑裂狭小-大口畸形综合征（AMS）患者在结膜-米勒肌复合体上植皮的远期效果，并回顾AMS上睑的处理方法。
作者在PubMed数据库中检索所有在任何字段中使用“睑裂狭小-大口畸形综合征”一词的文章。数据收集包括眼睑变化描述、手术年龄、术前角膜状况、眼睑修复类型和最终结果。对之前报道的2例同胞在单次重建手术后10年和15年进行了重新评估。还描述了1例来自秘鲁的新病例。
12篇文章中仅描述了15例AMS患者。60%的病例中，眼睑被描述为缺失。用于重建上睑的手术方式差异很大，包括局部皮瓣、眼睑共享手术，甚至还有一种伪装皮瓣。长期随访时，所有3例行上睑延长术并在米勒肌上覆盖全厚皮片的病例角膜清晰，有少量兔眼。
AMS患者的眼睑并非缺失，不应采用复杂的重建技术处理。在睑结膜内面覆盖全厚皮片可实现永久性的眼部保护。睑裂狭小-大口畸形综合征的上睑可通过在米勒肌上覆盖全厚皮片实现永久性延长。
