Dairi Abdulrhman Saleh, Shihata Mohammad, Bogis Abdulbadee A, Alrefai Mohammad, Aluthman Uthman, Jamjoom Ahmed
Department of Surgery, Faculty of Medicine, Umm Al-Qura University, Makkah, SAU.
Division of Cardiac Surgery, Cardiovascular Department, King Faisal Specialist Hospital and Research Center, Jeddah, SAU.
Cureus. 2021 Nov 8;13(11):e19359. doi: 10.7759/cureus.19359. eCollection 2021 Nov.
Cutis laxa syndrome is an uncommon connective tissue disorder affecting the major ultrastructure of the skin by progressive loss of elasticity. The results of this syndrome lead to the appearance of premature aging, which might also affect the internal organs. The disorder can be either congenital or acquired. The congenital form consists of autosomal dominant, autosomal recessive, and X-linked recessive patterns. The autosomal recessive pattern is the most common and severe one. Different systemic complications have been described in congenital cutis laxa syndrome, but the most serious and lethal one is cardiopulmonary abnormalities. In this report, we discuss the presentation of congenital cutis laxa syndrome with successful cardiovascular surgical management of multiple valvular heart diseases.
皮肤松弛症是一种罕见的结缔组织疾病,通过弹性的逐渐丧失影响皮肤的主要超微结构。该综合征的结果导致过早衰老的外观,这也可能影响内部器官。该疾病可以是先天性的或后天获得的。先天性形式包括常染色体显性、常染色体隐性和X连锁隐性模式。常染色体隐性模式是最常见和最严重的一种。先天性皮肤松弛症已被描述有不同的全身并发症,但最严重和致命的是心肺异常。在本报告中,我们讨论先天性皮肤松弛症的表现以及多种心脏瓣膜疾病成功的心血管外科治疗。