Kahramanmaras Sutcu Imam University Medical Faculty, Department of Anesthesiology and Reanimation Kahramanmaras, Turkey.
Int J Gen Med. 2010 Nov 10;3:359-63. doi: 10.2147/IJGM.S14267.
Marfan syndrome is an autosomal dominant connective tissue disorder characterized by a combination of clinical manifestations in different organ systems. Patients with Marfan syndrome (MFS) whose lifetimes are extended may be encountered as acute abdomen (appendicitis) cases apart from the obligatory reasons and emergencies arising naturally out of their disease, as in the case reported.
In a 28-year-old Turkish male, arachnodactyly, pectus excavatum, kyphoscoliosis and, according to pulmonary roentgenogram, a density increase in the left apical field were detected. In addition, according to the echocardiographic examination, Ebstein's anomaly, mitral valve prolapse, pulmonary hypertension, and inferior deficiency of mitral, aorta, and tricuspid valves were present. The patient was planned to be operated on with the prediagnosis of acute abdomen.
Taking into consideration the pathologies that may accompany MFS and the probable future complications, the patients must be closely monitored during anesthesia applications and required measures should be taken beforehand.
马凡综合征是一种常染色体显性遗传性结缔组织疾病，其特征是不同器官系统的临床表现的组合。马凡综合征（MFS）患者的寿命延长，除了自然发生的强制性原因和急症外，还可能出现急性腹痛（阑尾炎）病例，就像本例报告中一样。
一名 28 岁的土耳其男性，出现蜘蛛指（趾）、漏斗胸、脊柱后凸和胸廓侧凸，根据肺部 X 光片，左侧肺尖区密度增加。此外，根据超声心动图检查，存在埃布斯坦畸形、二尖瓣脱垂、肺动脉高压以及二尖瓣、主动脉瓣和三尖瓣下部分缺损。该患者计划在急性腹痛的诊断下进行手术。
考虑到可能伴随马凡综合征的病理改变以及未来可能出现的并发症，在麻醉应用期间必须对患者进行密切监测，并事先采取必要措施。
