2023 年 116 巻 9 号 p. 907-911
Loeys-Dietz syndrome is an autosomal dominant, systemic connective tissue disorder with vascular and skeletal symptoms similar to Marfan syndrome. Clinical findings vary from case to case, but almost all patients present with some vascular symptoms. The vascular symptoms of Loeys-Dietz syndrome are more frequent and more severe than those in Marfan syndrome. In particular, macrovascular lesions tend to cause aneurysm formation and dissection at an early stage, and there are many case reports of systemic vascular tortuosity and complications of small and medium-sized aneurysms and arterial dissection.
We encountered a case of Loeys-Diez syndrome with left vocal cord paralysis after acute aortic dissection. The patient was a 31-year-old man who was taking warfarin potassium and had vascular tortuosity in the left superior thyroid artery and other arteries. For left vocal cord paralysis, left arytenoid adduction with nerve-muscle pedicle flap transfer was performed. The patient was discharged without any problems during or after the surgery. However, on the 17th day after the surgery, he came to the emergency room due to delayed postoperative bleeding and laryngeal edema. On the same day, hemostasis and tracheostomy were performed.
Postoperative bleeding is one of the complications that should be considered in voice improvement surgery. We thought that it was necessary to make a surgical plan with a thorough understanding that cases of hereditary aortic diseases such as Marfan syndrome and Loeys-Dietz syndrome have vascular tortuosity and fragility. Even when performing surgery, it is necessary to perform protective perivascular operations and be aware of the possibility of wound healing failure after surgery.