Laranjeira João, Katerenchuk Vasyl, Duarte Joana, Lérias-Cambeiro Muriel, Lança Filipa
Anesthesiology, Unidade Local de Saúde de Santa Maria, Lisboa, PRT.
Anesthesiology, Unidade Local de Saúde da Arrábida, Setúbal, PRT.
Cureus. 2025 Feb 19;17(2):e79302. doi: 10.7759/cureus.79302. eCollection 2025 Feb.
Beals syndrome, also known as Beals-Hetch syndrome or congenital contractural arachnodactyly, is an autosomal dominantly inherited, rare connective tissue disorder characterized by flexion contractures, arachnodactyly, camptodactyly, severe kyphoscoliosis, and, less frequently, cardiovascular features. We describe the successful management of labor analgesia in a pregnant woman with Beals syndrome. During labor, a combined spinal/epidural technique was performed uneventfully, and intermittent top-ups were administered as needed at lower-than-usual volumes. Eutocic delivery occurred without complications, and both mother and baby were discharged three days later. Given the sparse literature about obstetric patients with Beals syndrome, we summarize the main anesthetic challenges and suggested approaches.
比尔斯综合征,也称为比尔斯 - 赫奇综合征或先天性挛缩性蜘蛛指症,是一种常染色体显性遗传的罕见结缔组织疾病,其特征为屈曲挛缩、蜘蛛指、手指屈曲畸形、严重脊柱后凸侧弯,以及较少见的心血管特征。我们描述了一名患有比尔斯综合征的孕妇分娩镇痛的成功管理。分娩期间,顺利实施了腰麻 - 硬膜外联合技术,并根据需要以低于常规的剂量进行间歇性追加给药。分娩顺利,无并发症发生,母婴于三天后出院。鉴于关于患有比尔斯综合征的产科患者的文献稀少,我们总结了主要的麻醉挑战及建议的处理方法。