Guillarducci Victor Toledo, Japolino Katarina Lanza, Seiberlich Emerson, Delgado Marina Ayres
Department of Surgery, Division of Anesthesiology, Hospital das Clínicas de Belo Horizonte, Universidade Federal de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.
Saudi J Anaesth. 2025 Oct-Dec;19(4):628-630. doi: 10.4103/sja.sja_12_25. Epub 2025 Sep 3.
Noonan syndrome (NS) is an autosomal dominant multisystem disorder characterized by congenital cardiac defects, craniofacial anomalies, and hematological abnormalities. With an incidence of 1 in 1000 to 2500 live births, NS is one of the most common syndromes associated with congenital heart disease, second only to trisomy 21. Pregnant patients with NS present unique anesthetic challenges due to the syndrome's diverse manifestations and the physiological changes of pregnancy. This report describes a 28-year-old woman with NS who underwent elective cesarean delivery at 36 weeks of gestation. Her medical history included repaired congenital heart defects, pulmonary stenosis, severe scoliosis, and asthma. Spinal anesthesia was successfully employed using a low-dose combination of local anesthetics and opioids to achieve effective sensory block while maintaining hemodynamic stability. The patient's normal coagulation profile allowed neuraxial anesthesia to be a feasible option despite potential technical difficulties associated with spinal abnormalities. This case underscores the importance of a multidisciplinary approach to pregnant patients with NS, including comprehensive preoperative evaluation and individualized anesthetic planning. Neuraxial anesthesia can be a safe and effective option when tailored to the patient's specific physiology, ensuring optimal maternal and fetal outcomes while minimizing risks.
努南综合征(NS)是一种常染色体显性多系统疾病,其特征为先天性心脏缺陷、颅面畸形和血液学异常。NS的发病率为每1000至2500例活产中有1例,是与先天性心脏病相关的最常见综合征之一,仅次于21三体综合征。患有NS的孕妇由于该综合征的多种表现以及妊娠期间的生理变化,会带来独特的麻醉挑战。本报告描述了一名28岁患有NS的女性,她在妊娠36周时接受了择期剖宫产。她的病史包括已修复的先天性心脏缺陷、肺动脉狭窄、严重脊柱侧弯和哮喘。通过使用低剂量局部麻醉药和阿片类药物的组合成功实施了脊髓麻醉,以实现有效的感觉阻滞,同时维持血流动力学稳定。尽管存在与脊柱异常相关的潜在技术困难,但患者正常的凝血状况使神经轴麻醉成为一种可行的选择。该病例强调了对患有NS的孕妇采取多学科方法的重要性,包括全面的术前评估和个性化的麻醉计划。当根据患者的特定生理状况进行调整时,神经轴麻醉可以是一种安全有效的选择,可确保最佳的母婴结局,同时将风险降至最低。