ANESTHETIC MANAGEMENT OF A CESAREAN SECTION IN A PATIENT WITH NOONAN SYNDROME: A MULTIDISCIPLINARY APPROACH

Background: Noonan syndrome (NS) is a genetic disorder associated with congenital heart defects, coagulation disorders, and skeletal abnormalities, posing significant challenges in anesthetic management, especially during pregnancy.

Case Presentation: We report the case of a 29-year-old primigravida with NS who was admitted for severe intrauterine growth restriction at 30 weeks of gestation. The patient had hypertrophic cardiomyopathy with previous surgical correction, requiring chronic anticoagulation. A scheduled cesarean section was planned due to fetal and maternal risks, with careful perioperative management including anticoagulation bridging, invasive monitoring, and advanced anesthetic techniques. General anesthesia was chosen due to severe scoliosis, anticoagulation status, and the risk of hemodynamic instability. The procedure was uneventful, and both the mother and neonate were stabilized postoperatively in intensive care units.

Discussion: This case highlights the anesthetic challenges in pregnant patients with NS, emphasizing the need for a multidisciplinary approach. The anesthetic strategy was tailored to the patients cardiac condition, respiratory function, and coagulation status, aligning with existing literature on NS-related obstetric and anesthetic management.

Conclusion: Multidisciplinary coordination, thorough preoperative assessment, and advanced perioperative monitoring are crucial in ensuring optimal maternal and fetal outcomes in NS patients undergoing cesarean delivery.