Anesthetic Management of a Neonate With Trisomy 18 Undergoing Repair of Ruptured Myelomeningocele in a Resource-Limited Setting

Anesthesia in Trisomy 18 Neonate

Abstract

Abstract

Background: Trisomy 18 (Edwards syndrome) is a rare chromosomal disorder characterized by multiple congenital anomalies, including craniofacial dysmorphism, congenital heart disease, and neurological malformations. These abnormalities create substantial perioperative challenges, particularly in neonatal anesthesia. We present the anesthetic management of a neonate with Trisomy 18 undergoing repair of a large ruptured myelomeningocele (MMC) in a resource-limited setting.

Case Presentation:  An 8-day-old female neonate weighing 3.3 kg with phenotypic features consistent with Trisomy 18 underwent surgical repair of a large ruptured MMC. Preoperative evaluation revealed micrognathia, short neck, low-set ears, high-arched palate, clenched fists, and rocker-bottom feet. The patient had previously been treated for neonatal sepsis and septic shock, but achieved hemodynamic stabilization without vasopressor support before surgery. Because preoperative echocardiography could not be performed due to resource limitations, perioperative management was conducted with consideration of possible congenital cardiac disease and limited cardiac reserve. Anesthetic induction was performed with ketamine and propofol, and endotracheal intubation was successfully achieved using video laryngoscopy with a 3-mm micro-cuffed endotracheal tube. Intraoperative hypotension developed during isoflurane administration and was managed by discontinuation of isoflurane and initiation of ketamine infusion. Despite active warming measures, intraoperative hypothermia (32–34°C) developed during the prolonged surgical procedure. Following fulfillment of extubation criteria, the patient was extubated in the operating room. A brief episode of apnea occurred after extubation but resolved with tactile stimulation. The patient was subsequently transferred to the Neonatal Intensive Care Unit in stable condition without the need for postoperative mechanical ventilatory support.

Conclusion: Anesthetic management of neonates with Trisomy 18 is particularly challenging because of multisystem anomalies, possible cardiac involvement, difficult airway risk, and susceptibility to hypothermia. In resource-limited settings, meticulous perioperative planning, careful hemodynamic management, active temperature control, and individualized anesthetic strategies are essential for achieving favorable perioperative outcomes.