Navigating the Anesthetic Challenges of Vertebral Defects, Anorectal Anomalies, Cardiac Anomalies, Tracheoesophageal Fistula (TEF)/Esophageal Atresia, Renal Anomalies, and Limb Abnormalities (VACTERL) Association: A Delicate Balancing Act.

ABSTRACT

Vertebral defects, anorectal anomalies, cardiac anomalies, tracheoesophageal fistula (TEF)/esophageal atresia, renal anomalies, and limb abnormalities (VACTERL) association is a rare congenital disorder presenting with a constellation of birth defects. The diagnosis is primarily clinical, and patients exhibit at least three of these anomalies. These patients' management involves a multidisciplinary approach tailored to the individual's condition. Anesthetic management is particularly challenging due to the diverse and complex anomalies. This article discusses the anesthetic management of a term newborn male (39 weeks, six days gestation, 3340 g) diagnosed with VACTERL association. The newborn was admitted to the neonatal intensive care unit (NICU) and scheduled for the surgical repair of TEF and derivative colostomy on the second day of life. To mitigate the risk of air leak and abdominal distension from positive pressure ventilation, a derivative colostomy was performed first under regional anesthesia preserving spontaneous ventilation. To achieve that, the patient was sedated with ketamine and dexmedetomidine, and an ultrasound-guided single-shot caudal block with ropivacaine was performed. Post-abdominal decompression, general anesthesia was induced, and intubation was managed via videolaryngoscopy. Thoracoscopic TEF repair required several pauses for ventilation and hemodynamic optimization. Dopamine was administered intraoperatively for blood pressure support. The newborn was extubated and started on enteral feeding by the seventh postoperative day, progressing well by the time of discharge. In this case, a derivative colostomy before TEF repair avoided positive pressure ventilation complications. Ultrasound-guided caudal block provided effective regional anesthesia with high success rates. Ketamine and dexmedetomidine offered balanced sedation with minimal respiratory compromise. Dopamine was used effectively to maintain adequate perfusion, monitored with invasive blood pressure and cerebral oximetry. Anesthetic management of newborns with VACTERL association undergoing simultaneous repair of TEF and anal atresia demands meticulous and tailored planning to address the specific needs and minimize associated risks. This case highlights the importance of comprehensive anesthetic management and its impact on the patient's outcome.