Anesthetic management of a parturient with VACTERL association undergoing Cesarean delivery

We present the anesthetic management of a parturient with VACTERL association undergoing combined regional and general anesthesia for Cesarean delivery. Defined as a syndrome, VACTERL association comprises at least three of the following abnormalities: vertebral, anal atresia, cardiac, tracheoesophageal, renal, and limb.

The patient's anatomic abnormalities and comorbidities comprised severe cervicothoracic scoliosis, kyphoscoliosis, congenitally fused ribs, and severe restrictive lung disease. She had a Mallampati class 3 airway, a right laterally flexed neck, and reduced mandibular protrusion. We performed a lumbar spine ultrasound for epidural placement which was used to provide peri- and postoperative analgesia. Due to the anticipated difficult tracheal intubation, the patient underwent an awake fibreoptic intubation and subsequently received general anesthesia. The patient's trachea was extubated on the first postoperative day, and she received adequate post-Cesarean epidural analgesia.

This case highlights the challenges that anesthesiologists face when managing parturients at extremely high risk for perioperative anesthetic morbidity due to the presence of severe pre-existing disease, anticipated difficult airway, and major spinal abnormalities complicating neuraxial anesthesia. We used a combined general and epidural anesthetic approach to control ventilation, provide effective postoperative analgesia, and reduce the risk of anesthetic-related perioperative morbidity. An individualized approach should be considered for the anesthetic management of high-risk pregnant patients with complex and multiple medical and surgical morbidities undergoing labour and delivery.