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Obstetric and Anesthetic Management of a Parturient with VACTERL Syndrome
Abstract Number: F 64
Abstract Type: Case Report/Case Series
This case report describes the obstetric and anesthetic management of a cesarean delivery in a woman with VACTERL association. VACTERL is an acronym for Vertebral defects, Anal atresia, Cardiac anomalies, Tracheo-Esophageal fistula, Renal and Limb defects. There is no consensus on the factors that contribute to the phenotypic manifestations. One published letter describes the anesthetic management of a parturient with VACTERL association. (Luce V et al. Anesth Analg 2004; 98: 874). Our patient presented early and was followed by a perinatologist, cardiologist, and pulmonologist. Her features included surgically corrected Tetralogy of Fallot and tracheo-esophageal fistula; severe scoliosis requiring Harrington rods (see X-ray); and congenital absence of a left thumb. She had no anal atresia or renal dysfunction. She had significant restrictive lung disease from persistent thoracic scoliosis and a short thorax.
Functionally, she remained NYHA 1 despite an admission to hospital at 30 weeks’ gestation for pneumonia that responded well to intravenous antibiotic therapy. Despite reduced lung volumes on pulmonary function testing she was mostly asymptomatic from the pulmonary standpoint. During pregnancy, echocardiography revealed persistent right ventricular hypertrophy and reduced systolic function, with moderate to severe pulmonic insufficiency, but no evidence of pulmonary hypertension. She also had evidence of mild tricuspid regurgitation with mild right atrial enlargement, but left ventricular systolic function was normal.
In view of a contracted pelvis it was decided to proceed with a primary cesarean delivery at 36 weeks’ gestation. The cardio-respiratory anomalies and a Mallampati IV airway, dictated a combined spinal epidural anesthetic after placing a pulmonary artery catheter and arterial line under sedation. Despite CSF flow through the spinal needle after difficult epidural needle placement with ultrasound guidance, the spinal anesthetic did not work. We then used general anesthesia, with a Glidescope® to facilitate endotracheal intubation. This was performed uneventfully and the cesarean delivery proceeded without complication. The patient was managed in an intensive care unit postoperatively for short-term invasive hemodynamic monitoring and made a good recovery. Further details of her obstetric issues and anesthetic care will be presented in the poster.