Diagnosis of absent right superior vena cava with intraoperative transesophageal echocardiography in a child with Holt-Oram syndrome: Anesthetic and perfusion implications.

Holt-Oram syndrome is a rare autosomal disorder with cardiac, vascular, and upper limb anomalies. Previous reports have described anesthetic and perioperative challenges including difficulty in arterial and venous cannulations, airway management and rhythm, and temperature abnormalities. There are no previous reports of absent right superior vena cava (SVC) in children with Holt-Oram syndrome. We present images of a case where the diagnosis of absent right SVC with persistent left SVC was made with intraoperative transesophageal echocardiography and discuss the anesthetic and perfusion implications of such findings.

Peripheral Cardiopulmonary Bypass in Two Patients With Symptomatic Tracheal Masses: Perioperative Challenges.

Tracheal tumors or masses causing critical airway obstruction require resection for symptom relief. However, the location and extent of these tumors or masses often preclude conventional general anesthesia and tracheal intubation. Peripheral cardiopulmonary bypass often is required before anesthetizing these patients. Herein, two cases of patients with tracheal masses, in whom awake peripheral cardiopulmonary bypass was instituted, are reported. The first case was that of an obese male child weighing 102 kg, with tracheal rhinoscleroma, who developed Harlequin, or north-south, syndrome after institution of femorofemoral venoarterial partial cardiopulmonary bypass. The second case was that of a female patient with adenoid cystic carcinoma of the trachea causing near-total central airway occlusion. She had severe pulmonary artery hypertension, which prevented the use of venovenous bypass. Instead, femoral vein-axillary artery venoarterial bypass was established to avoid Harlequin syndrome. Some of the challenges encountered were the development of Harlequin syndrome with risk of myocardial and cerebral ischemia, type and conduct of extracorporeal bypass, choice of monitoring sites, and provision of regional anesthesia for peripheral extracorporeal cannulations. Management of such patients needs frequent troubleshooting and multidisciplinary coordination for a successful surgical outcome.