Anesthesia for Uncommon Pediatric Diseases

Several uncommon pediatric diseases encountered by non-pediatric anesthesiologists bear discussion because a failure to recognize unique issues relating to them could lead to significant morbidity and mortality. Anterior mediastinal masses share the same space as major pulmonary and cardiovascular structures, which, if compressed, can compromise the airway, heart and great vessels. Awareness of the potential for major physiologic derangement and a multidisciplinary approach to their diagnosis and management will ensure a safe perioperative course. Congenital pulmonary airway malformation is one of the most frequent pulmonary malformations in children that requires surgical intervention by thoracoscopic approach in the first year of life. Their anesthetic management is often challenging due to their small size, need for lung isolation and maintaining adequate oxygenation during surgery. Understanding lung isolation technique in infants is necessary for the safe administration of anesthetics in these children. Arthrogryposis multiplex congenita syndrome is a rare, non-progressive, and congenital heterogeneous group of disorders characterized by congenital joint contractures. These children require frequent surgeries during childhood to address various musculoskeletal abnormalities. Their anesthetic management is often challenging due to comorbidities related to musculoskeletal deformities, neurologic, cardiovascular and respiratory systems involvement. Understanding the disease with thorough evaluation and preparation prior to anesthetic management is essential for a good perioperative outcome. Pheochromocytoma is a rare neuroendocrine tumor in children that may present unexpectedly for the management by anesthesiologists. It secretes catecholamines which can cause life threatening perioperative hemodynamic instability. The meticulous preoperative pharmacotherapy, intraoperative anesthetic management and postoperative monitoring are vital for a safe outcome.