Foreword 1: An Anesthesiologist's Perspective

In the past most infants and children with syndromes had anesthesia and surgery in children’s hospitals. These children entered the hospital a day or two before surgery. This gave the anesthesiologist time to see the patient a day or more before surgery, become familiar with the syndrome, understand the patient’s problems, and make a plan to ensure safe and efficient completion of surgery and anesthesia. Modern medical care has increased the number of patients with syndromes who survive, often with serious problems that require repeated surgery. Because of the way medicine is practiced today, these patients usually arrive in the hospital (often a community general hospital) on the day of surgery and have not been seen or evaluated by an anesthesiologist up to that time. Many of these children have problems that are potentially life-threatening or associated with severe difficulties during the induction, maintenance, and awakening from anesthesia.

There are thousands of syndromes, and new ones are described every year. It is difficult to remember even a fraction of these syndromes and their features, especially if we anesthetize these patients only occasionally. Insufficient information about the patients and their syndromes often leads to cancellation of surgery, loss of income by the parents who had to take a day away from work, and significant inconvenience for the family and the hospital. Consequently, anesthesiologists (and other health professionals) need a concise, informative, and easy-to-read source of information about syndromes and the risks they pose to patients who require anesthesia and surgery or intensive care management.

Syndromes: Rapid Recognition and Perioperative Implications meets this need. Having this information not only will improve patient safety, it will also reassure physicians caring for patients affected with complex medical conditions. Because there will be fewer delays or cancellations of surgery and fewer complications, parents will be more satisfied with the care of their children.

Many syndromes are associated with serious or potentially serious problems in anesthesiology, including cardiovascular disease, inability to extend the neck, inability to open the mouth, or the potential for increased intracranial pressure. By having the facts readily available about these pre-existing, complex medical conditions, anesthesiologists, and other healthcare professionals will be able to ask the right questions of the family and of other physicians involved in the care of the patient. Parents often have significant knowledge about their child’s problems but, under the stress of the situation, will often forget to mention them if not asked specifically. If physicians do not demonstrate proper knowledge about a condition, the parents of a child undergoing surgery may not have confidence that the “team” can provide safe care for their loved one. By understanding potential problems, the anesthesiologist will be able to plan accordingly and have available the right equipment (fiberoptic laryngoscope, light wand, etc.) or specialized assistance (someone to perform a tracheostomy) that may be required. For example, knowing that some syndromes are associated with severe acidosis and cardiac arrest during surgery would allow an anesthesiologist to modify the anesthesia technique and hopefully prevent these complications.

The organization of syndromes in this book allows anesthesiologists and other health professionals to find information about a given medical condition rapidly. Each entry gives, at a glance, an overview of the syndrome and its major characteristics, genetic inheritance, pathophysiology, diagnosis, and clinical features. For the anesthesiologist, it provides the precautions before anesthesia, anesthetic considerations, and pharmacological implications. Relevant references are available. This rapid overview allows the anesthesiologist and others to have immediate access to the information needed to provide effective and safe care for these infants, children, and adults affected with pre-existing medical conditions and who are often very ill.