Anesthesia for Genetic, Metabolic, and Dysmorphic Syndromes of Childhood

Anesthesia for Genetic, Metabolic, and Dysmorphic Syndromes of ChildhoodBaum VC, O’Flaherty JE. Philadelphia: Lippincott Williams & Wilkins, 1999. ISBN 0-7817-1552-0. 352 pp. $99.00. As dually trained anesthesiologists and pediatricians, Drs. Baum and O’Flaherty expertly discuss the anesthetic implications of 504 different syndromes in Anesthesia for Genetic, Metabolic, and Dysmorphic Syndromes of Childhood. This encyclopedic guide to unusual syndromes emanates from the authors’ firm conviction that, “Once the pathologic anatomy and physiology are understood, an appropriate anesthetic plan naturally follows.” This reviewer wholeheartedly agrees with the underlying philosophy of this book. The text presents syndromes alphabetically with brief entries organized into short discussions of underlying etiology (including genetics and abnormal gene product, when known); systemic abnormalities (with an enumeration of all possible anatomic findings and physiologic differences); miscellaneous information (which includes frequently fascinating historical facts); anesthetic considerations; and a brief bibliography. For ease of access, syndromes appear by the name felt to be most widely known by anesthesiologists. However, with cross-referencing, readers may locate the syndrome using alternate names as well. Seventy-eight figures, tables, and photographs supplement the text discussion. An introductory glossary provides succinct definitions of many medical terms commonly used to describe children with uncommon anatomy; for example, few anesthesiologists are likely to recall that “camptomelic” pertains to the permanent bending or bowing of a limb. Finally, six excellent appendices appear at the end of the book to remind readers of relevant biochemical pathways. Drs. Baum and O’Flaherty deserve great credit for this impressive textbook for they present in a comprehensive yet succinct fashion the characteristics of a multitude of childhood syndromes. This pediatric anesthesiologist found the coverage of syndromes to be quite extensive. Many syndromes discussed in this book do not appear in standard pediatric anesthesia texts or the standard reference Anesthesia and Uncommon Pediatric Diseases by Katz and Steward. For example, where else can one rapidly access information on the anesthetic management of a patient with propionic acidemia? The half-page entry on this syndrome includes a cogent summary of the underlying mitochondrial enzymatic defect with the relevant biochemical pathway diagrammed in the appendix. The entry enumerates the abnormal clinical findings in a systematic manner and then reminds the anesthesiologist to minimize protein catabolism by ensuring adequate perioperative glucose; to avoid acute decompensation into ketoacidosis by guarding against aspirated blood; and to anticipate perioperative respiratory insufficiency. The miscellaneous section discloses the interesting fact that propionic acid helps to give Swiss cheese its taste. Not only does this entry provide critical information to guide the successful anesthetic care of a challenging patient, one miscellaneous information helps fuel interesting discussions with one’s colleagues! For most syndromes presented, the text may serve as a stand-alone reference for health care professionals providing sedation, pain alleviation, or anesthesia for these special patients. The authors facilitate the search for further information by including the listing number of each syndrome in McKusick’s 2600-page text, Mendelian Inheritance in Man (accessible via web http://www.ncbi.nlm.nih.gov/Omim) and by selecting excellent recent references that appear at the end of each entry. Minor criticisms of this book concern the somewhat sparse distribution of the black and white photographs and diagrams. One hopes that a future edition will include more photographs of patients with these syndromes. Might the authors enlist the assistance of their pediatric anesthesia and surgical colleagues to augment their current collection of photographs? Additionally, can one persuade Lippincott, Williams & Wilkins to add color to the next edition? Color would enhance the teaching value of the photographs and make the book more appealing to browse. An additional minor complaint concerns syndromes that are not covered. This book lacks an entry on malignant hyperthermia even though the text aims to discuss anesthetically relevant genetic and metabolic syndromes of childhood. To the authors’ credit, they ask their readers to write with suggestions for additional subjects that should be included in future editions. This reviewer believes the text will be a valuable addition to the reference libraries of preadmission clinics, departmental anesthesia libraries, and operating room libraries in both general as well as pediatric hospitals. Those interested in diseases of childhood may also want to purchase this reasonably priced book for inclusion in their personal libraries. Anesthesiologists of all levels, from trainee to practitioner, will benefit from perusing this text both in a focused fashion when confronted with a patient with an unusual syndrome or in a more leisurely fashion when desiring interesting browsing material. While this reviewer prizes the first edition, she eagerly awaits future versions. Perhaps the next version might be made available via the Internet with hypertext links to McKusicks’Mendelian Inheritance in Man and other bibliographic references.