Eva Vogeley,Jodi Innocent
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Ethical Concepts, 1191 |
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Medical Malpractice, 1192
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Intentional Torts, 1193 |
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Informed Consent, 1194 |
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Implied Consent and Decisional Capacity, 1194 |
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Emancipated Minor and the Mature Minor Doctrine, 1195 |
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Emergency Medical Treatment and Active Labor Act, 1195 |
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Risk Management, 1195 |
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Research in Children, 1196 |
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Death and Organ Donation, 1197 |
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Summary, 1197 |
The topic of the ethics and medicolegal aspects of pediatric anesthesia is very broad and could fill volumes. Although the law may differ from state to state and from country to country, bioethical issues are generally applicable to all people. This chapter of necessity emphasizes some topics while neglecting others. Because medical malpractice and risk management are emphasized in most writings on the law and anesthesia, this topic is only summarized here. The emphasis of this chapter includes the intention torts, the doctrine of informed consent, and issues related to research involving children as subjects. A glossary of terms and abbreviations is provided in Box 36-1 .
BOX 36-1
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Glossary of Terms and Abbreviations |
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Assault: Causing apprehension of battery Battery: Harmful or offensive touching Capacity: Attribute of having legal competency to act Consortium: Inability of family members of the plaintiff to have normal relations with him or her Defendant: Party sued in a legal action Duty: Legal obligation, the breach of which can lead to liability EMTALA: Emergency Medical Treatment and Active Labor Act FDA: Food and Drug Administration HIPAA: Health Insurance Portability and Accountability Act of 1996 OHRP: Office for Human Research Protections Plaintiff: Party who initiates a lawsuit by filing a complaint Res ipsa loquitur: Literally, “the thing speaks for itself”; an exception to the rule that a plaintiff must show defendant's actions caused injury Standard of care: Level of skill and learning commonly possessed by members of a profession who are in good standing Tort: Civil wrong that results in an injury to another |
▪ ETHICAL CONCEPTS
The traditional ethical principles of beneficence, respect for persons, and justice[*] must guide physicians in all aspects of their professional lives, including the treatment of patients, the education of health care personnel, and research involving human subjects. As with overarching principles in general, it is difficult at times to apply these broad rules in individual instances. This is especially true in dealing with children, who do not have the capacity to consent on their own behalf.
The term “justice” encompasses the concept of fairness of distribution. The principle is often stated as requiring that people similarly situated should be treated similarly. Although pediatric anesthesiologists share the concerns of the greater medical community about issues of access to health care by families who are uninsured or underinsured, the principle of justice becomes more intense when there is inequality of treatment of pain based on age, gender, or both. There is a substantial body of literature dealing with the physiologic and autonomic concomitants of pain that makes it clear that even the smallest of preemies experiences distress when subjected to noxious stimuli. Because pain management often falls within the purview of departments of anesthesiology, there is the opportunity to ensure the compassionate and just treatment of all.
The Hippocratic oath reminds all physicians of the ethical mandate to do no harm. The principle of beneficence goes beyond this in that it requires the making of efforts to secure the well-being of patients. This, in turn, requires maximizing benefit and minimizing risk. It is important to remember that risks to patients involve more than physical risks. Especially in the area of anesthesiology, it is essential that physicians be mindful of psychological issues implicit in being rendered entirely dependent on a stranger, as occurs during general anesthesia. Sensitive and age-appropriate discussion can minimize these potential psychological harms to children and adolescents. For example, the expression “put to sleep” may have one meaning for an anesthesiologist but quite another for a child whose pet was recently euthanized.
The principle of respect for persons requires acknowledgement of autonomy. This means that people who have decisional capacity should be given sufficient information to make informed choices. In turn, such informed choices should be honored. As a corollary, however, respect for persons also requires protection of those with diminished capacity to make decisions. By legal definition, most minors are held to lack full decisional capacity. However, it is also clear that decisional capacity does not develop at a single age, legislatively defined. To the contrary, decisional capacity develops over the lifetime of an individual with considerable variation from person to person. For practitioners of pediatric anesthesia, the principle of respect for persons requires not only that the parents of children are given sufficient information to make choices about treatment but also that information is given to children in a manner that they can understand so that their assent to treatment can be obtained. Problematic are those situations in which a parental decision conflicts with that of a minor child. For example, what happens if a parent consents for anesthesia for a debulking operation for metastatic cancer when the child wants no further therapy? What if a teenager wants a cosmetic operation but the parent is opposed and refuses to consent for the operation? One of the unique features of pediatric anesthesiology is that the person consenting to anesthesia is not the patient but rather a legally authorized representative, usually a parent who can consent on the child's behalf. If consent is not obtained or the obtained consent is in some way defective, the treating physician runs the risk of committing medical negligence or an intentional tort, both of which are discussed in more detail later.
* These principles are those that were enunciated in the Belmont Report, which continues to serve as the guiding document for research in human subjects.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ MEDICAL MALPRACTICE
The mere utterance of the word “lawsuit” strikes terror in the hearts of most physicians. Although there is no doubt that some frivolous lawsuits are won not on the merits of the case but rather on the basis of emotional issues, it should be reassuring to pediatric anesthesiologists that in most instances a plaintiff must fulfill some clearly defined elements in order to prevail. These requirements are as follows.
▪ DUTY
In the law, a duty arises when there is a relationship that requires that a person conduct himself or herself according to a certain standard. If a physician is walking across a bridge and notices a person who is threatening to jump, it may be morally reprehensible for the physician simply to continue to walk across the bridge. It is not, however, an act of negligence because there is no relationship between the physician and the potential jumper that creates a duty for the physician to act in a certain way. In contrast, when a physician agrees to perform a medical service for a patient, then that physician has a duty to conduct himself or herself according to professional standards. In general, for a duty to arise, the physician and a patient must have entered into a physician-patient relationship. In some situations, however, a duty may be inferred even though no formal physician-patient relationship has been established. For example, it is not unusual for a pediatric anesthesiologist to be called to the emergency department or onto one of the wards in a hospital when a patient, previously unknown to the anesthesiologist, develops airway difficulties or otherwise requires intubation. If an anesthesiologist has agreed to be available for such an emergency situation, then he or she has a duty to such a patient, even though a formal relationship has not been previously established.
▪ BREACH OF THE DUTY OF DUE CARE
This element of a medical malpractice suit prevents a bad outcome alone from being sufficient to prevail against the treating pediatric anesthesiologist. The patient alleging malpractice must, in addition to showing that there was a bad outcome, establish that (1) there is a standard, a level of skill and learning, commonly possessed by members of the profession in good standing and (2) that the physician's action fell short of this standard.
Historically, the standard to which a physician's conduct was held was determined locally. This approach was based on the belief that the standard for a practitioner in a small rural community understandably differed from that of a specialist in a large metropolitan medical center. With the development of medical boards and specialty boards, the concept of a local standard was supplanted by a national standard related to one's specialty, rather than to one's location. To the extent that professional societies have promulgated practice guidelines, these can be used as evidence of a national standard. In the absence of such accepted guidelines, the standard of care is less clear and must be established through expert testimony. In cases in which competent members of the profession differ on what the standard should be (subscribe to different schools of thought), the adherence to one or the other approach does not constitute negligence.
An illustration may be helpful. Pediatric anesthesiologists may differ in whether a straight or curved blade is preferable when performing an intubation. The choice of one type over another does not constitute negligence. In contrast, there is a standard for ensuring that an esophageal intubation has not occurred. If a pediatric anesthesiologist has intubated a child's esophagus and continues on without confirming endotracheal tube placement, this would constitute a breach of the duty of due care.
The absence of Food and Drug Administration (FDA) approval for a drug or device has also been used by plaintiffs to establish a standard of care. Although physicians have the legal right to prescribe any FDA-approved medication for any condition or age group, it may be problematic when such a use results in an unfortunate or unforeseen outcome. If, for example, a medical device has been approved for ablation of varicose veins and an ophthalmologist uses it to treat blood vessels in an eye, the lack of FDA approval for this use might be persuasive evidence that there was a breach of the standard of care. In the area of pediatric anesthesia, however, the lack of FDA approval for the use of an agent in children does not in and of itself constitute evidence of breech of a national standard. Until very recently, there was little incentive for pharmaceutical companies to sponsor trials of drugs to permit FDA approval for children. Often, drugs used in adults were tried in children, were found to be successful, and became widely used without ever having undergone the stringent testing required for FDA approval. For example, the drug albuterol that is widely used by pediatricians and pediatric anesthesiologists to treat bronchospasm has never received FDA approval for young children. The lack of FDA approval for a drug that is widely used and adequately studied in children is unlikely to be held to be a breach of the standard of care. However, an off-label use of a drug in children that has not been widely studied may give rise to such a finding. Fortunately, legislation has been enacted that promises extension of exclusivity rights to pharmaceutical companies that are willing to undertake pediatric trials. It is hoped that this will give rise to more FDA approval of drugs used in children.
▪ CAUSATION
In order for a plaintiff to prevail in a malpractice action against an anesthesiologist, it is not sufficient to show that the physician had a duty and breached the standard of care. The plaintiff must also show that the act or omission of the anesthesiologist was what caused harm. Two different tests are used to determine if a physician's actions were the cause of a patient's harm. The first is the “but for” test. This test asks if “but for” the actions (or inactions) of the physician, would the patient have had the same untoward outcome? On the one hand, this test makes sense. If the patient's injuries would have occurred regardless of whether the physician did or did not act in a certain way, then it does not seem reasonable to hold the physician liable. Unfortunately, the “but for” test can give rise to unintended results that offend common sense. For example, an infant with congenital heart disease may not have had operative complications “but for” the fact that anesthesiologist had been willing to cover the heart cases on that day. This does not, however, prove that the anesthesiologist's decision to cover the heart cases was responsible for the harm. To avoid results that offend basic logic, courts may also apply the “significant factor” test in order to determine whether an anesthesiologist's action was an important cause of the operative injuries. If both these tests are met, then it is likely that the plaintiff has met the burden of proving causation.
In general, plaintiffs must specifically prove the element of causation. In a relatively few instances, the plaintiff does not have this burden because of the doctrine of res ipsa loquitur, which literally means, “The thing speaks for itself.” When his or her doctrine is invoked, the plaintiff no longer has the burden of proof; instead, the physician must prove that his or her actions were not the cause of the patient's harm. In order to invoke the doctrine of res ipsa loquitur, the plaintiff must demonstrate the following:
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The injury is of a type that does not ordinarily occur without negligence. |
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The instrumentality of the injury was under the exclusive control of the defendant(s). |
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The plaintiff did not contribute to the injury |
Some courts add a fourth requirement—that the actual explanation for the injury is more accessible to the defendant than the plaintiff. In the case of pediatric anesthesiologists, this fourth requirement is easily met, because their patients are often minors who are either unconscious or have been given sedatives in combination with agents that produce amnesia.
One of the most famous cases to invoke the doctrine of res ipsa loquitur is the case of Ybarra v. Spanard [154 P.2d 687 (Cal. 1944)]. In this case, the plaintiff underwent an appendectomy. After the operation, he had pain in his shoulder due to an injury sustained during the operation. He sued the surgeon, the hospital, and the anesthesiologist, alleging that at least one of them had to have been negligent for his injury to have occurred. The court found in favor of the plaintiff on the basis of res ipsa loquitur, holding that it was unreasonable to require the plaintiff to identify the party that had been negligent as he was anesthetized, that each of the plaintiffs owed him a duty of care, and that a shoulder injury was unlikely to result from appendectomy surgery absent negligence.
▪ DAMAGES
Even if a plaintiff shows that a pediatric anesthesiologist owed him a duty and there was a breach of the standard of care, he must also show that the resultant harm resulted in damages. Most jurisdictions require an actual physical harm before damages for nonphysical harms such as emotional distress may be awarded. Damage awards consist of what the fact finder (jury or judge) believes to be the actual harm (medical bills and actual economic losses, which in the case of children are mostly future loss of wages) and compensation of less tangible harms such as pain, suffering, and loss of consortium.
Negligence is not the only cause of action that can be brought against a pediatric anesthesiologist. Suits based on intentional torts have also been successfully brought against physicians.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ INTENTIONAL TORTS
The term “tort” refers to civil as opposed to criminal wrong-doing. As discussed earlier, most cases of medical malpractice come under the tort of negligence. The intentional torts present an area of special concern for health care professionals because many malpractice policies exclude coverage for these actions. Another important consequence of the distinction between intentional torts and negligence is that in an action for negligence, the physician is generally held liable only for the consequences that were foreseeable; the physician who commits an intentional tort is liable for every result stemming from his conduct regardless of its predictability or ability to be foreseen. The following illustration shows this distinction:
D intentionally hits P on the head, intending merely to annoy him. P is slightly injured and taken to the hospital. There, by gross and completely unforeseeable error, a nurse gives him a poison instead of medication and P dies. D will be liable for P's death, not just the minor injury. But if D had merely negligently given P the same minor injury, he would not be liable for the unanticipated death (Rest.2d,§435B, Illustration 1).
The intentional torts of most interest to physicians are those of battery and assault. Battery is the intentional infliction of a harmful or offensive bodily contact. Assault is the intentional causing of an apprehension of harmful or offensive bodily contact. It is important to note that the bodily contact need not be harmful but need only be offensive or to offend one's sense of dignity. A troubling example is illustrated by the case of Mohr v. Williams [104 N.W. 12 (Minn. 1905)]. In this case, a patient consulted an otolaryngologist about her right ear and consented to an operation on that ear. During the operation, the physician discovered that it was actually the left ear that was diseased and operated on it. The court held that the surgery on the left ear was an offensive contact that constituted battery even though there was no harm to the patient. The amount of damages awarded to the plaintiff in this case was $39.00. Nearly everything that pediatric anesthesiologists do to patients would constitute a battery were it not for obtaining consent. Even if obtained, if the informed consent process is incomplete or in some other fashion defective, then the action of the physician may constitute a battery or, more likely, medical negligence.
Although the intentional torts of assault and battery are those of which physicians are accused if valid consent is not obtained, several other intentional torts merit mention.
Claims for invasion of privacy can arise from unauthorized release of information concerning patients. With the implementation of the privacy rule of the Health Insurance Portability and Accountability Act of 1996 (HIPAA), most hospitals have reevaluated and refined their privacy policies. However, the temptation to discuss interesting cases with students on an elevator can still lead to inadvertent disclosures of private health information. Additionally, the increasing use by physicians of email to communicate with their patients carries the risk of protected health information being made available to persons who should not have such access. Finally, the convenience of the cellular phone must be balanced against the potential for a conversation being overheard by others. Prudence dictates that patients are informed if a cellular phone is being used so that they can decide whether to continue the cellular conversation or to call at another time.
Intentional infliction of emotional distress is another intentional tort that includes outrageous conduct that results in emotional trauma. This is the tort that perhaps may easily be avoided if the physician remembers to treat patients and families with respect at all times. Because the health care context is one that lends itself to at times challenging one's most strongly held beliefs, it is at times possible to get caught up in the emotionality of the moment and engage in conduct that could be construed as sufficiently outrageous to constitute intentional infliction of emotional distress. For example, a family who sought to discontinue life support alleged that hospital personnel accused them in a nonprivate area of the hospital of trying to murder the patient. The appeals court held that such conduct could be considered sufficiently outrageous as to permit a finding of intentional infliction of emotional distress [Gregg v. Calandra, 297 Ill.App.3d 639 (1998)].
False imprisonment is the unlawful restriction of a person's freedom, usually through physical restraint, although threats of harm can constitute false imprisonment in certain circumstances. During outpatient surgical procedures, most hospitals encourage a parent to remain in the waiting room. If a parent told the anesthesiologist that she planned to go out to a restaurant during her son's tonsillectomy and the anesthesiologist stated that if she left he would notify Child Protective Services and have her child placed in foster care, this might be held to constitute false imprisonment.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ INFORMED CONSENT
Depending on the state in which a physician practices, the courts may distinguish between lack of consent and lack of informed consent. As described, lack of consent to touch or treat a patient can result in a claim for battery. There is no need for physical injury, but only some contact; the matter of permission goes to the quality of the contact, and consent to being so touched is a defense [Chandler v. Cook, 438 Pa. 447, 265 A.2d 794 (1970)]. In contrast, in an informed consent claim grounded in negligence, the matter of permission goes to the scope of the contract between physician and patient, and the primary inquiry is whether the injury suffered was within the known risks of which the patient was informed, or whether the information, such as to alternative procedures, was complete [Grabowski v. Quigley, 454 Pa. Super. 27, 684 A2d 610, 616 (Pa, Super. 1996)]. For various procedural and evidentiary reasons, many attorneys will plead both battery and medical negligence when asserting a claim of lack of informed consent.
A claim of lack of informed consent also may be supported by state law. Moreover, a claim of lack of informed consent may be limited by applicable state law or case law to only those procedures that are considered invasive. For example, Pennsylvania requires that informed consent be obtained, except in emergencies, prior to conducting the following procedures (40 P.S. 1301.811-A):
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Surgery, including the related administration of anesthesia |
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Administration of radiation or chemotherapy |
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Administration of a blood transfusion |
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Inserting a surgical device or appliance |
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Administration of an experimental medication, use of an experimental device, or use of an approved medication or device in an experimental manner |
Valid informed consent discussion, of necessity, requires that the person obtaining consent have detailed knowledge of the procedure. It is unlikely that a surgeon can discuss anesthesia with as much depth as an anesthesiologist. Similarly, physicians in training may lack sufficient knowledge and experience to conduct a consent interveiw.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ IMPLIED CONSENT AND DECISIONAL CAPACITY
In some instances, consent to medical treatment can be implied by a patient's behavior. In the case of O—Brien v. Cunard SS Co [28 N.E. 266 (Mass. 1891)], decided in 1891, a passenger on a ship bound for the United States was told that she could not enter unless she had a certificate to show that she had been vaccinated. The patient stood in line and held up her arm. She then sued for battery, claiming that she had not consented to the vaccination. The court said that the behavior of the patient was such that it was reasonable for the physician to conclude that she had consented. The finding of implied consent in the modern medical setting, however, would be very unusual, and in some states that by regulation mandate the obtaining of written consent for certain procedures, the doctrine of implied consent cannot be used as a defense. The better practice is to adhere to the frequently quoted maxim, “If it isn—t evidenced in a writing, it didn—t happen.”
In order for consent to be valid, the person giving consent must have the capacity to do so. In general (with some exceptions listed here), children do not have the capacity to consent for medical treatment. In addition, persons who are premedicated, unconscious, or severely intoxicated do not have the capacity to consent. Commentators on decisional impairment have pointed out that in many cases there is a continuum of decisional capacity so that persons who are mildly intoxicated or persons with some degree of mental retardation can in some circumstances give legally effective consent, whereas in other circumstances they cannot. It is the obligation of the physician obtaining consent to determine the decisional capacity, either alone or in consultation with a colleague, and to present materials in a way that can be understood by the patient in light of their innate abilities, education, and other circumstances.
When treating minors, the issue of decisional capacity also goes to the authority of the person who is acting as the representative of the minor. In some states when children are placed in foster homes, parental rights are not terminated, and therefore the consent of the parent, rather than that of the foster parent, is required for almost all medical procedures. Even routine health care may not be the province of the foster parent but rather reside with the county agency.
There are some situations in which the patient's consent will be implied “as a matter of law” if certain conditions are met, including the following:
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The patient or person from whom consent must be obtained is incapacitated because of unconsciousness or a similar condition. |
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Failure to act would result in death or other serious harm. |
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There is no reason to believe that the patient would not consent. |
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A reasonable person would consent under the circumstances (Rest.2d, § 62). |
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ EMANCIPATED MINOR AND THE MATURE MINOR DOCTRINE
The use of the term “emancipated minor” is dependent on the context in which the term is used. A minor may be emancipated for some things, such as receiving public assistance, and not considered emancipated for others, such as signing a contract or entering into a lease for an apartment. A minor may be emancipated due to certain circumstances defined by statute or as a result of being legally granted such status by a court of law. In many states, minors are considered “emancipated” for purposes of consenting to health care if they have met certain conditions. The definition of “emancipated minor” for purposes of health care consent may vary in detail from state to state, but the law in Pennsylvania is quite typical. It states
Any minor who is eighteen years of age or older, or has graduated from high school, or has married, or has been pregnant, may give effective consent to medical, dental, and health services for himself or herself, and the consent of no other person shall be necessary (Act of Feb. 13, 1970, pl 19, No. 10 §1, 35 P.S. § 10101).
It should be noted that statutes such as this, while giving minors the ability to consent to medical care, do not necessarily give such persons the right to consent to participate in research.
Some jurisdictions, mostly through case law as opposed to legislation, have adopted the “Mature Minor Doctrine” as an exception to the usual rule requiring parental consent to medical treatment for the minor. A typical example comes from the case of Cardwell v. Bechtol [724 S.W.2d 739 (Tenn. 1987)], in which an adolescent sought care for back pain. The physician did not inquire about parental consent but rendered manipulative treatment because he believed that the adolescent was old enough to consent to the treatment on her own behalf. The parents of the adolescent brought suit when complications developed. The Supreme Court of Tennessee held that the adolescent was a mature minor and that the defendant could not be held to have committed a battery when he failed to obtain parental consent prior to treatment. An interesting extension of the concept of the “mature minor” was made in the case of Larry D. Belcher, Sr., Administrator of the Estate of Larry D. Belcher, Jr., Deceased, Plaintiff Below, Appellant v. Charleston Area Medical Center, a Corporation, Charleston Pediatric Group, a West Virginia Corporation, and M.B. Ayoubi, MD, Defendant Below, Appellees [422 S.E.2d 827 (West Va. 1992)] in which the parents told the treating physician that they did not want their 17-year-old son, who had muscular dystrophy, to be intubated unless their son desired it. The son died and was not resuscitated. The executors of the son's estate brought an action for wrongful death based on the theory that, as a mature minor, the son should have been consulted prior to the issuance of a do not resuscitate (DNR) order. The court held that if the son was a mature minor, he was entitled to consent to his medical treatment decisions. The case was remanded to the lower court to try the issue of whether the son in this case was a mature minor. Under the mature minor doctrine, whether a child has the capacity to consent depends upon the age, ability, experience, education, training, and degree of maturity or judgment obtained by the child. According to the court, in situations in which there is a conflict between the intentions of one or both parents and the minor, the physician's good faith assessment of the minor's maturity level would immunize him or her from liability for the failure to obtain parental consent.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ EMERGENCY MEDICAL TREATMENT AND ACTIVE LABOR ACT
When working in a health care facility, the physician has additional obligations to act even in the absence of written informed consent. The Emergency Medical Treatment and Active Labor Act (EMTALA) was included in the Consolidated Omnibus Budget Reconciliation Act of 1986 (COBRA) in response to some hospitals—refusal to treat indigent patients. EMTALA imposes the following requirements on all hospitals that participate in Medicare:
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An appropriate screening examination must be provided to anyone who comes to the health care facility seeking medical care. |
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If the health care facility determines that the individual has an emergency medical condition, the facility must treat and stabilize the patient. |
It should be noted that the EMTALA requirements apply for all patients, not just those who are covered by Medicare. The penalties for violation of EMTALA are quite draconian and include fines as well as exclusion from Medicare, and giving rise to private causes of action. In practical terms, this means that an anesthesiologist summoned to the emergency department has an obligation to intubate and otherwise stabilize a child, even if the parents have not yet consented to treatment.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ RISK MANAGEMENT
It goes without saying that the best way to avoid legal liability is to always get complete and informed consent from the person authorized to give consent and to practice impeccably so as to avoid all possible harm to patients. It is also common wisdom that if a physician has an excellent relationship with a patient and the patient's family, the likelihood of a lawsuit is lessened. In most cases, the avoidance of litigation based on interpersonal relationship is not available to pediatric anesthesiologists. In most instances, the interaction of a pediatric anesthesiologist is brief and takes place at a stressful time when there is an anticipation of surgery or another frightening procedure. This may be part of the reason why pediatric anesthesiologists may be sued, even if they are always mindful of the requirements for informed consent and scrupulously follow the standards of practice for their profession.
There are, however, other strategies that may help to minimize the risk. With the realization that untoward events occur despite flawless medical care, the pediatric anesthesiologist must be prepared to deal with unexpected outcomes. The best defense in any malpractice case is a careful, legible medical record with detailed preoperative assessments, anesthetic record, and postoperative notes. It is also prudent to discuss adverse events directly with the patient or the patient's family. It is tempting to defer these discussions to the surgeon, but an honest, caring, and open discussion between the anesthesiologist and patients and their families may prevent the filing of a suit. A careful note in the chart describing the adverse event, the factors that led to the event, and a description of actions that were undertaken to minimize harm is also essential when it is realized that it may be years between the adverse event and any resultant lawsuit. Because the statute of limitations for negligence suits only begins when a child reaches the age of majority, a lawsuit may be brought against a pediatric anesthesiologist as late as 20 years after the event. It goes without saying that any urge to revise the chart should be assiduously avoided. Erasures and marginal notes may be used by the plaintiff's attorneys to great advantage to cast doubt on one's integrity.
Finally, once a physician receives notice that a lawsuit has been filed, he or she should immediately contact the malpractice carrier and the legal department of the institution in which the activity giving rise to the claim occurred. To be a defendant in a malpractice suit can be emotionally devastating, and there is a tendency to want to solicit support and advice from friends and colleagues. As understandable as it is to try to gain solace, it is prudent to discuss the matter only with one's attorney, who cannot testify as to those conversations because of attorney-client privilege.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ RESEARCH IN CHILDREN
Any institution that receives federal monies for research, including grants from the National Institutes of Health (NIH), must have a federalwide assurance from the Office for Human Research Protections (OHRP) in order to conduct any research involving human subjects. As a part of this assurance, the institution must pledge to follow the Belmont Report in all research activities regardless of the source of funding. The overarching principles of the Belmont Report have been codified at 45 CFR 46 with similar regulations covering Food and Drug Administration-controlled test articles found at 21 CFR 50. Subpart D of both of these statutes contains the additional protections afforded children when they become research subjects. In addition to requiring written permission from parents, the regulations require that the institutional review board (IRB) responsible for the conduct of the research ensures that there are adequate provisions for soliciting the assent of children who are developmentally capable of giving consent. Although many IRBs and researchers use 7 years as the age when most children are capable of giving assent, this is not the position taken by the regulations. To the contrary, the regulations provide that this determination should be made on the basis of the age, maturity, and psychological state of the involved child.
Apart from the requirement of assent, the most important statutory difference between the statutory requirements for conducting research with children and that with adults is that research involving children falls into one of four permissible categories. Once a given risk-benefit category is assigned, additional protections must be met.
The categories of permissible research in children and their attendant requirements are as follows.
Under the federal regulations, “minimal risk” is defined as the probability and magnitude of harm encountered by healthy children in their ordinary life experiences including routine physical examinations and educational/psychometric testing. Under this definition, studies that involve the sampling of a small amount of blood or a urine test are considered minimal risks because such activities might be encountered during a routine physical examination. When research with children falls into this category, the only additional requirement is permission from parents and the assent of the child subjects.
* CFR is the abbreviation for the Code of Federal Regulations.
2. Research Involving Greater Than Minimal Risk but Presenting the Prospect of Direct Benefit to the Individual Subjects (45 CFR 46.405, 21 CFR 50.52)
Under this category, the research intervention must hold the possibility of contributing to the subjects—well-being. In addition to the requirements for permission from parents and assent from children, research under this category must be such that the risk is justified by the anticipated benefit to the subjects and that the relation of the anticipated benefit to the risk is at least as favorable to the subject as that presented by available alternative approaches.
Whether a placebo-controlled study can be approved under this category depends, in part, on whether the determination is made before or after randomization. The regulations are silent as to when the determination should be made, so there is variation among IRBs in interpretation. In general, however, placebo-controlled studies can be approved under this category if (1) there is no standard treatment for the condition under study or (2) the standard treatment carries significant risk or toxicity.
It is important to note that research cannot be conducted on healthy control subjects under this category because they do not have a disorder or condition. In advice from the OHRP, however, a genetic propensity to a disease may be construed as a condition. It might, then, be possible to conduct research on a child at risk of malignant hyperthermia under this category, even if the diagnosis had not yet been firmly established.
An additional requirement for research in this category is that the intervention or procedure presents experiences to the subject that are reasonably commensurate with those inherent in his or her actual or expected medical, dental, psychological, social, or educational situations. Thus, a child with leukemia might be able to undergo bone marrow aspiration as part of a research study as this is consistent with the child's actual medical experiences; a liver biopsy, on the other hand, probably could not be justified.
For an IRB to approve research under this category, two additional findings must be made. The risk must represent only a minor increase over minimal risk, and the IRB must find that the research is likely to yield generalizable knowledge about the subject's disorder or condition that is of vital importance for the understanding or amelioration of that condition. Many IRBs have difficulty applying these statutory requirements because there is little guidance to help with the interpretation of “minor increase over minimal risk” and “vital importance.” Further guidance from the OHRP is anticipated.
Finally, as an added protection for children in whom research is being conducted under this category, the written permission of both parents is required if they are reasonably available.
Research in this category cannot be approved by local IRBs but must be referred to OHRP or the FDA for consideration by a panel of experts, as well as an opportunity for public review and comment.
There has not been extensive case law interpreting these regulations governing research in children, but an important case was decided by the Maryland Supreme Court. In Grimes v. Kennedy Krieger Institute (366 Md 29, 782 A2d 807, 2001), the plaintiffs were children enrolled in a study to evaluate different methods of lead abatement in the Baltimore area. The plaintiffs alleged that they were encouraged to remain in the study houses so that their lead levels could be monitored over time even when there was knowledge that lead had not been completely removed. In addition, one child was found to have a significant elevation in his blood lead level, and the parents were never informed of this finding. The trial court found in favor of Kennedy Krieger Institute on the basis that the researchers had no duty to warn the subjects about the presence of lead dust. The court of appeals reversed and held that the federal regulations governing research may create an affirmative duty for researchers that are enforceable in state negligence actions. More important, the trial court stated the following:
In our view, otherwise healthy children should not be subjects of non-therapeutic research that has the potential to be harmful to the child. It is first and foremost the responsibility of the researcher and the research entity to see to the harmlessness of such non-therapeutic research. Consent of parents can never relieve the researchers of this duty (366 Md 29, 782 A2d 807, 2001).
The court further stated:
We hold that in Maryland a parent, appropriate relative, or other appropriate surrogate cannot consent to the participation of a child or other person under legal disability in non-therapeutic research or studies in which there is any risk of injury or damage to the health of the subject (366 Md 29, 782 A2d 807, 2001).
This latter holding has engendered considerable concern in the pediatric research community. There are many currently approved studies in institutions across the United States in which children are enrolled in nontherapeutic studies. Measurements of certain physiologic functions (e.g., nerve conduction studies or electromyograms) that do not lengthen the duration of anesthesia have been seen by many IRBs as constituting minimal risk. Under the Maryland holding, such studies are not approvable because they are nontherapeutic. At the time of this writing, the Maryland standard has not been adopted in any other jurisdictions.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ DEATH AND ORGAN DONATION
For centuries, the definition of death was based on the cessation of respiration and heartbeat. With the advent of the modern intensive care unit with respirators and extracorporeal membrane oxygenation devices, this definition was no longer useful. In 1974, the House of Delegates of the American Medical Association recognized a definition of death based on the irreversible cessation of all brain functions.
When death is declared, there is no longer a patient but only a dead body. This is so even if heartbeat and respirations and other functions are preserved while awaiting the harvesting of organs for transplantation. Insurance companies cease to pay for any medical or surgical treatments once death has been declared. If there is any payment for the services rendered to a dead body to preserve organs for transplantation, it is from the insurance company of the recipients.
An ethical dilemma that occurred at the Children's Hospital of Pittsburgh, which is likely to occur elsewhere (if it has not already), is when the parent of a child who has been declared dead but whose heartbeat and respirations are being maintained for transplantation wants to be present when the organs are harvested. Despite the medical acceptance of brain criteria for death, it is difficult for some parents to fully grasp this concept when their child's body remains warm and pink and has a pulse. At this institution, this dilemma was handled through counseling of the family and the nursing and support staff so that a solution was found that was acceptable to all. This situation may well illustrate the increasingly complicated and fascinating legal and ethical dilemmas that pediatric anesthesiologists will face over the next decade.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
▪ SUMMARY
The practice of pediatric anesthesiology not only requires an extensive knowledge of pediatrics and the principles of anesthesia but also demands an understanding of basic legal and ethical concepts. Although it is unpleasant to contemplate the possibility of a lawsuit, a basic understanding of the elements that must be proved by a malpractice plaintiff may provide some reassurance that merely having been named does not mean that the plaintiff will prevail. Pediatric anesthesiology is an exciting, fluid, and developing field, and an understanding of regulations governing research with children provides a foundation for contributing to the knowledge of an interesting and essential field that delivers excellent health care to children.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
Appendix A : Pediatric Drug Dosages
Franklyn P. Cladis
|
Generic Name (Trade Name) |
Route |
Dose |
Indication |
Side Effects |
|
Acetaminophen (Tylenol) |
PO PR |
10 to 15 nig/kg q 4 to 6 hr 20 to 40 mg/kg initial dose, then 20 mg/kg q 6 hr |
Pain/fever |
|
|
Ade nos ine |
IV |
0.1 mg/kg bolus; if not effective, 0.2 mg/kg (12 mg maximum single dose) |
SVT |
Flushing, chest pain, bronchospasm |
|
Albuterol |
Nebulized |
0.15 mg/kg in 2 mL normal saline solution |
Bronchospasm |
Tachycardia |
|
Alfentanil |
IV |
10 to 150 meg/kg bolus |
Anesthesia |
Apnea, bradycardia |
|
0.5 to 3 meg/kg per min |
Adjunct for anesthesia maintenance |
|||
|
Alprostad.il |
IV |
0.05 to 0.1 meg/kg per min |
Patency of ductus arteriosus |
Fever, apnea, arrhythmias |
|
Aminocaproic acid (Amicar) |
IV |
100 mg/kg bolus, infusion 30 mg/kg per hr |
Excessive bleeding from fibrinolysis |
Hypotension, bradycardia, arrhythmias |
|
Aminophylline |
PO, IV |
100 mg/kg load: 100 mg/kg dose every 6 hr 5 rng/kg load, infusion 1 mg/kg per hr |
Bronchospasm |
Arrhythmias |
|
Amiodarone |
IV |
5 rng/kg bolus slowly, infusion 5 to 10 meg/kg per min |
Ventricular arrhythmias |
Hypotension, arrhythmias (torsades de pointes) |
|
Ampie il lin |
IV |
50 mg/kg |
Endocarditis prophylaxis |
|
|
Aprotinin (Trasylol) |
IV Load |
10,000 units test done, 30,000 units/kg over 30 min |
To decrease bleeding, used mostly in cardiac surgery |
Anaphylaxis, celite ACT prolonged by aprotinin |
|
Load |
30,000 units/kg in the pump prime |
|||
|
IV infusion |
10,000 to 30,000 units/kg per hr (turn off during cardiopulmonary bypass and at end of surgery) |
|||
|
Atracurium (Tracrium) |
IV |
0.5 rng/kg intubation |
Neuromuscular blocker |
Histamine release |
|
5 to 15 meg/kg per min infusion |
||||
|
Atropine |
IV |
0.01 to 0.02 mg/kg |
Brady cardia |
|
|
Bretylium |
IV |
5 to 10 mg/kg load, q 10 to 20 min for a total of 30 mg/kg 1 to 2 mg/min infusion (>12 yr old) |
Refractory ventricular tachycardia and fibrillation |
Hypotension |
|
Bupivacaine (Marcaine) |
Neuraxial, peripheral |
2.5 mg/kg |
Epidural, caudal, peripheral nerve blockade |
Seizures, arrhythmias |
|
Butorphanol |
IV |
10 meg/kg |
Pain/fever |
CNS depression, respiratory depression |
|
Caffeine |
PO, IV |
5 to 10 mg/kg as caffeine base (10 to 20 mg/kg as caffeine citrate) |
Apnea of prematurity |
Tachyarrhythmias |
|
Calcium chloride |
IV |
10 to 20 mg/kg (via central venous line) |
Hypocalcemia, hypotension |
Tissue necrosis |
|
Calcium gluconate |
IV |
30 to 45 mg/kg |
Hypocalcemia, hypotension |
Tissue necrosis |
|
Cefazolin (Ancef) |
IV |
25 mg/kg |
Wound prophylaxis |
|
|
Chloral hydrate |
PO/PR |
50 to 75 mg/kg |
Sedation |
Arrhythmias |
|
Cimetidine (Tagamet) |
IV/PO |
5 to 15 mg/kg q 6 to 12 hr |
Gastric pH control |
Confusion, headache |
|
Cisatracurium (Nimbex) |
IV |
0.1 to 0.2 mg/kg bolus, infusion 1 to 5 meg/kg per min |
Neuromuscular blockade |
|
|
Clindamycin (Cleocin) |
IV |
5 to 10 mg/kg (slowly) q 6 to 8 hr |
Wound prophylaxis |
Thrombophlebitis, |
|
20 mg/kg (slowly) for SBE prophylaxis |
(penicillin [PCN] allergic) |
hypotension |
||
|
Clonidine |
Neuraxial |
1 to 3 meg/kg |
Enhance neuraxial blockade |
Hypotension, sedation |
|
Codeine |
PO |
0.5 to 1 mg/kg |
Pain |
Sedation, nausea, vomiting, respiratory depression |
|
Dantrolene |
IV |
2.5 mg/kg to maximum 10 mg/kg |
Malignant hyperthermia |
Slow to solubilize, drowsiness, muscle weakness |
|
Desmopressin (DDAVP) |
IV |
0.3 mcg/kg (over 15 min) |
Platelet dysfunction, von Willebrand disease |
Hyponatremia, hypertension, flushing |
|
IV |
2 to 4 meg/day in 2 doses (>12 years old) |
Diabetes insipidus |
Hyponatremia, zhypertension, flushing |
|
|
Dexamethasone (Decadron) |
IV |
0.5 to 1 mg/kg (maximum 16 to 20 mg) |
Croup, airway edema, cerebral edema |
Hypertension, headache, hype rglyce mia |
|
Diazepam (Valium) |
PO |
0.2 to 0.3 nig/kg 45 to 60 minutes preprocedure |
Preoperative sedation |
Hypotension, bradycardia |
|
IV |
0.05 to 0.3 mg/kg |
|||
|
Digoxin |
IV |
0.03 mg/kg <2 years old |
SVT, CHF |
Arrhythmias, atrioventricular block |
|
0.01 to 0.04 mg/kg >2 years old (digitalizing dose; V2 dose initially then V4 q 6 hr × 2) |
||||
|
IV maintenance |
2 to 10 mcg/kg per day (IV dose = 75% oral dose) |
|||
|
Diphenylhydantoin (Dilantin) |
IV |
12 to 20 mg/kg (over 30 min to avoid hypotension; limit infusion rate to 50 mg/min) |
Seizure, digoxin toxicity |
Hypotension, skin necrosis at IV site, ataxia, gingival hyperplasia |
|
Diphenhydramine |
IV |
0.5 to 2 mg/kg (maximum single dose 50 mg) |
Sedation, pruritus, acute |
Paradoxical excitement |
|
(Benadryl) |
(maximum 150 mg/day if <12 years old, 300 mg if > 12 years old) |
dystonic reactions |
||
|
Dobutamine |
IV |
2 to 15 mcg/kg per min (maximum dose 40 meg/kg/min) |
Heart failure |
Increased heart rate, ectopy, hypotension |
|
Dopamine |
IV |
2.5 to 20 mcg/kg per min |
Heart failure, increased renal blood flow—low dose |
Increased heart rate, ectopy |
|
Doxac uriu m |
IV |
0.05 to 0.1 mg/kg intubation |
Neuromuscular blocker |
|
|
Droperidol |
IV |
10 to 75 mcg/kg (maximum dose 1.25 mg) |
Antiemetic |
Hypotension, arrhythmias (FDA warning) |
|
d-Tubocurarine |
IV |
0.3 to 0.6 mg/kg |
Neuromuscular blocker |
Histamine release |
|
Edrophonium (Enlon, Tensilon) |
IV |
0.5 to 1.0 mg/kg |
Neuromuscular blocking reversal agent |
Bradycardia |
|
EM LA cream |
Topical |
0 to 3 mo maximum 1 g (10 cm2), 3 to 12 mo maximum 2 g (20 cm2), 1 to 6 years old maximum 10 g (100 cm2), 7 to 12 years old maximum 20 g (200 cm2) |
Dermal anesthesia for venipuncture |
Methemoglobinemia, blanching of skin |
|
Ephedrine |
IV |
0.02 to 0.2 mg/kg |
Hypotension |
Bradycardia, hypotension, confusion |
|
Epinephrine (Adrenalin) |
IV |
10 mcg/kg q 3 to 5 min, infusion 0.01 to 1 mcg/kg per min |
Cardiac arrest, hypotension, heart failure |
Tachycardia |
|
Endotracheal |
100 mcg/kg |
|||
|
Epinephrine, racemic |
Nebulized |
<2 years old 0.25 mL, >2 years old 0.5 mL of 2.25% solution in 3 mL normal saline solution |
Upper airway edema |
Tachycardia |
|
Esmolol |
IV |
500 mcg/kg per min loading dose for one minute 50 to 300 mcg/kg per min infusion |
, Hypertension, tachycardia |
Bradycardia |
|
Etomidate |
IV |
0.3 mg/kg (0.2 to 0.6 mg/kg) |
Anesthetic induction |
Myoclonus, pain on injection, adrenal suppression |
|
Fentanyl |
IV |
0.5 to 3 mcg/kg (may repeat q 30 min) |
Analgesia and sedation |
Apnea, cough, laryngospasm, chest wall rigidity, bradycardia |
|
2 to 10 mcg/kg bolus, infusion 1 to 4 mcg/kg per hr |
Adjunct for induction and maintenance of anesthesia |
|||
|
Fentanyl (Oralet, Actiq) |
PO |
5 to 15 mcg/kg (200 meg is smallest dose) |
Sedation preoperatively |
Sedation, pruritis, oxygen desaturati on |
|
Flu maze nil |
IV |
0.01 mg/kg (maximum dose 0.2 mg) |
Benzodiazepine antagonist |
Altered blood pressure, arrhythmias |
|
q 1 min up to 1 mg (maximum cumulative dose) |
||||
|
Furosemide (Lask) |
IV |
1 to 2 mg/kg q 6 hr (6 mg/kg per day maximum dose) |
Diuretic |
Hypokalemia, hyponatremia, potential ototoxicity |
|
Gentamicin |
IV |
1.5 to 2.5 mg/kg |
Endocarditis prophylaxis |
Nephrotoxicity, ototoxicity, potentiate neuromuscular blockade |
|
Glucagon |
IM, IV |
<20 kg 0.5 mg, >20 kg 1 mg |
Antihypoglycemic, relaxant for gastrointestinal tract |
Tachycardia |
|
Glucose |
IV |
200 mg/kg, 4 mg/kg per min |
Hypoglycemia |
|
|
G lyco pyrrol ate |
IV |
0.005 to 0.01 mg/kg |
Antisialagogue, |
Tachycardia, flushing |
|
(Robinul) |
bradycardia |
|||
|
Hydralazine |
IV |
0.05 to 0.3 mg/kg (15 min onset) |
Hypertension |
Tachycardia, flushing |
|
Hydrocortisone (Solu-Cortef) |
IV |
1 to 2 mg/kg q 6 hr |
Adrenal suppression, asthma |
Hypertension, hype rglyce mia |
|
Hydromorphone (Dilaudid) |
IV |
0.01 to 0.015 mg/kg |
Analgesia |
Sedation, nausea, vomiting, respiratory depression |
|
Hydroxyzine (Atarax, Vistarli) |
IM |
0.5 to 1 mg/kg q 4 to 6 hr |
Sedation |
Drowsiness |
|
I blip role n |
PO |
10 mg/kg q 6 hr |
Analgesic/antiinflammatory |
Inhibition of platelet aggregation, renal failure |
|
Inamrinone |
IV |
Load 3 to 4.5 mg/kg over 30 min, then 3 to 10 mcg/kg per min infusion |
Heart failure |
Arrhythmias, thrombocytopenia |
|
Insulin, regular |
IV |
Load 0.01 to 0.1 unit/kg, infuse 0.1 unit/kg per hr |
Hyperglycemia, DKA |
Hypoglycemia |
|
Isoproterenol (Isuprel) |
IV |
0.05 to 1 mcg/kg per min |
Bradycardia |
Tachycardia, ventricular arrhythmias, hypotension |
|
Ketamine |
IV |
0.5 to 1 mg/kg |
Sedation |
|
|
IV |
1 to 2 mg/kg |
Anesthesia |
Hallucinations, tachycardia, may increase intracranial and intraocular hypersalivation, pressure, apnea, vomiting |
|
|
IM |
3 to 5 mg/kg |
Sedation |
||
|
IM |
5 to 10 mg/kg |
Anesthesia |
||
|
PO |
5 to 10 mg/kg |
Sedation |
||
|
Ketorolac (Toradol) |
IV |
0.5 to 1 mg/kg |
Analgesia |
Inhibition of platelet aggregation, renal failure |
|
Labetalol |
IV |
0.2 to 0.5 mg/kg (q 5 min) |
Tachycardia, hypertension |
Hypotension, bradycardia |
|
Lidocaine |
IV |
1 mg/kg |
Ventricular arrhythmias |
Seizures |
|
IV |
20 to 50 mcg/kg per min infusion |
|||
|
Lidocaine |
Intratracheal |
3 to 4 mg/kg |
Topical anesthesia |
Arrhythmias, seizures |
|
Lorazepam |
IV |
0.05 to 0.1 mg/kg (maximum 4 mg) |
Sedation, anxiolysis |
Neurotoxicity in neonates (preservative) |
|
Mannitol |
IV |
0.25 to 1 g/kg (over 20 min) |
Increased intracranial pressure, diuresis |
|
|
Meperidine (Demerol) |
IV IM |
1 to 2 mg/kg 1 to 2 mg/kg |
Analgesia, preoperative sedation |
Accumulation of normeperidine in renal dysfunction |
|
Methadone |
IV |
0.1 mg/kg |
Analgesia |
Sedation, nausea, vomiting, respiratory depression |
|
Methohexital (Brevital) |
IV |
1 to 2 mg/kg |
Anesthesia |
Apnea |
|
PR |
25 to 35 nig/kg (10% solution) |
Sedation/anesthesia |
||
|
Methylene blue |
IV |
1 to 2 mg/kg may repeat in 1 hr |
Antidote for drug-induced methemoglobinemia |
Stains skin |
|
Methylprednisolone (Solu-Medrol) |
IV |
2 mg/kg load, 0.5 to 1 mg/kg q 6 hr |
Asthma exacerbation |
Hypertension, psychosis, glucose intolerance |
|
IV |
30 mg/kg over 15 min, infusion 5.4 mg/kg per hr for 23 hr |
Acute spinal cord injury |
||
|
Metoclopramide (Reglan) |
IV |
0.1 to 0.2 mg/kg |
Antiemetic |
Extrapyramidal reactions |
|
Midazolam (Versed) |
IV |
0.05 to 0.1 mg/kg |
Sedation |
Hypotension in neonates, hiccups |
|
IN |
0.2 to 0.3 mg/kg (5 to 10 min onset) |
Sedation |
Nasal irritation |
|
|
PO |
0.5 to 1 mg/kg (20 to 30 min onset) |
Sedation |
Bad taste |
|
|
Milrinone |
IV |
50 mcg/kg load, infusionc 0.25 to 0.75 mcg/kg per min |
Heart failure |
Hypotension |
|
Mivacurium |
IV |
0.2 to 0.3 mg/kg intubation; 10 to 15 mcg/kg per min infusion |
Neuromuscular blocker |
Histamine release |
|
Morphine |
IV/IM |
0.05 to 0.1 mg/kg |
Analgesia |
Histamine release, apnea, sedation, nausea, vomiting |
|
Naloxone (Narcan) |
IV/IM |
1 to 10 mcg/kg titrated |
Apnea/sedation after opioids |
Hypertension |
|
Naproxen (Naprosyn) |
PO |
5 to 10 mg/kg q 12 hr |
Analgesia, anti-inflammatory |
Inhibition of platelet aggregation, renal failure |
|
Ne o stigmi ne |
IV |
0.05 to 0.07 mg/kg |
Neuromuscular blockade reversal |
Bradycardia, secretions |
|
Nicardipine |
IV |
0.5 to 5 mcg/kg per min |
Induced hypotension, hypertension |
Hypotension |
|
Nitroglycerin |
IV |
0.5 to 3 mcg/kg per min |
Venodilation, myocardial ischemia |
Hypotension |
|
Nhroprusside |
IV |
0.5 to 10 mcg/kg per min |
Induced hypotension, hypertension |
Cyanide toxicky |
|
Norepinephrine (Levophed) |
IV |
0.1 to 1 mcg/kg per min |
Hypotension |
Arrhythmias |
|
Ondansetron (Zofran) |
IV |
0.05 to 0.1 mg/kg (maximum 4 mg) |
Antiemetic |
Headache |
|
Oxycodone |
PO |
0.05 to 0.15 mg/kg q 4 to 6 hr |
Analgesia |
Sedation, nausea, vomiting, respiratory depression |
|
Pancuronium (Pavulon) |
IV |
0.08 to 0.15 mg/kg |
Neuromuscular blocker |
Tachycardia |
|
Pentobarbital (Nembutal) |
IV |
1 to 3 mg/kg (maximum 100 mg) |
Sedation |
Respiratory depression, apnea |
|
Phenylephrine |
IV |
1 to 10 mcg/kg |
Hypotension |
Hypertension |
|
(Neo-Synephrine) |
IV |
0.1 to 0.5 mcg/kg/min infusion |
||
|
Potassium chloride |
IV |
0.5 mEq/kg (via CVP over 30 to 45 min) |
Hypokalemia |
Arrhythmias with rapid infusion |
|
Procainamide |
IV |
3 to 6 mg/kg per dose over 5 min q 5 to 10 min (maximum 15 mg/kg), infusion 20 mcg/kg per min |
Ventricular and atrial arrhythmias |
Hypotension, QT prolongation |
|
Prochlorperazine |
IV/IM |
0.1 to 0.15 mg/kgq6hr |
Antiemetic |
High incidence of |
|
(Compazine) |
PO/PR |
(IV not recommended) 0.4 mg/kg per day divided q 6 to 8 hr |
extrapyramidal reactions, sedation, hypotension |
|
|
Promethazine (Phenergan) |
IV/IM |
0.25 to 0.5 mg/kg q 4 to 6 hr |
Antiemetic |
Sedation |
|
Propofol |
IV |
2 to 3 mg/kg; 100 to 200 mcg/kg per min infusion |
Anesthesia |
Apnea, hypotension |
|
Propranolol (Inderai) |
IV |
0.01 to 0.1 mg/kg (over 10 min) |
Tachycardia, hypertension |
Hypotension, bradycardia |
|
Prostaglandin Ej |
IV |
0.05 to 0.1 mcg/kg per min |
Maintain patent ductus arteriosus |
Fever, apnea |
|
Protamine |
IV |
1 mg for every 100 U of heparin to be neutralized |
Heparin neutralization |
Hypotension, bradycardia, pulmonary hypertension |
|
Ranitidine (Zantac) |
IV PO |
1 to 1.5 mg/kg per day divided q 8 hr |
Gastric pH control |
|
|
2 to 3 mg/kg per day divided q 12 hr |
||||
|
Remifentanil |
IV |
1 to 4 mcg/kg bolus, infusion 0.1 to 0.5 mcg/kg per min |
Analgesia, adjunct to anesthesia |
Apnea, bradycardia |
|
Rocuronium (Zemuron) |
IV |
0.6 to 1.2 mg/kg |
Neuromuscular blocker |
|
|
Scopolamine |
IV/IM |
6 to 10 mcg/kg (maximum 0.3 mg/dose) |
Antisialagogue, amnestic |
Tachycardia, confusion |
|
Sodium bicarbonate |
IV |
0.5 to 1 mEq/kg |
Metabolic acidosis |
|
|
Succinylcholine |
IV |
1.5 to 2 mg/kg (fast onset) |
Neuromuscular blocker (depolarizing) |
Hyperkalemia, malignant hyperthermia trigger |
|
IM |
5 mg/kg |
Neuromuscular blocker |
||
|
Sufentanil |
IV |
0.25 to 1 mcg/kg bolus |
Anesthesia |
Apnea, chest wall rigidity |
|
IV |
0.1 to 1 mcg/kg per hr infusion |
Adjunct for maintenance of anesthesia |
||
|
Thiopental |
IV |
3 to 7 mg/kg |
Anesthesia |
Apnea |
|
Vancomycin |
IV |
10 mg/kg (over 30 min) |
Wound prophylaxis |
Hypotension, histamine release, red man syndrome |
|
Vas op ressi n |
IV |
0.0005 to 0.005 unit/kg per min |
Vasopressor, diabetes insipidus |
Hypertension |
|
Vecuronium (Norcuron) |
IV |
0.07 to 0.2 mg/kg |
Neuromuscular blocker |
|
|
0.8 to 1 mcg/kg per min infusion |
||||
|
Verapamil |
IV |
0.1 to 0.3 mg/kg (1 to 15 years old) |
SVT, CHF |
Bradycardia hypotension |
|
IM, intramuscular; IN, intranasa; IV, intravenous; CHF, congestive heart failure; PR, per rectum; SVT, supraventricular tachycardia; DKA, diabetic ketoacidosis; PO, orally; ACT, activated clotting time. |
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
Appendix B : Growth Curves[*]
|
FIGURE B-1 Girls from birth to 36 months: Length by age. |
|
FIGURE B-2 Girls from birth to 36 months: Weight by age. |
|
FIGURE B-3 Girls from 2 to 18 years: Stature by age. |
|
FIGURE B-4 Girls from 2 to 18 years: Weight by age. |
|
FIGURE B-5 Boys from birth to 36 months: Length by age. |
|
FIGURE B-6 Boys from birth to 36 months: Weight by age. |
|
FIGURE B-7 Boys from 2 to 18 years: Stature by age. |
|
FIGURE B-8 Boys from 2 to 18 years: Weight by age. |
* From Hoekelman RA, Blatman S, Friedman SB, editors: Primary pediatric care. St Louis, 1987, The CV Mosby Co. Modified from Hamill VV, Drizd TA, Johnson CL, and others: Am J Clin Nutr 32:607, 1979. Reproduced with permission by the American Journal of Clinical Nutrition. Copyright © 1979 American Society for Clinical Nutrition.
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
Appendix C : Normal Pulmonary Function Values
TABLE C-1 -- Vital capacity (VC) in relation to body height in boys
|
Height (cm) |
VC (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
1,418 |
1,238 |
1,623 |
|
116 |
1,452 |
1,269 |
1,662 |
|
118 |
1,523 |
1,330 |
1,743 |
|
120 |
1,596 |
1,394 |
1,827 |
|
122 |
1,671 |
1,460 |
1,912 |
|
124 |
1,748 |
1,527 |
2,001 |
|
126 |
1,828 |
1,597 |
2,092 |
|
128 |
1,909 |
1,668 |
2,186 |
|
130 |
1,994 |
1,742 |
2,282 |
|
132 |
2,080 |
1,817 |
2,381 |
|
134 |
2,169 |
1,895 |
2,482 |
|
136 |
2,260 |
1,974 |
2,587 |
|
138 |
2,354 |
1,056 |
2,694 |
|
140 |
2,450 |
2,140 |
2,804 |
|
142 |
2,548 |
2,226 |
2,917 |
|
144 |
2,649 |
2,315 |
3,032 |
|
146 |
2,753 |
2,405 |
3,151 |
|
148 |
2,859 |
2,498 |
3,273 |
|
150 |
2,968 |
2,593 |
3,397 |
|
152 |
3,079 |
2,690 |
3,524 |
|
154 |
3,193 |
2,790 |
3,655 |
|
156 |
3,310 |
2,892 |
3,788 |
|
158 |
3,429 |
2,996 |
3,925 |
|
160 |
3,551 |
3,102 |
4,065 |
|
162 |
3,676 |
3,211 |
4,207 |
|
164 |
3,803 |
3,323 |
4,353 |
|
166 |
3,934 |
3,437 |
4,503 |
|
168 |
4,067 |
3,553 |
4,655 |
|
170 |
4,203 |
3,672 |
4,811 |
|
172 |
4,342 |
3,793 |
4,970 |
|
174 |
4,484 |
3,917 |
5,132 |
|
176 |
4,629 |
4,044 |
5,298 |
|
178 |
4,776 |
4,173 |
5,467 |
|
180 |
4,927 |
4,304 |
5,639 |
|
Formula: log y = a + b · log x; y = VC (mL); x = Body height (cm); log VC (mL) = -2.5768 + 2.7799 · log height (cm); SD log y · x = ±0.0294; n = 86; r = +0.96. |
TABLE C-2 -- Vital capacity (VC) in relation to body height in girls
|
Height (cm) |
VC (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
1,365 |
1,128 |
1,651 |
|
116 |
1,396 |
1,154 |
1,689 |
|
118 |
1,461 |
1,207 |
1,767 |
|
120 |
1,527 |
1,262 |
1,847 |
|
122 |
1,595 |
1,318 |
1,930 |
|
124 |
1,665 |
1,376 |
2,014 |
|
126 |
1,736 |
1,435 |
2,101 |
|
128 |
1,810 |
1,496 |
2,190 |
|
130 |
1,886 |
1,559 |
2,281 |
|
132 |
1,963 |
1,623 |
2,375 |
|
134 |
2,043 |
1,688 |
2,471 |
|
136 |
2,124 |
1,756 |
2,569 |
|
138 |
2,207 |
1,824 |
2,670 |
|
140 |
2,293 |
1,895 |
2,774 |
|
142 |
2,380 |
1,967 |
2,879 |
|
144 |
2,469 |
2,041 |
2,987 |
|
146 |
2,561 |
2,117 |
3,098 |
|
148 |
2,654 |
2,194 |
3,211 |
|
150 |
2,750 |
2,273 |
3,327 |
|
152 |
2,848 |
2,354 |
3,445 |
|
154 |
2,948 |
2,436 |
3,566 |
|
156 |
3,050 |
2,521 |
3,689 |
|
158 |
3,154 |
2,607 |
3,815 |
|
160 |
3,260 |
2,695 |
3,944 |
|
162 |
3,369 |
2,784 |
4,075 |
|
164 |
3,479 |
2,876 |
4,209 |
|
166 |
3,592 |
2,969 |
4,346 |
|
168 |
3,708 |
3,065 |
4,485 |
|
170 |
3,825 |
3,162 |
4,628 |
|
172 |
3,945 |
3,261 |
4,772 |
|
174 |
4,067 |
3,362 |
4,920 |
|
176 |
4,191 |
3,465 |
5,071 |
|
178 |
4,318 |
3,569 |
5,224 |
|
180 |
4,447 |
3,676 |
5,380 |
|
Formula: log y = a + b · log x; y = VC (mL); x = Body height (cm); log VC (mL) = -2.2970 + 2.6361 · log x; SD log y ·x = ±0.0415; n = 87; r = +0.94. |
TABLE C-3 -- Total lung capacity measured in a body plethysmograph (TLCbox) in relation to body height in boys
|
Height (cm) |
TLCbox (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
1,966 |
1,732 |
2,232 |
|
116 |
2,011 |
1,771 |
2,283 |
|
118 |
2,101 |
1,851 |
2,385 |
|
120 |
2,194 |
1,932 |
2,490 |
|
122 |
2,289 |
2,016 |
2,599 |
|
124 |
2,387 |
2,102 |
2,709 |
|
126 |
2,487 |
2,109 |
2,823 |
|
128 |
2,589 |
2,281 |
2,940 |
|
130 |
2,695 |
2,374 |
3,059 |
|
132 |
2,803 |
2,469 |
3,182 |
|
134 |
2,913 |
2,566 |
3,307 |
|
136 |
3,026 |
2,666 |
3,436 |
|
138 |
3,142 |
2,767 |
3,567 |
|
140 |
3,260 |
2,872 |
3,701 |
|
142 |
3,381 |
2,978 |
3,839 |
|
144 |
3,505 |
3,087 |
3,979 |
|
146 |
3,631 |
3,199 |
4,123 |
|
148 |
3,761 |
3,313 |
4,269 |
|
150 |
3,893 |
3,429 |
4,419 |
|
152 |
4,028 |
3,548 |
4,572 |
|
154 |
4,165 |
3,669 |
4,729 |
|
156 |
4,306 |
3,793 |
4,888 |
|
158 |
4,449 |
3,919 |
5,051 |
|
160 |
4,595 |
4,048 |
5,217 |
|
162 |
4,744 |
4,179 |
5,386 |
|
164 |
4,896 |
4,313 |
5,558 |
|
166 |
5,051 |
4,449 |
5,734 |
|
168 |
5,209 |
4,587 |
5,914 |
|
170 |
5,370 |
4,730 |
6,096 |
|
172 |
5,534 |
4,874 |
6,282 |
|
174 |
5,701 |
5,021 |
6,472 |
|
176 |
5,870 |
5,171 |
6,665 |
|
178 |
6,043 |
5,323 |
6,861 |
|
180 |
6,220 |
5,468 |
7,061 |
|
Formula: log y = a + b · log x; y = TLCbox (mL); x = Body height (cm); log TLCbox (mL) = -2.0018 + 2.5698 · log height (cm); SD log y ·x = ±0.0276; n = 82; r = +0.96. |
TABLE C-4 -- Total lung capacity measured in a body plethysmograph (TLCbox) in relation to body height in girls
|
Height (cm) |
TLCbox (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
1,860 |
1,575 |
2,196 |
|
116 |
1,902 |
1,611 |
2,245 |
|
118 |
1,987 |
1,683 |
2,346 |
|
120 |
2,075 |
1,758 |
2,450 |
|
122 |
2,166 |
1,834 |
2,556 |
|
124 |
2,258 |
1,913 |
2,666 |
|
126 |
2,353 |
1,993 |
2,778 |
|
128 |
2,451 |
2,076 |
2,893 |
|
130 |
2,551 |
2,161 |
3,011 |
|
132 |
2,653 |
2,247 |
3,132 |
|
134 |
2,758 |
2,336 |
3,255 |
|
136 |
2,865 |
2,427 |
3,382 |
|
138 |
2,975 |
2,520 |
3,512 |
|
140 |
3,087 |
2,615 |
3,644 |
|
142 |
3,202 |
2,712 |
3,780 |
|
144 |
3,319 |
2,812 |
3,918 |
|
146 |
3,439 |
2,914 |
4,060 |
|
148 |
3,562 |
3,017 |
4,205 |
|
150 |
3,687 |
3,124 |
4,353 |
|
152 |
3,815 |
3,232 |
4,504 |
|
154 |
3,946 |
3,343 |
4,658 |
|
156 |
4,079 |
3,456 |
4,816 |
|
158 |
4,216 |
3,571 |
4,976 |
|
160 |
4,354 |
3,689 |
5,140 |
|
162 |
4,496 |
3,809 |
5,307 |
|
164 |
4,640 |
3,931 |
5,478 |
|
166 |
4,787 |
4,055 |
5,651 |
|
168 |
4,937 |
4,183 |
5,828 |
|
170 |
5,090 |
4,312 |
6,009 |
|
172 |
5,247 |
4,444 |
6,193 |
|
174 |
5,405 |
4,578 |
6,380 |
|
176 |
5,566 |
4,715 |
6,570 |
|
178 |
5,730 |
4,854 |
6,765 |
|
180 |
5,898 |
4,996 |
6,962 |
|
Formula: log y = a + b · log x; y = TLCbox (mL); x = Body height (cm); log TLCbox (mL) = -2.0377 + 2.5755 · log height (cm); SD log y ·x = ±0.0361; n = 72; r = +0.96. |
TABLE C-5 -- Functional residual capacity measured in a body plethysmograph (FRCbox) in relation to body height in boys
|
Height (cm) |
FRCbox (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
941 |
790 |
1,121 |
|
116 |
963 |
809 |
1,148 |
|
118 |
1,008 |
846 |
1,201 |
|
120 |
1,054 |
885 |
1,256 |
|
122 |
1,101 |
925 |
1,312 |
|
124 |
1,150 |
965 |
1,370 |
|
126 |
1,200 |
1,007 |
1,429 |
|
128 |
1,251 |
1,050 |
1,490 |
|
130 |
1,303 |
1,094 |
1,553 |
|
132 |
1,357 |
1,140 |
1,617 |
|
134 |
1,413 |
1,186 |
1,683 |
|
136 |
1,469 |
1,233 |
1,750 |
|
138 |
1,527 |
1,282 |
1,819 |
|
140 |
1,587 |
1,332 |
1,890 |
|
142 |
1,648 |
1,383 |
1,962 |
|
144 |
1,710 |
1,435 |
2,037 |
|
146 |
1,774 |
1,489 |
2,113 |
|
148 |
1,839 |
1,544 |
2,190 |
|
150 |
1,905 |
1,600 |
2,270 |
|
152 |
1,974 |
1,657 |
2,351 |
|
154 |
2,043 |
1,715 |
2,434 |
|
156 |
2,114 |
1,775 |
2,518 |
|
158 |
2,187 |
1,836 |
2,605 |
|
160 |
2,261 |
1,898 |
2,693 |
|
162 |
2,337 |
1,962 |
2,784 |
|
164 |
2,414 |
2,027 |
2,876 |
|
166 |
2,493 |
2,093 |
2,970 |
|
168 |
2,574 |
2,161 |
3,066 |
|
170 |
2,656 |
2,230 |
3,163 |
|
172 |
2,739 |
2,300 |
3,263 |
|
174 |
2,825 |
2,372 |
3,365 |
|
176 |
2,912 |
2,445 |
3,468 |
|
178 |
3,000 |
2,519 |
3,574 |
|
180 |
3,091 |
2,595 |
3,681 |
|
Formula: log y = a + b · log x; y = FRCbox (mL); x = Body height (cm); log FRCbox (mL) = -2.4915 + 2.6523 · log height (cm); SD log y ·x = ±0.0381; n = 82; r = +0.94. |
TABLE C-6 -- Functional residual capacity measured in a body plethysmograph (FRCbox) in relation to body height in girls
|
Height (cm) |
FRCbox (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
906 |
707 |
1,160 |
|
116 |
926 |
723 |
1,186 |
|
118 |
969 |
756 |
1,241 |
|
120 |
1,012 |
790 |
1,296 |
|
122 |
1,057 |
825 |
1,354 |
|
124 |
1,103 |
861 |
1,412 |
|
126 |
1,150 |
898 |
1,473 |
|
128 |
1,198 |
936 |
1,535 |
|
130 |
1,248 |
974 |
1,598 |
|
132 |
1,299 |
1,014 |
1,663 |
|
134 |
1,351 |
1,055 |
1,730 |
|
136 |
1,404 |
1,097 |
1,799 |
|
138 |
1,459 |
1,139 |
1,869 |
|
140 |
1,515 |
1,183 |
1,940 |
|
142 |
1,572 |
1,228 |
2,014 |
|
144 |
1,631 |
1,273 |
2,089 |
|
146 |
1,691 |
1,320 |
2,165 |
|
148 |
1,752 |
1,368 |
2,244 |
|
150 |
1,815 |
1,417 |
2,324 |
|
152 |
1,879 |
1,467 |
2,406 |
|
154 |
1,944 |
1,518 |
2,489 |
|
156 |
2,011 |
1,570 |
2,575 |
|
158 |
2,079 |
1,623 |
2,662 |
|
160 |
2,148 |
1,677 |
2,751 |
|
162 |
2,219 |
1,733 |
2,842 |
|
164 |
2,292 |
1,789 |
2,935 |
|
166 |
2,365 |
1,847 |
3,029 |
|
168 |
2,441 |
1,906 |
3,126 |
|
170 |
2,517 |
1,966 |
3,224 |
|
172 |
2,596 |
2,027 |
3,324 |
|
174 |
2,675 |
2,089 |
3,426 |
|
176 |
2,756 |
2,152 |
3,530 |
|
178 |
2,839 |
2,217 |
3,636 |
|
180 |
2,923 |
2,283 |
3,744 |
|
Formula: log y = a + b · log x; y = FRCbox (mL); x = Body height (cm); log FRCbox (mL) = -2.4314 + 2.6149 · log height (cm); SD log y ·x = ±0.0538; n = 72; r = +0.91. |
TABLE C-7 -- Forced expiratory volume in the first second (FEV1) in relation to body height in boys
|
Height (cm) |
FEV1 (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
1,135 |
952 |
1,352 |
|
116 |
1,163 |
976 |
1,386 |
|
118 |
1,222 |
1,025 |
1,456 |
|
120 |
1,282 |
1,076 |
1,528 |
|
122 |
1,345 |
1,128 |
1,602 |
|
124 |
1,409 |
1,182 |
1,679 |
|
126 |
1,475 |
1,238 |
1,758 |
|
128 |
1,544 |
1,295 |
1,839 |
|
130 |
1,614 |
1,354 |
1,923 |
|
132 |
1,686 |
1,415 |
2,009 |
|
134 |
1,761 |
1,478 |
2,098 |
|
136 |
1,837 |
1,542 |
2,189 |
|
138 |
1,916 |
1,608 |
2,283 |
|
140 |
1,997 |
1,676 |
2,380 |
|
142 |
2,080 |
1,746 |
2,479 |
|
144 |
2,165 |
1,817 |
2,580 |
|
146 |
2,253 |
1,891 |
2,685 |
|
148 |
2,343 |
1,966 |
2,792 |
|
150 |
2,435 |
2,043 |
2,901 |
|
152 |
2,529 |
2,123 |
3,014 |
|
154 |
2,626 |
2,204 |
3,129 |
|
156 |
2,725 |
2,287 |
3,248 |
|
158 |
2,827 |
2,372 |
3,369 |
|
160 |
2,931 |
2,460 |
3,493 |
|
162 |
3,038 |
2,549 |
3,620 |
|
164 |
3,147 |
2,641 |
3,749 |
|
166 |
3,258 |
2,734 |
3,882 |
|
168 |
3,372 |
2,830 |
4,018 |
|
170 |
3,489 |
2,928 |
4,157 |
|
172 |
3,608 |
3,028 |
4,299 |
|
174 |
3,730 |
3,130 |
4,445 |
|
176 |
3,854 |
3,235 |
4,593 |
|
178 |
3,982 |
3,342 |
4,744 |
|
180 |
4,112 |
3,451 |
4,899 |
|
Formula: log y = a + b · log x; y = FEV1 (mL); x = Body height (cm); log FEV1 (mL) = -2.8652 + 2.8729 · log height (cm); SD log y ·x = ±0.0380; n = 60; r = +0.94. |
TABLE C-8 -- Forced expiratory volume in the first second (FEV1) in relation to body height in girls
|
Height (cm) |
FEV1 (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
1,105 |
883 |
1,382 |
|
116 |
1,131 |
905 |
1,415 |
|
118 |
1,186 |
948 |
1,483 |
|
120 |
1,242 |
993 |
1,553 |
|
122 |
1,299 |
1,039 |
1,625 |
|
124 |
1,358 |
1,086 |
1,699 |
|
126 |
1,419 |
1,135 |
1,775 |
|
128 |
1,482 |
1,185 |
1,853 |
|
130 |
1,546 |
1,236 |
1,934 |
|
132 |
1,612 |
1,289 |
2,016 |
|
134 |
1,680 |
1,344 |
2,101 |
|
136 |
1,750 |
1,399 |
2,188 |
|
138 |
1,821 |
1,457 |
2,278 |
|
140 |
1,895 |
1,515 |
2,369 |
|
142 |
1,970 |
1,575 |
2,463 |
|
144 |
2,047 |
1,637 |
2,560 |
|
146 |
2,126 |
1,700 |
2,658 |
|
148 |
2,207 |
1,765 |
2,759 |
|
150 |
2,289 |
1,831 |
2,863 |
|
152 |
2,374 |
1,898 |
2,969 |
|
154 |
2,461 |
1,968 |
3,077 |
|
156 |
2,549 |
2,039 |
3,188 |
|
158 |
2,640 |
2,111 |
3,301 |
|
160 |
2,732 |
2,185 |
3,417 |
|
162 |
2,827 |
2,261 |
3,535 |
|
164 |
2,924 |
2,338 |
3,656 |
|
166 |
3,023 |
2,417 |
3,780 |
|
168 |
3,124 |
2,498 |
3,906 |
|
170 |
3,227 |
2,580 |
4,035 |
|
172 |
3,332 |
2,664 |
4,166 |
|
174 |
3,439 |
2,750 |
4,300 |
|
176 |
3,549 |
2,838 |
4,437 |
|
178 |
3,660 |
2,927 |
4,577 |
|
180 |
3,774 |
3,018 |
4,719 |
|
Formula: log y = a + b · log x; y = FEV1 (mL); x = Body height (cm); log FEV1 (mL) = -2.6056 + 2.7413 · log height (cm); SD log y ·x = ±0.0483; n = 51; r = +0.93. |
TABLE C-9 -- Peak expiratory flow rate (PEFR) in relation to body height in boys and girls
|
Height (cm) |
PEFR (L/sec) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
2.85 |
2.05 |
3.96 |
|
116 |
2.91 |
2.09 |
4.04 |
|
118 |
3.03 |
2.18 |
4.21 |
|
120 |
3.15 |
2.26 |
4.38 |
|
122 |
3.27 |
2.35 |
4.55 |
|
124 |
3.40 |
2.44 |
4.73 |
|
126 |
3.53 |
2.54 |
4.91 |
|
128 |
3.66 |
2.63 |
5.09 |
|
130 |
3.80 |
2.73 |
5.28 |
|
132 |
3.94 |
2.83 |
5.47 |
|
134 |
4.08 |
2.93 |
5.67 |
|
136 |
4.22 |
3.04 |
5.87 |
|
138 |
4.37 |
3.14 |
6.08 |
|
140 |
4.52 |
3.25 |
6.28 |
|
142 |
4.67 |
3.36 |
6.50 |
|
144 |
4.83 |
3.47 |
6.71 |
|
146 |
4.99 |
3.59 |
6.93 |
|
148 |
5.15 |
3.70 |
7.16 |
|
150 |
5.31 |
3.82 |
7.39 |
|
152 |
5.48 |
3.94 |
7.62 |
|
154 |
5.65 |
4.07 |
7.86 |
|
156 |
5.83 |
4.19 |
8.10 |
|
158 |
6.00 |
4.32 |
8.34 |
|
160 |
6.18 |
4.45 |
8.59 |
|
162 |
6.36 |
4.58 |
8.85 |
|
164 |
6.55 |
4.71 |
9.11 |
|
166 |
6.74 |
4.85 |
9.37 |
|
168 |
6.93 |
4.99 |
9.64 |
|
170 |
7.13 |
5.13 |
9.91 |
|
172 |
7.32 |
5.27 |
10.18 |
|
174 |
7.53 |
5.41 |
10.46 |
|
176 |
7.73 |
5.56 |
10.75 |
|
178 |
7.94 |
5.71 |
11.03 |
|
180 |
8.15 |
5.86 |
11.33 |
|
Formula: log y = a + b · log x; y = PEFR (L/sec); x = Body height (cm); log PEFR (L/sec) = -4.3722 + 2.3422 · log height (cm); SD log y ·x = ±0.0717; n = 76; r = +0.83. |
TABLE C-10 -- Maximum mid-expiratory flow (MMEF25-75% VC) in relation to body height in boys and girls
|
Height (cm) |
MMEF25–75% VC (L/sec) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
1.57 |
1.11 |
2.20 |
|
116 |
1.60 |
1.13 |
2.25 |
|
118 |
1.66 |
1.18 |
2.34 |
|
120 |
1.73 |
1.23 |
2.43 |
|
122 |
1.80 |
1.28 |
2.53 |
|
124 |
1.87 |
1.33 |
2.63 |
|
126 |
1.94 |
1.38 |
2.73 |
|
128 |
2.02 |
1.43 |
2.84 |
|
130 |
2.09 |
1.49 |
2.94 |
|
132 |
2.17 |
1.54 |
3.05 |
|
134 |
2.25 |
1.60 |
3.16 |
|
136 |
2.33 |
1.65 |
3.27 |
|
138 |
2.41 |
1.71 |
3.39 |
|
140 |
2.49 |
1.77 |
3.50 |
|
142 |
2.58 |
1.83 |
3.62 |
|
144 |
2.66 |
1.89 |
3.75 |
|
146 |
2.75 |
1.95 |
3.87 |
|
148 |
2.84 |
2.02 |
4.00 |
|
150 |
2.93 |
2.08 |
4.12 |
|
152 |
3.02 |
2.15 |
4.26 |
|
154 |
3.12 |
2.22 |
4.39 |
|
156 |
3.22 |
2.29 |
4.52 |
|
158 |
3.31 |
2.36 |
4.66 |
|
160 |
3.41 |
2.43 |
4.80 |
|
162 |
3.52 |
2.50 |
4.95 |
|
164 |
3.62 |
2.57 |
5.09 |
|
166 |
3.72 |
2.65 |
5.24 |
|
168 |
3.83 |
2.72 |
5.39 |
|
170 |
3.94 |
2.80 |
5.54 |
|
172 |
4.05 |
2.88 |
5.70 |
|
174 |
4.16 |
2.96 |
5.86 |
|
176 |
4.28 |
3.04 |
6.02 |
|
178 |
4.39 |
3.12 |
6.18 |
|
180 |
4.51 |
3.21 |
6.34 |
|
Formula: log y = a + b · log x; y = (MMEF25-75% VC) (L/sec); x = Body height (cm); log (MMEF25-75% VC) (L/sec) = -4.6651 + 2.3588 · log height (cm); SD log y ·x = ±0.0746; n = 108; r = +0.78. |
TABLE C-11 -- Maximum expiratory flow rate at 50% of vital capacity(V max50% VC, MEF50% VC) in relation to body height in boys and girls
|
Height (cm) |
Vmax50% VC (L/sec) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
1.86 |
1.35 |
2.55 |
|
116 |
1.89 |
1.38 |
2.59 |
|
118 |
1.96 |
1.43 |
2.69 |
|
120 |
2.04 |
1.48 |
2.79 |
|
122 |
2.11 |
1.54 |
2.90 |
|
124 |
2.19 |
1.59 |
3.00 |
|
126 |
2.27 |
1.65 |
3.11 |
|
128 |
2.34 |
1.71 |
3.21 |
|
130 |
2.43 |
1.77 |
3.33 |
|
132 |
2.51 |
1.83 |
3.44 |
|
134 |
2.59 |
1.89 |
3.55 |
|
136 |
2.68 |
1.95 |
3.67 |
|
138 |
2.76 |
2.01 |
3.79 |
|
140 |
2.85 |
2.08 |
3.91 |
|
142 |
2.94 |
2.14 |
4.03 |
|
144 |
3.03 |
2.21 |
4.16 |
|
146 |
3.12 |
2.28 |
4.28 |
|
148 |
3.22 |
2.35 |
4.41 |
|
150 |
3.31 |
2.42 |
4.54 |
|
152 |
3.41 |
2.49 |
4.68 |
|
154 |
3.51 |
2.56 |
4.81 |
|
156 |
3.61 |
2.63 |
4.95 |
|
158 |
3.71 |
2.71 |
5.09 |
|
160 |
3.81 |
2.78 |
5.23 |
|
162 |
3.92 |
2.86 |
5.37 |
|
164 |
4.02 |
2.94 |
5.52 |
|
166 |
4.13 |
3.01 |
5.67 |
|
168 |
4.24 |
3.09 |
5.82 |
|
170 |
4.35 |
3.17 |
5.97 |
|
172 |
4.47 |
3.26 |
6.12 |
|
174 |
4.58 |
3.34 |
6.28 |
|
176 |
4.69 |
3.42 |
6.44 |
|
178 |
4.81 |
3.51 |
6.60 |
|
180 |
4.93 |
3.60 |
6.76 |
|
Formula: log y = a + b · log x; y =V max50% VC (L/sec); x = Body height (cm); log V max50% VC (L/sec) = -4.2168 + 2.1771 · log height (cm); SD log y ·x = ±0.0689; n = 101; r = +0.79. |
TABLE C-12 -- Maximum expiratory flow rate at 25% of vital capacity (V max25% VC, MEF25% VC) in relation to body height in boys and girls
|
Height (cm) |
Vmax25% VC liters/s |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
0.94 |
0.63 |
1.41 |
|
116 |
0.96 |
0.64 |
1.44 |
|
118 |
1.00 |
0.67 |
1.49 |
|
120 |
1.04 |
0.69 |
1.55 |
|
122 |
1.08 |
0.72 |
1.61 |
|
124 |
1.11 |
0.75 |
1.66 |
|
126 |
1.16 |
0.77 |
1.73 |
|
128 |
1.20 |
0.80 |
1.79 |
|
130 |
1.24 |
0.83 |
1.85 |
|
132 |
1.28 |
0.86 |
1.91 |
|
134 |
1.32 |
0.89 |
1.98 |
|
136 |
1.37 |
0.92 |
2.04 |
|
138 |
1.41 |
0.95 |
2.11 |
|
140 |
1.46 |
0.98 |
2.18 |
|
142 |
1.51 |
1.01 |
2.25 |
|
144 |
1.55 |
1.04 |
2.32 |
|
146 |
1.60 |
1.07 |
2.39 |
|
148 |
1.65 |
1.11 |
2.46 |
|
150 |
1.70 |
1.14 |
2.54 |
|
152 |
1.75 |
1.17 |
2.61 |
|
154 |
1.80 |
1.21 |
2.69 |
|
156 |
1.86 |
1.24 |
2.77 |
|
158 |
1.91 |
1.28 |
2.85 |
|
160 |
1.96 |
1.31 |
2.93 |
|
162 |
2.02 |
1.35 |
3.01 |
|
164 |
2.07 |
1.39 |
3.09 |
|
166 |
2.13 |
1.43 |
3.18 |
|
168 |
2.19 |
1.47 |
3.26 |
|
170 |
2.25 |
1.50 |
3.35 |
|
172 |
2.30 |
1.54 |
3.44 |
|
174 |
2.36 |
1.58 |
3.53 |
|
176 |
2.42 |
1.62 |
3.62 |
|
178 |
2.49 |
1.67 |
3.71 |
|
180 |
2.55 |
1.71 |
3.80 |
|
Formula: log y = a + b · log x; y =Vmax25% VC (L/sec); x = Body height (cm); log Vmax25% VC (L/sec) = -4.5808 + 2.2116 · log height (cm); SD log y ·x = ±0.0874; n = 101; r = +0.76. |
TABLE C-13 -- Tidal volume (VT) in relation to body height in boys and girls
|
Height (cm) |
VT (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
279 |
185 |
421 |
|
116 |
283 |
188 |
428 |
|
118 |
293 |
194 |
442 |
|
120 |
302 |
200 |
456 |
|
122 |
311 |
206 |
470 |
|
124 |
321 |
213 |
484 |
|
126 |
331 |
219 |
499 |
|
128 |
341 |
226 |
514 |
|
130 |
351 |
232 |
529 |
|
132 |
361 |
239 |
544 |
|
134 |
371 |
246 |
560 |
|
136 |
381 |
253 |
575 |
|
138 |
392 |
260 |
591 |
|
140 |
403 |
267 |
607 |
|
142 |
413 |
274 |
624 |
|
144 |
424 |
281 |
640 |
|
146 |
435 |
289 |
657 |
|
148 |
447 |
296 |
674 |
|
150 |
458 |
303 |
691 |
|
152 |
469 |
311 |
708 |
|
154 |
481 |
319 |
726 |
|
156 |
493 |
327 |
743 |
|
158 |
505 |
334 |
761 |
|
160 |
517 |
342 |
779 |
|
162 |
529 |
350 |
798 |
|
164 |
541 |
358 |
816 |
|
166 |
553 |
367 |
835 |
|
168 |
566 |
375 |
854 |
|
170 |
578 |
383 |
873 |
|
172 |
591 |
392 |
892 |
|
174 |
604 |
400 |
911 |
|
176 |
617 |
409 |
931 |
|
178 |
630 |
418 |
951 |
|
180 |
643 |
426 |
971 |
|
Formula: log y = a + b · log x; y = VT (mL); x = Body height (cm); log VT (mL) = -1.3956 + 1.8643 · log height (cm); SD log y ·x = ±0.0903; n = 170; r = +0.65. |
TABLE C-14 -- Tidal volume (VT) in relation to age in boys and girls
|
Age (yr) |
VT (mL) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
6 |
278 |
178 |
432 |
|
7 |
313 |
201 |
486 |
|
8 |
347 |
223 |
539 |
|
9 |
380 |
244 |
590 |
|
10 |
412 |
265 |
640 |
|
11 |
443 |
285 |
689 |
|
12 |
474 |
305 |
737 |
|
13 |
504 |
324 |
784 |
|
14 |
534 |
343 |
830 |
|
15 |
563 |
362 |
875 |
|
16 |
592 |
381 |
920 |
|
17 |
620 |
399 |
964 |
|
Formula: log y = a + b · log x; y = VT (mL); x = Age (yr); log VT (mL) = 1.8438 + 0.7713 · log age (yr); SD log y ·x = ±0.0969; n = 170; r = +0.57. |
TABLE C-15 -- Minute ventilation (MV) in relation to body height in boys and girls
|
Height (cm) |
MV (mL/min) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
115 |
5,872 |
4,454 |
7,741 |
|
116 |
5,958 |
4,520 |
7,855 |
|
118 |
6,132 |
4,652 |
8,084 |
|
120 |
6,308 |
4,785 |
8,316 |
|
122 |
6,486 |
4,920 |
8,550 |
|
124 |
6,666 |
5,056 |
8,787 |
|
126 |
6,847 |
5,194 |
9,027 |
|
128 |
7,031 |
5,333 |
9,269 |
|
130 |
7,217 |
5,474 |
9,514 |
|
132 |
7,405 |
5,617 |
9,761 |
|
134 |
7,594 |
5,761 |
10,011 |
|
136 |
7,786 |
5,906 |
10,264 |
|
138 |
7,979 |
6,053 |
10,519 |
|
140 |
8,175 |
6,201 |
10,777 |
|
142 |
8,372 |
6,351 |
11,037 |
|
144 |
8,571 |
6,502 |
11,300 |
|
146 |
8,772 |
6,654 |
11,565 |
|
148 |
8,976 |
6,808 |
11,832 |
|
150 |
9,180 |
6,964 |
12,103 |
|
152 |
9,387 |
7,121 |
12,375 |
|
154 |
9,596 |
7,279 |
12,650 |
|
156 |
9,806 |
7,439 |
12,928 |
|
158 |
10,019 |
7,600 |
13,208 |
|
160 |
10,233 |
7,762 |
13,490 |
|
162 |
10,449 |
7,926 |
13,775 |
|
164 |
10,667 |
8,091 |
14,062 |
|
166 |
10,886 |
8,258 |
14,352 |
|
168 |
11,108 |
8,426 |
14,643 |
|
170 |
11,331 |
8,595 |
14,938 |
|
172 |
11,556 |
8,766 |
15,235 |
|
174 |
11,783 |
8,938 |
15,534 |
|
176 |
12,012 |
9,112 |
15,835 |
|
178 |
12,242 |
9,286 |
16,139 |
|
180 |
12,474 |
9,463 |
16,445 |
|
Formula: log y = a + b · log x; y = MV (mL/min); x = Body height (cm); log MV (mL/min) = -0.3035 + 1.6815 · log height (cm); SD log y ·x = ±0.0607; n = 170; r = +0.75. |
TABLE C-16 -- Minute ventilation (MV) in relation to age in boys and girls
|
Age (yr) |
MV (mL/min) |
||
|
Mean |
Lower Limits |
Upper Limits |
|
|
6 |
5,864 |
4,287 |
8,022 |
|
7 |
6,524 |
4,769 |
8,924 |
|
8 |
7,154 |
5,230 |
9,787 |
|
9 |
7,761 |
5,674 |
10,616 |
|
10 |
8,347 |
6,102 |
11,418 |
|
11 |
8,916 |
6,518 |
12,196 |
|
12 |
9,468 |
6,922 |
12,951 |
|
13 |
10,007 |
7,315 |
13,688 |
|
14 |
10,532 |
7,700 |
14,407 |
|
15 |
11,047 |
8,076 |
15,111 |
|
16 |
11,551 |
8,444 |
15,800 |
|
17 |
12,045 |
8,805 |
16,476 |
|
Formula: log y = a + b · log x; y = MV (mL/min); x = Age (yr); log MV (mL/min) = 3.2305 + 0.6910 · log age (yr); SD log y ·x = ±0.0689; n = 170; r = +0.66. |
Copyright © 2008 Elsevier Inc. All rights reserved. - www.mdconsult.com
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.
Copyright © 2005 Mosby, An Imprint of Elsevier
Appendix D : Index of Syndromes and Their Pediatric Anesthetic Implications
Franklyn P. Cladis
|
Name |
Description |
Anesthetic Implications |
References |
|
A |
|||
|
Aarskog syndrome |
Mutation in faciodigitogenital gene, growth retardation, rare congenital heart disease, mental retardation, hypermobility of cervical spine with possible hypoplasia, cryptorchidism |
Care with neck manipulation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Aase syndrome |
May be variant of Diamond Blackfan syndrome, anemia, radial hypoplasia |
Check preoperative hematocrit, radial anomalies make arterial catheter insertion more difficult |
Baum and O'Flaherty, 1999 |
|
Abetalipoproteinemia |
Absence of apolipoprotein B, neuropathy with sensory and motor loss, malabsorption of lipids |
May be deficient in fat-soluble vitamins with elevation in prothrombin time, succinylcholine is contraindicated with demyelination |
Baum and O'Flaherty, 1999 |
|
Achondrogenesis |
Types 1 and 2 result in severe defect in the development of bone and cartilage, micrognathia, very short stature, large cranium, micromelia, usually lethal |
May be difficult airway, difficult intravenous access, care with positioning |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Achondroplasia |
Premature fusion of the bones, macrocephaly, sleep apnea, chest wall deformities—scoliosis and small rib cage, brainstem compression, spine stenosis and fusion, small stature, obesity, normal intelligence |
May be difficult airway, may have cervical spine compression with positioning, altered lung mechanics secondary to chest wall deformity, regional anesthesia has been performed |
Kalla and others, 1986 ;Berkowitz and others, 1990 ;Baum and O'Flaherty, 1999 |
|
Adrenoleukodystrophy |
Adrenal cortical deficiency and CNS demyelination, secondary to accumulation of very long chain fatty acids, visual changes, retinopathy, hypotonia, ataxia, apraxia, neuropathy, spastic paraparesis, seizures, GERD |
Concern regarding succinylcholine with demyelination and muscle atrophy, risk of aspiration, inhibitors of dopamine may exacerbate movement disorder, may require stress-dose steroids |
Tobias, 1992 ; Nishina and others, 1993 ; Baum and O'Flaherty, 1999 |
|
Aicardia syndrome |
Seen only in females, agenesis of corpus callosum, hypotonia, infantile spasm and seizures, kyphoscoliosis |
Risk for aspiration, chronic antiseizure medication |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Alagille syndrome |
Congestive heart disease, biliary hypoplasia or atresia, HTN, vitamin K deficiency, renal dysplasia or stenosis |
May have cirrhosis and portal HTN, increased prothrombin time, preoperative cardiac evaluation, check renal function |
Choudry and others, 1998 ;Baum and O'Flaherty, 1999 |
|
Albright osteodystrophy |
See Pseudohypoparathyroidism |
||
|
Alport syndrome |
Sensorineural hearing loss, renal failure, can have myopathy or peripheral neuropathy |
Check renal function preoperatively, possible hyperkalemia with myopathy or neuropathy and succinylcholine |
Baum and O'Flaherty, 1999 |
|
Alström syndrome |
Vision and hearing loss, cardiomyopathy uncommon, progressive renal dysfunction, obesity |
Check renal function preoperatively, may need cardiac evaluation preoperatively |
Baum and O'Flaherty, 1999 |
|
Angelman syndrome |
Severe mental retardation, laughing, ataxia, seizures, “happy puppet syndrome” |
Chronic antiseizure medications |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Antley-Bixler syndrome |
Craniosynostosis, frontal bossing, choanal stenosis or atresia with severe upper airway obstruction, proptosis |
Choanal atresia may require early airway intervention (tracheostomy), may preclude the use of nasal tubes, eye protection for proptosis |
LeBard and Thiemann, 1998 ;Baum and O'Flaherty, 1999 |
|
Apert syndrome |
Craniosynostosis, midface hypoplasia, may have tracheal stenosis, congenital heart disease, mental retardation, increased ICP, syndactyly, renal anomalies, fusion of cervical vertebrae |
May be difficult intubation due to midface hypoplasia, cervical fusion and tracheal stenosis, preoperative cardiac evaluation, caution with premedication if ICP elevated, intravenous access may be difficult |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Argininosuccinic acid lyase and argininosuccinic acid synthetase deficiency |
Urea cycle defect with hyperammonemia, mental retardation, seizures |
Need high carbohydrate intake perioperatively with low protein intake, protein load (blood) may cause acute metabolic encephalopathy |
Baum and O'Flaherty, 1999 |
|
Arthrogryposis multiplex |
Multiple congenital contractures, small mandible, short neck, may be associated myopathy or neuropathy |
May be difficult intubation, difficult intravenous access, no known association with malignant hyperthermia |
Baum and O'Flaherty, 1999 |
|
Asplenia |
Duplication of right-sided structures, two right lungs, two right atria, usually a form of anomalous venous return, two sinoatrial nodes, absent spleen, may have malrotation |
Preoperative cardiac evaluation, risk of infection from encapsulated organisms |
Baum and O'Flaherty, 1999 |
|
Ataxia-telangiectasia syndrome |
Oculocutaneous telangiectalas, cerebellar ataxia, peripheral nerve degeneration, immunodeficiency, recurrent lung infections, predisposition to malignancies |
Aseptic technique with invasive procedures, potential hyperkalemia with succinycholine in setting of neuropathy, may have glucose intolerance |
Baum and O'Flaherty, 1999 |
|
B |
|||
|
Baller-Gerold syndrome |
Craniosynostosis, micrognathia, radial aplasia, congenital heart disease common, mental retardation, renal anomalies |
Micrognathia may make intubation difficult, preoperative cardiac evalu- ation, check renal function, radial artery catheterization may be difficult |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Bartter syndrome |
Abnormalities in Na+, K+, and Cl- transport with hypokalemia, hypochloremia, metabolic alkalosis, mental retardation, muscle weakness |
Check electrolytes (Na+, K+, and Cl-) preoperatively, may have ileus secondary to hypokalemia, may have decreased muscle tone |
Higa and others, 1993 ;Kannan and others, 1995 ;Baum and O'Flaherty, 1999 |
|
Becker muscular dystrophy |
Abnormality in size or amount of dystrophin, later age of onset and milder course, respiratory muscle weakness, cardiomyopathy |
Hyperkalemia with succinylcholine, possible risk of malignant hyperthermia, risk of aspiration, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Russell and Hirsch, 1994 |
|
Beckwith-Wiedemann syndrome |
Macroglossia, macrosomia, visceromegaly, increased risk of intra-abdominal tumors, hypoglycemia, neonatal polycythemia, omphalocele, may have congenital heart disease |
May have significant upper airway obstruction, preoperative cardiac evaluation, monitor blood glucose |
Gurkowski and Rasch, 1989 ;Suan and others, 1996 ; Baum and O'Flaherty, 1999 ; Butler and others, 2000 |
|
Bernard-Soulier syndrome |
Disorder of platelet function |
May require platelet transfusion |
Baum and O'Flaherty, 1999 |
|
Blackfan-Diamond syndrome |
Congenital red cell hypoplasia, steroid therapy common |
Check preoperative hemoglobin, may require transfusions, may need stress-dose steroids |
Baum and O'Flaherty, 1999 |
|
Branchio-oculofacial syndrome |
Defects of the branchial arch involving the eye and face, pseudocleft lip, renal anomalies |
No reports of difficult airway, check renal function preoperatively |
Baum and O'Flaherty, 1999 |
|
C |
|||
|
Camptomelic dysplasia |
Short stature, micrognathia, short neck, kyphoscoliosis |
May be difficult intubation, may have unstable cervical spine |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Carpenter syndrome |
Craniosynostosis, hypoplastic mandible, congenital heart disease common, may have increased ICP, mental retardation, obesity |
May be difficult intubation, preoperative cardiac evaluation, may need to manage elevated ICP |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Cat-eye syndrome |
Colobomas of iris, may have choanal atresia, congenital heart disease, anal atresia, renal abnormalities, radial anomalies |
Check renal function, preoperative cardiac evaluation, radial artery catheter may be difficult |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Catch-22 syndrome |
See DiGeorge syndrome |
||
|
Catel-Manzke syndrome |
Severe micrognathia, short neck, congenital heart disease common, |
May have significant upper airway obstruction, ventilation and intubation may be very difficult, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Central core disease |
Congenital myopathy with defect in ryanidine receptor |
Risk of malignant hyperthermia |
Baum and O'Flaherty, 1999 ;Frank and others, 1980 |
|
Cerebrocostomandibular syndrome |
Severe micrognathia, gap in ribs with small thoracic cage, congenital heart disease |
Intubation may be very difficult, severe restrictive lung disease, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 |
|
Cerebrooculofacioskeletal syndrome |
Degeneration of brain and spinal cord, hypotonia, hyporeflexia, micrognathia |
May have hyperkalemia with succinylcholine |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Charcot-Marie-Tooth syndrome |
Hereditary peripheral neuropathy, respiratory insufficiency later in life, muscle atrophy |
May have hyperkalemia with succinylcholine, may have perioperative respiratory complications |
Anotgnini, 1992 ; Greenberg and Parker, 1992 ; Baraka, 1997 ; Baum and O'Flaherty, 1999 |
|
CHARGE association |
Colobomas of the eye, heart disease (tetralogy of Fallot), atresia of choanae, retarded growth, genital anomalies (hypogonadism), ear anomalies (deafness) |
May be difficult intubation secondary to micrognathia, preoperative cardiac evaluation, respiratory distress from choanal atresia |
Stack and Wyse, 1991 ; Baum and O'Flaherty, 1999 ; Butler and others, 2000 |
|
Chédiak-Higashi syndrome |
Defect in granular cells (neutrophils, monocytes, lymphocytes), immunodeficient, partial albinism, peripheral neuropathy, may have platelet abnormalities, steroid therapy |
Risk for bacterial infections, use aseptic technique, may have hyperkalemia with succinylcholine, may require platelet transfusion, consider stress-dose steroids |
Baum and O'Flaherty, 1999 |
|
CHILD syndrome |
Congenital hemidysplasia, ichthyosiform erythroderma, limbdefects, congenital heart disease, hypomelia, renal agenesis |
Intravenous access may be difficult, preoperative cardiac evaluation, check renal function preoperatively |
Baum and O'Flaherty, 1999 |
|
Chotzen syndrome |
See Saethre-Chotzen syndrome |
||
|
Cockayne syndrome |
UV light- induced DNA damage, small mandible, small trachea, precocious coronary artery disease and HTN, ataxia, muscle atrophy, vertebral abnormalities, kyphoscoliosis |
Difficult intubation has been reported, may need smaller endotracheal tube, preoperative cardiac evaluation for ischemia, may need to treat HTN, check renal function preoperatively, succinylcholine may cause hyperkalemia with muscle atrophy |
Cook, 1982 ; Woolridge and others, 1996 ; Sasaki and others, 1997 ; Baum and O'Flaherty, 1999 |
|
Coffin-Siris syndrome |
Microcephaly, congenital heart disease, mental retardation, hypoplastic digits, renal anomalies, aggressive behavior |
Preoperative cardiac evaluation, check renal function preoperatively |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Cohen syndrome |
Micrognathia, mitral valve prolapse, mental retardation, hypotonia, muscle weakness, obesity |
May be difficult intubation, may be difficult ventilation secondary to obesity, subacute bacterial, endocarditis prophylaxis, perioperative complications secondary to obesity and muscle weakness |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Congenital adrenal hyperplasia |
Defect in the synthesis of cortisol with overproduction of an androgen cortisol precursor, virilization, clinical features depend on enzyme deficiency, may have salt wasting, may have hypoglycemia, may have hypokalemia and HTN, defect in aldosterone synthesis common |
Check Na+, K+, and glucose preoperatively, stress-dose steroids, salt losing can be treated with salt-containing intravenous fluids and mineralocorticoid |
White and others, 1987 ;Baum and O'Flaherty, 1999 |
|
Cornelia de Lange syndrome |
Micrognathia and short neck, occasional congenital heart disease, severe mental retardation, seizures, apnea, GERD, micromelia |
May be difficult intubation, risk of perioperative respiratory complications from GERD and apnea, may be difficult intravenous access |
Munoz Corsini and others, 1998 ; Baum and O'Flaherty, 1999 |
|
Costello syndrome |
Short neck, oral and nasal papillomas, hypertrophic cardiomyopathy, arrhythmias, congenital heart disease, mental retardation, swallowing difficulty and GERD |
Airway papillomas may make intubation difficult, preoperative cardiac evaluation, perioperative respiratory complications secondary to swallowing difficulty and GERD |
Dearlove and Harper, 1997 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Cri du chat syndrome |
Partial deletion of chromosome 5p, high pitched cry, may have micrognathia and short neck, long epiglottis, laryngeal deformity, severe mental retardation, recurrent aspiration, congenital heart disease common |
Difficult intubation described, preoperative cardiac evaluation, risk of aspiration at baseline, airway obstruction secondary to hypotonia |
Yamashita and others, 1985 ;Brislin and others, 1995 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Crouzon's syndrome |
Craniosynostosis, proptosis, hypoplastic maxilla, rarely elevated ICP |
Possible difficult intubation, eye care with proptosis |
Payne and Cranston, 1995 ;Baum and O'Flaherty, 1999 |
|
Cutis laxa |
Disorder of elastin synthesis, emphysema, aortic dilation, coronary artery disease, pulmonary HTN, pendulous skin |
Preoperative cardiac evaluation, intravenous access may be difficult |
Baum and O'Flaherty, 1999 |
|
Cystic fibrosis |
See Chapter 32 |
||
|
D |
|||
|
Dejerine-Sottas syndrome |
Motor and sensory neuropathy, distal muscle atrophy and weakness, may have autonomic abnormalities |
May develop hyperkalemia with succinylcholine, autonomic dysfunction may cause thermal lability, no known association with malignant hyperthermia |
Baum and O'Flaherty, 1999 |
|
Diastrophic dysplasia |
Short stature, can have subluxation of C2-3, micrognathia, laryngotracheal stenosis, limited joint mobility |
May be difficult intubation, may require smaller-than-expected endotracheal tube, cervical subluxation possible, care with positioning |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
DiGeorge syndrome (CATCH-22 syndrome) |
Microdeletion on chromosome 22, abnormalities of thymus, parathyroids, and great vessels, choanal atresia, may have micrognathia and short trachea, congenital heart disease, hypocalcemia and seizures in neonates, cellular immunodeficiency |
May be difficult intubation, endobronchial intubation more likely with short trachea, preoperative cardiac evaluation, avoid nasal tubes with choanal atresia, monitor calcium, irradiate blood products |
Flashburg and others, 1983 ;Wells and others, 1989 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Down syndrome (trisomy 21 syndrome) |
Trisomy 21, macroglossia, pharyngeal hypotonia, smaller trachea, obstructive sleep apnea, recurrent lung infections, congenital heart disease common, atlantoaxial instability, duodenal atresia, congenital hypothyroidism |
Airway obstruction may occur intraoperatively and postoperatively, reports of subluxation after intubation, may require neck films if symptomatic, may need smaller endotracheal tube, preoperative cardiac evaluation, bradycardia common after inhalation induction |
Williams and others, 1987 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 ;Williams and others, 1987 |
|
Dubowitz syndrome |
Small facies, mental deficiency, growth deficiency, rare congenital heart disease, micrognathia |
May be difficult airway, preoperative cardiac evaluation, may have behavioral disturbance |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Duchenne muscular dystrophy |
X-linked muscular dystrophy, lack of dystrophin in muscle, respiratory muscle weakness, restrictive lung disease, cardiomyopathy, heart block or arrhythmias, scoliosis, delayed gastric emptying, obesity in second decade |
Preoperative cardiac evaluation, postoperative mechanical ventilation possible, hyperkalemia with succinylcholine, unclear association with malignant hyperthermia, possible increased risk of aspiration secondary to respiratory muscle weakness and delayed gastric emptying |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Dutch-Kentucky syndrome (Hecht Beals syndrome) |
Limited mouth opening, mitral valve prolapse, flexion deformities |
Difficult intubation reported requiring fiberoptic technique, preoperative cardiac evaluation |
Browder and others, 1986 ;Geva and others, 1997 ;Baum and O'Flaherty, 1999 |
|
E |
|||
|
Eagle-Barrett syndrome |
See prune belly syndrome |
||
|
Edwards syndrome |
See Trisomy 18 |
||
|
Ehlers-Danlos syndrome |
Defect in gene encoding collagen, 10 types, possible tracheal dilation, lung cysts, mitral valve prolapse, aortic root dilation, congenital heart disease, coronary disease, joint laxity, poor wound healing, vascular abnormalities (aneurysm) with poor integrity, platelet abnormalities |
May bleed significantly from vascular aneurysm and bleeding diathesis, preoperative cardiac evaluation for congenital defects, premature ischemic heart disease, and conduction abnormalities, risk for pneumothorax, may be difficult intravenous access |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Ellis-van Creveld syndrome |
Dwarfism, micrognathia, may have short trachea, congenital heart disease, restrictive lung disease |
May be difficult intubation, endobronchial intubation more likely with short trachea, preoperative cardiac evaluation |
Wells and others, 1989 ;Baum and O'Flaherty, 1999 |
|
Epidermolysis bullosa |
Abnormality of dermis and mucous membranes, degree of skin separation, bullae formation and scarring depends on type, may limit mouth opening, airway bleeding and obstruction possible |
Any adhesive may cause skin sloughing and bullae formation; use nonadhesive technique to secure endotracheal tube, monitors, and intravenous catheters; protect skin from blood pressure cuff; pad pressure points; cover facemask with Xeroform or lubricate, may be difficult intubation; eye protection with lubricant; may be at risk for aspiration from esophageal disease |
Baum and O'Flaherty, 1999 |
|
Escobar syndrome |
See multiple pterygium syndrome |
||
|
F |
|||
|
Fabry disease |
Deficiency in lysosomal alphagalactosidase A, may have temporomandibular joint involvement, may have ischemic heart disease, HTN, valvular disease, cerebrovascular disease, extremity pain, lymphedema, renal failure, hypohidrosis, angiokeratomas |
May be difficult intubation from limited mouth opening, preoperative cardiac evaluation for ischemic heart disease and HTN, check renal function preoperatively, consider avoiding anticholinergics with hyperhidrosis |
Baum and O'Flaherty, 1999 |
|
Facioauriculovertebral syndrome |
See Goldenhar syndrome |
||
|
Fascioscapulohumeral muscular dystrophy |
Weakness of facial and ocular muscles, no cardiac involvement, neck and shoulder muscle weakness |
Hyperkalemia with succinylcholine, no reports of malignant hyperthermia |
Dresner and Ali, 1989 ; Baum and O'Flaherty, 1999 |
|
Familial dysautonomia |
See Riley-Day syndrome |
||
|
Familial periodic paralysis |
See Chapter 32 |
||
|
Fanconi syndrome |
Proximal renal tubular dysfunction (renal tubular acidosis), renal loss of amino acids, phosphate, glucose, bicarbonate, and potassium, may have muscle weakness from hypokalemia, may develop chronic renal failure |
Check electrolytes preoperatively (potassium, phosphate, chloride, and bicarbonate), check renal function preoperatively, check acid-base status preoperatively |
Joel and Rosales, 1981 ;Baum and O'Flaherty, 1999 |
|
Farber disease |
Deficiency of lysosomal enzyme ceramidase, ceramide deposition and granuloma formation may be disseminated, oral and laryngeal granulomas, pulmonary and cardiac granulomas, mental retardation, peripheral neuropathy with muscle atrophy |
May be difficult airway secondary to oral granulomas, preoperative cardiac evaluation for valvular granulomas, succinylcholine can cause hyperkalemia |
Baum and O'Flaherty, 1999 |
|
Femoral hypoplasia-unusual facies syndrome |
Craniosynostosis, cleft palate, micrognathia, may have congenital heart disease, femoral hypoplasia, may have renal anomalies |
May be difficult intubation from micrognathia, preoperative cardiac evaluation, check renal function preoperatively |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Fetal alcohol syndrome |
Secondary to in-utero exposure to alcohol, micrognathia, short neck, congenital heart disease, mental retardation, growth deficiency |
May be difficult intubation, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 |
|
Fetal hydantoin (Dilantin) syndrome |
Secondary to in-utero exposure to Dilantin, microcephaly, midface hypoplasia, webbed neck, congenital heart disease, may have mental retardation, growth delay, hirsutism |
May be a difficult intubation, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Fetal rubella syndrome |
Secondary to maternal rubella infection during first trimester, microcephaly, hearing loss, congenital heart disease, mental retardation, anemia and thrombocytopenia in the neonate, cataracts and glaucoma |
Preoperative cardiac evaluation, check preoperative hemoglobin and platelet count during neonatal period |
Baum and O'Flaherty, 1999 |
|
Fetal valproate syndrome |
Secondary to in utero exposure to valproic acid, congenital heart disease, meningomyelocele, radial anomalies |
Preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Fetal warfarin syndrome |
Secondary to in utero exposure to warfarin, microcephaly, congenital heart disease, severe mental retardation, seizures |
May have upper airway obstruction, preoperative cardiac evaluation, chronic antiseizure medication |
Baum and O'Flaherty, 1999 |
|
Fibrodysplasia ossificans progressiva syndrome |
Progressive ossification of muscles, joints, and subcutaneous tissue, affects neck, spine, shoulders, and jaw, may develop restrictive lung disease, cardiac conduction abnormalities, steroid therapy |
May be very difficult intubation due to limited cervical motion and mouth opening, preoperative cardiac evaluation for conduction defects and supraventricular tachycardia, may need stress-dose steroids |
Lininger and others, 1989 ;Newton and others, 1990 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Fragile X syndrome |
Defect in X chromosome, acromegalic facies, large ears, prognathism, mitral valve prolapse, mental retardation, behavioral issues, seizures |
Preoperative cardiac evaluation for mitral valve prolapse, may have behavioral difficulties, chronic antiseizure medication |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Fraser syndrome |
Absent palpebral fissures with skin overlying the eyes (cryptophthalomas), choanal atresia, tracheal stenosis, micrognathia, congenital cardiac defects, syndactyly, renal anomalies |
May be difficult intubation from laryngeal or tracheal stenosis, avoid nasal tubes if choanal atresia, preoperative cardiac evaluation, check renal function preoperatively |
Jagtap and others, 1995 ;Baum and O'Flaherty, 1999 |
|
Freeman-Sheldon syndrome (also Sheldon-Freeman syndrome and whistling face syndrome) |
Slowly progressive myopathy, increased muscle tone of face with microstomia, micrognathia, upper airway obstruction from increased tone of pharynx, dysphagia, joint contractures |
May be very difficult intubation from microstomia and micrognathia, fiberoptic intubation through an LMA has been described, aspiration risk with vomiting, hyperkalemia possible with myopathy, unclear association with malignant hyperthermia |
Jones and Dolcourt, 1992 ;Vas and Naregal, 1998 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Friedreich ataxia |
Degeneration of corticospinal, spinocerebellar, and pyramidal tracts, ataxia, cardiomyopathy is common |
Preoperative cardiac evaluation, hyperkalemia reported with succinylcholine |
Baum and O'Flaherty, 1999 |
|
Fryn's syndrome |
Micrognathia, congenital diaphragmatic hernia, congenital heart disease, mental retardation, may have renal anomalies |
Difficult intubation, may have significant respiratory distress or death from congenital diaphragmatic hernia, preoperative cardiac evaluation, check renal function preoperatively |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
G |
|||
|
Gaucher disease |
Lysosomal storage disease, trismus in infants, pulmonary HTN, seizures and apnea in infants, cranial nerve involvement, splenomegaly with thrombocytopenia and anemia |
May be difficult intubation from trismus, may be at risk for aspiration, check hematocrit and platelet count preoperatively |
Tobias and others, 1993 ;Baum and O'Flaherty, 1999 |
|
Goldenhar syndrome |
A subset of hemifacial microsomia: hemifacial microsomia, epibulbar dermoid, and rib, vertebral, scapular anomalies. |
May be very difficult intubation and mask ventilation, may have increased ICP, preoperative cardiac evaluation, check renal function preoperatively |
Scholtes and others, 1987 ;Madan and others, 1990 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Microtia with hearing loss, congenital heart disease, renal anomalies, may have hydrocephalus, radial anomalies |
|||
|
Gorlin syndrome |
Pigmented and atrophic skin changes, may have oral papillomas, congenital heart disease, GERD, renal dysplasia |
May be difficult ventilation and intubation from papillomas, care with positioning with joint hypermobility, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
H |
|||
|
Hajdu-Cheney syndrome |
Abnormality in skeletal tissue development, failure of cranial suture ossification, hypoplastic mandible, dental loss, cervical instability, short stature, cystic renal disease |
May be difficult intubation from hypoplastic mandible and cervical instability, check renal function preoperatively |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Hallerman-Streiff syndrome |
Birdlike facies, mandibular hypoplasia, tracheomalacia, chronic upper airway obstruction, may have cor pulmonale, joint hyperextensibility |
May be difficult intubation, can have significant airway obstruction, care with positioning |
Ravindran and Stoops, 1979 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Hallervorden-Spatz disease |
Iron deposition in CNS, oromandibular rigidity, dystonia, rigidity, dementia, spasticity |
Succinylcholine is contraindicated secondary to bedridden condition, oromandibular rigidity resolves under anesthesia, neuroleptic malignant syndrome has been reported |
Roy and others, 1983 ;Hayashi and others, 1993 ;Baum and O'Flaherty, 1999 |
|
Henoch-Schönlein purpura |
Usually postinfectious small vessel vasculitis, edema, renal dysfunction, lower extremity, and abdominal pain |
Check renal function preoperatively |
Baum and O'Flaherty, 1999 |
|
Holt-Oram syndrome |
Upper limb defects and congenital heart disease |
Preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 |
|
Hunter syndrome |
Mucopolysaccharidosis II from lack of iduronate sulfatase, coarse facial features, soft tissue stiffness of lips and mouth, joint stiffness, coronary artery narrowing, hydrocephalus |
Can be extremely difficult intubation, fiberoptic scope and LMA have been used, preoperative cardiac evaluation for ischemia, care with positioning |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Hurler syndrome |
Mucopolysaccharidosis IH from abnormality in α-L-iduronidase (clinically similar to Hunter syndrome but more severe and rapid clinical course), coarse facial features, soft tissue stiffness of lips and mouth, joint stiffness, coronary artery narrowing, hydrocephalus, mental retardation |
Extremely difficult intubation, fiberoptic scope and LMA have been used, preoperative cardiac evaluation for ischemia, care with positioning |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
J |
|||
|
Jarcho-Levin syndrome |
Dwarfism, limited cervical motion, short thoracic cage, rib anomalies, congenital heart disease |
May be difficult intubation, preoperative cardiac evaluation, may have restrictive lung disease |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Jervell and Lange-Nielsen syndrome |
See Prolonged QT syndrome |
||
|
Jeune syndrome |
Asphyxiating thoracic dystrophy, thoracic cage deformity, lung hypoplasia, may have pulmonary HTN, chronic renal failure |
Severe respiratory insufficiency, preoperative cardiac evaluation for pulmonary HTN and cor pulmonale, check renal function preoperatively |
Borland, 1987 ; Baum and O'Flaherty, 1999 ; Butler and others, 2000 |
|
K |
|||
|
Kabuki syndrome |
Similarity in facial appearance with Japanese Kabuki actors, congenital heart disease, may have pectus excavatum, mental retardation, renal anomalies |
Preoperative cardiac evaluation, check renal function preoperatively |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Kartagener syndrome |
Marked by situs inversus and immotile cilia, chronic sinusitis, chronic respiratory tract infection, bronchiectasis, dextrocardia, may be asplenic, immotile sperm |
Thick secretions may complicate airway management, nasal intubation relatively contraindicated in chronic sinusitis, situs inversus reverses many procedures: mainstem intubation occurs on left, reverse placement of electrocardio- graphic and defibrillator pads |
Baum and O'Flaherty, 1999 ;Ho and Friedland, 1992 |
|
Kasabach-Merritt syndrome |
Hemangioma and thrombocytopenia, high output cardiac failure, microangiopathic hemolytic anemia, may develop disseminated intravascular coagulation |
May need transfusion therapy with red cells, platelets, and fresh frozen plasma |
Baum and O'Flaherty, 1999 |
|
Kearns-Sayre syndrome |
Mitochondrial myopathy, external ophthalmoplegia, heart block, cardiomyopathy, proximal muscle weakness, ataxia, may have elevated lactate with exercise |
May develop lactic acidosis, consider avoiding lactated Ringer's, preoperative cardiac evaluation for heart block and myopathy, possible hyperkalemia with succinylcholine and mitochondrial myopathy, may be sensitive to non- depolarizing muscle relaxants, no clear association between mitochondrial myopathy and malignant hyperthermia |
Baum and O'Flaherty, 1999 |
|
Klinefelter syndrome (XXY syndrome) |
47, XXY karyotype, developmental delay, behavioral problems, hypogonadism, infertility |
Behavioral problems may complicate the perioperative period |
Baum and O'Flaherty, 1999 |
|
Klippel-Feil sequence |
Short neck and limited cervical spine immobility, micrognathia, congenital heart disease, may have renal anomalies |
Intubation may be extremely difficult, neurologic injury may occur with neck hyperextension, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Klippel-Trénaunay- Weber syndrome |
Unilateral extremity hypertrophy with arteriovenous fistulas and hemangiomas, may have high output failure, may have facial, intracranial, and epidural hematomas |
May have significant bleeding from arteriovenous fistulae, may have thrombocytopenia, preoperative cardiac evaluation for high output failure, neuraxial hemangiomas may complicate epidural and spinal anesthesia |
Baum and O'Flaherty, 1999 ;Ezri and others, 1996 |
|
L |
|||
|
Langer-Giedion syndrome |
Micrognathia, may have congenital heart disease, may have seizures, may have anemia, vertebral and rib anomalies |
May be difficult intubation, preoperative cardiac evaluation, check hematocrit preoperatively |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Larsen syndrome |
Flat face, subglottic stenosis, tracheomalacia, congenital heart disease, cervical spine instability, multiple joint dislocations |
Care with intubation with neck extension, smaller endotracheal tube with sub- glottic stenosis, preoperative cardiac evaluation, care with positioning with joint dislocations |
Stevenson and others, 1991 ; Baum and Tobias, 1996;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
LEOPARD syndrome |
Lentigines, electrocardiographic conduction abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia retarded growth, deafness |
May be deaf, preoperative cardiac evaluation for conduction defects and pulmonic stenosis |
Rodrigo and others, 1990 ;Baum and O'Flaherty, 1999 |
|
Leprechaunism |
Mutation of insulin receptor gene, hyperglycemia and hypoglycemia, precocious puberty |
Check perioperative glucose |
Baum and O'Flaherty, 1999 |
|
Lesch-Nyhan syndrome |
Developmental delay, hypertonia, spasticity, self-mutilation, hyperuricemia and renal stones, megaloblastic anemia |
Check hematocrit preoperatively, check renal function preoperatively |
Williams and others, 1997 ;Baum and O'Flaherty, 1999 |
|
Liddle syndrome |
Defect in sodium channel, HTN, hypokalemic metabolic alkalosis, may have renal failure |
Check preoperative electrolytes (K+, HCO3-), check renal function, check blood pressure preoperatively |
Baum and O'Flaherty, 1999 |
|
Limb-girdle muscular dystrophy |
Respiratory tract infections, conduction disorder, muscle weakness of pelvis and legs |
Preoperative cardiac evaluation for conduction disorders, succinylcholine may cause hyperkalemia, no known association with malignant hyperthermia |
Baum and O'Flaherty, 1999 |
|
Lowe syndrome |
Cataracts, mental retardation, renal failure, rickets |
Check renal function preoperatively, check electrolytes (Ca2+) preoperatively |
Baum and O'Flaherty, 1999 |
|
Lown-Ganong-Levine syndrome |
Accessory pathway that bypasses atrioventricular node, atrial tachyarrhythmias |
Reentrant tachycardia can be treated with adenosine or β-blockade |
Baum and O'Flaherty, 1999 |
|
M |
|||
|
Marfan syndrome |
Connective tissue disorder, defect in fibrillin, lens dislocations, pectus excavatum, pulmonary blebs, pneumothorax, dilation of aorta and pulmonary artery, aortic insufficiency and mitral valve prolapse, recurrent joint dislocations |
Preoperative cardiac evaluation for aortic and valvular pathology, aortic dissection possible, pneumothorax possible with positive pressure ventilation, care with positioning |
Wells and Podolakin, 1987 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Maroteaux-Lamy syndrome |
Mucopolysaccharidosis, heart failure common in second and third decade, recurrent respiratory infections, obstructive sleep apnea, short stature, decreased joint mobility, anemia and thrombocytopenia |
May be very difficult intubation, preoperative cardiac evaluation, care with positioning, check preoperative hematocrit and platelet count |
Baum and O'Flaherty, 1999 |
|
McArdle syndrome |
Glycogen storage disease (V) with subsequent myopathy, muscle pain, no cardiac involvement |
May develop hyperkalemia with succinylcholine |
Baum and O'Flaherty, 1999 |
|
McCune-Albright syndrome |
Fibrous dysplasia of bones, café-au-lait spots, precocious puberty, may have thyrotoxicosis and hyperadrenalism |
Care with positioning secondary to pathologic fractures, cushingoid patients may have difficult intravenous access |
Baum and O'Flaherty, 1999 |
|
Meckel-Gruber syndrome (Meckel syndrome) |
Microcephaly, short neck, micrognathia, congenital heart disease, may have occipital encephalocele, hydrocephalus, seizures, renal dysplasia |
May be difficult intubation, preoperative cardiac evaluation, check preoperative renal function, anticonvulsant medications may affect neuromuscular blockade and opioid metabolism |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
MELAS syndrome |
Mitochondrial myopathy, encephalopathy, lactic acidosis, stroke. |
Preoperative cardiac evaluation, possible hyperkalemia with succinylcholine, no clear association with malignant hyperthermia |
Baum and O'Flaherty, 1999 |
|
Blindness and hearing loss, cardiomyopathy, diffuse CNS degeneration with strokelike infarcts |
|||
|
Melnick-Needles syndrome |
Small face, micrognathia, glaucoma, respiratory track infections, may have pulmonary HTN, hypotonia |
May be difficult intubation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Menkes kinky hair syndrome (Menkes syndrome) |
Abnormal copper transport, microcephaly, cerebral degeneration, seizures, characteristic kinky hair, gastroesophageal reflux |
Difficult ventilation and intubation is described, anticonvulsant medications may affect neuromuscular blockade and opioid metabolism |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
MERRF syndrome |
Myoclonus, epilepsy, ragged red fibers Defect in mitochondrial DNA, hearing loss, epilepsy, myopathy, CNS degeneration |
May be acidemic, avoid lactated Ringer's in those with lactic acidosis, possible hyperkalemia with succinylcholine, no clear association with malignant hyperthermia |
Baum and O'Flaherty, 1999 |
|
Miller syndrome (postaxial acrofacial dysostosis syndrome) |
Craniofacial syndrome, malar hypoplasia, eyelid colobomas, micrognathia, congenital heart disease, postaxial limb deficiency, renal anomalies |
May be very difficult ventilation and intubation, preoperative cardiac evaluation, check preoperative renal function, may be difficult intravenous access, eye care essential |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Möbius sequence |
Congenital sixth and seventh cranial nerve palsy, micrognathia, feeding difficulties and aspiration, congenital cardiac defects possible, limb defects |
May be difficult to intubate, decreased ability to manage secretions, eye care essential, preoperative cardiac evaluation, may develop perioperative apnea or hypoventilation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Morquio syndrome |
Mucopolysaccharidosis (IV), short neck with decreased mobility, spine and thoracic deformities with respiratory insufficiency, aortic valve pathology, atlantoaxial instability, short stature |
Preoperative cervical spine evaluation, may be difficult intubation, preoperative cardiac evaluation for aortic valve, care with positioning |
Baum and O'Flaherty, 1999 |
|
Multiple pterygium syndrome |
Multiple pterygia, micrognathia, decreased range of motion of neck, rare congenital heart defects |
Difficult intubation described, LMA has been used, care with positioning, malignant hyperthermia has been reported—association with malignant hyperthermia not clear |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Multiple synostosis syndrome |
Multiple synostosis of digits with decreased joint mobility, developmental delay |
Care with positioning |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Myotonia congenita |
See Chapter 32 |
||
|
Myotonic dystrophy |
See Chapter 32 |
||
|
N |
|||
|
Nager syndrome (mandibulofacial dysostosis syndrome) |
Absent zygomatic arches, downsloping palpebrai fissures, colobomas, hearing loss, micrognathia, congenital heart disease, radioulnar synostosis, similar to Treacher Collins |
Difficult or impossible intubation described, intravenous access may be difficult due to limb anomalies, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Neu-Laxova syndrome |
Microcephaly, micrognathia, short neck, canine facies, congenital heart disease, renal anomalies, usually lethal |
May be difficult intubation, preoperative cardiac evaluation, check preoperative renal function |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Neurofibromatosis (von Recklinghausen disease) |
Neurofibromas of central and peripheral nervous system, café-au-lait spots and bone lesions, association with pheochromocytoma |
Neurofibromas may involve airway, increased sensitivity to succinylcholine and nondepolarizing muscle relaxants described, neurofibromas may involve neuraxial space |
Yamashita and others, 1977 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Noonan syndrome |
Webbed neck, low-set ears, micrognathia, pectus excavatum, congenital heart disease especially pulmonic stenosis, chylothorax, lymphedema, mental retardation, platelet and coagulation disorders, renal dysfunction |
May be difficult intubation, may have bleeding diathesis, preoperative cardiac evaluation, check preoperative renal function, intravenous access may be difficult with lymphedema |
Campbell and Bousfield, 1992 ; Schwartz and Eisenkraft, 1992 ; Baum and O'Flaherty, 1999 ; Butler and others, 2000 |
|
O |
|||
|
Oculodentodigital syndrome (oculodentoosseous dysplasia) |
Micro-ophthalmia, cleft lip or palate, micrognathia or mandibular overgrowth, small nose, syndactyly |
May be difficult intubation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Oral-facial-digital-syndrome (Type I) |
Cleft Up and palate, micrognathia, choanal atresia, mental retardation, hydrocephalus, syndactyly, digital asymmetry, polycystic kidney disease, affects females, Types II-IX clinically similar to Type I |
Upper airway obstruction possible, check renal function preoperatively |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Oromandibular-limb hypogenesis |
Small mouth, cleft palate, moebius, micrognathia, limb deficiency |
May be difficult intubation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Osier-Weber-Rendu syndrome (hereditary hemorrhagic telangiectasia) |
Vasculopathy with multiple telagiectases, pulmonary arteriovenous fistula with right-to-left shunting, paradoxical emboli, and bleeding, CNS arteriovenous fistulas and aneurysms, gastrointestinal bleeding |
Patients can have life-threatening bleeding from nose, lung, brain, and gastrointestinal tract, check hematocrit preoperatively, avoid nasal instrumentation, care with laryngoscopy to avoid bleeding, paradoxical emboli can occur, consider avoiding neuraxial blocks |
Waring and others, 1990 ;Radu and others, 1992 ;Baum and O'Flaherty, 1999 |
|
Osteogenesis imperfecta |
Mutation in a gene for collagen, four types, blue sclera in type I, hearing loss, kyphoscoliosis, restrictive hearing loss, extremely fragile bones, multiple fractures, hyperextensibilky, short stature |
Fractures may occur with any manipulation, care with intubation, care with positioning, consider radiologie evaluation for fractures, type I may have platelet dysfunction, malignant hyperthermia not clearly associated |
Barros, 1995 ; Porsborg and others, 1996 ; Baum and O'Flaherty, 1999 ; Butler and others, 2000 |
|
P |
|||
|
Pallister Hall syndrome |
Hypothalamic hamartoblastoma, panhypopkuitarism, imperforate anus, cleft lip and/or palate, micrognathia, dysplastic tracheal cartilage, hypoplastic lung, congenital heart disease, thyroid hypoplasia |
May be difficult intubation, abnormal tracheal cartilage may alter tracheal tube size, preoperative cardiac evaluation, preoperative evaluation of hypothalamic pituitary axis, may require stress-dose steroids |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Pearson syndrome |
Sideroblastic anemia, exocrine pancreatic dysfunction (may have Fanconi syndrome) |
Preoperative hematocrk, preoperative electrolytes (K+, Cl-, HCO3-, PO4-) |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Pendred syndrome |
Deafness, hypothyroid, goiter |
Preoperative thyroid studies |
Baum and O'Flaherty, 1999 |
|
Pentalogy of Cantrell |
Defect in ventral midline development, cystic hygroma, cleft lip and/or palate, congenital diaphragmatic hernia, pulmonary hypoplasia, omphalocele, renal anomalies, congenital heart disease |
Preoperative cardiac evaluation, may have significant pulmonary hypoplasia and pulmonary HTN |
Laloyaux and others, 1998 ;Baum and O'Flaherty, 1999 |
|
Peter's plus syndrome |
Ophthalmologic anomalies (glaucoma, decreased vision), micrognathia, congenital heart disease, dwarfism, developmental delay, seizures |
May be difficult intubation (rare), preoperative cardiac evaluation, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Pfeiffer syndrome |
Acrocephalosyndactyly, coronal and/or sagittal synostosis, midface hypoplasia, proptosis, obstructive sleep apnea, congenital heart defects, broad thumbs and broad great toes, may have fused elbows, usually normal intelligence |
May be difficult intubation (rare), may have increased ICP, preoperative cardiac evaluation, may have airway obstruction |
Tobias and others, 1998 ;Baum and O'Flaherty, 1999 |
|
Pierre Robin sequence |
Micrognathia, glossoptosis, airway obstruction, may also have cleft palate (not necessary for diagnosis) |
May be very difficult to intubate, successful intubation described with fiberoptic scopes, LMA, and Bullard, prone positioning, and tongue suture to displace tongue forward |
Populaire and others, 1985 ;Schelle and Schulman, 1991 ;Baraka, 1996 ; Perkins and others, 1997 ; Baum and O'Flaherty, 1999 ; Butler and others, 2000 |
|
Poland syndrome |
Hypoplasia of chest muscles and ribs on one side, rare dextrocardia, unilateral syndactyly |
Respiratory complications |
Sethuraman and others, 1998; Baum and O'Flaherty, 1999 |
|
Pompe disease |
Glycogen storage disease, macroglossia, respiratory and swallowing muscle weakness, cardiomegaly, hypotonia |
May be difficult to ventilate and intubate, increased risk for perioperative respiratory complications, preoperative cardiac evaluation |
McFarlane and Soni, 1986 ;Rosen and Broadman, 1986 ;Baum and O'Flaherty, 1999 |
|
Porphyria |
Defect in heme synthesis with overproduction of heme precursors, precipitating factors include infection, starvation, drugs, and pregnancy, acute attacks cause abdominal pain, autonomie instability with HTN, neuropathy, neuropsychiatrie abnormalities, electrolyte abnormalities. May also have hemolytic anemia, hepatic failure, and cirrhosis. |
Preoperative fasting should be minimized, can administer glucose-containing solution, premedication may decrease stress, avoid barbiturates, etomidate pentazocine. Ketamine may be safe, safe anesthetics include opioids, propofol, nitrous oxide, isoflurane, sevoflurane, desflurane, and muscle relaxants, neuropathy may cause respiratory muscle weakness and aspiration |
Jensen and others, 1995 ;Kanbak, 1997 ; Baum and O'Flaherty, 1999 |
|
Potter syndrome |
Secondary to oligohydramnios, low-set ears, beaked nose, micrognathia, lung hypoplasia, renal agenesis |
May be difficult intubation, respiratory failure secondary to pulmonary hypoplasia, check preoperative renal function |
Baum and O'Flaherty, 1999 |
|
Prader-Willi syndrome |
Secondary to partial deletion of chromosome 15, obesity, hypotonia, developmental delay, hypogonadism, obstructive sleep apnea, may have pulmonary HTN, hypotonia, developmental delay, hypogonadism, diabetes mellitus |
Anesthetic concerns related to obesity, difficult mask ventilation, potential difficult airway, decreased functional residual capacity, perioperative respiratory complications, monitor perioperative glucose |
McKenzie, 1991 ; Dearlove and others, 1998 ; Baum and O'Flaherty, 1999 ; Butler and others, 2000 |
|
Progeria |
Premature aging, micrognathia, beaked nose, premature coronary artery disease, cerebrovascular disease, HTN, diabetes mellitus |
Perioperative cardiac evaluation for ischemie heart disease, may be difficult intubation secondary to micrognathia and stiff joints |
Chapin and Kahre, 1979 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Prolonged QT interval syndrome (Jervell-Lange-Nielson syndrome; Romano-Ward syndrome) |
Prolonged QT interval on electrocardiogram, congenital deafness with Jervell-Lange Nielson syndrome |
Tachydysrhythmias and sudden death, torsades de pointes, prevention by avoiding drugs that prolong QT interval, volatile anesthetics prolong QT interval, avoid epinephrine, treat torsades with defibrillation, magnesium, correcting electrolytes, β-blocker |
Dunn and others, 1991 ;Richardson and others, 1992 ;Holland, 1993 ; Michaloudis and others, 1996 |
|
Proteus syndrome |
Hemihypertrophy, hypertrophie cardiomyopathy emphysematous lung disease, renal anomalies, cervical spine abnormalities |
Perioperative cardiac evaluation, may have abnormal lung function, check preoperative renal function |
Pennant and Harris, 1991 ;Baum and O'Flaherty, 1999 |
|
Prune-belly syndrome (Eagle-Barrett syndrome) |
Absence of abdominal wall musculature, urinary tract dilation, cryptorchidism, lung hypoplasia secondary to in-utero urinary tract obstruction with oligohydramnios, may have congenital heart disease |
Increased risk of perioperative respiratory complications secondary to decreased ability to cough, check preoperative renal function, preoperative cardiac evaluation |
Henderson and others, 1987 ;Baum and O'Flaherty, 1999 |
|
R |
|||
|
Rett syndrome |
Only in females, progressive encephalopathy beginning at 6 to 18 months, seizures, spasticity, behavioral problems |
Anticonvulsant medication may affect neuromuscular blockade and opioid metabolism |
Dearlove and Walker, 1996 ;Baum and O'Flaherty, 1999 |
|
Riley-Day syndrome (familial dysautonomia) |
Deficiency of dopamine hydroxylase, blood pressure instability, abnormal sweating, hypersensitivity to catecholamine, emotional lability, decreased respiratory drive to hypercarbia and hypoxia, decreased or lack of tear formation, decreased peripheral pain sensation |
Increased risk of aspiration, may have exaggerated response to inotropes, may have exaggerated depression of respiratory drive with narcotics, perioperative eye care, decreased postoperative pain requirements |
Stenquist and Sigurdsson, 1982 ; Axelrod and others, 1988 ; Baum and O'Flaherty, 1999 |
|
Riley-Smkh syndrome |
Macrocephaly, mild developmental delay, lipid storage myopathy, seizures |
Anticonvulsant medication may affect neuromuscular blockade and opioid metabolism, may develop hyperkalemia with succinylcholine |
Baum and O'Flaherty, 1999 |
|
Robinow syndrome (fetal face syndrome) |
Frontal bossing, micrognathia, may have congenital heart disease, genitourinary and renal anomalies |
May be difficult intubation, check preoperative renal function, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Rubinstein-Taybi syndrome |
Defect in cAMP-mediated induction of protein synthesis, microcephaly, micrognathia, obstructive sleep apnea, congenital heart disease in 30%, developmental delay, seizures, broad thumbs and toes |
May be difficult to intubate, preoperative cardiac evaluation, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism, may have delayed recovery from anesthesia |
Stirt, 1981 ; Dunkley and Dearlove, 1996 ; Baum and O'Flaherty, 1999 ; Butler and others, 2000 |
|
Russell-Silver syndrome |
Short stature, micrognathia, cafe-au-lait spots, normal intelligence hypoglycemia with fasting |
May be difficult to intubate, monitor perioperative glucose and use glucose-containing solutions |
Diner and others, 1994 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
S |
|||
|
Saethre-Chotzen syndrome |
Acrocephalosyndactyly, craniosynostosis, maxillary hypoplasia, normal intelligence, syndactyly |
No reports of difficult intubation, may have increased ICP |
Baum and O'Flaherty, 1999 |
|
Sanfilippo syndrome |
Mucopolysaccharidosis III, coarse facies, rare cardiac involvement, severe developmental delay, behavioral disturbances, seizures |
May be difficult intubation, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism, may have behavioral problems |
Kempthorne and Brown, 1983 ; Myles and Westhorpe, 1989 ; Baum and O'Flaherty, 1999 |
|
Schinzel-Giedion syndrome |
Growth and mental deficiencies, congenital heart disease, seizures, renal anomalies, choanal atresia |
Preoperative cardiac evaluation, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism, check preoperative renal function |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Schwartz-Jampel syndrome |
Myotonia secondary to sodium channel defect, micrognathia, joint contractures |
May be difficult intubation, myotonic contractures can occur during anesthetic or surgical manipulation, muscle relaxation does not relax contractions, hyperkalemia with succinylcholine, myotonic contractures can be precipitated by reversal of muscle relaxant, may be associated with malignant hyperthermia |
Ray and Rubin, 1994 ;Theroux and others, 1995 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Scimitar syndrome |
Partial anomalous pulmonary venous return, hypoplastic right lower lobe, recurrent pulmonary infections, pulmonary HTN |
Preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 |
|
Shprintzen syndrome |
See Velocardiofacial syndrome |
||
|
Shwachman syndrome |
Metaphyseal chondrodysplasia, recurrent pneumonia, hypotonia, short stature, exocrine pancreatic insufficiency pancytopenia, immunologic abnormalities |
Preoperative hematocrit and platelet count |
Baum and O'Flaherty, 1999 |
|
Smith-Lemli-Opitz syndrome |
Abnormality in cholesterol synthesis, microcephaly, micrognathia, lung hypoplasia, congenital heart disease, mental retardation, seizures, renal anomalies, thymic hypoplasia |
May be difficult intubation, preoperative cardiac evaluation, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism, blood should be irradiated if thymic hypoplasia, no clear association with malignant hyperthermia |
Peterson and Crouch, 1995 ;Haji-Michael and Hatch, 1996 ; Baum and O'Flaherty, 1999 ; Butler and others, 2000 |
|
Sotos syndrome |
Cerebral gigantism, excessive head growth, congenital heart disease, mental retardation, behavioral problems |
No reports of difficult airway, preoperative cardiac evaluation |
Jones and others, 1991 ;Baum and O'Flaherty, 1999 |
|
Stevens-Johnson syndrome |
Secondary to hypersensitivity to exogenous agents (drugs), erythema multiforme, urticarial lesions |
Placement of monitors may be difficult secondary to skin lesions, care with positioning |
|
|
Stickler syndrome |
Flat facies, hearing loss, cleft palate, pectus excavatum, hyperextensible joints, may be associated with Pierre Robin sequence |
May be difficult intubation, may have hearing loss |
Baum and O'Flaherty, 1999 |
|
Sturge-Weber syndrome |
Capillary hemangiomas in distribution of cranial nerve V, port wine stain, congenital glaucoma, may have mental retardation and seizures |
Hemangiomas of the mouth and upper airway may make intubation difficult, may have increased intraocular pressure, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism |
Batra and others, 1994 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
T |
|||
|
Tangier disease |
Low high-density lipoprotein and serum cholesterol, orange tonsils, neuropathic abnormalities with cranial, sensory, or motor findings, muscle wasting, splenomegaly, hemolytic anemia, platelet dysfunction |
Succinylcholine may cause hyperkalemia, check preoperative hematocrit and platelet count, possible premature ischemie heart disease |
Mentis, 1996 ; Baum and O'Flaherty, 1999 |
|
Thrombocytopenia-absent radius syndrome (TAR syndrome) |
Thrombocytopenia precipitated by stress, infection, surgery, congenital heart disease, bilateral absence of radii (thrombocytopenia worst during infancy) |
Preoperative cardiac evaluation, check preoperative platelet count |
Baum and O'Flaherty, 1999 |
|
Tourette syndrome |
Involuntary motor and vocal tics, behavioral abnormalities |
No specific anesthetic concerns |
Morrison and Lockhart, 1986 |
|
Treacher Collins syndrome |
Mandibulofacial dysostosis, malar hypoplasia, downsloping palbebral fissures, micro-ophthalmia, low-set ears, small mouth, micrognathia, obstructive sleep apnea, congenital heart disease |
May be extremely difficult airway fiberoptic endoscopy, LMA, and Bullard laryngoscope have all been used, preoperative cardiac evaluation |
Rasch and others, 1986 ;Brown and others, 1993 ;Inada and others, 1995 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Trisomy 8 syndrome |
Strabismus, micrognathia, webbed neck, congenital heart disease, mental retardation, seizures |
May be difficult intubation, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 |
|
Trisomy 9 syndrome |
May have microcephaly, cleft lip and palate, micrognathia, congenital heart disease, profound mental retardation |
May be difficult intubation, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism, preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 |
|
Trisomy 13 syndrome (Patau syndrome) |
Occipital scalp defect, low-set ears, cleft lip and palate, micrognathia, congenital heart disease common, severe mental retardation, seizures, renal anomalies, capillary hemangiomas, most die within 6 months, radial anomalies |
May be difficult intubation, preoperative cardiac evaluation, check preoperative renal function, placement of radial catheter may be difficult, anticonvulsant medication may effect neuromuscular blockade and opioids |
Pollard and Beasley, 1996 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Trisomy 18 syndrome (Edwards syndrome) |
Prominent occiput, microcephaly, micrognathia, short sternum, congenital heart disease, clenched hands, rocker bottom feet, renal anomalies |
May be difficult intubation, preoperative cardiac evaluation, check preoperative renal function |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Trisomy 21 syndrome |
See Down syndrome |
||
|
Tuberous sclerosis |
Triad of mental retardation, seizures, and adenoma sebaceum on face, hamartomas of the lung, cardiac rhabdomyomas, may develop endocrinopathies |
Oral adenomas may make intubation difficult, preoperative cardiac evaluation, spontaneous pneumothorax may occur, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism |
Lee and others, 1994 ;Schweiger and others, 1994 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Turner syndrome |
Caused by single X chromosome, micrognathia and short webbed neck, broad chest, congenital heart disease (coarctation), HTN, short stature, lymphedema of hands and feet, renal anomalies, may be hypothyroid |
May be difficult intubation, preoperative cardiac evaluation, intravenous access may be difficult secondary to lymphedema, check preoperative renal function |
Divekar and others, 1983 ;Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
V |
|||
|
VATER association |
Vertebral anomalies, anal atresia, tracheoesophageal fistula,esophageal atresia, radial and renal anomalies. Increased incidence in tracheoesophageal fistula, diabetic mothers, and trisomy 18 |
Preoperative cardiac evaluation, check radiographs of vertebrae and radii, check preoperative renal function |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Velocardiofacial syndrome (Shprintzen syndrome) |
Deletion of chromosome 22, microcephaly micrognathia, congenital heart disease, developmental delay, may have T-cell immunodeficiency, neonatal hypocalcemia |
May be difficult intubation, preoperative cardiac evaluation, blood should be irradiated if T-cell immunodeficiency check preoperative calcium in neonates |
Baum and O'Flaherty, 1999 |
|
von Hippel Lindau syndrome |
Retinal, CNS, and visceral hemangioblastomas, may be associated with pheochromocytoma, may have renal, pancreatic, or hepatic cysts, may develop cerebellar tumors |
Epidural analgesia has been described despite descriptions of spinal cord involvement, may have increased ICP, preoperative evaluation for pheochromocytoma |
Matthews and Halshaw, 1986; Mugawar and others, 1998 ;Baum and O'Flaherty, 1999 |
|
W |
|||
|
Walker-Warburg syndrome (HARD syndrome) |
Retinal defects, microtia, cleft lip and palate, CNS anomalies, hydrocephalus, seizures, mental retardation, muscular dystrophy |
May have increased intraocular pressure and ICP, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism, succinylcholine may cause hyperkalemia |
Baum and O'Flaherty, 1999 |
|
Watson syndrome |
Valvular pulmonary stenosis, slightly decreased intelligence, may have neurofibromas and café-au-lait spots |
Preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Weaver syndrome |
Macrocephaly, large tongue, short neck, developmental delay, seizures, behavioral problems |
May be very difficult intubation, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism |
Turner and Downing, 1985 ;Baum and O'Flaherty, 1999 |
|
Weber-Christian disease |
Fevers and panniculitis, necrosis of fat involving the retroperitoneal, pericardial, and meningeal areas, may have heart failure, involvement of retroperitoneal tissue may cause adrenal insufficiency, may develop restrictive pericarditis and seizures |
Preoperative cardiac evaluation for cardiac failure, avoid trauma to fat, anticonvulsant medication may affect neuromuscular blockade and opioid metabolism |
Baum and O'Flaherty, 1999 |
|
Weill-Marchesani syndrome |
Glaucoma, blindness, subvalvular aortic stenosis |
Preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 |
|
Werdnig-Hoffman disease |
Spinal muscular atrophy, anterior horn cell degeneration, decreased ability to swallow, respiratory tract infections, may have respiratory failure spine deformities, normal intelligence |
Increased risk of perioperative aspiration, may be chronically ventilated, succinylcholine may cause hyperkalemia |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 |
|
Whistling face syndrome |
See Freeman-Sheldon syndrome |
||
|
Williams syndrome (Williams-Beuren syndrome) |
Deletion of elastin gene on chromosome 7, elfin facies, severe congenital heart disease, high incidence of sudden death, mild developmental delay, may be very gifted musically, precocious social skills, “cocktail party personality,” rarely has behavioral problems, may be hypercalcemic |
Preoperative cardiac evaluation |
Baum and O'Flaherty, 1999 ;Butler and others, 2000 ;Kececioglu and others, 1993 |
|
Wiskott-Aldrich syndrome |
Recurrent pulmonary infections, vasculitis may affect coronary and cerebral arteries, renal insufficiency, thrombocytopenia, eczema, immune deficiency |
Check preoperative platelet count and hematocrit, irradiate blood products, consider preoperative cardiac evaluation of coronary arteries, check preoperative renal function, consider stress-dose steroids if taking steroids on a chronic basis |
Baum and O'Flaherty, 1999 |
|
Wolff-Parkinson-White syndrome |
Accessory pathway bypasses atrioventricular node, reentrant tachycardia, may have structural heart disease |
Adenosine and β-blockade terminate the tachycardia |
Lavoie and others, 1995 |
|
Z |
|||
|
Zellweger syndrome |
Dysfunction and absence of peroxisomes, micrognathia, congenital heart disease, hypotonia with respiratory insufficiency adrenal atrophy, contractures |
Preoperative cardiac evaluation, care with positioning, may require stress-dose steroids |
Baum and O'Flaherty, 1999 |
|
CNS, central nervous system; GERD, gastrointestinal reflux disease; HTN, hypertension; ICP, intracranial pressure; LMA, laryngeal mask airway. |
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