Motoyama & Davis: Smith's Anesthesia for Infants and Children, 7th ed.

PART FOUR – Associated Problems in Pediatric Anesthesia

Chapter 36 – Medicolegal and Ethical Aspects

Eva Vogeley,Jodi Innocent

  

 

Ethical Concepts, 1191

  

 

Medical Malpractice, 1192

  

 

Duty, 1192

  

 

Breach of the Duty of Due Care,1192

  

 

Causation, 1193

  

 

Damages, 1193

  

 

Intentional Torts, 1193

  

 

Informed Consent, 1194

  

 

Implied Consent and Decisional Capacity, 1194

  

 

Emancipated Minor and the Mature Minor Doctrine, 1195

  

 

Emergency Medical Treatment and Active Labor Act, 1195

  

 

Risk Management, 1195

  

 

Research in Children, 1196

  

 

Death and Organ Donation, 1197

  

 

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 

Glossary of Terms and Abbreviations

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.

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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:

  

1.   

The injury is of a type that does not ordinarily occur without negligence.

  

2.   

The instrumentality of the injury was under the exclusive control of the defendant(s).

  

3.   

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.

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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):

  

1.   

Surgery, including the related administration of anesthesia

  

2.   

Administration of radiation or chemotherapy

  

3.   

Administration of a blood transfusion

  

4.   

Inserting a surgical device or appliance

  

5.   

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:

  

1.   

The patient or person from whom consent must be obtained is incapacitated because of unconsciousness or a similar condition.

  

2.   

Failure to act would result in death or other serious harm.

  

3.   

There is no reason to believe that the patient would not consent.

  

4.   

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:

  

1.   

An appropriate screening examination must be provided to anyone who comes to the health care facility seeking medical care.

  

2.   

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.

  1. Research Not Involving Greater Than Minimal Risk (45 CFR 46.404, 21 CFR 50.51)[*]

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.

  1. Research Involving Greater Than Minimal Risk and No Prospect of Direct Benefit to the Individual Subjects but Likely to Yield Generalizable Knowledge About the Subject's Disorder or Condition (45 CFR 46.406, 21 CFR 50.53)

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.

  1. Research Not Otherwise Approvable That Presents an Opportunity to Understand, Prevent, or Alleviate a Serious Problem Affecting the Health or Welfare of Children (45 CFR 46.407 and 21 CFR 50.54)

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.

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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.

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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.

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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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