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PEDIATRICS Vol. 106 No. 2 August 2000, pp. 358-361

AMERICAN ACADEMY OF PEDIATRICS:
Fetal Alcohol Syndrome and Alcohol-Related Neurodevelopmental Disorders

Committee on Substance Abuse and Committee on Children With Disabilities

	ABSTRACT

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Prenatal exposure to alcohol is one of the leading preventable causes of birth defects, mental retardation, and neurodevelopmental disorders. In 1973, a cluster of birth defects resulting from prenatal alcohol exposure was recognized as a clinical entity called fetal alcohol syndrome. More recently, alcohol exposure in utero has been linked to a variety of other neurodevelopmental problems, and the terms alcohol-related neurodevelopmental disorder and alcohol-related birth defects have been proposed to identify infants so affected. This statement is an update of a previous statement by the American Academy of Pediatrics and reflects the current thinking about alcohol exposure in utero and the revised nosology.

The term fetal alcohol syndrome (FAS) refers to a constellation of physical, behavioral, and cognitive abnormalities.¹ In addition to the classic dysmorphic facial features, prenatal and postnatal growth abnormalities, and mental retardation that define the condition, approximately 80% of children with FAS have microcephaly and behavioral abnormalities.² As many as 50% of affected children also exhibit poor coordination, hypotonia, attention-deficit hyperactivity disorder, decreased adipose tissue, and identifiable facial anomalies, such as maxillary hypoplasia, cleft palate, and micrognathia. Cardiac defects, hemangiomas, and eye or ear abnormalities are also common.^3-5

The term fetal alcohol effects was developed originally to describe abnormalities observed in animal studies, but it was adopted quickly by clinicians to describe children with a variety of problems, including growth deficiency, behavioral mannerisms, and delays in motor and speech performance, who lacked the full complement of FAS diagnostic criteria. The lack of specificity and absence of definitive diagnostic criteria have made research and classification difficult, and a 1980 report from the Research Society on Alcohol suggested that fetal alcohol effects encompassed "any condition thought to be secondary to alcohol exposure in utero."⁶ Clearly, such a definition was cumbersome and allowed for wide divergence in intepretation.⁶ The Institute of Medicine in 1996 issued a report proposing the terms alcohol-related neurodevelopmental disorder (ARND) and alcohol-related birth defects (ARBD) to describe conditions in which there is a history of maternal alcohol exposure (defined as substantial regular intake or heavy episodic drinking) and an outcome validated by clinical or animal research to be associated with that exposure.⁷ This new terminology uses a pathophysiologic basis for the diagnostic categories to describe conditions resulting from prenatal alcohol exposure (Fig 1).

View larger version (62K): [in this window] [in a new window]   Fig. 1.   Diagnostic classification of fetal alcohol syndrome (FAS) and alcohol-related effects.*

Children with FAS, ARND, and ARBD may manifest cognitive, behavioral, and psychosocial problems that cause lifelong disabilities, although the resulting manifestations may vary with age and circumstances. Streissguth et al^5,8 traced the natural history of alcohol-affected children into adulthood and demonstrated the profound, pervasive, and persistent nature of the disorder. Abnormal cognitive functioning manifested itself in many domains, including specific mathematical deficiency, difficulty with abstraction (eg, time and space, cause-and-effect), and problems with generalizing from one situation to another. The affected persons also demonstrated poor attention and concentration skills, memory deficits, and impaired judgment, comprehension, and abstract reasoning. Behavioral issues, such as hyperactivity and impulsivity, and conduct problems, such as lying, stealing, stubbornness, and oppositional behavior, were common and were quantitatively and qualitatively different from those found in other forms of mental retardation.

None of the persons in the aforementioned study had achieved age-appropriate socialization or communication skills. Maladaptive social functioning was evidenced by their failure to consider consequences for their actions, lack of response to appropriate social cues, lack of reciprocal friendships, social withdrawal, sullenness, mood lability, teasing and bullying behavior, and periods of high anxiety and excessive unhappiness. Secondary disabilities, such as mental health problems, chemical dependency, failure to develop appropriate sexual behavior, and consequent legal problems, were also common in adults diagnosed with FAS. Current evidence suggests that while IQ scores <70 in this population increase the likelihood of such outcomes, early diagnosis and intervention may reduce the occurrence of secondary disabilities.⁹

As one of the most commonly identifiable causes of mental retardation, FAS is estimated to occur at the rate of 5.2/10 000 live births in the United States.^10-12 Higher rates are reported among selected subgroups (eg, 30/10 000 among Native Americans).^11-13 There seems to be a number of factors that determine the outcome of a pregnancy during which the mother consumes alcohol. Mills et al¹⁴ prospectively studied approximately 31 000 pregnancies in an attempt to determine how much alcohol pregnant women can consume safely. The consumption of 1 or more drinks (a drink is defined as 1.5 oz distilled spirits, 5 oz of wine, or 12 oz of beer) per day was associated with increased risk of giving birth to an infant with growth retardation. Although maternal age, parity, and health as well as specific fetal susceptibility may contribute to the infant's outcome, the potential for harm to the fetus is much stronger with large amounts of maternal alcohol consumption than with smaller amounts.¹⁵ Nevertheless, current data do not support the concept of a "safe level" of alcohol consumption by pregnant women below which no damage to a fetus will occur.

The economic effects of FAS, ARND, and ARBD based on the medical, surgical, behavioral, custodial, and judicial services required takes its toll on the individual, the family, and society. Annual cost estimates for the United States range from $75 million to $9.7 billion.¹⁶ The total lifetime cost of caring for a typical child with FAS may be as high as $1.4 million.⁵ The mental retardation related to FAS has by itself been estimated to account for as much as 11% of the annual cost of caring for all mentally retarded institutionalized residents of the United States and may account for up to 5% of all congenital anomalies.^17,18 The nonfiscal costs to families and affected children in terms of emotional and social effects are enormous.

	RECOMMENDATIONS

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1. Because there is no known safe amount of alcohol consumption during pregnancy, the Academy recommends abstinence from alcohol for women who are pregnant or who are planning a pregnancy.
2. Major efforts should be made at all levels of society to develop high-quality educational programs about the deleterious consequences of alcohol for the unborn child. This information should be integrated into a comprehensive drug prevention education curriculum for all elementary, junior high, and high school students. It also should be a part of similar education efforts in all postsecondary and adult centers of learning.
3. Pediatricians and other health care professionals who provide care for women and their newborns should increase their own awareness and that of their patients about FAS, ARND, and ARBD and their prevention. Pediatricians should increase their awareness of the prevalence of alcohol use by pregnant women in their communities and advocate for programs that identify the users and offer them treatment. When a child with problems related to maternal alcohol consumption is identified, alcohol treatment and prevention resources should be offered to the family and affected child.
4. Infants and children with a suspected diagnosis of FAS, ARND, or ARBD should be evaluated by a pediatrician who is knowledgeable and competent in the evaluation of neurodevelopmental and psychosocial problems associated with the diagnoses. The need for a skilled evaluation at an early age necessitates referral to a pediatric medical specialist as well as referral to early intervention and education agencies providing services under the provisions of the Individuals With Disabilities Education Act.
5. Parents of children given a diagnosis of FAS, ARBD, or ARND should receive appropriate support services for themselves and their child, including careful anticipatory guidance directed toward preventing similar problems in the future.
6. The Academy supports federal legislation that would require the inclusion of health and safety messages in all print and broadcast alcohol advertisements based on the US Surgeon General's warning: "Drinking during pregnancy may cause mental retardation and other birth defects. Avoid alcohol during pregnancy."
7. The Academy supports the development of state legislation that makes information about FAS, ARND, and ARBD available at marriage-licensing bureaus and other appropriate public places, including points of alcohol sale.
8. Pediatricians are encouraged to assume a leadership role in public education campaigns aimed at decreasing the incidence of FAS through reduction in alcohol use by pregnant women.

COMMITTEE ON SUBSTANCE ABUSE, 1999-2000
Edward A. Jacobs, MD, Chairperson
Stuart M. Copperman, MD
Alain Joffe, MD
John Kulig, MD, MPH
Catherine A. McDonald, MD
Peter D. Rogers, MD, MPH
Rizwan Z. Shah, MD

LIAISONS
Marie Armentano, MD
  American Academy of Child and Adolescent Psychology
Gayle M. Boyd, PhD
 National Institute of Alcohol Abuse/Alcoholism
Dorynne Czechowicz, MD
 National Institute on Drug Abuse

CONSULTANT
Richard B. Heyman, MD

STAFF
Stacey E. Spencer, MS

COMMITTEE ON CHILDREN WITH DISABILITIES, 1999-2000
Philip R. Ziring, MD, Chairperson
Dana Brazdziunas, MD
W. Carl Cooley, MD
Theodore A. Kastner, MD
Marian E. Kummer, MD
Lilliam Gonzalez de Pijem, MD
Richard D. Quint, MD, MPH
Elizabeth S. Ruppert, MD
Adrian D. Sandler, MD

LIAISONS
William C. Anderson
 Social Security Administration
Polly Arango
 Family Voices
Connie Garner, RN, MSN, EdD
 US Department of Education
Merle McPherson, MD
 Maternal and Child Health Bureau
Linda Michaud, MD
 Children's Hospital and Medical Center
Marshalyn Yeargin-Allsopp, MD
 Centers for Disease Control and Prevention

SECTION LIAISONS
Chris Johnson, MEd, MD
Lani S. M. Wheeler, MD

STAFF
Stacey E. Spencer, MS

	FOOTNOTES

The recommendations in this statement do not indicate an exclusive course of treatment or serve as a standard of medical care. Variations, taking into account individual circumstances, may be appropriate.

	ABBREVIATIONS

FAS, fetal alcohol syndrome; ARND, alcohol-related neurodevelopmental disorder; ARBD, alcohol-related birth defects.

	REFERENCES

Top Abstract Recommendation References

1. Jones KL, Smith DW, Ulleland CW, Streissguth P Pattern of malformation in offspring of chronic alcoholic mothers. Lancet. 1973; 1:1267-1271 [Medline]
2. Riley E, Mattson S, Sowell ER, Jernigan TL, Sobel DF, Jones KL Abnormalities of the corpus callosum in children prenatally exposed to alcohol. Alcohol Clin Exp Res. 1995; 19:1198-1202 [CrossRef][Medline]
3. Clarren SK, Smith DW The fetal alcohol syndrome. N Engl J Med. 1978; 298:1063-1067 [Medline]
4. Jones KL Fetal alcohol syndrome. Pediatr Rev. 1986; 8:122-126 [Abstract/Free Full Text]
5. Streissguth AP, Aase JM, Clarren SK, Randels SP, LaDue RA, Smith DF Fetal alcohol syndrome in adolescents and adults. JAMA. 1991; 265:1961-1967 [Abstract]
6. Aase JM, Jones KL, Clarren SK Do we need the term "FAE"? Pediatrics 1995; 95:428-430 [Abstract/Free Full Text]
7. Stratton K, Howe C, Battaglia F, eds. Fetal Alcohol Syndrome: Diagnosis, Epidemiology, Prevention and Treatment. Washington, DC: National Academy Press; 1996:4-21
8. Streissguth A, Barr H, Sampson PD, Bookstein FL Prenatal alcohol and offspring development: the first 14 years. Drug Alcohol Depend. 1994; 36:89-99 [CrossRef][Medline]
9. Streissguth A, Kanter J, eds. The Challenge of Fetal Alcohol Syndrome: Overcoming Secondary Disabilities. Seattle, WA: Washington Press; 1997
10. Cordero JF, Floyd RL, Martin ML, Tracking the prevalence of FAS. Alcohol Health Res World. 1994; 18:82-85
11. Centers for Disease Control and Prevention Surveillance for fetal alcohol syndrome using multiple sources, Atlanta, GA, 1981-1989. MMWR Morb Mortal Wkly Rep. 1997; 46:1118-1120 [Medline]
12. Abel EL Fetal alcohol syndrome: the American paradox. Alcohol Alcohol. 1998; 33:195-201 [Abstract/Free Full Text]
13. Egeland GM, Perham-Hester KA, Gessner BD, Ingle D, Berner JE, Middaugh JP Fetal alcohol syndrome in Alaska, 1977 through 1992: an administrative prevalence derived from multiple data sources. Am J Public Health. 1998; 88:781-786 [Abstract/Free Full Text]
14. Mills JL, Granbard BI, Harley EE, Rhoads GG, Berendes HW Maternal alcohol consumption and birth weight: how much drinking in pregnancy is safe? JAMA 1984; 252:1875-1879 [Abstract]
15. Kaminski M Maternal alcohol consumption and its relation to the outcome of pregnancy and child development at 18 months. Int J Epidemiol. 1992; 21:S79-S81 [Medline]
16. Hanwood HJ, Napolitano DM Economic implications of the fetal alcohol syndrome. Alcohol Health Res World. 1985; 10:38-43
17. Abel EL, Sokol RJ Incidence of fetal alcohol syndrome and economic impact of FAS-related anomalies. Drug Alcohol Depend. 1987; 19:51-70 [CrossRef][Medline]
18. Charness ME, Simon RP, Greenberg DA Ethanol and the nervous system. N Engl J Med. 1989; 321:442-454 [Medline]

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