FETAL ALCOHOL SYNDROME

Background
Medical Management Considerations
References
Resources
Publication Information

BACKGROUND
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Description and Cause

Fetal Alcohol Syndrome (FAS) is a constellation of physical and mental birth defects that is the direct result of prenatal alcohol exposure (maternal alcohol ingestion during pregnancy). The National Council on Alcoholism outlines three necessary criteria for diagnosis: abnormalities in growth, central nervous system dysfunction, and characteristic facial features. FAS is the most common preventable cause of mental retardation. Fetal Alcohol Effect (FAE), or Alcohol Related Neurodevelopmental Disorder (ARND), is a less severe condition with similar symptoms. This includes those children who may have significant neuropathology related to fetal alcohol exposure, but may not have classical findings of growth failure and facial dysmorphology The term Fetal Alcohol Spectrum Disorders (FASD) can also encompasses another diagnostic category called Alcohol Related Birth Defects (ARBD) this could also be used interchangeably with FAE or ARND.
 
Although there is much debate on the amount of alcohol ingestion required to cause Fetal Alcohol Syndrome, animal research shows that a single exposure to high levels of ethanol can kill nerve cells in the developing brain. In general, FAS occurs in the following frequencies based on the amount and frequency of alcohol ingestion during pregnancy:

  • 4 to 6 drinks*/day -- 40% FAS  
  • 2-4 drinks/day -- 11% FAS
  • 1 drink/day -- 1% FAS

*One drink is approximately 12 oz. beer, 4 oz. wine, or 1 oz. liquor/spirits.

Note: The effect of alcohol on the growing, developing embryo and fetus is still not completely understood. The exact mechanisms by which alcohol induces malformations may be a direct toxic effect of ethanol or a combination of ethanol and metabolite acetaldehyde.

Occurrence

  • Fetal Alcohol Syndrome: 0.5 - 2:1,000 live births (FAS is believed to be widely under-diagnosed)
  • Fetal Alcohol Spectrum Disorder and (Fetal Alcohol Effect/Alcohol Related Neurodevelopmental Disorder): 3-10:1000 live births

 Characteristic Features

  • Central nervous system dysfunction (80%+)
  • Ptosis, strabismus, epicanthal folds
  • Infantile irritability (80%+)
  • Impulsivity and hyperactivity (50%)
  • Poor coordination (50%)
  • Hypotonia (50%)
  • Decreased adipose tissue (50%)
  • Visual and hearing problems (20-50%)
  • Cardiac abnormalities (20-30%)
  • Hemangiomas (20-30%)
  • Skeletal abnormalities
  • Urogenital problems
  • Dental abnormalities

Diagnosis

  • History of maternal alcohol ingestion during pregnancy
  • Low birth weight (80%+)
  • Microcephaly (80%+)
  • Prenatal and postnatal growth deficiency (80%+)
  • Characteristic facial features (80%+) (small head size, narrow eye slits, flat midface, low nasal ridge, loss of groove between nose and upper lip)

Common Behavior & Emotional Consequences

  • Difficulty with habilitation
  • Difficulty with self regulation
  • Impulsivity
  • Attention Deficit Hyperactivity Disorder
  • Slow CNS processing
  • Difficulties in memory, abstracting and with arithmetic
  • Poor judgment
  • Time and space disorientation

 Common Associations

  • Attention Deficit Hyperactivity Disorder (50%)
  • Psychiatric disorders, such as depression and anxiety disorders
  • Cleft Lip +/- palate
  • Genital defects
  • Kidney defects
  • Alcohol and/or drug dependence

Secondary Disabilities

  • Alcohol and other substance abuse
  • Inappropriate sexual behaviors
  • Disrupted school experience
  • Mental health problems
  • Trouble with the law

MEDICAL MANAGEMENT CONSIDERATIONS
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Note: These considerations are in addition to the normal medical care provided to an individual without Fetal Alcohol Syndrome. In general, children with Fetal Alcohol Syndrome do not require significantly more than routine medical care. All recommendations can be addressed through clinical examination by the primary care provider, unless otherwise noted.

Ongoing (all ages)

  • Monitor for growth deceleration (if head growth decelerates, obtain brain imaging studies because this is unusual in FAS)
  • Obtain renal imaging studies if urinary tract infections or other urogenital problems appear
  • Refer for echocardiogram if clinical suspicion of anatomical or functional cardiac concern
  • Monitor for mental or behavioral change

 Infancy (Birth to 1 year)

  • Perform complete physical and neurological exam to detect congenital anomalies
  • Assess central nervous system anomalies (MRI)
  • Assess cardiac function
  • Assess vision
  • Assess hearing (BAER)
  • Refer to registered dietician for nutrition
  • Refer for physical and occupational therapy evaluation if needed
  • Discuss recurrence risk with birth mothers that may approach 100%
  • Refer to early intervention programs and Fetal Alcohol Syndrome/disability support groups
  • Discuss possibility of adoption or foster care for more stable social environment

 Childhood (1 to 13 years)

  • Assess vision every 2 years
  • Monitor speech and language progress
  • Refer for speech and language therapy evaluation if necessary
  • Refer for psychiatric evaluation if necessary
  • Consider stress and behavioral management training
  • Recommend and arrange for dental care
  • Refer for orthodontic treatment in late childhood if necessary
  • Monitor school progress
  • Minimize changes or disruptions in caregiver(s) and environment

 Adolescence and Adulthood (13 years and over)

  • Monitor for alcohol and/or drug abuse; counsel to avoid alcohol; refer to substance abuse counseling as needed
  • Monitor for mental or behavioral change; provide appropriate mental health interventions and referrals as needed
  • Provide education regarding sexual development
  • Minimize changes or disruptions in caregiver(s) and environment
  • Discuss alternative community living resources
  • Discuss long-term financial plans
  • Monitor prevocational training and vocational activities
  • Discuss community-supported employment opportunities

 Teaching Strategies

  • Place in calm, orderly environment
  • Establish clear, consistent rules
  • Use simple concrete instructions
  • Help student set realistic goals
  • Monitor performance to facilitate success
  • Avoid repeat failures and prevent loss of control
  • Balance structure with responsibility
  • Plan ahead for change and supervise transitions
  • Teach functional social skills
  • Work closely with student's family members
  • Take a life span approach
  • Develop good work habits and skills for the present and future

REFERENCES
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Peer-reviewed Journal Articles/Academies

American Academy of Pediatrics. (1993). Fetal Alcohol Syndrome and Fetal Alcohol Effects. Pediatrics 91(5), 1004-1006.

Bagheri, M. M., et al. (1998). Fetal Alcohol Syndrome: Maternal and Neonatal Characteristics. Journal of Perinatal Medicine 26(4), 263-269.

Centers for Disease Control and Prevention, National Center for Environmental Health, Division of Birth Defects and Developmental Disabilities. (2005).  FAS Guidelines for Referral and Diagnosis Retrieved on January 30, 2006 from  http://www.cdc.gov/

Johnson V.P., et al. (1996). Fetal Alcohol Syndrome: Craniofacial and Central Nervous System Manifestations. American Journal of Medical Genetics 61(4), 329-339.

National Academy of Sciences, Institute of Medicine. (1996). Fetal Alcohol Syndrome. Retrieved on January 30, 2006 from http://www.nap.edu/

Stratton, K., Howe, C., & Battaglia, F. (1996). Fetal Alcohol Syndrome: Diagnosis, Epidemiology, Prevention, and Treatment. National Academy of Sciences, Institute of Medicine, Committee to Study Fetal Alcohol Syndrome.

Special Interest Groups/Other Publications

Hagerman, R. J. (1999). Fetal Alcohol Syndome. In: Neurodevelopmental Disorders: Diagnosis and Treatment (pp. 3-60.) New York: Oxford University Press.

Leppert, M. & Hofman, K. (1996). In: Developmental Disabilities in Infancy and Childhood vol II: The Spectrum of Developmental Disabilities. In A.J. Capute, & P.J. Accardo (Eds) Baltimore: Paul H. Brookes Publishing Co.

National Organization on Fetal Alcohol Syndrome. (2005). What is Fetal Alcohol Syndrome (FAS). Retrieved on January 30, 2006 from http://www.nofas.org/

Streissguth, A. (1997). A Guide for Families and Communities: Fetal Alcohol Syndrome. Brooks Publishing.

RESOURCES
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California Department of Developmental Services
916-654-1690
http://www.dds.ca.gov
 
California Fetal Alcohol Spectrum Organization
http://www.calfas.org
 
California Regional Centers
http://www.dds.ca.gov/RC/RCList.cfm
 
The Clearinghouse for Drug Exposed Children (UC San Francisco)
415-476-9691
 
Exceptional Parent Magazine
800-247-8080
http://www.eparent.com
 
FAS/FAE Newsletter
907-456-2866
 
The Fetal Alcohol Network Newsletter
610-384-1133
 
Fetal Alcohol Syndrome Consultation, Education and Training Services, Inc.
http://www.fascets.org
 
The Fetal Alcohol Syndrome Family Resource Institute
253-531-2878
http://www.fetalalcoholsyndrome.org
 
March of Dimes Birth Defects Foundation
914-428-7100
http://www.modimes.org
 
National Clearinghouse for the Prevention of Perinatal Abuse of Alcohol and Other Drugs
800-354-8824
 
National Family Empowerment Network: Supporting Families Affected by FAS and FAE 800-462-5254
 
National Organization on Fetal Alcohol Syndrome
202-785-4585
http://www.nofas.org
 
Substance Abuse and Mental Health Services Administration
http://www.samhsa.gov
 
National Center on Birth Defects and Developmental Disabilities
404-498-3947
http://www.cdc.gov/ncbddd

PUBLICATION INFORMATION
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This document does not provide advice regarding medical diagnosis or treatment for any individual case, and any opinions or statements contained in this document are not intended to serve as a standard of medical care. Physicians are encouraged to view the considerations presented in this document in light of evolving scientific information. This document is not intended for use by the layperson. Reproduction of this document may be done with proper credit given to California Department of Developmental Services.

Last updated July 2008

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