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Orofacial Clefts at a Glance

  • Orofacial clefts are one of the most common birth defects in the United States occurring in 1 in 750 births.

  • Orofacial clefts affect 1 in 450 births in Utah. 

  • Utah has the highest incidence of orofacial clefts in the United States.

  • More than 100 affected children are born in Utah each year.  Of these, approximately 65 have cleft lip with or without cleft palate and 35 have cleft palate alone.

  • About 5,430 babies with an oral facial cleft are born each year in the United States.

  • The lifetime cost for medical treatment, educational services and lost productivity averages more than $101,000.

  • About 15% of all babies with oral clefts die before age 1, usually because of associated birth defects.

What are orofacial clefts?

Orofacial clefts are birth defects where mouth or roof of mouth do not form properly.  A cleft is a separation in a body structure, often resulting from the failure of tissues to grow together properly. Oral facial clefts may involve the lip, the roof of the mouth (hard palate) or the soft tissue in the back of the mouth (soft palate).

What are the major types of oral facial clefts?

Cleft lip is a separation of two sides of lip and often includes the bones of the maxilla and/or the upper gum.  A cleft lip varies from notch in lip to a cleft extending up into nose.  Sometimes there is a cleft or notch in the gum as well.

Cleft palate is an opening in roof of mouth.  The two sides of the palate fail to join together or fuse.  Most cleft palates involve the hard palate at the front of the roof of the mouth, but the soft palate at the back of the roof of the mouth can also have a cleft.
Cleft lip with or without a cleft palate and isolated cleft palate are two different conditions.  .Babies with cleft lip/palate have a cleft lip and sometimes a cleft palate. In isolated cleft palate, the cleft palate occurs by itself, without cleft lip or other malformations. These two forms of oral-facial clefts are considered separate birth defects. There is variability in the severity of oral facial clefts.

Embryology

The lip and palate develop separately, but both form very early in fetal development.  Oral facial clefts occur 5 to 9 weeks after conception if tissues in the developing mouth fail to merge together and fully fuse.

The cause of oral facial clefts is thought to be multifactorial due to genes as well as environmental factors.  Oral facial clefts are often associated with other birth defects as part of a syndrome. More than 200 syndromes are associated with orofacial clefts including Stickler’s syndrome, 22q11 deletion syndrome, Van der Woude syndrome.   Often syndromes associated with oral facial clefts have a recognized chromosomal or genetic cause.

Sometimes a cleft palate is part of the Pierre-Robin sequence.  Pierre-Robin sequence is characterized by a combination of three features thought to be interrelated: an abnormally small lower jaw is abnormally small (micrognathia), displacement of the tongue downwards (glossoptosis), and a cleft palate.

What is the impact of oral facial clefts?

Orofacial clefts generally require surgical repair.  Often multiple surgeries are needed to reconstruct the lip and palate. Orofacial clefts are associated with other health and medical problems.  These include feeding problems and sometimes airway issues; ear infections; speech problems; and dental or orthodontic problems.
The medical costs associated with orofacial clefts are substantial. Just the direct medical costs to treat a child with cleft lip and palate was estimated to be over 22,000 dollars (in 2002 dollars, N. Waitzman, 2005). For the estimated 100 children born with these conditions every year in Utah, their direct medical costs would therefore be in the order of 2.2 million dollars.  When the costs of educational resources and lost productivity are added, the average lifetime cost for an individual with an oral facial cleft is estimated to be $101,000.
About 15% of all babies with oral clefts die before age 1, usually because of associated birth defects. Data from the California Birth Defects Monitoring  Program suggests that about one in four of those with cleft lip (with/without cleft palate) and half with only cleft palate have other major birth defects.

 Can oral facial clefts be prevented?

Several studies have suggested that multivitamins containing folic might lower the risk of having a child with an orofacial cleft. The reduction in risk may be as high as 25 to 50%.   It is recommended that all women of child-bearing age who may become pregnant take 400mcg of folic acid daily at least one month before becoming pregnant.

Smoking in pregnancy increases the risk of having a baby with orofacial cleft.  Don’t smoke during pregnancy.

How are orofacial clefts detected?

Sometimes orofacial clefts are diagnosed by prenatal ultrasound, but there is no systematic screening for orofacial clefts.  Most clefts are readily diagnosed in the newborn period, although sometimes a cleft of the soft palate is not recognized.

How common are orofacial clefts in Utah?

Orofacial clefts affect 1 in 450 births in Utah.  For reasons that are not fully understood, Utah has the highest incidence of orofacial clefts in the United States.

More than 100 affected children are born in Utah each year.  Of these, approximately 65 have cleft lip with or without cleft palate and 35 have cleft palate alone. 

From 1995 to 2004, more than 900 cases of orofacial clefts were identified. The rate of cleft lip with or without cleft palate was approximately twice that of cleft palate alone.

Rates of orofacial clefts are similar among Utah whites, Hispanics, and Asians (Figure 2). The rate among Native Americans may be higher but the numbers are too small to be certain.  (Figure 2) The number of affected births among African Americans is also too small to make conclusions.

What is the role of UBDN in tracking and preventing orofacial clefts?

Since 1995, the UDOH Utah Birth Defects Network (UBDN) has been tracking rates of orofacial clefts, evaluating their origin, and, more recently, researching their causes.
Current activities of the UBDN include

  1. tracking rates across the state and in different population groups;
  2. working with national and international partners in evaluating potential reasons for the high rate in Utah;
  3. searching for further clues on causes. Researchers in Utah are also planning to estimate the medical costs associated with orofacial clefts and to assess the quality of life of affected children and families.

What services are available for families of a child with an orofacial cleft?

A multidisciplinary team approach is important for the care of children with oral facial clefts.  The following team is recognized by Cleft Palate Foundation
Cleft Palate / Craniofacial Clinic
Primary Children’s Medical Ctr
100 N Medical Dr
Salt Lake City, UT 84113
Phone:801.588.2787
Alt. Phone: 801.588.2670
Fax: 801.588.3633
Email: leann.roling@ihc.com

Developmental services from birth to three years of age are provided to qualifying infants and children with orofacial clefts by the Utah Early Intervention Program, located within the Bureau of Children with Special Health Care Needs, Utah Department of Health. Services include child health assessment, service coordination, occupational and physical therapy, and speech and language therapy. Information on these services is available at: http://www.utahbabywatch.org
Developmental and educational services for children ages three and older are provided by local school districts for children who qualify.
A further resource is the Utah Collaborative Medical Home, which is a project designed to provide information, tools, and resources for Primary Care Physicians to enhance their ability to care for children with special health care needs. Information on the Utah Collaborative Medical Home is available at their web site: http://www.medhomeportal.org

Orofacial Support Groups and Information Resources

About Face USA
P.O. Box 158
South Beloit, IL 61080-0158
Phone:  (888) 486-1209 (toll-free)
AboutFaceUSA@comcast.net

Children's Craniofacial Association
13140 Coit Road, Suite 307
Dallas, TX 75240
Phone:  (800) 535-3643 or (214) 570-9099
CCA@ccakids.com

FACES: The National Craniofacial Association
P. O. Box 11082
Chattanooga, TN 37401
Phone:  (800) 332-2373
faces@mindspring.com

American Cleft Palate-Craniofacial Association (ACPA)
1504 East Franklin St., Suite 102
Chapel Hill, NC 27514-2820
Phone:  (919) 933-9044
Fax:  (919) 933-9604
http://www.acpa-cpf.org/

The Cleft Palate Foundation (CPF)
CLEFTLINE: 1-800-24-CLEFT (24 hours, toll free)
http://www.cleftline.org/

Let's Face It
Box 29972
Bellingham, WA 98228-1972
Phone:  (360) 676-7325
letsfaceit@faceit.org

Cleft Advocate
5201 Sequin Drive
Las Vegas, NV  89130
Phone: 702.228.8662
Fax: 702.341.5351
Website:  http://www.cleftadvocate.org
E-mail:  debbie@cleftadvocate.org

AboutFace USA/cleftAdvocate
PO Box 969
Batavia, IL  60510-0969
Phone: 888. 486.1209
Fax: 630.761.2985
Website:  www.aboutfaceusa.org
E-mail:  AboutFaceUSA@comcast.net