$Unique_ID{BRK03609} $Pretitle{} $Title{Cleft Palate and Cleft Lip} $Subject{Cleft Palate and Cleft Lip Hare Lip Cheiloschisis} $Volume{} $Log{} Copyright (C) 1984, 1985, 1986, 1989, 1990, 1992 National Organization for Rare Disorders, Inc. 32: Cleft Palate and Cleft Lip ** IMPORTANT ** It is possible that the main title of the article (Cleft Palate and Cleft Lip) is not the name you expected. Please check the SYNONYMS listing to find the alternate name and disorder subdivisions covered by this article. Synonyms Hare Lip Cheiloschisis General Discussion ** REMINDER ** The Information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Cleft Lip and Palate are common malformations that are noticeable at birth (congenital). A cleft is an incomplete closure of the palate or lip, or both. This defect is caused when the pair of long bones that form the upper jaw (maxillae) do not fuse properly during the development of the embryo. The cleft may be barely noticeable or result in severe deformities requiring surgical correction. Symptoms There are several varieties of Cleft Lip and Palate malformations. Over 200 syndromes have cleft lip and/or palate as a feature. The most severe types involve the lip, gum, bone, and the soft and hard palates. Less severe clefts may involve only one of these structures. Clefts may occur on one or both sides of the lip and/or palate. Children with Cleft Lip and Palate usually have a flat nose and lips that appear spread out or splayed. Generally there are abnormalities in dental development such as absent, extra or deformed teeth. Other features may include abnormal growth of the cartilage on one or both sides of the nose (ipsilateral alar nasal cartilage), and a wider than normal space between the eyes (mild ocular hypertelorism). Children with this disorder are at increased risk for middle ear infections (otitis media) and hearing loss. Speech may be impaired. Causes When Cleft Lip and Palate occur as part of another syndrome, the cause is that of the syndrome. (For disorders that have Cleft Palate as a symptom of the disorder, choose "Cleft Palate" as your search term in the Rare Disease Database). When Cleft Lip and Palate occur in isolation, the cause is less clear. Both a genetic change (mutation) and environmental factors may be involved. Affected Population Cleft Lip and Palate affect more than 5500 newborns each year in the United States. The frequency in newborn infants is 1:700, but this ratio varies according to ethnic background. Orientals are the most likely to be affected while Americans of African descent are the least likely to be affected. Males are at least twice as likely as females to be affected by cleft lip, but females are more prone to cleft palate. Therapies: Standard The treatment of Cleft Lip and/or Palate requires the coordinated efforts of a team of specialists. Pediatricians, dental specialists, surgeons, speech pathologists, psychologists and others must systematically and comprehensively plan the child's treatment and rehabilitation. Cleft lip may be surgically corrected. Generally surgeons repair the lip when the child is still an infant. A second surgery is sometimes necessary for cosmetic purposes when the child is older. Cleft palate may be repaired by surgery or covered by an artificial device (prosthesis) that closes or blocks the opening. Surgical repair can be carried out in stages or in a single operation, according to the nature and severity of the defect. The first palate surgery is usually scheduled during the toddler period. With adequate assistance, many children with cleft palates speak as well as other children by the time they enter school. Speech and language development need to be assessed by a speech pathologist during the preschool years. Dental problems associated with cleft palate are generally correctable. Braces and orthodontic appliances are frequently needed later to straighten teeth that have grown in crooked. Routine testing of hearing should be scheduled for all preschool children with clefts of the palate. They may require tubes placed in their ears to drain congestion and improve hearing. Therapies: Investigational Researchers continue to study the genetic causes of Cleft Lip and Palate. New surgical and dental corrective procedures are being researched and developed. Other studies continue to concentrate in the areas of facial and psychological development and other associated problems with speech, chewing and swallowing. Although the palate of cleft palate patients generally closes during early childhood, difficulties may persist if the palate is excessively short in relation to the throat (pharynx). Researchers are studying a teflon- glycerine paste that is applied to the rear of the pharynx in a minor surgical procedure. A rounded bump or ledge is formed, bringing the pharynx and palate into the proper relationship with each other. The hardened paste remains in place indefinitely; no side effects have been observed. Children as young as eight years old have been treated with this procedure. For further information on this procedure contact: William N. Williams, D.D.S. University of Florida College of Dentistry Box J-424 Gainesville, FL 32610 (904) 392-4370 Research on birth defects and their causes is ongoing. The National Institutes of Health (NIH) is sponsoring the Human Genome Project that is aimed at mapping every gene in the human body and learning why they sometimes malfunction. It is hoped that this new knowledge will lead to prevention and treatment of genetic disorders in the future. This disease entry is based upon medical information available through October 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Cleft Lip and Cleft Palate National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Forward Face 560 First Ave. New York, NY 10016 (212) 263-5205 (800) 422-FACE A Cleft Palate Team is a group of specialists who are primarily interested in the care of children having clefts. For information about local teams contact: The Cleft Palate Foundation, Inc. 1218 Granview Ave. Pittsburgh, PA 15211 (800) 24-CLEFT (800) 23-CLEFT FACES National Association for the Craniofacially Handicapped P.O. Box 11082 Chattanooga, TN 37401 (615) 266-1632 National Craniofacial Foundation 3100 Carlisle St., Suite 215 Dallas, TX 75204 (800) 535-3643 Craniofacial Family Association 170 Elizabeth Street Suite 650, Toronto, Ontario MG5G1X8 Canada Society for the Rehabilitation of the Facially Disfigured, Inc. 550 First Ave. New York, NY 10016 (212) 340-5400 About Face 123 Edward Street Toronto, Ontario M5G 1X8 Canada Let's Face It Box 711 Concord, MA 01742 (508) 371-3186 NIH/National Institute of Dental Research (NDR) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4261 For Genetic Information and Genetic Counseling Referrals: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References SMITH'S RECOGNIZABLE PATTERNS OF HUMAN MALFORMATION, 4th ed.: Kenneth Lyons Jones M.D.; W. B. Saunders Company, 1988. Pp. 196-199. MENDELIAN INHERITANCE IN MAN, 10th Ed.: Victor A. McKusick, Editor: Johns Hopkins University Press, 1992. Pp. 230-231. BIRTH DEFECTS ENCYCLOPEDIA, Mary Louise Buyse, M.D., Editor-In-Chief; Blackwell Scientific Publications, 1990. Pp. 405-406, 410-411. NELSON TEXTBOOK OF PEDIATRICS, 14TH Ed.; Richard E. Behrman, Editors; W.B. Saunders Company, 1991. Pp. 926-927. MANAGING THE CLEFT LIP AND PALATE PATIENT, F.L. Kaufman; Pediatr Clin North Am (Oct 1991; 38(5)): Pp. 1127-1147.