Hemihypertrophy (Hemihyperpla... Health Article

Treatment and management

The treatment for hemihypertrophy is different for each individual and depends on the specific symptoms. If leg-length differences are present, corrective shoes can increase the sole for the unaffected leg to prevent scoliosis and walking difficulties. Orthopedic devices such as braces or, more rarely, surgery to lengthen the normal leg may be indicated. Surgery to retard growth of the overgrown leg is controversial and not recommended. Surgery for congenital defects or laser surgery for birth marks may be indicated. Plastic surgery may be considered to correct very discrepant facial features.

A protocol to screen for childhood cancers has been proposed, which includes abdominal ultrasound every three months until age six, every six months until puberty, and careful medical follow-up of patients into adulthood. Surgical intervention is appropriate if cancers are detected. Monitoring of serum alpha fetoprotein levels may also be useful as a marker of hepatic tumors.

Appropriate special education services are necessary for those with mental retardation. Counseling related to social stigmatism may be necessary if severe disfigurement is an issue.

Prognosis

Hemihypertrophy does not alter life span, although complications from associated abnormalities such as childhood cancer and mental retardation can cause problems. Asymmetry of the limbs can interfere with their proper function and cause pain. Insecurities due to disfigurement are possible and can be addressed through support groups or therapy.

BOOKS

Buyse, M. L., ed. "Hemihypertrophy." Birth Defects Encyclopedia. Boston: Blackwell Scientific Publications, 1990.

Goodman, R. M., and R. J. Gorlin. "Hemihypertrophy." The Malformed Infant and Child. New York: Oxford University Press, 1983.

PERIODICALS

Biesecker, L. G., et al. "Clinical Differentiation Between Proteus Syndrome and Hemihyperplasia: Description of a Distinct Form of Hemihyperplasia." American Journal of Medical Genetics 79(1998): 311-318.

Hoyme, H. E., et al. "Isolated Hemihyperplasia (Hemihypertrophy): Report of a Prospective Multicenter of the Incidence of Neoplasia and Review." American Journal of Medical Genetics 79(1998): 274-278.

ORGANIZATIONS

Klippel-Trenaunay Support Group. 5404 Dundee Rd., Edina, MN 55436. (612) 925-2596.

Proteus Syndrome Foundation. 6235 Whetstone Dr., Colorado Springs, CO 80918. (719)264-8445. abscit@aol.com. <http://www.kumc.edu/gec/support/proteus.html>.

WEBSITES

"Hemihypertrophy." Online Mendelian Inheritance in Man. <http://www.ncbi.nlm.nig.gov/entrez/dispomim.cgi?id=235000>.

National Organization of Rare Disorders. <http://www.rarediseases.org>.

Amy Vance, MS, CGC