Munson Health
 
Achondroplasia

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by Alan R

(Achondroplastic Dwarfism)

 

Treatment

Unfortunately, there is currently no treatment that can cure this condition. Scientists are exploring ways to create alternate growth factors which can bypass the missing receptor and lead to normal bone growth. They may offer the possibility of enhanced stature to future families who have children with achondroplasia.

Medication

Treatment with human growth hormone has been used for over a decade. It effectively increases bone growth rate, at least in the first year of life. There have been few studies looking at whether children treated with growth hormone achieve greater adult height.

Surgery

Surgery is sometimes needed to correct specific skeletal deformities.
  • Spinal fusion —a surgery to permanently connect otherwise separate vertebrae
  • Laminectomy —a surgery to open the spinal canal to relieve pressure on the compressed spinal cord from spinal stenosis
  • Osteotomy—the bones of the leg are cut and allowed to heal in the correct anatomical position. This procedure is for patients with severe knock-knee or bowed legs.
Osteotomy has primarily been used to correct deformities. In recent years, bone lengthening procedures have been used for many short children, including those with achondroplasia. The procedures are lengthy, traumatic, and demanding for both children and their families. Complications, sometimes serious, are common. One center has reported an average leg length gain of about seven inches and an average increase in arm length of about four inches for achondroplastic individuals who undergo surgery. The combination of growth hormone therapy followed by lengthening surgery may provide benefit in achieving near-normal stature and proportions.
 

RESOURCES

American Academy of Pediatrics
http://www.healthychildren.org

Little People of America
http://www.lpaonline.org

 

CANADIAN RESOURCES

Canadian Paediatrics Society
http://www.cps.ca

Little People of Ontario
http://www.lpo.on.ca/

 

References


Achondroplasia. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated January 10, 2011. Accessed July 24, 2013.


Aldegheri R, Dall'Oca C. Limb lengthening in short stature patients. J Pediatr Orthop B . 2001 Jul;10(3):238-47.


Aviezer D, Golembo M, Yayon A. Fibroblast growth factor receptor-3 as a therapeutic target for achondroplasia--genetic short limbed dwarfism. Curr Drug Targets . 2003;4(5):353-65.

 

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