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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Parkes Weber syndrome


Other Names for this Disease
  • Cutaneous flush with underlying multiple micro arteriovenous fistulas, soft tissue and skeletal hypertrophy of the affected limb
  • PKWS
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Treatment


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How might Parkes Weber syndrome be treated?

For capillary malformations (such as port wine stains) that are of cosmetic concern, individuals may be referred to a dermatologist. For arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs), the risks and benefits of intervention (i,e, embolization versus surgery) may be considered, usually with input from a multi-disciplinary team (e.g., specialists in interventional radiology, neurosurgery, surgery, cardiology, and dermatology). For risks associated with heart failure, referral to a cardiologist may be warranted. Hypertrophy (overgrowth) of the limb and/or difference in size between limbs may be treated surgically by an orthopedist.[1]

Supportive care may include compression garments (tight-fitting pieces of clothing on the affected limb to reduce pain and swelling); these may also protect the limb from bumps and scrapes, which can cause bleeding. Heel inserts may be used if the legs are different lengths, which can aid in walking normally. Various pain medications and antibiotic medications may be prescribed as needed.[2]
Last updated: 6/9/2011

References
  1. Pinar Bayrak-Toydemir, David Stevenson. RASA1-Related Disorders. GeneReviews. February 22, 2011; http://www.ncbi.nlm.nih.gov/books/NBK52764/. Accessed 6/9/2011.
  2. Parkes Weber syndrome. Children's Hospital Boston. 2011; http://childrenshospital.org/az/Site2919/mainpageS2919P1.html. Accessed 6/8/2011.


Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Parkes Weber syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.