Credit: Sadanandan Mohan, George Varghese, Sanjay Kumar ,Dinesh P. Subramanian, Department of Oral and Maxillofacial Surgery, Government Dental College, Kottayam at NJMS
Parks Weber Syndrome is defined as a disorder of the vascular system, a complex network of blood vessels in the body1. The vascular system consists of arteries, veins and capillaries. Arteries carry blood rich in oxygen from the heart to various organs and tissues in the body; veins carry the blood back to the heart, whereas, capillaries are tiny blood vessels connecting arteries and veins. A characteristic feature of Parks Weber syndrome is vascular abnormalities which are present from birth. Vascular abnormalities include capillary malformation and arteriovenous fistulas (
AVFs are either present from birth, surgically created for hemodialysis treatment or acquired due to trauma or erosion. Hemodialysis is a treatment for kidney failure, doing some of the work your kidneys did when they were healthy. Your blood travels through tubes from your body, during treatment, into the dialysis machine where it goes through a filter which removes extra fluid and waste. Once the blood is cleaned, it travels back into your body from the dialysis machine with the help of tubes. In addition, the blood flow near the surface of the skin increases because of capillary malformations making them look like large, pink and flat stains on the skin. Capillary malformations are also called “port-wine stains” sometimes because of their color2.
The capillary malformations occur with multiple micro-AVFs, affecting the blood circulation. The AVFs are often associated with heart failure and abnormal bleeding, both of which are life-threatening complications. Other characteristic features of Parks Weber syndrome include overgrowth of a limb which is mostly common in one of the legs. Nonetheless, one of the limbs is larger than the corresponding one because of an abnormal growth of soft tissues and bones. The symptoms of Parks Weber syndrome are similar to those that occur in capillary malformation-arteriovenous malformation (CM-AVM). In many cases, CV-AVM and Parks Weber syndrome have the same genetic cause.
The signs and symptoms of Parkes Weber syndrome are birthmarks on the skin, caused by capillary malformations. The malformations look like pink, flat and large stains since they increase the blood flow near the surface of the skin. They are sometimes called port-wine stains because of the color. Other signs include excessive growth or hypertrophy of the affected limb, soft tissue and bone, for this reason, there is a difference in size between the non-affected and affected limb. The abnormal connections between the arteries and veins cause multiple arteriovenous fistulas, which can be associated as a life-threatening complication including heart failure and abnormal bleeding.
References
- Kumar, V., Jorwal, P., Biswas, A., & Deorari, V. (2019). Parkes Weber syndrome. QJM: An International Journal of Medicine.
- Banzic, I., Brankovic, M., Maksimović, Ž., Davidović, L., Marković, M., & Rančić, Z. (2017). Parkes Weber syndrome—Diagnostic and management paradigms: A systematic review. Phlebology, 32(6), 371-383.
- Stefan Stefanov-Kiuri, M.D.Alvaro Fernandez-Heredero, M.D., Parkes Weber Syndrome (2015), The New England Journal of Medicine.
- https://nejm.org
