PAMJ Clinical Medicine (Jun 2020)

Diagnosis of Parkes Weber syndrome affecting a newborn baby´s upper left limb in a low resource setting: a case report

  • Francklin Djifack Tétinou,
  • Aimé Noula Mbonda,
  • Simplice Penka Takoupo,
  • Nadia Adjifack Tétinou,
  • Gilles Libeng Nong,
  • Stéphane Nguembu

DOI
https://doi.org/10.11604/pamj-cm.2020.3.74.24463
Journal volume & issue
Vol. 3, no. 74

Abstract

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Vascular malformations (VMs) is a term used to describe vessels developmental abnormalities. These malformations involve arteries, veins, capillaries or lymphatics vessels. It can result to high-flow arteriovenous malformations (AVMs), associated with shunting of large amounts of arterial blood into the venous system; these malformations can lead to venous engorgement, and lately to high-output cardiac failure. Parkes Weber syndrome (PWS) is a form of VMs in which we have significant arteriovenous shunts. Patient with PWS have dilated and frequently visible pulsate varicose veins with other visible signs of arteriovenous shunt. There is frequent bones involvement. PWS is still frequently misdiagnosed as Klippel-Trenaunay syndrome, which is a triad of malformations involving the capillary, venous, and lymphatic vessels, without an arteriovenous fistula. Most cases of PWS are sporadic; however some few cases are caused by mutations in the RASA1 gene. Here we report a case of congenital PWS of the left upper limb in a low income country.

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