Analysis of Hemodynamics by MR-Angio and Embolization of Klippel-Trenaunay-Weber Syndrome: Five Year Follow-up

Abstract

A newborn male child was admitted with hypoxemia, high output failure, hypertrophic right lower limb with a segmental pink naevus flammeus, lymphatic edema, subcutaneous varices and multiple arteriovenous fistulae (AVF) spinal and pelvine; findings of the Klippel-Trenaunay- / Parkes-Weber-Overlap Syndrom (KTW). Because of a threatening right heart insufficiency caused by the shuntvolume, the child was embolized as early as 5 months of age. In the course of 5 years, a total of 17 spinal and pelvic feeding arteries were occluded in 6 sessions using Histoacryl, Platincoils or Ethibloc. The low weight of the child's body (4.3 kg) and cardiac insufficiency (CI) limited the amount of contrast agent and fluid. To reduce the amount of contrast agent used for diagnostic purposes we analyzed the pathological hemodynamics by magnetic resonance angiography (MRA). Within 5 years normal bodily development took place with no sings of exponential growth of the affected leg. We understand the “newly formed” fistulae in the current angiography as a local hemodynamic activation of persistent primitive arteriovenous communication caused by mesodermal disturbances, the degree of which correlate with the prognosis.