Massive hemorrhage in patient with Klippel-Trenaunay-Weber syndrome
DOI:
https://doi.org/10.25751/rspa.8845Keywords:
Klippel-Trenaunay-Weber syndrome, Massive hemorrhage, angiodysplasia, aneurysmectomy, femoral-popliteal bypass surgery, coagulopathy, introperative Cell-SaverAbstract
Klippel Trenaunay Weber syndrome (SKTW) is rare congenital disease characterized by cutaneous hemangioma, bone and soft tissue hypertrophy and venous/lymphatic abnormalities related with arterio-venous malformations and coagulopathy.
There are about 1000 case reports found in the literature, of which only 12 include anesthetic approach.
The authors report a case of a patient diagnosed with SKTW submitted to a right superficial femoral artery aneurysmectomy and femoral-popliteal bypass surgery, under general anesthesia complicated with massive hemorrhage (total blood loss of 8050mL against 500-1000mL estimated for this kind of procedure), due to angiodisplasia of right leg.
The authors discuss an aggressive approach to massive blood loss, mainly regarding the use of introperative Cell-Saver and fluid rapid infusers (Level 1 ®).
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