TY - JOUR
T1 - Fibroadipose vascular anomaly treated with sirolimus
T2 - Successful outcome in two patients
AU - Erickson, Jonathan
AU - McAuliffe, William
AU - Blennerhassett, Lewis
AU - Halbert, Anne
PY - 2017/11/1
Y1 - 2017/11/1
N2 - Fibroadipose vascular anomaly (FAVA) is a rare, complex mesenchymal malformation combining fibrofatty replacement of the affected muscles and slow-flow vascular malformation. The condition is characterized by localized swelling, severe pain, phlebectasia, and contracture of the affected limb. Treatment paradigms are not well established for this rare, recently recognized condition. We report two cases of FAVA in which treatment with sirolimus produced rapid, dramatic improvement in pain and quality of life.
AB - Fibroadipose vascular anomaly (FAVA) is a rare, complex mesenchymal malformation combining fibrofatty replacement of the affected muscles and slow-flow vascular malformation. The condition is characterized by localized swelling, severe pain, phlebectasia, and contracture of the affected limb. Treatment paradigms are not well established for this rare, recently recognized condition. We report two cases of FAVA in which treatment with sirolimus produced rapid, dramatic improvement in pain and quality of life.
KW - fibroadipose vascular anomaly
KW - medical therapy
KW - pediatric dermatology
KW - sirolimus
KW - vascular malformation
U2 - 10.1111/pde.13260
DO - 10.1111/pde.13260
M3 - Article
C2 - 29144050
SN - 0736-8046
VL - 34
SP - E317-E320
JO - Pediatric Dermatology
JF - Pediatric Dermatology
IS - 6
ER -