title: Parkes Weber syndrome-Diagnostic and management paradigms: A systematic review.
abstract: Objectives Parkes Weber syndrome is a congenital vascular malformation which consists of capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation. Although Parkes Weber syndrome is a clinically distinctive entity with serious complications, it is still frequently misdiagnosed as Klippel-Trenaunay syndrome that consists of the triad capillary malformation, venous malformation, and lymphatic malformation. Methods We performed a systematic review investigating clinical, diagnostic, and treatment modalities of Parkes Weber syndrome (PubMed/MEDLINE, Embase, and Cochrane databases). Thirty-six publications (48 patients) fulfilled the eligibility criteria. Results The median age of patients was 23 years (IQR, 8-32), and 24 (50.0%) were males. Lower extremity was affected in 42 (87.5%) and upper extremity in 6 (12.5%) patients; 15 (31.3%) patients developed high-output heart failure; 12 (25.0%) patients had chronic venous ulcerations, whereas 4 (8.3%) manifested distal arterial ischemia. The spinal arteriovenous malformations were reported in six (12.5%) patients and coexistence of aneurysmatic disease in five (10.4%) patients. The most frequently utilized invasive treatments were embotherapy followed by amputation and surgical arteriovenous malformation resection, and occasionally stent-graft implantation. All modalities showed clinical improvement. However, long follow-up and outcome remained unclear. Conclusion A diagnosis of Parkes Weber syndrome should be made on the presence of capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation (as main defect) in overgrowth extremity. Arteriovenous malformation presents the criterion for distinguishing Parkes Weber syndrome from Klippel-Trenaunay syndrome, which is substantial for treatment strategy. The primary management goal should be patient's quality of life improvement and complication reduction. Embolization alone/combined with surgical resection targeting occlusion or removal of arteriovenous malformation "nidus" reliably leads to clinical improvement.
authors: 1 Faculty of Medicine, University of Belgrade, Belgrade, Serbia.|2 Clinic for Vascular and Endovascular Surgery, Clinical Center of Serbia, Belgrade, Serbia.
authors: Igor
authors: I
authors: Banzic
authors: 3 Department of Cardiology, Erasmus MC, Thoraxcenter, Rotterdam, The Netherlands.
authors: Milos
authors: M
authors: Brankovic
authors: 1 Faculty of Medicine, University of Belgrade, Belgrade, Serbia.|2 Clinic for Vascular and Endovascular Surgery, Clinical Center of Serbia, Belgrade, Serbia.
authors: Živan
authors: Ž
authors: Maksimović
authors: 1 Faculty of Medicine, University of Belgrade, Belgrade, Serbia.|2 Clinic for Vascular and Endovascular Surgery, Clinical Center of Serbia, Belgrade, Serbia.
authors: Lazar
authors: L
authors: Davidović
authors: 1 Faculty of Medicine, University of Belgrade, Belgrade, Serbia.|2 Clinic for Vascular and Endovascular Surgery, Clinical Center of Serbia, Belgrade, Serbia.
authors: Miroslav
authors: M
authors: Marković
authors: 4 Clinic for Cardiovascular Surgery, University Hospital Zurich, Zurich, Switzerland.
authors: Zoran
authors: Z
authors: Rančić
mesh_terms: D000293:Adolescent; D000328:Adult; D001165:Arteriovenous Malformations / Q000175:diagnosis / Q000628:therapy; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000175:diagnosis*; D044148:Lymphatic Abnormalities / Q000175:diagnosis / Q000628:therapy; D008297:Male; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular; D011788:Quality of Life; D012306:Risk; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000628:therapy*; D055815:Young Adult
keywords: Klippel–Trenaunay syndrome; Parkes Weber syndrome; arteriovenous malformation; congenital vascular malformation