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Parkes Weber syndrome-Diagnostic and management paradigms: A systematic review.
Phlebology. 2017 Jul; 32(6):371-383.P

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+Show Affiliations

1 Faculty of Medicine, University of Belgrade, Belgrade, Serbia. 2 Clinic for Vascular and Endovascular Surgery, Clinical Center of Serbia, Belgrade, Serbia.3 Department of Cardiology, Erasmus MC, Thoraxcenter, Rotterdam, The Netherlands.1 Faculty of Medicine, University of Belgrade, Belgrade, Serbia. 2 Clinic for Vascular and Endovascular Surgery, Clinical Center of Serbia, Belgrade, Serbia.1 Faculty of Medicine, University of Belgrade, Belgrade, Serbia. 2 Clinic for Vascular and Endovascular Surgery, Clinical Center of Serbia, Belgrade, Serbia.1 Faculty of Medicine, University of Belgrade, Belgrade, Serbia. 2 Clinic for Vascular and Endovascular Surgery, Clinical Center of Serbia, Belgrade, Serbia.4 Clinic for Cardiovascular Surgery, University Hospital Zurich, Zurich, Switzerland.

Pub Type(s)

Journal Article
Review
Systematic Review

Language

eng

PubMed ID

27511883

Citation

Banzic, Igor, et al. "Parkes Weber syndrome-Diagnostic and Management Paradigms: a Systematic Review." Phlebology, vol. 32, no. 6, 2017, pp. 371-383.
Banzic I, Brankovic M, Maksimović Ž, et al. Parkes Weber syndrome-Diagnostic and management paradigms: A systematic review. Phlebology. 2017;32(6):371-383.
Banzic, I., Brankovic, M., Maksimović, Ž., Davidović, L., Marković, M., & Rančić, Z. (2017). Parkes Weber syndrome-Diagnostic and management paradigms: A systematic review. Phlebology, 32(6), 371-383. https://doi.org/10.1177/0268355516664212
Banzic I, et al. Parkes Weber syndrome-Diagnostic and Management Paradigms: a Systematic Review. Phlebology. 2017;32(6):371-383. PubMed PMID: 27511883.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Parkes Weber syndrome-Diagnostic and management paradigms: A systematic review. AU - Banzic,Igor, AU - Brankovic,Milos, AU - Maksimović,Živan, AU - Davidović,Lazar, AU - Marković,Miroslav, AU - Rančić,Zoran, Y1 - 2016/08/09/ PY - 2016/8/12/pubmed PY - 2018/10/31/medline PY - 2016/8/12/entrez KW - Klippel–Trenaunay syndrome KW - Parkes Weber syndrome KW - arteriovenous malformation KW - congenital vascular malformation SP - 371 EP - 383 JF - Phlebology JO - Phlebology VL - 32 IS - 6 N2 - 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. SN - 1758-1125 UR - https://www.unboundmedicine.com/medline/citation/27511883/Parkes_Weber_syndrome_Diagnostic_and_management_paradigms:_A_systematic_review_ L2 - http://journals.sagepub.com/doi/full/10.1177/0268355516664212?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -