caa a22 4500 606246282 CHVBK 20210128101339.0 cr unu---uuuuu 210128e20150401xx s 000 0 eng 10.1007/s12262-014-1201-8 doi (NATIONALLICENCE)springer-10.1007/s12262-014-1201-8 Parkes Weber Syndrome Involving Right Lower Limb: A Case Report [Elektronische Daten] [Benod Kondapavuluri, R. Bharadwaj, Shama Shaikh, Akshay Chand, Vidyasagar Chaturvedi] Vascular malformations (VMs) are developmental abnormalities of the vascular system. Malformations may involve any segment of the vascular tree: arteries, capillaries, veins or lymphatics. High-flow arteriovenous malformations (AVMs) are associated with shunting of large amounts of arterial blood into the venous system; these lesions can have dynamic and dramatic hemodynamic manifestations, such as massive arteriolisation with gross venous engorgement, organomegaly of concerned anatomical region and high-output cardiac failure. Patients with Parkes Weber syndrome have clinically significant micro- or macrofistulous arteriovenous shunts, affecting usually one extremity. The patient has dilated, frequently visible pulsatile varicose veins and other visible signs of AV shunting. The abnormality is sporadic; it is likely a somatic mutation. There is frequent intraosseous involvement. The presence of high AV shunts differentiates Parkes Weber syndrome from Klippel-Trenaunay syndrome which is a clinical triad of capillary malformation, soft tissue and bone hypertrophy and venous and lymphatic malformations. The mutations in RASA 1 gene leads to Parkes Weber syndrome characterised by the presence of multiple, small (1-2cm in diameter) capillary malformations mostly localized on the face and limbs. We report a case of congenital AVM of the lower limb causing cardiac decompensation. The patient was safely and successfully treated by performing a knee disarticulation. Association of Surgeons of India, 2014 Vascular malformations nationallicence Arteriovenous shunts nationallicence Massive arteriolisation nationallicence Cardiomegaly nationallicence Parkes Weber syndrome nationallicence Kondapavuluri Benod Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut Bharadwaj R. Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut Shaikh Shama Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut Chand Akshay Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut Chaturvedi Vidyasagar Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut Indian Journal of Surgery Springer India 77(2015-04-01), 130-134 0972-2068 77<130 2015 77 12262 https://doi.org/10.1007/s12262-014-1201-8 text/html Onlinezugriff via DOI BK010053 XK010053 XK010000 Metadata rights reserved Springer special CC-BY-NC licence nationallicence 1 research-article jats NATIONALLICENCE NATIONALLICENCE NL-springer NATIONALLICENCE 856 40 https://doi.org/10.1007/s12262-014-1201-8 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kondapavuluri Benod Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut NATIONALLICENCE 700 1- Bharadwaj R. Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut NATIONALLICENCE 700 1- Shaikh Shama Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut NATIONALLICENCE 700 1- Chand Akshay Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut NATIONALLICENCE 700 1- Chaturvedi Vidyasagar Dr. D.Y. Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India aut NATIONALLICENCE 773 0- Indian Journal of Surgery Springer India 77(2015-04-01), 130-134 0972-2068 77<130 2015 77 12262