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Year : 2004  |  Volume : 70  |  Issue : 2  |  Page : 119-122

Klippel trenaunay parkes-Weber syndrome

Departments of Dermatology & Sexually Transmitted Diseases, Lady Hardinge Medical College & Associated S.S.K. & K.S.C. Hospitals, New Delhi

Correspondence Address:
SEC 28 HNO 466, Noida, UP, 201303
[email protected]

How to cite this article:
Mendiratta V, Koranne R V, Sardana K, Hemal U, Solanki R S. Klippel trenaunay parkes-Weber syndrome. Indian J Dermatol Venereol Leprol 2004;70:119-22

How to cite this URL:
Mendiratta V, Koranne R V, Sardana K, Hemal U, Solanki R S. Klippel trenaunay parkes-Weber syndrome. Indian J Dermatol Venereol Leprol [serial online] 2004 [cited 2021 Jan 20];70:119-22. Available from:

A 5-year-old girl was referred with the complaints of enlargement of the right upper limb and multiple bluish-red swellings since birth, and recurrent episodes of fever, pain and bleeding in the affected limbs for the last 4 years. There was no history of any systemic complaints. The antenatal history was not significant, and there was no family history of similar complaints.

Examination of the child revealed a conscious, slightly distressed child with moderate pallor and fever. Cutaneous examination showed soft, well defined, warm, mildly tender swellings over the right elbow, having multiple discrete 0.3 cm x 0.5 cm to 0.5 cm x 0.7 cm sized erythematous to dark blue colored angiokeratomatous lesions [Figure - 1]. No bruit was heard on auscultation. This was associated with a diffuse soft tissue swelling of the upper arm, chest and upper back and increase of the girth of the limb on the right side of the body with multiple lymphangiomas. A port wine stain, 6 cm x 5 cm in size, was also observed, extending from the inner side of the right upper arm into the axilla and posteriorly to the upper scapular area. Systemic examination was normal.

Laboratory investigation revealed Hb 7.9 g% with a normal platelet count. An X-ray of the right arm, forearm and chest wall showed a soft tissue swelling over the right arm extending distally up to the elbow and proximally to the right chest wall, with the underlying bone (the humerus) thinned out, suggestive of atrophy. A Doppler study showed a multichanneled slow-flowing arteriovenous malformation with evidence of thrombosis with soft tissue thickening [Figure - 2]. Ultrasonography of the abdomen was normal.

The child was given analgesics and antibiotics for the thrombophlebitis and was given aspirin. She was referred to the pediatric surgery department for the management of the limb enlargement and arteriovenous malformation.

In 1900, Klippel and Trenaunay described a rare congenital disorder having abnormalities in the mesodermal components, clinically characterized by
(a) a capillary malformation, usually a port wine stain, over the affected extremity or at a site other than the hypertrophied limb (crossed dissociated form), (b) soft tissue or bony hypertrophy or both, (c) varicose veins or venous malformation, sometimes with persistent lateral embryonic veins.[1] Any two features are required for the diagnosis of Klippel Trenaunay syndrome (KTS).[2] Parkes-Weber described arteriovenous malformation, an infrequent finding in a “hemangiectactic hypertrophied lower limb”.[3] Intrauterine injury to the sympathetic ganglia causing dilatation of the arteriovenous anastomosis,[4] abnormalities in the deep veins obstructing the venous flow,[5] and mixed ectodermal and mesodermal dysplasias[6] have been suggested as some of the possible underlying mechanisms. There have also been case reports with a positive family history.[7]

Arteriovenous malformations are usually high flow malformations. They may be silent in the neonate or clinically may have a raised temperature, soft tissue enlargement or bruit on auscultation. Various reports on KTS have observed a vascular malformation of the slow flow type only and none has reported an arteriovenous malformation.[8],[9] Severe arteriovenous malformations may give rise to congestive heart failure. Disseminated intravascular coagulation has been reported in a patient with Parkes-Weber syndrome after a lower leg fracture.[10] Arteriovenous malformations are progressive lesions and their evolution is helped by puberty, pregnancy, infections and trauma.

Vascular anomalies may be divided into two major categories: tumors (hemangiomas) and vascular malformations.[4],[6] Vascular malformations are developmental anomalies that are further classified on the basis of the channel abnormality as arterial, venous, lymphatic, capillary, combined, arteriovenous and arteriovenous fistulas.

The vascular malformation in the Parkes-Weber syndrome is an arteriovenous malformation, which grows in proportion to the growth of the child and also in relation to the hemodynamic changes such as increased blood flow, causing vessel dilatation, obstruction and thrombosis.

Our patient had a large port wine stain over the upper limb with raised temperature but without any bruit on auscultation and clinically a diagnosis of KTS was considered. Subsequently, on Doppler evaluation a slow-flowing arteriovenous malformation was discovered.

The majority of patients with KTS require non-operative management. Epiphysiodesis is recommended only if the leg length discrepancy exceeds 2 cm in the growing child. Symptomatic varicose veins or localized arteriovenous malformation can be removed.[9] On the other hand, presence of arteriovenous malformation would require surgical intervention and correction with the help of endovascular surgery[11] as the hemodynamic alterations would give rise to high output cardiac failure, and the treatment is recommended at the time of diagnosis.

The lesions are detectable with the use of the continuous wave Doppler even before they are audible with a stethoscope. Good judgment before surgery and precise diagnosis with the help of venography/MRI is required.[11] Our patient represents an interesting case of Klippel Trenaunay-Weber syndrome [Table - 1]. A distinction between the two may sometimes be difficult,[12] as highlighted in our case. 

   References Top

1.Grevelink SV, Mulliken JB. Vascular anomalies and tumors of skin and subcutaneous tissues. In: Freedberg IM, Eisen AZ, Wolff K, et al, editors. Fitzpatrick's Dermatology in general medicine. 6th ed. New York: McGraw-Hill; 2003. p. 1002-19.  Back to cited text no. 1    
2.Gloviezki P, Stanson AW, Stickler GB, Johnson CM. Klippel Trenaunay syndrome: the risks and benefits of vascular interventions. Surgery 1991;110:469-79.  Back to cited text no. 2    
3.Parkes-Weber F. Angioma formation in connection with hypertrophy of limbs and hemihypertrophy. Br J Dermatol 1907;19:231-5.  Back to cited text no. 3    
4.Bliznak J, Staple TW. Radiology of angiodysplasias of the limbs. Radiology 1974;110:35-44.  Back to cited text no. 4  [PUBMED]  
5.Sevelle M. Klippel and Trenaunay's syndrome: 768 operated cases. Ann Surg 1985;201:365-73.  Back to cited text no. 5    
6.Baskerville PA, Ackroyd JS, Browse NL. The etiology of the Klippel Trenaunay syndrome. Ann Surg 1985;202:624-7.  Back to cited text no. 6  [PUBMED]  
7.Advoet GE, Jorens PG, Roelen LM. Genetic aspects of Klippel Trenaunay syndrome. Br J Dermatol 1992;126:603-7.  Back to cited text no. 7    
8.Samuel M, Spitz L. Klippel Trenaunay syndrome: clinical features, complications and management in children. Br J Surg 1995;82:757-61.  Back to cited text no. 8  [PUBMED]  
9.Jacob AG, Driscoll DJ, Skaughnessy WJ, Stanson AW. Klippel Trenaunay syndrome: spectrum and management. Mayo Clin Proc 1998;73:28-36.  Back to cited text no. 9    
10.Yamamoto H, Muneta T, Asahina S, Furuya K, Suzuki K. Lower leg fracture with Parkes Weber syndrome complicated by disseminated intravascular coagulation. J Orthop Trauma 1995;9:449-52.  Back to cited text no. 10  [PUBMED]  
11.Burrows PE, Fellows KE. Techniques for management of pediatric vascular anomalies In: Cope C, editors. Current techniques in Interventional Radiology. 2nd ed. Philadelphia: Current Science; 1995. p. 11-27.  Back to cited text no. 11    
12.Frieden I, Enjolras O, Esterly N. Vascular Birthmarks and Other Abnormalities of Blood Vessels and Lymphatics.In: Schachner LA, Hansen RC, Happle R, et al, eds. Pediatric Dermatology. 3rd ed. Edinburgh: Mosby; 2003:833-62.  Back to cited text no. 12    


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