ISSN - 0973-0958

Pediatric Oncall Journal

Klippel Trenaunay Syndrome

Altaf Naseem, U N Reddy.
Department of Pediatrics, Deccan College of Medical Sciences, Hyderabad, India.

Dr.Altaf Naseem, Assistant Professor, Department of Paediatrics, Deccan College of Medical Sciences, Santhosh Nagar, Hyderabad.
Spot Diagnosis

  Klippel Trenaunay Syndrome
What is the diagnosis?

Klippel Trenaunay Syndrome. It is characterized by a triad of port wine stain, varicose veins and bony and soft tissue hypertrophy involving an extremity {1}. Most cases are sporadic {2}, although few cases in the literature report an autosomal dominant pattern of inheritance {3}. The exact cause of this syndrome remains to be elucidated, underlying mixed mesodermal and ectodermal dysplasia is likely responsible {4}. It generally affects the single extremity although cases of multiple affected limbs have been reported. Diagnosis is mainly clinical {1}. Treatment is conservative and symptomatic. Hypertrophy of limb may lead to subsequent vertebral scoliosis, gait abnormalities and compromise of function {5}. Important differentials are Maffucci syndrome and Proteus syndrome.

1. Samuel M, Spitz L. Klippel-Trenaunay syndrome: clinical features, complications and management in children. Br J Surg. 1995, 82: 757-761
2. Aelvoet GE, Jorens PG, Roelen LM. Genetic aspects of the Klippel-Trenaunay syndrome. Br J Dermatol. 1992, 126: 603-607.
3. Ceballos-Quintal JM, Pinto-Escalante D, Castillo-Zapata I. A new case of Klippel-Trenaunay-Weber {KTW} syndrome: evidence of autosomal dominant inheritance. Am J Med Genet. 1996, 63: 426-427
4. Baskerville PA, Ackroyd JS, Browse NL. The etiology of the Klippel-Trenaunay syndrome. Ann Surg. 1985, 202: 624-627
5. Jacob AG, Driscoll DJ, Shaughnessy WJ, Stanson AW, Clay RP, Gloviczki P. Klippel-Trénaunay syndrome: spectrum and management. Mayo Clin Proc. 1998, 73: 28-36.

E-published: June 2009, Vol 6 Issue 6 Art # 34
Compliance with ethical standards
Funding:  None  
Conflict of Interest:  None
Cite this article as:
Naseem A, Reddy U N. Klippel Trenaunay Syndrome. Pediatr Oncall J. 2009;6: 35.
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