Skin Hemangiomas with Limb Hypertrophy

 
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Question :
Rajani H S, Narayanappa D
Department of Pediatrics, JSS Medical College, JSS Academy of Higher Education and Research, Mysore, India

Address for Correspondence: Dr.Rajani H S, Assistant Professor, Department of Pediatrics, JSS Medical College, JSS Academy of Higher Education and Research, Mysore 570004, India. Email: drrajanihs@jssuni.edu.in

A 9-month-old male child had large irregular compressible bluish purple hemangiomas and port vein stains in the right gluteal region and right lower limb laterally with asymmetric hypertrophy of right lower limb (Figure 1). Blood investigations were normal. Viscera, bones, spinal canal, gastrointestinal and genitourinary tracts were not involved. Ultrasound showed numerous subcutaneous cystic lesions. Figure 1: Bluish purple hemangiomas and port vein stains in the right gluteal region and right lower limb laterally with asymmetric hypertrophy of right lower limb

What is the diagnosis?

Discussion :
Klippel-Trenaunay syndrome. It is a sporadic rare cutaneous vascular disorder characterized by a triad of a port wine stain, varicose veins, and hemangiomata along with bony or soft tissue hypertrophy of an extremity associated with life-threatening complications.1,2 Complications include limb-length discrepancy leading to impaired gait and pain, thromboembolism, bleeding, venous insufficiency and soft-tissue infection.3 A primary mesodermal abnormality in fetal development leads to persistence of microscopic arteriovenous communications.1 The differential diagnosis are Parkes Weber syndrome, Proteus syndrome, Macrodystrophia lipomatosa, Beckwith-Wiedemann syndrome, neurofibromatosis, soft tissue sarcomas and lymphangioma.4 Ultrasound guided foam sclerotherapy is the state of the art new treatment to potentially close many large vascular malformations.5 Compression therapies and surgical debulking can be considered.6

References :
  1. Zea MI, Hanif M, Habib M, Ansari A. Klippel-Trenaunay Syndrome: a case report with brief review of literature. J Dermatol Case Rep. 2009;3:56-9.
  2. 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.
  3. Sharma D, Lamba S, Pandita A, Shastri S. Klippel-trénaunay syndrome - a very rare and interesting syndrome. Clin Med Insights Circ Respir Pulm Med. 2015;9:1-4
  4. Lacerda LdaS, Alves UD, Zanier JF, Machado DC, Camilo GB, Lopes AJ. Differential diagnoses of overgrowth syndromes: the most important clinical and radiological disease manifestations. Radiol Res Pract 2014; 2014: 947451
  5. Smith PC. Chronic venous disease treated by ultrasound guided foam sclerotherapy. Eur J Vasc Endovasc Surg. 2006; 32:577-583
  6. Noel AA, Gloviczki P, Cherry KJ Jr, Rooke TW, Stanson AW, Driscoll DJ. Surgical treatment of venous malformations in Klippel-Trénaunay syndrome. J Vasc Surg. 2000; 32:840-847

Correct Answers : yes  100%

Last Shown : Oct 2019
 
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