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Skin Hemangiomas with Limb Hypertrophy
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
Klippel-Trenaunay syndrome, varicose veins,hemangiomata
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
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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
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What is the diagnosis?
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 |
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Funding: None
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Conflict of Interest: None
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DOI: https://doi.org/10.7199/ped.oncall.2019.64 |
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Cite this article as:
S R H, D N. Skin Hemangiomas with Limb Hypertrophy. Pediatr Oncall J. 2019;16: 131. doi: 10.7199/ped.oncall.2019.64
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