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Klippel-Trenaunay-Weber Syndrome with Kasabach-Merritt Coagulopathy and Hydronephrosis
Lata Bhat1, Supriya Bisht, Kavita Khanijo
1Department of Neonatology, Fortis Hospital, Noida, Uttar Pradesh, India. Correspondence to: Dr Supriya Bisht, Department of Neonatology, Fortis Hospital, B-22, Sector 62, Noida, Uttar Pradesh, India. supriyabisht07@gmail.com.
Background:
Klippel-Trenaunay-Weber Syndrome is a rare syndrome, consisting of vascular malformation of blood and lymph vessels.
Case Characteristics:
A newborn female with respiratory distress from birth, and having vascular malformation involving left thigh.
Observation:
The neonate also had hydronephrosis and developed complication of Kasabach Merritt syndrome.
Message:
Urogenital abnormalities can be present in Klippel-Trenaunay-Weber syndrome but hydronephrosis is rare. Mortality is high with development of Kasabach Merritt syndrome.
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