Klippel-Trenaunay-Weber Syndrome with Kasabach-Merritt Coagulopathy and Hydronephrosis
- Indian Pediatr. 2015 Nov;52(11):987-8. doi: 10.1007/s13312-015-0760-5.
- 1. Department 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. [email protected].
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.