Klippel-Trenaunay-Weber Syndrome: A clinical case report

Klippel-Trenaunay-Weber Syndrome (KTWS) is a rare condition and presents the main characteristics of cutaneous hemangiomas, the presence of varicose veins, and hyperplasia of the soft and hard tissues on different parts of the body. This syndrome is characterized by an extreme individual variability, given that the majority of patients do not clearly present this classic trio. This condition is diagnosed at birth or in the first years of life, defined as a non-hereditary, but congenital, abnormality caused by a gene mutation in one autosomal dominant or recessive gene. The clinical signs are present at birth and are highly variable according to their severity. A female patient, 6 years of age, sought out an orthodontist, complaining of a facial asymmetry and presenting a history of ectopic eruptions. In the anamnesis, the patient's guardian reported that the child suffers from KTWS. This condition is characterized by an extreme degree of variability when it affects the craniofacial region, with the majority of patients not showing the classic trio of signs. KTWS is a rare and unknown condition; however, it does require interdisciplinary knowledge, considering, first and foremost, the diverse vascular changes associated with other maxillofacial and dental needs. (C) 2017 Japanese Society of Pediatric Dentistry. Published by Elsevier Ltd. All rights reserved.