Klippel-Trenaunay-Weber Syndrome: A clinical case report

Publication date: Available online 8 December 2017 Source:Pediatric Dental Journal Author(s): Mirian Azevedo Pereira da Silva, Marcelo Rodrigo Vaz de Melo, Maria Isabel de Oliveira e Britto Villalobos, Cláudia Assunção e Alves Cardoso, Cláudia Scigliano Valério, Flávio Ricardo Manzi 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...
Source: Pediatric Dental Journal - Category: Dentistry Source Type: research