Cleft lip and/or palate with mucous cysts of lower
Overview
Van Der Woude syndrome (VDWS) consists of the following characteristics: cleft lip with or without cleft palate, isolated cleft palate, pits or mucous cysts on the lower lip, and hypodontia. It is the most common syndromic form of cleft lip and palate (CLP), accounting for 2% of all CLP cases (usually CLP is nonsyndromic). Affected individuals have normal intelligence. It was first characterized in 1954
Causes
VDWS is an autosomal dominant or sporadic inheritance caused by mutations of the IRF6 gene, located on chromosome 1 at 1q32-q41. (Popliteal pterygium syndrome can also be caused by mutations of this gene.)
Diagnosis
A single experienced dentist, using a tongue depressor under natural light, carried out the clinical examination of babies coming to the hospital for their initial examination (with the consent of the parents). The cysts found were classified, according to their location, as palatal and alveolar cysts, according to the dental arch (maxillary and mandibular) and oral area (anterior and posterior). Data on the prevalence of cysts among the male and female genders with different types of clefts were submitted for statistical analysis using the chi-square test, with a significance level of 5%. The other data were documented in terms of numbers and percentages.