RESUMEN
Van der Woude syndrome (VWS) is a rare developmental disorder with an autosomal dominant inheritance and variable expressivity, occurring in about 1 of every 1,00,000-2,00,000 people. This syndrome is remarkably variable. It is characterized by orofacial manifestations like lower lip pits, cleft lip and/or cleft palate, hypodontia, cleft or bifid uvula, syngnathia, narrow high arched palate, ankyloglossia and hyper nasal voice. We report two interesting cases of VWS with characteristic orofacial features and an unusual additional finding of bilateral commissural pits. The purpose of this article is to facilitate understanding of etio-pathogenesis, clinical manifestations, role of genetic counseling and with special emphasis on commissural pits as an additional feature in VWS.
RESUMEN
Cemento-osseous dysplasia is a group of disorders known to originate from periodontal ligament tissues. Florid cemento-osseous dysplasia clearly appears to be a form of bone and/or cementoid tissues restricted to jaw bones. This lesion is usually asymptomatic and is incidentally detected during routine radiographic examination. The diagnosis is made by clinical and radiographic examinations, and biopsy is not necessary. The patient must manifest the typical changes in atleast two quadrants. An asymptomatic individual requires only a periodic follow-up to ensure that there is no change in the disease behavior. Surgical management is indicated only for a progressive lesion that produces orofacial disfigurement. This paper presents a rare case of a 60-year-oldfemale who was clinically and radiographically diagnosed as having florid cemento-osseous dysplasia in the maxilla and mandible. Discussion is presented with emphasis on clinical and radiographic manifestations.