Van der Woude syndrome: Report of two cases with supplementary findings.

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.

Florid cement-osseous dysplasia of maxilla and mandible: a rare clinical case

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.

Radiographic analysis of ameloblastoma: A retrospective study.

Background: Ameloblastoma is benign odontogenic tumor, usually affecting the posterior region of mandible. It is seen in the third to fifth decades of life. Radiographically the lesion is variable in appearance and may be unilocular or multilocular, with well-defined cortical borders in the mandible and ill-defined margins in the maxilla. Objective: To analyze cases of ameloblastoma, with emphasis on the radiographic findings. We also review the current literature briefly and discuss the clinical and radiographic findings. Materials and Methods: The present hospital-based retrospective study was conducted by reviewing the clinical and radiographic records of ameloblastoma cases from 2009 to 2011, available in the archives of the department. The data of a total of 14 patients were analyzed. Results: We observed that the patients affected with ameloblastoma were in the age-group of 19-68 years. The male: female ratio was 1.3:1. The mandible (78.57%) was more commonly affected than the maxilla (14.28%). Six patients (42.86%) had unilateral involvement and eight cases (57.14%) had bilateral involvement. The multilocular and unilocular types of ameloblastoma were noted in 12 (85.72%) and 2 cases (14.28%), respectively. The soap-bubble (50.00%), spider-web (21.43%), and honeycomb (14.28%) appearances were seen in the multilocular variety. Root resorption of variable degree was distinctly observed in 11 cases (78.57%). Conclusion: Radiographs are an important aid for the diagnosis of oral lesions of various types, especially those that involve bone. It is important for the practicing clinicians to know the salient features of ameloblastoma which are peculiar to the local population.

Cemento-ossifying fibroma.

Cemento-ossifying fibromas (COFs) are benign lesions affecting the jaws and other craniofacial bones. They commonly affect adult females between the third and fourth decade of life, predominantly occurring in the premolar/molar region of the mandible. Most of the lesions typically show slow and often expansile growth, centrally within the jaw and characteristically behave in a benign form, but occasionally they may present as an aggressive gigantiform lesion. Radiographically, they appear as well-defined unilocular or multilocular intraosseous masses. The lesion is invariably encapsulated and of mixed radiolucent densities. The tumor may grow quite extensively; thus, the term "aggressive" is sometimes applied. Surgical resection is the treatment of choice. They are insensitive to radiotherapy and recurrences are uncommon. Clinical, radiographic and histopathologic features of COF and other fibro-osseous lesions are overlapping and may cause confusion in classification, diagnosis and treatment.