Mansi Tailor

Hereditary ectodermal dysplasia: A retrospective study

Background: Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat. Objective: To review and analyze cases of ED with an emphasis on clinical manifestations and parents marriage history. Methodology: The present retrospective study was conducted by assessing the clinical records of nineteen cases of ED, available in the archives of the department; for age, gender, family history of consanguineous marriage and clinical manifestations. Results: It was observed that ED was more prevalent in males, with a ratio of 1.7:1. The hypohydrotic type was more common (78.95%) than hydrotic type (21.05%). The marriage history of parents revealed that 66.67% had consanguineous marriage and had 68.42% offsprings affected with ED; whereas 33.33% had history of non-consanguineous marriage and had 31.58% offsprings affected with ED. The clinical manifestations observed were- dry skin(94.74%); scaly skin(42.11%); sparse hair on scalp, eyebrows and eyelashes(100%); frontal bossing(63.18%); saddle nose (57.89%); hypertelorism (47.37%); nail abnormality(52.63%); normal sweat glands(21.05%); abnormal sweat glands(78.95%); hypoplastic maxilla(52.63%); protuberant lips (57.89%); palmo-plantar keratosis(21.05%); wrinkled & hyper pigmented facial skin(84.21%); partial anodontia(94.74%); conical shaped teeth(84.21%); high arched palate(68.42%); thin alveolar bone(100.00%); taurodontism(21.05%) and cleft lip & cleft palate(05.26%). The number of teeth present in all the cases ranged from 0 to 19. Conclusion: ED patients suffer from social problems and poor psychological and physiological development as a result of unacceptable esthetics and abnormal function of orofacial structures. Oral rehabilitation thus becomes mandatory, although it is often difficult; particularly in pediatric patients.

Oral mucocele: A clinical and histopathological study.

Background: Oral mucocele is the most common benign minor (accessory) salivary gland lesion, caused due to mechanical trauma to the excretory duct of the gland. Clinically they are characterized by single or multiple, soft, fluctuant nodule, ranging from the normal color of the oral mucosa to deep blue. It affects at any age and is equally present in both sexes with highest incidence in second decade of life. They are classified as extravasation or retention type. Objectives: To analyze the data between 2010 and 2011 of, clinically and histopathologically diagnosed 58 oral mucoceles for age, gender, type, site, color, cause, symptoms and dimension. Results: Oral mucoceles were highly prevalent in the age group of 15-24 years, were seen in 51.72% of males and 48.28% of females, with a ratio of 1.07:1. The extravasation type (84.48%) was more common than the retention type (15.52%). The most common affected site was lower lip (36.20%) followed by ventral surface of the tongue (25.86%). The lowest frequency was observed in floor of mouth, upper lip and palate. The maximum numbers of mucoceles were asymptomatic (58.62%), and the color of the overlying mucosa had color of adjacent normal mucosa (48.28%). It was also observed that most of the mucoceles had diameter ranging from 5 to 14 mm. The causative factors of the lesion were lip biting (22.41%), trauma (5.18%) and numerous lesions (72.41%). Conclusion: Oral Mucoceles are frequently seen in an oral medicine service, mainly affecting young people and lower lip, measuring around 5 to 14 mm and the extravasation type being the most common.

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.

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.

Benign osteoblastoma- an unusual rare clinical presentation

Benign Osteoblastoma is an uncommon osteoblastic tumor that rarely involves facial bones. It is an uncommon lesion that accounts for 1% of all bone tumors and about 3% of all benign bone tumors. The data search has revealed that only 59 cases affecting the facial bones are reported in the literature. An unusual new case of benign osteoblastoma is presented with emphasis on clinical, radiographic and microscopic aspects, as well as differential diagnosis, treatment and follow-up are discussed. The importance of the correct diagnosis of this lesion is stressed due to presence of similarity to other bone lesions. The purpose of this article is to add one more case of this rare lesion to the academic literature and to provide a descriptive review.

Prevalence of commissural lip pits in population of central Gujarat: A hospital-based study

Background: Commissural lip pits (CLPs) fall among the rare congenital deformities recorded. CLP usually follows dominant hereditary pattern and may be associated with other congenital defects. CLP can appear as unilateral depression or bilateral depression. Objective: To assess the prevalence, age distribution, site and gender predilection of CLP in a central Gujarat population. Materials and Methods: Outdoor patients over a period of 6 months were examined for clinical evidence of CLPs. Results: A total of 14,403 patients were examined for the presence of CLP. CLP was identified in 381 patients with a prevalence of 2.65%. The occurrence of CLP was more in male patients. The maximum number of patients was in the age groups of 20–29 years. No significant statistical difference was observed in the distribution of CLP. Unilaterally distributed CLP was more common on the left side. Conclusion: Our study showed 2.65% prevalence of isolated CLPs with a male predilection. There was almost equal distribution of unilateral and bilateral CLP. In unilateral cases, the left side was affected the most. Although literature says lip pits can be an associated feature of certain syndromes, there were no syndromic patients in our study.

Prevalence of most commonly reported tobacco-associated lesions in central Gujarat: A hospital-based cross-sectional study.

Background: Oral cancer is a major health problem in tobacco users worldwide and is one of the ten most common cancers. India alone accounts for 1/3 rd of the world′s oral cancer and has a high rate of potentially malignant disorders (PMDs). The most common predisposing factors are smoking, smokeless tobacco, betel nut in quid form (pan), alcohol, spicy food, and sharp broken tooth. There are various tobacco-associated lesions (TALs) which can be diagnosed at very early stage. This study was conducted to rule out association of smoking and smokeless tobacco with occurrence of TALs and its dose-response relationship. Materials and Methods: Total of 60,018 patients attending the Department of Oral Medicine and Radiology from January 2013 to December 2014 with different oral and dental symptoms were screened. Of these, 4795 patients satisfying inclusion and exclusion criteria formed the cohort of the present study. All the patients were informed regarding the study and an informed verbal consent was obtained, following which they were interviewed for tobacco-related habits and examined by the trained dental professionals for the presence of any lesion. Along with patients′ demographic details, information regarding the type of habit, duration, and frequency was recorded. Statistical Analysis: Statistical analysis was performed using STATA 13.1 software (STATA 13.1 software by Stata Corp Ltd India Continent) by applying Pearson′s Chi-square test with Fisher′s exact test, Independent t-test, and ANOVA test. Results: The overall study population showed maximum cases having habit of smokeless tobacco (37.9%) and smoking tobacco (36.5%). The overall prevalence of TALs was found to be 7.98%. Our study found strong relation of duration and frequency of habit with respect to occurrence of the lesions. Conclusion: TALs are often subtle and asymptomatic. Therefore, it is important for the clinician to maintain a high index of suspicion, especially if risk factors such as tobacco habits are present.

Imaging in the diagnosis of a rare anomaly: Cleidocranial dysplasia

Cleidocranial dysplasia (CCD) is a rare autosomal dominant and developmental anomaly of skeleton characterized by generalized dysplasia of the bones and teeth. CCD is caused by mutation of transcription factor RuNx2, although 40% of the cases appear spontaneously with no apparent genetic cause. The disorder presents with partial or complete absence of clavicles, open anterior fontanelle, and cranial sutures, presence of multiple wormian bones, short stature, wide pubic symphysis, short middle phalanges of the 5 th finger, over retained deciduous teeth, multiple impacted permanent and supernumerary teeth and various other abnormalities. The frequency of this disorder is one/million individuals. Early diagnosis of CCD is essential for initiating the appropriate treatment approach. In confirmed cases, genetic counseling for family planning should certainly be advised. Because of its rarity, we report the classical case of CCD, with an emphasis on the importance of clinical and radiographic findings.

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 at least 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-old female 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.