Cases reported "Tooth Abnormalities"

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1/272. Rare dental abnormalities seen in oculo-facio-cardio-dental (OFCD) syndrome: three new cases and review of nine patients.

    Oculo-facio-cardio-dental syndrome is a very rare condition. So far, only nine cases have been documented. We report on three additional female patients representing the same entity. The clinical findings were: congenital cataract, microphthalmia/microcornea, secondary glaucoma, vision impairment, ptosis, long narrow face, high nasal bridge, broad nasal tip with separated cartilages, long philtrum, cleft palate, atrial septal defect, ventricular septal defect, and skeletal anomalies. The following dental abnormalities were found: radiculomegaly, delayed dentition, oligodontia, root dilacerations (extension), and malocclusion. For the first time, fusion of teeth and hyperdontia of permanent upper teeth were seen. In addition, structural and morphological dental changes were noted. These findings expand the clinical spectrum of the syndrome.
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2/272. Treatment of an impacted dilacerated maxillary central incisor.

    Impacted incisor With Dilaceration refers to a dental deformity characterized by an angulation between crown and root causing noneruption of the incisor. Surgical extraction used to be the first choice in treating the severely dilacerated incisor. In this article, a horizontally impacted and dilacerated maxillary central incisor was diagnosed radiographically. By combining two stages of the crown exposure surgery with light force orthodontic traction, the impacted dilacerated incisor was successfully moved into proper position. However, long-term monitoring of the stability and periodontal health is critical after orthodontic traction.
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ranking = 0.090909090909091
keywords = dental
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3/272. Taurodontism in association with supernumerary teeth.

    The dental, radiological, genetic and dermatoglyphic findings of an additional patient with taurodontism in association with supernumerary teeth were presented and the findings of the patient were compared with those in the literature.
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keywords = dental
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4/272. A Rapp-Hodgkin like syndrome in three sibs: clinical, dental and dermatoglyphic study.

    Rapp-Hodgkin ectodermal dysplasia is an autosomal dominant disorder characterized by distinctive craniofacies, cleft lip or palate, oligodontia or anodontia, hypoplasia of the nails, and a decrease in or absence of the sweat glands and hair follicles. We have identified a family in which three children display clinical features similar to Rapp-Hodgkin syndrome. The father and two other sisters of the patient had normal facial features, but had short stature and had dental anomalies, the latter suggestive of ectodermal dysplasia. The overall clinical, dental, and dermatoglyphic findings of these patients are discussed in relation to reports of families with Rapp-Hodgkin syndrome.
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keywords = dental
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5/272. EEM syndrome: report of a family and results of a ten-year follow-up.

    We report on a Brazilian kindred in which two sibs presented with the complete form of EEM (ectodermal dysplasia, ectrodactyly, and macular dystrophy) syndrome with hypotrichosis, dental anomalies, syndactyly, and retinal changes with prominent pigmentation in the posterior pole of the retina. In this family, we also observed another sib with syndactyly, as well as a first cousin with ectrodactyly. A 10-year follow-up demonstrated gradually decreasing visual acuity and progression of retinal degenerative anomalies.
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ranking = 0.090909090909091
keywords = dental
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6/272. incontinentia pigmenti (Bloch-Sulzberger syndrome): report of case.

    incontinentia pigmenti is an inherited disorder with predominantly ectodermal abnormalities. The dental effects, delayed eruption, hypodontia, and microdontia, are very similar to anhidrotic ectodermal dysplasia. It is important that children with incontinentia pigmenti gain access to specialist dental care including pediatric dentistry, orthodontics, prosthodontics and oral surgery.
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keywords = dental
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7/272. Rieger's syndrome: (oligodontia and primary mesodermal dysgenesis of the iris) clinical features and report of an isolated case.

    An 11-year-old female manifested bilateral prominent Schwalbe's line, iridal adhesions, displaced and irregular pupils. She had no evidence of juvenile glaucoma. The major nonocular finding in this patient was the congenital absence of her primary and permanent maxillary central and lateral incisors. Although missing or malformed teeth may occur in other conditions such as Down's syndrome, focal dermal hypoplasia syndrome, anhidrotic ectodermal dysplasia, chondroectodermal dysplasia, and incontinentia pigmenti, the clinician should include Rieger's syndrome in his differential diagnosis in patients with oligodontia in combination with malformation of the anterior chamber of the eye. Rieger's syndrome is a rare condition characterized by ocular and dental abnormalities, namely mesoectodermal dysgenesis of the iris and cornea of the eyes and hypodontia of the dentition. This report presents a sporadic case of Rieger's syndrome in an 11-year-old female and discusses the pertinent clinical features of this condition.
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ranking = 0.090909090909091
keywords = dental
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8/272. Clinical problems associated with unusual cases of talon cusp.

    CASE REPORT: Talon cusp is an uncommon dental anomaly manifested as an accessory cusp-like structure on the crown of anterior teeth. This report describes two unusual cases of talon cusp. Case 1 showed bilateral anomalous cusps on the palatal aspects of maxillary supernumerary teeth, causing premature contact and tooth impaction. In case 2, a double-fused talon cusp was projected from the palatal surface of a large geminated central incisor. A talon cusp is not an innocuous defect, as it may provide a substantial diagnostic, treatment planning and procedural challenge. early diagnosis and management are important to avoid complications.
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keywords = dental
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9/272. Dysosteosclerosis: a case with unique dental findings and SEM evaluation of a hypoplastic tooth.

    A ten-year-old boy, who had the typical dental findings of dysosteosclerosis such as yellowish, hypoplastic teeth, retarded eruption, which upon eruption, decayed rapidly, is presented. To date this is the first known case reported with a congenital absence of the first permanent molars. Furthermore, SEM evaluation of the enamel and dentin was performed on a tooth from a patient with dysosteosclerosis for the first time. These studies showed weak ultrastructural compositions due to irregular calcification.
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ranking = 0.45454545454545
keywords = dental
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10/272. Globodontia in the otodental syndrome.

    A family of Polish extraction was studied in which massive, globe-shaped posterior teeth were found in a father and two of his sons, similar to those described in a previous family in which persons with this type of teeth also had a high-frequency sensorineural deafness. The audiograms in this family showed high-frequency air-conduction thresholds in the father and one son with globodontia and in other relatives without the tooth defect. The other son with abnormal teeth had a normal-appearing audiogram. Absence of premolar teeth and yellow-white spots of local hypomaturation of enamel on canine teeth were also findings in this kindred, as reported or observed in other kindreds. The disorder illustrates the problem of variable expressivity of a trait which makes it difficult to predict the risk of having an affected child when only one feature of a syndrome is present in a relative of a fully affected patient.
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ranking = 0.36363636363636
keywords = dental
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