Cases reported "Anodontia"

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11/38. Facemask therapy with rigid anchorage in a patient with maxillary hypoplasia and severe oligodontia.

    In this report, we describe the treatment of a 10-year-old girl with a Class III skeletal relationship with maxillary hypoplasia and severe oligodontia. The maxillary arch was in a complete crossbite relationship with the mandibular arch. The treatment plan called for displacing the maxillary complex anteriorly with a facemask. Because of the lack of available teeth, a rigid anchorage implant was used in combination with the remaining teeth to provide anchorage. A titanium lag screw was placed in the maxillary alveolus. Three weeks later, 800 g of orthodontic force was applied. A significant anterior displacement of the nasomaxillary complex was achieved with the facemask. At the end of treatment, a temporary removable partial denture was placed.
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ranking = 1
keywords = hypoplasia
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12/38. Hemifacial microsomia and hypodontia: a case report.

    A 6-year-old girl with hemifacial microsomia was examined. Abnormalities found were: severely malformed pinna of the right ear, right malar hypoplasia and unilateral mandibular hypoplasia. Dental examination revealed normal intraoral soft tissues with all deciduous teeth present. Radiographic examination disclosed that the maxillary and mandibular right and left second premolars were not developing. The mandibular ramus was short in length and the mandibular condyle had not developed on the right. The patient had no renal, cardiac or skeletal anomalies and her hearing was normal. No previous publications have been located which report the association of hypodontia and hemifacial microsomia.
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ranking = 0.4
keywords = hypoplasia
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13/38. Hypodontia, impacted permanent teeth, spinal defects, and cardiomegaly in a previously diagnosed case of the Yunis-Varon syndrome.

    The Yunis-Varon syndrome is a rare hereditary disorder with ectodermal and skeletal anomalies that include agenesis or hypoplasia of the clavicles, craniofacial disproportion with severe micrognathia, digital anomalies, prenatal and postnatal growth deficiency, and neonatal death. Only 12 cases have been reported in the literature. Although neonatal death is a significant feature of this syndrome, two case reports describe children, both males, who were 30 months and 3 years of age at the time of investigation. The 30-month-old child was reexamined at 11 years of age, and had further clinical and radiologic features that included hypodontia, impacted permanent teeth, spinal defects, cardiomegaly, bilateral hearing loss, and metatarsus adductus. Children who survive the neonatal period and continue to thrive with many of the features of the Yunis-Varon syndrome as well as the new features described in this article may not have a distinct yet related syndrome.
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ranking = 0.2
keywords = hypoplasia
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14/38. poland-Moebius syndrome: a case with oral anomalies.

    The combination of Moebius and poland anomalies is rarely described in the literature. While some authors believe this association is an independent syndrome, others think that poland, Moebius and poland-Moebius syndromes are variations of the same condition. We report a case of poland-Moebius syndrome in a 6-year-old girl who presented with bilateral convergent strabismus, the inability to abduct her eyes beyond the midline and brachydactyly of her right hand. oral manifestations included incompetent lips and an abnormal tongue. Other facial features included hypoplasia of her mandible and her left ear at a slightly lower level than her right ear. Panoramic and lateral skull radiographs confirmed the absence of certain teeth and the hypoplastic mandible. The diagnosis of poland-Moebius syndrome was made on the basis of cranial nerve involvement and oro-facial manifestations.
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ranking = 0.2
keywords = hypoplasia
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15/38. Enamel defects and Lyonization in focal dermal hypoplasia.

    We report a pattern of enamel hypoplasia in focal dermal hypoplasia similar to that found in females with X-linked amelogenesis imperfecta. Three cases of focal dermal hypoplasia are described, with specific focus on the oral and dental features. In these cases the teeth all had vertical grooving with notching of the incisal or cuspal tips. Also recorded were blunt roots of taurodont form with open apices and missing teeth in 1 case. Oral papillomas were present in 2 cases. The pattern of enamel defects is attributed to Lyonization, which is consistent with the pattern of skin and bone lesions typically seen in focal dermal hypoplasia. This supports the proposal that focal dermal hypoplasia is X-linked. The authors conclude that the pattern of dental defects in focal dermal hypoplasia is consistent with Lyonization.
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ranking = 216.92021678355
keywords = dermal hypoplasia, focal dermal hypoplasia, hypoplasia, focal
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16/38. A point mutation of the ED1 gene in a Japanese family with X-linked hypohidrotic ectodermal dysplasia.

    X-linked hypohidrotic ectodermal dysplasia (EDA) is characterized by the hypoplasia or absence of hair, teeth and sweat glands. In this study, the authors investigated the ED1 gene in a Japanese family with X-linked hypohidrotic ectodermal dysplasia. The only affected male fulfils the diagnostic criteria for this disorder. His parents were not consanguineous and both of them were healthy. After informed consent, genomic dna was isolated from the peripheral blood lymphocytes or oral buccal epithelial cells of all members of the family. A polymerase chain reaction fragment containing exon 9 of the ED1 gene was amplified using primers. The patient's amplified fragment, as well as those from his father, mother and sister, were directly sequenced. The sequence from the patient revealed a point mutation (G1149A) in exon 8 of the ED1 gene, which changes codon 291 from glycine to arginine. Heterozygosity was demonstrated in his mother and sister. This mutation has not been reported previously. The amino acid substitution is predicted to disrupt the transmembrane domain, which strongly implies that this is the disease-causing mutation in the family.
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ranking = 0.2
keywords = hypoplasia
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17/38. Three successive generations of women with anhidrotic/hypohidrotic ectodermal dysplasia.

    We describe the findings of anhidrotic/hypohidrotic ectodermal dysplasia in three successive generations of a family. All three women had variable alopecia, anhidrosis, hypodontia and malar hypoplasia. Chromosomal studies revealed a defect of the 2q12 region in all three patients. Previous studies have reported rare cases of autosomal dominant ectodermal dysplasia associated with defects in the 2q11-13 region1. These rare disorders are characterized by common anomalies of at least two elements of the ectoderm and its appendages--namely, the skin, teeth, hair, nails and sweat glands. These patients also frequently have chronic dental problems with early loss of teeth and recurrent lung, ear and nose infections secondary to a defect in mucous membrane function. The majority of reported cases of ectodermal dysplasias have historically been X-linked recessive, but our findings indicate that an autosomal version may be more prevalent than previously thought.
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ranking = 0.2
keywords = hypoplasia
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18/38. Maxillary distraction osteogenesis to treat maxillary hypoplasia: comparison of an internal and an external system.

    BACKGROUND: Distraction osteogenesis (DO) has become a mainstream surgical technique for patients with jaw deformities. In cases of maxillary hypoplasia, DO with a rigid external distraction (RED) system has been used for maxillary advancement; however, DO with internal devices is currently popular. MATERIALS: This article describes DO with an internal device and a RED system in 2 patients with maxillary hypoplasia with oligodontia. The first patient, a young girl, had a concave profile due to maxillary hypoplasia and 9 congenitally missing permanent teeth. At age 11 years 11 months, she received DO with an internal device. The second patient, a boy aged 11 years 7 months, was treated with DO with a RED system. RESULTS: In the girl, the maxilla was advanced 5.0 mm without any dentoalveolar compensation. In the boy, the maxilla was advanced 7.0 mm, but undesirable mesial movement of posterior teeth was observed. CONCLUSIONS: DO with internal devices is simpler and more useful than the RED system for maxillary hypoplasia with oligodontia.
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ranking = 1.6
keywords = hypoplasia
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19/38. Kartagener's syndrome: unusual dental morphology.

    The present paper describes the dental presentation of a female patient with Kartagener's syndrome. There are no reports in the literature identifying dental anomalies in patients with this condition. The subject presented with the congenital absence of an upper lateral incisor, enamel hypoplasia and aberrant tooth morphology.
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ranking = 0.2
keywords = hypoplasia
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20/38. The effects of periradicular inflamation and infection on a primary tooth and permanent successor.

    Primary teeth and the permanent successors must be understood as interdependent units, where each one of them interacts with and depends on each other. Pulpal inflammation/infection of a primary tooth and the spread of this condition over the periradicular tissues can lead to alterations in the dental germ of the permanent successor and to the surrounding structures if no therapy is done, i.e. endodontics or extraction. This work will present cases of permanent teeth that showed alteration in eruption and / or in development, as a consequence of inflammation / infection of the preceding primary teeth, such as: hypoplasia, morphological alteration on the dental crown or total arrest of. radicular formation. The teeth analysed in this study belong to patients who attended the Universidade Federal de Santa Catarina Children's dentistry Clinic. The earlier these lesions are diagnosed, the less were the destructive effects and the consequences on the primary tooth/permanent germ unit.
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ranking = 0.2
keywords = hypoplasia
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