Cases reported "Anodontia"

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1/9. 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 = 1
keywords = incontinentia pigmenti, incontinentia, pigmenti
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2/9. incontinentia pigmenti: seven cases with dental manifestations.

    incontinentia pigmenti (Bloch-Sulzberger syndrome) is an uncommon genodermatosis that usually affects female infants. The condition is characterized by four cutaneous stages and is frequently associated with dental, ocular, central nervous system and structural anomalies. A large case series of seven patients, all female, who presented to the Department of Paediatric dentistry at the Eastman Dental Hospital over the last 16 years is reported. The dental features of these cases were typical and included missing teeth, microdontia and delayed eruption. In two of the seven cases, both maxillary canines were palatally impacted.
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ranking = 0.36104510083853
keywords = pigmenti
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3/9. Clinical features of incontinentia pigmenti with emphasis on dermatoglyphic findings.

    incontinentia pigmenti is a syndrome characterized by both systemic and oral manifestations. Presented here are the dental, clinical, radiological, genetic and dermatoglyphic findings of a 6 year old female case and her family members. The following features were apparent: oligodontia in maxillary and mandibular arches in both dentition, peg-shaped incisors and brown lesions on the body surface. No other problems were observed. The case had remarkable dermatoglyphic findings such as hypothenar loops associated with distally displaced axial triradii on both palms, reduced total finger and summed palmar a-b ridge-counts, decreasing plantar pattern intensity on the left sole. The other family members had similar dermatoglyphic characteristics. The paternal grand father, the father and the brother had eye defects.
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ranking = 4.0902612752096
keywords = incontinentia pigmenti, incontinentia, pigmenti
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4/9. Surviving male with incontinentia pigmenti: a case report.

    incontinentia pigmenti, or Block-Sulzberger Syndrome, is an X-linked dominant disorder with characteristic skin, hair, eye and tooth abnormalities. It is classically considered a male-lethal disorder with recurrent miscarriages of male foetuses. A few cases of surviving males with incontinentia pigmenti have been reported in the medical literature. This article reports the medical and dental findings of a boy diagnosed with incontinentia pigmenti.
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ranking = 6.0902612752096
keywords = incontinentia pigmenti, incontinentia, pigmenti
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5/9. Orthodontic and orthopedic treatment of a patient with incontinentia pigmenti.

    incontinentia pigmenti is an uncommon, inherited disorder with predominantly ectodermal manifestations that is associated with skin (100%)), dental (90%), skeletal (40%), central nervous (40%), and ocular (35%) deformities. It is an X-linked dominant disease, usually lethal in males and occurring in female infants. The dental effects include delayed eruption, partial anodontia, microdontia, and cone or peg-shaped teeth. The dental, clinical, and radiological findings in a 16-year-old female are presented here. The patient had peg-shaped teeth and a unilateral maxillary transverse discrepancy associated with oligodontia in the maxillary and mandibular arches. Orthodontic treatment included rapid maxillary expansion and fixed orthodontic therapy for prosthetic purposes and elimination of the functional midline shift.
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ranking = 4.0902612752096
keywords = incontinentia pigmenti, incontinentia, pigmenti
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6/9. incontinentia pigmenti (Bloch-Sulzberger syndrome). A case report.

    incontinentia pigmenti is a rare genodermatosis with multiple manifestations affecting the skeletal, ocular, dental, and other tissues. More than 95% of reported cases occur in females, and more than 60% of reported cases involve some dental abnormality. A brief review of the literature and a case in a 7-year-old girl with the disease are presented.
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ranking = 0.45130637604816
keywords = pigmenti
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7/9. A dentofacial deformity associated with incontinentia pigmenti: report of a case.

    A case of IP in a 16-year-old girl has been presented. This patient manifested classic ectodermal and mesodermal anomalies. We present this case to illustrate a rare etiologic factor in the development of dentofacial deformities that can be treated in the conventional manner.
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ranking = 4
keywords = incontinentia pigmenti, incontinentia, pigmenti
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8/9. Male cases of incontinentia pigmenti: case report and review.

    Male patients with Bloch-Sulzberger incontinentia pigmenti (IP type II) are rare and more severely affected than their female counterparts, with a significant occurrence of sex chromosome aneuploidy. This document introduces a new male IP type II patient and reviews 48 males reported with IP. Twenty-eight of the 49 patients meet current criteria for diagnosis of IP type II. The phenotype is variable and the incidence of documented developmental delay is 25%. Five patients had klinefelter syndrome (47,XXY). Most patients were reported prior to 1961 when chromosome analysis was not available. biopsy and laboratory reports considered to be "consistent with" the diagnosis of IP were seen in patients meeting criteria as well as those who would not currently be given the diagnosis. The histologic findings considered diagnostic are varied. This variability may be accounted for by differences in stage of disease, biopsy site, histologic technique, and reporting style. Conversely, this may indicate that the diagnostic weight given to the biopsy should be reconsidered. eosinophilia was not a consistent finding. overall, differences in reporting, ascertainment, and length of follow-up lead to difficulty in interpreting or predicting the natural history of males with IP type II. Based on the existing literature, they appear to have a higher rate of mental retardation than the general population, but there does not appear to be a correlation between severity of physical and mental involvement. The presence of sex chromosome aneuploidy documented in the more recent cases emphasizes the need for chromosome analysis in any male patient suspected of IP type II.
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ranking = 5
keywords = incontinentia pigmenti, incontinentia, pigmenti
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9/9. Case report: orthodontic treatment of dental problems in incontinentia pigmenti.

    incontinentia pigmenti is an uncommon genodermatosis that occurs in female infants. The characteristic dental defects are partial anodontia and the presence of some peg-shaped teeth. This report describes a patient with incontinentia pigmenti who exhibited multiple missing teeth in both arches. The patient had malpositioned teeth and decreased occlusal vertical dimension associated with the missing teeth. Orthodontic treatment was required for prosthetic purposes. The dental anomalies of incontinentia pigmenti and the treatment procedure are presented.
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ranking = 6.0902612752096
keywords = incontinentia pigmenti, incontinentia, pigmenti
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