1/15. Dental and oral lesions in two patients with focal dermal hypoplasia (Goltz syndrome).This report is concerned with the manifestations associated with focal dermal hypoplasia (Goltz syndrome) in two patients. Goltz syndrome is a rare polydysplastic syndrome. Systemic findings were similar to those previously reported in the literature and are illustrated. More detailed descriptions of the dental abnormalities are presented. The possibility of root resorption being a previously unreported part of the syndrome is hypothesised.- - - - - - - - - - ranking = 1keywords = dermal hypoplasia, focal dermal hypoplasia, hypoplasia, focal (Clic here for more details about this article) |
2/15. focal dermal hypoplasia: management of complex dental features.A 17 year-old female who presented for treatment of grossly carious lower first molar teeth had multiple features of focal dermal hypoplasia (FDH). These included enamel pitting and hypoplasia, anomalies of shape, size and positioning of teeth, as well as soft tissue papillomas and telangiectasis of tongue. A case report and a synopsis of the syndrome (FDH) are presented, the oral aspects of the condition are reviewed and the management of the complex dental anomalies is discussed.- - - - - - - - - - ranking = 0.53314715904339keywords = dermal hypoplasia, hypoplasia (Clic here for more details about this article) |
3/15. focal dermal hypoplasia -- oral and dental findings.This report describes the case of an 8-year-old girl with focal dermal hypoplasia. As well as deformities affecting her hands and feet she had marked facial asymmetry, one diminutive eye and coloboma affecting the other. Intraorally, the patient had papillomas at the base of the tongue and tonsils. Her teeth showed irregular spacing, hypodontia, enamel hypoplasia, anomalous tooth form and delayed development. Radiographically, several teeth showed abnormal form. The patient's previous surgical experiences had adversely affected her behaviour and treatment has been limited to patient motivation and oral hygiene instructions, acclimatization, and simple restorative care.- - - - - - - - - - ranking = 0.73314715904339keywords = dermal hypoplasia, focal dermal hypoplasia, hypoplasia, focal (Clic here for more details about this article) |
4/15. airway management of a patient with weill-marchesani syndrome.weill-marchesani syndrome is a genetically determined rare systemic connective tissue disorder consisting of brachymorphy, brachydactyly, aortic stenosis, and ophthalmologic abnormalities, such as ectopia lentis, microspherophakia, and secondary glaucoma. The primary lesion is fibrous tissue hyperplasia. Airway control and intubation may be difficult in patients with WMS because of stiff joints, poorly aligned teeth, and maxillary hypoplasia with a narrow palate. We describe the successful airway management of a patient with WMS and laryngeal stenosis, using an intubating laryngeal mask following failed conventional laryngoscopic intubation.- - - - - - - - - - ranking = 0.00060745875615356keywords = hypoplasia (Clic here for more details about this article) |
5/15. CNS midline anomalies in the Opitz G/BBB syndrome: report on 12 Brazilian patients.We report on 12 Brazilian boys with the Optiz G/BBB syndrome associated with CNS midline anomalies, namely, Dandy-Walker anomaly (two patients), enlarged cisterna magna (four patients), enlarged 4th ventricle (four patients), and callosal a/hypoplasia (two patients). These signs clearly show the involvement of the CNS midline in the Opitz G/BBB syndrome.- - - - - - - - - - ranking = 0.00060745875615356keywords = hypoplasia (Clic here for more details about this article) |
6/15. ectodermal dysplasia, ectrodactyly, clefting, anophthalmia/microphthalmia, and genitourinary anomalies: nosology of Goltz-Gorlin syndrome versus EEC syndrome.We report on two unrelated Brazilian girls born to normal and nonconsanguineous parents and presenting ectodermal dysplasia, ectrodactyly, clefting, tear duct anomalies, and micro/anophthalmia. The clinical picture presented by these patients suggests the diagnosis of Goltz-Gorlin (focal dermal hypoplasia) syndrome and EEC syndrome.- - - - - - - - - - ranking = 0.13313492507181keywords = dermal hypoplasia, hypoplasia (Clic here for more details about this article) |
7/15. Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome presenting with a large nephrogenic cyst, severe oligohydramnios and hydrops fetalis: a case report and review of the literature.OBJECTIVES: To report a case of EEC syndrome with a large nephrogenic cyst detected by prenatal ultrasonography. methods: Prenatal ultrasonographic detection, genetic counselling, termination of pregnancy, radiographic study, autopsy and ultrastructural study of scalp hair. The literature on EEC syndrome with genitourinary anomalies and prenatal diagnosis was also reviewed. RESULTS: A 6-cm cyst in the right side of abdominal cavity was detected in a fetus of 24 weeks' gestational age with severe oligohydramnios and hydrops fetalis. The autopsy revealed bilateral renal dysplasia with a large nephrogenic cyst at the right side and markedly hypoplastic urinary bladder and pulmonary hypoplasia. The fetus also had ectrodactyly and syndactyly of hands and feet and ectodermal dysplasia and left cleft lip and palate. Ultrastructure of hair from scanning electron microscopy revealed no obvious abnormality. CONCLUSION: This is a rare case of fetal malformation partly detected prenatally. In order to detect this syndrome, careful ultrasound search for acral anomalies should be performed in cases with renal malformation or obstructive uropathy.- - - - - - - - - - ranking = 0.00060745875615356keywords = hypoplasia (Clic here for more details about this article) |
8/15. pycnodysostosis. Report of a case and review of the Japanese literature, with emphasis on oral and maxillofacial findings.We report a case of pycnodysostosis and review 54 cases of this syndrome in the Japanese literature, with special emphasis on oral and maxillofacial findings. Common findings were as follows: hypoplasia of maxilla and mandible, hypopneumatization of the maxillary sinuses, loss of mandibular angle, a grooved palate, and malpositioned teeth.- - - - - - - - - - ranking = 0.00060745875615356keywords = hypoplasia (Clic here for more details about this article) |
9/15. Astomia-agnathia-holoprosencephaly association. prenatal diagnosis of a new case.A case of agnathia-astomia-holoprosencephaly with prenatal ultrasound diagnosis at 23 weeks is reported and discussed. This lethal neurocristopathy, well known in mammalians, is rarely observed in humans. prenatal diagnosis features are intrauterine growth retardation, mandibular absence or major hypoplasia, holoprosencephaly, cyclopia or hypotelorism, and in some instances frontal proboscis. This malformation is usually sporadic, but may be genetically determined as an autosomal recessive trait, since two cases in the same sibship have been reported.- - - - - - - - - - ranking = 0.00060745875615356keywords = hypoplasia (Clic here for more details about this article) |
10/15. Radiographic observations of pyknodysostosis. Report of a case.A case of pyknodysostosis in a 32-year-old woman has been reported. The patient presented typical skeletal changes, separated cranial sutures, open fontanelles, partial aplasia of the terminal phalanges of the fingers and toes, and brachyphalanges. The oral findings included lesions resembling periapical cementoma, hypoplasia of the maxilla and mandible, crowding of teeth, and a median raphe.- - - - - - - - - - ranking = 0.00060745875615356keywords = hypoplasia (Clic here for more details about this article) |
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