1/8. 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 = 1keywords = complex (Clic here for more details about this article) |
2/8. Chondroectodermal dysplasia: a case report.Chondroectodermal Dysplasia is a disease complex consisting of bilateral manual polydactyly, chondrodysplasia of long bones resulting in acromelic dwarfism, hydroitic ectodermal dysplasia affecting principally the nails, teeth and hair and congenital heart malformations. It is necessary to identify this disease at its early stage in order to render prompt treatment. The oral manifestations are characteristic for this particular disease so that a dental surgeon can identify this condition and refer the case to a cardiologist and orthopedician for corrective surgeries. In this article, a case of a 2 1/2-year old child with chondro-ectodermal dysplasia is presented along with radiographic investigations and treatment plan.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
3/8. Limb anomalies following chorionic villus sampling: a registry based case-control study.Using data from the Italian Multicentric Birth Defect Registry a case-control study was performed to verify if chorionic villus sampling (CVS) was associated with transverse limb defects (TLD), with or without features of oro-mandibular-limb hypogenesis complex (OMLHC), in the exposed offspring. The results show that the risk of TLD and OMLHC is increased following CVS, and is particularly high for CVS performed early in pregnancy, i.e., under 70 days of gestational age. These results, together with a review of other epidemiologic studies, biological data and clinical reports, strongly suggest a causative role of CVS as a risk factor for TLD and indicate that at this stage CVS before 70 days of gestational age should be discouraged as an option for prenatal diagnosis and that all patients wishing to undergo CVS should be informed about the possible risk of the procedure.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
4/8. Agnathia malformation complex associated with a cystic distention of the oral cavity and hydranencepahly.A fetus with a severe variant of the agnathia malformation complex (AMC) was delivered following prenatal diagnostic evaluation with ultrasonography. The constellation of anomalies that accompanied the agnathia included holoprosencephaly, hydranencephaly, situs inversus, and polysplenia. Recently, several authors have reported the association between the agnathia, holoprosencephaly, and situs inversus. We present evidence which suggests that, when hydranencephaly is also present, this may represent the most severe variant of the AMC. Our case is presented, the literature is reviewed, and a hypothesis regarding the embryopathologic mechanism is discussed.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
5/8. Holzgreve-Wagner-Rehder syndrome: Potter sequence associated with persistent buccopharyngeal membrane. A second observation.Here we present a second example of the syndrome first reported by Holzgreve et al. [1984] in this journal, i.e., Potter sequence with persistent buccopharyngeal membrane type II, postaxial polydactyly, cleft palate, cardiac anomalies, intestinal nonfixation, and intrauterine growth retardation. This specific complex MCA syndrome is not associated with a detectable chromosome abnormality.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
6/8. Agnathia-holoprosencephaly: a developmental field complex involving face and brain. Report of 3 cases.Agnathia-holoprosencephaly (A-H) is a developmental field complex involving a graded series of defects in the jaws, mouth, tongue, ears, eyes, and brain. Two general groups can be recognized: agnathia with holoprosencephaly (more severe) and agnathia without holoprosencephaly (less severe). This report describes three new cases of agnathia without holoprosencephaly and reviews the recent literature. By combining published cases with those ascertained through a survey of genetic centers in the united states, it appears that there have been at least 24 occurrences of A-H in the past 25 years. An inductive defect of the prechordal mesoderm that also affects neural crest cells is presented as the cause for this developmental field complex. Because of the etiologic heterogeneity associated with developmental field defects, the genetic counselor must provide a wide range of recurrence risks when dealing with the A-H complex.- - - - - - - - - - ranking = 1.4keywords = complex (Clic here for more details about this article) |
7/8. A case of lateral facial cleft, cleft lip and palate, anophthalmia, microtia, clavicular agenesis and asternia.We present a female infant with a complex pattern of congenital malformations including a lateral facial cleft, cleft lip and palate, anophthalmia, microtia, clavicular agenesis and asternia. A differential diagnosis is proposed and discussed.- - - - - - - - - - ranking = 0.2keywords = complex (Clic here for more details about this article) |
8/8. Isolated complex limb reduction abnormality diagnosed antenatally.Congenital limb reduction defects are rare lesions that are usually associated with other anomalies. Terminal defects are the commonest reduction defects. The aetiology seems to be multifactorial. We present a case of congenital terminal deficiency of the same side of the upper and lower limbs diagnosed by ultrasound scanning during the second trimester. The fetus had an absent right fibula and right foot, a hypoplastic right hand and left talipes equinovarus. The pregnancy was terminated. Micrognathia was the only associated anomaly found on postmortem examination. The cause of this isolated anomaly is unknown but, because of the very variable presentation of limb reduction defects, could possibly fit into the categories so far described in the literature.- - - - - - - - - - ranking = 0.8keywords = complex (Clic here for more details about this article) |