Cases reported "Ectodermal Dysplasia"

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11/587. radius hypoplasia, radial palsy, and aplasia cutis due to amniotic band syndrome.

    amniotic band syndrome is one of the many causes of aplasia cutis congenita. It is usually seen as a constriction band surrounding a limb or as a membrane that adheres to some part of the body. This syndrome can be associated with various malformations. An infant with amniotic adhesions producing aplasia cutis, radial palsy, and hypoplasia of the radius is presented. Early treatment led to total functional recovery of the affected limb. ( info)

12/587. breast reconstruction in ectodermal dysplasia.

    patients with ectodermal dysplasia may request breast reconstruction. In addition to abnormalities of other ectodermally derived structures, the breast and nipple-areolar complex may be absent or hypoplastic. Although this group of patients may have concerns with hair, nails, teeth, or even upper limb malformations, this report focuses on reconstruction of the breast anomalies. Four unrelated patients with ectodermal dysplasia who have undergone breast reconstruction are discussed. ( info)

13/587. Severe congenital absence of skin in a preterm infant.

    A severe case of aplasia cutis congenita in a preterm infant is described. Although major problems with thermoregulation and fluid balance were anticipated, these parameters were relatively easy to control once the patient was stabilized. Meticulous skin care and rapid formation of a membranous-like fibrous tissue layer covering the denuded areas probably played an important role in minimizing excessive fluid and heat loss. The prognosis in aplasia cutis congenita is determined by the underlying associated anomalies, the severity of skin lesions and, in our case, the maturity of the infant who died from complications of prematurity. ( info)

14/587. 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. ( info)

15/587. Johanson-Blizzard syndrome: a prenatal ultrasonographic diagnosis.

    Johanson-Blizzard syndrome is a rare autosomal recessive disorder characterized by aplasia of alae nasi, pancreatic insufficiency, aplasia cutis, anorectal anomalies and postnatal growth restriction. In this case report, we describe the prenatal sonographic findings of Johanson-Blizzard syndrome in a 21-week pregnancy of a consanguineous couple. Sonographic findings of aplastic alae nasi (beak-like nose) and dilated sigmoid colon led to the prenatal diagnosis. This is the first report of the prenatal sonographic diagnosis of Johanson-Blizzard syndrome. ( info)

16/587. ectodermal dysplasia with hypodontia in a set of Nigerian twins--a case report.

    A set of Nigerian male twins with features of ectodermal dysplasia born to apparently normal parents are presented. The main findings in both children were hypodontia, abnormally shaped teeth and hypotrichosis. The general treatment modalities of the condition, especially in a tropical environment, are discussed. ( info)

17/587. Aplasia cutis congenita--etiological relationship to antiphospholipid syndrome?

    A case of aplasia cutis congenita is reported. The mother suffered from antiphospholipid syndrome and a possible etiological relationship between this and aplasia cutis congenita is discussed. ( info)

18/587. Aplasia cutis congenita associated with fetus papyraceous.

    This report describes a healthy infant born with an isolated, truncal cutis aplasia defect in association with a fetus papyraceous. Effective healing of the cutaneous defect occurred over the course of a month. A 2-year follow-up demonstrate stable wound coverage. This rare association of aplasia cutis congenita, with a fetus papyraceous points most likely to the vascular causation of the cutaneous defect. ( info)

19/587. The use of tricalcium phosphate to preserve alveolar bone in a patient with ectodermal dysplasia: a case report.

    The prosthodontic management of the child with ectodermal dysplasia is made difficult because of the under-development of the alveolar ridges. This paper describes a case where tricalcium phosphate was placed in sockets immediately following the extraction of the primary incisor teeth to help maintain alveolar bone width, offering a valuable alternative treatment option in the prosthodontic management of the child patient with ectodermal dysplasia. ( info)

20/587. Aplasia cutis congenita with epidermolysis bullosa: a case report.

    A child with epidermolysis bullosa and aplasia cutis congenita is presented. This is a very rare condition that represents an unusual manifestation of epidermolysis bullosa. Management of aplasia cutis congenita is controversial and may be conservative, surgical, or a combination of the two. In this patient, surgical treatment with split-thickness skin grafts failed, and conservative treatment using silver-sulfadiazine cream dressings was instituted. ( info)
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