Cases reported "Cleft Lip"

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1/208. Congenital fistula of the palate.

    Four cases of congenital fistula of the palate are presented. All four patients had a fistula which was situated in the vault with a bifid uvula, submucous separation of the palatal muscles, deformities of the palatal plates and unilateral cleft lip. Velopharyngeal incompetence appeared in primarily treated children. The aetiology and surgical treatment of the congenital defect are discussed.
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ranking = 1
keywords = defect
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2/208. Congenital facial neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome).

    Four patients with clinical features of Goldenhar-Gorlin syndrome who showed facial paralysis on clinical examination are presented. The fourth case died following surgery for cleft lip. autopsy revealed hypoplasia of the right facial nerve in its intracranial segment, with small right facial nucleus in the brain stem. Nosological aspects of the Goldenhar-Gorlin syndrome are discussed. Peripheral facial paralysis, as a part of this syndrome, is reviewed in the light of clinical and pathological findings and in its relationship to cardiac anomalies. It is suggested that Goldenhar-Gorlin syndrome is a part of a so-called cardiofacial syndrome.
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ranking = 255.66090473288
keywords = dysplasia
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3/208. Confirmation of Kapur-Toriello syndrome in an Italian patient.

    We report a patient showing the phenotype described by Kapur and Toriello (Am J Med Genet 41:423-425, 1991) in two sibs. Clinical manifestations include severe mental retardation, cleft lip/palate, and distinctive nose, eye, and intestinal defects. This additional case contributes to a better definition of the apparently new MCA/MR syndrome proposed by Kapur and Toriello.
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ranking = 1
keywords = defect
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4/208. Evidence that AEC syndrome and Bowen--Armstrong syndrome are variable expressions of the same disease.

    Several clinical disorders combine ectodermal dysplasia (ED) and cleft lip and/or palate (CL/P). These conditions have been recognized as a group of diseases with a narrow phenotypic spectrum and multiple points of overlap. We report a patient with a clinical diagnosis of AEC syndrome (ankyloblepharon, ectodermal defects, and CL/P) who additionally has some features observed in a different ED-CL/P disorder, Bowen-Armstrong syndrome. Because of this clinical overlap, we suggest that AEC syndrome and Bowen-Armstrong syndrome may be variable manifestations of the same pathologic entity.
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ranking = 25101.199727308
keywords = ectodermal dysplasia, ectodermal defect, dysplasia, defect
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5/208. Salivary gland aplasia with cleft lip and palate: a case report and review of the literature.

    We report the case of a patient with lifelong symptoms of xerostomia and a repaired bilateral cleft lip and palate. The clinical evaluation demonstrated aplasia of the major salivary glands. A review of the literature pertaining to salivary gland aplasia is presented, along with a summary of the data regarding patient gender, defect sites, hereditary background, and combined manifestations. The diagnostic methods, possible pathogenesis, and management are also discussed.
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ranking = 10.51714733775
keywords = aplasia, defect
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6/208. Maxillary distraction for the management of cleft maxillary hypoplasia with a rigid external distraction system.

    Maxillary hypoplasia is a common finding in patients with repaired orofacial clefts. Management of this condition has been a challenge to the reconstructive team. The introduction of distraction osteogenesis to treat craniofacial skeletal dysplasias has opened alternative approaches to manage these severe conditions. In this article, the authors present their technique to distract the hypoplastic cleft maxilla using a rigid external distraction device. The clinical assessment, indications, orthodontic procedure, surgical technique, and distraction protocol are reviewed. A case report shows the use of the technique. This technique allows the reconstructive team to treat patients in all age groups with predictable and stable results.
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ranking = 63.915226183221
keywords = dysplasia
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7/208. Prosthetic reconstruction in the cleft lip and palate patient with an extracoronal resilient attachment retained removable partial overdenture: case report.

    There are still some difficulties in prosthetic reconstruction of cleft lip and palate patients with conventional prostheses or implant retained prostheses. The most common difficulties are insufficient alveolar bone quality and quantity, inadequate soft tissue, and abutment teeth. The patient we report on was a 23-year-old man with a clinical diagnosis of right incomplete cleft lip and palate combined with midface dysplasia. The maxillary six anterior teeth were reconstructed. The maxillary right central incisor and canine were used as abutments for an extracoronal resilient attachment (ERA) retained removable partial overdenture. The STERN ERA SYSTEM is a hinged resilient attachment with an ideal stress breaking characteristic, a good retentive function, and easy chairside replacement. The 2-year follow-up examination revealed an adequate esthetic appearance with good retention and stability of the prosthesis. A removable partial overdenture using the teeth adjacent to the cleft area as abutments with an adequate attachment design is an alternative method for prosthetic reconstruction of cleft lip and palate deformity.
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ranking = 63.915226183221
keywords = dysplasia
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8/208. Working with parents to promote health.

    A cleft lip, with or without cleft palate, is the most common craniofacial malformation. The issue of whether health can be attained if the person has a physical defect is discussed. health education can be considered as a means whereby individuals learn to maintain, restore and promote health. Consideration is given to health promotion and the prevention of ill health in relation to infant feeding. Skill teaching, if successfully completed, may be regarded as a fundamental cornerstone to health promotion.
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ranking = 1
keywords = defect
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9/208. Frontonasal dysostosis in two successive generations.

    Frontonasal dysostosis (also called frontonasal "dysplasia") comprises ocular hypertelorism, median facial cleft affecting nose and/or upper lip, unilateral or bilateral cleft of the alae nasi, anterior cranium bifidum occultum, or a widow's peak. Usually it is a sporadic disorder, although a few familial cases have been reported. We describe a 2-year-old girl with anterior cranium bifidum occultum, lipoma of genu and anterior part of the corpus callosum, and hypertelorism. Her mother had a history of a nasal drip at birth caused by a defect in the cribriform plate and phenotypically, a widow's peak. This observation suggests either autosomal dominant or X-linked dominant inheritance. The family illustrates the importance of identifying mild expression of frontonasal dysostosis before genetic counseling.
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ranking = 64.915226183221
keywords = dysplasia, defect
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10/208. Nasal abnormalities in facial clefts.

    After a short review of their development rare clefts of the nose occurring together with facial clefts are described in a number of clinical cases. These may be divided into 6 groups: 1. clefts of the nose; 2. oblique facial clefts; 3. nasal abnormalities in cleft lip and palate cases; 4. nasal abnormalities in median cleft palate cases; 5. aplasia of the premaxilla; 6. syndromes associated with nasal abnormalities and facial clefts. The discussion deals with the literature, aetiology, prognosis and therapeutic aspects. The publication of individual rare cases is suggested.
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ranking = 1.5861912229583
keywords = aplasia
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