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Unusual oral findings in dermatosparaxis (Ehlers-Danlos syndrome type VIIC).
A 13-year-old patient with dermatosparaxis (Ehlers-Danlos syndrome type VIIC), an autosomal recessive disorder of procollagen-I-N-proteinase, is presented. The oral findings comprise micrognathia, hypodontia, localized microdontia, opalescent tooth discoloration, root dysplasia, pulp obliteration, severe gingival hyperplasia, frontal open bite, and severe restriction of TMJ mobility. The reported anomalies suggest the need for expanding the present phenotypic spectrum. This is the first report on oral findings in the syndrome.
Prevention and early recognition of major orofacial disorders.
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The role of research in craniofacial surgery.
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Correction of microstoma.
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Oral findings in a group of newborn Swedish children.
Oral examinations were performed of 1021 newborn Swedish children, of whom 101 were re-examined after 2-3 or 4-5 months. The most common findings, registered in 74.9% of the children, were of oral mucosal cysts situated either palatally or on the alveolar ridges. The majority of the palatal cysts disappeared shortly after birth, and some alveolar cysts appeared after birth. Ankyloglossia was found in 2.5% of the children, and Fordyce spots in 1.0%. No natal teeth were found. The upper labial frenum was attached to the crest of the alveolar ridge in 76.7% of the children, palatally in 16.7% and buccally in 6.7%. The relationship of the alveolar ridges was recorded: the anterior segment of the mandibular ridge was distal to the maxillary in 99% of cases, and, posteriorly, the mandibular ridges were lingual to the maxillary in 97.6%. An open bite was found in 39.8% of the children.
Familial occurrence of syngnathia congenita syndrome.
A case of familial occurrence of syngnathia congenita in mother and child is described; this syndrome has been seen only sporadically until now. Due to the distinct appearance of the intraoral chords--they were situated on both sides between the ridges of the jaw and also between the left half of the bifid uvula and the retrolingual region--a relationship to the autosomal-dominantly transmitted Cleft Palate Lateral Synechiae Syndrome (CPLS-Syndrome) is suspected. Complete penetrance with different expressivity may explain the different clinical manifestations of the cases seen up to now and the absence of bifid uvula in the mother of our patient. The probable pathogenesis during embryological development is discussed.
Ranulas and their mimics: CT evaluation.
The spectrum of computed tomographic (CT) findings in ten patients with pathologically proved simple and diving ranulas is reviewed. These retention cysts originate within the sublingual space from obstruction of the sublingual or minor salivary glands; when they enlarge, the cysts herniate to involve the submandibular and inferior parapharyngeal spaces (the so-called diving or plunging ranula). CT findings in 38 additional patients with a variety of cystic lesions in the floor of the mouth are contrasted with findings in cases of ranulas. The relevant anatomy is reviewed. This experience indicates that a unilocular, cystic mass emanating from the sublingual space and extending into the adjacent submandibular and/or inferior parapharyngeal spaces can be considered a diving ranula in virtually all cases. A unilocular, cystic mass entirely within the sublingual space can be considered a simple ranula in most instances, although absolute distinction between a simple ranula and an epidermoid cyst cannot be made radiographically.
The production of developmental abnormalities of the oral structures in Amblystoma punctatum.
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Meeting the challenges of craniofacial-oral-dental birth defects.
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Cleft palate and congenital synechiae syndrome: a case report.
OBJECTIVE: A 1-day-old baby girl with a cleft of the secondary palate and a soft tissue band connecting the upper and lower jaws and preventing mouth opening was referred to the cleft lip and palate team by her pediatrician. This case represents a further example of an interesting but rare anomaly known as congenital alveolar synechia syndrome that requires early management to allow normal feeding and oral development.
Macrostomia, preauricular tags, and external ophthalmoplegia: a new autosomal dominant syndrome within the oculoauriculovertebral spectrum?
OBJECTIVE: First and second branchial arch involvement during early embryonic development results in a wide spectrum of anomalies that encompass diverse, superimposed, and heterogeneous phenotypes within the so-called oculoauriculovertebral spectrum. Nine members of a Brazilian family presenting typical branchial arch involvement in association with external opthalmoplegia are reported. CONCLUSION: Macrostomia or abnormal mouth contour, preauricular tags, and uni- or bilateral ptosis were present in association in several patients. To our knowledge, this is the first report on this type of autosomal dominant condition. Clinical and genetic aspects are discussed.
Mucogingival deformities.
The presence of mucogingival deformities often have an impact on patients in terms of esthetics and function. The variety of the conditions makes it difficult to place gingival and alveolar mucosa deformities under a single definition. Mucogingival deformities, as defined in this paper, may be congenital, developmental, or acquired defects. These may occur around natural teeth or implants and in edentulous ridges. They may be localized to soft tissues or be associated with defects in the underlying bone. They may show different degrees of severity and extension. A classification of mucogingival deformities should provide a method for identifying the different conditions in order to improve diagnosis, etiologic identification, research, treatment, and insurance evaluation.
[Method of correction of microstomia and deviation of the mouth angles by rotation of trapeziform and triangular flaps of the mouth mucosa].
In the paper, the author suggests an essentially new method of dilation of the oral orifice and elimination of mouth angles deviations basing on the principle of new redistribution of local tissues by means of rotation of a trapeziform graft of the mucosa with its submucous layer from the buccal and adoral region. In this method one uses more adequate tissues, which reserves in case of microstomia are located in the buccal and adoral region. The successful utilization in 60 patients of the suggested method of elimination of microstomia arising due to different causes enabled the author to recommend it widely for reparative surgery.
[Preoperative examination of the patient with dento-maxillary deformities].
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[Reconstitution of lip, tongue and glottis function].
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[Stomatological aspects in lentiginous hypertrichosis apropos of 2 personal cases].
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