11/16. An unusual complication of mandibular distraction.BACKGROUND: The authors present an unusual complication of mandibular distraction in a child with the curious condition of multiple pterygium syndrome is presented. CASE REPORT: The patient was a Caucasian male with severe pterygia in his neck. As a result of his limited mouth opening and restricted upper airway leading to obstruction, he underwent lengthening of his mandible by distraction, which significantly improved his breathing. During his follow-up, it was observed that an unusually elongated permanent molar was present in an abnormal position. CONCLUSION: This case highlights the need to carefully plan the sites for osteotomy and the potential for damage to the developing permanent dentition in young children.- - - - - - - - - - ranking = 1keywords = mouth (Clic here for more details about this article) |
12/16. Severe postburn contracture of cheek mucosa leading to false temporomandibular joint ankylosis.A case of burn injury to lips and cheek mucosa is reported who developed severe contracture of cheek mucosa leading to inability to open mouth. Patient was misdiagnosed as a case of temporomandibular ankylosis and was managed on those lines to no relief. When the contracture was released and soft tissue defect repaired with nasolabial flap, patient gained near normal mouth opening.- - - - - - - - - - ranking = 2keywords = mouth (Clic here for more details about this article) |
13/16. Congenital contractures, edema, hyperkeratosis, and intrauterine growth retardation: a fatal syndrome in Hutterite and Mennonite kindreds.We present clinical findings in infants from three kindreds (two Hutterite and one Mennonite) with an apparently unique, fatal disorder. The major manifestations consist of severe intrauterine growth retardation, congenital contractures, and tense skin which is easily eroded. The skin is tightly drawn over the face, giving an abnormal appearance consisting of a narrow, pinched nose, small mouth, limited jaw mobility, and ectropion (in one). One infant had first-degree hypospadias. Apart from this, there were no organ malformations and the infants did not have hydrops. Histologically, the skin showed hyperkeratosis. It is postulated that this is a tissue dysplasia and that all of the clinical effects are secondary. The disorder appears to be an autosomal recessive trait. The two Hutterite families are from different endogamous subdivisions. They are related as fourth cousins once-removed and fifth cousins in multiple ways through the six nearest common ancestors of all four parents. There are 25 founders (11 couples and three individuals) who are common ancestors. We computed the probability of joint descent of the four alleles in each pair of parents and in a sample of alberta Hutterite couples, assuming that each of the common founders in turn was the original carrier. For an allele from one particular founder couple, there is a relatively greater probability of identity by descent for each pair of parents than on the average for other couples of the same endogamous subdivision.- - - - - - - - - - ranking = 1keywords = mouth (Clic here for more details about this article) |
14/16. Infantile systemic hyalinosis in a black infant.A black girl was born with flexion contractures and experienced pain on movement by 1 week of age. She subsequently developed perioral papules, gingival hyperplasia, perianal nodules, torticollis, diarrhea, rectal prolapse, and inability to open her mouth. Her skin became increasingly sclerodermatous, and velvety, hyperpigmented plaques arose over bony prominences. A skin biopsy specimen showed hyaline material in the papillary dermis with lack of elastic fibers. Ultrastructural examination revealed fibrillogranular material around fibroblasts and blood vessels. This child had the clinical, histologic, and ultrastructural features of infantile systemic hyalinosis. This disorder has not been described in a black infant. Previous case reports of infantile systemic hyalinosis are reviewed and unusual features of our case are discussed.- - - - - - - - - - ranking = 1keywords = mouth (Clic here for more details about this article) |
15/16. Marden-Walker phenotype: spectrum of variability in three infants.The physical, radiographic, and pathologic findings in 3 new patients with Marden-Walker syndrome (MWS) are compared with those of previously described children with the syndrome. Over 75% of the children with MWS have blepharophimosis, psychomotor retardation, small mouth, micrognathia, kyphosis/scoliosis, and multiple contractures. Minimal diagnostic criteria have yet to be defined attesting to the broad range of variability and potential genetic heterogeneity in this disorder.- - - - - - - - - - ranking = 1keywords = mouth (Clic here for more details about this article) |
16/16. Freeman-Sheldon syndrome: case management from age 6 to 16 years.OBJECTIVE: This is the first description of long-term follow-up of a case of Freeman-Sheldon syndrome. microstomia was treated with a mouth expander for 2 to 3 hours per day before active orthodontic treatment. Separate impressions were necessary in each quadrant of both upper and lower jaws because of limited mouth opening. CONCLUSION: Orthodontic treatment improved the patient's Class II malocclusion, which was accompanied by crowding and a deep bite.- - - - - - - - - - ranking = 2keywords = mouth (Clic here for more details about this article) |
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