[Oral pathology in children].
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A rare case of the in utero observation of agnathia, microstomia, and synotia associated with hydramnios is presented. The correct diagnosis is made by observing an absent mandible in a patient with hydraminios.
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Cheilognathopalatoschisis of the "common" type is a disturbance which goes beyond the actual region of the cleft and almost regularly involves the dental system as a whole, namely in the form of hypodontia. Whether the cleft and hypodontia anlage also affect the growth of both jaws in the sense of progenia is still not known. Equally, the question whether the pathogenetically different types of cheilognathopalatoschisis and isolated cleft palate can be separated etiologically from each other is not yet definitely clarified.
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Jefferson fracture is a very rare disease which occurs in only 2-13% of all cervical spinal fracture cases and in only 1.3% of total spinal fracture cases. A combination of an atlas-axis fracture occurs relatively frequently and with a higher incidence of neurological morbidity than isolated fractures. However, a Jefferson fracture, which is an isolated C-1 fracture, occurs very rarely. A 58-year-old woman was involved in a traffic accident and admitted to our hospital. She had a large scalp laceration in the parietal region and complained of nuchal pain. Neurological examination revealed nothing abnormal. A cervical x-p (lateral view) revealed no abnormal findings, but an open-mouth view revealed an 8 mm displacement (in total) in the lateral mass of the atlas. A cervical CT revealed a Jefferson fracture. Crutchfield traction was performed for 9 days followed by external immobilization with a halo-vest to allow the patient to be ambulatory quickly. A posterior occipitocervical fusion was performed with an iliac bone autograft using the Sof'wire Cable system for late cervical stability and reducing the period of rigid external immobilization. The postoperative state was uneventful. The halo-vest was removed 10 weeks after surgery. An x-p obtained 3 months postoperatively showed good stability of the cervical spine. The Sof'wire Cable system proved to be very useful.
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The case of a 4-month-old male with de novo partial trisomy for chromosome 14 involving the p13 leads to q24 portion is reported. He presented with growth and psychomotor retardation, peculiar facies due to nose-mouth anomalies, monolateral microphtalmia, high arched palate, and anomalies of hands and feet. These symptoms are found also in the other 8 cases of partial trisomy 14 reported in the literature. This confirms a characteristic chromosomal syndrome although the breaking points on the extra chromosome 14 are not the same in the 9 cases. The clinical picture of our case calls for careful investigations of the chronology of bone age and of the immunologic situation in further cases of total and partial trisomy 14.
A family is described in which two male infants have microcephaly, abnormal ears, anti-mongoloid slant, small mouth, cleft palate, flexed overlapping fingers with syndactyly of digits three and four, syndactyly of the second to the fifth toes, and normal karyotype. This seems to be a new syndrome.
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