1/8. An atypical Hallermann-Streiff syndrome. Focus on dental care and differential diagnosis.The Hallermann-Streiff syndrome (HSS) is a rare congenital disorder characterized by dyscephaly, birdlike facies, hypoplastic mandible, congenital cataracts, microphthalmia, hypotrichosis, skin atrophy, proportionate short stature, and dental anomalies. A case of a 29-year-old man with atypical HSS with neither cataracts, hair and skin alterations, nor short stature is reported, with special consideration to oral findings and dental management. Dental extractions, scaling, restorations, and endodontics were performed under local anesthesia. Later, orthodontic rehabilitation with fixed brackets was carried out. Finally, a removable partial denture for the maxillary arch was designed using transparent acrylic, and this also served as a retention splint. Young patients with HSS and other similar syndromes must be involved in personalized oral health prevention programs as early as possible. Despite numerous systemic anomalies, some of these patients may undergo conventional dental procedures under local anesthesia in the dental office.- - - - - - - - - - ranking = 1keywords = facies (Clic here for more details about this article) |
2/8. Familial syndrome of unusual triangular facies associated with cleft palate, malocclusion, midfacial hypoplasia and sensorineural hearing loss in two siblings. Is it a new autosomal recessive syndrome?We present a previously undescribed syndrome characterized by triangular facial appearance, mid-facial hypoplasia, cleft palate and mild sensorineural hearing loss in two siblings. The parents were unrelated. The patients' stature and intelligence were normal. We suggest that the inheritance is autosomal recessive.- - - - - - - - - - ranking = 4keywords = facies (Clic here for more details about this article) |
3/8. Orofacial features of Scheie (Hurler-Scheie) syndrome (alpha-L-iduronidase deficiency).Scheie syndrome is a rare inborn error of metabolism, a mucopolysaccharidosis in which deficiency of the lysosomal enzyme alpha-L-iduronidase leads to tissue accumulation of mucopolysaccharides. Scheie syndrome is a forme fruste of Hurler syndrome (gargoylism), and some patients have the phenotype of Hurler-Scheie compound syndrome. The craniofacial abnormalities include coarse facies, mandibular condylar hypoplasia, retarded tooth eruption, and cystic jaw radiolucencies--particularly about the molars. Corneal clouding may lead to blindness but, in contrast to some other mucopolysaccharidoses, mental handicap is rare in Scheie syndrome. Cardiac valve incompetence is common, as are recurrent respiratory infections. Two brothers with Hurler-Scheie syndrome are presented and the oral and systemic complications each patient had described. The syndrome is discussed with particular reference to the orofacial features.- - - - - - - - - - ranking = 1keywords = facies (Clic here for more details about this article) |
4/8. Hallermann-Streiff syndrome: report of case.This syndrome, known also as oculomandibulocephaly, is a symmetric second branchial arch defect resulting in dyscephaly with bird facies and hypoplastic mandibles, among other defects; nearly half of all affected persons show dental anomalies. It should be considered a congenital disorder consisting mainly of developmental anomalies of the skull and facial bones.- - - - - - - - - - ranking = 1keywords = facies (Clic here for more details about this article) |
5/8. The treatment of some disorders of the maxillo-mandibular apparatus. III. the maxillo-septal facies, ipsilateral deficiency of facial mesenchyme (mesectoderm) and condylar displacement.The results of treatment of 67 patients with septo-maxillary syndrome and associated temporomandibular joint dysfunction are reported. Five case histories are described in detail. The features of the syndrome emphasized are the maxillo-septal facies, ipsilateral deficiency of facial mesenchyme (mesectoderm) and condylar displacement. Associated with condylar displacement was early osteo-arthritis of the temporomandibular joint complex. It is suggested that Costen's syndrome needs restatement.- - - - - - - - - - ranking = 5keywords = facies (Clic here for more details about this article) |
6/8. Muscular contraction headache and dental imbalance.A physical explanation for the contraction of the muscles of mastication, in muscular contraction headache, is described. It is suggested the role of tension is over emphasized as the main aetiological factor. In the younger dentate group, the presence of dental imbalance requires diagnosis by a dental practitioner. In the older, edentulous group, the family physician can easily diagnose mandibular overclosure from the facies (Fig. 1) or absence of molar teeth (Fig. 7) especially if combined with wearing the same set of dentures for more than ten years. When these signs are present, the family physician should consider referring such patients for a dental opinion.- - - - - - - - - - ranking = 1keywords = facies (Clic here for more details about this article) |
7/8. Hallerman-Streiff syndrome: case report and recommendations for dental care.Hallerman-Streiff syndrome is a rare congenital anomaly characterized by a peculiar bird facies, mandibular and maxillary hypoplasia, dyscephaly, cataracts, microphtalmia, hypotrichosis, skin atrophy, and short stature. Dental abnormalities are present in 80 percent of the cases and include malocclusion, crowding, severe caries, supernumerary and neonatal teeth, enamel hypoplasia, hypodontia, premature eruption of primary dentition, agenesis of permanent teeth, and anterior displacement or absence of condyles. Very few cases have been described in the dental literature. The predisposition to severe caries, together with other problems, makes it imperative that young patients be started in a strong prevention program as early as possible. This is a case report of a five-year-eleven-month-old white male who presented for a dental examination at The Children's Hospital in Denver, CO. The findings and recommendations for treatment are discussed.- - - - - - - - - - ranking = 1keywords = facies (Clic here for more details about this article) |
8/8. The oro-facial manifestations of trisomy 8 mosaicism: a case report.trisomy 8 mosaicism is a relatively common autosomal chromosomal disorder characterized by features such as mental retardation, characteristic facies, skeletal abnormalities and congenital heart disease. The case of a 4-year-old boy is described with particular reference to the oro-facial manifestations, which included cleft palate, pronounced anterior open bite, complete reserved unilateral buccal cross-bite, slightly increased width of alveolar processes, and gingival enlargement.- - - - - - - - - - ranking = 1keywords = facies (Clic here for more details about this article) |