1/14. MR and CT imaging in the Dyke-Davidoff-Masson syndrome. Report of three cases and contribution to pathogenesis and differential diagnosis.Cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis, and mental retardation. These findings are due to cerebral injury that may occur early in life or in utero. The radiological features are unilateral loss of cerebral volume and associated compensatory bone alterations in the calvarium, like thickening, hyperpneumatization of the paranasal sinuses and mastoid cells and elevation of the petrous ridge. The authors describe three cases. Classical findings of the syndrome are present in variable degrees according to the extent of the brain injury. Pathogenesis is commented.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/14. Tessier type VI-VII cleft combination associated with congenital bimaxillary fusion and anophthalmia.Congenital intermaxillary fusion is a rare anomaly. Combination of the anomaly with any type of facial cleft is extremely rare. death in a majority of these patients as a result of feeding and aspiration problems in early life may have caused the reports to be limited. In this article a 5-year-old patient, probably the first in the literature having Tessier type VI-VII facial cleft combination associated with bimaxillary fusion and anophthalmia on the right side, is presented. The patient has survived on fluid meal through a very small opening for 5 years. The features of the case are presented, and the time and method of the management of such a rare anomaly are discussed with a review of the literature.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/14. A review of myeloproliferative disease with presentation in the head and neck region.The diagnosis of an essential thrombocytosis is demonstrated in this presentation of a well-looking 53 year old man who had a five-year history of increasing facial asymmetry as evidenced by deviation of his mandible to the right and malocclusion. The enlarged mandibular condyle was the first manifestation of his underlying myeloproliferative disorder. His management will be discussed. Neoplastic diseases of the multipotent haematopoietic stem cells result in four major diseases: chronic myelogenous leukaemia (CML); polycythaemia vera (PV); agnogenic myeloid metaplasia with myelofibrosis (AMM/MF); essential thrombocytosis (ET). CML: demonstrates increased production of neutrophils and marked splenomegaly. It is divided into a chronic phrase typified by hyperplasia of mature bone marrow elements and a blastic or acute phase which evolves into a proliferation of immature marrow elements and can develop into acute myelogenous leukaemia. PV: associated with increased production of all myeloid cells but dominated by increased red blood cells with splenomegaly. AMM/MF: allows the neoplastic stem cells to proliferate and lodge in multiple sites outside the bone marrow. splenomegaly and fibrosis of marrow spaces also occurs. ET: resulting in a markedly elevated platelet count in the absence of a recognizable stimulus. Treatment revolves around measures to maintain hydration, to relieve arthralgias, to prevent thrombotic episodes, and to prevent infections.- - - - - - - - - - ranking = 7keywords = life (Clic here for more details about this article) |
4/14. osteochondroma of the mandibular condyle: a case report and review of the literature.osteochondroma is rarely found in the oral and maxillofacial regions. A rare case of osteochondroma affecting the mandibular condyle of a 46-year-old Japanese woman is reported. Clinical examination revealed facial asymmetry, malocclusion, and a palpable hard mass in the right temporomandibular joint (TMJ). Radiologically, the lesion was visualized as a radiopaque mass in the same region, but no destructive features were evident. Three-dimensional computed tomography was employed for estimating the stereographic extension of the lesion, which seemed to develop from the anterior portion of the condylar neck, and extend to the condylar head. The patient underwent tumor excision and condyloplasty under a clinical diagnosis of benign TMJ tumor. The histopathological diagnosis was osteochondroma of the mandibular condyle, and the lesion consisted of proliferative bony and hyalinized cartilage-like tissues. Moreover, a cartilage cap, a characteristic feature of osteochondroma, was also observed. Thirty-eight cases of osteochondroma of the mandibular condyle described in the English literature, including the present case, were reviewed. The mean patient age was 39.7 years with a peak in the fourth decade, which was older than patients with tumors in the axial skeleton. There was no sexual predominance for tumors in either the mandibular condyle or axial skeleton. The histopathogenesis of this tumor developing in the mandibular condyle was also discussed.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/14. Improving quality of life with a team approach: a case report.An adolescent female who presented amelogenesis imperfecta with severe anterior open bite, long face, facial asymmetry, high angle, and Class III skeletal pattern was treated with an interdisciplinary (orthodontics, orthognathic surgery, and prosthodontics) treatment approach. Presurgical orthodontic treatment was followed by surgical maxillary posterior impaction with anterior advancement and mandibular setback operation with vertical chin reduction and genioplasty. After the surgery, anterior ceramic laminate veneers and posterior full ceramic onlay-crowns were performed. The results showed that function and esthetics were achieved successfully with interdisciplinary collaboration.- - - - - - - - - - ranking = 4keywords = life (Clic here for more details about this article) |
6/14. TMJ fractures in children and adolescents: treatment guidelines.TMJ fractures must be focused not only as a cause of direct damage to osseous structures, but also of future disturbances of dentofacial development. Treatment is aimed at restoring normal joint function, occlusion and symmetry. Any disturbance of condylar cartilage will result in alteration of mandibular development. The subsequent deformity of jaw and face will depend not only on the type, intensity extent and chronology of the noxious agent, but also on the particular time of occurrence and growth activity. Thus the effect will be more decided if the disturbance occurs early in life, during childhood, when growth activity is greater and mandibular shape and size have not been assumed yet. This report will include basic information on both prevention and first aid in these types of injuries. The correct application of these precautions immediately following the trauma should improve the short and long-term outcome. Information on diagnosis and treatment of lesions of the bone and soft tissues, which may coexist with dental trauma, a critical first step in the overall management of traumatized patients, will be given. Follow-up procedures of these patients will be illustrated. The guidelines described in this paper for the treatment of traumatic TMJ fractures in children and youths are based on our clinical experience. They are intended as an aid to practioners in the management and treatment of these traumas, by professional must always use professional judgement. There are no guarantees of any positive results associated with the use of these guidelines, although it is felt that time and proper treatment will maximize the chances of success.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/14. Ocular manifestations of mosaic trisomy 22: a case report and review of the literature.Mosaic trisomy 22 is rare, but can be compatible with prolonged life. patients with mosaic trisomy 22 usually present with intrauterine growth retardation, mental retardation, failure to thrive, and craniofacial asymmetry. We report the case of a five-year-old boy who had a birth weight of 3.8 kg and normal developmental milestones. He presented with unilateral ocular manifestations of ptosis, double elevator palsy, high myopia, and choroidal coloboma involving the macula. Cytogenetic evaluation showed a low level of trisomy 22 in peripheral blood lymphocytes (1 in 100) and in cultured fibroblasts from a conjunctival biopsy of the affected eye (1 in 60). Our case demonstrates the value of chromosomal analysis of the tissues involved rather than just karyotyping of the blood lymphocytes to detect mosaicism in patients with localised and unilateral congenital malformations.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/14. Shprintzen (velo-cardio-facial) syndrome: a rare case.Shprintzen syndrome (velo-cardio-facial, VCFS) is a very rare morbid entity, seen in either familial or sporadic forms, with major clinical findings such as facial dysmorphism, cleft palate, cardiovascular (especially conotruncal-anomalies), mild/moderate mental retardation, or, more commonly, observed learning difficulty. Tendency to behavioral disorders and bipolar schizophrenic diseases may be present in these cases. Autosomal dominant inheritance has been reported. VCFS appears as a consequence of microdeletion in the 22q11 chromosomal band. Although each syndrome has different clinical reflections, genetically the defect is located on the same chromosome.A 4-year-old boy was admitted to our clinic with a syndromic face and the diagnosis of tetralogy of fallot. The patient underwent total correction of the cardiac defect. Atypical facial appearance, cleft palate, and malformed hand-fingers were present. With the aid of pediatric and genetic consultation (fluorescence in situ hybridization test), Shprintzen syndrome was confirmed. Both early and late postoperative periods of the patient were uneventful. The patient was closely consulted by a specialist psychologist during and after the hospitalization period.These children can be integrated into social life earlier through early surgical intervention for cardiac defects and facial deformations as well as neurologic and/or neuropsychiatric evaluation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/14. Management and subsequent 13-year progress of a mandibular fracture with malocclusion in a child--case report.Under acute conditions, maxillofacial injuries may be treated without the opportunity for an assessment of occlusal irregularities, even when there are mandibular fractures, because life-threatening injuries have priority over occlusion. Consequently, mandibular fractures may result in post-trauma malocclusion and facial deformity. The case history reported is of a male patient who had been involved in a traffic accident in childhood and suffered mandibular fractures. The initial incomplete management resulted in persistent deformation of the mandible, disturbance of dental occlusion and difficulty in mastication. These irregularities were corrected during childhood by non-operative orthodontic treatment. When the patient reached adulthood, some permanent teeth were malformed because the fractures had damaged some tooth germs. However, the permanent dentition in general was almost normal as a result of the corrected primary dentition. Although the alveolar deformity due to the injury remained, the mandibular base was satisfactorily remodelled. The case reported supports the view that early restoration of normal dental occlusion before the eruption of permanent teeth contributes to the establishment of good functional dental occlusion of the permanent teeth.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/14. lymphangioma and cystic hygroma. Correction of facial growth disharmony and obstructive sleep apnea.Soft tissue tumors and masses of the mouth and face can adversely affect the growth of facial bones. Cystic hygroma and lymphangioma, when massive, can create serious deformities of the maxilla and mandible. These deformities are correctible, surgically, and may be preventable if surgery and orthodontics are coordinated early in life. During and after surgery, airway management is paramount, but paradoxical effects such as obstructive sleep apnea may be observed.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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