1/38. microstomia following facial burns.Deep burns to the face and lips often lead to the formation of scar tissue and contracture of the perioral tissues with marked reduction of the ability of the patient to open his mouth. The mouth tends to be turned into a wrinkled, rigid, unyielding structure, resembling the semi-rigid mouth of the fish. Such burns are most frequently caused by electricity or flames, and less frequently by chemical substances. The deformity caused by microstomia is painful both to the patient and to his family. Additionally, there is serious functional loss, it is practically impossible to smile, speech becomes difficult, and the movement of the mandible is limited. In severe cases feeding has to be performed with a straw. oral hygiene is compromised and access for the administration of dental care is impossible, hence limited to extractions. Techniques to prevent or, if not prevented, surgically correct the resultant microstomia are described, followed by a case report on a pediatric patient, whose microstomia was surgically corrected several years following the injury.- - - - - - - - - - ranking = 1keywords = ear (Clic here for more details about this article) |
2/38. Transmission of the dysgnathia complex from mother to daughter.We report the first observation of parent-to-child transmission of dysgnathia, a rare disorder characterized by severe mandibular hypoplasia or agenesis, ear anomalies, microstomia, and microglossia. Patient 1 was noted prenatally by ultrasound to have severe micrognathia and, after birth, abnormal ears with canal stenosis and non-contiguous lobules located dorsally to the rest of the pinnae, normal zygomata, severe jaw immobility and microstomia with an opening of only 4 to 5 mm, hypoplastic tongue, and cleft palate. The 21-year-old mother of patient 1 was born with severe micrognathia requiring tracheostomy, microglossia, cleft palate with filiform alveolar bands, abnormal pinnae, and decreased conductive hearing. Dysgnathia is thought to result from a defect in the development of the first branchial arch. A similar phenotype has been seen in Otx2 haplo-insufficiency and endothelin-1 homozygous null mice, suggesting that these genes contribute to branchial arch development. Our report of a long-surviving mother and her daughter with non-syndromal dysgnathia may lead to identification of the molecular basis of these findings and provide insight into the genetics of first branchial arch formation. The survival of patient 1 and patient 2 beyond the neonatal period has implications for improvements in prenatal diagnosis and counseling and for neonatal treatment of this condition.- - - - - - - - - - ranking = 4keywords = ear (Clic here for more details about this article) |
3/38. Oro-dental and craniofacial anomalies in leopard syndrome.'leopard syndrome' is a syndrome affecting many systems or organs. The main anomalies are summarized in the acronym LEOPARD in which each letter corresponds to the damage of a given organ. In the presented case, there are oro-dental and craniofacial anomalies. The relationship between the leopard syndrome and the given anomalies and the possibilities of the management and the follow-up of the diseased are studied. The clinical case presented is a 4-year-old boy with leopard syndrome showing retardation of dental development, delayed development and possible agenesis of permanent teeth, and craniofacial anomalies (osseous hypodevelopment). The bibliographical study shows that leopard syndrome is due to damage of the neural crest cells. Thus, the dental and craniofacial anomalies arise since neural crest cells participate in the formation of the teeth and some craniofacial bones. Therefore, dental and craniofacial anomalies might be expected in some cases of the disease. The therapeutic management of the lesions, and the follow-up of the patient would be done by a multidisciplinary team.- - - - - - - - - - ranking = 1keywords = ear (Clic here for more details about this article) |
4/38. Fibrous ankylosis after distraction osteogenesis of a costochondral neomandible in a patient with grade III hemifacial microsomia.Distraction osteogenesis has recently become a mainstay for treatment of craniofacial syndromes with mandibular hypoplasia. This article presents the difficult case of a patient with a previous costochondral rib graft who underwent mandibular distraction and developed a fibrous pseudoarthrosis at the distraction site. This was attributed in part to an associated temporomandibular joint ankylosis. Resorption of the pseudoarthrosis occurred once the distractor was removed. It appears that distraction osteogenesis of a mandible with an ankylosed temporomandibular joint can result in healing with a fibrous union, presumably because of movement at the distraction site when masticating. This can result in a pseudo "temporomandibular joint" at the distraction site. A temporomandibular joint arthroplasty was performed, followed by repeat distraction. We conclude that if there is an ankylosed temporomandibular joint or a stiff temporomandibular joint that may ankylose during the course of the distraction process, then a temporomandibular joint arthroplasty should be performed before or at the time the distractor is placed.- - - - - - - - - - ranking = 1keywords = ear (Clic here for more details about this article) |
5/38. Hypomandibular faciocranial dysostosis in consanguineous parents revealed by ultrasound prenatal diagnosis.We report here the fourth case of hypomandibular faciocranial dysostosis (HFD). The diagnosis was made at birth on the association of severe retrognathia, microstomia, severe hypoglossia with glossoptosis, persistent buccopharyngeal membrane, median cleft palate, bifid uvula, down-slanting palpebral fissures, short nose with anteverted nares, laryngeal hypoplasia, and low-set ears. A severe microstomia and micrognathia were detected by ultrasound at 31 weeks of gestation. Interestingly, even though the present case exhibits many facial dysmorphic features characteristic of HFD, craniosynostosis was absent. This report suggests that craniosynostosis is not mandatory for the diagnosis of this condition. Furthermore, we present a new argument for an autosomal recessive mode of inheritance for HFD.- - - - - - - - - - ranking = 1keywords = ear (Clic here for more details about this article) |
6/38. Dominant inheritance of cleft palate, microstomia and micrognathia--possible linkage to the fragile site at 16q22 (FRA16B).We report a family in which a father and his three children are affected with microstomia, micrognathia and partial or complete cleft of the hard and soft palate. The probands were non-identical twins, a boy and a girl, both noted to have the above features soon after birth. Their father was diagnosed with a submucous cleft of the palate at the age of 4 years and their older brother has milder facial features and a bifid uvula. All affected family members were demonstrated to have a fragile site on chromosome 16q22 but otherwise normal karyotypes. Of interest is a previously described family with autosomal dominant inheritance of U-shaped cleft palate, microstomia, micrognathia and oligodontia where all affected members were shown to have the fragile site at 16q22 in a proportion of their cells [Bettex et al. (1998) Eur J Pediatr Surg 8:4-8]. We propose that these two conditions are the same and represent a distinctive syndrome involving aberrant orofacial development that may be linked to the fragile site at 16q22.- - - - - - - - - - ranking = 1keywords = ear (Clic here for more details about this article) |
7/38. Application of cast magnetic attachments to sectional complete dentures for a patient with microstomia: a clinical report.patients with microstomia who must wear removable dental prostheses often state that they are unable to insert or remove the prosthesis because of the constricted opening of the oral cavity. This article presents a cast iron-platinum magnetic attachment system applied to sectional collapsed complete dentures for an edentulous patient with microstomia. With the use of lingual and palatal midline hinges and a cast iron-platinum magnetic attachment, the sectional prosthesis was successfully and easily inserted and provided adequate function in the patient's mouth.- - - - - - - - - - ranking = 1keywords = ear (Clic here for more details about this article) |
8/38. Freeman-Sheldon syndrome: a case report.Freeman-Sheldon syndrome, also called "whistling-face syndrome," is a very rare genetic condition, occurring both sporadically and by transmission through autosomal dominant or recessive mode, which affects primarily the face and skeleton. Characteristics include microstomia of the mouth, which gives the person a whistling appearance, a flat face, club feet, contracted joint muscles of the fingers and hands, and underdeveloped nose cartilage. This article describes a case of Freeman-Sheldon syndrome in a 10-year-old male. The most apparent deformities are associated with orofacial and skeletal development.- - - - - - - - - - ranking = 2keywords = ear (Clic here for more details about this article) |
9/38. microstomia caused by swallowing of caustic soda: report of a case.A case of microstomia caused by swallowing caustic soda is presented. A 54-year old man developed a progressive stricture of the circumoral region following accidental ingestion of caustic soda when he was 9 years old. He was treated by a general surgeon who performed bilateral commissurotomies when he was 19 years old and lived normally until he needed major dental prosthetic treatment. His dentist was unable to perform the treatment due to the mouth stricture. The surgical option was to perform bilateral buccal mucosal flaps. A review of the literature and the surgical technique are presented.- - - - - - - - - - ranking = 3keywords = ear (Clic here for more details about this article) |
10/38. Hemifacial microsomia and hypodontia: a case report.A 6-year-old girl with hemifacial microsomia was examined. Abnormalities found were: severely malformed pinna of the right ear, right malar hypoplasia and unilateral mandibular hypoplasia. Dental examination revealed normal intraoral soft tissues with all deciduous teeth present. Radiographic examination disclosed that the maxillary and mandibular right and left second premolars were not developing. The mandibular ramus was short in length and the mandibular condyle had not developed on the right. The patient had no renal, cardiac or skeletal anomalies and her hearing was normal. No previous publications have been located which report the association of hypodontia and hemifacial microsomia.- - - - - - - - - - ranking = 3keywords = ear (Clic here for more details about this article) |
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