1/78. Hemifacial microsomia: oral, clinical, genetic and dermatoglyphic findings. Oral, clinical, genetic and dermatoglyphic findings of a female patient with hemifacial microsomia are described and compared with those cited in the literature. ( info) |
| 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. ( info) |
3/78. Simultaneous maxillary and mandibular distraction osteogenesis with a semiburied device. Distraction osteogenesis is a technique utilizing natural healing mechanisms to generate new bone; it is commonly used to lengthen the hypoplastic mandible. Distraction of the maxilla and mandible as a unit is an obvious extension of the technique. We describe the application of a semiburied distractor to simultaneously lengthen the mandible and maxilla and level a canted occlusal plane in three cases. The indications for bimaxillary distraction are reviewed, including its advantages, disadvantages and limitations. ( info) |
4/78. Mandibular distraction osteogenesis with multidirectional extraoral distraction device in hemifacial microsomia patients: three-dimensional treatment planning, prediction tracings, and case outcomes. Distraction osteogenesis of the craniofacial skeleton with the use of several different types of distraction devices (i.e., extraoral, intraoral, unidirectional, multidirectional, and customized) have been documented. However, the details of treatment planning and the method of predicting the distraction of the mandible in patients with hemifacial microsomia have not been published previously. This paper presents a technique for (1) three-dimensional treatment planning for mandibular distraction, (2) three-dimensional prediction tracings with conventional radiographs (panoramic, lateral, and posterior-anterior cephalometric), and (3) correlating the treatment planning and clinical applications. Lastly, 2 patients with hemifacial microsomia planned and treated with this approach are reported. ( info) |
5/78. Distraction osteogenesis of the ascending ramus for mandibular hypoplasia using extraoral or intraoral devices: a report of 8 cases. PURPOSE: This report presents the results of distraction osteogenesis using unidirectional extraoral and intraoral devices in 8 patients with different grades of vertical mandibular ramus hypoplasia. patients AND methods: Eight patients with hypoplastic mandibles underwent unilateral lengthening of the ascending ramus using unidirectional extraoral or intraoral devices. Intraoral mandibular distraction was performed on 5 patients with deficiencies of the vertical ramus up to 24 mm. External devices were used in 3 patients with more severe hypoplasias. An intraoral osteotomy was performed, and progressive distraction at rates of 0.5 mm/12 hours was initiated after 5 days. Once the desired length was reached, the device was maintained in place for 8 to 12 weeks. Three-dimensional computed tomography scans were taken in all the patients to plan the procedure and to compare the changes postoperatively. RESULTS: Successful distraction osteogenesis was achieved in all patients. The amount of mandibular lengthening ranged from 17 to 32 mm. Complications with the external devices such as rotation of the proximal bony fragment (2 cases) and loosening of the external screws at the end of the consolidation period (1 case) were observed. CONCLUSIONS: The results suggest that the intraoral device can be used as the method of choice for distraction osteogenesis of the ascending ramus of the mandible in patients with large deficiencies. Preoperative and postoperative 3-dimensional computed tomographic scans are essential in treatment planning. ( info) |
6/78. Treatment of hypoglossia-hypodactyly syndrome without extremeity anomalies. Three cases of hypoglossia-hypodactyly syndrome without limb deformities are reported. All exhibited different degrees of tongue hypoplasia, micrognathia, retrognathia with a very narrow space between the left and right halves of the mandible, constricted isthmus, and only one lower incisor. bone lengthening for the midline mandibular hypoplasia and orthodontic treatment were performed in the three cases with satisfactory results. ( info) |
7/78. 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. ( info) |
8/78. Behcet's disease: dental and oral soft tissue complications. Behcet's disease is a chronic, multisystem disorder. It has 3 primary components: recurrent inflammations of the eye, ulcerations of the oral mucous membranes, and ulcerations of the genitalia. Diagnosis of Behcet's disease relies mainly on history taking and clinical manifestations. This article describes the oral soft tissue and dental complications and the prosthetic problems encountered in a young patient. If dentists encounter patients with chronic ocular inflammations and recurrent oral mucous membrane ulcers, the index of suspicion should increase. ( info) |
9/78. Maxillofacial prosthetic rehabilitation of a midfacial defect complicated by microstomia: a clinical report. Severe limitation in the oral opening, though an uncommon clinical presentation, makes gaining access to the oral cavity difficult for any dental procedure. This article describes the maxillofacial prosthetic management of a patient with a midfacial defect complicated by postsurgical microstomia. Intraoral and extraoral prostheses restored the patient's speech, dental articulation, mastication, lip support, esthetics, and anterior oral seal. ( info) |
10/78. 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. ( info) |