1/9. Maxillofacial implications and surgical treatment of arthrogryposis multiplex congenita.A case of arthrogryposis multiplex congenita that affected a patient's temporomandibular joint is described. Preoperatively, the patient's interincisal opening was limited to 9 mm. Elective surgery was performed, which consisted of bilateral coronoidotomies, right and left meninscectomies, capsular release, and lateral pterygoid myotomies. Physical therapy was initiated postoperatively. Eighteen weeks after the surgery, the patient was able to open 18 mm and force open to 20 mm. The patient also noted significant improvement in speech and jaw function in the postoperative period.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
2/9. Klippel-Trenaunay syndrome (angio osteohypertrophy syndrome): a report of 3 cases.Klippel-Trenaunay syndrome was first reported in 1900 by Klippel and Trenaunay. The syndrome is characterized by the triad of vascular nevi, venous varicosity, and hyperplasia of soft tissue-and possibly bone-in the affected area. The original description was made with regard to the extremities. The syndrome is diagnosed on the basis of 2 or 3 symptoms. The 3 patients reported here presented with hemangioma and hypertrophy of the jawbone and concomitant malocclusion. There were clinically no obvious varicose veins. It appears that gravity plays an important role in the venous drainage from the head and neck region, and this could be the reason for the rarity of varicose veins in the head and neck region when compared with lower extremities. In addition, 2 of the 3 cases showed exophytic pedunculated growth arising from the gingiva, which has not been reported in any previous case reports. The following report presents the clinical features in the orofacial region and highlights the clinical significance of this syndrome.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
3/9. Abnormal anterior belly of the digastric muscle: a proposal for the classification of abnormalities.We recognized an abnormal anterior belly of the digastric muscle in an 83-year-old male cadaver. Three muscle bundles were observed on the left anterior belly: (i) attached to the left digastric fossa; (ii) attached to the right digastric fossa; and (ii) attached to the raphe of the mylohyoid muscle. Four muscle bundles were recognized on the right anterior belly: (i) attached to raphe of the mylohyoid muscle; (ii, iii) attached to the exterior surface on the base of the mandible from the raphe of the mylohyoid muscle; and (iv) attached to the interior surface on the base of the mandible from the raphe of the mylohyoid muscle. The raphe of the mylohyoid muscle was curved significantly to right and the four abnormal bundles found on the right anterior belly (see above) were attached to its curved point.- - - - - - - - - - ranking = 10.50896533994keywords = mandible (Clic here for more details about this article) |
4/9. Thelarche variant in a girl with angelman syndrome.A case of angelman syndrome (AS) with thelarche variant in a 4.5-year-old girl is presented. Clinical suspicion of AS was raised at the age of 15 months when she presented with mental retardation and epilepsy, absence of speech, ataxic gait with jerky movements, hyperactivity and paroxysmal episodes of laughter. Moreover, she had facial dysmorphic features such as microbrachycephaly, mid-facial hypoplasia, macrostomia and prominent mandible. Dinucleotide repeat polymorphism (DNRP) analysis, identified absence of maternal alleles at D15S543, D15S113 and GABRB3 loci, findings consistent with AS. Studies on CYP19 locus (outside the 15q11-13 region) revealed the presence of two different alleles, thus excluding the possibility of paternal isodisomy of chromosome 15 in this patient. breast development at the age of 4.5 years, accompanied by accelerated growth velocity and bone age suggested the diagnosis of variant thelarche. This is the second case of AS with sexual precocity reported and whether this combination is a coincidence or not remains to be clarified.- - - - - - - - - - ranking = 5.25448266997keywords = mandible (Clic here for more details about this article) |
5/9. Facial anomalies in patients with cytochrome-c-oxidase (COX) deficiency: a dysneurulation.The authors report 3 cases of cytochrome-c-oxidase deficiency (2 cases of kearns-sayre syndrome and 1 case of chronic progressive external ophthalmoplegia) with central nervous system alterations and facial anomalies. The facial anomalies are high forehead, wide nasal bridge, upturned nose, long and flat philtrum (alterations depending on frontal-nasal-premaxillary structures which derive from prosencephalic neural crests), hypoplastic maxilla and mandible, ophthalmoplegia (alterations of maxilla and III-VI cranial nerve nuclei, which derive on the mesencefalic neural crests), low set ears, short neck (alterations of the 3rd, 4th branchial arch derivatives, which arise from rhombencephalic neural crests). The authors conclude that cytochrome-c-oxidase deficiency in embryonic stage can injure, in kearns-sayre syndrome and chronic progressive external ophthalmoplegia, distal tissues of face and central nervous system depending on neural crests, and that the symptomatology of these diseases can be ascribed to dysneurulation.- - - - - - - - - - ranking = 5.25448266997keywords = mandible (Clic here for more details about this article) |
6/9. Maxillo-nasal dysplasia (binder syndrome): antenatal discovery and implications.Binder syndrome (BS) or maxillo-nasal dysplasia is an uncommon developmental anomaly affecting primarily the anterior part of the maxilla and nasal complex. The characteristic findings are a failure of development in the premaxillary area with associated deformities of the nasal skeleton and the overlying soft tissues. Affected individuals typically have an unusually flat, underdeveloped midface (midfacial hypoplasia), with an abnormally short nose and flat nasal bridge, underdeveloped upper jaw, relatively protruding lower jaw and/or a 'reverse overbite' (or class III malocclusion). A case of BS was diagnosed at 24 weeks of gestation using two- and three-dimensional ultrasound. The first sign was an isolated flattened fetal nose in the mid-sagittal plane. Further ultrasound imaging showed the absence of the naso-frontal angle, giving impression of flat forehead and small fetal nose. We discuss about this entity.- - - - - - - - - - ranking = 5418.4565682461keywords = lower jaw, jaw (Clic here for more details about this article) |
7/9. Autosomal dominant osteopetrosis and maxillomandibular osteomyelitis.Osteopetroses represent a heterogeneous group of rare, hereditary bony dysplasias. They range from a devastating neurometabolic disease (including severe malignant infantile osteopetrosis) to 2 more benign conditions principally affecting adults: autosomal dominant osteopetrosis (ADO) type I and type II. The present study describes the maxillofacial manifestations associated with the 2 subgroups of ADO. In this paper, we present the oldest patient described in the literature with ADO type I (76 years old). We also document the first ADO type II patient described in the literature with synchronic osteomyelitis of the mandible and the maxilla.- - - - - - - - - - ranking = 5.25448266997keywords = mandible (Clic here for more details about this article) |
8/9. Facial dimple with accessory bone and teeth.Accessory jaws (distomus), with or without teeth, rarely occur. This condition has been observed most commonly in association with lateral facial clefts. A case of a 6-year-old child with a unilateral facial dimple which is interpreted as a putative vestige of a lateral facial cleft and accessory maxilla is reported. Possible aetiology is addressed and previous cases are reviewed.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
9/9. The Robin sequence associated with partial maxillary duplication and multiple facial clefts: a case report.A case is presented of a male child of recent Yugoslav immigrants born with bilateral facial clefts, bilateral cleft lip and palate and severe micrognathia. Dental evaluation at 2 years revealed a very prominent premaxilla with hypoplastic maxillary deciduous central incisors. The mandible was markedly retrognathic with the patient exhibiting drooling. Panoramic radiographs revealed the presence of all deciduous teeth in addition to a duplication of the right maxillary molar teeth.- - - - - - - - - - ranking = 5.25448266997keywords = mandible (Clic here for more details about this article) |