1/11. Mesenchymal chondrosarcoma associated with Goldenhar's syndrome.Goldenhar's syndrome is characterised by bony abnormalities of the face, jaw and vertebral column. We report the first case of the development of a primary malignant neoplasm (mesenchymal chondrosarcoma) initially misdiagnosed as a meningioma, occurring in association with this syndrome.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
2/11. A theory on the embryogenesis of oculo-auriculo-vertebral (Goldenhar) syndrome.Oculo-auriculo-vertebral (OAV) syndrome is made up of anomalies, mainly of first and second branchial arch derivatives. Characteristic features include structural malformations of the external and middle ears, face, and jaw. It has been previously suggested that hemorrhage involving the first and second branchial arches causes hypoplasia and malformation of the face and auricle, but this theory cannot explain the multisystemic and protean manifestations of this disease. The theory set forth describes the cutaneous, facial, vertebral, and systemic anomalies in the OAV spectrum as a result of ectodermal nondisjunction early in development with subsequent mesodermal tethering. A subgroup of OAV may therefore be a disorder of ectodermal nondisjunction involving the otic placode similar to the spectrum of diseases such as occult spinal dysraphism that is associated with the same mechanism in the embryonic neuraxis. This would imply a molecular mechanism involving cell adhesion molecules that unify the two disease processes and explain the multisystem anomalies of the OAV syndrome.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
3/11. Early treatment of severe mandibular hypoplasia with distraction mesenchymogenesis and bilateral free fibula flaps.The technique of distraction has revolutionized the treatment of mandibular hypoplasia; however, presently large mandibular defects still require bone grafts. Microvascular grafting is commonly used in adults. Conversely, in pediatric reconstruction, nonvascularized rib grafts remain standard. Unfortunately, resorption of nonvascularized bone remains a major issue, particularly when soft tissue is hypoplastic. This case study represents a combination of techniques in the treatment of severe mandibular deficiency, and introduces the concept of distraction mesenchymogenesis. The patient was a 2 1/2-year-old boy with severe bilateral Pruzansky class III mandibular hypoplasia. He had a permanent open mouth posture, an overjet of 23 mm, and was unable to move the lower mandibular segment. His oropharyngeal airway diameter was 2.2 mm and he was tracheostomy dependent. The patient was treated with distraction of the lower jaw mesenchyme followed by bilateral functional free fibular microvascular flaps containing reinnervated muscle. This created a well-vascularized body, ramus, and condyle bilaterally within an adequate soft-tissue envelope. Postoperatively, the overjet was reduced to 5 mm. The patient can now actively move his mandible. Airway diameter increased to 10 mm, and the patient is able to tolerate intermittent tracheostomy plugging. This innovative combination of techniques allows early intervention, limits graft resorption, and improves airway control.- - - - - - - - - - ranking = 74.366048642619keywords = mandible, jaw (Clic here for more details about this article) |
4/11. Microvascular temporomandibular joint and mandibular ramus reconstruction in hemifacial microsomia.Microvascular temporomandibular joint (TMJ) and mandibular ramus reconstruction was performed in a 4-year-old hemifacial microsomia patient with multiple craniofacial and extracranial anomalies (goldenhar syndrome). Her major craniofacial anomalies included bilateral cleft lip and palate, left macrostomia, left microtia, and complete absence of the left vertical mandibular ramus and TMJ. Most of her other anomalies had been corrected surgically before TMJ and vertical mandibular ramus reconstruction, which was accomplished with a metatarsophalangeal (MTP) joint transplantation. The MTP joint was placed in hyperextended position in the skull base inserting the proximal phalanx under the remnants of the zygomatic arch and replacing the vertical mandibular ramus with the metatarsal bone. Straight mouth opening, correction of the midline, and normalized lateral movements of the mandible were accomplished. The graft includes two epiphyseal plates, which should maintain growth of the transplant. During the follow-up period (16 months) the achieved results have been maintained without adverse effects. The present technique appears to be a promising alternative in the treatment of children with Pruzansky type 3 hemifacial microsomia.- - - - - - - - - - ranking = 73.366048642619keywords = mandible (Clic here for more details about this article) |
5/11. Multiple accessory tragi as a clue to the diagnosis of the oculo-auriculo-vertebral (Goldenhar) syndrome.We present a newborn with multiple features of the oculo-auriculo-vertebral spectrum (OAVS), or Goldenhar's syndrome. Although not reported previously, aplasia cutis congenita might be within the spectrum of anomalies seen in the syndrome. Of particular importance to the dermatologist is the finding of multiple accessory tragi in a linear distribution from the preauricular skin, along the mandible, to the lateral neck, which might be a clue to the diagnosis of OAVS. This syndrome is associated with additional systemic anomalies that can often be found by simple screening techniques.- - - - - - - - - - ranking = 73.366048642619keywords = mandible (Clic here for more details about this article) |
6/11. prenatal diagnosis of hemifacial microsomia and ipsilateral cerebellar hypoplasia in a fetus with oculoauriculovertebral spectrum.Oculoauriculovertebral spectrum, or goldenhar syndrome, is characterized by varying degrees of prevalently unilateral underdevelopment of craniofacial structures (orbit, ear, mandible) and spinal anomalies. We report the prenatal ultrasonographic diagnosis made at 24 weeks' gestation in a family with a negative history. The prenatal diagnosis was suspected due to the presence of marked hemifacial microsomia and moderate ipsilateral cerebellar hemisphere hypoplasia in the absence of facial clefting.- - - - - - - - - - ranking = 73.366048642619keywords = mandible (Clic here for more details about this article) |
7/11. A case of oculo-auriculo-vertebral spectrum with long-term and comprehensive management.AIM: To show that a patient with severe oculo-auriculo-vertebral spectrum can achieve satisfactory occlusion and facial appearance through long-term and comprehensive management. methods: A patient, with oculo-auriculo-vertebral spectrum, had bilateral cleft lip and palate, macrostomia, malformed pinna, and unilateral hypoplasia of the zygomatic arch and mandible, which resulted in an asymmetric facial appearance characteristic of hemifacial microsomia. After bilateral lip repair and palatal closure, surgical repair of the pinna, nose, and cheiloplasty was performed. The patient was seen at the authors' dental hospital at 12 years 2 months of age. After lateral expansion of the maxillary arch, an edgewise multibracket appliance was placed and presurgical orthodontic treatment was begun. Combined maxillary and mandibular osteotomies were performed when the patient was 19 years of age, followed by genioplasty, multiple repair of the mandibular body and lip, and soft tissue transplantation to the left cheek, to improve facial symmetry. RESULTS AND CONCLUSION: After prosthetic treatment at 23 years of age, a stable and functional occlusion with a satisfactory facial appearance was achieved.- - - - - - - - - - ranking = 73.366048642619keywords = mandible (Clic here for more details about this article) |
8/11. Surgical correction of Goldenhar's syndrome.Goldenhar's syndrome is a variant of hemifacial microsomia - one of the more common congenital syndromes of the first and second arch. The Goldenhar's type is characterized by varying degrees of underdevelopment of craniofacial structures. The major deformities involve the mandible and the ear; but they also include the orbit and vertebral column, specific defects pathognomonic of this condition. Medical management requires reconstructive surgery of which treatment must take into consideration the timing of surgery and the effects of surgery upon the growth and development of the poorly formed structures. This paper presents a discussion of the pathogenesis and the favorable and unfavorable factors affecting the selection and timing of surgery. The literature is reviewed and two additional cases are added with an evaluation of the effects of early reconstruction. A single-stage procedure, utilizing rib autograft, is described for correction of the defective hemimandible, glenoid fossa, and zygoma. The results are evaluated by means of photographs and radiographs with a follow-up period of at least one and one-half years. The data suggest a remodeling of the grafts to resemble normal structures, and a downward growth of the maxilla to fill a space created by the reconstruction of the mandible and expansion of the lower portion of the face. Multiple stages of surgery as well as unnecessary delays in performing surgical procedures are challenged by the report.- - - - - - - - - - ranking = 220.09814592786keywords = mandible (Clic here for more details about this article) |
9/11. Distraction osteogenesis in maxillofacial surgery using internal devices: review of five cases.PURPOSE: The purpose of this report is to show the feasibility and potential advantages of using internal devices for distraction osteogenesis in the management of maxillofacial skeletal deficiencies. patients AND methods: Distraction osteogenesis was used to correct a variety of maxillofacial skeletal deformities in five patients. One patient underwent bilateral Le Fort III advancement aided by distraction, three patients underwent mandibular ramus lengthening, and one patient underwent segmental alveolar reconstruction by distraction. The devices were activated by either a transcutaneous or transmucosal pin. After achievement of the desired skeletal transport, the activating pins were disengaged and removed from the distraction device. This allowed the distraction device to remain submerged and to stabilize the site of the consolidating bone. RESULTS: All patients achieved lengthening of their jaws. However, premature consolidation was noted in two patients, and one patient had significant relapse. CONCLUSIONS: Development of internal distraction devices is important to address the limitations of currently available biphasic systems. Potential benefits of internal devices include 1) elimination of skin scarring caused by translation of transcutaneous fixation pins, 2) improved patient compliance during the fixation or consolidation phase because there is no external component, and 3) improved stability of the attachment of the device to the bone.- - - - - - - - - - ranking = 1keywords = jaw (Clic here for more details about this article) |
10/11. Four-year-old girl with Goldenhar-sequence and severe obstructive sleep apnea, symptoms, diagnosis and therapy.Recently sleep related breathing disorders in children have received considerable attention. Unfortunately, cardiorespiratory polygraphy is expensive and time-consuming, and up until now today only a few pediatric hospitals have complete facilities for all-night cardiorespiratory polygraphy. Nevertheless, the prevalence of sleep related breathing disorders in children is higher than has previously been thought. To show the importance of proper diagnosis of sleep related breathing disorders, we report a case of a 4-year-old girl with severe obstructive sleep apnea caused by Goldenhar-sequence (facio-auriculo-vertebral dysplasia). Despite some treatment efforts to correct the aplastic right mandible, the symptoms of obstructive sleep apnea were not immediately recognized and remained untreated. Untreated obstructive sleep apnea led to growth and mental retardation. After polygraphic confirmation of obstructive sleep apnea and appropriate interdisciplinary treatment, the 4-year-old girl made rapid advances in growth and mental development.- - - - - - - - - - ranking = 73.366048642619keywords = mandible (Clic here for more details about this article) |
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