1/23. E.N.T. manifestations of Von Recklinghausen's disease.Von Recklinghausen's Disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
2/23. Hemophiliacs bone pseudotumors.Four cases of proved hemophiliac pseudotumors caused by intraosseous bleeding are reported. Five lesions were found at the uncommon locations involving the cranial vault, mandible, phalanx, distal femur and distal tibia. The conventional radiographic and computed tomographic findings are expansile osteolytic destruction, cortical thinning, partial breaking cortex or pathological fracture, and sometimes associated soft tissue mass. Ultrasonographic feature of one case at the phalanx shows cortical expansion and thinning contained mixed echogenicity in the medullary canal with soft tissue extension. T99m DTPA of one case at the distal femur shows increased vascular flow and uptake at right distal tibia and left distal femur.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
3/23. osteoradionecrosis of the cervical vertebrae and occipital bone: a case report and brief review of the literature.osteoradionecrosis (ORN) is a common complication of radiation therapy. We present the first case reported in the literature of ORN involving the first and second cervical vertebrae and occipital bone in a patient who was treated with surgery and radiation therapy 9 years prior for a TxN3M0 squamous cell carcinoma of the left neck arising from an unknown primary origin. A brief review of the pathophysiology and treatment of this pathological process is also presented. Although the mandible is the most commonly affected site in the head and neck, ORN may develop in an unusual location without any preceding trauma and display an insidious but rapidly progressive course. The pathophysiology of ORN is believed to be a complex metabolic and homeostatic deficiency created by radiation-induced cellular injury and fibrosis, which is characterized by the formation of hypoxic, hypovascular, and hypocellular tissue. The irradiated bone loses its capability to increase the metabolic requirements and nutrient supply required to replace normal collagen and cellular components lost through routine wear. This results in tissue breakdown and the formation of a chronic nonhealing wound. infection plays only a contaminant role, with trauma being a possible initiating factor. diagnosis of ORN begins with a complete physical examination, including fiberoptic examination and biopsy of any suspicious lesion to eliminate the possibility of recurrent tumor. Treatment of ORN commonly requires the debridement of necrotic bone and hyperbaric oxygen therapy. The head and neck surgeon must possess a high degree of suspicion to promptly diagnose ORN and initiate early treatment. Because of similarities in clinical presentation, the most important step in the initial management of suspected ORN is to eliminate the possibility of tumor recurrence or a new primary.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
4/23. Type I gaucher disease: extraosseous extension of skeletal disease.OBJECTIVE: To investigate the frequency and morphology of extraosseous extension in patients with gaucher disease type I. DESIGN AND patients: MRI examinations of the lower extremities were analyzed in 70 patients with gaucher disease type I. Additionally, the thoracic spine and the midface were investigated on MRI in two patients. RESULTS: Four cases are presented in which patients with gaucher disease type I and severe skeletal involvement developed destruction or protrusion of the cortex with extraosseous extension into soft tissues. In one patient, Gaucher cell deposits destroyed the cortex of the mandible and extended into the masseter muscle. In the second patient, multiple paravertebral masses with localized destruction of the cortex were apparent in the thoracic spine. In the third and fourth patient, cortical destruction with extraosseous tissue extending into soft tissues was seen in the lower limbs. CONCLUSIONS: Extraosseous extension is a rare manifestation of Gaucher bone disease. While an increased risk of cancer, especially hematopoietic in origin, is known in patients with gaucher disease, these extraosseous benign manifestations that may mimic malignant processes should be considered in the differential diagnosis of extraosseous extension into soft tissues. A narrow neck of tissue was apparent in all cases connecting bone and extraosseous extensions.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
5/23. Jacob's disease: report of two cases and review of the literature.Jacob's disease is a rare condition consisting of new joint formation between the coronoid process of the mandible and the inner aspect of the zygomatic arch. Strictly speaking, it was first described by the French anatomist Oscar Jacob in 1899, although in 1853 von Langenbeck had described coronoid process hyperplasia. The pathogenesis of both conditions remains unknown. In this paper we present two new cases and a complete review of the literature on Jacob's disease, of which we have found only 12 cases. Due to the low prevalence of this condition, its diagnosis is not straight forward.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
6/23. Craniofacial and dental manifestations of proteus syndrome: a case report.The proteus syndrome is a rare congenital hamartomatous condition that is characterized by a wide range of malformations, sometimes involving the face. Common manifestations include partial gigantism, congenital lipomas, and plantar hyperplasia. In this report we describe the craniofacial clinicopathological development in a girl with proteus syndrome from age 6 to 20 years. The patient had pronounced hemifacial hypertrophy, exostoses in the left parietal region, and enlargement of the inferior alveolar nerve and mandibular canal in the affected region. The dental development of the affected left mandible and maxilla was characterized by extremely premature development and eruption of the primary and permanent teeth and by pronounced idiopathic root resorptions. The multidisciplinary management of the patient and the treatment outcome is reported. A review of the Proteus patients in the literature who exhibited manifestation in the craniofacial region is presented.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
7/23. Giant-cell reparative granuloma of the tibia.Giant-cell reparative granuloma (GCRG) occurs in the jaw, temporal bone, and short tubular bones of the hands and feet. Although GCRG can affect long bones, only small numbers of such cases have been sporadically reported. This report describes a giant-cell reparative granuloma in the proximal tibia in a 60-year-old woman, describes features of GCRG in long bones and reviews the literature. A 60-year-old female patient was referred to us with complaints of moderately tender swelling of the right leg. Whole-body scintigraphic scanning was performed, which incidentally also disclosed a distal femoral lesion. The patient was admitted for surgery and incisional biopsies were performed on both lesions. pathology analysis of the specimen from the tibia showed new bone lamellae encircled by osteoblasts and multinucleated giant cells which were more numerous in the haemorrhagic regions of the stroma; the latter displayed fibroblasts, histiocytes and inflammatory cells. The specimen from the femoral lesion showed typical features of a benign enchondroma. The patient was readmitted for surgery. The femoral enchondroma was curetted and the cavity was packed with bone graft. The tibial GCRG was treated with marginal resection, autogenous and allogenous bone grafting and intramedullary nailing. Follow-up examination after two years showed no clinical or radiological evidence of a recurrence. Although GCRG is uncommon, it should be considered whenever a lucent, expansile, and possibly destructive lesion of a long bone is encountered. It should be distinguished from true giant cell tumours occurring in the same locations because they have different biologic behaviours.- - - - - - - - - - ranking = 0.091647209044092keywords = jaw (Clic here for more details about this article) |
8/23. Giant-cell reparative granuloma of the temporal bone: a case report and review of the literature.Giant-cell reparative granuloma (GCRG) is an unusual, non-neoplastic fibrous lesion that most often arises in the mandible and maxilla. GCRG of the temporal bone is exceedingly rare. To the best of our knowledge, only 17 cases have been previously reported in the international medical literature. Although no case of metastasis has been reported, this malignancy can be locally aggressive, and it often recurs following incomplete excision. We report the case of a young woman with a very large GCRG of the right temporal bone. We discuss the clinical picture, differential diagnosis, histologic evaluation, appearance on computed tomography and magnetic resonance imaging, and treatment options. We also review the cases of temporal bone GCRG that have been reported in the literature so far.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
9/23. sphenoid sinus brown tumor, a mass lesion of occipital bone and hypercalcemia: an unusual presentation of primary hyperparathyroidism.Brown tumor is a focal lesion of the bone caused by primary or, less commonly, secondary or tertiary hyperparathyroidism (HPT). While the mandible is the most frequently involved bone in the head and neck region, atypical involvement of the cranium in the area of the sphenoid sinus is exceedingly rare. In the literature, a unique case of brown tumor of the sphenoid sinus was reported in a patient with primary HPT. We present a case of sphenoid sinus and occipital bone brown tumor associated with primary HPT. A 47-yr-old woman presented a 2-yr history of headaches, dizziness, diffuse body and articular pain, fatigue, and a 6-month history of intermittent nausea and vomiting, polydipsia, and polyuria. magnetic resonance imaging (MRI) demonstrated an expansive mass lesion in the sphenoid sinus with erosion of the sellar floor and medial wall of the right orbit, and expansion in the medulla of bone. Examination of biopsy specimens obtained from sphenoid sinus mass confirmed the diagnosis of brown tumor. The biochemical laboratory studies showed elevation of parathyroid hormone and confirmed the diagnosis of primary HPT. Excision of a parathyroid adenoma affected the metabolic status into normalizing. At the follow-up of 12 months postoperatively, the size of sphenoid sinus brown tumor decreased and the mass of occipital bone disappeared. In conclusion, this is a first report of primary HPT masquerading as a destructive fibrous sphenoid sinus brown tumor associated with a mass lesion of occipital bone and hypercalcemia in the literature.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
10/23. osteonecrosis of the jaws in periodontal patients with a history of bisphosphonates treatment.BACKGROUND/AIM: osteonecrosis of the jaws is being increasingly reported in patients with bone metastasis from a variety of solid tumours and disseminated multiple myeloma receiving intra-venous bisphosphonates. The signs and symptoms that may occur before the appearance of clinical evident osteonecrosis include changes in the health of periodontal tissues, non-healing mucosal ulcers, loose teeth and unexplained soft-tissue infection. A series of nine periodontally involving patients showing osteonecrosis of the jaws that appeared following the intra-venous use of bisphosphonates is reported. MATERIAL AND methods: Nine consecutive patients with osteonecrosis of the jaws were prospectically studied. patients' past medical histories and the drugs that they had received for their malignant disease were systematically documented. Clinical, histopathological and radiographic features and proposal for treatment modalities of osteonecrosis are also reported. RESULTS: Of the nine patients (six women and three men) observed, all had osteonecrosis in the mandible; two had maxillary involvement as well. All nine patients had a history of extraction of periodontally hopeless teeth preceding the onset of osteonecrosis. In two patients, the lesions also appeared in edentulous areas spontaneously. All the patients had received intra-venous bisphosphonates as treatment for their disseminated haematological neoplasms or metastatic bone disease. The duration of bisphosphonate therapy at presentation ranged from 10 to 70 months (median: 33 months). CONCLUSIONS: jaw osteonecrosis appears to be associated with the intra-venous use of bisphosphonates. Dental professionals should be aware of this potentially serious complication in periodontal patients receiving long-term treatment with bisphosphonates.- - - - - - - - - - ranking = 1.6415304633086keywords = mandible, jaw (Clic here for more details about this article) |
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