1/64. 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) |
2/64. 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) |
3/64. A case report on aggressive fibromatosis with bone involvement.Aggressive fibromatosis is a locally infiltrative fibroblastic tumour that arises from fascial planes of soft tissue but does not metastasize. It is known to invade muscle, subcutaneous tissue and neurovascular structures. However, bone involvement is very rare and there has been few reports of bone involvement. We present a case of a young man with aggressive fibromatosis of the right lower leg with fibula involvement.- - - - - - - - - - ranking = 0.039401453228164keywords = lower (Clic here for more details about this article) |
4/64. Bone amyloidoma in a diabetic patient with morbid obesity.Bone localisations of amyloidosis are rare, usually diffuse and associated with myeloma. We report the case of a patient with massive obesity complicated by diabetes, hypertension, sleep apnea and liver steatosis, who complained of rapidly worsening bilateral polyradiculalgia of the lower limbs. After sufficient weight loss made nuclear magnetic resonance imaging feasible, a spinal tumour was visualised on the 5th lumbar vertebra, extending to soft tissues. Total excision was performed, and pathological studies revealed an amyloid bone tumour with no evidence of myeloma.- - - - - - - - - - ranking = 0.039401453228164keywords = lower (Clic here for more details about this article) |
5/64. 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) |
6/64. Disseminated hydatid disease causing paraplegia and destruction of the hip.A patient who had had been treated by surgery for spinal echinococcosis in a Mediterranean country emigrated to northern europe. After surgery, the echinococcosis disseminated and he developed chronic lytic and sinus-draining hydatid disease of the left hip and neuromuscular weakness of the left lower extremity. Seventeen years after the spinal surgery, he was referred to our hip service for a possible total hip replacement (THR) after receiving adequate chemotherapy against echinococcosis for nearly 3 years. Because of the poor results reported by others, we decided against THR; two of the four previous patients described in the current literature who had THR died due to complications.- - - - - - - - - - ranking = 0.039401453228164keywords = lower (Clic here for more details about this article) |
7/64. 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 = 1.0788029064563keywords = mandible, lower (Clic here for more details about this article) |
8/64. Campomelic dwarfism.The campomelic syndrome is a short-limb, usually fatal, neonatal dwarfism. It is characterized by bowed lower limbs, especially the tibia, usually associated with a cutaneous dimple over the anterior skin. There is delayed calcification of the epiphyses and delayed mineralization of the spine and pelvis. The cartilages of the tracheobronchial tree are hypoplastic, contributing to death from respiratory problems. Other associated abnormalities are unusual appearing facies, cleft palate, absent olfactory nerves and talipes equinovarus. Less commonly seen are cardiac, renal and brain anomalies.- - - - - - - - - - ranking = 0.039401453228164keywords = lower (Clic here for more details about this article) |
9/64. 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) |
10/64. 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) |
| | Next -> |