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1/20. 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.
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2/20. cysticercosis in otolaryngology.

    cysticercosis is not likely to be the first diagnosis the otologist has in mind when regarding tumors in the head and neck area. The fact that this disease may present a diagnostic and therapeutic problem is illustrated by three cases, appearing in the tongue, buccal mucosa, and midline of the neck, respectively. The local finding of a soft nontender mass, the typical calcifications in the soft tissues visible on roentgenograms, and the histopathological findings are described and illustrated. attention is drawn to the fact that cerebral cysticercosis, which is a very grave disease, may cause tinnitus, nystagmus, and hearing disturbances.
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3/20. Bronchogenic cysts of the neck in adults.

    Bronchogenic cysts are congenital sacs that result from maldevelopment of the primitive foregut. Although they occur predominantly in the chest, there are reports of lesions in extrathoracic locations. The majority of reported bronchogenic cysts located in the neck are found in the pediatric population; a review of the literature reveals few reports of bronchogenic cysts of the neck among adults. The diagnosis of a bronchogenic cyst relies on the histology and location of the lesion. Here, we review our experience in the diagnosis and management of 2 adult patients with pathologically proven bronchogenic cysts. Both patients presented with solitary neck masses that proved to be bronchogenic cysts on histologic examination. Our purpose is to define the histopathologic and clinical characteristics of bronchogenic cysts and discuss the features that distinguish them from other cervical cysts. In conclusion, congenital bronchogenic cysts can occur in the neck of adults and should be considered in the differential diagnosis of cystic cervical masses in adults, as well as children.
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4/20. 4 years' experience of head and neck tuberculosis in a south london hospital.

    In a south london department of otorhinolaryngology and head and neck surgery, 33 cases of tuberculosis were diagnosed in 4 years. The most common presentation was cervical adenitis (58%) and in some cases the initial investigations suggested malignant disease. Most of the patients were of non-British origin but none proved to be hiv seropositive. Fine-needle aspiration was positive for tuberculosis in 7 of 19 patients. 21 patients required a surgical procedure for diagnosis.
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5/20. otolaryngology problems in the immune compromised patient--an evolving natural history.

    As the human immunodeficiency virus is being detected in increasing numbers of asymptomatic individuals at risk, newer earlier patterns of disease have become apparent--including cranial and cervical herpes zoster, oral hairy leukoplakia, and oral candidiasis--thus linking viral and other disease to the development of acquired immunodeficiency disease (AIDS). Many similarities between patients with AIDS and other immunosuppressed patients have emerged. As immunosuppressed patients survive longer, they begin to manifest cancers such as lymphomas and squamous cell cancers in addition to Kaposi's sarcoma. Otolaryngologists can learn to identify and treat otitis and sinusitis in the immunosuppressed patient, to identify predictive early signs such as oral hairy leukoplakia, herpes simplex virus, and oral candidiasis, and to diagnose and treat Kaposi's sarcomas of the head and neck, lymphomas, squamous cell cancers, and opportunistic infections as the immunodeficiency disease progresses.
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6/20. Significance of abnormal indium In 111-labeled leukocyte accumulation in the head and neck region.

    The localization of occult infection is often a difficult clinical problem. In 1975, labeling of leukocytes with indium In 111 became technically feasible, and subsequent clinical experience with 111In-labeled leukocyte scanning demonstrated high sensitivity and specificity for acute infection. To our knowledge, experience with white blood cell scanning in the head and neck has not been previously reported. The University of california, san francisco, experience with 111In-labeled white blood cell scanning was reviewed. Between 1982 and 1987, 520 whole-body 111In-labeled leukocyte studies were performed, of which 60% were interpreted as abnormal. Eighteen studies were abnormal in the head and neck region, and the medical records of these patients were retrospectively reviewed. Abnormal findings correlated with other evidence of disease that involved mucosal surfaces, the neck, and the mediastinum. Sites in the nasopharynx with nasogastric tubes and tracheotomy sites were abnormal without associated clinical evidence of infection. Further clinical experience is needed to correlate white blood cell scan findings with disease in the head and neck and to define the role of the scan in diagnosis and management of otolaryngological disorders.
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7/20. Iatrogenic subcutaneous cervicofacial and mediastinal emphysema.

    subcutaneous emphysema of the head, neck and mediastinum occurs with a variety of disease processes. Most cases involve the passive escape of air from the aerodigestive tract into subcutaneous tissues. The many causes include head and neck surgical procedures, tracheal and esophageal trauma, intraoral trauma, foreign bodies and neoplasms of the aerodigestive tract, and pulmonary barotrauma from mechanical ventilation or in patients with pulmonary disorders. subcutaneous emphysema secondary to active injection of air has recently been reported following certain dental procedures. An interesting case of diffuse cervicofacial and mediastinal emphysema following a difficult extraction of an infected lower molar tooth with a high-pressure air drill is presented. The patient required airway observation and high-dose antibiotic therapy. Early recognition of this unique problem is essential in preventing such life-threatening complications as airway obstruction, mediastinitis, deep neck infection, and cardiac failure. Diagnostic and therapeutic recommendations are included.
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8/20. Otolaryngologic management of posttransplant lymphoproliferative disease in children.

    Twelve of 14 children (86%) given a pathologic diagnosis of posttransplantation lymphoproliferative disease (PTLD) at the Children's Hospital of Pittsburgh presented with head and neck symptoms, and are included in this retrospective analysis. Upper airway obstruction was the most common symptom, evident in nine children (75%). Ten children (83%) had febrile illnesses with dysphagia, odynophagia, and evidence of hypertrophy of components of Waldeyer's ring. Associated findings included cervical adenopathy, sinusitis, and otitis media. The two remaining children had an intratracheal and paratracheal mass, respectively. Excision of obstructing lymphoid tissue with proper handling of the specimen is advocated for diagnosis and relief of airway obstruction. Systemic therapy is necessary for treatment of PTLD and includes reduction of immunosuppression. erythromycin causes elevation in cyclosporine levels and should be avoided in patients taking this drug.
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9/20. AIDS, otolaryngology and a case of adenoid cystic carcinoma of the parotid arising in a patient with the aids-related complex.

    The incidence of acquired immunodeficiency syndrome (AIDS) and aids-related complex (ARC) is increasing, and as head and neck manifestations of this entity are common it is important that otolaryngologists have an up-to-date knowledge of this condition. In this paper the epidemiological aspects of the disease are reviewed and the head and neck manifestations discussed. An interesting case of adenoid cystic carcinoma of the parotid arising in a patient with ARC is presented.
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10/20. Principles and hazards of electrocautery in otolaryngology.

    Electrocautery is used extensively in otolaryngology--head and neck surgery. Its commonplace use and excellent safety record may lull the surgeon from heeding the device's potential dangers. User error causes the majority of patient injuries. A thorough understanding of the electrophysical principles and hazards is essential for even the occasional user. Three cases of electrosurgical injury are reported to demonstrate problem areas in electrocautery usage. The basic electrophysical properties are described, and guidelines for prevention of injury to the patient are reviewed.
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