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11/20. arthrogryposis multiplex congenita: otolaryngologic diagnosis and management.

    arthrogryposis multiplex congenita (AMC) is an uncommon congenital disorder characterized by multiple fixed joint deformities and non-progressive neuromuscular dysfunction. A small fraction of these infants will present with otolaryngologic problems resulting from cranial nerve weakness, muscle dysplasia, or structural dysharmony of the head and neck. The charts of 50 patients with AMC were reviewed to determine the incidence of these findings. A summary of the literature is presented discussing the etiology, pathophysiology, diagnosis and management of this interesting clinical problem.
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12/20. eikenella corrodens as a cause of recurrent and persistent infections of the head and neck.

    Four patients had eikenella corrodens infections involving the head and neck in different ways. The organism is discussed as a pathogen in mixed infections and as the predominant organism in other processes. As part of the resident microflora of mucous membrane surfaces, it is an important potential pathogen in a number of otolaryngologic clinical settings. Careful microbiological culturing and susceptibility testing reveal the causative organism and guide the antibiotic therapy.
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13/20. Otolaryngologic manifestations of Hansen's disease.

    A Mexican migrant farm worker whose condition was previously undiagnosed was examined at the University of utah Medical Center. He had an unusual peripheral neuropathy, ulcerative and nodular skin lesions, and multiple head and neck complaints. Results of the history, physical examination, and multiple biopsies led to the diagnosis of lepromatous leprosy. Although an uncommon disease in the united states, leprosy remains a common cause of head and neck pathologic conditions in many parts of the world and must be considered in the appropriate clinical setting.
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keywords = neck
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14/20. Digital subtraction angiography: applications in otolaryngology--head and neck surgery.

    Digital electronic technology has made its most notable contribution to radiology in the form of the CT scanners which have so greatly benefited medical practice. Experience with intravenous angiography using a digital fluoroscopy unit developed at the University of arizona gives promise of a further major benefit in the area of angiography. The majority of examinations are conducted on an outpatient basis with a risk level comparable to that of an intravenous pyelogram. The authors review their experience with this unit, including it's diagnostic and therapeutic applications to congenital and acquired vascular diseases, neoplasms, and vascular trauma of the head and neck.
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ranking = 5
keywords = neck
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15/20. Diagnosis and management decisions in infections of the deep fascial spaces of the head and neck utilizing computerized tomography.

    Infections of the deep spaces of the head and neck may still result in major consequences despite the advent of antibiotics. Abscesses in these areas merit special consideration by today's head and neck surgeon because of their relative rarity and the life-threatening complications that may follow inadequate treatment. Diagnosis and management decisions are enhanced by use of computerized tomography (CT) as an adjunctive study. The EMI scan may demonstrate either cellulitis of the neck requiring no surgery or a space abscess displacing the adjacent structures thus requiring surgical drainage. anatomy of the significant fascial planes and spaces of the neck will reviewed employing CT utilizing 3 mm cuts. Specific case presentations feature early diagnosis and management.
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keywords = neck
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16/20. head and neck manifestations of uncommon infectious diseases.

    Certain uncommon systemic infections may be present with head and neck manifestations either initially or during the course of the disease. A high index of suspicion is required on the part of the otolaryngologist with the subsequent procedures leading to the appropriate diagnosis. The manifestations of infectious diseases such as erysipelas, histoplasmosis, rabies, tetanus, botulism, and cysticercosis must be understood by the head and neck specialist. For successful management, many of the infections require prompt identification and initiation of therapy. Airway maintenance, ventilatory support, and medical chemotherapy may be required.
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keywords = neck
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17/20. head and neck manifestations of eosinophilia-myalgia syndrome.

    eosinophilia-myalgia syndrome (EMS) is a multisystemic disease that occurs in patients who have consumed products containing L-tryptophan. Prominent features include incapacitating myalgias, arthralgias, neuropathies, and eosinophilia. Despite the frequent association of dysphagia, dyspnea, and the potential for aspiration, the otolaryngology literature is devoid of information on EMS. In order to determine the frequency of otolaryngic symptoms, questionnaires were distributed to patients with EMS in 33 different US states. Among the 28 various head and neck manifestations studied, 70% of EMS patients complained of generalized muscle spasms, 66% xerostomia, 62% dyspnea, and 56% dysphagia. In addition, the epidemiology, clinical presentation, diagnostic criteria, and treatment options are discussed. This paper assesses the frequency of otolaryngic manifestations of EMS, as well as introduces this syndrome to the otolaryngologist-head and neck surgeon. It is important for the otolaryngologist to be aware of EMS and its manifestations and treatments so that patients with this potentially lethal disease can receive appropriate evaluation and expeditious treatment.
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ranking = 6
keywords = neck
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18/20. Wegener's granulomatosis. The great masquerade: a clinical presentation and literature review.

    Wegener's granulomatosis is classically a systemic disease which has masqueraded as many other disorders. The patient presenting with head and neck manifestations is often initially referred to the otolaryngologist. Although the natural history of this disease is fairly well understood, its etiology and pathogenesis are not clear. The response of Wegener's granulomatosis to appropriate therapy can be dramatic, whereas without treatment it is a progressive and mutilating disorder with life-threatening complications. Two cases which were recently managed at our center are presented in an attempt to illustrate the complexity of this disorder and the diagnostic and therapeutic dilemma which it presents. A literature review was carried out and a recent concept, the Multistep Evolution Hypothesis, as well as diagnostic advances in serological testing, are discussed. cyclophosphamide and systemic steroids, the mainstay of therapeutic intervention, as well as more recent modalities including plasmapheresis, folate antagonists, oral trimethoprim-sulfamethoxazole and local radiotherapy are reviewed.
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keywords = neck
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19/20. Otolaryngologic manifestations of multiple pterygium syndrome.

    Escobar Syndrome, or Multiple pterygium Syndrome (MPS), is a rare syndrome with multiple congenital anomalies involving the head and neck area and limbs. Affected individuals have multiple pterygia, camptodactyly and/or syndactyly as the main features of this syndrome. patients with MPS have a characteristic facies, including ptosis, antimongoloid slant of the palpebral fissures, hypertelorism, micrognathia, neck pterygia, and a sad flat, emotionless look to the face. We present a case of MPS recently treated at our institution for the purpose of further describing the clinical features of this syndrome, emphasizing the otolaryngologic manifestations. Increased awareness of MPS will facilitate appropriate management of this syndrome.
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ranking = 2
keywords = neck
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20/20. Otolaryngologic manifestations of sarcoidosis: presentation and diagnosis.

    Neuro-otologic manifestations of sarcoidosis are rare. dizziness in particular is a rare presenting complaint of the patient afflicted with this systemic granulomatous disorder. head and neck and sinonasal presentations of this disease are more common. We reviewed our experience with six such patients who presented for management of their otolaryngologic (ORL) manifestations in order to delineate the involvement of the otolaryngologist in the treatment and diagnosis of these patients, with a focus upon the relevant tests and procedures in the otolaryngologist's de novo diagnosis of sarcoidosis. Studies ordered in the course of otolaryngologic evaluation and their utility in the diagnosis of sarcoidosis by the otolaryngologist are reviewed and classified into useful, supportive, and ancillary. The otolaryngologist played an important role in diagnosis, with four of six patients diagnosed to have sarcoidosis on the basis of their otolaryngologic presentations. biopsy was performed by the otolaryngologist for diagnosis of sarcoidosis in all four of these cases. steroids were central to treatment. Oral steroid therapy was the principal treatment: both patients with neuro-otologic sarcoid were successfully managed with oral steroids. Intralesional steroids were necessary to treat the skin lesion. One of six patients patients experienced complications related to steroid use.
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