Otolaryngologic manifestations of acquired syphilis.
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Biomedical subjects
Publications and source records attributed to M Strome.
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OBJECTIVES: Inclusion body myositis (IBM) is an inflammatory myopathy with a 40% reported incidence of dysphagia. A protracted course, refractory to medical therapy, frequently leads to consultation with an otolaryngologist for dysphagia management. We studied the incidence, symptoms, and mechanisms of dysphagia in patients with IBM. STUDY DESIGN: Retrospective study of medical records and self-reported follow-up survey; dysphagia is defined as difficulty in swallowing. MATERIALS: Twenty-two patients with biopsy-proven IBM. RESULTS: The rate of dysphagia was more than 80% (16 of 19), twice as high as previously reported. Progressive dysphagia was associated with a significantly worse functional class. Relevant management guidelines are established, including the timing for appropriate surgical intervention. CONCLUSION: Progressive dysphagia may signify more aggressive IBM or an episodic worsening in status. Recognition of the disease manifestations will afford proper patient management. Informed otolaryngologists can have a favorable impact on the dysphagia associated with IBM.
Contraction of paralyzed striated muscles has been restored by stimulating reinnervating pedicles with currents of low intensity. In order to allow clinical application, stable, long-term excitability must emulate the parameters necessary for the stimulation of normal motor nerves. In 6 dogs, the ansa hypoglossi nerve was implanted into the contralateral denervated sternohyoid muscle and surrounded with a bipolar cuff electrode. Three of the reinnervating pedicles were chronically paced with a Medtronic Itrel II Multiprogrammable Pulse Generator (0.5 V, 0.2 second on [30 pulses per second, 0.21-millisecond pulse width], 2.9 seconds off). At reexploration after 8 months (6 months for 1 dog), frank contraction confirmed by electromyography tracings occurred in all animals with currents in the range of 0.1 to 0.5 mA. Muscle force was further manipulated by selective release of blocking currents (600 Hz, 1.7 to 0.4 mA) superimposed over regular stimulation (50 Hz, 0.3 to 1.7 mA). Nerve and muscle vitality were histologically confirmed. Long-term, low-intensity conduction capabilities, fine tuning, good tolerance of implanted electrodes, and lack of fatigue suggest that reinnervating pedicles may be successfully used for pacing when clinically indicated.
BACKGROUND: Treating the neck after organ-preservation treatment with radiotherapy or chemoradiotherapy can be problematic. METHODS: To develop management guidelines, we reviewed the results of a 100-patient phase-3 trial that had compared outcome after radiotherapy alone with outcome after chemoradiotherapy for head and neck cancer. Patients were randomly assigned to receive radiotherapy alone or concurrent chemoradiotherapy. After completing therapy, patients were reassessed, and surgery was recommended for persistent disease at the primary site or neck and for all patients with stage N2-3 neck nodes regardless of clinical response. RESULTS: Of the 47 patients with stage NO-1, 43 had a complete response (CR); of the 18 N1 patients, all but 4 had a CR. One of these 4, as well as 5 others among the NO-1 patients, underwent neck dissection (n = 6). No disease was found on pathologic examination, and no patient had neck recurrence. Of the remaining 41 N0-1 patients, 3 had disease progression and received no further therapy. Of the 38 others, 4 had neck recurrence, with 3 recurring at the primary site. Of the 53 with stage N2-3, 23 had less than a complete response (<CR), and 30 had a CR. In 35 N2-3 patients, neck dissection was performed as planned. Of these 35, 18 had a CR in the neck; 4 had positive nodes on pathologic examination. The other 17 had a <CR in the neck; 8 had positive nodes on pathologic examination. One patient in this group of 17 had regional recurrence after a pathologically negative neck dissection. Of the 18 N2-3 patients who did not undergo planned neck dissection, 6 had tumor progression and had no further therapy. The other 12, all with a CR in the neck, were followed, and 3 had neck recurrence; none successfully salvaged. Despite a CR in 30 N2-3 patients, 7 had persistent disease or eventual neck recurrence. Adding neck dissection minimized neck recurrence (p = .05). In N2-3 patients, disease-specific survival was significantly better in patients with a CR in the neck (p = .002). Disease-specific survival was not affected by neck dissection (p = .40) but was significantly affected by viable tumor in the specimen (p = .03). CONCLUSION: Based on these results and the realization that it is difficult to follow patients for recurrent neck cancer, that salvage is often unsuccessful, and that patients dying from uncontrollable neck disease have an extremely poor quality of life, we recommend neck dissection for all N2-3 patients regardless of the neck response and for N1 patients without a CR.
BACKGROUND: A phase III randomized comparison of radiotherapy alone versus combination chemotherapy and concurrent continuous-course radiotherapy was performed at the Cleveland Clinic Foundation. METHODS: Between March 1990 and June 1995, 100 patients with resectable stage III and IV squamous cell head and neck cancer were randomized to either Arm A: radiotherapy alone, 68-72 Gy at 1.8-2.0 Gy per day; or to Arm B: the identical radiotherapy with concurrent chemotherapy. Chemotherapy consisted of 5-fluorouracil, 1000 mg/m2/day, and cisplatin 20 mg/m2/ day, both given as continuous intravenous infusions over 4 days beginning on day 1 and day 22 of the radiotherapy. At 50-55 Gy, patients were clinically reassessed. If a response was evident, radiotherapy was completed. In non-responding patients, however, radiotherapy was terminated and surgery recommended. After completion of all treatment, salvage surgery was performed, if possible, for any residual primary or nodal disease or for any subsequent locoregional recurrence. RESULTS: Except for an overrepresentation of T1 patients on Arm A, the treatment arms were equivalent. Toxicity was greater in the patients on Arm B with a higher incidence of grade III and IV neutropenia, thrombocytopenia, cutaneous reaction, and mucositis. Feeding tubes were also required more often, and weight loss was greater on the chemotherapy arm. No toxic deaths occurred. With a median follow-up of 36 months, the Kaplan-Meier 3-year projections of relapse-free survival are 52% for Arm A and 67% for Arm B (p = .03), and the likelihood of developing hematogenous metastases is 21% for Arm A and 10% for Arm B (p = .04). Although overall survival is not significantly different, overall survival with successful primary site preservation was 35% for Arm A and 57% for Arm B (p = .02). This difference remains statistically significant in the subsets of patients with laryngeal and hypopharyngeal primaries but not in patients with oropharyngeal primaries. CONCLUSIONS: Continuous-course radiotherapy and concurrent combination chemotherapy is an intensive, toxic but tolerable treatment regimen, which, when compared with radio therapy alone, can produce an improvement in relapse-free survival, a decrease in distant metastases, and an improvement in overall survival with successful primary site preservation.
Laryngeal adduction for swallowing chiefly involves contraction of the thyroarytenoid and lateral cricoarytenoid muscles to seal the glottic chink. Vocal cord elongation supplements closure through cricoarytenoid activation. Relaxation of the posterior cricoarytenoid muscle is also involved in the swallowing process. Recent interest has focused on stimulating the laryngeal nerves to protect the lower airway from conditions where normal muscular coordination may be disrupted (e.g., in aspiration following stroke). Unfortunately, electrical stimulation results in a generalized contraction of all the dependent intrinsic laryngeal muscles because the larger, more excitable axons fire before their smaller counterparts can be activated. In the physiological state, however, the smaller fibers are recruited first. The current study focuses on electronic manipulation of force in the glottic muscles involved in deglutition. We used a stimulator that could selectively activate the intrinsic laryngeal muscles based on their specific motor unit architectures. In 5 dogs, the circuit recruited the axons in the recurrent and superior laryngeal nerves from small to large. The muscles were identified according to the differential recruitment rates of their compound muscle action potentials as they appeared on the graph. The smaller axons in the thyroarytenoid recruited faster than the large ones found in the lateral cricoarytenoid muscles, with intermediate figures observed with the cricothyroid. The posterior cricoarytenoid presented with the slowest recruitment rates, as expected from this muscle's highest contingent of larger motor units. Latencies between the onsets of stimulations and muscle saturations also appeared stable. This approach to manipulating glottic force saves energy because it allows stimulating the adductory muscles with minimal interference from their abductor antagonist.
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The specific performance of intrinsic laryngeal muscles has been difficult to evaluate, especially in living subjects. To reproduce natural contractions, we artificially induced orderly recruitment of their innervating axons according to the size principle. In 5 dogs, both recurrent laryngeal nerves (RLNs) were stimulated with 50 through 10 Hz, 300 to 1000 microA currents while 600 Hz, 1000 to 0 microA decreasing blocking currents were administered. Surface electromyography electrodes placed on the thyroarytenoid, posterior cricoarytenoid, and lateral cricoarytenoid muscles were used to determine the amplitudes (in mA) of compound muscle action potentials. There was a highly statistically significant difference (P<.004) between the thyroarytenoideus which had the fastest rate of recruitment (8.38%), and posterior cricoarytenoideus, which had the slowest (4.81%). There was an intermediate recruitment rate (6.72%) of the lateral cricoarytenoideus, a divergence attributed to a more equal distribution in fast and slow types of myofibers and a smaller sample. We submit that RLN axons can be recruited in an orderly manner according to their sizes and that the rates are unique to the muscle classes they innervate. The parameters defining these contraction patterns may offer key information for laryngeal pacing.
The treatment of laryngotracheal stenosis remains challenging. Even with the wide variety of current techniques available, optimal reconstruction is not possible for all patients. However, the future of laryngotracheal reconstruction is promising with ongoing development of innovative techniques for repair, as well as the prospect of laryngeal transplantation.
Laryngeal transplantation was actively investigated in the late 1960s and early 1970s using a dog model. An expanded knowledge base in immunobiology, pharmacology, and deglutition makes reassessment timely after an approximately 20-year hiatus. The basic parameters to be evaluated include varied methods of preservation, optimal immunosuppressive drug regimens, and the role of radiation therapy. Our current research protocol addressing the latter considerations is outlined, including a brief introduction of a new animal model developed specifically for this purpose. We will ultimately attempt to answer the question: Is laryngeal transplantation feasible?
In earlier laryngeal transplantation studies by Takenouchi, et al., the longest ischemic interval tolerated was 45 minutes. A new animal model and enhanced preservative solutions made reassessment timely. This study evaluated two determinants of graft viability: 1. the duration of ischemia and 2. the composition of the preservative media. Three groups of viable transplants were assessed. Groups I and II were preserved with iced heparinized saline with respective ischemic intervals of 3 and 6 hours. Group III was preserved with the Wisconsin solution during a 20-hour ischemic interval. All animals were sacrificed at 24 hours. Representative sections of group I confirmed viability whereas group II exhibited both clinical and histologic evidence of irreversible vascular change. In contrast, most representative sections in group III had little demonstrable change. These data suggest that laryngeal allografts can endure prolonged ischemic intervals if properly maintained.
This study was designed to determine the efficacy of corn oil as an alternative vehicle to olive oil for emulsifying cyclosporin (CsA) in preventing rejection of transplanted rat larynges. The issue of varied site absorption was also addressed. Thirty animals were transplanted to get 5 viable transplants at two weeks for three varied sites of administration; intramuscular (IM), subcutaneous (SQ) and intraperitoneal (IP). Five mg/kg of CsA in corn oil was the dose administered based on earlier data generated in our laboratory. Postulating selective absorption, the indirect measure of laryngeal histopathology, i.e. rejection, was chosen over blood levels for evaluation. In the IM group 2 grafts evidenced mild rejection whereas 3 showed marked cellular and vascular rejection. The SQ group had 1 mild, 1 moderate and 3 with severe rejections. The IP group had one moderate rejection and 4 severe rejections. Qualitatively the IM and SQ groups were similar. The IP group histologically evidenced far greater cellular rejection. CsA 5mg/kg emulsified in corn oil did not differ substantively in histologic scope or pattern of rejection from CsA in olive oil in experimental rat laryngeal transplantation. Further, the data did not support a change in the administration of CsA from an intramuscular site.
Since 1983, Cyclosporin A (CsA) has been the most successful primary drug in preventing rejection of organ transplants. This study was designed to determine the efficacy and dose response of CsA in preventing rejection of LBNF-1 rat allografts to Lewis recipients. Four groups of animals were studied. Group I served as the control, and groups II, III, and IV were given daily intramuscular doses of CsA for 1 month. The groups were given doses of 5 mg/kg, 7.5 mg/kg, and 10 mg/kg, respectively. Sixty-eight animals were transplanted to get eight viable transplanted animals at 1 month in each CsA group. Laryngeal viability was assessed with both clinical and histological parameters. Groups II, III, and IV had representative clinically viable larynges. The histology varied and had some correlation with CsA dosage. Group II evidenced changes ranging from mild to severe rejection. Group III was more homogeneous with the most severe change being characterized as mild-to-moderate rejection. Group IV was the most uniform with all representative specimens showing only limited infiltration of inflammatory cells with intact mucosa and submucosal glands (mild rejection). None of the CsA groups evidenced the squamous metaplasia characteristic of the control group. CsA can prevent rejection of laryngeal allografts from LBNF-1 donors to Lewis recipients.
This study used both clinical and histopathologic criteria to define the sequence and time parameters of rejection in histoincompatible vascularized rat laryngeal allografts. Clinical onset of rejection was characterized at 1 week by graft edema. Pathologic examination at this time revealed arterial wall thickening and a diffuse lymphocytic and macrophage mucosal infiltrate. At 14 days, the graft was encased in an inflammatory exudate with no visible cartilaginous structure. This correlated microscopically with a lymphocytic infiltrate of the lamina propria, loss of minor salivary glands, and squamous metaplasia of the surface respiratory epithelium. Arterial thrombosis was always correlated with tissue necrosis, but venous occlusion, occurring more than 2 days after transplantation, was not an independent determinant of graft viability. Definition of the time sequence and histopathology of rejection will allow future determination of the efficacy of various immunosuppressive regimens.
A new rat model was developed to reexamine the potential for laryngeal transplantation. The final anatomic derivation evolved from two earlier developmental phases. The first model had only a single arterial anastomosis; the second had an end-to-end arterial anastomosis with an end-to-end arteriovenous shunt. The final product employed an end-to-side arterial shunt and an end-to-side arteriovenous shunt for revascularization. The allografts were sited in tandem with the intact recipient larynges and were not innervated. A total of 16 animals were studied in phase 3; 2 died and the remaining 14 had a 64% arterial patency at intervals of 1 to 14 days. Our purpose is to detail the relevant technical considerations of this new model and compare it with historical controls.
The otolaryngologic manifestations of Down syndrome often masquerade as common and seemingly trivial conditions. Behind this facade lies a broad spectrum of disease which can detract from an inherently diminished level of function and jeopardize survival. This paper examines the pertinent associations between Down syndrome and various pathological entities related to the head and neck. Evaluation of current therapeutic modalities reinforces the need for early diagnosis and treatment so that the potential of this patient group is fulfilled.
Clinically important aspiration following the creation of a phonatory myomucosal shunt has occurred in 16% of our cases. Gax collagen was used in three cases therapeutically with excellent results. We report the technique used, the properties that make it efficacious, and the case specifics.
The ethics, justification, history, future, and rejection of laryngeal transplantation are examined in this article. The authors believe that laryngeal transplantation will be a viable alternative for a select group of patients before the end of this century.