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Laryngeal involvement in systemic lupus erythematosus.

Laryngeal involvement in systemic lupus erythematosus (SLE) can range from mild ulcerations, vocal cord paralysis, and edema to necrotizing vasculitis with airway obstruction. In this report, four cases showing the range of severity of this disease manifestation are presented, accompanied by a comprehensive review of the literature. The clinical course of 97 patients with laryngeal involvement with SLE are reviewed, of whom 28% had laryngeal edema and 11% had vocal cord paralysis. In the majority of cases, symptoms such as hoarseness, dyspnea, and vocal cord paralysis resolved with corticosteroid therapy. Other, less common causes of this entity included subglottic stenosis, rheumatoid nodules, inflammatory mass lesions, necrotizing vasculitis, and epiglottitis. The clinical presentation of laryngeal involvement in patients with SLE follows a highly variable course, ranging from an asymptomatic state to severe, life-threatening upper airway compromise. With its unpredictable course and multiple causations, this complication remains a diagnostic and therapeutic challenge to physicians involved in the care of patients with SLE.

Adult↗

Bilateral abductor paresis masquerading as asthma.

Rare upper airway lesions may be mistaken for asthma. A 16-year-old Hispanic male athlete presented to our allergy clinic with a 4-month history of wheezing and snoring with hoarseness and progressive fatigue on exertion or during sleep. His mother taped periods of harsh stridor and sleep apnea. There was no family history of vocal cord abnormalities. A year before the onset of symptoms, he suffered injury to his oral cavity with a loss of consciousness during a wrestling match. He denied dysphagia or dysphonia. He failed to respond to bronchodilators, cromolyn, or prednisone therapy during 4 weeks. On referral to our clinic, his physical examination and tape recording were characterized by harsh inspiratory stridor. His pulmonary function tests were significant for peak flow depressed out of proportion to FEV1 with reduced FVC, no response to bronchodilator, and flattened inspiratory loop unresponsive to cough or panting. Fluoroscopy and endoscopy of the upper airway was consistent with "marked bilateral limitation of vocal cord abduction." Sleep study demonstrated desaturation with CO2s in the 60s during sleep. He was started on continuous positive airway pressure, 10 cm at night, with no desaturation or sleep disturbance on follow-up.

Adolescent↗

Congenital laryngeal defects.

Embryologic disorders of the larynx are rare. The main symptoms are stridor and hoarseness, symptoms which in the newborn always necessitate further investigation. Both the laryngotracheo-esophageal cleft and the congenital laryngeal web are discussed in relation to the embryologic development of the larynx and to diagnosis and therapy. The use of the operating microscope is stressed.

Esophagus↗

Carcinoma of the larynx in a child.

Squamous cell carcinoma of the larynx in children is a rare entity, only 21 cases have been reported in patients 10-years-old or younger. A 9-year-old girl presented because of dyspnea and mild hoarseness of one week duration. Laryngoscopy and bronchoscopy showed a supraglottic mass, which proved to be poorly differentiated squamous cell carcinoma. No neck nodes or systemic metastasis were present. The tumor regressed with radiation therapy (6400 rads) but local recurrence and lung metastasis were evident 6 weeks later. Chemotherapy was refused by the parents and the patient died in one month. It is believed that squamous cell carcinoma of the larynx in children parallels that of adults in many respects including response to treatment. However, our attempt to save the larynx by avoiding surgery and using radiotherapy alone was not successful. Squamous cell carcinoma of the larynx in children may be more aggressive than that of adults. The scarcity of cases impedes establishment of treatment protocols in children. Initial aggressive management using surgery, radiotherapy, and possibly chemotherapy may be warranted in children.

Age Factors↗

Ectodermal dysplasia: the otolaryngologic manifestations and management.

Ectodermal dysplasia is a rare group of diseases presenting special problems in management for the otolaryngologist, but the full spectrum of otorhinolaryngologic manifestations has been previously unrecognized in the otolaryngologic literature. The anhidrotic form, characterized by deficient sweating, sparse hair growth and deficient teeth, with associated decreased mucous production in the aerodigestive tract leads to chronic upper respiratory tract infections, otitis, dysphagia, hoarseness, bronchitis and sometimes hemoptysis.

Acute Disease↗

Voice problems after pediatric laryngotracheal reconstruction: videolaryngostroboscopic, acoustic, and perceptual assessment.

Children with laryngotracheal stenosis undergo laryngotracheal reconstruction (LTR) to permit tracheostomy decannulation. We conducted voice assessments of 8 pediatric LTR patients with voice problems. Perceptual ratings and acoustic measures were taken in 5 patients and videolaryngoscopy/laryngostroboscopy was conducted in all 8. Results of perceptual evaluation were abnormal for hoarseness, breathiness and pitch. Acoustic analysis revealed a markedly decreased fundamental frequency of phonation, diminished frequency range, decreased vocal intensity, and shortened maximum phonation time. Videolaryngostroboscopic findings are summarized as follows: 2 patients had severe glottal insufficiency, 3 patients had vocal fold asymmetry in stiffness and position, and 3 patients demonstrated phonation using supraglottal structures. Use of the stroboscope contributed to diagnosis in 3 of 8 patients. Reverse (inhalatory) phonation was observed in three patients. Concomitant articulation problems were exhibited frequently. Sentence production was shortened by the decreased phonatory duration. When coupled with the abnormal voice quality, speech intelligibility was significantly affected. Voice problems following decannulation were observed to persist as a long-term difficulty in some LTR patients.

Adolescent↗

Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year-old female: case report and review of the literature.

OBJECTIVE: To alert practitioners to the risk of sudden airway obstruction and death in patients with Wegener's granulomatosis. DESIGN: Case report and literature review. SETTINGS: University and Community hospitals. PATIENT: A 17-year-old white female. INTERVENTION: (1) evaluation and treatment for mental status changes over 2 months. (2) Evaluation and surgical biopsy of nasal septal perforation under general anesthesia at a university children's hospital. (3) Evaluation and observation at a community hospital 2 days later. (4) autopsy. RESULTS: Serology performed 3 days ante-mortem revealed (at 2 days post-mortem) cytoplasmic anti-neutrophil cytoplasmic antibody positive at 1:128. Autopsy was significant for microscopic fibrosis and granulomas in the kidneys and essentially total obstruction of the subglottis and upper trachea by a 3.5 x 1 x 1 cm mass of fibrosis and granulomas overlying circumferentially necrotic mucosa. This mass was centered on the crico-tracheal junction. CONCLUSIONS: Wegener's granulomatosis can lead to proliferative tissue growth with acute airway obstruction in the larynx and trachea, and death. Any patient with WG under age 20, as well as patients with WG and laryngotracheal symptoms (e.g. strider, hoarseness, wheezing) would benefit from evaluation of the airway.

Adolescent↗

Transesophageal echocardiography and magnetic resonance imaging for the assessment of saccular aneurysm of the transverse thoracic aorta.

We report a patient who initially presented with hoarseness and was admitted to our hospital with chest pain, caused by a saccular aneurysm of the thoracic aortic arch. The initial diagnosis was made by cross-sectional echocardiography, the extension and morphology of the saccular aneurysm being detected by transesophageal echocardiography. Magnetic resonance imaging confirmed the measurements of the aneurysm and clearly showed the anatomic relation with surrounding structures and arch vessels. The patient refused operation and died during in-hospital stay. A rupture of the thoracic aneurysm was the cause of death.

Aged↗

Glottic and supraglottic carcinoma: a retrospective comparison of radiotherapy alone with sandwich therapy in 366 patients.

From 1971 through 1982, 442 patients with laryngeal carcinoma were seen at the Leiden University Hospital. They were treated either with radiotherapy alone, sandwich therapy (pre- and postoperative radiotherapy) or by surgery followed by postoperative irradiation. Three hundred and sixty-six patients with glottic or supraglottic tumours could be analysed with respect to two different treatments, complications of treatment and some prognostic factors. Two endpoints of analysis were used: disease-free interval and survival to cancer death. In patients with glottic or supraglottic carcinoma, the survival of patients with advanced disease, treated with radiotherapy only, was worse as compared to the survival of the same category of patients who were treated with sandwich therapy (p less than 0.005). In patients with small glottic tumours, radiotherapy alone was mostly used. In small supraglottic tumours, the survival with both therapy policies was equal. Persistent hoarseness in patients with small glottic tumours, treated with radiotherapy only, is of predictive value for the development of a recurrence (p less than 0.001). There was no influence on prognosis of histological differentiation of the tumour. It appeared that interruption of radiotherapy for more than two days had an adverse effect on survival in patients with glottic carcinoma (p = 0.0001). Finally, the occurrence of second malignancies was analysed. It was found that 19% had a second malignancy. Almost 60% of them were lung cancers.

Aged↗

Radiotherapy for primary carcinoma of the trachea.

Between 1940 and 1985, 24 cases of primary carcinoma of the trachea were registered at the London Regional Cancer Centre. The most common presenting symptoms were hoarseness, haemoptysis and cough. Twenty patients had epidermoid carcinoma and four had adenoid cystic carcinoma. Because of different clinical behaviours, the two histologies were separately analyzed. Of the 20 patients with epidermoid carcinoma, 19 received radiotherapy as primary treatment and one patient did not receive radiotherapy because of advanced disease. Radiation doses ranged from 4000 to 6000 cGy and most patients had megavoltage irradiation. Treatment result was disappointing. Only one patient remained disease-free at 15-month follow-up and all other patients had persistent or recurrent tracheal tumour. Median survival for all 20 patients was 5 months (range 1 to 19 months). Of the four patients with adenoid cystic carcinoma, two had primary surgery and postoperative radiotherapy and two had primary radiotherapy. Two patients died of disease, at 5 months and 8 years from diagnosis. Two surviving patients had 15-month follow-up: one had persistent disease and the other was free from recurrence. In this study, radiotherapy within the range of doses given was found to be an ineffective primary treatment for tracheal carcinoma.

Adult↗

Functional roles of the superior laryngeal nerve afferents in electrically induced vocalization in anesthetized cats.

Our purpose was to elucidate the functional roles of the laryngeal afferents in controlling vocalization. We investigated the effects of laryngeal deafferentation (sectioning the internal branch of the superior laryngeal nerve (ISLN)) on respiration and voice quality during electrically-induced vocalization in twelve ketamine anesthetized cats. Co-ordinated vocal activity was obtained by electrical stimulation to the pontine call site. After the bilateral ISLN section, the respiratory, expiratory and inspiratory durations during induced vocalization became 0.56 +/- 0.15, 0.44 +/- 0.12 and 0.67 +/- 0.19 (mean +/- S.D., n = 9) times, respectively, compared with those before the ISLN section. A decrease in respiratory duration was also observed when local anesthetics were applied to the laryngeal mucosa. The laryngeal deafferentation increased the degree of hoarseness with a decrease in the fundamental frequency. Since the laryngeal deafferentation caused a decrease in the intralaryngeal adductor activities, it was suspected that the voice quality change was partly caused by the reduction in adductor activities. It was thus concluded that feedback via laryngeal afferents plays an important role in controlling vocalization.

Anesthesia↗

Clinical implication of symptoms in patients with non-small cell lung cancer treated with definitive radiation therapy.

To clarify clinical significance of symptoms presented at the beginning of treatment, we analyzed the symptoms in 240 patients with non-small cell lung cancer treated with definitive radiation therapy. Symptoms were classified into four groups: no symptom (Grade 0), cough, sputum/hemosputum and fever up (Grade 1), chest pain and breathlessness (Grade 2), appetite loss, body weight loss, SVC syndrome, hoarseness, and pain in the upper limb/shoulder (superior sulcus tumor) (Grade 3), and their therapeutic outcomes were examined. The 2- and 5-year overall actuarial survival rates for patients with squamous cell carcinoma were 38.5% and 15.4% for Grade 0, 40.5% and 20.1% for Grade 1, 17.9% and 2.6% for Grade 2, and 15.8% and 5.3% for Grade 3. A statistical difference was noted in survival between Grades 0-1 and Grades 2-3 (P < 0.01), but was not seen between Grades 0 and 1, or between Grades 2 and 3. As for patients with stage III disease alone, the difference in survival was still significant between Grades 0-1 and 2-3 (P < 0.05). In patients with adenocarcinoma/large cell carcinoma, however, no obvious relationship was found between symptoms and prognosis, except for body weight/appetite loss. In conclusion, though the symptoms closely related to clinical stage and performance status and not an independent prognostic factor, chest pain and breathlessness correlated with poor therapeutic outcome as well as body weight loss and T3-4 related symptoms, while cough, sputum (hemosputum), and fever were favorable symptoms in patients with squamous cell carcinoma who received definitive radiation therapy.

Actuarial Analysis↗

Lipoid proteinosis.

Lipoid proteinosis (Urbach-Wiethe disease) is a rare, recessively inherited disorder that is characterized by the deposition of hyaline-like material in the skin, oral cavity, and other tissues. It usually appears in infancy with hoarseness. We report a case of lipoid proteinosis in a 10-year-old boy that demonstrates the characteristic clinical, histologic, and ultramicroscopic features of this disease.

Child↗

Jugular foramen syndrome as a complication of metastatic melanoma.

INTRODUCTION: The jugular foramen (Vernet) syndrome with neuropathy of cranial nerves IX-XI is more commonly due to metastatic lesions than primary neoplasms. Hypoglossal nerve involvement is referred to as the Collet Sicard variant of the jugular foramen syndrome. MATERIALS AND METHODS: We report an elderly man who presents with dysphagia, dysarthria, hoarseness, and a 12-pound weight loss. Examination demonstrated paralysis of the right vagus and hypoglossal nerve. Communication and collaboration with the radiologist resulted in identification of the lesion. Computed tomographic (CT) guided needle aspiration confirmed metastatic melanoma. DISCUSSION: The onset of cranial neuropathy may allow the site of lesion to be predicted based on knowledge of the cranial base and neural anatomy. Diagnostic evaluation is tailored to evaluate the area in question. Active, multidisciplinary collaboration is essential for success.

Accessory Nerve↗

Arytenoid subluxation from blunt laryngeal trauma.

Isolated arytenoid dislocation and subluxation are uncommon laryngeal injuries most often resulting from endotracheal intubation. However, these diagnoses must be entertained in all patients having sustained laryngeal trauma. Complaints of dysphonia, pain with phonation, or odynophagia in the setting of laryngeal trauma should include evaluation for possible arytenoid displacement after an airway is secured. Prolonged hoarseness or odynophagia after endotracheal intubation should alert the physician to the possibility of a cricoarytenoid joint injury. This represents the first reported case of isolated arytenoid injury resulting from blunt external trauma to the larynx. The patient had a stable airway without intervention, and the displaced joint spontaneously relocated with resolution of the cricoarytenoid edema and hemarthrosis. We propose that the cricoarytenoid joint was subluxed probably due to edema, hematoma, and/or cricoarytenoid hemarthrosis sustained from blunt laryngeal trauma. We furthermore propose that some cases of cricoarytenoid subluxation may be treated without operative intervention.

Adult↗

Relapsing polychondritis in an adolescent.

Relapsing polychondritis (RP) is an uncommon systemic disorder with a highly variable course. A 17-year-old woman recently presented with a 1-month history of depression, weight loss, chest wall tenderness, hoarseness, and dysphagia. Physical examination revealed cachexia, low-grade fever, pharyngeal erythema, and tenderness of the right auricle, anterior chest, cricothyroid cartilage, and both knees. Laboratory studies included a hematocrit of 34% and a sedimentation rate of 50 mm/hr. Initial improvement on oral corticosteroids was followed by respiratory distress. At that time calcified tracheal cartilage, subglottic stricture, and a saddle nose deformity were present. Despite therapy with steroids, dapsone, and pulse cyclophosphamide, the respiratory distress reoccurred, eventually necessitating tracheostomy. Tracheal cartilage biopsy confirmed the presumptive diagnosis of RP. Bilateral auricular chondritis developed after initial presentation, as did acute vertigo. Although seen in all age groups, less than 10% of cases of RP are seen in children and adolescents. Auricular chondritis (89% of all cases), inflammatory asymetric arthritis (81% of all cases), nasal chondritis (72% of all cases), respiratory tract chondritis (56% of all cases), and audiovestibular abnormalities (46% of all cases) were present in our patient. Relapsing polychondritis may follow a slowly evolving or rapidly progressive course. Appropriate diagnosis and aggressive therapy are recommended to lessen the morbidity and mortality.

Adolescent↗

Extent of sympathetic blockade after stellate ganglion block with bupivacaine.

A study of the extent of sympathetic blockade after stellate ganglion block was assessed using liquid crystal thermography. Two volumes (10 and 20 ml) of bupivacaine 0.5% plain were used. Irrespective of the volume used cranial sympathetic block always occurred and thoracic sympathetic block never occurred. While upper cervical block was present in all patients, lower cervical sympathetic block was present only in the 20-ml group (P less than 0.05). The larger volume was associated with a significant incidence of hoarseness due to spread of local anaesthetic onto adjacent laryngeal nerves.

Brain↗

A 1-year follow-up of low birth weight infants with and without bronchopulmonary dysplasia: health, growth, clinical lung disease, cardiovascular and neurological sequelae.

A cohort of 86 low birth weight (LBW infants (< 1751 g) including 23 subjects with bronchopulmonary dysplasia (BPD) and 23 matched controls without BPD were checked up at corrected ages of 1, 2, 4, 6, 8 and 12 months. Thirty-six of the subjects were re-hospitalized, mostly because of infections, there being no significant difference in the number of hospitalizations between BPD cases and their controls. Delayed growth was frequent, but a catch-up growth was seen. Eighteen of the subjects (5 with BPD and 7 of controls) were of height below 2 SD at a corrected age of 12 months. Twenty-four (14 with BPD and 3 controls) had persistent respiratory symptoms during the follow-up period and 4 of the BPD subjects still had such symptoms at the age of 1 year. Hoarseness was more frequent in BPD cases than in the controls (8/1 cases). One subject with BPD had transient symptoms of cor pulmonale and 1 without BPD developed transient systemic hypertension. Nine subjects (4 with BPD and 2 controls) had a diagnosis of cerebral palsy at the age of 1 year.

Bronchopulmonary Dysplasia↗