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Sudden onset of velopharyngeal insufficiency in Klippel-Feil syndrome.

A 17-year-old girl with Klippel-Feil vertebral anomalies presented with sudden onset of VPI and hoarseness after a minor neck torsion injury. Her symptoms were felt to be due to compression of the brain stem by the odontoid process (through basilar invagination). The VPI and hoarseness responded to conservative management with oral steroids.

Adolescent↗

[Relapse of acute monocytic leukemia present with skin and tracheal involvement].

A 34 year-old female was admitted because of anemia and leukopenia. Her bone marrow contained abundant blastic cells, which were histochemically positive for peroxidase and alpha-naphthyl butyrate esterase, but negative for ASD chloroacetate esterase. She was diagnosed as acute monocytic leukemia (FAB, M5a). Complete remission was achieved after the administration of BHAC, daunorubicin, 6MP and prednisolone, and she was discharged after consolidation therapies. But shortly later, she noticed hoarseness and erythematous nodules on her breast and abdomen. Though the examinations of peripheral blood and bone marrow did not show any abnormality, hoarseness rapidly worsened and she complained of dyspnea. X-ray and CT scan demonstrated narrowing of the trachea under the cricoid cartilage, and trans-tracheal biopsy revealed leukemic involvement. In addition, erythematous skin lesion showed the infiltration of leukemic cells by biopsy. Although radiation and chemotherapy was initiated, she died of pneumonia. We tried to discuss the laryngo-tracheal and skin involvement of acute monocytic leukemia as early symptoms of relapse.

Adult↗

Vocal cord paralysis.

The information presented in this article demonstrates that unilateral or bilateral vocal cord paresis or paralysis in infants and children is difficult to diagnose and difficult to manage. In an attempt to provide the otolaryngologist with a concise set of relevant guidelines, the following rules for management are presented here. 1. Suspect bilateral abductor vocal cord paralysis (BAVP) when a neonate or infant presents with high-pitched inspiratory stridor and evidence of airway compromise. Factors that should increase the suspicion of BAVP include associated Arnold-Chiari malformation; congenital anatomic abnormality involving the mediastinum (for example, tracheoesophageal fistula, vascular ring, other vascular anomalies); dysmorphic syndromes, especially those involving brainstem dysfunction; and manifest findings indicative of neuromuscular disorder. The neonate or infant with Arnold-Chiari malformation and inspiratory stridor has bilateral abductor vocal cord paralysis until proven otherwise. 2. Suspect unilateral vocal cord paresis or paralysis in an infant or child with hoarse voice, low-pitched cry, or breathy cry or voice. The infant who develops mild stridor and hoarse cry following surgical repair of a patent ductus arteriosus or tracheoesophageal fistula has a unilateral vocal cord paralysis until proven otherwise. 3. Direct laryngoscopy with the flexible fiberoptic nasopharyngolaryngoscope and photodocumentation using a videocassette recorder offers the best method for diagnosis of vocal cord paresis or paralysis. Additional diagnostic studies that may be helpful include radiographic studies, CT scan, MRI scan, electromyography of the larynx, and, in older children, stroboscopy. 4. In using a flexible direct laryngoscope be careful not to interpret all motions of the vocal cords or arytenoids as evidence to preclude the diagnosis of vocal cord paralysis or paresis and be careful not to mistake the anterior intraluminal portion of a normal cricoid for an "anterior glottic web." 5. Tracheotomy is often required in order to assure adequate airway during infancy for children with BAVP. However, with the advent of sophisticated cardiorespiratory monitoring equipment and methods for monitoring blood oxygen and carbon dioxide levels, tracheotomy can be delayed until attempts have been made to improve the adequacy of the airway with neurosurgical intervention or other procedures.(ABSTRACT TRUNCATED AT 400 WORDS)

Child↗

Post-operative sore throat: effect of lignocaine jelly and spray with endotracheal intubation.

The effects of laryngeal lignocaine spray and/or lignocaine jelly as lubricants were studied on the incidence of sore throat, hoarseness, or tracheal irritability as evidenced by either a tendency to cough or frank coughing after intubation with a Sensiv tube (Searle Medical Products). Pressure in the medium-volume, low-pressure cuff was controlled and kept below 2.5 kPa (25 cmH2O) during anaesthesia. The side-effects of 94 surgical patients were recorded in a double-blind manner in the recovery room and on the first post-operative day. The lowest number of patients (62%) with side-effects was found in the control group and the greatest (95%) after the combination of lignocaine jelly and spray (P less than 0.05). The number of side-effects after lignocaine jelly alone was 85% and after the spray alone 68%. Women were more prone to have side-effects, especially sore throat and hoarseness, than men (P less than 0.05). Induced hypotension did not increase the incidence of side-effects. Endotracheal cuff pressures were also studied in 114 patients during N2O-O2 anaesthesia and in 54 patients after the replacement of N2O with nitrogen. The minimum occluding pressure required was 1 kPa (10 cmH2O). In 42% of the patients receiving N2O a limiting value of 2.5 kPa (25 cmH2O) was reached during anesthesia in a mean time of 74 min (range 25-180 min). After the replacement of N2O with nitrogen, the cuff pressure decreased from 1.8 kPa (18 cmH2O) to 0.7 kPa (7 cmH2O) over 40 min. It is concluded that lignocaine jelly with the use of a spray significantly increases post-operative side-effects.

Administration, Topical↗

[Huge jugular foramen neurinoma extending to the pharyngeal region: a case report].

The authors report a rare case of a huge jugular foramen neurinoma extending to pharyngeal region in a 64 year-old woman who visited an otolaryngologist with complaints of dysphagia, hoarseness and headache in 1984. At that time a submucosal lump was noted in her left pharyngeal region. Biopsy of the tumor proved it to be neurinoma. A CT scan disclosed a dumbell shaped jugular foramen neurinoma and noncommunicating hydrocephalus. Because her symptoms slowly progressed and cerebellar signs as well as signs of increased intracranial pressure was noted, she was referred to our hospital in 1986. She had shown typical sings of left Vernet syndrome, VIIth, VIIIth cranial nerve impairment as well as cerebellar, long tract and increased ICP signs. A subtotal removal was performed from the extracranial and posterior fossa in one stage. The tumor seemed to originate from the Xth cranial nerve. The histological diagnosis of neurinoma was confirmed. Postoperatively, although her dysphagia increased for several weeks, a tracheostomy was not necessary. She was discharged 2 months later and returned to her usual occupation as a house wife. Slight dysphagia and hoarseness were her only chronic symptoms. So far, 88 cases of jugular foramen neurinoma have been reported, 15 of them including our case from Japan. Our case is probably the largest of these reported tumors. Tumors in jugular foramen often masquerade as an acoustic neurinoma or other tumor. However, recently this can usually be diagnosed preoperatively with a careful neurological examination and by means of neuroradiological investigations such as jugular venography, CT scan and MRI.

Cerebral Angiography↗

Voice disorders in the elderly.

A review of the literature clearly shows that connective tissue degeneration in the larynx, particularly of elastic and collagen fibers, is more prevalent in males than in females. Reinke's edema or polypoidal degeneration of vocal cords may or may not be more common in females. Whether or not the above statements are true, tissue atrophy causes a problem in males because the voice becomes higher pitched, weak or reedy, less masculine, whereas polypoidal change in the older female larynx results in a lower pitch, husky voice that would be acceptable in a male but makes the female voice more male-like and undesirable. Functional misuses of laryngeal muscles come into play when patients try to compensate for these changes. The etiology of dysphonia in the elderly gets even more confusing when psychological factors such as loneliness and depression add their effects to laryngeal muscle misuse. Laryngeal cancer is still probably the most common cause of hoarseness in older persons. Unfortunately the biopsy to rule out cancer in a person who is hoarse from degenerative or functional causes will often greatly worsen the dysphonia and render voice therapy less effective.

Aged↗

Cardiovocal syndrome in infancy.

Chronic hoarseness is a rare presenting or accompanying sign of congestive heart failure in infancy. Two infants had a paralyzed left vocal cord, secondary to traction on their left recurrent laryngeal nerve. Both had significant underlying congenital heart disease (type 2 aortopulmonary window, ventricular septal defect, patent ductus arteriosus; large ventricular septal defect). After surgical repair of their congenital heart defect, both infants had normal vocal cord function. Direct laryngoscopy is necessary to diagnose the cause of persistent hoarseness in infancy. If a paralyzed left vocal cord is found, a comprehensive cardiovascular diagnostic work-up is warranted.

Angiocardiography↗

Laryngeal complications in a patient with inactive systemic lupus erythematosus.

Laryngeal complications in systemic lupus erythematosus (SLE) are rarely described. They range from hoarseness to life-threatening respiratory distress. To our knowledge, previous reports describe laryngeal involvement with SLE occurring only during periods of active disease. We saw a patient with inactive SLE in whom hoarseness and exertional dyspnea developed as a result of arytenoiditis and vocal cord paresis during steroid tapering. The condition responded dramatically to readjustment of her steroid dosage. Involvement of the larynx with SLE is a potentially life-threatening complication and may occur in patients with either active or inactive disease. It is an indication for close observation and steroid therapy in patients with SLE.

Airway Obstruction↗

The prevalence of tuberculous laryngitis in pulmonary tuberculosis in Tanzanians.

To assess the prevalence of tuberculous laryngitis in Tanzanians a study was made of 341 patients admitted for pulmonary tuberculosis to the chest unit of Muhimbili Medical Centre, University Hospital of Dar-es-Salaam. Laryngeal involvement, assessed by indirect laryngoscopy, was present in 27% of previously untreated patients. Hoarseness, which invariably accompanied lesions in the glottic region, was found in 53%. If a patient with pulmonary tuberculosis complains of hoarseness then concomitant tuberculous laryngitis is the first differential diagnostic consideration. Tuberculous laryngitis is usually related to advanced stages of pulmonary tuberculosis. Indirect laryngoscopy is an essential part of routine investigations for pulmonary tuberculosis.

Adolescent↗

[Therapy of isolated acute laryngitis with fusafungin. A phase IV study].

Four-hundred and eighty-four patients with acute laryngitis were examined before and after treatment with fusafungin under phase IV conditions. After one week of therapy such complaints as hoarseness, sore throat and difficulties in swallowing were improved in about 90% of the patients, while the leading complaint of "hoarseness" had disappeared in 60%. Inflammatory findings, documented by a physician, showed comparable results. An analysis of subgroups (ineffectively treated patients and patients with long-lasting symptoms and complaints) shows that fusafungin was effective therapeutically for treating for acute laryngitis. This study also shows that the generally accepted high rate of spontaneous recovery does not exist.

Acute Disease↗

[Clinical aspects in oral, pharyngeal and laryngeal carcinoma--results of an overall Swiss cohort study].

Squamous cell carcinomas of the oral cavity, pharynx and larynx cause early symptoms and are fairly accessible. Nevertheless, in more than half of cases they are diagnosed at an advanced stage. This paper identifies patients at high risk of developing one of these carcinomas, describes the typical clinical findings and shows how, with a high index of suspicion and a proper examination technique, diagnosis at an earlier stage could be achieved. The study is based on the data of 636 patients with squamous cell carcinoma of the oral cavity (174 patients), oropharynx (177), hypopharynx (97), and larynx (188). 87% of the patients were male, and 90% were aged between 40 and 80 years. 85% of the patients smoked and drank alcohol regularly. The key symptoms were pain, dysphagia, hoarseness, a painless neck mass, or a visible ulcerating lesion. On average a 4-month period elapsed from the onset of symptoms to the histological diagnosis. The diagnostic delay was significantly shorter in patients with pain or visible organic changes (ulcerating lesions, neck masses) than in patients with functional symptoms (dysphagia and hoarseness).

Adult↗

Indirect laryngoscopic evaluation of vocal cord function in patients undergoing transhiatal esophagectomy.

Vocal cord function was assessed by indirect laryngoscopy before and after transhiatal esophagectomy for carcinoma of the middle and lower esophagus. Unilateral vocal cord palsy was found in 16 (34 percent) of 47 patients, but three of these patients were not hoarse and coughed normally. Four patients were hoarse postoperatively but had normal vocal cord function on indirect laryngoscopic examination. All vocal cord palsies but one were on the left side and the left side of the neck was used for esophageal mobilization with the only exception of the one patient who had a right cord palsy after a right-sided neck dissection. Patients with impaired vocal cord function did not have a significantly increased stay in the intensive therapy unit or in the hospital, nor was there an increase in pulmonary complications. Vocal cord palsy is a recognized complication of transhiatal esophagectomy, and although it remains unresolved whether or not the recurrent laryngeal nerve damage occurs at the level of the aortic arch or in the neck, the present evidence suggests the latter site. The clinical impression of postoperative vocal cord palsy is often incorrect.

Adenocarcinoma↗

[A case of Werner syndrome associated with spastic paraparesis and peripheral neuropathy].

A 33-year-old woman, whose parents were consanguineously married, was admitted to our hospital because of progressive difficulty in walking and hoarseness in speech. She had been in good health as a child, at age 16 ulcerations appeared on the skin of feet, and have been recurrent. AT age 22, bilateral cataracts were removed. From the age of 26 years, the deformities in the toes and fingers worsened and she had difficulty in walking. At age 32, hoarseness was noticed. The patient was a diminutive woman and the skin was dry, thin and hyperpigmented. The neurological examination revealed mental retardation, spastic paraparesis and polyneuropathy. Electrophysiological studies revealed a slowing of central and peripheral nerve conduction. Sural nerve biopsy revealed a significantly higher incidence of de- and remyelination and a loss of myelinated fibers. These data suggest that the central and peripheral nervous systems are affected in Werner syndrome.

Adult↗

Chondrosarcoma of the larynx: CT features.

PURPOSE: To define the typical CT features of chondrosarcoma of the larynx. PATIENTS AND METHODS: Results of CT studies, performed on 10 patients with pathologically proved chondrosarcoma of the larynx, were retrospectively reviewed and correlated with clinical presentation. RESULTS: In all patients, the mass was detected on CT. The most frequent site of origin of the tumor was the cricoid cartilage (nine cases) followed by the thyroid cartilage (one case). Coarse or stippled calcification within the tumor was the most helpful radiologic finding and was seen in every case. In eight patients, the tumor had both an endolaryngeal and an extralaryngeal growth pattern, whereas in two patients the tumor was entirely endolaryngeal. Hoarseness, dyspnea, and dysphagia were the most common symptoms. In all patients presenting with dyspnea, the tumor exhibited endolaryngeal components. In patients presenting with hoarseness, three tumors had endolaryngeal and extralaryngeal components and two tumors were entirely endolaryngeal. CONCLUSION: Cross-sectional imaging afforded excellent evaluation of the airway as well as the extralaryngeal component of the tumor.

Aged↗

Giant left ventricular aneurysm.

A 65 year old lady with known inferior left ventricular aneurysm presented with acute dysphagia and hoarseness. A barium swallow examination confirmed extrinsic compression of the oesophagus. Successful aneurysmectomy resulted in complete relief of the dysphagia and hoarseness. The clinical and radiological features are reviewed.

Aged↗

Chlamydia pneumoniae as a new source of infectious outbreaks in nursing homes.

OBJECTIVE: To determine the extent and severity of illness and mode of transmission of Chlamydia pneumoniae infection in 3 nursing home outbreaks. DESIGN AND SETTING: Retrospective cohort study in 3 nursing homes in Ontario from September to November 1994. SUBJECTS: A total of 549 residents and 65 staff members. MAIN OUTCOME MEASURES: Morbidity and mortality were determined by a review of disease surveillance forms, residents' charts, and a self-administered questionnaire to staff. Single and paired serum samples for C pneumoniae serological testing and nasopharyngeal swabs for C pneumoniae culture were collected, and direct fluorescent antibody assays were performed to confirm C pneumoniae infection. RESULTS: The attack rates for confirmed and suspected cases combined were 68%, 46%, and 44% among residents in nursing homes A, B, and C, respectively, and 34% among nursing home C staff. A total of 16 cases of pneumonia confirmed by chest x-ray and 6 deaths were identified. The spectrum of illness among nursing home C residents included a new cough in 58 (100%), fever in 37 (64%), sore throat in 14 (24%), and hoarseness in 8 (14%). Staff members at nursing home C were more likely to report hoarseness (P<.001) and sore throat (P<.001). Residents who smoked had onset of illness earlier than nonsmokers (P=.007), which perhaps is related to airborne transmission in a designated smoking room. CONCLUSIONS: Chlamydia pneumoniae caused serious morbidity and mortality among residents and morbidity among staff; C pneumoniae is an important cause of respiratory disease outbreaks in nursing homes, and diagnostic tests must be readily available for early recognition of C pneumoniae infections.

Aged↗

Recessive epidermolysis bullosa simplex associated with plectin mutations: infantile respiratory complications in two unrelated cases.

Plectin is a 500 kDa protein involved in cytoskeleton-plasma membrane attachment with a wide tissue distribution including cutaneous and airway epithelia, muscle and neuronal tissue. Recently, mutations in the gene encoding plectin (PLEC1) have been implicated in the pathogenesis of an autosomal recessive variant of epidermolysis bullosa simplex in which cutaneous blistering starting in the neonatal period is associated with muscular dystrophy in later life. In this study, we report two unrelated patients, both of consanguineous parentage, who presented with cutaneous blistering and a hoarse cry from birth. Both experienced inspiratory stridor and respiratory distress, necessitating emergency tracheostomy in one case. Immunoreactivity to monoclonal antibodies against plectin was absent or markedly reduced in skin biopsies from both patients. Electron microscopy revealed a low intraepidermal plane of cleavage and hypoplastic hemidesmosomes with a reduced association with keratin intermediate filaments. Direct sequencing of PLEC1 in each case demonstrated two novel homozygous frameshift deletion mutations, 5069del19 and 5905del2, which both create downstream premature termination codons. Although currently neither patient has symptoms of muscle disease, the identification of mutations in PLEC1 may be predictive for the future development of muscular dystrophy. Recessive epidermolysis bullosa simplex resulting from abnormalities in plectin should be considered in the differential diagnosis blistering, hoarseness and stridor in infancy.

Child, Preschool↗

[Phonosurgery of chronic vocal cord edema].

INTRODUCTION: Chronic (Reinke's) oedema of the vocal folds is a frequent and declicate objective of phonosurgery. It is characterized by a marked bilateral subepithelial oedema, which develops by degrees, as a non-specific reaction of the vocal folds to various irritative noxious agents (especially smoking), in patients with some predisposition. It is found, by the light and electron microscopes and immunohistochemistry, that oedema is characterized by subepithelial fissure-like spaces, which accumulate a protein-rich fluid, and develops like neobursae. Therefore, mechanical factors and functional influences may also contribute to the development of Reinke's oedema. The voice is low pitched and with various degrees of hoarseness. Reinke's oedema alters the mechanical properties of the cover, which becomes very pliable and with reduced stiffness, incapacitating the vocal fold for production of high tones. Hoarseness is induced in subject with associated laryngitis, or disbalance in mechanical properties of the vocal folds. Hyperkinetic pattern of voice production can often be seen in patients with Reinke's oedema, which is a compensatory results of reduced functional capability of the vocal folds. Stroboscopy reveals a prolonged closed phase of the vibratory cycles and strikingly marked mucosal waves. MATERIAL AND METHOD: A series of 371 patients with Reinke's oedema was operated by direct microlaryngoscopy, under the general anaesthesia. The "excessive" mucosa was removed by bimanual micro-procedure, while the care was not taken to severe layers deeper than a superficial part of the intermediate layer of the vocal fold (Reinke's space). In this procedure we used the micro-forceps and scissors, to detach oedema parallel to the free edge of the vocal fold, at its upper and lower demarcation lines, beginning from the posterior part of oedema. Another 27 patients were operated by indirect procedures. Microstroboscopy (IMS) was used in subjects, while videostroboscopy (IVS) was carried out in another 18 patients. These procedures were used when general anaesthesia was contraindicated, and in patients with Reinke's oedema of the first degree (initial oedema). In these patients only a mucosal strip was removed from the upper surface of the vocal fold, apart from the free edge. Oedema was removed bilaterally, while the formation of the postoperative web was prevented by regular examinations of the patient. In several cases of adherence between the two folds in their anterior commissure, the problem was solved indirectly by the use of a curved forceps and under the topical anaesthesia. Postoperative voice rest and administration of steroids were mandatory. Surgical and functional results were followed-up by stroboscopy over the period of at least three years after surgery. The majority of patients were additionally treated by the voice therapy, while the decision about its use was made three weeks after surgery. RESULTS: Functional results of our therapeutic strategy were satisfactory in our series of 398 subjects with Reinke's oedema. In comparison with other benign lesions of the vocal folds, it was more time-consuming and required a more frequent use of the postoperative voice therapy (Table 1). Thus, we have not encountered recurrences. DISCUSSION: During the last 10 years we operated on 1550 patients with various benign lesions of the vocal folds, including 398 subjects with Reinke's oedema (25.7%). Excision of the "excessive" mucosa may appear today as a procedure which is too radical if compared with many techniques which have been offered during the last decades: conservative excision and suction, squeezing technique, laser. Nevertheless, the histological structure of Reinke's oedema, with subepithelial fissure-like spaces indicated that the latter procedures can hardly be expected to prevent recurrences. It was found that the use of laser was not favourable in this area for its deteriorative local effect.

Chronic Disease↗