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Laryngeal papillomatosis with airway obstruction in an infant.

Laryngeal papillomatosis in infants and children is a benign condition, but the location and a marked tendency for recurrence makes the disease both dangerous and troublesome. This case report deals with a little girl who had suffered hoarseness and wheezing since she was born. The diagnosis of laryngeal papillomatosis was made when she was 17 months old. By that time, the tumour had reached a size that necessitated a tracheotomy to secure the airway. The symptoms and differential diagnoses are discussed, and it is stressed that chronic hoarseness and wheezing sounds in infants and children should make a doctor suspect laryngeal papillomatosis. Laser treatment and anaesthetic management of small children with a compromised airway are discussed. As tracheal intubations and tracheotomy increase the risk of the disease spreading to the trachea and bronchi, an example is given of treating laryngeal papillomatosis with potassium titanyl phosphate (KTP) laser, using a laryngeal mask as an airway to avoid tracheal intubation. Whether this procedure can reduce the need to perform a tracheotomy in some of these small patients remains to be seen.

Airway Obstruction↗

Bone marrow transplantation for infantile ceramidase deficiency (Farber disease).

Infantile ceramidase deficiency (Farber disease) is an uncommon, progressive lysosomal storage disease characterized by multiple ceramide-containing nodules (lipogranulomata) in the subcutaneous tissue and upper aerodigestive tract, painful periarticular swelling, psychomotor retardation, and varying degrees of ocular, pulmonary or hepatic involvement. Management of Farber disease has been limited to symptomatic supportive care, and few affected infants survive beyond 5 years of age. We performed an allogeneic bone marrow transplant (BMT) from an HLA-identical heterozygous sister in a 9.5-month-old female with minimally symptomatic Farber disease who received a pre-transplant regimen of busulfan and cyclophosphamide. Ceramidase activity in peripheral blood leukocytes increased from 6% before transplant to 44% (donor heterozygote level) by 6 weeks after BMT. By 2 months after transplant, the patient's subcutaneous lipogranulomata, pain on joint motion, and hoarseness had resolved. Despite modest gains in cognitive and language development, hypotonia and delayed motor skills persisted. Gradual loss of circulating donor cells with autologous hematopoietic recovery occurred; VNTR analyses showed 50% donor DNA in peripheral blood cells at 8.5 months after BMT and only 1% at 21 months after transplant. Interestingly, leukocyte ceramidase activity consistently remained in the heterozygous range despite attrition of donor cells in peripheral blood. This novel observation indicates ongoing hydrolase production by non-circulating donor cells, possibly in the mononuclear phagocytic system, and uptake by recipient leukocytes. Although lipogranulomata and hoarseness did not recur, the patient's neurological and neurocognitive status progressively declined. She died 28 months after BMT (age 37.5 months) with pulmonary insufficiency caused by recurrent aspiration pneumonias. Allogeneic BMT improves the peripheral manifestations of infantile ceramidase deficiency, but may not prevent the progressive neurological deterioration, even when carried out in minimally symptomatic patients.

Bone Marrow Transplantation↗

The elastin gene is disrupted in a family with a balanced translocation t(7;16)(q11.23;q13) associated with a variable expression of the Williams-Beuren syndrome.

The Williams-Beuren syndrome (WBS) is a complex developmental disorder with multisystemic manifestations including supravalvular aortic stenosis (SVAS), a so-called elfin face, a hoarse voice, and a specific cognitive phenotype. Most WBS patients have a >1 Mb deletion on one of their chromosomes 7 in q11 but except for elastin, whose haploinsufficiency causes the cardiovascular malformations, it is unknown which genes in the deletion area contribute to the phenotype. We have investigated a family with a cytogenetically balanced translocation t(7;16)(q11.23;q13) in which affected individuals manifested a broad spectrum of clinical phenotypes ranging from a hoarse voice as the only feature to the full WBS phenotype. Molecular cytogenetic and DNA sequence analyses of the translocation breakpoint showed that the cytogenetic rearrangement disrupts the elastin gene locus within intron 5 in the exact same manner in all translocation carriers. The recently described large inversion of the 7q11.23 region was not present in this family. Our data demonstrate that disruption of the elastin gene by a translocation breakpoint may cause classical WBS, atypical WBS, SVAS, or no recognisable phenotype, and provide a clear example for extensive phenotypic variability associated with a position effect in humans.

Adult↗

Pathologic voice type and the acoustic prediction of severity.

We hypothesized that acoustic measures would predict dysphonic severity with differential results for pathological voice types. An instructional program based upon synthesized voice signals was developed to facilitate an awareness of prototypical voice types. Eighty phonatory samples representing normal subjects as well as patients with unilateral vocal fold paralysis, vocal nodules, and functional dysphonia were analyzed acoustically on the basis of four measures: average fundamental frequency (F0), jitter, shimmer, and harmonic/noise ratio (H/N ratio). Following training, 29 listeners classified 62% of the phonatory samples on the basis of breathy, hoarse, rough, and normal. Dysphonic severity of rough voices was predicted more successfully by H/N ratio (r2 = .73) than by shimmer (r2 = .43). Dysphonic severity of breathy voices was predicted only by the combined features of less jitter, more shimmer, and lower H/N ratio (r2 = .74). No combination of acoustic variables was successful in the prediction of the hoarse voice type.

Adolescent↗

Multivariate statistical analysis of flat vowel spectra with a view to characterizing dysphonic voices.

The aim of this article is to show how dysphonic voices can be characterized by means of a multivariate statistical analysis of flat vowel spectra. The spectral contour was obtained by means of a wavelet transform of the logarithmic magnitude spectrum, which was subsequently flattened to remove interspeaker variability related to the excitation and vocal tract filter functions. The results of the statistical analysis of flat spectra were the following. Firstly, principal components analysis produced markers that separated noisy from clean spectra. Secondly, the heuristic search for harmonic peaks or interharmonic dips could be omitted. Thirdly, conventional spectral markers of noise appeared as special instances of the markers that were derived statistically. Fourthly, the levels of visually assigned hoarseness and the first two principal components were significantly correlated. The assignment of different levels of (visual) hoarseness to different vowel timbres could be explained by the variability associated with the spectral contour.

Algorithms↗

Primary non-Hodgkin's lymphoma of the thyroid gland: a population based study.

During a 9-year-period, 50 cases of primary non-Hodgkin's lymphoma (NHL) of the thyroid gland were reported to a population-based lymphoma registry covering western Denmark giving an incidence of 2.06 x 10(-6) cases per year. The male:female ratio was 1:4, and the mean age was 72.8 years for women and 62.8 years for men. On histomorphological reclassification 83% of the cases showed a high grade and 17% a low grade morphology, 98% had a B-phenotype and 2% a T-phenotype. In at least 33 of the cases, primary NHL of the thyroid gland was preceded by Hashimoto's thyroiditis and at least 25 of the patients had a high grade NHL which was transformed from Hashimoto's thyroiditis through a low grade B-cell lymphoma of MALT type. The most frequent presenting symptoms were goitre (100%), hoarseness (57%), stridor/dyspnoea (55%) and dysphagia (45%); thirty-six percent of the patients were hypothyroid at the time of diagnosis. Seventy-six percent of the patients had localized disease (stages 1-2) and 24% had disseminated lymphoma (stages 3-4). Five year survival was 34.5% and 5 year cause-specific survival 46.2%. The following factors were associated with a poor prognosis: stage 3-4 disease, elevated S-urate, presence of hoarseness and age > 66 years. Morphological subtype did not correlate significantly with survival.

Adult↗

Solitary plasmacytosis of the larynx in a patient with non-Hodgkin's lymphoma.

Solitary plasmacytosis of the larynx is a clinically unusual event. It may cause hoarseness or airway obstruction and usually needs surgical excision. Plasmacytosis is distinguished from extramedullary plasmacytoma (EMP) in that the latter primarily involves the head and neck and needs more intensive therapy such as radiotherapy. Many reports have described the histopathology and immunohistochemistry of EMP of the larynx; however, no literature in English has described solitary plasmacytosis of the larynx. We report a 44-year-old female patient with non-Hodgkin's lymphoma and systemic lupus erythromatosus with Sjögren's syndrome. She complained of 1-year period of hoarseness that became exacerbated in the most recent 3 weeks. A wide-base and smooth-surface mass at the left supraglottic area was noted by direct laryngoscope. Computed tomography scan revealed a well-defined radiopaque mass. The histopathology of a specimen excised by carbon dioxide laser-assisted laryngomicrosurgery was interpreted initially as plasmacytoma. Immunohistochemical staining for kappa and lambda light chains demonstrated polyclonal plasma cells, and the definitive diagnosis was plasmacytosis. After 6 months follow-up, the subjective quality of her voice improved. Laryngoscopic examination revealed no evidence of recurrence.

Adult↗

[Perceptive and instrumental examination of voice quality in patients with lip-jaw-palate clefts].

BACKGROUND: In Anglo-American literature, prevalences as high as 41% are reported for voice disorders in cleft lip and palate (CLP) patients. Because of considerable variability in the reported findings and because no prevalence data are available for the German-speaking area, a screening study was conducted. METHOD: 154 patients with CLP were examined. Voice quality was assessed perceptively according to the rbh-system (roughness, breathiness, hoarseness). Suprasegmentals and nasal resonance were also assessed. Using a new computer programme for the apparative analysis of voice quality, data for pitch, intensity and pertubation (jitter and shimmer) were obtained for 4 sustained vowels. RESULTS: Based on perceptual analysis, we found that the prevalence of severe voice disorders in CLP patients was 6.5% which is lower than reported in most other studies. The incidence of voice disorders in CLP patients is numerically only slightly higher than in the normal population. The acoustic measurements confirm the results of perceptual analysis. Since the prevalence of severe voice disorders was low, none of the pertubation quotients differentiated between modal and disordered voices. A weak correlation was found between jitter and the ratings for hoarseness. CONCLUSIONS: 1. While it is appropriate to assume a higher potential risk for vocal dysfunction in CLP patients the actual prevalence in the patients examined is numerically only slightly higher than in normals. 2. For patients with voice disorders, apparative diagnostics of voice quality is a useful and practical adjunct to perceptual analysis which helps profiling individual patients and helps the professional to make decisions for therapy.

Adolescent↗

Contributions of the video-assisted approach to thyroid and parathyroid surgery.

BACKGROUND: The authors report their initial experience with partial and total thyroidectomy, and neck exploration for hyperparathyroidism using the video-assisted approach. PATIENTS AND METHODS: Between June 1999 and March 2001, 114 patients underwent a thyroid (n = 57) or parathyroid (n = 57) operation using a video-assisted cervical approach. Video-assisted neck exploration was conducted in all cases under general anaesthesia leading to a partial or total thyroidectomy, and to a selective adenoma removal in PHPT or to a subtotal parathyroid resection in SHPT. RESULTS: IN THE THYROID GROUP: The mean cranio-caudal and transversal diameter of the resected specimen were respectively 4.9 +/- 0.9 and 2.8 +/- 0.6 cm, and mean total lobar weight was 11.7 +/- 5.8 g. Conversion to conventional surgery was required in 5 patients (8.8%). The mean operative time was 133.9 +/- 26.9 and 86.5 +/- 22.5 minutes for total and partial thyroidectomy respectively. The laryngeal nerve was identified in 96% of cases. The mean length of skin incision was 24.0 +/- 2 mm. There were 3 cases of postoperative hypocalcemia, and 2 cases of postoperative hoarseness. The postoperative hospital stay was less than 24 hours for 72.7% of patients. The pain intensity at day one (VAS) was 2.1 +/- 1.3. IN THE PARATHYROID GROUP: Seven of the 44 patients who underwent PHPT (15.9%) and 4 of the 13 patients who underwent surgery for SHPT (30.8%) were converted to a conventional surgical technique. The mean operative time in PHPT and SHPT was 47.3 +/- 22.3 minutes and 136.8 +/- 18.7 minutes, respectively. Recurrent laryngeal nerve was identified in 53.1% of the patients. The median diameter and weight of the resected parathyroid glands were 1.5 cm (range 0.8-2.7) and 0.9 g (range 0.5-7), respectively. The length of skin incision was 24 +/- 2 mm. All but 2 patients are currently cured. Postoperative complications included hematoma and transient hoarseness each in one patient (1.75%). The median pain intensity at day one (VAS) was 0.5 (range: 0 to 3.6). In the PHPT group, the postoperative hospital stay was less than 24 hours for 56.7% of the patients, and less than 48 hours for 91.9% of them. CONCLUSION: The video-assisted approach for thyroid and parathyroid surgery is feasible, safe and effective in selected cases. Benefits for the patients should be further assessed in future prospective comparative trials.

Follow-Up Studies↗

Progress in laryngeal framework surgery.

This paper reviews progress in laryngeal framework surgery since it was first reported about 25 years ago. The success of this type of surgery requires both a basic knowledge of the physiology of phonation, in order to make decisions about the surgical procedure, and surgical skill, in order to accomplish the intended procedure successfully. The main reason for hoarseness is imperfect closure of the glottis, but the second most important reason for hoarseness, increased stiffness of the vocal fold, cannot be corrected by mere medialization of the vocal fold. Laryngeal framework surgery is different in concept from conventional surgery, which is intended to remove the lesion. Controversial points discussed here regarding type I thyroplasty include: (i) whether the cartilage window should be removed; (ii) materials for fixation of the window; and (iii) comparison of type I thyroplasty with arytenoid adduction. A new surgical treatment for spasmodic dysphonia and its results in three patients are described briefly. Surgery for raising the vocal pitch requires further improvement. In the future, laryngeal framework surgery will have wider application in treatment of dysphonias, such as asthenic voice due to atrophy in professional singers or aging, pitch problems in females and gender identity disorder and spasmodic dysphonia.

Glottis↗

Investigating respiratory responses to metalworking fluid exposure.

In response to worker and union representative concerns, the association between metalworking fluid exposure and respiratory symptoms was investigated in a cross-sectional survey, in a large automotive machining location. A self-administered respiratory symptom-screening questionnaire was sent to 2935 current employees. MWF exposure levels were assigned to respondents on a departmental basis based on average and "peak" area aerosol measurements. MWF exposure, years in the plant, and smoking status were regressed on presence or absence of daily or weekly respiratory symptoms, as well as upper and lower respiratory symptom groupings derived from principal components factor analysis. The response rate was 81 percent. Symptom prevalence was high: 29 percent of subjects reported weekly or daily phlegm; 23 percent, dry cough; 42 percent, runny or plugged nose. Average aerosol concentration in departments with exposure ranged from 0.02 to 0.84 mg/m(3), and peak levels from 0.02 to 2.85 mg/m(3). Average exposures ranging from 0.25 to 0.84 mg/m(3), as compared to exposures in the range of 0.02 to 0.09 mg/m(3), were statistically significantly associated with wheezing, chest tightness, sore throat, and hoarse throat, as well as with the upper respiratory symptom grouping. When peak exposure was included in the regression, it exerted a stronger effect than average exposure level on dry cough, phlegm, wheezing, fever/chills, and hoarse throat, as well as on upper and lower respiratory symptom groupings. These effects were independent of smoking status. Exposure-symptom trends for the average and peak departmental area concentration categories were statistically significant for the upper and lower respiratory symptom groupings and for most individual symptoms. We have observed an association of increasing upper and lower respiratory symptoms with estimated MWF exposure, measured independently, at average departmental aerosol concentrations well below the NIOSH recommended personal exposure level of 0.5 mg/m(3). The results have been used to prioritize exposure reduction efforts in the workplace.

Dose-Response Relationship, Drug↗

A simple subspace approach for speech denoising.

For pathological voices, hoarseness is mainly due to airflow turbulence in the vocal tract and is often referred to as noise. This paper focuses on the enhancement of speech signals that are supposedly degraded by additive white noise. Speech enhancement is performed in the time-domain, by means of a fast and reliable subspace approach. A low-order singular value decomposition (SVD) allows separating the signal and the noise contribution in subsequent data frames of the analysed speech signal. The noise component is thus removed from the signal and the filtered signal is reconstructed along the directions spanned by the eigenvectors associated with the signal subspace eigenvalues only, thus giving enhanced voice quality. This approach was tested on synthetic data, showing higher performance in terms of increased SNR when compared with linear prediction (LP) filtering. It was also successfully applied to real data, from hoarse voices of patients that had undergone partial cordectomisation. The simple structure of the proposed technique allows a real-time implementation, suitable for portable device realisation, as an aid to dysphonic speakers. It could be useful for reducing the effort in speaking, which is closely related to social problems due to awkwardness of voice.

Humans↗

Importance of the intraoperative identification of the external branch of the superior laryngeal nerve during thyroidectomy: electromyographic evaluation.

OBJECTIVE: To establish the frequency and degree of postthyroidectomy injury of the external branch of the superior laryngeal nerve (EBSLN) by means of electromyography and to assess whether intraoperative identification of the nerve and the presence of malignancy influence morbidity. PATIENTS AND METHODS: Prospective, randomized, blind study. Comparison of 100 superior thyroid poles subjected to surgery because of benign and malignant disease, divided in three groups: G1, thyroidectomies, without searching for the EBSLN; G2, thyroidectomies, searching for the EBSLN; G3, Control, lobes, contralateral to the lobectomy, not surgically manipulated. The electromyographic function of the EBSLN was assessed in all cases, classifying the degree of injury and clinically assessing its function. Central tendency measures were used, as well as X (2) and multiple variance analysis. RESULTS: In G1, with 50 dissections of the superior pole, 25 injuries were found in 15 patients (10 bilateral and 5 unilateral). In G2, EBSLN injury was found after 13 explorations of the superior pole in 8 patients (5 bilateral and 3 unilateral). G3 patients did not present EBSLN injury. X (2) among groups and degree of injury revealed that intraoperative identification of the EBSLN decreases its injury. No evidence were found that injury frequency is increased by the presence of thyroid malignancy, extracapsular infiltration, or size of tumor. Clinical manifestations of hoarseness were present in 14% of G1 patients and 8% of G2 patients. DISCUSSION: The rate of EBSLN injury increased when not exploring and/or identifying it expressly. The presence of cancer, extracapsular extension, or size of the thyroid tumor exerted no influence on the frequency of injury; localization of the nerve was the only factor affecting injury. Hoarseness correlated with the degree of injury. CONCLUSION: EBSLN must be localized expressly to decrease the risk of injury.

Adolescent↗

Comparison of the LMA-ProSeal and LMA-Classic in children.

BACKGROUND: The LMA-ProSeal is a new laryngeal mask airway with a rear cuff and drainage tube that allows a higher seal pressure than the LMA-Classic for the same intra-cuff pressure, and it permits drainage of gastric secretions and access to the alimentary tract. The LMA-ProSeal can be used in children but it does not have a rear cuff. This study compared the LMA-ProSeal and the LMA-Classic in children for ease of insertion, airway sealing pressure and fibre-optic visualization. METHODS: Sixty ASA I-II children undergoing herniorrhaphy, orchiopexy or myringotomy were included. The patients were randomly assigned to size 2 LMA-Classic trade mark or size 2 LMA-ProSeal groups for airway management. We assessed success rates at first attempt of insertion, airway sealing pressure, fibre-optic position, success rates of gastric tube placement and postoperative blood staining of the device, tongue-lip-dental trauma and hoarseness. RESULTS: There was no statistical difference between the two groups for the success rates at first attempt of insertion, airway sealing pressure and fibre-optic position. Gastric tube insertion was successful in 90% of cases in the LMA-ProSeal group. The LMA-Classic had a higher rate of postoperative blood staining, but there was no tongue-lip-dental trauma or hoarseness in either group. CONCLUSION: We conclude that ease of insertion and airway sealing pressure are similar between the LMA-ProSeal and the LMA-Classic in children.

Anesthesia, Inhalation↗

Clinical factors and prognosis in non-small cell lung cancer.

We evaluated the relationship of clinical characteristics and survival in 1,635 patients with non-small cell lung cancer (NSCLC) treated in Brazil. The following variables were included: sex, age, smoking, Karnofsky's performance status (PS), weight loss, symptoms at diagnosis (cough, dyspnea, hemoptysis, chest pain, wheezing, and hoarseness), presence of superior vena cava syndrome (SVCS), histologic type, TNM stage, and therapeutic modality (surgery, chemotherapy [CT] and radiotherapy [RT]). Multivariate prognostic models were obtained by Cox regression. Patients unsuitable for surgery or who had recidivant disease were elected to further RT and/or CT, and long-term results in this group were equivalent to those in the group treated only by surgery. A diagnosis of bronchioloalveolar carcinoma, small tumors, absence of hoarseness, treatment by surgery, and RT were independent factors related to good overall survival in stage I and II. Weight loss and clinical signs of SVCS were related to poor prognosis in stage III. PS, diagnosis of adenocarcinoma or undifferentiated carcinoma, absence of weight loss and dyspnea, NO or N1 disease, ability to receive RT, CT, and to perform some palliative surgical procedure were good prognostic factors in stage IV. Clinical features of patients with NSCLC at diagnosis offer additional information to estimate their prognosis.

Adult↗

Case report: candida infection presenting as laryngitis.

Three patients with acute leukemia are described who developed hoarseness as an initial presentation of candida infection. Two of these patients subsequently developed systemic candidiasis. Patients with acute leukemia who develop hoarseness should be investigated for laryngeal candidiasis and considered for systemic antifungal therapy.

Adult↗

Alkalinization of intracuff lidocaine improves endotracheal tube-induced emergence phenomena.

UNLABELLED: We sought to evaluate the effect of filling an endotracheal tube cuff with 40 mg lidocaine alone (Group L) or alkalinized lidocaine (Group LB) in comparison to an Air Control group (Group C) on adverse emergence phenomena in a randomized controlled study (n = 25 in each group). The incidence of sore throat was decreased for Group LB in comparison to Group L during the 24 postextubation hours. The difference between Group L and Group C remained significant in the two postextubation hours only. Plasma lidocaine levels increased when lidocaine was alkalinized (C(max) were 62.5 +/- 34.0 ng/mL and 3.2 +/- 1.0 ng/mL for Groups LB and L, respectively). Cough and restlessness before tracheal extubation were decreased in Group LB compared with Group L and in Group L compared with Group C. Nausea, postoperative vomiting, dysphonia, and hoarseness were increased after extubation in Group C compared with the liquid groups, and a better tolerance was recorded with Group LB compared with Group L. The increase of arterial blood pressure and cardiac frequencies during the extubation period was less in the liquid groups than in the control group and less in Group LB compared with Group L. We concluded that use of intracuff alkalinized lidocaine is an effective adjunct to endotracheal intubation. IMPLICATIONS: Use of 40 mg of alkalinized lidocaine, rather than lidocaine or air, to fill the endotracheal tube cuff reduces the incidence of sore throat in the postoperative period. This approach also decreases hemodynamic effects, restlessness, dysphonia, and hoarseness.

Anesthesia Recovery Period↗

Intracuff pressures do not predict laryngopharyngeal discomfort after use of the laryngeal mask airway.

BACKGROUND: The laryngeal mask airway (LMA) is a large foreign body that exerts pressure on the pharyngeal mucosa, which may lead to throat discomfort. To determine whether intracuff pressures are associated with such discomfort, a randomized, double-blind study was performed to determine the effect of high versus low intracuff pressures. METHODS: Seventy healthy women were randomly allocated to two groups with different LMA intracuff pressures: 30 mmHg (low pressure) or 180 mmHg (high pressure). Pressures were controlled with a microprocessor-controlled monitor. Insertion of the LMA was performed by one investigator and facilitated with propofol and verified fiberoptically. Anesthesia was maintained with enflurane and nitrous oxide. The LMAs were removed while the patients were still asleep. Patients assessed their laryngopharyngeal complaints (sore throat, dysphagia, hoarseness) at 8, 24, and 48 h after operation on a 101-point numerical rating scale. RESULTS: No significant difference was found in the overall incidence of complaints between both groups (low pressure: 50%; high pressure: 42%). On the day of surgery, dysphagia (38%) was more frequent than sore throat (16%) or hoarseness (6%) (P < 0.05) within the high-pressure group. In the low-pressure group, the incidence of these complaints was not significantly different (33%, 20%, and 23%, respectively). On the following day, dysphagia was still present in 20% of the women in both groups, and other symptoms comprised 10% or less of the reported complaints. CONCLUSIONS: Differences in LMA intracuff pressures did not influence either the incidence or severity of laryngopharyngeal complaints.

Adult↗