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A 21-year-old man with 3-month history of hoarseness and dyspnea was suspected to have Wegener's granulomatosis with initially appearing in the trachea. In spite of unspecific biopsies, the clinicopathological picture and the high titles of anticytoplasmic autoantibodies (ACPA) confirmed the diagnosis. The early treatment with cyclophosphamide impeded the evolution to graver systemic forms of the disease.

A 63 year old woman presented with a 3 year history of exertional dyspnoea. Spirometry suggested extrathoracic airway obstruction (decreased inspiratory flow and saw-tooth pattern of flow-volume curves), and bronchoscopy revealed structural lesions and a trembling motion in the arytenoid region, causing upper airway obstruction on forced respiratory efforts. As there were no abnormal findings other than the lesions, the exertional dyspnoea was probably caused by impaired movement of the arytenoid region.

The stoma following laryngectomy may be placed either within the incision or separately. To determine if this variation had a bearing on its ultimate size, measurements of the stoma size were carried out in 61 patients who underwent laryngectomy by one of these methods. It was found that the stoma was nearly twice the size in patients in whom the stoma had been sited within the main wound and that there was a minimal need for the use of a tube or stoma button post-operatively in these patients.

To better define the clinical course of congenital subglottic stenosis, the authors reviewed a cohort of 27 children managed with long-term tracheotomy and interval bronchoscopy. Rates of spontaneous resolution were 19% at 6 months, 39% at 12 months, and 69% at 18 months (Kaplan-Meier survival analysis). Children older than 4 months of age at tracheotomy tended to have more rapid resolution of their stenoses (P = .08). Overall, the median time to decannulation was 15.8 months (95% confidence interval, 13.9 to 17.6 months). One death was possibly tracheotomy-related, and 12 (44%) of the children experienced complications or sequelae resulting in new or extended hospitalization. Considering the prolonged cannulation necessary for spontaneous resolution of congenital subglottic stenosis, the role of long-term tracheotomy as the sole form of management may be limited. Once the need for intervention is apparent, the authors recommend early consideration of cricoid split or laryngotracheoplasty as alternatives to long-term tracheotomy.

We prospectively studied and compared clinical features, treatment, course of illness, and long-term morbidity and mortality rates for Wegener granulomatosis in 23 childhood-onset patients with those of 135 adult-onset patients who were studied concurrently. Treatment was usually provided with glucocorticoids and cyclophosphamide. The mean follow-up period was 8.7 years for childhood-onset and 7.6 years for adult-onset Wegener granulomatosis. Most aspects of Wegener granulomatosis were similar in childhood-onset and adult-onset patients. Permanent morbidity from disease occurred in 86% of both groups. However, some features were significantly different. Wegener granulomatosis in childhood-onset patients was complicated five times more often by subglottic stenosis and twice as often by nasal deformity. Treatment-related permanent morbidity occurred in 22% of childhood-onset patients and 45% of adult-onset patients. After similar periods of cyclophosphamide therapy and follow-up, cyclophosphamide-related malignancies were less likely (0% vs 11%) to have developed in childhood-onset patients. Although 89% of patients treated with glucocorticoids and cyclophosphamide had remission, prolonged delay in achieving remission and relapses led in both patient groups to freedom from active disease for approximately 50% of the total patient-years. As a result, morbidity was substantial and has led to comparative studies of alternative therapies.

Five-week-old New Zealand white rabbits were divided into three groups: control, anterior-posterior cricoid split with stent, and cricoid quartersection (anterior-posterior-lateral cricoid split) with stent. The stents were removed 2 weeks after placement. The rabbits were allowed to grow for six months, at which time they were killed and the larynges were studied. Average, minimal, and maximal subglottal cross-sectional areas were calculated for each animal and adjusted for weight. The cricoid quartersection group had significantly greater average, minimal, and maximal subglottal cross-sectional areas compared to controls and significantly greater minimal and maximal cross-sectional areas compared to the anterior-posterior cricoid split group. The anterior-posterior cricoid split group and the control group did not significantly differ with respect to any of the three parameters. The results indicate that anterior-posterior cricoid split and cricoid quartersection do not adversely affect subglottic growth. Cricoid quartersectioning was superior to the anterior-posterior cricoid split in its ability to expand subglottic airway dimension.

It recently has been recognized that platelet-activating factor (PAF) may be a mediator of asthma exacerbation. We had the opportunity to analyze bronchoalveolar lavage fluids from an asthmatic infant, which were characterized by neutrophil infiltration. The patient's lungs were washed on three occasions with saline during asthmatic attacks. PAF was found in each case on the basis of its ability to cause the immediate aggregation of washed rabbit platelets. The PAF detected was equivalent to 1-1.4 pmol of 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine, three quarters of which were recovered in cell-associated form. By contrast, we did not detect PAF in bronchoalveolar exudates from patients with laryngeal stenosis or with respiratory distress syndrome. LysoPAF, the direct precursor as well as initial metabolite of PAF, was also analyzed after being converted to PAF by acetylation. There was a wide variation in the amount of lysoPAF present in individual patients, suggesting that lysoPAF levels cannot be taken as an indicator for the presence of PAF.

In the surgical treatment of the paralyzed larynx, a compromise often needs to be made between an orifice size needed to preserve voice and that needed for adequate inspiratory airflow rates. To assess the negative pressures needed to generate normal airflows across a narrowed vocal cord aperture, we measured pressure and flow changes across cadaveric larynges while altering aperture size. Best-fit quadratic equations for each aperture area selected were derived and showed that if the aperture were 0.5 cm2 or less, the resistance to normal breathing would be significantly increased. Aperture sizes of 0.67 cm2 or greater are not associated with such an increase in resistance.

Laryngotracheal reconstruction with expansion surgery is a reliable method for treatment of laryngotracheal stenosis in children. It allows for decannulation of the patient and restoration of vocal communication. Previous information regarding vocal quality in these patients has been scant. Sixteen children who underwent successful laryngotracheal reconstruction were evaluated using a standardized voice assessment during each of the following: phoneme prolongation, counting, storytelling, and spontaneous conversation. Interjudge ratings were obtained to establish reliability. The location and severity of the laryngotracheal stenosis and the method of treatment were reviewed. Twelve children have a functional voice compared to six preoperatively. Overall, vocal parameters suggested a particular pattern consisting of a lower than optimal pitch and a restricted pitch range. Laryngotracheal reconstruction does make oral communication possible for children who would otherwise have persistent tracheotomy. However, vocal quality appears to be disturbed in most of the patients.

Altogether 19 patients with chronic laryngo- and tracheostenosis of different etiology were investigated. All the patients were broken into 3 groups (1--less than 1 year, 2-1-3 years, 3--over 3 years) with regard to the time of canulla bearing. The patients were examined with 99mTc-human serum albumin particles. Major disorders in the capillary blood supply of both lungs were detected. These changes were focal-diffuse. Abnormalities were mainly observed in the apical area of both lungs and in the mediastinal zone. Redistribution of the pulmonary blood supply in the direction of the upper parts of the lungs was revealed. Lung abnormalities increased depending on the time of canulla bearing.

Non-invasive procedures are proposed to aid the diagnosis of childhood laryngotracheal pathology and to monitor the course of such disease. The procedures capitalize on the one-to-one relationship which exists between the acoustic phenomena (stridors) associated with respiration and the configuration of the respiratory tract. Careful analysis of these acoustic patterns can thus assist in identifying and localizing constrictions, in diagnosis, and in monitoring disease severity. Based on the acoustical analysis of the stridor generated by children with congenital stridor, subglottal laryngitis, and trachea stenosis, the present paper demonstrates that a close relationship exists between the specific pathology and the spectrum of the associated respiratory stridor.

Laryngotracheal reconstruction in children with autogenous rib cartilage has proved successful in relieving laryngotracheal stenosis. To date, no objective measurements have been available to assess the upper airway's functional ability in these children after decannulation. A pilot study analyzing pulmonary function in 18 children successfully decannulated after laryngotracheal reconstruction was performed. The children ranged in age from 24 months to 12 years and were several months postdecannulation at the time of testing. The results of this pilot study offer an objective assessment of functional capacity after laryngotracheal reconstruction in older children and serve as a basis for objective follow-up. In younger children, pulmonary function values were difficult to obtain and proved to be of no significant value to the overall assessment of functional capacity of the airway.

There are several studies showing that patients with idiopathic obstructive sleep apnea (OSA) have a narrow and collapsible pharynx that may predispose them to repeated upper airway occlusions during sleep. We hypothesized that this structural abnormality may also extend to the glottic and tracheal region. Consequently, we measured pharyngeal (Aph), glottic (Agl), cervical tracheal (Atr1), midtracheal (Atr2), and distal (Atr3) tracheal areas during tidal breathing in 66 patients with OSA (16 nonobese and 50 obese) and 8 nonapneic controls. We found that Aph, Agl, and Atr1, but not Atr2 or Atr3, were significantly smaller in the OSA group than in the control group. Obese patients with OSA had the smallest upper airway area, although the nonapneic controls had the largest areas. Multiple linear regression analysis revealed that the pharyngeal area, cervical tracheal area, and body mass index were all independent determinants of the apnea-hypopnea index, accounting for 31% of the variability in apnea-hypopnea index. Aph, Agl, and Atr showed significant correlation with the body mass index. We conclude that sleep-disordered breathing is associated with diffuse upper airway narrowing and that obesity contributes to this narrowing. Furthermore, we speculate that a common pathophysiological mechanism may be responsible for this reduction in upper airway area extending from the pharynx to the proximal trachea.

Recently the association of hypersomnia and respiratory insufficiency without lesion in the respiratory organ has attracted attention of many investigators. Obese patients with such a condition have been called the Pickwickian syndrome. In this report, two non-obese patients with a similar condition were presented, one with micrognathia and frequent apneic episodes during sleep, and the other with laryngeal stenosis due to paralysis of the bilateral laryngeal nerves and chronic laryngitis. Tracheostomy had a prompt and long-lasting therapeutic effect to make their sleep stable and also to relieve their excessive daytime sleepiness. These findings suggest that the obstruction or stenosis of the upper airway during sleep disturbed their nocturnal sleep, and that their excessive daytime sleepiness was a phenomenon compensating for their disturbed nocturnal sleep.

The airway resistance of 32 patients with tracheostomies due to tracheal or laryngeal stenoses was measured using the autoflow and the oscillation method. The resistance to flow between the mouth and tracheostomy was determined with the autoflow method. While measuring with the oscillation method the tracheostoma was blocked with stomahaesive . The comparison of both methods resulted in a good correlation (r2 = 0.83). Hence, the oscillation method can be used to determine airway resistance in patients with stenosis of the larynx or trachea, irrespective of whether or not they have a tracheostome .

The techniques and observations of earlier authors on laryngeal electromyography are briefly reviewed. Our personal experience with the use of a percutaneous method in a series of 27 patients is described and discussed. The percutaneous technique is a simple, safe method and permits the examination to be performed on a routine out-patient basis. The presence of denervation, the degree of paralysis, evidence of recovery, can be readily obtained and this permits a more accurate assessment and prognosis to be made.

In young rabbits (4-week-old) the growth of the subglottis was studied after an endolaryngeal soft tissue trauma. The injury induced the formation of a subglottic stenosis. In contrast to earlier observations on endolaryngeal trauma extending into the cricoid cartilage, the cricoid developed normally to the adult size and shape at 24 weeks. Similar to the earlier findings, the subepithelial layer showed considerable thickening as the result of formation of scar tissue, ectopic cartilage and fatty tissue with interruption of the elastic tunica (= conus elasticus). It was concluded that after endolaryngeal trauma in rabbits two types of subglottic stenosis can develop, determined by the depth of the injury.

When considering management of subglottic stenosis, all conservative efforts to establish a satisfactory airway must be considered prior to surgical reconstruction. However, the approach which allows for the earliest possible decannulation is the one which is sought because of high morbidity and mortality of tracheostomy in the pediatric population.

We used a body plethysmograph to determine airway resistances in 485 cases of laryngeal and tracheal stenoses. 143 cases who had resistances exceeding 60 mm H2O/l.a.sec underwent surgery. A vocal cord was laterally fixed in 49 patients suffering from bilateral recurrent paralysis. Optimal results were obtained at a postoperative resistance level of 30 mm H2O/l.a.sec (standard value: 14.77 +/- 6.53 - n - 387). The patients could carry out work of medium intensity and had a steady voice. We performed tracheal interventions in 94 cases of tracheal stenoses. A mean postoperative resistance of 29.9 mm HWO/l.a.sec, with a tracheal diameter of 7-8 mm was attained. In practice, only a few patients found the remaining obstruction a hindrance, during work of maximal intensity. No recurrences were observed after treatment. Airway resistance exceeding 150 mm H2O/l.a.sec were found in 13 new admissions and 73 times in those undergoing therapy. In these cases asphyxiation threatens. These patients must be tracheotomized or intubated immediatly.

Bronchopulmonary dysplasia has now become the most common chronic lung disease of early childhood, and is a sequel of neonatal intensive care. The condition is characterized by interstitial fibrosis, agenesis of pulmonary alveoli, and distension of the remaining lung tissue. The resultant stiffness of the lung leads to noisy, labored respiration with retraction of the soft tissues of the thorax during inspiration which may easily be mistaken for the signs of subglottic stenosis. Two cases which illustrate the problem are presented. The mechanism underlying the clinical picture is discussed.

We used a body-plethysmograph to determine air-way resistances in 485 cases of laryngeal and tracheal stenoses. We decided in 143 cases to intervene after observing resistance exceeding 60 mm H2O/l and sec. A vocal chord was lateral fixated in 49 patients suffering bilateral recurrent paralysis. Optimal results were obtained at a postoperative resistance level of 30 mm H2O/l and sec (standard value: 14.77+/-6.53--n = 387). The patients could carry out work of medium intensity and had a steady voice. We performed tracheal interventions in 94 cases of tracheal stenoses. A mean, post-operative resistance of 29.9 mm H2O/l and sec, with a tracheal diameter of 7--8 mm was attained. In practice, only a few patients found the remaining obstruction a hindrance during work of maximal intensity. No recurrences were observed after treatment. Airway resistances exceeding 150 mm H2O/l and sec were found in 13 new admissions and 73 times in those undergoing therapy. In these cases asphyxiation threatens. These patients have to be tracheotomized or intubated immediately.

A study was made of blood hydrocortisone content for a period of 24 hours in the patients with congenital diseases of the heart before and after the operation, in patients with acute respiratory disease, severe pneumonia, and also with acute laryngeal stenosis of influenzal etiology. It was revealed that under pathological conditions the adrenal cortex took part in the compensatory reactions of the organism at any time of the day. As a result of this hydrocortisone concentration is steadily maintained at a definite level in the course of the 24 hours. These changes depended on the intensity of the stressor and the severity of metabolic disturbances in the organism.

The authors report the cases of ten children treated for sub-glottic laryngeal stenosis, in the Department of Pr. Narcy in Hospital Robert-Debre. Medical treatment of the laryngeal stenosis had failed in these cases. Treatment, most often surgical, of the gastro-oesophageal reflux present in these ten cases enabled these children to be cured. A review of the literature stresses the role and responsibility of gastro-oesophageal reflux in laryngeal pathology. Based on their experience, the authors suggest: systematic investigation for gastro-oesophageal reflux during management of laryngeal stenosis, especially when laryngeal inflammation is encountered; the adoption of an interventionist attitude vis-a-vis gastro-oesophageal reflux which would seem to have an important pathogenic role in certain laryngeal stenoses.

Stenosis of the posterior laryngeal commissure with arytenoid fixation following intubation trauma or laryngeal fracture poses a treatment dilemma. Over the past 2 1/2 years we have managed four patients with this problem by mobilization of the fixated cricoarytenoid joint, arytenoid-pharyngeal mucosal advancement flap, brief splinting of the arytenoids in the fully abducted position, and early speech therapy. As of this time, three of the four patients have been both decannulated and have achieved adequate to near normal voices. Most significantly, the previously fixated arytenoids are now mobile, confirming the efficacy of the principles presented herein.

The single-frequency forced oscillation technique was used to estimate the total respiratory impedance, resistance, and reactance at 2, 4, and 12 Hz of children who had cricoid stenosis (CS; n = 11), provoked bronchoconstriction (PB; n = 6), or cystic fibrosis (CF; n = 13). The selection criteria for patients who had CS and CF were abnormal forced expiratory volume in 1 sec (FEV!) and/or maximal expiratory flow at 50% vital capacity (Vmax 50%). Sixteen of the 17 patients who had CS or PB were found to have resistance (Rrs) values outside the normal range at 4 Hz; at 2 Hz, 14 were found to have abnormal values and, at 12 Hz, only 9. The reactance in the CS and PB groups was somewhat less discriminative than Rrs at all frequencies. Of the 13 patients who had CF, only 4 had abnormal Rrs values; in this clinical condition, the reactance at 2 Hz was the most discriminative variable, being abnormal in 6 of the 13 patients. Irrespective of the clinical group, the Rrs was, in absolute terms, highest at 2 Hz, and it decreased with increasing frequency. This pattern of negative frequency dependence was, however, not obviously specific for any of the tested clinical conditions and offered no information in addition to a single low-frequency determination.