Lauren D. Holinger

Etiology of Stridor in the Neonate, Infant and Child

Of 219 patients whose primary presenting symptom was stridor, 150 were males, 69 were females. All were under 2½ years of age; more than half were four months of age or younger. Congenital anomalies caused stridor in 191 (87.2%). Congenital laryngeal anomalies accounted for the stridor in 132 (60.3%); there were 35 (16.0%) patients with congenital tracheal anomalies, 11 (5.0%) with congenital bronchial anomalies, 12 (5.5%) with infectious conditions, 12 (5.5%) with internal laryngeal trauma and 15 (6.8%) patients with other conditions. The cause of stridor was undetermined in two patients. Sixteen vascular anomalies were diagnosed and classified according to the part of the tracheobronchial tree which was involved. Twenty-six patients required tracheotomy. Fifty-eight (26.5%) were referred with an erroneous presumptive diagnosis for which they were being treated. The mean length of time from onset of symptoms to determination of the correct diagnosis by endoscopy was four months; it varied from one day to 16 months. The importance of early endoscopy for the diagnosis of conditions causing stridor cannot be overemphasized. The occurrence of more than 1 anomaly in 99 (45.2 %) of the 219 patients demonstrates the importance of complete endoscopic examination of all patients with stridor.

Etiology of bilateral abductor vocal cord paralysis: a review of 389 cases.

The etiology of 389 cases of partial or complete bilateral abductor vocal cord paralysis has been determined and classified. One hundred and forty-nine were infants and children 12 years of age and under; 240 were adults, age 13 and older. In the infants and children the paralyses were congenital in 82 cases, of which 43 were associated with other congenital anomalies, and 39 were without associated anomalies. Fifty-nine cases were considered acquired, most being secondary to underlying congenital anomalies, particularly the associated findings of meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. Eight cases of paralysis in this age group were of undetermined etiology. Of the 240 adult cases of bilateral vocal cord paralysis, 138 followed thyroidectomy. Fifty-two cases were associated with various neurologic disorders, including poliomyelitis, Parkinsons disease, cerebrovascular accident, Guillain-Barré syndrome, multiple sclerosis, neoplasm, and other miscellaneous neurologic conditions. Sixteen cases were due to malignant neoplasms of the neck and mediastinum. The remaining 34 cases constitute a miscellaneous group which includes foreign bodies, bilateral neck dissection, infection, congenital lesions, trauma, and idiopathic paralyses. The characteristic symptoms of bilateral abductor vocal cord paralysis include normal or near normal phonation with inspiratory stridor which may progress to complete respiratory obstruction. These symptoms are due to the stationary but flaccid midline position of the vocal cords which places them in a phonating position, where they both obstruct the airway and produce a fairly clear voice or cry. This paradoxical combination of symptoms was frequently found to be responsible for a failure or delay in diagnosis.

Subglottic stenosis in infants and children.

Of 158 cases of subglottic stenosis 115 were congenital and 43 acquired. Current follow-up has been obtained in 146 (92%) which constitutes the determinate group. Although stridor was the most common presenting symptom of the congenital group, 34% presented with recurrent or prolonged episodes of croup. Tracheotomy was required in 47 of the 107 determinate cases (44%). Further management of the congenital cases was based on the experience that children outgrow this disorder; periodic dilatation may augment the natural process. Of those infants and children tracheotomized, all but five have been decannulated, and there was one death — a mortality rate of 2.1%. Acquired subglottic stenosis proved to be a more difficult management problem. Tracheotomy was necessary in 38 of the 39 determinate cases (97%). Repeated active dilatations for prolonged periods were usually required as well as endoscopic removal of granulation tissue. Of those infants and children tracheotomized, all but eight have been decannulated. There were nine deaths; five were due to unrelated underlying disease; four were attributable to complications of long-term tracheostomy. Thus, in the entire series, 85 infants and children required tracheotomy and five deaths may be attributable to long-term tracheostomy complications — a mortality rate of 5.9%.

Surgical management of severe laryngomalacia

Infants and children with laryngomalacia exhibit varying degrees of upper airway obstruction and dysphagia. Although the disorder is usually self‐limited, the potential exists for symptoms so severe that operative intervention cannot be avoided. Relief of progressive airway compromise traditionally has involved bypassing the obstruction with tracheotomy. Recently, endoscopic surgical management of the most severe cases has been reexamined by the authors and others.

Synchronous Airway Lesions and Outcomes in Infants With Severe Laryngomalacia Requiring Supraglottoplasty

BACKGROUND Beta-Carotene has been reported to produce regressions in patients with oral leukoplakia, a premalignant lesion. However, previous studies have all been of short duration, with clinical response as the end point. OBJECTIVE To evaluate the duration of response and the need for maintenance therapy in subjects who respond to beta-carotene. METHODS In this multicenter, double-blind, placebo-controlled trial, subjects were given beta-carotene, 60 mg/d, for 6 months. At 6 months, responders were randomized to continue beta-carotene or placebo therapy for 12 additional months. RESULTS Fifty-four subjects were enrolled in the trial, with 50 being evaluable. At 6 months, 26 subjects (52%) had a clinical response. Twenty-three of the 26 responders completed the second, randomized phase. Only 2 (18%) of 11 in the beta-carotene arm and 2 (17%) of 12 in the placebo arm relapsed. Baseline biopsies were performed in all patients, with dysplasia being present in 19 (38%) of the 50 evaluable patients. A second biopsy was obtained at 6 months in 23 subjects who consented to this procedure. There was improvement of at least 1 grade of dysplasia in 9 (39%), with no change in 14 (61%). Nutritional intake was assessed using food frequency questionnaires. There was no change in carotenoid intake during the trial. Responders had a lower intake of dietary fiber, fruits, folate, and vitamin E supplements than did nonresponders. Beta-carotene levels were measured in plasma and oral cavity cells. Marked increases occurred during the 6-month induction. However, baseline levels were not restored in subjects taking placebo for 6 to 9 months after discontinuation of beta-carotene therapy. CONCLUSIONS The activity of beta-carotene in patients with oral leukoplakia was confirmed. The responses produced were durable for 1 year.

A randomized trial of adjuvant chemotherapy in head and neck cancer.

Ninety-five patients with squamous cell carcinoma of the head and neck were entered into a randomized study testing a two-week course of induction chemotherapy with methotrexate and leucovorin given prior to regional therapy. In addition, following regional therapy, patients randomized to chemotherapy were to receive similar methotrexate courses every three months for one year. Poor tolerance to this regimen after radiation and surgery led to a change in the chemotherapy following regional therapy to a combination of Adriamycin (Adria Laboratories, Columbus, Ohio) and cisplatin every three weeks for four cycles after the first 35 patients had been entered. Nine cases were ineligible and four lacked any follow-up data, leaving 82 analyzable cases. Using Cox regression analysis, no differences in the percentage of patients achieving disease control, the relapse-free survival, or the overall survival were identified between any treatment group. As has been described in many pilot studies of induction chemotherapy of head and neck cancer, chemotherapy responders had a more favorable disease-free survival than chemotherapy nonresponders in the total group of patients receiving adjuvant chemotherapy. However, correcting for imbalances in the expected three year disease-free survival of these patients, based on their disease site and stage, erased this difference, indicating tumor response to this regimen of chemotherapy is not an independent factor affecting disease outcome. The division of patients into arbitrary prognostic categories based on the expected outcome for each specific tumor site and stage proved to be a useful method for balancing treatment groups, given the multiple site-stage combinations within the upper aerodigestive tract. The defined prognostic categories were the single most sensitive predictors of relapse-free and overall survival.

Laryngocele and saccular cysts.

The symptoms, endoscopic findings, treatment and results of 46 patients with laryngoceles and saccular cysts are presented. Thirty-four were adults; 12 were infants and children under three years of age. Twenty-two adults had anterior saccular cysts, nine had lateral saccular cysts; three had laryngoceles. Ten infants and children had saccular cysts; two had laryngoceles. A laryngocele is an abnormal dilatation of the saccule which communicates with the lumen of the larynx, fills with air but on occasion may be temporarily distended with mucus; laryngoceles may be congenital or acquired. A saccular cyst is a mucus-filled dilatation of the saccule which does not communicate with the laryngeal lumen; saccular cysts are classified as lateral or anterior. Laryngoceles and saccular cysts represent abnormalities of the laryngeal saccule; a developmental spectrum exists among the normal saccule, large saccule, laryngocele and saccular cyst. The treatment of saccular cysts in infants and children is primarily repeated aspiration. In adults, symptomatic laryngoceles and large lateral saccular cysts are treated by an external approach; endoscopic aspiration and unroofing of small lateral saccular cysts is sometimes adequate and is attempted first. Anterior saccular cysts are treated by endoscopic excision biopsy. Carcinoma of the larynx may be found in association with a laryngocele or saccular cyst and must be diligently searched for by biopsies in the region of the saccular orifice. A smooth mass involving the area of the false vocal cord and aryepiglottic fold cannot be assumed to be a lateral saccular cyst; biopsies of the ventricle and saccule and deep incisional biopsies of the mass are indicated to rule out a carcinoma originating in the ventricle or saccule.

Tracheal surgery in children: an 18-year review of four techniques.

OBJECTIVE Review the short- and long-term outcomes of a single institution experience in infants with congenital tracheal stenosis, comparing four different operative techniques used from 1982 through 2000. METHODS Hospital and clinic records of 50 infants and children who had surgical repair of congenital tracheal stenosis secondary to complete tracheal rings were reviewed. Age at surgery ranged from 7 days to 72 months (median, 5 months, mean 7.8+/-12 months). Techniques included pericardial patch tracheoplasty (n=28), tracheal autograft (n=12), tracheal resection (n=8), and slide tracheoplasty (n=2). All procedures were done through a median sternotomy with cardiopulmonary bypass. Seventeen patients had a pulmonary artery sling (35%), and 11 had an intracardiac anomaly (22%). RESULTS There were three early deaths (6% early mortality), two after pericardial tracheoplasty and one after autograft. There were six late deaths (12% late mortality), five after pericardial tracheoplasty and one after slide tracheoplasty. Length of stay (median) was 60 days (pericardial tracheoplasty), 28 days (autograft), 14 days (resection), and 18 days (slide). Reoperation and/or stent placement was required in seven patients (25%) after pericardial tracheoplasty, in two patients (17%) after autograft, in no patients after resection, and in one patient (50%) after slide tracheoplasty. CONCLUSIONS Our current procedures of choice for infants with congenital tracheal stenosis are resection with end-to-end anastomosis for short-segment stenoses (up to eight rings) and the autograft technique for long-segment stenoses. Associated pulmonary artery sling and intracardiac anomalies should be repaired simultaneously.

Respiratory obstruction and apnea in infants with bilateral abductor vocal cord paralysis, meningomyelocele, hydrocephalus, and Arnold-Chiari malformation

This report describes 21 infants and children with bilateral abductor vocal cord paralysis and associated meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. Two life-threatening forms of respiratory distress are distinguished: (1) upper airway obstruction due to bilateral abductor cord paralysis and (2) apnea. Clinically significant episodes of apnea were documented in 13 infants. Ten infants had evidence of aspiration and dysphagia. Vocal cord paralysis, apnea, aspiration, and dysphagia were frequently temporally related to increased intracranial pressure.

Aerodigestive Tract Foreign Bodies in the Older Child and Adolescent

This study was undertaken in order to establish the incidence of aerodigestive tract foreign body accidents among older children and adolescents, and to investigate the circumstances surrounding these events. A review of patients treated over a 5-year period identified 367 children from whom aerodigestive tract foreign bodies were removed. Seventeen percent of these patients were 5 years of age or older. Among these children, 88% aspirated nonfood items, half of which were school supplies; 78% of the group 5 years old and under aspirated food items. Among older children with esophageal foreign bodies, 31 % had food impactions, compared with 7% in the younger group; 70% of these children had a history of some anatomic abnormality of the esophagus. These data suggest that older children and adolescents represent a distinct group of patients at risk for foreign body accidents. Pediatricians and parents of children in this age group should discourage the practice of using the oral cavity as a repository for school supplies, and should stress the need for adequate preparation and mastication of food, particularly among children with esophageal abnormalities.