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Congenital laryngeal anomalies.

The physician who is called upon to evaluate stridor in the newborn must be familiar with common congenital lesions of the larynx. Congenital stridor is a symptom of an underlying problem that must be accurately diagnosed to be properly and effectively managed. This requires a complete history and physical examination to discover the type of stridor and its association with feeding difficulties or cyanosis. Careful evaluation of the cry is important. Radiologic examinations are of great value in providing additional information concerning laryngeal anomalies. The mainstay of diagnosis is endoscopic examination. Familiarity with these anomalies, their symptoms, diagnostic methods, and treatment will provide for better care of the pediatric patient with stridor.

Child↗

Respiratory insufficiency as the primary presenting symptom of multiple-system atrophy.

BACKGROUND: Respiratory stridor, sleep-disordered breathing, and respiratory insufficiency are part of the clinical spectrum of multiple-system atrophy (MSA). We have encountered cases where these were presenting symptoms, with the diagnosis of MSA being initially unrecognized. OBJECTIVE: To describe cases in which breathing difficulties were the initial and primary manifestation of MSA. DESIGN: Database review from January 1, 1996, through October 31, 2005. SETTING: Mayo Clinic, Rochester, Minn. PATIENTS: All patients diagnosed as having MSA, cross-referenced for apnea, hypopnea, or hypoventilation. On review, we included only cases in which respiratory dysfunction was the primary initial clinical event in MSA, excluding equivocal cases. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Characteristics and clinical course of patients. RESULTS: Six cases were identified in which substantial respiratory insufficiency occurred as an early, presenting symptom of MSA. Three patients had been examined emergently for acute respiratory distress before the ultimate diagnosis of MSA; the other 3 patients were diagnosed as having obstructive sleep apnea unresponsive to therapy, with bilateral vocal cord paralysis found on ear, nose, and throat examination. Stridor was noted early in the course in all. All patients required tracheostomy, and all eventually developed features consistent with probable MSA. CONCLUSIONS: Multiple-system atrophy may occasionally present as primary respiratory failure or dysfunction, with initially mild motor and autonomic symptoms. Otherwise unexplained central respiratory failure, bilateral vocal cord paralysis, stridor, or refractory central sleep apnea should prompt consideration of MSA.

Aged↗

Functional upper airway obstruction in adolescents.

Functional upper airway obstruction is an uncommon manifestation of a conversive reaction characterized by recurrent stridor attacks caused by adduction of the vocal cords during inspiration. The oxygen saturation never drops to pathologic levels. The stridor is not accompanied by an appropriate degree of anxiety and is not associated with other symptoms. Patients benefit from verbal reassurance and speech therapy, but stridor attacks tend to recur and psychiatric consultation seems necessary in most cases. We present the case histories of three adolescent patients with nonorganic upper airway obstruction and describe the features that may facilitate the diagnosis of this condition. Early diagnosis and intervention may prevent unnecessary and potentially harmful investigations and therapy.

Adolescent↗

Long-term outcome of surgically treated acquired subglottic stenosis in infancy.

There is little information about the long-term outcome of infants with acquired severe subglottic stenosis (SGS) who require surgical intervention. We retrospectively identified infants with acquired subglottic stenosis who required anterior cricoid split (ACS) or tracheostomy for primary airway management; some of these children later required laryngotracheoplasty (LTP). All children were treated at our hospital from 1989-1997. During follow-up, we assessed patients for persistent symptoms (stridor at rest, exercise limitation, difficulty with respiratory tract infections, recurrent croup, and voice alteration), and we measured lung function when possible. We identified 34 infants with acquired SGS: 13 treated primarily with tracheostomy, and 21 with ACS. Nine patients could not be extubated following ACS and required tracheostomy, while the 12 who were extubated were followed up at a mean time of 76 months postoperatively; 3 had moderate stridor at rest, 1 moderate exercise limitation, and none had recurrent croup. Four of 5 who had lung function measured had moderately severe extrathoracic airflow limitation. From the tracheostomy group (n = 13) and the failed ACS group (n = 9), 2 patients were decannulated without further surgery, 17 underwent LTP, and 2 have LTP planned. Fifteen of the 17 patients who had LTP have been decannulated. Follow-up, at a mean time of 58 months postoperatively, showed none with stridor at rest, 3 with moderate exercise limitation, none with recurrent croup, and 2 with moderate voice alteration. All 5 patients who had lung function measured had airflow limitation, 1 being severe. In conclusion, ACS facilitates extubation in selected patients with severe, acquired SGS of infancy, and the long-term outcome of patients successfully extubated is excellent. Failure of ACS invariably means tracheostomy, and subglottic repair by LTP is associated with a good long-term outcome.

Airway Obstruction↗

Distal tracheal compression.

The consultants concur that distal tracheal stenosis is a challenging problem in the pediatric patient but don't all agree on the presentation or management. Drs. Cotton and Crysdale agree that these children are usually seen with biphasic stridor. Dr. Parsons believes that biphasic stridor is not particularly common but inspiratory stridor is. The experts divide the differential diagnosis into intrinsic and extrinsic categories. Dr. Cotton lists tracheomalacia, complete tracheal rings and masses, such as an isolated hemangioma or granuloma. The usual extrinsic lesion is a vascular anomaly, the most common being an innominate artery. Dr. Crysdale's differential diagnosis includes tracheal stenosis, either congenital or acquired, and tracheomalacia. Vascular compression is most common as an extrinsic cause. Dr. Parsons includes tracheomalacia, complete tracheal rings, segmental tracheal stenosis, isolated masses, and vascular anomalies, such as an aberrant innominate artery. He also states that a tracheoesophageal fistula must be considered. Dr. Cotton prefers MRI and possibly a barium swallow for assessing vascular architecture. Even finding an air bronchogram on a chest x-ray can pinpoint an area of stenosis. The best test is direct laryngoscopy with video-photography. While Drs. Parsons and Crysdale agree that direct laryngoscopy is the best, Dr. Crysdale also suggests MRI or CT and adds that a contrast bronchogram is a most useful test.(ABSTRACT TRUNCATED AT 250 WORDS)

Airway Obstruction↗

Vincristine-induced recurrent laryngeal nerve paralysis in children.

Stridor developed in 2 children during vincristine therapy for malignancies. Indirect laryngoscopy revealed left vocal cord paralyses in both patients. One child had generalized neurotoxicity from vincristine including hypotonia, decreased gastrointestinal motility, and painful paresthesias while laryngeal nerve paralysis was the only neurotoxic manifestation in the other patient. Stridor resolved in both patients after discontinuing or decreasing the dose of vincristine. Visualization of the airway not only confirms the diagnosis, but also rules out treatable causes of stridor in the febrile, immunocompromised patient.

Child↗

Use of endotracheal silicone stents for relief of tracheobronchial obstruction.

In this article we describe our initial experience with bifurcated and longitudinal silicone stents that can be inserted entirely endoscopically. A total of 10 patients were stented; half had upper airways obstruction resulting from malignant disease and half had anastomotic obstruction after single-lung (3 patients), double-lung (1 patient), or heart-lung transplantation (1 patient). All patients derived immediate relief of life-threatening stridor. Stents were in place for between 5 days and 2 1/2 years (mean, 232.9 days). In the patients with malignant disease, the stents have provided effective relief from stridor for the remainder of their lives. In the transplant recipients, the medium-term results are encouraging, with the stents providing effective relief from stridor, although the longitudinal stents have been associated with distal migration, requiring that the stents be replaced on up to five occasions. The stents have not been associated with infection in the nonimmunosuppressed patients, and during the relatively short follow-up period there has been no tissue reaction to the material.

Adult↗

[Vascular tracheal compression presenting as bronchiolitis in infants].

BACKGROUND: Anomalies of the aortic arch and pulmonary arteries may produce compression of the trachea with chronic stridor or wheezing aggravated by crying, feeding and flexion of the neck. CASE REPORTS: Three infants, 3, 5 and 2 months-old, respectively, were admitted suffering from acute bronchiolitis. The first patient had intermittent wheezing for one month; lateral chest X-rays showed an opaque area inserted between the trachea and oesophagus and computed tomography showed pulmonary artery sling with tracheal stenosis which was successfully operated. The second patient displayed pulmonary atelectasia which required bronchoscopy; this technique showed tracheal stenosis which was due to pulmonary artery sling. The third patient had a history of congenital stridor: he required endotracheal intubation; a bronchoscopy performed because persistent pulmonary atelectasia showed tracheal stenosis which was secondary to a double aortic arch. The condition was improved after surgical correction. CONCLUSIONS: A history of stridor and/or wheezing in patients admitted for bronchiolitis must lead to consider the possibility of vascular compression of the trachea and to perform specific investigations.

Aorta, Thoracic↗

Paradoxical vocal cord motion--a case report.

Paradoxical vocal cord motion (PVCM) is an unusual cause of stridor, which is associated with some underlying causes, such as central nervous system lesion, gastroesophageal reflux or psychogenic problem. Once a diagnosis of PVCM is made, acute management with reassurance and sedation instead of aggressive airway intervention is required. Speech therapy, psychotherapy combination with anti-reflux medication is considered to be useful in long-term management. We present a 58 year-old male patient who had suffered from several episodes of acute onset of stridor, short of breath and tachypnea since one year ago. He was initially treated as an asthmatic patient with poor response. Aneurysm of ascending aorta by angiography, and mild gastroesophageal reflux with hiatal hernia by panendoscopy were noted. Then, the paradoxical vocal cord motion during inspiration phase was confirmed by flexible fiberoptic nasopharyngoscope after the consultation with an otolaryngologist. The emergency of his air-hunger was relieved quickly after psychological intervention. Now, he is free of stridor attack under anti-reflux therapy and psychotherapy.

Botulinum Toxins↗

A newborn case of congenital laryngeal cyst complicated with pneumothorax and pneumomediastinum.

Benign congenital laryngeal cysts are rare entities. They often cause chronic hoarseness and severe stridor. Case reports of congenital laryngeal cyst complicated with pneumothorax and pneumomediastinum are very rare. A 3,112 g full-term male newborn developed stridor which got worse during crying for 12 h after birth. Chest retractions were present with inspiration. Chest X-rays showed the presence of right pneumothorax and pneumomediastinum. Transnasal flexible laryngoscopic examination revealed a large cystic mass, which occupied almost the entire supraglottic airway. The operation was performed with the techniques of laryngomicrosurgery under general anesthesia. The cystic wall was punctured and serous liquid contents were aspirated. Excision of the entire cystic lesion was performed. The next day, extubation was performed without any troubles. The stridor had disappeared and the pneumothorax and pneumomediastinum were improved without further medical intervention. The histopathological examination revealed that the cystic wall consisted of normal squamous epithelial cells. It is reasonable to think that the high airway pressure due to congenital laryngeal cyst was responsible for pneumothorax and pneumomediastinum.

Cysts↗

Microdebrider-assisted supraglottoplasty.

OBJECTIVE: Although laryngomalacia is a common cause for infantile stridor, few patients eventually go on to require surgical intervention. When surgery is required, the location of tissue excised may vary depending on the endoscopic findings, but only two methods of tissue excision are described-cold knife excision and carbon dioxide laser. We present our experience of using the microdebrider to excise tissue during supraglottoplasty. METHODS: Over the last 12 months, patients were identified who had undergone supraglottoplasty, and their final outcomes with regards to resolution of stridor, cor pulmonale, and/or failure to thrive were assessed. A description of the indications for supraglottoplasty and the actual technique utilizing the microdebrider is included. RESULTS: Five patients were identified as having undergone a microdebrider-assisted supraglottoplasty. All five had resolution of their stridor following surgery. No new complications such as aspiration or supraglottic stenosis were identified. No revision surgeries were required. The microdebrider was used to trim the aryepiglottic folds and/or redundant arytenoid mucosa in all cases. CONCLUSIONS: The microdebrider appears to be a safe and effective tool to remove redundant tissue during supraglottoplasty.

Debridement↗

The relationship between chronic cough and paradoxical vocal fold movement: a review of the literature.

SUMMARY: Chronic cough (CC) and paradoxical vocal fold movement (PVFM) are debilitating conditions. PVFM has been given many labels, including vocal cord dysfunction, Munchausen's stridor, functional inspiratory stridor, nonorganic functional or psychogenic upper airway obstruction, factitious asthma, psychogenic stridor, emotional laryngeal wheezing, and episodic laryngeal dyskinesia. Although CC and PVFM have been considered separate entities in many reports, there is preliminary support for the notion that there may be an underlying link between these two conditions. Speech pathologists have become increasingly involved in the treatment of these patients and therefore need to understand the theoretical background of these disorders, the pathophysiological links between the two, and the impact of voice disorders on these populations. The aim of this article is to review the current literature on CC and PVFM from a speech pathology perspective to provide a model for defining and conceptualizing the disorders and to provide a framework for management and future research.

Behavior Therapy↗

Effectiveness of dexamethasone in preventing extubation failure in preterm infants at increased risk for airway edema.

We studied 50 preterm infants who had multiple or traumatic endotracheal intubations, or whose duration of endotracheal intubation was > or = to 14 days, and who were considered at high risk for airway edema. These infants were enrolled in a prospective, randomized, controlled clinical trial to assess whether prophylactic dexamethasone therapy would be effective in the prevention of postextubation stridor and respiratory distress. At study entry, both groups had similar weights, postnatal ages, methylxanthine use, ventilator settings, blood gas values, and pulmonary function test results (dynamic compliance, total respiratory resistance, tidal volume, peak-to-peak transpulmonary pressure, minute ventilation, and peak inspiratory and expiratory flow rates). Patients underwent blood gas studies, physical examinations, and pulmonary function testing at baseline (4 hours before extubation) and again 2 to 4 hours and 18 to 24 hours after extubation. Twenty-seven infants received dexamethasone, 0.25 mg/kg per dose, at baseline, and then every 8 hours for a total of three doses; 23 infants received saline solution at corresponding times. Eighteen to twenty-four hours after extubation, total pulmonary resistance increased by 225% from baseline in the control group compared with 33% in the dexamethasone group (p < 0.006), and the dexamethasone group had a greater tidal volume, a greater dynamic compliance, and a lower arterial carbon dioxide pressure. Of 23 control infants, 10 had postextubation stridor compared with 2 of 27 dexamethasone-treated patients (p < 0.006). Of the 23 control patients, 4 required reintubation compared with none of the treated group (p < 0.05). We conclude that the prophylactic use of corticosteroids for the prevention of postextubation stridor and respiratory distress is efficacious in low birth weight, high-risk preterm infants.

Carbon Dioxide↗

Acute apnea caused by an epiglottic cyst.

A life threatening complication in the course of routine sedation in a 5 year old child is reported in a case study. A retention cyst of the epiglottis was found to be responsible for obstruction of the larynx leading to acute apnea. The patient history revealed recurrent episodes of stridor previously diagnosed and treated as acute laryngotracheobronchitis as well as border line psychomotoric retardation. Cerebral magnetic resonance imaging was performed for neuroradiological evaluation. After administration of sedation the child presented with stridor and acute apnea. Emergency orotracheal intubation could prevent tracheotomy but was complicated by the unexpected presence of a tumor at the base of the tongue. Further evaluation revealed a large epiglottic cyst. After endoscopic removal of the cyst no further episodes of apnea or stridor were noted.

Acute Disease↗

Nasopharyngeal teratoma involving the temporal bone.

Teratoma is the most common nasopharyngeal tumor in neonates. The tumor is usually limited to the oropharynx and is manifest by stridor and respiratory distress. Complete excision affects a cure. An unusual case of nasopharyngeal teratoma extending into the temporal bone, causing facial paralysis and conductive hearing loss, in addition to stridor, is presented. Stridor was relieved by transoral partial excision shortly after birth. Complete removal of the tumor by way of a subtemporal and infratemporal fossa approach was performed at 14 months of age. The defect was filled with a rectus abdominis muscle graft with microvascular anastomoses. The facial nerve was reconstructed secondarily with a sural nerve graft. The patient has no recurrence tumor and has good facial function at 30 months. This case demonstrates the first known case of facial paralysis due to nasopharyngeal teratoma. The surgical approach for tumor removal: lateral infratemporal fossa dissection, and the method of reconstruction: free rectus abdominis muscle flap with microvascular anastomoses, had so far not been described in a patient this young.

Humans↗

[Fatal double tracheo-esophageal vascular compression and neurofibromatosis].

BACKGROUND: Vascular rings are a classical cause of tracheal and esophagus compression. We report the case of such an abnormality in an infant with neurofibromatosis. CASE REPORT: A 1 week-old male infant with a familial neurofibromatosis presented a stridor with severe respiratory distress. A vascular ring was demonstrated and operated on. The stridor persisted after surgery. A postoperative oesophagogram and tracheobronchoscopy showed an irregular compression of the oesophageal lumen, thought to be due to a residual extrinsic compression. Because the postoperative echocardiogram showed an extensive tumoral infiltration of both auricles, it was decided to not operate again the child. The postmortem examination revealed a disseminated neurofibromatosis infiltrating trachea, bronchi and also the wall of esophagus. CONCLUSION: Persisting stridor and oesotracheal compression postoperatively requires search for another cause. Association of vascular ring and neurofibromatosis is probably not fortuitous.

Aorta, Thoracic↗

Laryngeal lymphatic malformation in a newborn.

Stridor in a neonate evokes a series of prompt evaluations to determine the etiology. Common etiologies include inherent structural defects (such as laryngomalacia), vocal fold paralysis, acquired infectious etiologies, or extrinsic compressions. We report a neonate with a lymphatic malformation (lymphangioma) initially confined to the larynx that presented with stridor and progressive respiratory failure. Only seven other cases have been reported to present with lymphatic malformation confined solely to the larynx. There are several different modalities available to treat this condition, but recurrences are common. The presentation and management are discussed below. Physicians should be aware that stridor in a neonate can result from this unusual intrinsic obstruction of the larynx.

Humans↗

Tenuous airway in children with trisomy 21.

Multiple congenital anomalies are closely linked to Down syndrome (trisomy 21). Cardiac malformations are found in 40% of patients, a large number of whom will require a major surgical procedure. The importance of postextubation stridor in these children is frequently underestimated. A retrospective review of 99 trisomy 21 patients who underwent cardiovascular surgery revealed postextubation stridor in 24 (24.2%). Significant factors for the development of stridor included younger age (P=0.04), lower growth percentile for weight (P=0.03), and increased frequency of reintubation (P=0.04). Subglottic stenosis was found in 6 (6.1%). In 4 of these patients, an endotracheal tube of larger diameter than predicted for age was used. All 6 patients were less than the 10th percentile for weight. We conclude that Down syndrome patients deserve special considerations and modifications of standard intubation techniques for successful airway management.

Age Factors↗