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Laryngomalacia: a proposed classification form.

Laryngomalacia is a common cause of stridor in children. The disorder has a heterogenous presentation, from the mildest form, which resolves with maturation, to the most severe form, requiring tracheotomy. While there is a vast literature on the subject, there is neither stratification nor correlation of clinical presentation, endoscopic appearance, treatment and outcome. In order to statistically evaluate the choice of treatment based upon presentation, patients must first be classified by relevant predictors of disease severity. A form is proposed to classify the clinical presentation of laryngomalacia by recording relevant historical and anatomic factors. Historical factors are classified by (1) severity of stridor; (2) weight gain; (3) age at presentation; and (4) neurologic status, forming the mnemonic SWAN. The principal anatomic site of collapse is recorded as: (1) postero-lateral; (2) posterior; or (3) anterior. Endoscopic findings consistent with gastroesophageal reflux disease (GERD) or gross aspiration are noted. Photographic and/or video documentation is performed when possible. A pilot study was undertaken to determine the ease of use of this instrument. Ten children, four boys and six girls, were classified. Ages ranged from 1-day-old to 19 months, with a mean of 9 months. Five children were examined in the clinic and five in the operating room. The form was readily and easily applied, and allowed the heterogeneity of the disorder to be organized. Wider application of this form across institutions, with classification of patients with laryngomalacia by historical and anatomic factors, should allow the accumulation of sufficient numbers of patients to allow statistical analyses of treatment and outcome as they relate to the initial presentation of this disorder of airway dynamics.

Female↗

Congenital vascular anomalies and persistent respiratory symptoms in children.

Congenital cardiovascular anomalies are a rare but treatable cause of stridor in the paediatric population. Despite this, we and the authors of other series have found a delay in referral for endoscopic diagnosis. The duration of symptoms prior to endoscopic diagnosis ranged from 6 weeks to 10 years. It has been estimated that up to 3% of the population have congenital anomalies of the aortic arch complex and we feel that many patients currently being diagnosed as having recurrent croup or atypical asthma must be excluded from this group. We had 16 cases of congenital vascular anomalies causing tracheobronchial compression. Fifteen were due to innominate artery compression and one was due to a double aortic arch. Presenting symptoms included stridor (100%), persistent cough (75%), chronic dyspnoea (75%), reflex apnoea (60%), recurring respiratory tract infections (56%) and dysphagia (25%). Direct laryngoscopy and bronchoscopy was diagnostic in all cases and is our investigation of choice. Eleven underwent aortopexy (69%), one had division of the non-dominant aortic arch (6%) and four were managed conservatively (25%). They were followed for up to 9 years post-operatively and 75% had complete resolution of their symptoms. We feel that only a high clinical index of suspicion will enable these potentially fatal anomalies to be diagnosed.

Aorta, Thoracic↗

Adverse events of procedural sedation and analgesia in a pediatric emergency department.

STUDY OBJECTIVE: To determine the adverse event and complication rate for the use of procedural sedation and analgesia for painful procedures and diagnostic imaging studies performed in a pediatric emergency department. METHODS: This prospective case series was conducted in the ED of a large, urban pediatric teaching hospital. Subjects were patients younger than 21 years seen between August 1997 and July 1998, who required intravenous, intramuscular, oral, rectal, intranasal, or inhalational agents for painful procedures or diagnostic imaging. All patients who underwent procedural sedation and analgesia were continually monitored. Adverse events and complications were recorded. The ED controlled substance log was checked weekly and all sedations were reviewed. Adverse events were defined as follows: oxygen desaturation less than 90%, apnea, stridor, laryngospasm, bronchospasm, cardiovascular instability, paradoxical reactions, emergence reactions, emesis, and aspiration. Complications were defined as adverse events that negatively affected outcome or delayed recovery. RESULTS: Of 1,180 patients who underwent procedural sedation and analgesia in the ED, 27 (2.3%) experienced adverse events, which included oxygen desaturation less than 90% requiring intervention (10 patients) [supplemental oxygen (9), bag-mask ventilation (1)], paradoxical reactions (7), emesis (3), paradoxical reaction and oxygen desaturation requiring supplemental oxygen (2), apnea requiring bag-mask ventilation (1), laryngospasm requiring bag-mask ventilation (1), bradycardia (1), stridor and emesis (1) and oxygen desaturation requiring bag-mask ventilation with subsequent emesis (1). There was no statistically significant difference in mean doses for all procedural sedation and analgesia medication regimens between those children who experienced adverse events and those who did not. No single drug or drug regimen was associated with a higher adverse event rate. In addition, there was no significant difference in the adverse event rate between males and females, among the different ages, or among the different indications for procedural sedation and analgesia. No patient required reversal of sedation with naloxone or flumazenil, endotracheal intubation, or hospital admission because of complications from procedural sedation and analgesia. CONCLUSION: The adverse event rate for procedural sedation and analgesia performed by pediatric emergency physicians was 2.3% with no serious complications noted.

Adolescent↗

A new diagnostic approach to vascular rings and pulmonary slings: the role of MRI.

UNLABELLED: The conventional diagnostic work-up of a patient suspected of having a vascular cause for stridor, or dysphagia, includes esophagography and bronchoscopy to delineate the abnormal structure without imaging the structure itself. Cine-angiography is regarded as the golden standard, but is not routinely performed. Magnetic resonance imaging (MRI) is non-invasive and has the important advantage over cine-angiography of depicting all structures in the field of view. Color Doppler echocardiography depicts the great vessels, but not the esophagus and trachea. In 14 patients with obstructive symptoms and in four patients without obstructive symptoms MRI successfully imaged the abnormal structure, as was the case in two symptomatic patients using computer tomography. In this series, the findings were confirmed at surgery or by cine-angiography. CONCLUSION: we suggest that in patients suspected of having a vascular cause for stridor or dysphagia, MRI should be performed. If there is need for a screening procedure, color Doppler echocardiography should be used and if that is equivocal or non-conclusive, esophagography and bronchoscopy should be used. If MRI is difficult to interpret, it should be augmented by magnetic resonance angiography before considering cine-angiography.

Adult↗

Airway problems in patients with rheumatologic disorders.

The intensivist should be aware of the upper airway manifestations of the common rheumatologic disorders which may lead to ICU admission or which may potentially pose a problem during airway management. Information should be obtained from the patient, the patient's family, and the patient's primary physician, if possible. One should be fully prepared with various options in case a problem arises with an airway. Equipment for managing a difficult airway should be available. Alternate methods of managing the airway (e.g., the laryngeal mask airway, fiberoptic scopes, and the WU Scope) (Achi Corporation, Fremont, CA) are of great help in dealing with airway problems. The potential for cervical spine instability exists in patients with rheumatologic disorders. Intubating with care and avoiding spinal movement both seem to be more important than any particular mode of intubation in preserving neurologic function. One should make a concentrated and serious effort to be as gentle as possible and to avoid even minimal trauma to the mucosa in these patients, because they are at risk for mucosal edema and subsequent postextubation stridor. In cases of stridor, helium-oxygen mixtures may be of help and may eliminate the need for reintubation. When difficulty in establishing an airway is anticipated, it is prudent to attempt airway control in the operating room with surgical assistance standing by should cervical tracheotomy is required.

Connective Tissue Diseases↗

Paradoxical vocal fold movement: a case report.

We present a case of one patient with respiratory stridor that was resolved by speech therapy. Paradoxical vocal fold movements were observed by flexible fiberoptic videolaryngoscopy in this patient during episodes of wheezing and dyspnea. Otherwise, normal vocal fold movement was observed in normal conditions of breathing (out of the crisis) and during phonation. Many different terms have been used to describe this entity in the literature, and it is crucial that clinicians recognize the subtle signs of functional stridor. Paradoxical vocal fold motion has to be considered as an important cause of respiratory disease. Its recognition and treatment are discussed in this report.

Adult↗

[Episodic laryngeal dysfunction].

Episodic laryngeal dysfunction (ELD), also known as paradoxical vocal cord dysfunction or laryngeal dyskinesia, is characterized by abnormal closure of the vocal cords during inspiration (and sometimes at the very start of expiration). It can manifest in different ways depending on the patient's age. In the newborn, it is usually associated with stridor restricted to the inspiratory breath following crying, and the natural history is always one of rapid resolution. Occasionally, a more severe form presents with intense dyspnea and marked inspiratory stridor in a baby that usually has extensive gastroesophageal reflux which has not been treated adequately enough to improve the dyspnea or the associated vasovagal attacks. A tracheotomy may be necessary. It is rare for this disease to present between the ages of 2 and 8 years. Thereafter, it may present as a form of pseudo asthma resistant to bronchodilators and anti-inflammatory drugs. The dyspnea can be very severe and lead the family to seek hospital admission, at least during the initial episodes. There is a female preponderance. The key to making the diagnosis is the complete reversibility of the patient's symptoms when they are distracted. Exertion asthma can be mimicked by forms of ELD that occur only by effort (apart from the profile of the lung function tests). There may be significant gastroesophageal reflux. Medical intervention (hospitalization and tracheotomy) must be avoided, treatment being essentially behavioral. Finally, some cases of paradoxical adduction of the vocal cords have been described with the use of neuroleptics, brain stem compression, cortical lesions and, much more rarely, motoneuron disease. This diagnosis requires a high index of suspicion, particularly in patients with asthma whose presentation or clinical course with treatment is atypical.

Age Factors↗

An unusual case of laryngomalacia presenting as asthma refractory to therapy.

BACKGROUND: Laryngomalacia is the most common cause of stridor in infants, but few reports exist of clinically relevant laryngomalacia in adults. OBJECTIVE: To present and discuss an unusual late presentation of laryngomalacia and its significance in the evaluation and management of asthma. METHODS: An 18-year-old woman presented to an academic medical center with symptoms of "wheezing" on inspiration and exertion, with relatively normal spirometric findings. She was clinically diagnosed as having asthma at the age of 13 years, but her symptoms were poorly controlled by maximal medical therapy. Further evaluation with rhinolaryngoscopy demonstrated laryngomalacia characterized by redundant soft tissue overlying the right arytenoid cartilage and aryepiglottic fold. RESULTS: The patient demonstrated positive bronchoprovocation, with a 33% decrease in forced expiratory volume in 1 second after the administration of histamine, 1 mg/mL. However, with the otolaryngology evaluation, it was determined that her laryngeal findings were clinically significant. She subsequently underwent operative laryngoscopy with carbon dioxide laser excision of the laryngeal abnormality, resulting in improvement in her symptoms and a marked decrease in her need for asthma medication. CONCLUSIONS: We report an unusual case of laryngomalacia presenting as asthma that was successfully treated with laser surgical excision. This case emphasizes the necessity of differentiating classic wheezing from stridor and upper airway obstruction.

Adolescent↗

Functional airway obstruction mimicking tongue angioedema.

BACKGROUND: Functional causes of upper airway obstruction have focused primarily on psychogenic stridor associated with paradoxical vocal cord dysfunction. Angioedema can involve upper airway structures and be life-threatening. CASE REPORT: We report a 12-year-old female with upper airway obstruction from posterior tongue swelling which was determined to be a conversion reaction. METHODS & RESULTS: A lateral neck film revealed severe tongue swelling. Examination revealed a calm, cooperative patient in no distress but exhibiting inspiratory and expiratory stridor. A computed tomography scan revealed soft tissue fullness at the base of the tongue without associated lymphadenopathy. Laboratory evaluation was normal. With anesthetic induction in the operating room, there was complete relaxation of the upper airway with no evidence of tongue swelling, mass, or other abnormality. Following tongue biopsy, she had no reoccurrence of the tongue mass. CONCLUSION: This case represents a childhood conversion reaction of functional airway obstruction where tongue manipulation simulated tongue swelling radiographically consistent with angioedema.

Airway Obstruction↗

[Treatment laryngomalacia: experience with 22 cases].

UNLABELLED: Laryngomalacia is the most frequent cause of stridor in childhood, and in most of the cases, spontaneous resolution occurs by the age of 2 years. Approximately 10% of the cases (severe laryngomalacia) require surgery. This condition is of unknown etiology and its diagnosis is made by fiberoptic laryngoscopy, which shows shortening of the aryepiglottic folds, and/or redundant arytenoid mucosa, and/or anterior-posterior epiglottic prolapse. AIM: Our objective was to verify the main clinical and anatomical affections and to highlight the clinical parameters for clinical follow-up and surgical indication in patients with laryngomalacia. STUDY DESIGN: Transversal cohort study. MATERIAL AND METHOD: Twenty-two children diagnosed with laryngomalacia in the Pediatric Otorhinolaryngology of UNIFESP-EPM, from January 2001 to December 2003, whose clinical and surgical follow-up were performed by the same examiner, were enrolled in this study. RESULTS: Out of twenty-two evaluated children, 2 (9.1%) presented with severe laryngomalacia and pectus excavatum (funnel chest). At polysomnography, no child presented any significant respiratory event during sleeping. Those two children with severe laryngomalacia were submitted to supraglottoplasty with resection of the aryepiglottic folds. CONCLUSION: We concluded that stridor and shortening of the aryepiglottic folds are preponderant in children with laryngomalacia. The polysomnographic exam did not prove to be a good parameter for clinical follow-up, neither for surgical indication. The most important parameters were pectus excavatum and failure to thrive. Supraglottoplasty is effective with low rate of morbidity.

Cohort Studies↗

Acute epiglottitis in adults (the Singapore experience).

Acute epiglottitis in adults is a potentially fatal but self-limiting disease of increasing incidence world-wide. Forty-two patients, seen consecutively over a four year period at the ENT Department, Singapore General Hospital were reviewed retrospectively. A strong male predominance with a peak age incidence in the sixth decade was noted. A severe sore throat and dysphagia with disproportionate signs of oropharyngeal inflammation was the main presenting picture. Only three patients had stridor on presentation. Vigilant monitoring of the airway with empirical high-dose intravenous ampicillin, cloxacillin and steroids resulted in a dramatic clinical improvement in most patients and none developed stridor after admission. The yield from throat swabs and blood cultures were low. Two patients developed complications, a Ludwigs angina and an epiglottic abscess. Recurrent epiglottitis was a problem in one patient. There was low morbidity and no mortality on the management regime outlined.

Acute Disease↗

Branchial cleft and pouch anomalies.

We present a retrospective study of 106 patients with branchial cleft and pouch anomalies who presented to the Hospital for Sick Children between 1948 and 1990. The relevant embryology of the branchial apparatus is summarized and a theoretical description of individual anomalies given. Second branchial cleft sinuses were the most common anomalies, and the majority were managed simply, with adequate excision and a low recurrence rate. Five cases of first branchial cleft anomalies are presented, emphasizing the delay in diagnosis, the need for complete excision to prevent recurrence, and for a parotidectomy incision to protect the facial nerve from damage. The two third branchial pouch anomalies presented with a cystic neck swelling, one with recurrent infection and discharge, and the other with stridor. In both, the diagnosis was made at operation. The single fourth branchial pouch cyst was an unexpected finding in a patient with stridor.

Branchial Region↗

Hamartoma of the larynx causing neonatal respiratory distress.

A new-born boy with severe stridor was found to have a laryngeal hamartoma. The airway was secured by means of a tracheostomy, and the hamartoma was subtotally resected endoscopically using a carbon dioxide laser. A good functional result was achieved, even though decannulation was delayed until the age of 33 months. This is the ninth known case of hamartoma of the larynx and the sixth presenting with stridor in the neonatal period.

Hamartoma↗

Vocal fold abductor paralysis as a solitary and fatal manifestation of multiple system atrophy.

A patient is presented who had bilateral abductor vocal fold paralysis pathologically proven to be due to multiple system atrophy (MSA) in the absence of other neurological features. MSA is a degenerative neurological condition that includes olivopontocerebellar atrophy, Shy-Drager syndrome and striatonigral degeneration. The usual predominant features of MSA are cerebellar ataxia, autonomic dysfunction and Parkinsonism. Stridor is present in over one third of patients and has been reported previously as a presenting symptom in MSA: however previously reported patients have always gone on to develop other neurological symptoms. The usual investigations of bilateral abductor vocal fold paralysis caused by MSA will not reveal the pathological process and we believe that magnetic resonance imaging (MRI) of the medulla and brain stem and autonomic function tests are probably the investigations of choice. It is a worthwhile exercise attempting to identify MSA as the cause of stridor as the prognosis is good in the medium term if appropriate support is offered.

Aged↗

Double epiglottis in Weyer's acrofacial dysostosis.

While evaluating a 61-year-old patient for stridor we incidentally detected a double epiglottis. The patient was also diagnosed of having Weyer's acrofacial dysostosis which is characterized by hexadactyly affecting all four extremities, small and deeply set nails, dental deformities with small, conical teeth and mandibular hypoplasia. The double epiglottis was not the cause for the stridor. Because of the covert symptomatology of double epiglottis it is suggested that the association with Weyer's syndrome is common. Embryological evidence and a review of the literature on laryngeal abnormalities is discussed.

Epiglottis↗

Failed tracheostomy under local anaesthesia...plan B?

Severe stridor of recent onset is a challenge to deal with because of the lack of investigations on which to base the management plan. We describe a case of an elderly lady who presented to us with a short history of severe stridor. We encountered unanticipated difficulties with tracheostomy under local anaesthesia as the thyroid was replaced by a diffuse mass and the airway had to be secured by an awake fibre-optic intubation. Awake fibre-optic intubation is thought to be a relative contraindication in acute upper airway obstruction, but occasionally tracheostomy under local anaesthesia may not be possible and in experienced hands an awake fibre-optic intubation is a reasonable alternative.

Aged, 80 and over↗

Complications of emergency tracheal intubation in severely head-injured children.

A high incidence of unsuccessful attempts and complications has been reported when emergency tracheal intubation (ETI) is performed outside the hospital in severely injured children. The aim of this prospective series was to analyse the incidence and related risk factors of complications of emergency tracheal intubation. The time to complete successful ETI and occurrence of incidents, e.g. cough reflex, hypoxia or spasm were related to the experience of the physician performing intubation and the use of drugs to facilitate ETI. The incidence of hypoxia, hypercarbia, postintubation complications such as extubation stridor and long-term sequelae were noted. Of the 188 children, 78% were successfully intubated at the site of the accident, 10% upon arrival at a local hospital from where they were secondarily transferred and 12% upon admission to our trauma centre. The most severely injured children were intubated in the field in 98% of cases without failure, nor life-threatening complications related to ETI. The experience of the operator influenced the number of attempts and the time to complete successful intubation. Immediate incidents were noted in 25% of children, e.g. cough in 18%. The regimen of drugs, but not level of consciousness, influenced the incidence of immediate incidents; without drugs, more than 67% experienced incidents. Early tracheal intubation and controlled ventilation resulted in adequate ventilation upon arrival (mean PaO2 of 35.8+/-24 kPa, mean PaCO2 of 4.35+/-1 kPa). Long-term complications, including transient stridor upon extubation in 33% of the cases, and laryngeal granuloma or tracheal stenosis, were comparable to those in other series. ETI in shocked patients and pulmonary infection in hospital, but not the technique of ETI, increased the risks of long-term complications. Emergency tracheal intubation can be performed safely in the field, and results in adequate ventilation during transportation of severely injured children, provided that it can be performed by trained physicians using adequate drugs to facilitate intubation.

Child↗