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Granular cell tumour of the larynx.

Granular cell tumour (GCT) is a benign tumour with abundant eosinophilic cytoplasm filled with granules of varying sizes. These granules are the defining characteristic of the GCT and are believed to represent lysosomes in varying stages of fragmentation. The commonly used term granular cell myoblastoma, found in the older literature, is a misnomer because the tumour is clearly not of muscle origin. Among the major theories of origin, some support the tumour's derivation from neuronal tissue, histiocytes, fibroblast or Schwann cells. In the larynx, pseudoepitheliomatous hyperplasia may predispose to confuse the GCT with squamous cell carcinoma. The most common region of GCT is in the head and neck, accounting for approximately 30 to 50 per cent of all lesions. The larynx is relatively an uncommon location for these tumours, accounting for approximately three to 10 per cent of the reported cases. Affected patients typically present with persistent hoarseness, stridor, haemoptysis, dysphagia, and otalgia, but the tumour may be asymptomatic and be discovered only incidentally during a routine examination. Complete excision with an attempt to maintain normal structures generally results in cure. We present the case of a patient with typical features of a GCT of the larynx. The gross appearance, histopathology and brief discussion of the current literature are also presented.

Adult↗

Primary tuberculous tracheitis.

We report a case of primary tuberculous tracheitis in an otherwise healthy woman who presented with cough and stridor due to diffuse tracheal narrowing by tuberculous pseudomembranous lesion, which resolved completely with antituberculosis chemotherapy.

Antitubercular Agents↗

Metastasis to the larynx from a prostatic carcinoma. A case report.

We present the fourth reported case of metastases of adenocarcinoma from the prostate to the larynx. The patient was a 64-year-old male who presented with stridor from a smooth subglottic mass six months after he had been diagnosed as having adenocarcinoma of the prostate with bone secondaries. We have reviewed the mechanisms by which such a tumour might metastasize to the larynx and we feel that ossification in the laryngeal cartilages may be important in the development of such metastases.

Adenocarcinoma↗

Benign mucous membrane pemphigoid of the upper aero-digestive tract: rare paraneoplastic syndrome presentation in renal cell carcinoma.

Benign mucous membrane pemphigoid is a rare autoimmune disorder affecting the upper aero-digestive tract and conjunctivae. This is a case presentation of benign mucous membrane pemphigoid affecting the oral mucosa, pharynx, oesophagus and larynx, leading to cicatricial lesions in the pharynx and larynx, causing dysphagia, hoarseness and stridor. The alternative forms of management for laryngeal scarring due to this disease are explained. The patient was later diagnosed with advanced renal cell carcinoma, raising the possibility of cicatricial pemphigoid manifesting as a paraneoplastic syndrome of underlying renal cell carcinoma.

Aged↗

Tracheoesophageal compression associated with substernal goitre. Correlation of symptoms with cross-sectional imaging findings.

OBJECTIVE: The aim of this study was to compare the subjective tracheoesophageal pressure symptoms associated with substernal goitres with objective cross-sectional radiographic measurements. DESIGN: Patients with substernal goitre were questioned regarding tracheoesophageal compression symptoms and these data were analysed for correlation with radiographic and intra-operative findings. RESULTS: Twenty-six patients were included. Patients with airway symptoms including dyspnoea, stridor and coughing were more likely to have significant tracheal narrowing on pre-operative computed tomography (CT) imaging. There was also a significant correlation between the perceived severity of lump sensation in the throat and the presence of tracheal and oesophageal displacement and retrotracheal extension of the thyroid mass. CONCLUSION: Pre-operative CT measurements of tracheal and oesophageal displacement, retrotracheal extension and the degree of tracheal compression correlate well with the presence and severity of tracheoesophageal pressure symptoms. None of the radiographic findings that are exclusive to substernal goitre (percentage of substernal mass, goitre size at the level of the thoracic inlet, ratio of goitre size to the vertebral body at the thoracic inlet and retrosternal isthmus size) had any correlation with clinical symptoms in this study.

Airway Obstruction↗

Fatal aortoesophageal fistula in two cases of tight vascular ring.

Vascular rings are rare vascular congenital anomalies causing oesophageal and tracheal compression. An aortoesophageal fistula is a devastating, in part iatrogenic, complication of vascular rings. It is seen with increasing frequency, and can be misleading, since differential diagnosis with other causes of haematemesis and melaena is often difficult, especially in infants. We report two infants with aortoesophageal fistulas secondary to double aortic arches forming a vascular ring. In both, the diagnosis was missed, and massive haemorrhage led to death. In both cases, the fissuration on the oesophageal and aortic sides of the fistula had sharp edges, highly suggestive of an iatrogenic laceration caused by manipulation of nasogastric tubes. The key for the diagnosis of vascular rings is, therefore, clinical suspicion and awareness of this condition. Prompt identification in infants with stridor, wheezing, or respiratory distress can prevent prolonged intubation, thus avoiding the formation of an aortoesophageal fistula and hopefully preventing a fatal outcome.

Aorta, Thoracic↗

D-2-hydroxyglutaric aciduria: further clinical delineation.

It has recently been recognized that D-2-hydroxyglutaric aciduria is a distinct neurometabolic disorder with a severe and a mild phenotype. Whereas the clinical and neuroimaging findings of the severe phenotype were homogeneous among the patients, the findings in the mild phenotype were much more variable, leaving the clinical picture poorly defined. We were able to collect the clinical, biochemical and neuroimaging data on an additional 8 patients with D-2-hydroxyglutaric aciduria, 4 with the severe and 4 with the mild phenotype. With the new information, it becomes clear that the mild phenotype shares the essential characteristics of the severe phenotype. The most frequent findings, regardless of the clinical phenotype, are epilepsy, hypotonia and psychomotor retardation. Additional findings, mainly occurring in the severe phenotype, are episodic vomiting, cardiomyopathy, inspiratory stridor and apnoeas. The most consistent MRI finding is enlargement of the lateral ventricles, occipital more than frontal. Regardless of the clinical phenotype, early MRI shows in addition subependymal cysts and signs of delayed cerebral maturation. Later MRI may reveal multifocal cerebral white-matter abnormalities. Two patients had vascular abnormalities, but it is as yet unclear whether these are related to D-2-hydroxyglutaric aciduria or are incidental findings.

Brain↗

Isolated neonatal swallowing dysfunction: a case series and review of the literature.

Our purpose was to describe the natural history of isolated neonatal swallowing dysfunction (INSD). Nine infants with INSD are described. Eight presented within two weeks of birth. Symptoms included choking and cyanotic spells with feeds, recurrent aspiration, apnea, stridor, and vomiting. Three had nonspecific neurological abnormalities and were diagnosed later in life with underlying disorders (myotonic dystrophy, CHARGE association, velocardiofacial syndrome). All required tube feeding. Six tolerated nasogastric feedings and received a gastrostomy tube. Three failed nasogastric feeds and required jejunal feedings. The gastrostomy tube was removed in 7/9 at a mean age of 37 +/- 9 months. In conclusion, INSD has a good long-term prognosis. The presence of minor neurological abnormalities at presentation suggests another underlying disorder. Nasogastric feeding followed by a gastrostomy is recommended in those without gastroesophageal reflux. Jejunal feedings are necessary in some. While most improve over time, they may need nutritional support for 3 years or more.

Deglutition Disorders↗

Brainstem in multiple system atrophy: clinicopathological correlations.

1. Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder that manifests with parkinsonism, cerebellar ataxia, and autonomic failure in various combinations. 2. Orthostatic hypotension, neurogenic bladder, laryngeal stridor and sleep apnea, and rapid eye movement (REM) sleep behavior disorder are prominent manifestations of MSA. 3. In MSA, there is severe depletion of catecholaminergic neurons of the C1 and A1 areas in the ventrolateral medulla, and this may contribute to orthostatic hypotension and endocrine disturbances in this disorder, respectively. 4. Loss of corticotrophin-releasing factor (CRF) neurons in the pontine micturition area may contribute to neurogenic bladder dysfunction. 5. Respiratory abnormalities may reflect loss of cholinergic neurons in the arcuate nucleus of the ventral medulla. 6. Loss of cholinergic mesopontine neurons, in the setting of loss of locus ceruleus neurons and preservation of rostral raphe neurons, may contribute to REM sleep abnormalities in MSA.

Autonomic Pathways↗

[Sleep and movement disorders].

The three different states of being (wakefulness, NREM and REM sleep) are associated with profound neurophysiological and neurochemical changes in the brain. These changes explain the existence of movement disorders appearing only or preferentially during sleep, and the effects of sleep on movement disorders. Sleep-related movement disorders are of clinical relevance for multiple reasons: 1) high frequency (e.g. restless legs syndrome (RLS)); 2) diagnostic relevance (e.g. REM sleep behavior disorder (RBD) as first manifestation of Parkinson disorder); 3) diagnostic uncertainty (e.g. parasomnias vs nocturnal epilepsy); 4) association with injuries (e.g. RBD, sleepwalking), sleep disruption/daytime sleepiness (e.g. RLS), and psycho-social burden (e.g. enuresis); 5) requirement of specific treatments (e.g. nocturnal epilepsy, stridor, RBD). This article gives an overview on clinical manifestations, pathophysiology, work-up and treatment of sleep-related movement disorders (e.g. RLS, bruxism), parasomnias (e.g. sleepwalking, RBD), sleep-related epilepsies, and on sleep-associated manifestations of movement disorders (e.g. Parkinson disease, multiple system atrophy).

Adult↗

Anaphylactic reaction to lychee in a 12-year-old girl: cross-reactivity to latex?

There are very few case reports on allergic reactions to lychee in the literature - so far only in adults. We report on a 12-year-old girl who developed swelling of lips, pruritus, generalized urticaria and dyspnea 30 min after eating a raw lychee. A second event occurred after eating a piece of cake covered with a fruit cocktail. All other foods were well tolerated. In infancy the girl had suffered from atopic dermatitis, which disappeared in childhood; for the previous 2 yr she had presented with seasonal allergic rhinoconjunctivitis. Upon oral provocation, she developed restlessness, flush, generalized urticaria and inspiratory stridor 50 min after eating half a lychee. The diagnostic work up showed a clear positive skin prick test to raw lychee and specific immunoglobulin E (IgE) in serum to latex but not to lychee. In the cellular antigen stimulation test (CAST) carried out with lychee extracts in several concentrations, the same positive results could be found confirming an allergic reaction. Cross-reactivity of lychee to latex was shown by inhibition experiments using the UniCAP 100-system. In conclusion, it seems worthwhile considering the rare allergy to lychee in the case of unclear food-allergic reactions and lychee should be added to the list of foods cross-reacting with latex.

Anaphylaxis↗

Vocal cord paralysis as a consequence of peritonsillar infiltration with bupivacaine.

Reduction of postoperative pain is an important goal in the perioperative management of tonsillectomy patients. This is particularly the case for children, who often exhibit resistance to intramuscular or rectal administration of drugs. Peritonsillar bupivacaine infiltration, a relatively safe method of pain control, is in some centers frequently used by otolaryngologists for pain relief. We present the case of a 5-year-old girl who developed bilateral vocal cord paralysis following preoperative peritonsillar bupivacaine infiltration. After an uneventful tonsillectomy and extubation, stridor and respiratory distress developed. Bilateral vocal cord paralysis was seen on laryngoscopy. The patient was reintubated and five hours later was successfully extubated without further sequelae. Anesthesiologists and surgeons should be aware of this uncommon complication than can occur with the use of peritonsillar bupivacaine infiltration for pain control in tonsil surgery.

Anesthetics, Local↗

Pulmonary function pre- and post-total laryngectomy.

Pulmonary function pre- and post-total laryngectomy was assessed in 16 patients without overt inspiratory stridor preoperatively. It could be shown that total laryngectomy is associated with a significant decrease in vital capacity and in an increase in inspiratory flow-volume values 9 days postoperatively. The inspiratory values tended to increase slightly over the next half year. The measured expiratory flow-volume values were preoperatively already significantly lower than the predicted values, and no significant changes could be observed immediately after the operation nor after 6 months. These alterations have to be taken into account when judging the pulmonary function after total laryngectomy.

Adult↗

Chondronecrosis of the cricoid cartilage after intubation. Two case reports.

Chondronecrosis of the cricoid cartilage is a rare complication of intubation. The records of two children were reviewed. An 8-month-old girl with myelomonocytic leukaemia developed chondronecrosis 10 days after a 2-day period of ventilation. A 4-year-old girl comatose after poisoning by the histamine antagonist, alimemazine, developed chondronecrosis after a 2-day period of intubation. The complication was suspected when extubation led to dyspnoea owing to laryngeal stridor and was confirmed by direct laryngoscopy. We review the development of the condition, the causative factors, treatment and prevention.

Antipruritics↗

Effectiveness of a croup clinical pathway in the management of children with croup presenting to an emergency department.

OBJECTIVE: The aim of this study was to evaluate the safety and effectiveness of a clinical pathway for croup in an emergency department (ED). METHODOLOGY: This before-and-after intervention study on all consecutive children aged 6 months to 10 years who presented to our ED with moderate/severe croup was conducted over a 6-month period. Children with a clinical croup score (CSS) of 2 or more and resting stridor were considered eligible for entry into the study. Children were treated with either oral dexamethasone, or a combination of oral dexamethasone suspension and nebulized adrenaline. Children were clinically assessed, observed in the emergency short-stay ward and discharged or admitted according to the clinical pathway. The following outcomes were measured: admission rates, hospital re-presentation, length of stay, and adverse clinical events. Children in the post-intervention group were followed up by telephone within 48 h of discharge. RESULTS: There were 157 patients recruited in the pre-intervention group and 110 in the post-intervention group. Significant reductions were reported in the length of stay (18.9 h compared with 5.2 h), hospital admission (52.9% compared with 18.0%) and intensive care admission (10.2% compared with 0.0%) after the introduction of the croup clinical pathway. No children in the study experienced an adverse clinical event. Follow-up interviews of parents indicated that the new treatment strategy was well received. CONCLUSION: The use of the croup clinical pathway in the ED is safe and effective in guiding consistent management, resulting in reduced admission rates, earlier discharge home, and no reported adverse events.

Anti-Inflammatory Agents↗

Flexible endoscopy of aerodigestive tract in small infants.

BACKGROUND: Flexible endoscopy (FE) is a useful method for diagnosing airway problems. Congenital or acquired airway lesions in infants may lead to respiratory distress that requires comprehensive investigation and management. This study was designed to evaluate the use of FE in small infants. METHODS: Infants who had symptoms of respiratory distress and received FE when they were less than 1-year-old were studied and their medical history, diagnoses, interventions, and complications from FE were investigated. RESULTS: The study population consisted of 568 small infants (334 boys and 234 girls) who weighed 5.1 +/- 2.4 kg, and received FE when they were 4.5 +/- 3.6 months of age. Most patients (91.2%) received diagnostic FE and the remainder (8.8%) received therapeutic procedures. Stridor (38.0%) was the most common indication for FE and laryngomalacia (33.3%) was the most frequent finding. Synchronous FE diagnosis was found in 351 (61.8%) cases. No major complications associated with FE were found. CONCLUSION: Flexible endoscopy allows direct visualization of dynamic motion of the small aerodigestive tract. Laryngomalacia was the most common FE finding of respiratory distress in small infants. Synchronous FE lesions were frequently found in this young age group and it necessitated a thorough investigation of the entire aerodigestive tract.

Endoscopy↗

How to enter the pediatric airway for bronchoscopy.

BACKGROUND: Bronchoscopies are performed in childhood for diagnostic reasons (e.g. evaluation of stridor, unexplained cough, possible malformations) and therapeutic reasons (e.g. foreign body removal, management of the difficult airway). METHODS: Various procedures of entering the pediatric airways are presented, based on an overview of the literature and the experience of the authors. RESULTS: The advantages and disadvantages of direct fibreoptic bronchoscopy, bronchoscopy via face mask, via laryngeal mask airway, via tracheal tube, and for combined flexible and rigid bronchoscopy are discussed. In addition, practical aspects of bronchoscopy are considered, including local anesthesia, oxygen supplementation, monitoring, antibiotic treatment. CONCLUSIONS: Although inspection of the pediatric airways has become a well-accepted routine procedure with a high diagnostic yield, and bronchoscopies are well tolerated, it is important that the most appropriate means of access to the airways is chosen according to the indications and the age of the child.

Age Factors↗

Laryngotracheal stenosis in children after intubation. Report of five cases.

Acquired laryngotracheal stenosis is a serious long term complication of tracheal intubation with an incidence of 0.7 to 8% in intubated children. We report five cases of subglottic stenosis over a 4.5-year period and review the pathology and pathogenesis of the condition. A diagnosis of subglottic stenosis was suspected when extubation failed due to the onset of dyspnoea or laryngeal stridor. An incidence of 0.9% of all intubated children was observed. We conclude that the main components contributing to stenosis are pathologies with decreased mucosal capillary perfusion pressure and intubation conditions.

Child, Preschool↗