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Lipoblastoma of the neck: a rare cause of respiratory problems in children.

Lipoblastomatous tumours are rare, and they occur primarily in children younger than 3 years of age. They are benign and may be divided in lipoblastomas and lipoblastomatosis. A case with cervical lipoblastoma causing respiratory difficulty is reported, and a clinical characterisation of patients with lipoblastomatous tumours in the neck is presented. A 6-year-old boy with complains of stridorous respiration and significant reduction in physical capacity was referred to the ENT Department, Odense University Hospital, Denmark. He was treated with total surgical resection of a soft and slowly growing tumour in the left side of the neck, extending from the base of the skull to the upper part of the mediastinum. The histological examination showed a lipoblastoma. After surgery all symptoms disappeared, and the patient was without any operative sequelae. Including the actual case, a review of English literature resulted in the identification of 37 patients with cervical lipoblastoma or lipoblastomatosis. However, in most cases the information was sparse, and only 13 patients were eligible for an analysis of basic clinical and demographic data. The median age was 25 months (range: 7-75 months), and the median tumour size 9 cm (range: 3-18 cm). No difference in sex distribution was found. Lipoblastomas (85%) seemed more frequent than lipoblastomatosis (15%). Stridorous respiration was present in 31%. It is concluded that a considerable part of lipoblastomatous tumours in the neck are combined with respiratory difficulties (31%), and that complete but gentle surgical treatment in most cases will restore normal physiological conditions.

Child↗

Laryngeal dystonia in xeroderma pigmentosum.

We report on three patients with xeroderma pigmentosum group A (XPA) who showed laryngeal stridor in their 20s. The stridor appeared on feeding and emotional excitation, was exaggerated during respiratory infection and was life-threatening on some occasions. Adduction of the vocal cords during inspiration, observed by laryngoscopy, confirmed laryngeal dystonia in all cases. This type of focal dystonia may be characteristic in XPA and requires special attention during the management of these patients to avoid serious complications.

Adult↗

Pierre Robin sequence and double aortic arch: a case report.

We present an 8-day-old female with two admissions for respiratory failure. On the first admission, the diagnosis of Pierre Robin sequence (PRS) and laryngomalacia was made after assessment with chest radiography, echocardiography, and flexible fiberoptic laryngoscopy. Four days after discharge, the child presented with stridor and respiratory distress, and a new cardiac murmur was noted after admission. Repeat echocardiography, with confirmatory direct laryngobronchoscopy, revealed a double aortic arch (DAA) with distal tracheal compression. This case illustrates the necessity of a complete otolaryngic evaluation, including direct laryngobronchoscopy, to search for a synchronous airway lesion in any neonate with severe respiratory distress associated with stridor.

Aorta, Thoracic↗

Laryngeal inflammatory pseudotumour: an unusual cause of airway obstruction.

We report a rare case of a laryngeal pseudotumour in a child presenting with stridor, which was treated by endoscopic laryngeal resection, without the need for a tracheostomy. A short review of the literature is also presented. Although rare, laryngeal pseudotumour should be considered as part of the differential diagnosis of stridor and upper airway obstruction in children.

Airway Obstruction↗

Airway emergency in tuberculosis.

We report a case of acute fatal stridor in a patient newly diagnosed with pulmonary tuberculosis and human immunodeficiency virus (HIV) infection. No evidence of direct airway encroachment was identified at autopsy. We review mechanisms by which tuberculosis may cause stridor and discuss the implications of co-existent HIV infection with reference to the recent literature. The report highlights the need for recognition of acute or evolving airway compromise as an uncommon manifestation of tuberculosis.

Adult↗

Fetal tracheal reconstruction with cartilaginous grafts engineered from mesenchymal amniocytes.

BACKGROUND/PURPOSE: This study was aimed at determining whether cartilaginous grafts engineered from mesenchymal cells normally present in the amniotic fluid could be used in fetal tracheal repair. METHODS: Ovine mesenchymal amniocytes were expanded in culture, labeled with green fluorescent protein, and seeded onto biodegradable scaffold tubes maintained in chondrogenic medium. After chondrogenic differentiation of the constructs was confirmed, they were used to repair either partial or full circumferential tracheal defects in allogeneic fetal lambs (n = 7). Newborns were evaluated for signs of airway compromise. Implants were harvested over a 10-day period postnatally for multiple analyses. RESULTS: All 5 lambs that survived to term were able to breathe spontaneously at birth, 4 (80%) of them without stridor. However, variable degrees of stridor developed over time in all but one animal. Mild-to-moderate tracheal stenosis was present in all specimens. Histologically, grafts contained green fluorescent protein-positive cells, were lined with pseudostratified columnar epithelium, and remodeled into a predominantly fibrous cartilage pattern. Implants showed no significant changes in glycosaminoglycans, collagen, and elastin content at harvest. CONCLUSIONS: Engineered cartilaginous grafts derived from mesenchymal amniocytes may become a viable alternative for tracheal repair. The amniotic fluid can be a practical cell source for engineered tracheal reconstruction.

Amniotic Fluid↗

Juvenile laryngeal papillomatosis.

Always ask about hoarseness and quality of voice in a history of any child presenting with cough or asthma-like symptoms. Children presenting with what appears to be an acute onset of hoarseness, without any physical signs of airways obstruction, should be reviewed after two weeks. If there is chronic hoarseness, referral to an ENT specialist should be considered with a view to laryngoscopy. If the child develops clinical signs of acute airway obstruction such as stridor or respiratory distress, prompt paediatric review is indicated. When referring, it is important to emphasise whether or not there is chronic hoarseness in order to differentiate the diagnosis from croup. Juvenile Laryngeal Papillomatosis may present with cough, pneumonia, dysphagia, or stridor, as well as hoarseness. These patients are often misdiagnosed as having asthma or allergies.

Airway Obstruction↗

Life-threatening vocal cord paralysis in a patient with group A xeroderma pigmentosum.

We report a 19-year-old male with group A xeroderma pigmentosum who presented life-threatening vocal cord paralysis. At 3 months of age, he became sensitive to sunlight, and at the age of 4 years he was diagnosed with group A xeroderma pigmentosum. The neurologic symptoms progressed slowly thereafter. From the age of 18 years, he reported the development of occasional episodic inspiratory stridor and dyspnea, but the cause remained unknown. At the age of 19, he had a common cold and became severely dyspneic and cyanotic. Immediate examination of the glottis upon arrival by an otorhinolaryngologist using a fibroscope indicated complete paralysis of both vocal cords, and tracheal intubation resulted in marked improvement of respiration. Tracheostomy was performed thereafter. Inspiratory stridor and dyspnea are the common symptoms in this disease, and some patients with group A xeroderma pigmentosum undergo a tracheostomy, but the pathogenesis remains unknown. To our knowledge, vocal cord paralysis has never been reported in patients with group A xeroderma pigmentosum. This case is presented to illustrate the importance of fibroscopy in the examination of vocal cords in patients with group A xeroderma pigmentosum before the development of life-threatening events.

Adult↗

[Severe laryngomalacia: surgical indications and results in 33 patients].

INTRODUCTION: Laryngomalacia is the most common course of stridor in children: with a 50% to 75% incidence. About 50% to 60% of congenital laryngeal anomalies that present with stridor are due to laryngomalacia. In most cases, the disease followed a benign course but the prognosis is less favorable in 10% to 15% of cases. These patients may require surgical intervention. MATERIALS AND METHODS: This prospective study included 33 patients referred to our institution from May 1998 to May 2003 for severe laryngomalacia. The diagnosis of severe laryngomalacia was based on clinical and if necessary paraclinical data. An endoscopic laser resection of arytenoid mucosal excess associated if necessary with suprahyoid epiglottectomy was performed in all patients. RESULTS: Mean age of the children was 7.5 Months (range, 2 weeks-4 Years). Ninety-six percent of the patients had complete resolution of symptoms before the fourth postoperative week. Ninety one percents of the patients had effective oral feeding within the first Month (48% immediately after surgery). The average hospital stay was 6 days (range, 3 to 14). Weight gains were found to be satisfactory in all cases since children were discharged the hospital. CONCLUSION: Endoscopic laryngeal surgery is an appropriate therapy for treatment of severe forms of laryngomalacia. It is a safe and effective surgical procedure.

Child, Preschool↗

Substernal goiter.

The literature on substernal goiter from the seventeenth century to the present is reviewed. Substernal goiter may be defined as any thyroid enlargement that has its greater mass inferior to the thoracic inlet. Truly ectopic mediastinal goiters are rare, and most substernal goiters arise from and maintain some attachment to the cervical thyroid gland. Patients are generally in the fifth decade of life, and women predominate. Most patients experience dyspnea, stridor, or dysphagia, but 15 to 50% are asymptomatic; symptoms are often positional, and acute stridor may occur. Ten to twenty percent have no cervical mass or tracheal deviation on examination, and virtually all patients are euthyroid. Standard chest roentgenograms are often diagnostic, but computed tomographic or radioactive iodine scans may be helpful. The presence of a substernal goiter in all but the highest-risk patients is an indication for resection, usually through a cervical collar incision; an occasional patient will require sternotomy or thoracotomy. Death or major complications should be rare postoperatively. Substernal goiters are adenomatous and benign, but carcinoma occurs in 2 to 3% and may be occult. Patients should be followed closely, as these goiters may recur.

Adolescent↗

Vascular rings and slings: long-term follow-up of pulmonary function.

Between 1968 and 1983, 54 patients underwent surgery for symptomatic aortic arch and pulmonary artery anomalies at St Christopher's Hospital for Children. Presenting symptoms included stridor, wheeze, apnea, recurrent pulmonary infections, or dysphagia. Diagnosis was established with chest roentgenogram, bronchoscopy, barium esophagram, and arteriography. Four types of vascular anomalies were encountered; double aortic arch (24 patients), right aortic arch with left ligamentum arteriosum (17 patients), anomalous innominate artery (10 patients), and pulmonary artery sling (three patients). There were no intraoperative deaths and only one postoperative death. All surviving patients had immediate relief of their severe respiratory or swallowing symptoms. Mild respiratory symptoms persisted postoperatively from 3 months to 4 years and included frequent or severe upper respiratory infections, persistent cough, stridor and pneumonia. Five of the 53 surviving patients were lost to follow up. The remaining 48 patients were followed from 6 months to 14 years and all but one patient noted complete resolution of all respiratory symptoms. Twenty-nine patients at follow up were old enough to undergo pulmonary function testing including vital capacity, functional residual capacity, and inspiratory and expiratory flow volume loops. Seventeen of these 29 asymptomatic patients consented to these studies, and nine of these patients had abnormal flow volume loops indicative of significant central airway obstruction; the other eight studies were normal. We conclude that surgical repair for vascular rings and slings is safe and symptomatically efficacious. However, anatomic tracheal or bronchial distortion persists in a significant number of these patients as evaluated by pulmonary function studies.

Adolescent↗

Factors influencing acquired subgottic stenosis in infants.

A prospective study of factors that might contribute to the development of acquired subglottic stenosis was undertaken in newborn infants with endotracheal tubes in place for 7 days or more. Duration of intubation, the number of endotracheal tubes inserted, the duration of mechanical ventilation, the presence of post-extubation stridor, and the size of the endotracheal tube in relation to gestational age significantly correlated with the development of subglottic stenosis. Patients at risk for significant subglottic stenosis were those with post-extubation stridor and those with tubes in place for 25 days or longer. On the basis of these findings, it is recommended that endotracheal tubes be chosen such that the ratio of nominal tube size divided by the patient's gestational age in weeks is less than 0.1.

Birth Weight↗

Management of Chiari II complications in infants with myelomeningocele.

Vocal cord paralysis with inspiratory stridor during infancy is a well-recognized complication of the Arnold-Chiari malformation associated with myelomeningocele. Management of these symptoms, however, remains controversial. The outcome in 19 infants with symptoms managed by us during 1978-1984 was therefore reviewed. Ten infants had stridor alone at presentation (clinical grade 1), four infants also had apnea (grade 2), and five infants also had associated cyanotic spells and dysphagia (grade 3). Of 14 ventricular shunt revisions performed, seven were associated with resolution: in five of eight infants with grade 1, two of four with grade 2, and none of two with grade 3 symptoms. Of the 10 posterior fossa decompressions performed, two were associated with clinical resolution: in one of four infants with grade 1, one of two with grade 2, and none of four with grade 3 symptoms. Mortality over 6 months following onset of symptoms was absent among infants with grade 1, one with grade 2, and three with grade 3 symptoms. We propose that differences in outcome among the three groups of infants reflect differences in pathologic processes within the brain stem. We speculate that infants with grade 2 or 3 symptoms have more extensive brain stem damage, such as hemorrhage, infarction, and necrosis. Use of our classification system may be helpful in further studies on intervention modalities in these infants.

Arnold-Chiari Malformation↗

Respiratory distress in a pregnant woman with congenital vocal cord paralysis.

BACKGROUND: Vocal cord paralysis is one of the most common congenital laryngeal lesions. Hoarseness and dysphonia are common in unilateral cases. Stridor and respiratory distress are seen in bilateral cases. CASE: A 33-year-old primigravida with bilateral congenital vocal cord paralysis presented in the third trimester with a 2-week history of bronchitis and progressive shortness of breath. She developed stridor, her respiratory status deteriorated, and she was intubated with difficulty. She went into cardiac arrest, was resuscitated and placed on a ventilator, and a tracheotomy was performed. Her condition improved, she remained stable until 38 weeks, and was delivered by cesarean. CONCLUSION: Congenital vocal cord paralysis may result in life-threatening respiratory distress during pregnancy. Tracheotomy placement may be useful in the management of these patients.

Adult↗

Clinical presentation as a guide to the identification of GERD in children.

OBJECTIVE: to determine if there is a correlation between common otolaryngologic symptoms and presence of gastroesophageal reflux disease (GERD) in children. METHODS: charts of 295 children presenting with suspicion of GERD were reviewed for presenting symptoms including: (1) airway symptoms: stertor, stridor, frequent cough, recurrent croup, wheezing, nasal congestion, obstructive apnea, blue spells, hoarseness, throat clearing; (2) feeding symptoms: wet burps, globus sensation, frequent emesis, dysphagia, choking/gagging, sore throat, halitosis, food refusal, stomach aches, arching, drooling, chest pain, irritability, and failure to thrive. At least one positive test of barium esophagram, gastric scintiscan, pH probe or esophageal biopsy resulted in inclusion in the GERD positive group. RESULTS: 214 children had GERD diagnosed while 81 had no positive tests for GERD. Between the GERD positive and GERD negative groups, the significantly different symptoms were stertor (P=0.040), cyanotic spells (P=0.043), frequent emesis (P=0.007), failure to thrive (P=0.006), and choking/gagging (P=0.044). Three pooled variables were created: airway flow (stertor, stridor, cyanotic spells), airway irritation (frequent cough, recurrent croup, throat clearing), and feeding (dysphagia, failure to thrive, frequent emesis). GERD patients who were 2 years or less were compared to those older than 2 years and all three of these pooled variables were significantly different between these groups (P<0. 001). CONCLUSION: children who present with a certain constellation of airway or feeding symptoms are more likely to have a positive GERD test. Children 2 years old or less are more likely to present with airway symptoms or feeding difficulties while children older than 2 years are more likely to present with airway irritation.

Airway Obstruction↗

Airway obstruction caused by a congenital epiglottic cyst.

Congenital epiglottic cyst is a rare affliction with potential for airway obstruction. A newborn boy was referred to our department for evaluation of respiratory distress and inspiratory stridor 7 h after birth. Through a transnasal fiberoptic laryngoscopy examination, a diagnosis of an obstructive upper laryngeal cyst was made. Immediate endoscopic surgery was performed 20 h after birth to completely remove the lesion. Two days after surgery, the patient resumed normal breathing pattern and showed no further episodes of stridor or airway obstruction.

Airway Obstruction↗

Endoscopic division of the ary-epiglottic folds in severe laryngomalacia.

OBJECTIVE: Laryngomalacia is the commonest cause of congenital stridor. The underlying anatomical abnormality associated with this condition is a prolapse of the supraglottic tissues into the laryngeal inlet during inspiration, and may involve the epiglottis, ary-epiglottic folds and the corniculate mounds of the arytenoids. However, it has been noted that the most consistent structural abnormality seen in these cases is the shortening of the ary-epiglottic folds and marked side to side curling of the epiglottis. We describe the follow-up and outcome of 33 cases treated by the simple division of the ary-epiglottic folds. METHOD: All case notes were reviewed with respect to indications of operation, age of operation, endoscopic findings, operative technique, complications and follow-up until resolution of symptoms. RESULTS: Surgical outcomes could only be ascertained in only 32 patients. Twenty-two cases (68.7%) showed complete resolution of stridor and associated complications of laryngomalacia. In the remaining ten cases who could be followed up (31.2%), seven patients (21.8%) showed partial resolution with no further surgery required, two patients (6.2%) required additional excision of redundant mucosa as second procedure, one patient (3%) with associated cleft lip/palate and tracheomalacia had to undergo a tracheostomy. There was improvement in feeding after surgery in all the 12 patients (100%) who had had pre-operative feeding difficulties. All the four patients with cyanosis pre-operatively were cured, but one of the two patients with apnoeic episodes pre-operatively continued to have apnoeic spells despite resolution of their laryngomalacia. CONCLUSION: Simple endoscopic excision of the ary-epiglottic folds is a quick, reliable, highly effective procedure with very few complications. We recommend its use as the first line option in the management of severe laryngomalacia, with more extensive methods reserved for the very occasional case of primary failure.

Arytenoid Cartilage↗

Laser division of the aryepiglottic folds for severe laryngomalacia.

Laryngomalacia is an entity of ill-defined etiology. The clinical situation is one of inspiratory stridor present at birth, or within the newborn period. The stridor may increase in severity for the first several months of life and thereafter spontaneously begins to resolve. In the majority of cases the condition resolves spontaneously by approximately two years of age. This report will present the surgical management of severe laryngomalacia in infants using the carbon dioxide laser to surgically divide the aryepiglottic fold with resolution of the laryngomalacia.

Epiglottis↗