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Tuberculosis is still a major paediatric problem in many parts of Africa, and the diagnosis may be missed because of the unusual and varied clinical features in some children. This is illustrated by 5 cases which have been described who presented with (a) unilateral swelling of the leg associated with inguinal lymphadenopathy; (b) subcutaneous emphysema; (c) 'cystic lung' disease; (d) respiratory stridor and (e) abnormal behaviour.
Exercise-induced anaphylaxis (EIA) is a unique and an increasingly recognized syndrome consisting of premonitory symptoms and signs of generalized body warmth, pruritus, and erythema, which progresses on continued exertion to confluent urticaria, laryngeal edema with stridor or hoarseness, and gastrointestinal colic and frequently culminates in vascular collapse. Previous studies of five individuals with this condition have demonstrated significant elevations of serum histamine concurrent with the early clinical manifestations after experimental exercise. To assess relevant morphologic alterations in the skin of these patients, cutaneous mast cells were examined by light and transmission electron microscopy before and during the initial erythema elicited by exertion. The marked alterations observed in mast cells immediately after exercise consisted of (1) loss of electron density and internal substructure of granules, (2) fusion of granule membranes with those of adjacent granules and with mast cell membranes creating conduits to the extracellular space, and (3) an apparent decrease in the number of intact granules per cell. Biopsy specimens obtained before exercise from patients with EIA and from two normal individuals who served as control subjects were identical, and the control subjects had normal mast cell morphology after exercise. Serum histamine levels were significantly elevated in patients with EIA after exercise at the time of biopsy, whereas control subjects had normal levels. These observations provide evidence that EIA is a distinct form of physical allergy associated with mast cell degranulation similar in morphology to that of human pulmonary mast cell IgE-Fc-dependent activation secretion. Characterization of this disorder is important because its prevalence may be underestimated, and its clinical consequences, which may include some morbidity, are not fully known.
An 8-month-male infant was admitted to our institute in order to investigate his developmental delay. He had facial features-long palpebral fissures with eversion of the lower lateral eyelids, arched eyebrows with lateral sparseness, depressed nasal tip, large, prominent and cupped ears. From these characteristical features, he was diagnosed as having Kabuki make-up syndrome (KS). When he was 2 months old, he was admitted to our institute because of intractable stridor and liver dysfunction associated with cytomegalovirus (CMV) infection. In KS, increased susceptibility to infection is described. We suspected persistent CMV infection because of an increased susceptibility to infection in KS. Recently acute idiopathic thrombocytopenic purpura (ITP) was diagnosed. According to the correlation between the number of CMV DNA copies and his platelet count, it is speculated that ITP would occur when the number of CMV DNA copies was elevated.
Vocal cord cysts are rare, and typically present with symptoms of dysphonia in older children and adults. There is often a background of voice abuse. We present the first recorded and photographed case of an intracordal cyst presenting in a neonate. The patient presented with symptoms of episodic stridor, respiratory distress, and feeding difficulties, which resolved rapidly after simple incision of the cyst. Theories for the aetiology of intracordal cysts are discussed. The authors believe that this case lends support to the theory that intracordal cysts are congenital in origin.
Congenital vallecular cyst is fairly uncommon in neonates and infants. Although benign in nature, it may cause stridor and even life-threatening airway obstruction in early infancy. A 3-month-old male baby presented with failure to thrive and respiratory distress was found to have a vallecular cyst. Marsupialization with CO(2) laser was performed.
Neurofibromatosis is characterized by multiple café-au-lait spots and cutaneous neurofibromas. Laryngeal involvement in neurofibromatosis is rare and is usually manifested by obstructive airway symptoms. We encountered a 1 year 8-month-old girl who was a victim of neurofibromatosis with the presentation of stridor. Fiberoptic laryngoscopy revealed a round smooth mass over the right aryepiglottic fold which prolapsed into the larynx during inspiration. Carbon dioxide laser excision was performed and histopathologic examination revealed a plexiform neurofibroma. The patient had regular follow up for almost 4 years. Even though there seemed to be a recurrence, she remained symptom free.
Hereditary vocal cord palsy is an uncommon cause of neonatal stridor that has significant implications for patients, their families, and their future progeny.
Exercise-induced laryngomalacia (EIL) is characterized by inspiratory stridor that is brought on by exercise (i.e. competitive sports) and fails to respond to treatment with bronchodilators (Smith et al., Ann Otol Rhinol Laryngol 1995;104:537-541). During exercise, (1) spirometric flow volume loops show evidence of variable extrathoracic obstruction, and (2) laryngoscopy shows inspiratory prolapse of supraglottic structures with partial glottic obstruction. Only five cases of probable EIL have been reported in the literature (Smith et al., Ann Otol Rhinol Laryngol 1995;104:537-541; Lakin et al., Chest 1984;86:499-501; Bittleman et al., Chest 1994;106:615-616; Bent et al., Ann Otol Rhinol Laryngol 1996;105:169-175; Chemery et al., Rev Mal Respir 2002;19:641-643). Here, a case of laryngomalacia induced by exercise in a previously asymptomatic 10-year-old athlete with a remote history of laryngomalacia in infancy is presented, and a review of previously reported cases is provided.
The case history is presented of a 29-month-old boy with the characteristic skin lesions of epidermolysis bullosa letalis, requiring steroid therapy. After an episode of gastrointestinal haemorrhage, probably caused by the steroids, the child experienced progressive stridor and respiratory failure and died 10 days later. The postmortem findings in the larynx were of marked mucosal inflammation and cystic swelling of the serumucinous glands producing severe narrowing of the supraglottic airway. The laryngeal cysts were thus entirely different in nature to the cutaneous bullae.
A saccular or congenital cyst of the larynx is a mucus-filled dilatation of the laryngeal saccule and can distort the aryepiglottic fold, the false cord or the laryngeal ventricle. The most common symptom of the laryngeal cyst is respiratory distress with inspiratory stridor and the diagnosis can usually be made by laryngoscopy. Treatment includes incision and drainage together with extirpation of the cranial wall of the cyst through a laryngoscope. Sometimes tracheostomy is also needed. Usually there are no complications afterwards and the development of these infants is perfectly normal. Our 3 cases and clinical features of this very unusual disorder are presented.
In the diagnosis of esophageal foreign bodies in children, stridor and dyspnea as well as dysphagia are important. The history is often negative. Long-standing esophageal foreign bodies often present as a neck mass and develop Zenker-type diverticula in the cricopharyngeal sphincteric area. CT scanning has proven to be highly useful in delineating radio-transparent foreign bodies and their precise location in this clinical situation. Two cases with dysphagia and three cases with respiratory distress are presented and discussed.
Mechanical ventilation of the newborn is now widely used in neonatal intensive care. The oro-tracheal route of intubation is simpler, but for long-term ventilation has been considered unstable. A method of fixation of oro-tracheal tubes is described which overcomes this instability. Five hundred consecutive ventilated infants were intubated by the oro-tracheal route and the tube was fixed by the method described. Of the 500 ventilated infants, 213 died without being extubated. Of the 287 survivors, 44 developed a degree of post-extubation stridor. No surviving infant developed clinical evidence of subglottic stenosis and in almost 200 postmortem examinations laryngeal narrowing was not identified. The method of oro-tracheal fixation described is stable and may reduce the incidence of subglottic stenosis.
Patients with Hunter syndrome may have symptoms of hoarseness, stridor and breathing difficulties as a result of laryngeal and tracheal involvement. In planning their evaluation, we must prefer non-invasive methods as X-ray or CT scan, and avoid doing endotracheal intubation or bronchoscopies. Review of adult cases in the literature and description of the only case of a child with Hunter syndrome having life-threatening complications of his upper airways is discussed in this report. In this case and in the literature we cannot exclude intubation or bronchoscopy as a serious aggravating factor, causing further narrowing of the larynx.
INTRODUCTION: Acute epiglottitis (AE) continues to cause life-threatening airway obstruction in children. The aim of this retrospective study was to evaluate deficiencies in the management of AE, to clarify the role of airway endoscopy, and to establish the cause of death in this disease. MATERIAL: The subjects of our retrospective study were 24 children with AE treated in the paediatric intensive care unit (PICU) of the University of Cologne between 1980 and 1994. The records of all patients were reviewed. RESULTS: Laryngoscopy with fibreoptic or small rigid endoscopes allowed accurate diagnosis of AE, particularly in patients presented with atypical clinical signs. Furthermore, endoscopic information of the degree of inflammation were helpful in the critical decision, whether artificial airway was required. The number of patients managed without intubation increased (8% vs. 45%) since airway endoscopy became available in 1989. No evidence of beta-lactamase-producing strains of Haemophilus influenzae was observed. The most remarkable finding was the high mortality in AE (12.5%). Affected children were admitted in poor post-hypoxia conditions following outdoor cardiorespiratory arrest. CONCLUSION: The most decisive factor to decrease mortality seems to be timely appropriate presentation at referring centres, if AE is suspected or stridor remains unclear. Fibreoptic airway endoscopy represents a major step forward in the management of acute epiglottitis, and this requires close interdisciplinary collaboration between paediatricians, anaesthetists and otorhinolaryngologists.
The laryngeal mask airway (LMA) was first described in 1983, and has since been used for administration of general anesthesia, resuscitation and as an aid to bronchoscopy or intubation. When used for anesthesia, the LMA has the advantage of lessening potential subglottic injury as compared to an endotracheal tube. Children with mild subglottic stenosis often have minimal symptoms of airway compromise at baseline. However, a minor degree of subglottic irritation may result in dyspnea (as in upper airway infection or following laryngeal intubation). We have begun using the LMA in such patients undergoing a general anesthetic for head and neck procedures unrelated to the airway. The present report describes two such children who underwent rhinologic and ophthalmologic surgery using the LMA. No perioperative stridor or respiratory distress was noted, and the LMA did not interfere with the procedure. In addition, we describe a modification of the standard LMA to further facilitate surgical access to the head and neck.
We reviewed the 9 year experience at the Children's Hospital of Philadelphia with patients requiring tracheotomy for a diagnosis of congenital airway abnormalities. Of the 56 patients, 28 (50%) had cardiovascular, or chromosomal abnormalities, neurologic conditions, or congenital syndromes, 24 (43%) were born prematurely, and 13 (23%) were found to have gastroesophageal reflux. Only 18 (32%) went on to eventual decannulation of their tracheotomy with a mean tracheotomy duration of 1.75 years. The majority of patients (75%) had multiple presenting signs. Stridor was the most common (54%), followed by accessory respiratory effort (39%), cyanosis (30%), apnea (29%), and failure to thrive (23%). Twenty eight patients (50%) had multiple airway abnormalities contributing to their need of a tracheotomy for airway protection orr ventilator dependence. Laryngeal abnormalities were found in 71% of patients, tracheal abnormalities in 48% of patients, bronchial abnormalities in 11%, and upper airway obstruction in 14%. Of the laryngeal abnormalities, laryngomalacia was the most common, followed by subglottic stenosis, glottic web, and vocal cord paralysis. Tracheomalacia was the most common tracheal abnormality. The relatively large percentage of patients with cardiovascular or other major malformations, and prematurity, accounts for comorbid factors in the need for prolonged tracheotomy (and low early decannulation rate). Although gastroesophageal reflux was found in a recognizable portion off the patients, it is unclear whether this represents a comorbid condition.
In children with stridor, a detailed evaluation of the airway is often required to assess precisely its anatomical and functional status. Various methods of assessment have been developed and airway management may include, as well as rigid and flexible endoscopy, the use of imaging techniques such as plain X-rays, a barium oesophagogram, ultrasound, a CT scan, a magnetic resonance image (MRI) and an angiogram, as well as respiratory function tests including acoustic rhinometry and flow volume loops or even pH monitoring. This article aims to highlight the valuable information these alternative techniques can provide.
Primary malignant tumours of the trachea are extremely rare in infants. This report describes an 11-month-old boy suffering from severe dyspnea with a 5-month history of stridor and 'bronchitis'. A hard mass could be palpated below the right lobe of the thyroid gland. Roentgenograms and endoscopy showed an exophytic tumour filling more than 80% of the tracheal lumen. A tracheal resection and a subtotal thyroidectomy with primary anastomosis was performed. An invasive squamous cell carcinoma of the trachea was diagnosed. This is the first reported case in an infant in the English literature. A local recurrence was found on MRI 19 weeks later. The infant died at 16 months of age.