Multidisciplinary management of children with craniofacial syndromes with particular reference to the airway.
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Biomedical subjects
Publications and source records attributed to K Pearman.
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Recurrent respiratory papillomatosis is a relatively rare disease caused by members of the human papilloma virus family. Lesions may occur anywhere throughout the respiratory tract but show a predilection for squamo-columnar epithelial junctions, frequently leading to hoarseness and upper airway obstruction. Rarely, it can progress to squamous cell carcinoma. The impact of recurrent respiratory papillomatosis on patients, their families, and the health care system is considerable. Unfortunately, despite extensive investigational studies, no cure is available for the disease. This article reviews the aetiology of and therapeutic options for recurrent respiratory papillomatosis.
A 3-yr-old girl developed severe progressive juvenile laryngeal papillomatosis (JLP) 2 yr after liver transplantation (Tx) for biliary atresia. The papillomata were resistant to withdrawal of immunosuppression, to laser surgery, and to subcutaneous interferon (3 MU/m2, three times weekly), necessitating tracheostomy. Oral ribavirin therapy (25 mg/kg/day) in combination with no immunosuppression effectively prevented the rapid recurrence of JLP over 5 yr. Hence, oral ribavirin is a potentially useful therapy in this life-threatening situation and may also be of benefit in other children with severe JLP.
A rare presentation of an antrochoanal polyp is reported. A 14-year-old boy presented with obstructive sleep apnoea and subnormal growth velocity for height and weight over a 1-year period. Examination revealed a post-nasal mass which following removal was confirmed histopathologically as an antrochoanal polyp. Relief of the airway obstruction was promptly followed by catch-up growth and subsequent normal growth velocities. The possible mechanisms underlying the cachexia are explored including the possible association with the obstructive sleep apnoea.
Numerous factors contribute towards a late diagnosis of laryngeal malignancy in childhood. These include its rarity, the similarity of its early symptoms to those of other benign, common childhood conditions as well as the relative difficulty encountered during paediatric laryngeal examination. We believe that these cases are of sufficient interest when they occur to warrant reporting since the consequences of late diagnosis in these young patients can be serious. We present a case of an 11-year-old boy with advanced squamous cell carcinoma of the larynx (T3N0M0), who was successfully treated with primary total laryngectomy and bilateral selective neck dissections to avoid the potential additional morbidity of radical radiotherapy.
OBJECTIVE: To describe the histopathological changes of the mucosa in laryngomalacia; look for any relationship with gastro-oesophageal reflux and to describe the histological changes of reflux laryngitis in laryngomalacia. METHODS: We examined serial histological sections from nine cases of laryngomalacia, who had aryepiglottoplasty and compared the histopathological features with five cases of postintubation inflammatory laryngitis and five age-matched autopsy specimens of normal larynx. RESULTS: Five of the cases of laryngomalacia had mild inflammation in the form of basal cell hyperplasia and chronic inflammation close to the basement membrane. Deeper subepithelium was oedematous. Two cases had moderate and two cases severe inflammation. The latter showed ulceration and a dense band of chronic inflammation in the immediate subepithelium with underlying oedema. Three of the cases had gastro-oesophageal reflux proven by barium swallow. Two of these showed intraepithelial eosinophils. CONCLUSIONS: A band of inflammation of variable intensity just beneath the epithelium with oedema deep to it is the most important histological feature of laryngomalacia. The presence of intraepithelial eosinophils appears to be a histological indication for reflux aetiology of the inflammation.
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We report a child of 3 years 9 months with the Marshall-Smith syndrome (MSS), characterised by the typical facial features, developmental delay, and advanced bone age. After the diagnosis was made at 5 months of age, careful observation for respiratory complications and failure to thrive was initiated. By 3 1/2 years of age, although our patient had no life threatening respiratory complications, investigation showed significant upper airway obstruction, which has been successfully treated. Aggressive treatment for failure to thrive has also allowed her to maintain a weight on the 50th centile. The purpose of this report is to suggest that early diagnosis and aggressive management may improve the ultimate prognosis with respect to the respiratory and feeding difficulties seen in this rare syndrome.
We describe a patient who developed upper respiratory tract symptoms following long-term treatment of Wilson's disease with penicillamine. These symptoms were attributed to areas of pharyngeal thickening and were treated with a laser. Histological examination of the lesions showed proliferations of abnormal elastic fibres similar to those previously described at other sites, especially the skin, in patients receiving penicillamine. This drug impairs the maturation and reduces the stability of elastic fibres and although elastic tissue throughout the body is affected, we are aware of no previous reports of penicillamine-induced changes presenting with upper respiratory tract symptoms.
A detailed analysis was made of nasal capnograms obtained from sleeping children with differing degrees of sleep-induced respiratory obstruction, the severe form being known as obstructive sleep apnoea. Clinical observations of these children were compared with analyses of nasal capnography data. During periods when the degree of obstruction was consistent throughout the first hour of sleep, the means and coefficients of variation of several parameters, derived from the capnograms were calculated. The coefficients of variation showed a close relationship to the degree of obstruction. In comparison, measures of end-tidal carbon dioxide concentration proved to be a very insensitive index of the degree of obstruction. The conclusion is that the usefulness of nasal capnography in predicting and grading obstruction can easily be extended by the use of statistical measures of dispersion and that this concept warrants further work.
A case of angiofibroma affecting the right maxilla of a new born baby is presented with histological evidence. We believe this to be the first congenital case of this condition to have been reported.
Twelve recordings of overnight pulse oximetry representing a range of severity of obstructive sleep disturbance were evaluated by 7 experienced clinicians. Each recording was then scored by counting the number of minutes in each hour affected at any time by a fall in saturation to below 90%. Comparison of the clinical evaluation and the overall scores gave a clear level of 5 minutes per hour at and above which all the assessors agreed that the charts had clinically important desaturation. Overnight pulse oximetry was then performed on 25 children aged between 12 months and 10 years about to undergo adenotonsillectomy. The charts were scored by the method above with a score of 5 or more indicating a positive result for hypoxic episodes. The scores for shortened analysis periods of 1, 2, 3 and 4 hours duration were compared with the overall score and no cases which were negative, up to and including 4 hours, became positive in the overall result. Thus recording pulse oximetry for 4 hours provides sufficiently accurate information for clinical purposes.
A randomised clinical trial of antiseptic nasal carrier cream (Naseptin) and silver nitrate cautery in the treatment of epistaxis in children was carried out. Fifty-four percent responded to cautery with silver nitrate and 50% to treatment with Naseptin antiseptic carrier cream. There were 24 patients in each group. No statistically significant difference was observed between the groups when tested with the chi 2 test. We believe that the first line treatment of epistaxis in children should be the use of antiseptic nasal cream.
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Many infants with tracheotomies remain cannulated for prolonged periods while the underlying cause of airway obstruction is either treated or natural resolution is awaited (usually by growth). To enable these children to enjoy a relatively normal family environment despite a tracheotomy, it is desirable that they should be managed at home for at least part of the time. For the past 8 years we have routinely used soft polyvinyl chloride paediatric tracheotomy tubes (Shiley) in our patients. These tubes have proved to be relatively resistant to obstruction with secretions and are changed at 1- to 2-week intervals. They can be modified by making a series of three to four 2-mm through-and-through fenestrations around the shoulder in order to improve speech production and facilitate decannulation. Parents are tutored in tracheotomy care, which includes tube changing, humidification and suction. They are then permitted to take their child home from hospital when they are considered to be competent. Twenty-eight children (13 boys, 15 girls) with a mean age of 14.5 weeks (range 1-525 weeks) at the time of tracheotomy have been managed at home using this system. The median period of hospitalisation was 12 weeks (range 5-75 weeks), and the median duration of home management was 94 weeks (range 13-394 weeks). Sixteen patients have been successfully decannulated, 11 remain cannulated and 1 died at home from sudden infant death syndrome. Despite supportive measures, the majority of the children developed intermittent chest infections.(ABSTRACT TRUNCATED AT 250 WORDS)
Ten to 15% of all salivary gland tumors arise from the minor salivary glands, which are distributed throughout the upper aerodigestive tract. Most of these tumors occur in the oral cavity, and there is a much higher incidence of malignancy than is the case with the major salivary glands. The pathologic appearance, clinical features, treatment, and prognosis of minor salivary gland tumors are discussed. Particular reference is made to the role of adjuvant radiotherapy in improving the control rate after surgical excision.
The anatomy of the submandibular salivary gland and common conditions that cause gland enlargement are described. We also describe a simple technique for removal of a calculus from Wharton's duct and a method for submandibular gland excision.
Salivary disease in childhood is uncommon. Clinical features are much the same as in adult disease, however, congenital, inflammation, and functional disease is more common. Management is governed by the same general principles in all age groups. Congenital abnormalities are rare. Inflammation, either bacterial or viral, is the most common disease. Chronic sialadenitis usually resolves at puberty. Neoplasia is rare, and the majority of tumors are benign and occur in the parotid gland. Malignancy is suggested by rapid growth, pain, skin fixation, and lymphadenopathy. Surgical treatment of sialorrhea should only be considered after careful assessment. Relocation of the submandibular ducts is the treatment of choice.