To the knowledge of the authors, the present report encompasses the largest series of children treated for CA. The high incidence of comorbid conditions mandates thorough medical evaluation, particularly for otological, airway, cardiac, and gastrointestinal tract diseases.

Serum fentanyl concentrations after intranasal administration exceed the minimum effective steady state concentration for analgesia in adults. The use of intranasal fentanyl during halothane or sevoflurane anesthesia for BMT is associated with diminished postoperative agitation without an increase in vomiting, hypoxemia, or discharge times.

Angioedema is an immunologically mediated, anatomically limited, nonpitting edema that can lead to life-threatening airway obstruction. To predict the risk of airway compromise in angioedema, we retrospectively reviewed 93 episodes in 80 patients from 1985 to 1995. Intubation or tracheotomy was necessary in 9 (9.7%) cases. Angiotensin-converting enzyme inhibitor use in 36 cases (39%) was associated with intensive care unit (ICU) admission (P = 0.05). ICU stay correlated significantly with presentation with voice change, hoarseness, dyspnea, and rash (P < 0.05). Voice change, hoarseness, dyspnea, and stridor were present in patients requiring airway intervention (P < 0.05). On the basis of our data, we propose a staging system by which airway risk may be predicted from the anatomic site of presentation. Patients with facial rash, facial edema, lip edema (stage I), and soft palate edema (stage II) were treated as outpatients and on the hospital ward. Patients with lingual edema (stage III) usually required ICU admission. All patients with laryngeal edema (stage IV) were admitted to the ICU. Airway intervention was necessary in 7% of stage III patients and in 24% of stage IV cases. No deaths were caused by angioedema. Airway risk in angioedema may be predicted by anatomic site of presentation, allowing appropriate triage with preparation for airway intervention in selected cases.

Neuro-otologic manifestations of sarcoidosis are rare. Dizziness in particular is a rare presenting complaint of the patient afflicted with this systemic granulomatous disorder. Head and neck and sinonasal presentations of this disease are more common. We reviewed our experience with six such patients who presented for management of their otolaryngologic (ORL) manifestations in order to delineate the involvement of the otolaryngologist in the treatment and diagnosis of these patients, with a focus upon the relevant tests and procedures in the otolaryngologist's de novo diagnosis of sarcoidosis. Studies ordered in the course of otolaryngologic evaluation and their utility in the diagnosis of sarcoidosis by the otolaryngologist are reviewed and classified into useful, supportive, and ancillary. The otolaryngologist played an important role in diagnosis, with four of six patients diagnosed to have sarcoidosis on the basis of their otolaryngologic presentations. Biopsy was performed by the otolaryngologist for diagnosis of sarcoidosis in all four of these cases. Steroids were central to treatment. Oral steroid therapy was the principal treatment: both patients with neuro-otologic sarcoid were successfully managed with oral steroids. Intralesional steroids were necessary to treat the skin lesion. One of six patients patients experienced complications related to steroid use.

Pediatric tracheotomy at our institution is associated with an overall 19.9% incidence of complications. Although the rate of major complications such as accidental decannulation or death is low, rates of peristomal skin breakdown and development of granuloma are more frequently reported and can occur at any point following tracheotomy. Further work is necessary to understand and mitigate wound care issues in post-tracheotomy care.

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Mutations in the Connexin 26 (Cx26) gene have been found to account for approximately 20% of all childhood deafness. This number approaches 50% in documented recessive cases of hearing loss. Two mutations, 35delG and 167delT, account for the majority of reported mutations in this gene, but to date, more than 60 mutations have been described. No other single gene has yet been identified that contributes this significantly to the aetiology of hearing loss. Several mutations in this gene have been found to predominate in specific ethnic populations (167delT in Ashkenazi Jews and 235delC in Japanese individuals). While the majority of mutations found in Cx26 result in frame shifts and premature terminations, a number of missense mutations have also been identified. The V37I missense mutation has been reported as both a polymorphism and as a potentially disease-causing missense mutation. The present authors have identified three unrelated individuals with sensorineural hearing loss who are homozygous for this mutation. One individual is of Philippine ancestry, another is from a Chinese and Cambodian background, while the third is of Chinese ancestry, raising the possibility that this mutation may be more frequent among populations in eastern Asia.