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Vascular endothelial growth factor–A (VEGF-A) is known to play an important role in the angiogenic response essential for tumor growth in a variety of human and experimental tumors. This study was designed to investigate whether VEGF-A may play a role in the pathogenesis of recurrent respiratory papillomatosis (RRP). A retrospective study with institutional review board approval was performed at a tertiary care medical center on 12 patients with a history of laryngeal RRP. Their ages at the time of initial diagnosis ranged from 19 to 96 months (mean, 56 months). All patients had involvement of right and left true vocal cords. All patients required multiple endoscopic procedures (range, 4 to 66; mean, 12). Normal pediatric larynx samples from 5 autopsy patients were used as controls. Formalin-fixed, paraffin-embedded sections of laryngeal squamous papillomas from the 12 patients with a diagnosis of RRP and the 5 control patients were examined by in situ hybridization for the presence of messenger RNA (mRNA) for VEGF-A and vascular endothelial growth factor receptor 1 (VEGFR-1) and vascular endothelial growth factor receptor 2 (VEGFR-2). The biopsy specimens were from the true vocal cord (N = 10) or subglottis (N = 2) in the patients with RRP and consisted of large sections of larynx including the true vocal cord in the control patients (N = 5). Strong expression of VEGF-A mRNA was noted in the squamous epithelium of papillomas of all 12 patients. Strong expression of VEGFR-1 and VEGFR-2 was noted in the endothelial cells of the underlying vessels in all 12 patients. Neither strong labeling of VEGF-A mRNA nor labeling of its receptors was noted in the control patients. We conclude that the angiogenic growth factor VEGF-A is strongly expressed in the epithelium of squamous papillomas in RRP. Also, VEGFR-1 and VEGFR-2 mRNAs are strongly expressed by underlying vascular endothelial cells, suggesting an important role in the pathogenesis of RRP.

Lymphatic malformations (LMs) are uncommon congenital lesions that may occur throughout the body, although the head and neck region is the most common site. Most LMs are seen at birth. However, they may present in adolescence or adulthood, mainly as a result of trauma or infection. We report the case of a 7-year-old boy who presented with an LM of the cervicofacial region causing airway compression. We discuss the causes of delayed presentation of these congenital lesions. An overview of the causation, natural history, diagnosis, and treatment options is presented.

Hemangioma is a well-recognized cause of airway obstruction in the infant with stridor. Corticosteroid and laser therapy are effective in stabilizing the airway in most cases. There are, however, some extensive airway lesions that are not adequately managed by these modalities. This report describes the use of recombinant interferon alfa-2a in 15 patients with life-threatening airway hemangiomas. All patients had failed corticosteroid and/or laser therapy. Multiple upper airway sites were involved, including the base of the tongue, supraglottis, subglottis, trachea, and mediastinum. Eleven patients have completed therapy and are doing well. Four patients have resolving lesions on the drug regimen. Life-threatening airway lesions unresponsive to conventional treatment should be considered for a trial of interferon alfa-2a.

Rhabdomyosarcoma (RMS), the most common malignant soft-tissue tumor in the pediatric population, occurs most often in the head and neck region. Major advancements in the management of these aggressive tumors have been achieved in the last 20 years on three fronts: chemotherapy, radiation therapy, and surgery. Surgery, with the advent of skull-base, craniofacial, and free-tissue-transfer reconstructive techniques, has once again become part of the primary management of these tumors. Prior to 1960, RMS was uniformly fatal; now, with the introduction of multimodality treatment regimens, two thirds of patients can expect long-term survival.

Purpose of review The study of errors in medicine has proliferated in the past few years. This spark was ignited by the Institute of Medicine's report in 2000, titled 'To Err is Human'. Studies of errors have been performed in both medical and surgical specialties. Surgical skill and technology has proliferated over the last century to such an extent that outcomes will probably not improve from focusing finite resources on attempts to enhance surgical skill or developing new instrumentation. Advancing medicine in the 21 Century will probably depend on our ability to address and ameliorate errors and adverse events in healthcare. Directing attention to this previously little studied realm of medicine has the ability to dramatically reduce morbidity and mortality. Small changes have the ability to result in outcomes that are far beyond what is predicted by incremental measures. Recent findings There have been studies identifying and classifying errors in otolaryngology in out-patient and in-hospital settings, addressing allergy sera misadministration, providing quality assurance for pathology reports in head and neck malignancies, implementing computerized patient records, and detailing problems that occur when patients have a test ordered and the results are not followed up. Summary The study of errors in medicine is going to dramatically increase in the next decade. The practicing otolaryngologist must be vigilant for errors, use surgical time-outs, should have a system in place to track tests ordered and results received, and most importantly have the capability to identify and ameliorate errors as they occur on an individual and institutional level.

Benign lesions of the airway in infants and children may endanger the airway and compromise laryngeal function. The CO2 laser provides an elegant method of transoral surgical excision without compromising laryngeal function or airway competence. It avoids the risks of such other treatment modalities as external surgical approaches, prolonged steroid therapy, and radiation. It is applicable to a wide variety of lesions without the necessity of tracheotomy. Hospitalization is minimized. Ten cases are presented to show the wide applicability of this technique. Gross pathology and pre- and postoperative findings are demonstrated and discussed, as is the safety and efficacy of the laser soft-tissue interaction.

Infection of the supraglottic larynx is a well-recognized clinical entity in both adults and children. The anatomical relationships of the various components of the larynx have been studied extensively, especially as they relate to the spread of malignant disease. However, the relationship of these spaces and compartments in inflammatory disease has not been heretofore appreciated. This report describes for the first time histologic evidence that infection of the supraglottic larynx may spread to the paraglottic space, thus causing compromise of the glottic region. This potential carries significant implications for planning the appropriate therapeutic measures.

Congenital subglottic hemangioma is a potentially life-threatening lesion which frequently presents before the age of 6 months. Many forms of therapy have been advocated for the treatment of this difficult lesion. The use of the carbon dioxide laser has been advocated in the past and this report represents an update in treating 31 patients from 1975 to 1983. The success rate was 94 % and the complication rate was 6.5%. This form of therapy is an effective and valuable addition to those already available for this troublesome lesion.

Stenosis of the larynx and/or trachea presents perplexing problems. No one technique has proved totally satisfactory in the management of all varieties of stenosis. Recent reports have described the successful use of the CO2 laser in the endoscopic management of stenosis of the larynx and trachea. Failures of this technique need emphasis to assure appropriate selection of therapeutic method. Retrospectively, 49 cases of laryngeal stenosis, 6 cases of tracheal stenosis and 5 cases of combined laryngeal and tracheal stenosis were studied (total 60 patients) following treatment at the Boston University Affiliated Hospitals. Follow-up ranged from 1 to 8 years. Multiple procedures were required in 35 laryngeal patients. Of the laryngeal patients 39 were successfully managed (average number of procedures in successful cases 2.18). Of 11 tracheal patients with combined laryngeal and tracheal procedures, 3 were successfully managed (average number of procedures in successful cases 6). Failures in laryngeal stenosis included four patients in whom an adequate airway was not established though voice was present while maintaining tracheostomies. Thirteen patients failing endoscopic management required open surgery with good result. Factors associated with poor result or failure include circumferential scarring with cicatricial contracture, scarring wider than 1 cm in vertical dimension, tracheomalacia and loss of cartilage, previous history of severe bacterial infection associated with tracheostomy, and posterior laryngeal inlet scarring with arytenoid fixation. In these circumstances, multiple procedures, more extensive alternative open surgical techniques, or maintenance of tracheostomy were necessary. In successful cases only three or fewer procedures on average were required. The factors associated with failure or success of endoscopic methods in the management of laryngotracheal stenosis, including use of the CO2 laser and soft Silastic stents, are analyzed.

Congenital choanal atresia frequently demands immediate recognition in the newborn, and provision of a nasal airway as soon as possible. A variety of techniques are available to correct this problem, but none is entirely satisfactory. Transpalatal procedures are associated with significant morbidity in the newborn, and transnasal microsurgical procedures are less predictable when a thick bony plate is present in the choanal obstruction. Transnasal resection with the CO2 laser has been carried out in seven patients (ten sides), whose ages ranged from 16 hours to 14 years. From this early experience, it appears that the procedure is entirely feasible and convenient. It seems to have significant advantages over conventional microsurgical techniques. Eight out of ten choanae have remained patent for four months or more. A lumen keeper is needed during the immediate postoperative period for at least two to three weeks. The procedure is associated with minimal morbidity.

Recurrent respiratory papillomatosis is most common in childhood but it affects all age groups; it represents a diathesis of the aerodigestive tract so that lesions may develop at various sites — the nares, lips, pharynx, nasopharynx, larynx, tracheobronchial tree, and the lungs. Ablation of all visible papillomas with the surgical laser achieved remissions in approximately one-third of patients for one year or more; since relapses may occasionally occur 2 to 20 years later, cure can never be assumed. At the present time, management is directed towards total ablation of all visible papilloma consistent with preservation of the airway and voice; reduction of the tumor burden to minimal proportions is thought to allow the maximum opportunity for remission. As the host-papilloma relationship is unraveled, it may be possible in the future to provoke an immune response so that remissions can be predicted and produced consistently.

A new microlaryngeal instrument set has been developed for phonatory surgery in adults and for pediatric laryngology. This 20-instrument set consists of forceps (cupped and alligator type) and scissors with 1-mm distal grasping and cutting tips. Open tip and whistle tip variety suction instruments with tip diameters of 1.0,1.5, and 2 mm are also available. Included as well are fine (1.0-mm) probes for palpation and laser welding and smoke evacuation double-action grasping forceps. This microlaryngeal instrument set has been extremely useful in more than 80 clinical applications.

Acute epiglottitis is a true pediatric emergency. The intense inflammation of the supraglottic larynx may completely obstruct the larynx within several hours. This infection is secondary to infiltration of Hemophilus influenzae type b (HIB), and 90% to 95% of patients have positive blood cultures. During this period of generalized septicemia HIB involvement of other soft tissue sites may occur. The goal of this article is to alert otolaryngologists to the possibility of extraepiglottitic HIB involvement. In particular, HIB meningitis, concurrent with supraglottitis, will be discussed. Although the overall incidence of multiple site involvement is relatively low, it is important to be aware of the possibility, since the outcome may be significantly altered.

Congenital and acquired lesions of the pediatric airway may lead to significant morbidity and mortality. A review of 616 endoscopic procedures, carried out over a 2-year period, was undertaken. Of these procedures, 270 were diagnostic and 346 were therapeutic. This paper outlines the clinical indications, the role of radiology, and the diagnoses revealed by this retrospective review. Of particular interest was the fact that 11.5% of the patients had lesions in more than one anatomic site in the upper aerodigestive tract. The complications were divided into intraoperative, immediate postoperative, and delayed. In total, there was a 1.6% complication rate.

Recent reports of isolation of bacteria from effusions of the middle ear taken from asymptomatic children have suggested a possible role for persistent bacterial infection in the etiology of prolonged effusions. To investigate the significance of anaerobic bacteria in such effusions, we obtained 51 samples of middle ear effusion (MEE) from 30 children. After careful cleansing of the tympanic membrane with 70% alcohol, we performed tympanocentesis prior to performing a myringotomy. Fastidious bacteriologic methods failed to yield a single anaerobic isolate from any of these specimens. We conclude that anaerobic infection of MEE is exceedingly uncommon.

A variety of congenital and acquired lesions of the larynx are now managed effectively with the use of the carbon dioxide laser. This instrument seems to be the most effective of all available lasers for this type of therapy. Argon and neodymium:yttrium aluminum garnet lasers do not appear appropriate for such lesions for several reasons. On occasion, patients with congenital and acquired subglottic stenosis, as well as those afflicted with rare tumors of the larynx, require open procedures as part of their treatment regimen. The need for appropriate equipment and competent pediatric anesthesia is paramount when undertaking cases of this type. These patients offer unique challenges to a laryngologist, but correction of their problems is effectively managed by appropriate application of current technology.