Clinical update on 10 children treated with intralesional cidofovir injections for severe recurrent respiratory papillomatosis.

OBJECTIVES: To continue assessment of the benefits and risks of intralesional administration of cidofovir, an acyclic nucleoside phosphonate, for treating severe recurrent respiratory papillomatosis (RRP) in pediatric patients, and to discuss guidelines for larger prospective multi-institutional studies of the use of cidofovir. DESIGN: Prospective case series. SETTING: Tertiary care children's hospital. PATIENTS: A total of 10 patients with severe RRP (defined as requiring debulking procedures to maintain airway patency at least once a month) underwent intralesional cidofovir therapy. The original 5 patients have received more than 1 year of follow-up since their last cidofovir injection, and 5 subsequent patients have been treated with a revised injection protocol. INTERVENTION: Microsuspension laryngoscopy with intralesional injection of cidofovir after repetitive carbon dioxide laser treatments and mechanical debulking of papillomas. MAIN OUTCOME MEASURES: Papilloma stage at the time of serial laryngoscopies. Histologic examination of biopsy specimens of laryngeal tissue obtained 1 year or more after last cidofovir injection. RESULTS: There was evidence of marked improvement in the 4 of the 5 new patients enrolled under the revised injection protocol, continuation of a disease-free state in 1 of the original 5 patients, and sustained improvement in 4 of the 5 original patients, resulting in a significantly reduced interval of intervention. CONCLUSIONS: Intralesional cidofovir therapy continues to show benefit in the treatment of severe RRP in pediatric patients. Safety profiles have not been fully established, but current histologic data are reassuring.

Intralesional cidofovir for recurrent respiratory papillomatosis in children.

OBJECTIVE: To assess the potential benefit of intralesional administration of cidofovir, an acyclic nucleoside phosphonate with activity against several DNA viruses, for treating severe respiratory papillomas in pediatric patients. DESIGN: Prospective case series. SETTING: Tertiary care children's hospitals. PATIENTS: Five pediatric patients with severe recurrent respiratory papillomatosis requiring laryngoscopy with carbon dioxide laser therapy more frequently than once a month to maintain airway patency. Each patient underwent between 12 and 33 laryngoscopies with laser treatment prior to being injected with cidofovir. INTERVENTION: Microsuspension laryngoscopy with intralesional injection of cidofovir (Vistide) in conjunction with mechanical debulking and carbon dioxide laser of papillomas. MAIN OUTCOME MEASURE: Papilloma stage at time of serial laryngoscopies. RESULTS: One patient was disease free and 3 patients demonstrated a dramatic response to adjuvant therapy with cidofovir at the 9-month follow-up visit after the last injection of cidofovir. One patient showed an improvement in papilloma stage that was possibly related to concurrent therapy with interferon. CONCLUSIONS: Intralesional injection of cidofovir seems to be of benefit in the treatment of severe respiratory papillomatosis in pediatric patients. Larger prospective studies with longer follow-up will be required before cidofovir can be considered an accepted means of managing this difficult disease.

Documentation of the prevalence of penicillin-resistant Streptococcus pneumoniae isolated from the middle ear and sinus fluid of children undergoing tympanocentesis or sinus lavage.

With increasing pneumococcal resistance to penicillin and other antibiotics, use of antibiotic therapy for children with upper respiratory tract infections such as otitis media and sinusitis has become difficult. Selecting an appropriate treatment regimen has become more challenging due to frequent concomitant microbial resistance to multiple antibiotics. In a prospective, nonrandomized study, we obtained middle ear and sinus aspirate specimens from all children undergoing outpatient tympanocentesis or sinus lavage for any indication at our institution over two 4-week periods. One hundred fifty-four specimens were obtained. Of these, 12 grew Streptococcus pneumoniae, 7 of which were resistant to penicillin. A 6-month retrospective review of these patients' medical histories evaluated their antibiotic use prior to surgical intervention. An association between penicillin resistance and recent use of 2 or more antibiotics in children with positive S pneumoniae cultures was confirmed, as has been documented in prior reports. Those with penicillin-resistant S pneumoniae also demonstrated a higher incidence of multidrug-resistant organisms.

Adenotonsillectomy in the very young patient: cost analysis of two methods of postoperative care.

Postoperative management of the patient younger than 36 months undergoing adenotonsillectomy has been the subject of many debates. Concerns for early postoperative complications such as airway obstruction, emesis, dehydration, and hemorrhage have led many physicians to consider overnight hospitalization following adenotonsillectomy in very young children. Trends in health care management have had increasing focus on cost effective means of treating patients to limit unnecessary expenditure on the part of the patient, physician, and hospital facility. The purpose of this retrospective review was to analyze two methods of early postoperative management in children less than 36 months old undergoing adenotonsillectomy at the Children's Hospital, San Diego from 1992 to 1997. Three hundred and seven cases were reviewed. Same-day discharge was compared with overnight inpatient observation based on the cost analysis of these two methods of postoperative care. Postoperative care was based on length of stay in the recovery room and as an inpatient. Expense of postoperative care was based on cost calculation for the recovery room and overnight hospitalization. Of the 307 patients, 194 went home the day of surgery and 113 were observed overnight in the hospital. Average hospital cost was higher in the outpatient group than in the inpatient group (P < 0.001). This difference reflects longer recovery room stay (350 min) in the outpatient group compared to the inpatient group (108 min) (P < 0.001). Outpatient adenotonsillectomy in the patient under 36 months may be safe; however, prolonged recovery room stays may actually make outpatient surgery less cost-effective than overnight admission. Recovery room costs are significantly higher per unit time than costs of inpatient hospitalization. Further investigation of cost-effective outpatient observation units may improve cost containment in the outpatient surgical setting.

Cervical thymic anomalies.

Because of its infrequent occurrence, cervical thymic tissue is rarely considered in the differential diagnosis of neck masses. Consequently, diagnosis is most often made by pathologic examination of the excised specimen. The preponderance of reported patients have been children and adolescents, typically asymptomatic. The clinical presentation, evaluation and surgical management of five new cases of cervical thymic anomalies ranging from infancy to adulthood are described. The authors also review the embryology and histopathology of these lesions and discuss their recommended approach to the evaluation and management of cervical thymic anomalies.

Surgical strategies for otitis media.

OBJECTIVE: As we enter the new millennium, Otitis Media remains the most common cause for an acute care visit to the pediatrician. This paper discusses the various surgical approaches to children who do not respond to medical management for acute otitis, recurrent acute otitis and for chronic otitis media. In addition to discussing the relevant alternatives of intervention, attention is directed to special populations of patients who are likely to demand a great deal of care, the problems that are encountered with typanostomy tubes and the concept of a new era of a "post-tympanostomy tube legacy." CONCLUSION: Suggestions are offered as to how we can alter our approach to these patients requiring surgical intervention and thereby reduce long term sequelae.

Acute coalescent mastoiditis in an infant: an emerging trend?

There is some evidence to suggest that the incidence and complications of ACM may be increasing. However, in the current era of widespread access to health care and broad-spectrum antibiotics, an intratemporal or intracranial complication from acute otitis media may not initially be suspected. The reported case is significant in that the patient was very young, had no underlying disease or immunocompromise, and did not have a known antecedent acute otitis media. With the emergence of resistant streptococcal species and prolonged survival in immunocompromised patients, the relative incidence of complications caused by acute otitis media will probably continue to rise, making it imperative that we raise our index of suspicion for previously rare infectious complications of relatively common diseases.

Minimal endoscopic approach to subperiosteal orbital abscess.

OBJECTIVE: To limit endoscopic abscess drainage to the opening of the ethmoid cells involved, adjacent to the subperiosteal orbital abscess. DESIGN: Prospective study. PATIENTS: Twenty children with subperiosteal orbital abscess complicating acute sinusitis. INTERVENTION: Endoscopic opening of the medial wall of the bulla ethmoidalis and of the lamina papyracea. RESULTS: The limited endoscopic approach allowed subperiosteal orbital abscess drainage in all patients with positive clinical outcomes. Extensive ethmoidectomy was not necessary to achieve drainage. CONCLUSIONS: Endoscopic subperiosteal orbital abscess drainage does not require complete ethmoidectomy as was previously performed and can be limited to the opening of the bulla ethmoidalis and the lamina papyracea through the bulla ethmoidalis.

Discoordinate pharyngolaryngomalacia.

In cases of severe laryngomalacia, laser division of the aryepiglottic folds (AEFs) or endoscopic supraglottoplasty may be an ineffective solution. Failure of this technique is rare and the reasons for failure are not well established. The purpose of this study was to describe those cases of laryngomalacia in which endoscopic treatment did not reverse the clinical situation. We introduce the concept of discoordinate pharyngolaryngomalacia (DPLM). DPLM was defined as severe laryngomalacia with complete supraglottic collapse during inspiration, without shortened AEFs or redundant mucosa, and with associated pharyngomalacia. Twenty-seven of 82 children with severe laryngomalacia presented a DPLM. Endoscopic treatment was performed in 16 children and the surgical procedure was inadequate to reverse the clinical problem in these patients. In 10 children correction of additional sites of obstruction was required (uvulopharyngopalatoplasty, surgery of choanal atresia, aortopexy). Tracheostomy was necessary in 13 children. Bi-level positive airway pressure (BiPAP) was used successfully in 2 children and tracheotomy was avoided. Treatment still needs to be better defined.

Pediatric aerodigestive foreign body injuries are complications related to timeliness of diagnosis.

Foreign body (FB) injury from aspiration or ingestion is a common pediatric health problem. Diagnosis relies on clinical judgment plus medical history, physical examination, and radiographic evaluation. A multi-institutional review of 1269 FB events revealed that 85% were correctly diagnosed following a single physician encounter. However, 15% of the children had an elusive diagnosis (>1 week), despite previous evaluation. Delays in diagnosis were seven times more likely to occur in aspirations than in ingestions. Secondary injuries (e.g., pneumonia and atelectasis) occurred in 13% of airway FBs but in only 1.7% of esophageal FBs. Plain radiographs were used in 82% of children, and special studies (e.g., fluoroscopy) in only 7%. We conclude that diagnosis of FB injury in children is frequently achieved at the initial evaluation but that continued surveillance by follow-up visits to health care facilities from parents and other caretakers is important, to reduce pulmonary injuries.

Nager acrofacial dysostosis: management of a difficult airway.

Nager acrofacial dysostosis, first described by Nager and deReynier in 1948, is a rare syndrome characterized by mandibulofacial dysostosis with associated radial defects. The facial features include downward slanting palpebral fissures, absent eyelashes in the medial third of the lower lids, mandibular and malar hypoplasia, dysplastic ears with conductive deafness, and variable degrees of palatal clefting. Upper limb malformation is a constant feature of Nager syndrome and ranges from thumb hypoplasia to absence of the radial ray. The maxillo-mandibular hypoplasia and associated retroplaced tongue set the stage for early and significant upper airway obstruction. The craniofacial anomalies and associated trismus make emergent airway intubation challenging. We present a case of Nager syndrome with life threatening airway obstruction unresponsive to conservative management. This case and a review of the literature emphasize the importance of early tracheotomy for these patients.

One-stage tracheal reconstruction of congenital tracheal stenosis.

Congenital tracheal stenosis (CTS) is a condition difficult to manage and associated with a high mortality rate. The principles of one-stage laryngotracheoplasty have been adapted to the surgical management of CTS. Three children underwent a one-stage anterior tracheoplasty with costal cartilage grafting and without cardiopulmonary bypass. Extubation was performed within 13 days. One child developed granulation tissue one month postoperatively, and this was removed endoscopically. The three children were free of respiratory obstruction signs during follow-up ranging from 8 months to 3 years. Postoperative endoscopies have shown growth of the grafted trachea.

Pediatric ethmoidectomy.

The ethmoid sinuses play a key role in the health or disease of the entire paranasal sinus complex. Multiple underlying inflammatory processes may easily obstruct the narrow ostia of each ethmoid air cell, leading to infection. By virtue of its unique anatomic position and relationship to the ostiomeatal complex, the ethmoid labyrinth becomes the key to treatment of acute and chronic sinusitis. Surgical intervention for sinus disorders in children has grown in popularity. This article reviews the anatomy and pathophysiology of ethmoid sinusitis and discusses the role of surgery for these disorders.

Coccidioides immitis subperiosteal abscess of the temporal bone in a child.

A destructive granulomatous lesion of the temporal bone caused by Coccidioides immitis disseminated from a pulmonary lesion was found in a 4-year-old immunocompetent child. To our knowledge, it is the first case of coccidioidomycosis of the temporal bone reported in the world literature. The child presented with pain in her right ear and a 6-month history of intermittent fever, which partially responded to multiple courses of antibiotics. A tender erythematous postauricular swelling consistent with a subperiosteal abscess subsequently developed over 1 month. A mastoidectomy showed granulation tissue with pockets of purulence, and histologic evaluation of the specimen revealed spherules of C immitis, later verified by culture. The patient responded to intravenous amphotericin B therapy, without evidence of disease recurrence. Coccidioides immitis is endemic in regions of the Southwestern United States, with extremely infectious characteristics and relative resistance to medical therapy. Coccidioidomycosis should be considered in the differential diagnosis of a granulomatous lesion of the temporal bone.

Open surgical excision of subglottic hemangioma in children.

Subglottic hemangioma is an unusual entity which may involute spontaneously without aggressive surgical intervention; although tracheotomy is sometimes necessary. The actual time course for involution is not clear by reviewing the literature [1,14]. Numerous treatment modalities are described for the treatment of this condition, including tracheotomy, CO2 laser ablation and intralesional steroid injection with or without endotracheal intubation. Surgical resection of subglottic hemangioma is an option which is described in the literature and may be utilized in certain selected cases. We present seven cases of subglottic hemangioma treated at three institutions which were resected via a crico-tracheotomy approach. Postoperative follow-up for these patients range from 6 months to 4.5 years. Although conservative measures are still advocated as the treatment of choice for subglottic hemangioma, open surgical resection may be indicated in selected cases resulting in a satisfactory outcome.

Airway compromise secondary to vascular compression in a neonate.

Respiratory distress in the neonate may be due to extrinsic tracheobronchial compression. We recently observed a neonate with left mainstem bronchus obstruction resulting in respiratory distress and mediastinal shift. Initial management required intubation and positive pressure ventilation. Bronchoscopic evaluation demonstrated compression of the proximal left main bronchus. The distal bronchus appeared normal. Bronchography, echocardiography and an MRI of the chest confirmed proximal compression of the bronchus and suggested that the cause was an aneurysmal left pulmonary artery and patent ductus arteriosus. With medical therapy there was resolution of the bronchial compression. Re-evaluation by bronchoscopy and echocardiography revealed a normal left main stem bronchus, a normal left pulmonary artery and a closed ductus arteriosus. This clinical scenario suggests that transient pulmonary hypertension may result in significant airway compression due to the proximity of the left main stem bronchus to the left pulmonary artery.

Nasal hamartoma: case report and review of the literature.

Nasal masses in the pediatric population present with nasal obstruction, epistaxis and chronic rhinorrhea. We report on a 6-year-old boy with long-standing nasal obstruction. A large left nasal mass was evident on physical examination and CT scan. Techniques of functional endoscopic sinus surgery were used to resect the mass, which, on pathologic examination, proved to be a hamartoma. Hamartomas are non-neoplastic malformations, or inborn errors of tissue development. They are characterized by an abnormal mixture of tissues indigenous to that area of the body. Review of the literature revealed 5 previously reported cases of nasal hamartomas, only 2 of these in children. Our case is unique in that the hamartoma arose from the ethmoid and maxillary sinuses; previously reported cases arose from the septum and vestibule. This is the first reported case treated using a functional endoscopic sinus surgery approach.

Neonatal otitis media. An update.

Neonatal otitis media may be an isolated infection or part of a more complex sepsis syndrome. Because early studies suggested that the microbiologic characteristics of neonatal otitis media included a high risk of gram-negative coliforms and Staphylococcus aureus, tympanocentesis was recommended routinely. Subsequent studies have supported empiric medical therapy in selected patients, reserving tympanocentesis for patients in whom medical treatment fails. Because of these conflicting recommendations, records from 37 neonates with otitis media who underwent tympanocentesis from 1986 through 1991 were studied retrospectively. Cultures in one outpatient (11%) and four inpatients (13%) yielded Escherichia coli, all of which were sensitive to amoxicillin. No cultures of S aureus were identified. Sterile cultures and pneumococcus isolates were found most frequently. No septic or intracranial complications were noted. All patients were discharged on regimens of routine antibiotics for otitis media.