Trends in the management of pediatric chronic sinusitis: survey of the American Society of Pediatric Otolaryngology.

BACKGROUND: The management of chronic sinusitis (CS) in children has yet to be fully elucidated. The objective of this study is to assess practice trends within the pediatric otolaryngology community for the management of children with CS. METHODS: A multiple choice survey of the members of the American Society of Pediatric Otolaryngology (ASPO) was performed to assess for various factors related to the management of CS in children. RESULTS: A total of 175 ASPO members responded to the survey. The majority of respondents initially treat patients medically with oral antibiotics (95%), topical steroids (90%), and nasal saline sprays (68%). Fifty-five percent performed adenoidectomy as part of the treatment of CS, with 81% performing the operation before endoscopic sinus surgery (ESS). Compared with 3 years before the survey, 47% of respondents performed approximately the same number of ESS cases, whereas 35% reported doing fewer cases annually. Seventy-two percent of practitioners do not routinely perform a second-look surgery. CONCLUSIONS: The majority of pediatric otolaryngologists use oral antibiotics, nasal steroids, and saline lavage, and will perform adenoidectomy when managing patients with CS. More than a third of pediatric otolaryngologists are using more stringent criteria for surgery and performing less extensive surgery than 3 years before the survey. Surgical outcomes for CS do not appear to have changed over the past 3 years.

Mutation of the POU-domain gene Brn4/Pou3f4 affects middle-ear sound conduction in the mouse.

Mutagenesis of the POU-domain gene Brn4/Pou3f4 causes defects in the cochlear duct, semicircular canal, temporal bone and stapes footplate. The footplate defect suggested a middle-ear conductive component to the hearing loss associated with this mutation. This was examined by measuring velocity transfer functions at the umbo of wild type and knockout mice during sound stimulation of the tympanic membrane. When the median umbo velocity of test frequencies in the two groups were compared, the mid-range frequencies of the knockout mice showed a statistically reliable reduction in velocity (maximum of 13 dB) and high variability among animals. These results indicated that mutation of the POU-domain gene, Brn4, changed middle-ear sound conduction when measured at the umbo. The origin of the abnormal velocity response was sought by puncturing a hole in the pars flaccida (PF), and subsequently, measuring movements at the umbo and the head of the long arm of the incus. This hole permitted us to measure velocity at the tip of the incus long arm, just above the incudostapedial joint. The comparison of umbo behavior in both groups with PF perforated showed a loss of sensitivity in the mid-range frequencies of the knockout animals. A comparison of incus velocity in the two groups also exhibited a velocity reduction in the mid-range frequencies of the knockout animals. The reduction at the incus, however, was milder than observed at the umbo. The effect of the perforation in, and variability of, the knockout incus responses may have masked a more potent mid-range frequency effect. Nevertheless, evaluation of the stapes and oval window in knockout mice showed variable pathology from ear to ear. The presence of this pathology, the mid-frequency loss in incus sensitivity and the variability in incus velocity among animals suggested that abnormal stapes behavior in Brn4 deficient mice may determine the response of the ossicles, and thus account for the abnormal mid-frequency umbo behavior seen in knockout animals.

Pediatric submandibular triangle masses: a fifteen-year experience.

BACKGROUND: The purpose of this study was to evaluate the surgical results of pediatric submandibular triangle masses, with specific attention to neoplastic processes. METHODS: We retrospectively reviewed the medical records of 105 patients aged 6 months to 21 years who underwent surgery in the submandibular triangle at a major pediatric tertiary care hospital from 1987 to 2001. RESULTS: One hundred five patients who underwent surgery in the submandibular triangle were included in the study. Twenty patients had neoplastic processes, six of which were of primary salivary origin (two mucoepidermoid carcinomas and four pleomorphic adenomas). Twenty-four patients underwent excision of inflamed or infected lymph nodes, and 23 patients underwent excision of inflamed or infected submandibular glands. Thirty-eight patients were included who underwent surgery for sialorrhea or to gain access for another surgical procedure. Complications included tumor recurrence, transient and permanent marginal mandibular nerve weakness, ranula, postoperative fluid collection, and cellulitis. Duration of follow-up ranged from no follow-up to 11 years. CONCLUSION: Surgical excision of submandibular triangle masses is uncommon. We present our experience with these lesions, with a discussion of diagnosis, surgical indications, and surgical complications.

Cellular neurothekeoma of the maxilla.

Neurothekeomas are uncommon benign soft tissue tumors of nerve sheath origin. They occur predominately in the head and neck or upper trunk of children and young adults. A 15-month-old boy presented with an enlarging mass of the right maxilla. Radiologic imaging demonstrated an expansile lesion of the nasomalar region. An incisional biopsy resulted in the diagnosis of neurothekeoma. This lesion should be considered as part of the differential diagnosis of pediatric soft tissue head and neck masses. We discuss the presentation, evaluation, and treatment of these rare benign lesions.

Actinomycosis of the temporal bone: a report of a case.

Actinomycosis is a chronic suppurative infection of the cervicofacial region caused by Actinomyces species, which are anaerobic, gram-positive filamentous bacteria. Although actinomycosis has a propensity for involving the oral cavity, rare cases of actinomycosis involving the temporal bone have been published. We report the case of a 14-year-old girl who presented with clinical, audiometric, and radiologic findings consistent with right chronic suppurative otitis media that persisted despite tympanomastoidectomy. Findings on histologic evaluation of a specimen obtained during revision surgery were consistent with a diagnosis of actinomycosis. Although actinomycosis of the temporal bone is rare, it should be considered in the differential diagnosis of chronic suppurative temporal bone infections that are resistant to standard therapy.

Sialorrhea: a management challenge.

Sialorrhea (drooling or excessive salivation) is a common problem in neurologically impaired children (i.e., those with mental retardation or cerebral palsy) and in adults who have Parkinson's disease or have had a stroke. It is most commonly caused by poor oral and facial muscle control. Contributing factors may include hypersecretion of saliva, dental malocclusion, postural problems, and an inability to recognize salivary spill. Sialorrhea causes a range of physical and psychosocial complications, including perioral chapping, dehydration, odor, and social stigmatization, that can be devastating for patients and their families. Treatment of sialorrhea is best managed by a clinical team that includes primary health care providers, speech pathologists, occupational therapists, dentists, orthodontists, neurologists, and otolaryngologists. Treatment options range from conservative (i.e., observation, postural changes, biofeedback) to more aggressive measures such as medication, radiation, and surgical therapy. Anticholinergic medications, such as glycopyrrolate and scopolamine, are effective in reducing drooling, but their use may be limited by side effects. The injection of botulinum toxin type A into the parotid and submandibular glands is safe and effective in controlling drooling, but the effects fade in several months, and repeat injections are necessary. Surgical intervention, including salivary gland excision, salivary duct ligation, and duct rerouting, provides the most effective and permanent treatment of significant sialorrhea and can greatly improve the quality of life of patients and their families or caregivers.

Pediatric intraparotid Castleman's disease.

Castleman's disease (CD) is a rare, benign lymphoepithelial disease of unknown cause that presents most commonly in the mediastinum. There are 2 histologic types of CD: the hyaline vascular type and the plasma cell type. In the head and neck, 98% of these lesions are of the hyaline vascular type. The differential diagnosis of CD includes reactive lymphadenopathy, lymphoproliferative disorders, lymph node metastasis, and other conditions. Approximately 80 cases of CD have been reported in children; head and neck involvement in children is extremely rare. A case of a child with intraparotid CD is presented; the clinical course, histopathologic presentation, radiographic findings, and management of CD are reviewed.

Choanal atresia: a twenty-year review of medical comorbidities and surgical outcomes.

OBJECTIVE: To review medical comorbid conditions and surgical outcomes for children treated for choanal atresia (CA). STUDY DESIGN: Retrospective review (1979-1998). METHODS: Participants included 78 children, aged newborn to 18 years, in the setting of an academic pediatric hospital. Main outcome measures were type and number of procedures and airway patency. RESULTS: Seventy-eight children (48 female and 30 male patients) were treated and followed for CA, with an average follow-up of 35 months. Thirty-five children (45%) had unilateral CA, and 43 children (55%) had bilateral CA. There was no statistically significant sex difference between unilateral and bilateral CA. Concomitant medical problems were common, mostly otitis media with effusion (32%), upper and lower airway diseases (32% and 23%, respectively), cardiac anomalies (19%), and gastrointestinal tract disorders (18%). Statistically significant correlations were found for bilateral CA and cardiac disorders (P =.04), CHARGE syndrome ( P=.002), obstructive sleep apnea ( P=.003), hematological problems (P =.001), and prematurity or failure to thrive (P =.006). Surgery was effective in establishing a patent airway in all cases. Average ages at first surgery were 25.2 months for unilateral and 2.4 months for bilateral CA. Unilateral CA required, on average, 2.7 total procedures, including dilation and removal of stents, compared with bilateral CA, which required 4.9 procedures. Nearly all children underwent correction by means of the transnasal approach. CONCLUSIONS: 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.

A staging system for congenital cholesteatoma.

OBJECTIVE: To develop a staging system for congenital cholesteatoma in predicting the likelihood of residual disease. DESIGN: Retrospective analysis of data from a case series, to identify predictors of residual disease. SETTING: Tertiary care pediatric hospital. PARTICIPANTS: Children undergoing surgical removal of congenital cholesteatoma. There were 156 patients, with 160 cholesteatomas; 4 children had bilateral disease. INTERVENTIONS: Each case was scored as to quadrants of the middle ear involved, ossicular involvement, and mastoid extension. MAIN OUTCOME MEASURE: Surgically confirmed residual disease at any time after the initial procedure. RESULTS: Four stages were defined as follows: stage I, disease confined to a single quadrant; stage II, cholesteatoma in multiple quadrants, but without ossicular involvement or mastoid extension; stage III, ossicular involvement without mastoid extension; and stage IV, mastoid disease. There was a strong association between stage and residual disease, ranging from a 13% risk in stage I to 67% in stage IV. CONCLUSIONS: This simple staging system may be particularly useful in standardizing the reporting of congenital cholesteatoma and in adjusting for severity in evaluating outcomes. It also provides information that is useful in counseling parents.

Adenovirus-mediated ex vivo gene transfer of human vascular endothelial growth factor in a rabbit laryngotracheal reconstruction model.

Free autologous cartilage, which is used in laryngotracheal reconstruction (LTR), may undergo progressive necrosis as a result of delayed revascularization. Angiogenic growth factors, such as vascular endothelial growth factor (VEGF), promote angiogenesis in the ischemic environment. We studied the effect of ex vivo gene transfer of VEGF121 on cartilage angiogenesis and graft survival in a rabbit model of LTR. Sixty rabbits underwent LTR with auricular cartilage. The grafts were treated at 1 x 10(9) plaque-forming units with 1) VEGF121 (n = 20), 2) LacZ reporter gene (n = 20), or 3) saline solution (n = 20). Graft neovascularization and survival were histologically assayed at 1 and 10 weeks. Angiogenesis was enhanced at both 1 and 10 weeks after treatment with VEGF121 as compared to controls (p < .001). No statistical improvement in graft survival was evident after treatment with VEGF121. Ex vivo gene transfer to cartilage may be a promising gene therapy strategy to enhance revascularization--and, potentially, cartilage survival--under the proper conditions.

Congenital cholesteatoma: 20 years' experience at The Children's Hospital of Philadelphia.

OBJECTIVE: We report our experience with congenital cholesteatoma over a span of 20 years with an emphasis on presenting characteristics and predictors of outcome. METHODS: We conducted a retrospective review from 1981 through 2000. RESULTS: One hundred seventy-two congenital cases were identified in 167 patients. Five patients had bilateral disease. The majority (72%) were found in boys, with an average age of 5.0 years. Hearing loss was slight to moderate. When confined to 1 quadrant, cholesteatoma was anterosuperior in 82% of cases; 47% had cholesteatoma in 2 or more quadrants. Ossicular chain involvement was found in 43% of all cases, and mastoid extension was evident in 23%. The rate of recurrent disease was directly related to the extent and number of quadrants involved. CONCLUSION: To our knowledge, this is the largest series of congenital cholesteatomas to be reported. This review confirms the male predominance and predilection for the anterosuperior quadrant. The extent of cholesteatoma and its relation to residual disease should be used as a guide for planning a second-look procedure.