Predicting surgical outcomes in pediatric cervicofacial nontuberculous mycobacterial lymphadenitis.

OBJECTIVES: We examined surgical outcomes in children with cervicofacial nontuberculous mycobacterial lymphadenitis and attempted to identify predictors of complications. METHODS: A retrospective chart review from 2 tertiary pediatric centers was used to identify .11 presentation or operative variables (age at surgery, gender, symptom duration, pain, violaceous skin changes, skin breakdown, fluctuance, purified protein derivative positivity, operative procedure, use of nerve integrity monitoring, and use of skin flap advancement) and to compare these to 5 postoperative complications (facial nerve dysfunction [paresis or paralysis], poor scarring, recurrence, wound infection, and wound dehiscence without infection). RESULTS: The 45 patients analyzed for presentation or operative variables (28 female, 17 male; average age, 31.2 months) typically presented with painless masses averaging 8.2 weeks in duration, along with violaceous skin changes in 29 of the 45 cases (64%) and skin breakdown in 9 cases (20%). The surgical procedures included parotidectomy with or without selective lymphadenectomy in 38 of the 45 cases (84%) and lymphadenectomy alone in 7 cases (16%). Skin resection and cervicofacial advancement flap reconstruction was performed in 20 cases (44%). Nerve integrity monitoring was utilized in 32 cases (71%). In the 44 patients analyzed for postoperative complications, we found facial nerve paresis in 14 (31.8%), poor scarring in 9 (20.5%), wound infection in 6 (13.6%), recurrence in 4 (9.1%), and facial nerve paralysis in 2 (4.5%). Nine of the 14 cases (64.3%) of initial facial nerve paresis resolved. At final follow-up, facial nerve paresis persisted in 5 of the 14 children (35.7%) with initial postoperative paresis and in 1 of the 2 children (50.0%) with initial postoperative paralysis. Facial nerve paralysis persisted in the other child with initial postoperative paralysis. Overall, 6 of these 7 patients (85.7%) with persistent facial nerve dysfunction had follow-up of less than 1 month. All transient and permanent facial nerve dysfunction was in the distribution of the marginal mandibular nerve only. No statistically significant predictors of complications were identified. CONCLUSIONS: We report acceptable but not insignificant rates of marginal mandibular distribution facial nerve injury, poor scarring, wound infection, and recurrence following resection of cervicofacial nontuberculous mycobacterial lymphadenitis in children that must be discussed with patients and parents before operation. No presentation or operative variables predicted the complications.

Cochlear implantation in infants less than 12 months of age.

OBJECTIVE: Infants are diagnosed with severe to profound hearing loss at an earlier age due to the advent of universal newborn hearing screening. This offers the opportunity to provide intervention in the form of cochlear implantation at an earlier age than was previously possible. The purpose of this investigation is to evaluate the risk of cochlear implant surgery in children less than 12 months of age. DESIGN: Retrospective review of children who underwent cochlear implantation before 12 months of age. SETTING: Patients were identified from a database of pediatric cochlear implant patients at a tertiary care center. All patients were diagnosed with severe to profound hearing loss by otoacoustic emission and auditory brainstem response. Follow-up ranged from 2 months to 5 years. RESULTS: Fourteen of 15 patients had full insertions of the electrode hardware. Less than full insertion and post-operative CSF otorrhea occurred in one patient with severe cochlear abnormalities. There were no other perioperative surgical complications. The average speech detection threshold was 27.6 dB (20-45 dB) at approximately 1-3 months post-stimulation and 25 dB (15-30 dB) at approximately 5-7 months. CONCLUSION: In our experience, we feel cochlear implantation is safe for infants as young as 6 months of age. The current standard at our institution is to implant by 7 months of age for prelingual deafness as opposed to waiting additional time until 12 months of age before the brain is presented with speech.

Infectious complications in pediatric cochlear implants.

OBJECTIVES: Infectious complications may cause significant delay in cochlear implant device initiation and programming and be a source of additional morbidity. We reviewed our experience with infectious complications in the pediatric age group to determine specific sources that may not be seen in adults. STUDY DESIGN: A retrospective analysis from a single implant center. METHODS: Cases of pediatric cochlear implants were reviewed for data on infectious complications. Complications were identified as "major" or "minor," "early" or "delayed." Information was gathered regarding any comorbid, chronic health condition. Data related to the causative organism(s) were collected. RESULTS: Two hundred sixty-eight cases of pediatric implants were reviewed. Twenty-two cases were identified (an infection rate of 8.2%), all classified as "major." The majority, 12, were classified as "delayed" complications. Twenty-one cases required explantation with 14 successfully reimplanted. Five cases (in 4 patients) or 23% were associated with a specific chronic pediatric condition including two children with tracheostomies. Among implanted children who had chronic health conditions, 42% developed implant-related infections. Among otherwise healthy implanted children, only 6.6% developed implant-related infections. Resistant bacterial infections were not identified. CONCLUSIONS: Health conditions in the pediatric age group were associated with 23% of our complications, a risk factor not previously identified in the literature. These children, demonstrating seven times the infection rate of healthy children, should be carefully observed postoperatively. Overall, cochlear implantation in children continues to be associated with a low risk of infectious complications.

High-resolution magnetic resonance imaging of human cochlea.

OBJECTIVES: High-resolution MRI (MRI) of human inner ear structures provides several advantages over other imaging modalities. High-resolution visualization of inner ear ultrastructure in a noninvasive manner may provide important information about inner ear disease that is not obtainable in other ways. The study was performed to demonstrate the capabilities of MRI at high resolution on the human cochlea, vestibular structures, and facial nerve. Comparative analyses of MRI anatomy with that seen on histological dissection were made. The aim of the study was to define the anatomy of human cadaveric cochlea using a 9.4-Tesla magnetic resonance scanner, currently the most powerful magnetic resonance magnet available. STUDY DESIGN: Experimental pilot study of cadaveric human cochleae. METHODS: Serial scanning using a 9.4-Tesla magnetic resonance imager on normal preserved and fresh cadaveric inner ears was performed in different planes. RESULTS: The images revealed detailed anatomy of the modiolus, utricle, saccule, semicircular canals, and facial nerve. Specifically, identifiable structures within the cochlea included the osseous spiral lamina, Reissner's membrane, membranous spiral lamina, spiral ligament, and others. CONCLUSIONS: Data established through the acquisition of images from cadaver cochlea, facial nerve, and vestibular complex provide a foundation for developing steps for testing temporal bones and, eventually, patients with Meniere's disease and other inner ear disease. The present ongoing project will provide information on baseline images of the inner ear using high-resolution MRI.

Spontaneous tracheal and subglottic tears in neonates.

OBJECTIVES/HYPOTHESIS: Spontaneous rupture of the trachea or subglottis as a complication of difficult delivery has not been reported in the United States literature. There have been a few cases reported in the European literature. The present report describes a series of newborns with this complication and discusses the signs and treatment options of this difficult, life-threatening problem. STUDY DESIGN: Retrospective review. METHODS: Newborns born between 1996 and 2001 who were treated for spontaneous subglottic or tracheal rupture at a tertiary care children's hospital neonatal intensive care unit were reviewed. RESULTS: Four cases of spontaneous subglottic rupture were seen at the hospital. In three of the four cases the tracheas were intubated on an emergency basis after subcutaneous air was noted in the anterior aspect of the neck. In the fourth patient the trachea was not intubated until the subglottic tear was visualized intraoperatively. Two of the four patients died. One died without securing of an airway; the other died of complications of prolonged hypoxia. Eight cases from European literature of spontaneous neonatal subglottic and tracheal tears are reviewed and are compared with the cases presented in the current report. CONCLUSIONS: Early detection of airway rupture by flexible endoscopy is essential for timely diagnosis and appropriate treatment. Standard endotracheal intubation can exacerbate the problem and should be deferred if possible until direct airway visualization can be accomplished. Signs associated with tracheal tears include subcutaneous emphysema, respiratory distress, pneumothorax, and pneumomediastinum. These should lead to emergent consultation with otolaryngologists for examination and securing of the airway.

Effects of exogenous interferon gamma on patients with treatment-resistant chronic rhinosinusitis and dysregulated interferon gamma production: a pilot study.

OBJECTIVE: To evaluate the effects of exogenous interferon gamma treatment in patients with chronic rhinosinusitis and evidence of aberrant production of interferon gamma (IFN-gamma) and its regulatory cytokines. METHODS: Ten patients with treatment-resistant chronic rhinosinusitis (4 males and 6 females) treated with exogenous interferon gamma (50 micro g/m2) were retrospectively evaluated by assessing clinical outcomes compared with clinical and laboratory findings before interferon gamma treatment. RESULTS: Dysregulated IFN-gamma production was suspected to be characterized by (1) decreased interleukin 12 production (n = 1), (2) defects in interleukin 12 receptor signaling (n = 4), (3) intrinsic defects in interleukin 12 (n = 4), and (4) decreased IFN-gamma production. Eight patients had a history of chronic otitis media with positive bacterial cultures of sinus lavage samples. Adverse skin reactions to various antibiotics were reported in 7 patients. Asthma was reported in 4 patients. Along with sinusitis symptoms, these conditions were better controlled in all 9 patients who received exogenous interferon gamma for longer than 3 months. In 1 patient, interferon gamma treatment was discontinued after 3 weeks secondary to "presumed" tremor that was later diagnosed as a tic. Repeated surgical procedures and hospitalizations were reported in 2 patients after interferon gamma treatment secondary to recurrent chronic otitis media/mastoiditis/catheter infection and G-tube leakage. Interferon gamma treatment was discontinued in 1 of these patients because of a concern about neutropenia that occurred after catheter infection. Adverse effects of using exogenous interferon gamma were generally limited to local skin reactions. CONCLUSION: Exogenous interferon gamma may be a therapeutic option in a subset of patients with treatment-resistant chronic rhinosinusitis and evidence of dysregulated IFN-gamma production.

Mandibular distraction to relieve airway obstruction in children with cerebral palsy.

OBJECTIVE: To determine the effectiveness of distraction osteogenesis of the mandible for relief of airway obstruction in neurologically impaired children. DESIGN: Prospective pilot study. SETTING: Tertiary care children's hospital. PATIENTS: Five children with upper airway obstruction secondary to hypotonia were identified by airway endoscopy, pulse oximetry, and polysomnography. Four children were tracheotomy-dependent because of upper airway obstruction, and 1 was being considered for tracheotomy because of progressive airway obstruction. INTERVENTIONS: All patients underwent distraction osteogenesis of the mandible for relief of their airway obstruction. MAIN OUTCOME MEASURES: Treatment success was determined by endoscopy, continuous pulse oximetry, and polysomnography. RESULTS: Four of the 5 children underwent distraction osteogenesis of the mandible with successful resolution of airway obstruction or tracheotomy decannulation. One child did not show adequate improvement of upper airway obstruction and remained tracheotomy-dependent. Follow-up was 2 to 40 months. CONCLUSIONS: Children with cerebral palsy and hypotonia of the upper airway may achieve relief of their chronic airway obstruction following distraction osteogenesis of the mandible. Appropriate selection criteria must be adhered to, including demonstration of tongue base obstruction on flexible laryngoscopy.

Diagnosis and management of pulmonary metastasis from recurrent respiratory papillomatosis.

OBJECTIVE: We sought to review the current and proposed management, as well as bring about discussion, of managing the patient with distal tracheal and pulmonary parenchymal involvement by recurrent respiratory papillomatosis (RRP). DESIGN, SETTING, AND PATIENTS: We conducted a review of 6 patients with pulmonary metastasis from RRP at 3 academic tertiary care hospitals. Interventions included surgical and medical management with antiviral, chemotherapeutic, and/or immune-modulating agents. RESULTS: Although treatment with alpha-2-beta interferon, isotretinoin, and methotrexate have not proved to eradicate pulmonary involvement by RRP, possible epithelial stabilization and slowing of disease progression are noted. CONCLUSIONS: The rates of distal tracheal and pulmonary metastasis as seen in our cohort were higher than previously reported. Approximately 12% of our patients with RRP have distal tracheal spread and as many as 7% of all patients with RRP at our institutions have pulmonary dissemination. Also, high suspicion for malignant conversion to squamous carcinoma in the patient with pulmonary spread should be maintained. In addition, aggressive treatment, although not proved to eradicate the pulmonary disease, should be undertaken due to the high morbidity and mortality associated with pulmonary dissemination of RRP in our cohort.

Transtracheal oxygen catheters in a pediatric population.

This is the first report to evaluate transtracheal oxygen catheter (TTOC) use in a pediatric patient series. Seven pediatric patients (4 boys and 3 girls) received TTOCs in 2 tertiary care medical centers. The medical indications included bronchopulmonary dysplasia in 4 patients and tracheomalacia in the other 3. The average age at the time of placement was 22 months (range, 2 weeks to 37 months). Catheter placement for 4 patients was through an open tracheotomy stoma. In 3, placement was through a percutaneous technique. The follow-up ranged from 2 weeks to 5 years. There were no long-term complications. Transient needs for supplemental oxygen were all met by the TTOC system. In 4 patients, the catheter has been removed because of resolution of the supplemental oxygen requirements. Minor complications included skin site infection and mucus plugging. In 1 patient, accidental dislodging of the catheter led to its replacement in the operating room. In 1 percutaneous placement, a pneumothorax occurred and resolved without any persistent morbidity. We conclude that transtracheal oxygen delivery can be a reasonable alternative to a nasal cannula or formal tracheotomy in selected pediatric patients in whom long-term oxygen delivery, but not an alternate airway, is required. In order to avoid complications, meticulous technique must be adhered to in using the percutaneous approach for placement.

Cine magnetic resonance imaging for evaluation of focal tracheomalacia: innominate artery compression syndrome.

BACKGROUND: The contribution of an 'aberrant innominate artery' to respiratory distress syndromes has been a matter of debate nearly since the introduction of this concept. Recent advances in dynamic imaging are proving to be of value in assessing tracheal function in patients with respiratory distress. We therefore evaluated patients with innominate artery compression syndrome using the cine magnetic resonance imaging (CMRI) modality. OBJECTIVES: To apply the CMRI modality to evaluate patients with respiratory distress who exhibited tracheal compression at the level of the innominate artery. METHODS: A cohort of three patients in respiratory distress underwent bronchoscopy, followed by CMRI using a Siemens 1.5T Vision system. RESULTS: These three patients exhibited tracheal compression at the level of the innominate artery in agreement with their findings during bronchoscopy. All three exhibited dynamic tracheal compression that varied with the respiratory cycle. The degree of tracheal compromise was readily appreciated using the dynamic, real-time CMRI modality. Due to the severity of symptoms, the two children underwent innominate arteriopexy with complete resolution of their symptoms. CONCLUSIONS: CMRI provides extremely rapid acquisition of images, as well as integrated information regarding relationships of mediastinal structures. By providing functional imaging of tracheal patency during the respiratory cycle, CMRI may provide additional insight into innominate artery compression syndrome as more patients are evaluated.

Postoperative facial nerve function in pediatric parotidectomy: a 12-year review.

OBJECTIVE: To analyze the incidence of postparotidectomy facial nerve dysfunction in pediatric patients and to evaluate the association between patient demographics or underlying pathology and functional outcomes. STUDY DESIGN: Case series with chart review. SETTING: Tertiary-care pediatric hospital. SUBJECTS AND METHODS: We reviewed charts of patients younger than 18 years who underwent parotidectomy performed by a pediatric otolaryngologist between 1999 and 2011 at a tertiary pediatric center. Distributions of postoperative facial nerve dysfunction and timing of recovery were noted. Chi-square and t-test analyses were conducted to determine the association between demographics or pathology and functional outcomes. RESULTS: Forty-three patients met inclusion criteria: 41 underwent superficial parotidectomy, and 2 underwent total parotidectomy. Leading indications for surgery were atypical mycobacterial infection (16/43 [37.2%]) and first branchial cleft anomaly (8/43 [18.6]). The facial nerve was sacrificed in 1 (2%) patient with Ewing's sarcoma involving the nerve. The incidence of immediate facial nerve paresis was 21% (9/43), involving the marginal mandibular nerve (n = 7), buccal branch (n = 1), and both marginal mandibular and frontal branches (n = 1). Full recovery of nerve function in patients with paresis occurred within 1 month (n = 2), 2 months (n = 1), 6 months (n = 3), or 10 months (n = 2). CONCLUSION: Postparotidectomy paresis of distal branches of the facial nerve is a common occurrence in the pediatric population and should be discussed during preoperative evaluation. The risk is comparable to that of the adult population. Age, gender, and pathologic diagnosis were not predictive of postoperative nerve dysfunction.

Symptom timeline preceding cochlear implant failure: an institutional experience.

OBJECTIVE: Review cochlear explants and determine the incidence of device and medical failures and develop a pattern of symptoms indicating probable implant failure. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Subjects were selected if they underwent cochlear explantation. Data were reviewed to determine initial symptom and symptom timeline preceding implant failure. RESULTS: A total of 847 implants were performed from 1988 to 2008, with 128 devices explanted. Of total implants, 72% were Advanced Bionic devices, and 28% were Cochlear Nucleus devices. Overall failure rate was 128 of 847 (15%), with 51 (6%) medical failures and 77 (9%) device failures. Patients with Advanced Bionic devices underwent 102 explants (16.7% failure rate), with 35% medical failures and 65% device failures. Patients with Nucleus devices underwent 26 explants (11% failure rate), with 58% medical failures and 42% device failures. Medical failures included infected devices and wound dehiscence, with the pattern averaging 4.5 months to explantation. Hard device failures included sudden malfunction and slow decline in function, with pattern of failure over 4.2 months. Soft failures included tinnitus and discomfort, with failure over 8 months. CONCLUSIONS: There were common patterns when evaluating both medical and device failures. By recognizing patterns of symptoms that may indicate probable implant failure, otolaryngologists can better counsel patients on what to expect with their implants when they present with certain symptoms.

Effect of endoscopic sinus surgery on pulmonary status of adults with cystic fibrosis.

OBJECTIVE: Functional endoscopic sinus surgery (FESS) provides symptomatic relief of sinus disease in patients with cystic fibrosis (CF), but it is unclear whether it has beneficial effects on lung disease in this population. This study assessed the effect of FESS on the respiratory status of adult patients with CF. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary medical center. SUBJECTS: Thirty-two adult patients with CF who underwent 45 operative cases. METHODS: Clinical information retrieved for the 12-month periods preceding and following to determine the effect of FESS on the rate of decline in lung function, as well as intravenous antibiotic use and hospitalization for pulmonary exacerbation. RESULTS: The rate of decline in forced expiratory volume in 1 second and forced vital capacity was not significantly different in the 12 months before and after FESS. Functional endoscopic sinus surgery did not reduce days hospitalized or days on intravenous antibiotics for a respiratory exacerbation in the pre- vs postoperative period. Limiting the analysis to the 30 surgeries that were performed in patients with concomitant respiratory symptoms (ie, excluding the 15 surgeries performed for sinus symptoms alone) did not significantly alter the results. Covariates of importance in CF, including CFTR genotype, gender, or microbiology, did not affect the study results. CONCLUSION: These results did not demonstrate an effect of FESS on progression of lung disease in patients with CF, but further research is needed because low statistical power has made some of the negative findings inconclusive.

Deposition of aerosolized particles in the maxillary sinuses before and after endoscopic sinus surgery.

BACKGROUND: Recent studies suggest that topical therapy is beneficial in many conditions underlying chronic sinusitis. Current literature has documented low aerosolized particle deposition efficiency into the paranasal sinuses. Mathematical modeling suggests that three factors influence the deposition efficiency: particle size, pressure gradient, and size of the sinus ostium. Ostium size is the most dominant factor. Therefore, we sought to determine if maxillary antrostomy and ethmoidectomy would increase the deposition efficiency. METHODS: Five cadavers underwent pre- and postoperative scintigraphy after administration of aerosolized Tc-99M. Images were obtained with a gamma-camera and regions of interest (ROIs) were drawn around the maxillary sinuses. Counts per minute in the pre- and postoperative ROIs were then compared using the paired t-test. RESULTS: Results indicated a significant increase in deposition of radioactivity in the maxillary sinuses in the postoperative state (p < 0.01). CONCLUSION: Topical therapy for chronic sinusitis may be more feasible in the postoperative population.

Effective treatment of alpha-mannosidosis by allogeneic hematopoietic stem cell transplantation.

OBJECTIVES: To study the efficacy of hematopoietic stem cell transplantation (HCT) for ameliorating the clinical manifestations of alpha-mannosidosis. STUDY DESIGN: Four patients with alpha-mannosidosis underwent allogeneic HCT at the University of Minnesota. Diagnosis was established by assay of leukocyte alpha-mannosidase activity level. Physical features, donor engraftment, leukocyte alpha-mannosidase activity, neuropsychologic function, and hearing were monitored before and after transplantation, with follow-up ranging from 1 to 6 years. RESULTS: All 4 patients showed slowing of their neurocognitive development and sensorineural hearing loss before HCT. All patients are alive, with normalization of leukocyte enzyme activity after HCT. Intellectual function has stabilized, with improvement in adaptive skills and verbal memory function in 3 of 4 patients. Hearing has improved to normal or near normal for speech frequencies in 3 patients. No new skeletal abnormalities have developed. CONCLUSIONS: HCT can halt the progressive cognitive loss in patients with alpha-mannosidosis. Early diagnosis and treatment with HCT is critical for optimal results.