Facial neuromas in children: delayed or immediate surgery?

OBJECTIVE: The objective of this study was to investigate the clinical characteristics and outcome of facial nerve neuromas in children. To date, no specific study has focused on children, and the management of these tumors is not codified. DESIGN AND SETTINGS: A review of case series treated in a tertiary care center of pediatric otolaryngology. SUBJECTS: The treatment and outcomes for 7 children (4 infants and 3 adolescents) were analyzed. RESULTS: Six patients underwent complete removal of tumor and immediate restoration of the nerve continuity. The grade of facial palsy improved in 4 of the 6 children, but did not get better than grade 3 (House classification). The remaining patient was managed conservatively and remained stable clinically and radiologically after 9 years follow-up. CONCLUSIONS: These findings support the reasonable strategy of combining conservative assessment of these slow-growing tumors with regular clinical and radiologic evaluations and radical surgery using various procedures. The choice depends on the age of the child, the extent and growth rate of the tumor, the grade of facial palsy, and the hearing function.     

Cranionasal fistula in pediatric population: is endonasal endoscopic approach effective?

PURPOSE: To study the efficacy and safety of endoscopic endonasal repair of CSF leak in pediatric population. STUDY DESIGN: A prospective study in a tertiary care center. PATIENTS AND METHODS: All the cases with a age of less than 14 years with a diagnosis of CSF rhinorrhoea were included in the study. These were subjected to detailed examination, radiological evaluation using computed tomographic cisternography and MRI if required. These cases were then subjected to endoscopic endonasal repair of the fistula using the technique described in the text. RESULTS: There were 25 cases in the study with a male predominance and trauma responsible for 22 of these and spontaneous leak in 3 of the cases. The majority of these leaks were from cribriform plate area. After the repair, there was no leak in 24 of these cases and 1 case with recurrence did well after second surgery. DISCUSSION: The prior work on the entity of CSF rhinorrhoea in children consists of a few case reports only and ours is the largest series on endoscopic repair of the CSF leak in children showing it to be safe and effective with a success rate of 96%. CONCLUSION: Endoscopic closure of the cranio nasal fistula is associated with a high degree of success (96% in our series) and has significantly less morbidity and mortality when compared with the craniotomy approach. Hence it can be concluded that it is the standard of care for the pediatric population as well.     

Is fungal infestation of paranasal sinuses more aggressive in pediatric population?

OBJECTIVE: Comparison of characteristic features, radiology, management and recurrence pattern of fungal sinusitis between children and adults. METHODS: A prospective study conducted in the department of Otorhinolaryngology, Head and Neck Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh in which all the cases presenting with the features of allergic fungal sinusitis (AFS) between January 2000 and January 2005 were enrolled. These cases were divided into two groups, group 1 comprised of cases with age less than 15 years and group 2 with age more than 15 years. Detailed history, physical examination and nasal endoscopic examination and computed tomography (CT) scan of the paranasal sinuses was done in all the cases. The cases with prior history of sinonasal surgery were excluded from the study. All patients refractory to medical management were subjected to endoscopic sinus surgery. All the cases were followed up for a period ranging from 6 to 39 months to see for the recurrence. The data of both the groups was analysed statistically using chi square test. RESULTS: The study population comprised of 200 cases, with 68 cases in group 1 and 132 cases in group 2. The most common symptom in both the groups was presence of nasal obstruction. The children had higher incidence of having unilateral disease (46 out of 68) compared with adults, where it was 38 out of 132. The bony erosion was seen more often in group 1. Surgery was done endoscopically in all the cases. The intra orbital or intra cranial extension was seen in 58 cases of group 1 and 47 cases of group 2 (p<0.001). Recurrence was seen in 18 (15 with intraorbital and 3 with intracranial extension) cases in group 1 and 13 cases (11 with intraorbital and 2 with intracranial extension) in group 2 (p<0.005). CONCLUSION: In our study, we found a higher incidence of facial deformities, proptosis, intraorbital/intracranial extension and a higher rate of recurrence in group 1, therefore, suggesting a more aggressive nature of AFS in children than adults mandating an early diagnosis, proper management and regular follow up in these cases.     

Auditory neuropathy or endocochlear hearing loss?

AIMS: The purpose of the study was to define boundaries between endocochlear hearing loss and auditory neuropathy in children with congenital profound hearing loss and positive otoacoustic emissions. PATIENT: A child presented with bilateral profound hearing loss, which was confirmed by the absence of evoked auditory potentials at 110 dB and with conserved otoacoustic emissions. The lack of any relevant medical history, a normal neurologic pediatric examination, and the improvement obtained with powerful hearing aids suggested an endocochlear cause. Genetic testing identified mutations in OTOF, responsible for the DFNB9 recessive form of hearing loss. RESULTS: In recent years, cases of children with hearing loss associated with positive otoacoustic emissions have been labeled as "auditory neuropathy." Classically, this form of hearing loss is refractory to the use of hearing aids and cochlear implants. Mutations in OTOF lead to inner hair cells dysfunction, whereas the outer hair cells are initially functionally preserved. As this form of endocochlear hearing loss can be detected at a molecular level, genetic testing can be proposed for cases of nonsyndromic auditory neuropathy, as those children could benefit from cochlear implantation. CONCLUSION: It is advisable to reserve the term "auditory neuropathy" for patients who present hearing loss and conserved otoacoustic emissions in the context of a neurologic syndrome or for children with suggestive perinatal history. In other cases, genetic testing for mutations in OTOF should be carried out.     

Pathologic evaluation of routine tonsillectomy and adenoidectomy specimens in the pediatric population: is it really necessary?

OBJECTIVE: To evaluate the necessity of histopathologic examination for routine tonsillectomy and/or adenoidectomy in terms of unexpected malignancy by evaluating a large group of pediatric patients retrospectively with review of the literature. MATERIALS AND METHODS: A retrospective chart review of all patients under the age of 19 who underwent tonsillectomy and/or adenoidectomy between January 1990 and January 2005 was carried out. The records were analyzed concerning each patient's age, sex, indication for surgery, type of surgical procedure and the result of histopathologic examination of the specimen. The patients operated for chronic or recurrent infections and obstructive hypertrophy were included in the study. Moreover, the English literature was searched in Medline for articles published between 1949 and March 2005 and the studies dealing with pathologic analysis of tonsillectomy and/or adenoidectomy specimens were reviewed. RESULTS: The total number of patients was 2826. After excluding 83 patients because of insufficient data, 2743 patients with an age distribution from 1 to 18 years (mean: 7.53) were reviewed. There were 1534 males (56%) and 1209 females (44%). Tonsillectomy and adenoidectomy were performed together on 1930 patients (70%) while tonsillectomy and adenoidectomy alone were performed on 287 (10%) and 526 (20%) patients, respectively. Evaluation of the pathology reports revealed no malignancies. Review of the literature identified 14 articles and 5 of them included only pediatric patients. The rate of unexpected malignancies observed in these pediatric series varied between 0 and 0.18%. CONCLUSION: After being evaluated by an experienced otolaryngologist, pathologic evaluation of all specimens may not be necessary if a child undergoing routine tonsillectomy and/or adenoidectomy is not found to have certain preoperative risk factors.     

Laryngotracheal reconstruction in infants and children: are single-stage anterior and posterior grafts a reliable intervention at all pediatric hospitals?

Objective

To review outcomes of pediatric laryngotracheal stenosis treated by single-stage laryngotracheal reconstruction with anterior and posterior cartilage grafts and compare decannulation rate for single-stage laryngotracheal reconstruction with rates published at larger (>200 beds) pediatric tertiary care hospitals.

Methods

A 4-year retrospective chart review (2004–2008) of all patients undergoing procedures coded with 2008 CPT codes 31582 (laryngoplasty for laryngeal stenosis with graft or core mold, including tracheotomy) and 31587 (laryngoplasty, cricoid split) for a pediatric, tertiary-care hospital. Interventions were single-stage laryngotracheal reconstruction with anterior and posterior cartilage grafts, and the main outcome measure was the decannulation rate after single-stage laryngotracheal reconstruction.

Results

We identified 44 patients with subglottic stenosis, of whom 13 underwent single-stage laryngotracheal reconstruction with anterior and posterior cartilage grafts. The mean age at surgery was 2.2 years (range, 5 months to 4 years). Twelve of 13 children had Cotton-Myer grade III stenosis. Ninety-two percent (12 of 13) of children remain decannulated. The mean follow up was 52 months.

Conclusions

Single-stage laryngotracheal reconstruction with anterior and posterior cartilage grafts appears to be a safe and effective technique for managing patients with high-grade subglottic stenosis at intermediate size children's hospitals. Our overall decannulation rate of 92% compares favorably to that reported in the literature (84–96%).     

Acute supraglottitis in adults: What's the optimal airway intervention?

Objectives

To validate the Friedman stage and define the proper timing for airway intervention in adult cases of acute supraglottitis, we evaluated the clinical courses and management of adult patients.

Methods

202 adult patients with acute supraglottitis were included in this study. The diagnosis of supraglottitis was established by flexible nasolaryngoscopic examination. Friedman stages were classified in each patient. In this study, the first three days of serial nasolaryngoscopic findings for each patient were evaluated.

Results

All patients were treated successfully with only conservative methods. There were no significant differences between patients with Friedman stages I or II/III. Among the patients that had serial nasolaryngoscopic findings, only one patient presented with progressive swelling of the epiglottis, and there were no cases of persistent swelling.

Conclusion

The airway intervention threshold should be raised from Friedman stages II–III. And, airway intervention should not be needed if patients are tolerant of their respiratory discomfort.     

Auditory neuropathy: endocochlear lesion or temporal processing impairment? Implications for diagnosis and management

BACKGROUND/OBJECTIVE: Auditory neuropathy/dys-synchrony, characterized by absent auditory brainstem responses, normal otoacoustic emissions or cochlear microphonics, and word discrimination disproportional to the pure-tone audiogram, may be accompanied by perceptual consequences that could jeopardize language acquisition in affected children. However, the related evidence is constantly changing leading to a serious debate. The aim of the present paper is to review the current knowledge on auditory neuropathy/dys-synchrony, and to present the therapeutic strategies that can be employed in its management, taking into account the potentially underlying pathophysiology. MATERIALS/METHODS: Literature review from Medline and database sources. Related books were also included. STUDY SELECTION: Controlled clinical trials, prospective and retrospective cohort studies, nested-based case-control and analytical family studies, laboratory and electrophysiological studies, animal models, case-reports, joint statements and review articles. DATA SYNTHESIS: Auditory neuropathy/dys-synchrony, in contrast to what is widely believed, is a very frequent disease, responsible for approximately 8% of newly diagnosed cases of hearing loss in children per year. Hyperbilirubinemia and hypoxia represent major risk factors, whereas generalized neuropathic disorders, or a genetic substrate involving the otoferlin gene, are responsible for the phenotype of auditory neuropathy/dys-synchrony in certain cases. Auditory nerve myelinopathy and/or desynchrony of neural discharges are the most probable underlying pathophysiologic mechanisms. Genetic testing may be helpful in cases of non-syndromic prelingual children. Auditory neuropathy/dys-synchrony management aims at restoring the compromised processing of auditory information, either through conventional amplification and/or alternative forms of communication, or by cochlear implantation (combined with intensive speech and language therapy). CONCLUSION: Auditory neuropathy/dys-synchrony is more frequent than considered in the past, especially amongst hearing-impaired children. Accurate diagnosis, based on subjective and objective hearing assessment techniques (including the various electrophysiological assessment measures), and timely treatment of the affected children is of paramount importance, with hearing aids, intensive speech and language therapy (and sign language when indicated) providing the mainstay of habilitation, and cochlear implantation representing a valid therapeutic alternative.     

The role of mitomycin in the prevention and treatment of scar formation in the pediatric aerodigestive tract: friend or foe?

OBJECTIVE: To evaluate the role of mitomycin in the prevention and treatment of scar formation in the pediatric aerodigestive tract. DESIGN: Prospective study; institutional review board-approved clinical trial. SETTING: Tertiary care pediatric medical center. PATIENTS: Fifteen patients; choanal atresia in 5 patients, airway stenosis in 8 patients, hypopharyngeal stenosis in 1 patient, and esophageal stenosis in 1 patient. OUTCOME: The efficacy and safety of mitomycin in the prevention of scar formation. INTERVENTION: All patients underwent surgical repair of the stenotic area, followed by topical application of mitomycin (1 mL of 0.4 mg/mL) for 4 minutes. RESULTS: Ten patients (67%) showed major improvement, 4 patients (27%) showed minor improvement, and 1 patient (7%) showed no improvement. CONCLUSION: Topical application of mitomycin can play an effective role in the prevention and treatment of scar formation in the aerodigestive tract.     

Auditory neuropathy in hyperbilirubinemia: is there a correlation between serum bilirubin, neuron-specific enolase levels and auditory neuropathy?

This study evaluated whether a correlation exists between increased serum bilirubin and neuron-specific enolase (NSE) assays (a biochemical index of neuronal damage) and auditory neuropathy. Nineteen term neonates without hemolysis whose serum bilirubin levels were above 20 mg/dl and 27 healthy term newborns with bilirubin levels <13 mg/dl were included in the study. Auditory brainstem responses (ABRs) and transient evoked otoacoustic emissions (TEOAEs) of patients with hyperbilirubinemia were obtained before discharge. This preliminary study did not show any correlation between the serum NSE and bilirubin values. However, infants who had auditory neuropathy had significantly higher NSE levels, and thus these patients, being in the high-risk group, need close follow-up.     

Otitis media: Is precursor of delayed reading in Arabic speaking children?

Objective

To investigate the relation between otitis media in the language acquisition years and the occurrence of delayed reading between the ages of 7 and 10.

Method

Participants were 40 children, half of whom had a history of otitis media between the ages of birth and three years and half who were free of the disease. These children, now ages 7–10, were tested with the Stanford Beint and Arabic Dyslexia Assessment Test.

Results

Children with a history of otitis media scored over a year below grade level in reading and significantly below controls on Arabic Dyslexia Assessment tests as well as on the Verbal IQ factor on the Stanford Binet.

Conclusions

Children with early onset otitis media (birth to three years) tend to be at greater risk for delayed reading than age-matched controls.     

Resonance frequency analysis of osseo-integrated implants for bone conduction in a pediatric population – A novel approach for assessing stability for early loading

Objectives

The purpose of our study was to directly measure the stability of a bone anchored hearing device (BAHD) abutment using resonance frequency analysis (RFA) in a pediatric population. RFA was used to guide early loading of the abutment following single stage surgery.

Methods

The principle behind RFA is to obtain a numerical value relating to stability. A Smartpeg (1 cm commercially manufactured attachment) is screwed onto the abutment – its resonance in a magnetic field is measured with an Osstell recording device. The degree of movement (vibration) is inversely proportional to the stability of the abutment and a numerical figure, the Implant Stability Quotient (ISQ), is derived. RFA measurements were obtained at surgery, 4 weeks and 16 weeks post implant surgery. Patients were fitted with the new CochlearTM Baha^® BI300 series implant using a one-stage procedure and based on RFA stability measures, loading of the sound processor occurred any time from 1 week after implant surgery if the RFA measure was 60 units or over.

Results

Twenty two consecutive patients were recruited. Eight patients had bilateral BAHD's fitted giving a total of 30 implants. The age range was 2–16 years with an average age of 9 years at time of fitting. The time interval from surgery to loading the processor ranged from 1 to 16 weeks, with an average time of 6 weeks. The mean ISQ value at time of surgery was 61.29 (95% CI = 2.03), at 4 weeks was 61.92 (95% CI = 2.97) and at 16 weeks was 63.45 (95% CI = 3.18).

Conclusions

Our study shows we have been able to operate a successful program of earlier BAHD loading using single stage surgery in children. This is supported by favorable RFA measures of implant stability with average ISQ values of over 60 units.     

Auditory neuropathy in hyperbilirubinemia: is there a correlation between serum bilirubin,neuron-specific enolase levels and auditory neuropathy?

This study evaluated whether a correlation exists between increased serum bilirubin and neuron-specific enolase (NSE) assays (a biochemical index of neuronal damage) and auditory neuropathy. Nineteen term neonates without hemolysis whose serum bilirubin levels were above 20 mg/dl and 27 healthy term newborns with bilirubin levels <13 mg/dl were included in the study. Auditory brainstem responses (ABRs) and transient evoked otoacoustic emissions (TEOAEs) of patients with hyperbilirubinemia were obtained before discharge. This preliminary study did not show any correlation between the serum NSE and bilirubin values. However, infants who had auditory neuropathy had significantly higher NSE levels, and thus these patients, being in the high-risk group, need close follow-up.     

‘Hemostatic pause’ in pediatric tonsillectomy?

A randomized prospective study was performed on 101 children undergoing dissection tonsillectomy in two different sequences. In the ‘pause’ sequence, a period of inactivity lasting 1.5 min (‘hemostatic pause’) with the Boyle-Davis gag relaxed and the fossae packed with gauze swabs was implemented after the tonsils were excised. Hemorrhage was controlled exclusively by ligatures. The duration of tonsillectomy and the number of ligatures used were accurately recorded. The procedure was identical in the ‘no pause’ group but the pause period was omitted. No reactionary haemorrhage occurred. There was no significant difference in the operating time between the two groups, but the mean number of ligatures required was significantly reduced in the ‘pause’ sequence. We conclude that ‘hemostatic pause’ in tonsillectomy reduces the amount of ligatures needed for satisfactory hemostasis.