Bilateral cavernous sinus thrombosis complicating acute unilateral pansinusitis in a 15-year-old boy.

Cavernous sinus thrombosis (CST) is a rare and potentially fatal complication of acute sinusitis. Timely diagnosis and management is, therefore, essential in preventing death and neurological disability. Here, we describe the case of a paediatric patient with bilateral CST secondary to acute unilateral pansinusitis that presented with rapidly progressing bilateral periorbital oedema. Initial imaging was negative. This case serves to emphasise the importance of maintaining a high index of suspicion when managing paediatric patients with suspected CST with persistent symptoms. Expeditious investigation and management of our patient in this case resulted in a positive outcome, with resolution of symptoms and no residual neurological deficit.

Long-term quality of life in children with complex needs undergoing cochlear implantation.

OBJECTIVES: An estimated 30-40% of children undergoing cochlear implantation (CI) have additional disabilities and traditional outcome measures are often not reliable or appropriate. Few studies have evaluated long-term quality of life (Qol) in this group. Our aim was to assess long term QoL of children implanted with complex needs against a cohort of children implanted without. We also compared both groups for secondary outcome measures such as parental opinion, complications and hours of device wear. METHODS: A retrospective casenote review of all children undergoing assessment between 2005 and 2010 was undertaken. Children with complex needs were identified using the Scottish Complex Needs Group Criteria (ISD 2006). Quality of life was measured using the GCBI (Glasgow Children's Benefit Inventory) which is a validated questionnaire suitable for the retrospective assessment of health-related QoL following paediatric surgical interventions. It consists of a 200-point scale which ranges from -100 (maximal harm) to +100 (maximal benefit) and can be analysed in four QoL subdomains (health, vitality, physical, learning). Parents were also asked to complete a simple questionnaire asking if they would recommend CI to other families and whether they would wish their child to undergo CI if given the option again. RESULTS: 174 children were implanted and 17 (10%) were found to have complex needs. Follow-up ranged from 4 to 9 years. Total GCBI scores were similarly high between groups but significantly higher scores were found in the emotion (p = 0.05) and learning domains (p = 0.03) in the complex needs group. Linear regression analysis was used to show that total GCBI score was not influenced by the number of categories of impairment (R2 = 0.0123) in the complex needs group. All parents of children with complex needs said that they would proceed with implantation again or recommend to a friend. The complication rate in the complex needs group was higher (29 vs 15%), with device failure being particularly more common. Data-logging was consistent between both groups. A higher rate of development of significant other health issues that could affect outcome was found in the complex needs group (35% vs 8%). CONCLUSIONS: Our study suggests that children with complex needs have comparable long-term QoL scores to those without and seem to particularly benefit in the domains of learning and emotion. Complications are higher in this group and there remains an increased risk of further development of medical conditions following early implantation, which may influence progress.

Case 266: Pontine Tegmental Cap Dysplasia.

History A 1-year-old boy was referred for cochlear implant assessment after he received a diagnosis of bilateral profound sensorineural hearing loss at neonatal hearing screening shortly after birth. The child was born at term via uneventful delivery, and there was no history of familial hearing loss or maternal illness. Tympanic membranes were normal, and hearing loss was confirmed with auditory brainstem testing, which showed no response from either ear. Hearing aids were provided from 3 months of age, but no behavioral responses were noted when these were worn. He was also noted to have some mild developmental delay throughout his 1st year of life and was slow to crawl, roll over, and stand up. Physical examination showed no syndromic features or physical abnormalities. Ophthalmology confirmed normal vision and visual movements but bilateral anesthetic corneas. He had corneal abrasions due to minor repeated corneal trauma, and left-sided tarsorraphy was performed at 6 months. Facial nerve function, swallow, and voice quality were normal. To assess suitability for a cochlear implant, the patient underwent MRI of the temporal lobe and brain and thin-section CT of the temporal bones. The patient subsequently underwent left cochlear implantation.

Case 266.

History A 1-year-old boy was referred for cochlear implant assessment after he received a diagnosis of bilateral profound sensorineural hearing loss at neonatal hearing screening shortly after birth. The child was born at term via uneventful delivery, and there was no history of familial hearing loss or maternal illness. Tympanic membranes were normal, and hearing loss was confirmed with auditory brainstem testing, which showed no response from either ear. Hearing aids were provided from 3 months of age, but no behavioral responses were noted when these were worn. He was also noted to have some mild developmental delay throughout his 1st year of life and was slow to crawl, roll over, and stand up. Physical examination showed no syndromic features or physical abnormalities. Ophthalmology confirmed normal vision and visual movements but bilateral anesthetic corneas. He had corneal abrasions due to minor repeated corneal trauma, and left-sided tarsorraphy was performed at 6 months. Facial nerve function, swallow, and voice quality were normal. To assess suitability for a cochlear implant, the patient underwent MRI of the temporal lobe and brain ( Figs 1 - 4 ) and thin-section CT of the temporal bones ( Figs 5 , 6 ). The patient subsequently underwent left cochlear implantation. Figure 1: Sagittal midline T1-weighted 1.5-T MR image (repetition time msec/echo time msec, 541/15). Figure 2: Axial T2-weighted MR image (6703/116, 4-mm section thickness) at the level of the pontomesencephalic junction. Figure 3: Axial T2-weighted MR image one level inferior to that shown in Figure 2 . Figure 4a: (a) Axial 1.5-T single-slab three-dimensional (3D) turbo spin-echo MR image (1200/271) at the level of the right internal auditory canal. (b) Corresponding axial 1.5-T single-slab 3D turbo spin-echo MR image at the level of the left internal auditory canal. Figure 4b: (a) Axial 1.5-T single-slab three-dimensional (3D) turbo spin-echo MR image (1200/271) at the level of the right internal auditory canal. (b) Corresponding axial 1.5-T single-slab 3D turbo spin-echo MR image at the level of the left internal auditory canal. Figure 5: Unenhanced axial thin-section (0.7-mm section thickness) CT image of the temporal bone at the level of the internal auditory canals and middle cochlear turns. Figure 6: Unenhanced axial thin-section (0.7-mm section thickness) CT image of the temporal bone at a level slightly inferior to that shown in Figure 5 .

Cochlear implantation and alcohol misuse.

OBJECTIVE: To assess the outcome of patients referred to our national centre for cochlear implantation who have a history of alcohol misuse. METHODS: A retrospective case-note review from 1989 - current was performed. Information was collected on aetiology/duration of deafness, current alcohol habits, and co-morbidities. For those implanted, we assessed implant type, anaesthetic, and complications. Our team rated each patients' engagement with the rehabilitation programme and collected 9-month post-implant Bamford-Kowal-Bench (BKB) and 1-year post-implant categories of auditory performance (CAP) scores. RESULTS: Fourteen patients with a history of harmful drinking or alcohol dependency were identified. Aetiology of deafness was varied but most commonly included sensorineural hearing loss of unknown aetiology (7), neomycin ototoxicity (3), and head injury (2). Twelve were suitable for implantation and 11 have been successfully implanted. Three patients experienced a post-operative complication (one major/two minor). Most (eight) were rated as having good engagement with the rehabilitation process and three rated as average. Nine-month post-implant BKB scores ranged from 10 to 92 (mean 56). One-year post-implant CAP scores range from 4 to 8. CONCLUSION: Patients with a background of alcohol misuse are being increasingly referred to our service. Alcohol is often implicated in the aetiology of deafness or can be sequelae of the psychological issues sometimes experienced by deaf patients. Even in the case of active alcohol misuse at the time of implantation, all surviving patients are currently active users and our results show that these patients can do well following implantation with the right support.

Standardising reporting of cervical lymphadenopathy in paediatric neck ultrasound: a pilot study using an evidence-based reporting protocol.

OBJECTIVES: Cervical lymphadenopathy is common in children and can arise from a wide range of aetiologies. Ultrasound can be a useful imaging tool for initial investigation but is known to be operator dependent. We aimed to compare the content of ultrasound reporting in this clinical scenario before and after the introduction of an evidence-based reporting protocol. METHODS: We performed a prospective 8-month pilot study assessing the content of ultrasound reports generated from scans to investigate suspected cervical lymphadenopathy in children referred to our tertiary referral otolaryngology service. We found wide variation in report content and inconsistent reporting of certain radiological features. In response to this we performed a literature search to identify key, clinically relevant ultrasonographic features for cervical lymphadenopathy and then in consultation with our radiology colleagues, devised a protocol to facilitate the reporting of these key features. Content of reports was then prospectively re-audited over a further 8-month period. RESULTS: 23 reports were assessed before and 26 after introduction of the reporting protocol. Fisher's exact test was used to analyse the data. We found a statistically significant (p < 0.05) improvement in the frequency of reporting of various key features such as nodal distribution, shape, echogenicity, calcification, necrosis and vascular pattern. CONCLUSIONS: The introduction of a standardised protocol has helped to streamline the reporting of ultrasounds to investigate cervical lymphadenopathy within our department. In the absence of any national guidelines on the reporting of paediatric neck ultrasound in this scenario, we propose that our protocol could be used by other departments to improve standardisation and as a teaching aid.

Development of a screening service for neonatal ear deformity using neonatal hearing screeners and an information leaflet.

OBJECTIVES: Early splinting of neonatal ear deformities has been proven to be successful but the opportunity to splint is frequently missed due to lack of awareness amongst healthcare personnel. We aimed to develop a regional screening service using neonatal hearing screeners and an information leaflet to allow for the early detection and treatment of such children. METHODS: We created an information leaflet that was distributed by hearing screeners to all parents in Greater Glasgow at the time of the child's neonatal hearing assessment, with a contact number allowing parents to self refer. All neonates referred were seen at a dedicated clinic within a week and suitability for splints determined. We aimed to assess acceptability of the service, splinting result as rated by parents and otolaryngologist and also costs involved. RESULTS: Over a 15 month period, 13,403 leaflets were distributed. 88 babies were referred (0.7%) and 54 were found suitable for splinting. 78% of parents rated the efficacy of splints as either excellent or very good and 96% said they would recommend the service to a friend. Median age at first review was 4 days. We found a weak but statistically significant correlation between age at first review and the surgeon rated outcome from splinting (Spearman's rho=-0.321, p=0.038), with those babies commencing treatment early generally having a better splinting result. We also found that age at first review correlated with duration of splinting required (Spearman's rho=0.357, p=0.008), with younger babies generally requiring shorter splinting times. Cost analysis revealed a saving of £482.76 per child when comparing splint treatment to potential later corrective ear surgery costs. CONCLUSIONS: Our screening service is both acceptable to parents and efficient in allowing for early correction of ear deformity in the majority of cases. By detecting treatable children early, we propose that the introduction of routine screening and splinting on a wider basis will avoid the psychological burden of ear deformity in childhood and also avoid the need for later corrective surgery.

Severe laryngeal dysplasia in a 20-year-old nonsmoker treated with CO2 laser excision: a case report and review of the literature.

OBJECTIVE: We present the unusual case of a 20-year-old female nonsmoker with severe laryngeal dysplasia managed with endoscopic CO(2) laser resection with frozen-section analysis. We use her case as a platform for discussion of the current evidence surrounding laryngeal dysplasia, particularly etiology and the use of lasers in management. BACKGROUND DATA: Laryngeal dysplasia represents a series of progressive epithelial changes that can eventually lead to invasive laryngeal carcinoma. Typically, most patients are male, in their 50s or 60s, and are smokers; to our knowledge our patient represents the youngest reported case of this condition in a woman with no established risk factors. We discuss the implications of this on her subsequent management with the CO(2) laser. METHODS: With the help of intraoperative photographs, we describe our excisional technique with the CO(2) laser, model 315M Superpulse class 4 (Irradia AB, Stockholm, Sweden) at a 6-W continuous power setting. RESULTS: At 6 months after surgery, our patient remains disease free with good voice quality. CONCLUSIONS: Our patient appears to defy all the usual etiologic stereotypes that usually govern the development of laryngeal dysplasia. CO(2) laser excision plus intraoperative frozen-section analysis remains a safe and reliable method for the treatment of laryngeal dysplasia; the reassurance of histologically clear margins minimizes the extent of radical laser excision necessary and provides good functional results. We provide a comprehensive overview of the current literature on laryngeal dysplasia etiology and treatment, particularly the use of lasers in management.

A double-blind randomized controlled trial of management of recurrent nosebleeds in children.

BACKGROUND: To establish whether a treatment regimen of silver nitrate cautery and 4 weeks of antiseptic nasal cream is superior to antiseptic cream treatment alone in the management of pediatric epistaxis. STUDY DESIGN: Double-blind randomized controlled trial. SUBJECTS AND METHODS: Children with epistaxis and visible anterior septal vessels were invited to participate. Patients were randomized to receive treatment or control. Treatment patients received silver nitrate cautery, followed by antiseptic cream for 4 weeks. Control patients received sham cautery followed by antiseptic cream for 4 weeks. RESULTS: A total of 109 patients were randomized and results were available for 93 (85%). Of those receiving cautery, 21 (45.7%) of 46 had no bleeding in the 4 weeks before follow-up. Of those receiving only antiseptic cream 14 (29.8%) of 47 had no bleeding. (chi(2) = 2.49; P = 0.114). More children in the active treatment group had an improvement in their symptoms compared with controls (42 of 46; 91.3%) in the treatment group vs 33 of 47 (70.2%) controls (chi(2) = 6.626; P = 0.01; relative risk reduction = 71 percent, number needed to treat = 4.7). CONCLUSION: When using subjective improvement in symptoms as the outcome measure, silver nitrate cautery with antiseptic cream twice daily for 4 weeks appears to give a small but statistically significant benefit when compared to antiseptic cream alone.

Childhood epistaxis and nasal colonization with Staphylococcus aureus.

OBJECTIVE: Epistaxis is very common in children but its cause remains unknown. We postulate that nasal colonization with Staphylococcus aureus leads to inflammation, crusting, and ultimately new vessel formation. STUDY DESIGN: A prospective case-control study. SUBJECTS AND METHODS: Sixty-seven children were recruited, 42 with epistaxis (22 had crusting in the nasal vestibule; 20 did not) and 25 control subjects. A microbiology swab was taken from the anterior nasal cavity of each child. RESULTS: All groups were equally likely to have a positive culture. S aureus was more common in the epistaxis group (P = 0.008) compared with the control group. There was no difference in the prevalence of S aureus between crust and noncrust groups. Epistaxis patients were much less likely to have isolates of respiratory pathogens or a skin commensal. CONCLUSION: Children with epistaxis are more likely to have nasal colonization with S aureus than controls. Our data would support the hypothesis that S aureus replaces existing nasal flora and causes inflammation and new vessel formation.

Atypical mycobacterial infection of the head and neck in children: a 5-year retrospective review.

OBJECTIVE: We review the management of 31 cases of atypical mycobacterial lymphadenitis presenting to a tertiary referral pediatric otolaryngology department between February 2002 and February 2007. STUDY DESIGN: Retrospective case review. SUBJECTS AND METHODS: Cases were identified by using the hospital's correspondence and pathology databases. Demographic and clinical information was collected. Descriptive and nonparametric inferential statistics (SPSS; SPSS Inc, Chicago, IL) were calculated. RESULTS: Four patients were treated conservatively with triple-therapy antibiotics. Twenty-seven underwent surgical excision plus 3 months of clarithromycin postoperatively. Thirteen percent developed nodal recurrence; this appeared to be unrelated to the operation performed (chi(2)(8) = 5.8, P = 0.69) or the management modality used (P = 0.35). Patients with previous surgery were more likely to have recurrent infection (chi(2)(2) = 9.3, P = 0.01). CONCLUSIONS: In our experience, nodal excision plus postoperative clarithromycin remains the best treatment option for most children, although those with limited disease may benefit from combination antibiotic therapy. Previous incision and drainage leads to high recurrence rates after later excision and therefore, heightened awareness of these infections is essential to ensure appropriate early management.

Rare case of non-refluxing non-obstructive megaureter in an adult.

Non-refluxing non-obstructive megaureter is a rare condition, usually diagnosed in asymptomatic neonates. We present the case of a 39-year-old male, who presented with a 6-month history of recurrent UTIs and intermittent frank haematuria. IVU, flexible cystoscopy and micturating cystogram confirmed characteristically dilated ureters, without evidence of reflux or obstruction. We review the aetiology, investigation and management of this rare condition.