Effect of intratympanic steroid administration on sensorineural hearing loss associated with acute otitis media.

OBJECTIVE: To evaluate a small cohort of patients who presented with symptoms and signs consistent with acute infective sensorineural hearing loss who were treated with intratympanic steroids. METHOD: Seven patients received a 7-day course of oral antibiotics and oral prednisolone followed by 3 intratympanic injections of methylprednisolone and 1 week of topical dexamethasone drops. RESULTS: Hearing improved in 57 per cent of patients (four out of seven). The mean improvement in this group was 24 dB (range, 10-52 dB). The magnitude of the sensorineural hearing loss at presentation was less in those who responded to intratympanic steroid therapy than in non-responders (mean pure tone average of 30 dB versus 65 dB pre-intratympanic steroids, and 14 dB versus 83 dB post-intratympanic steroids, respectively). CONCLUSION: The results of our study suggest that intratympanic steroids provide a valuable contribution to the treatment of acute infective sensorineural hearing loss and may provide additional benefit by virtue of a concentrated local steroid effect in patients who do not respond to antibiotics.

Comparison of quality of life outcomes following different mastoid surgery techniques.

BACKGROUND: Mastoid surgery carried out to treat chronic otitis media can lead to improvement in objective and subjective measures post-operatively. This study investigated the subjective change in quality of life using the Glasgow Benefit Inventory relative to the type of mastoid surgery undertaken. METHOD: A retrospective multicentre postal survey of 157 patients who underwent mastoid surgery from 2008 to 2012 was conducted. RESULTS: Eighty-three questionnaire responses were received from patients who underwent surgery at one of three different hospitals (a response rate of 53 per cent). Fifty-seven per cent of patients had a Glasgow Benefit Inventory score of 0, indicating no change in quality of life post-operatively. Thirty-five per cent scored over 50, indicating significant improvement. The only significant difference found was that women fared worse after surgery than men. CONCLUSION: The choice of mastoid surgery technique should be determined by clinical need and surgeon preference. There is no improvement in quality of life for most patients following mastoid surgery.

Great Ormond Street Hospital treatment guidelines for use of propranolol in infantile isolated subglottic haemangioma.

OBJECTIVE: Treatment options for large subglottic haemangioma include steroids, laser ablation, open excision, tracheostomy and, more recently, propranolol. This article aims to present the Great Ormond Street Hospital guidelines for using propranolol to treat infantile isolated subglottic haemangioma by ENT surgeons. METHODS: The vascular malformations multidisciplinary team at Great Ormond Street Hospital has developed guidelines for treating infantile haemangioma with propranolol. RESULTS: The Great Ormond Street Hospital guidelines for propranolol treatment for infantile subglottic haemangioma include investigation, treatment and follow up. Propranolol is started at 1 mg/kg/day divided into three doses, increasing to 2 mg/kg/day one week later. On starting propranolol and when increasing the dose, the pulse rate and blood pressure must be checked every 30 minutes for the first 2 hours. Lesion response to treatment is assessed via serial endoscopy. CONCLUSION: Recent reports of dramatic responses to oral propranolol in children with haemangioma and acute airway obstruction have led to increased use. We advocate caution, and have developed guidelines (including pre-treatment investigation and monitoring) to improve treatment safety. Propranolol may in time prove to be the best medical treatment for subglottic haemangioma, but at present is considered to be still under evaluation.

Peri-operative complications after adenotonsillectomy in a UK pediatric tertiary referral centre.

OBJECTIVES: Adenoidectomy and/or tonsillectomy are commonly performed in tertiary pediatric hospitals for the management of obstructive sleep apnea, often in children with significant comorbidities. This study examines the peri-operative course of a large series of complex patients undergoing such surgery at a major pediatric centre, reporting particularly cases of respiratory compromise requiring intensive care admission, both electively and unplanned. METHODS: This study was conducted by the pediatric ENT department at Great Ormond Street Hospital. All children undergoing adenoidectomy and/or tonsillectomy from July 2003 to December 2010 were included in this study. This involved a retrospective review of the case notes and hospital databases, with particular emphasis on those children requiring admission to the pediatric intensive care unit. RESULTS: A total of 1735 consecutive admissions for adenoidectomy and/or tonsillectomy (1627 individual patients aged 4-197 months, median 46 months) were included between 2003 and 2010 (998 adenotonsillectomies, 182 tonsillectomies and 555 adenoidectomies). In this group, 999/1627 patients (61.4%) had a diagnosis of sleep disordered breathing or sleep apnea, including 258 who had polysomnography. 407/1627 (25.0%) had no specific comorbidities which were felt likely to influence their surgical outcome. Established high risk factors included age less than 24 months (292), Down syndrome (99), neuromuscular problems (314), craniofacial abnormalities (94), storage diseases (23), morbid obesity (20), cardiovascular disease (133), respiratory disease (261), hemoglobinopathy (76) and coagulophathy (34). 300/1735 admissions were day cases and 1082/1735 were observed for one night. 353/1735 required more than one night in hospital (294 for two to three nights). 7/1735 had primary hemorrhage necessitating return to the operating room, all after tonsillectomy. 41/1735 (38 with major comorbidities) required peri-operative intensive care admission, mostly for respiratory support. Of these, 7 were admitted pre-operatively to intensive care, and 17 were planned post-operative transfers. Only 17/1735 required unanticipated post-operative admission to intensive care. Odds ratio analysis suggested a significantly higher chance of PICU admission in children with particular comorbidities (Down Syndrome, cardiac disease, obesity, cerebral palsy, craniofacial anomalies, mucopolysaccharidoses and hemoglobinopathy) when compared to children without comorbidities. Adenotonsillectomy was associated with a higher risk of PICU admission than adenoidectomy alone, but patient age less than 24 months was not associated with significantly higher rates of PICU admission. There were no peri-operative mortalities in this cohort. CONCLUSIONS: The peri-operative course was largely uneventful for the majority of children undergoing surgery during this period, particularly given the high prevalence of sleep apnea and other risk factors in this cohort. Major complications were uncommon, with 2.4% of these selected, typically high risk cases requiring peri-operative intensive care admission. Importantly, only 1% of all admissions required unanticipated transfer to intensive care. This has informed changes in peri-operative management in this unit, with implications for other pediatric tertiary referral centres.

Surgical site infections in paediatric otolaryngology operative procedures.

OBJECTIVE: An assessment of the rate of surgical site infections associated with elective paediatric otolaryngology surgical procedures. METHODS: Prospective data was collected for a 3-week period for all children undergoing surgery where either mucosa or skin was breached. The parents of the children were requested to complete a questionnaire at 30 days after the operation. RESULTS: Data was collected on 80 consecutive cases. The majority of cases were admitted on the day of the procedure. The procedures included adenotonsillectomy (24), grommets (12), cochlear implantation (6), bone-anchored hearing aid (2), submandibular gland excision (1), branchial sinus excision (1), cystic hygroma excision (3), nasal glioma excision (1), microlaryngobronchoscopy (13), tracheostomy (3) and other procedures (14). Nearly half the cases had more than one operation done at the same time. 26/80 (32.5%) patients had a temporary or permanent implant inserted at the time of operation (grommet, bone-anchored hearing aid, cochlear implant). 25/80 (31%) operative fields were classed as clean and 55/80 (68.7%) as clean contaminated operations. The duration of the operation varied from 6 min to 142 min. Hospital antibiotic protocol was adhered to in 69/80 (86.3%) cases but not in 11/80 cases. In our series, 3/80 (3.7%) patients had an infection in the postoperative period. CONCLUSIONS: Surgical site infections do occur at an appreciable rate in paediatric otolaryngology. With the potential for serious consequences, reduction in the risk of surgical site infections is important.

Branchial anomalies in children.

BACKGROUND: Branchial cleft anomalies are the second most common head and neck congenital lesions seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95% of the branchial anomalies. This article analyzes all the cases of branchial cleft anomalies operated on at Great Ormond Street Hospital over the past 10 years. METHODS: All children who underwent surgery for branchial cleft sinus or fistula from January 2000 to December 2010 were included in this study. RESULTS: In this series, we had 80 patients (38 female and 42 male). The age at the time of operation varied from 1 year to 14 years. Amongst this group, 15 patients had first branchial cleft anomaly, 62 had second branchial cleft anomaly and 3 had fourth branchial pouch anomaly. All the first cleft cases were operated on by a superficial parotidectomy approach with facial nerve identification. Complete excision was achieved in all these first cleft cases. In this series of first cleft anomalies, we had one complication (temporary marginal mandibular nerve weakness. In the 62 children with second branchial cleft anomalies, 50 were unilateral and 12 were bilateral. In the vast majority, the tract extended through the carotid bifurcation and extended up to pharyngeal constrictor muscles. Majority of these cases were operated on through an elliptical incision around the external opening. Complete excision was achieved in all second cleft cases except one who required a repeat excision. In this subgroup, we had two complications one patient developed a seroma and one had incomplete excision. The three patients with fourth pouch anomaly were treated with endoscopic assisted monopolar diathermy to the sinus opening with good outcome. CONCLUSION: Branchial anomalies are relatively common in children. There are three distinct types, first cleft, second cleft and fourth pouch anomaly. Correct diagnosis is essential to avoid inadequate surgery and multiple procedures. The surgical approach needs to be tailored to the type of anomaly of origin of the anomaly. Complete excision is essential for good outcomes.

Single stage laryngotracheal reconstruction using endoscopic posterior grafting in isolated posterior glottic stenosis in children - how we do it.

OBJECTIVE: Posterior glottic stenosis is a well recognised though rare condition. A wide range of treatment options have been proposed including endoscopic laser techniques and open techniques with grafting. The objective of this study was to present our experience of single stage laryngotracheal reconstruction with endoscopic technique of placement of posterior graft in isolated posterior glottic stenosis. METHODS: This study was conducted at the Department of Paediatric Otolaryngology at Great Ormond Street Hospital, London. The senior authors (ALT, DMA) were involved in the management of two children with isolated posterior glottic stenosis operated on as single stage reconstruction. RESULTS: The two children in this small series had isolated posterior glottic stenosis. The aetiology in both the cases was previous prolonged intubation. Both patients underwent a successful placement of the posterior graft endoscopically, though it was a challenging task because of space constraints. None of these children required an intra-operative or post-operative tracheostomy. There were no complications in these patients. At follow up endoscopic examinations, both these patients have had their airway widened successfully. CONCLUSION: Single stage laryngotracheal reconstruction with endoscopic placement of posterior graft in cases with isolated posterior glottic stenosis is a good alternative to open surgical techniques, although is technically a challenging procedure.

Surgical excision as primary treatment modality for extensive cervicofacial lymphatic malformations in children.

OBJECTIVE: There has been much recent focus on sclerotherapy treatment of lymphatic malformations with OK432. Surgical treatment however can have a number of advantages, including complete curative excision. The aim of this study was to evaluate the results of surgical excision as the primary (first) treatment for this condition. This group includes a number of children with very extensive disease as well as some with smaller lesions. METHODS: Prospectively collected database with additional information from medical records of children with cervicofacial lymphangiomas treated over 10 years at a tertiary paediatric referral centre. For this study only children who underwent surgical excision as the primary treatment modality were included. RESULTS: Total of 118 children with lymphatic malformations were treated under the care of the senior author over a 10 year period. Of these 53 patients, who underwent surgical excision as the primary treatment modality for cervicofacial lymphatic malformations were included in this study. Forty-one patients who underwent sclerotherapy as the initial treatment were excluded. Also excluded were 6 patients who underwent thoracic surgery and 18 who were treated conservatively. The majority of the patients (41, 77.3%) underwent only a single surgical procedure (36 - surgical excision, 5 - laser excision). At the first follow up after the primary surgery, the result was complete resolution of symptoms in 29 patients, near complete resolution in 13 patients (together 79.3%) and partial response in 11 (19.7%) patients. Twenty-three patients with disease localised only to the neck, all (100%) had a complete or near complete resolution of the disease after the primary surgery. Complete/near complete response was achieved in 98% cases with macrocystic disease, regardless of the location. Minor complications occurred in 11.3% patients. No permanent nerve weaknesses occurred. CONCLUSION: Cervicofacial lymphatic malformations in children should be managed in a multidisciplinary setting. Surgery remains a very important treatment modality. The majority of patients (80%) in this study had complete or near complete resolution with one surgical procedure. Isolated neck lesions have the best outcomes (100% resolution in this study). Patients with macrocystic disease, achieved complete or near complete resolution in 97% of cases, regardless of the location. Some children with extensive disease will need multiple treatments. Surgical excision as the primary treatment modality in selected cases is safe and reliable technique and has good aesthetic and functional outcomes in experienced hands.

A rare cause of paediatric epistaxis: lobular capillary haemangioma of the nasal cavity.

The authors describe a case of a 14-year-old male child presenting with massive anterior epistaxis on a background of recurrent episodes of epistaxis. Immediate management constituted anterior nasal packing. Endoscopic nasal examination revealed a 5 mm purple vascular lesion anterior to the right-middle turbinate. The initial working diagnosis of juvenile nasopharyngeal angiofibroma which is most common in this population was excluded following MRI. The lesion was excised via an endoscopic approach with no complications. Histological analysis confirmed the diagnosis of lobular capillary haemangioma. The patient made an uneventful recovery and remains on follow-up with no recurrence.

Melioidosis and the vacuum-assisted closure device: a rare cause of a discharging neck wound, and a new approach to management.

OBJECTIVE: We report a case of melioidosis presenting as a discharging neck abscess, and we describe the use of a vacuum-assisted closure device in its management. METHOD: We report the case of a 44-year-old, Afro-Caribbean woman with melioidosis. We also present the results of a literature search using the search terms 'melioidosis', 'Burkholderia pseudomallei' and 'vacuum-assisted closure device'. RESULTS: Microbiological analysis identified the causative organism as being the bacterium Burkholderia pseudomallei, and its antimicrobial sensitivities to imipenem and ciprofloxacin. A vacuum-assisted closure device was used to manage the patient's melioidosis of the neck; we believe this is the first report of such treatment. CONCLUSIONS: Melioidosis is rare in the UK and western world; however, exposure can occur during travel to endemic areas. We therefore draw attention to this infection as part of the differential diagnosis of a neck abscess. We propose the use of vacuum-assisted closure devices as useful adjuncts to the management of discharging neck wounds.

New presentation of familial medullary thyroid carcinoma in 87-year-old patient with high-risk RET proto-oncogene codon 620 mutation.

OBJECTIVE: We report a case of familial medullary thyroid carcinoma in an 87-year-old woman, despite the patient having a high-risk codon 620 mutation. METHOD: Medline and PubMed were searched for cases and literature reviews relating to the following keywords: 'codon 620', 'medullary thyroid carcinoma', 'multiple endocrine neoplasia' and 'RET proto-oncogene'. RESULTS: We report the case of an 87-year-old woman who presented with a goitre, later identified as medullary thyroid carcinoma. Genetic analysis revealed a RET proto-oncogene codon 620 mutation. Genetic testing has revolutionised the management of medullary thyroid carcinoma. The genetic basis of hereditary medullary thyroid carcinoma lies with the RET proto-oncogene. Several disease-causing mutations of this gene have been identified and their clinical prognosis described. The penetrance of these mutations is high; as such, carriers progress to develop medullary thyroid carcinoma at a young age. Mutations at the codon 620 position are classified as high-risk for early development of medullary thyroid carcinoma; thus, the current recommendation is for prophylactic thyroidectomy at five years of age. CONCLUSIONS: In this case, the progress of hereditary medullary thyroid carcinoma was unique, considering the late presentation of medullary thyroid carcinoma despite the presence of the high-risk RET proto-oncogene codon 620 mutation. The authors wish to highlight the importance of this case, as it may present a counter-argument to the current recommendations for early, prophylactic thyroidectomy in codon 620 mutation carriers in order to prevent early development of medullary thyroid carcinoma.

Driving standards in tracheostomy care: a preliminary communication of the St Mary's ENT-led multi disciplinary team approach.

OBJECTIVES: To assess tracheostomy care and improve standards following the introduction of an ENT-led multidisciplinary tracheostomy ward round service. DESIGN: Prospective third cycle audit. SETTING: Tertiary academic London hospital serving an inner city population of multi-ethnic background (St Mary's Hospital, Paddington, London). PARTICIPANTS: Patients with a tracheostomy discharged from ITU to general wards. IMPLEMENTED ACTIONS: Establishment of an ENT-led Tracheostomy Multidisciplinary Team (TMDT). Weekly TMDT ward round to manage patients with a tracheostomy. ENT-led educational and training sessions for allied healthcare professionals. MAIN OUTCOME MEASURES: Compliance with local tracheostomy care guidelines (St Mary's tracheostomy care bundle) and time to tracheostomy tube decannulation. RESULTS: Preliminary results of 10 patients show improved compliance with tracheostomy care guidelines, established in 2004, rising to 94%. Average time to decannulation was significantly reduced from 21 to 5 days (P-value = 0.0005, Mann Whitney Wilcoxon Test). The mean total tracheostomy time was reduced from 34 to 24 days although this was not statistically significant (P-value = 0.13, Mann Whitney Wilcoxon Test). CONCLUSIONS: The introduction of regular ENT-led multidisciplinary input for patients with a tracheostomy significantly improved compliance with nursing care standards. There was also a reduction in the total length of time tracheostomy tubes remain in situ, with time to decannulation significantly reduced.