CHronic Rhinosinusitis Outcome MEasures (CHROME), developing a core outcome set for trials of interventions in chronic rhinosinusitis.

STATEMENT OF PROBLEM: Evaluating the effectiveness of treatments in chronic rhinosinusitis (CRS) have been limited by both a paucity of high quality randomised trials, and the heterogeneity of outcomes in those that have been reported. Core outcome sets (COS) are an agreed, standardized set of outcomes that should be measured and reported by future trials as a minimum and will facilitate future meta-analysis of trial results in systematic reviews (SRs). We set out to develop a core outcome set for interventions for adults with CRS. METHOD(S) OF STUDY: A long-list of potential outcomes was identified by a steering group utilising a literature review, thematic analysis of a wide range of stakeholders' views and systematic analysis of currently available Patient Reported Outcome Measures (PROMs). A subsequent e-Delphi process allowed 110 patients and healthcare practitioners to individually rate the outcomes in terms of importance, on a Likert scale. MAIN RESULTS: After 2 rounds of the iterative Delphi process, the 54 initial outcomes were distilled down to a final core-outcome set of 15 items, over 4 domains. PRINCIPAL CONCLUSIONS: The authors hope inclusion of these core outcomes in future trials will increase the value of research on interventions for CRS in adults. It was felt important to make recommendations regarding how these outcomes should be measured, although additional work is now required to further develop and revalidate existing outcome measures.

Mucopolysaccharidosis I; Parental beliefs about the impact of disease on the quality of life of their children.

BACKGROUND: Hematopoietic stem cell transplants, alongside enzyme replacement therapy and good multi-disciplinary care, have dramatically improved the life expectancy in children with Mucopolysaccharidosis (MPS) I, with better objective and functional outcomes. Despite these improvements, children with both the attenuated (non-Hurler) and severe (Hurler) variants of the disease have marked residual morbidity. Children with MPS I suffer with head and neck disease including obstructive sleep apnoea and hearing loss. The impact of these on quality of life has been poorly researched and no previous work has been published looking at patients' perception of their own health, an important domain when considering the impact of treatment. METHODS: This exploratory qualitative study aimed to discover the effect of head and neck disease, alongside that of MPS I as a whole, on the quality of life of affected children. A grounded theory approach was used to conduct this study. Children and their parents were invited to participate in semi-structured interviews. The transcribed interviews were coded and emergent themes explored until saturation occurred. RESULTS: The families of eleven children with MPS I were interviewed, five with Hurler's and six with the attenuated non-Hurler's. Important themes to emerge were- the fear of dying associated with obstructive sleep apnoea, difficulties communicating at school due to the delayed acquisition of language, chronic pain and restricted mobility, physical differences and restricted participation in social activities such as sports secondary to the musculoskeletal disease burden. The overall theme running through the analysis was the desire to fit in with ones peers. CONCLUSION: Parents and children with MPS 1 worry about 'fitting-in' with broader society. The presence of airway disease has a profound impact on the emotional well being of parents whilst language delay and musculoskeletal disease have the biggest impact on the quality of life of the children themselves. It is important to understand the impact of MPS I on the quality of life of children and their families so that we may improve future treatment and management of this sub-group of children who have an increasing life span.

Subcranial craniofacial resection for advanced sinonasal malignant tumours involving the anterior skull base.

BACKGROUND: The subcranial approach is a modification of traditional craniofacial resection. It provides similar broad access to the anterior skull base, but with lower mortality and morbidity. It has been the surgical technique of choice at our institution since 2006 for treating advanced stage sinonasal tumours (American Joint Committee on Cancer stage III or above). This paper reports our experience and outcomes. METHOD AND RESULTS: Eighteen patients underwent subcranial craniofacial resection over a seven-year period, this being combined with a second adjunctive procedure in 89 per cent of cases. Forty per cent of patients required reconstruction of the primary defect. No peri-operative deaths occurred. One patient had a transient cerebrospinal fluid leak. The major complication rate was 33 per cent, of which 67 per cent were directly related to soft tissue reconstruction. Tumour recurrence rate was 17 per cent and the five-year disease-free survival estimate was 40 per cent. CONCLUSION: The subcranial approach is a safe and effective technique that may be used to successfully treat advanced sinonasal malignancies with anterior skull base extension.

The impact of commissioning for rhinosinusitis in England.

OBJECTIVES: To assess the compliance of clinical commissioning groups (CCGs) in England with the ENT-UK rhinosinusitis commissioning guide produced in collaboration with the Royal College of Surgeons England and the National Institute of Clinical Excellence. We also aimed to assess the ease of accessibility of data from CCGs. DESIGN: Audit of compliance of English CCGs with the ENT-UK rhinosinusitis commissioning guide. SETTING: CCGs in England PARTICIPANTS: A total of 58 of the 221 CCGs in England were included and chosen because they were the first CCGs authorised by NHS England, or alternately, the CCGs forecasted to have a deficit in their first year of operation. Their websites were reviewed; when information was not easily accessible, a freedom of information request was submitted to the relevant CCG. MAIN OUTCOME MEASURES: Compliance with commissioning guidelines for rhinosinusitis. RESULTS: Thirteen percent of CCGs had restrictive referral criteria in place, largely unrelated to published evidence-based guidance. The routine use of multiple courses of oral steroids, prescription of antibiotics, CT scanning within primary care, and delaying referral for a year, prior to referral to a specialist were recommended against published advice. CONCLUSIONS: Restricting access to surgery may contribute to poorer outcomes and a decrease in the patient's quality of life. This is against the NHS constitution and is open to legal challenge. We encourage all ENT surgeons to review policies of their local CCG and engage with commissioners to ensure that their patients have evidence-based care.

Patient-reported outcomes in children suffering with mild to moderate tonsillitis versus those in children with severe tonsillitis.

OBJECTIVE: To quantify the impact that mild to moderate tonsillitis has on quality of life in children and compare it to that of severe tonsillitis. METHODS: In this prospective study, parents of children aged 0-16 years completed the Paediatric Throat Disorders Outcome Test, and quality of life scores in the tonsillitis groups were compared. RESULTS: A hundred children were recruited: 58 had severe tonsillitis and were offered surgery, and 42 had mild to moderate tonsillitis and were managed conservatively. The mean outcome test scores in those children undergoing surgery were 36.7 for tonsillectomy patients and 36.9 for adenotonsillectomy patients, compared with a score of 31.5 for the mild to moderate tonsillitis patients (p = 0.019). CONCLUSION: Children with mild to moderate disease had significantly better quality of life scores than those with severe disease. It is thought that those with mild to moderate disease have short-term improvements in general quality of life after surgery, which disappear in the medium term. This transient improvement needs to be balanced against the morbidity of the surgery and the cost burden to the National Health Service. The results of this study support the national drive towards limiting tonsillectomy to children with severe tonsillitis or obstructive sleep apnoea.

A natural obturator in hereditary haemorrhagic telangiectasia.

OBJECTIVE: Most patients with hereditary haemorrhagic telangiectasia suffer with frequent episodes of epistaxis. The aim of this case report is to highlight the effect on epistaxis, occurring in hereditary haemorrhagic telangiectasia, when nasal airflow ceases. CASE REPORT: We present the interesting case of a patient with hereditary haemorrhagic telangiectasia who experienced cessation of her recurrent, refractory epistaxis through the development of coexisting polyp disease. The patient's enlarged, grade three nasal polyps were behaving as physiological obturators, limiting airflow through her nose. This reduced the intranasal trauma and subsequent frequency of her nosebleeds. CONCLUSION: Epistaxis is a debilitating part of hereditary haemorrhagic telangiectasia, and poses a frequent management challenge. Our patient was more tolerant of her grade three nasal polyps than of her recurrent epistaxis. This case highlights the importance of reducing nasal airflow when treating patients with hereditary haemorrhagic telangiectasia.