Publications by authors named "Piyush Jani"

39 Publications

Genetic testing for hereditary hyperparathyroidism and familial hypocalciuric hypercalcaemia in a large UK cohort.

Clin Endocrinol (Oxf) 2020 Oct 12;93(4):409-418. Epub 2020 Jun 12.

Department of Endocrinology, Cambridge University Hospital NHS Foundation Trust, Cambridge, UK.

Background: Primary hyperparathyroidism (PHPTH) is a common endocrine disorder and an estimated 10% of cases are hereditary, related to syndromes including; multiple endocrine neoplasia (MEN) type 1, MEN type 4, MEN2A and hereditary hyperparathyroidism-jaw tumour syndrome. Establishing the underlying genetic cause for PHPTH allows for personalized and cost-effective management. Familial hypocalicuric hypercalcaemia (FHH) is a benign disorder of hypercalcaemia associated with an inappropriately low urinary calcium excretion, which is quantified by the calcium creatinine clearance ratio (CCCR). Recent NHS England National Genomic Test Directory testing criteria for familial hyperparathyroidism state testing patients presenting with PHPTH and CCCR > 0.02 presenting (i) <35 years of age, or (ii) <45y with one of (a) multiglandular disease, or (b) hyperplasia on histology, or (c) ossifying fibroma(s) of the maxilla and/ or mandible, or (d) a family history of unexplained PHPTH. The testing criterion for FHH is a CCCR < 0.02.

Aims And Methods: A retrospective review of patients referred for genetic testing over a 4 year period for suspected hereditary HPTH was performed. Genetic analysis was performed by next-generation sequencing of the following genes; MEN1, CDC73, CASR, CDKN1A, CDKN1B, CDKN2B, CDKN2C, RET, GCM2, GNA11, and AP2S1 in NHS-accredited Regional Genetic laboratories. Aims of this study were to better define testing criteria for suspected hereditary PHPTH in a UK cohort.

Results: A total of 121 patients were included in this study (92 female) with a mean age of 41 years (SD 17). A pathogenic germline variant was identified in 16% (n = 19). A pathogenic variant was identified in the PHPTH genes CDC73 in a single patient and MEN1 in six patients (6% of total), in the FHH genes, CASR in 11 patients and AP2S1 in a single paediatric case (10% of total). A variant of uncertain significance (VUS) was identified in eight patients (6%) but over the course of this study familial segregation studies and computational analysis enabled re-classification of four of the variants, with two VUS's in the CASR gene being upgraded to likely pathogenic variants. Age at diagnosis and multiglandular disease as sole risk factors were not predictive of a pathogenic germline variant in this cohort but a positive family history was strongly predictive (P = .0002). A significant difference in the mean calcium creatinine clearance ratio (CCCR) in those patients with an identified CASR pathogenic variant versus those without (P = .0001) was demonstrated in this study. Thirty-three patients were aged over 50 years and the diagnostic rate of a pathogenic variant was 15.1% in those patients >50 years of age compared to 15.9% in those <50 years. Five patients >50 years and with a CCCR of <0.01, were diagnosed with a pathogenic variant in CASR.

Conclusion: Family history was the strongest predictor of hereditary PHPTH in this cohort. This study has highlighted the importance of re-evaluating VUS's in order to inform patient management and enable appropriate genetic counselling. Finally, this study has demonstrated the need to consider genetic testing for PHPTH in patients of any age, particularly those with additional risk factors.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/cen.14254DOI Listing
October 2020

Thyroidectomy in octogenarians is not associated with poorer postoperative outcomes.

Head Neck 2019 08 4;41(8):2500-2506. Epub 2019 Mar 4.

Department of Otolaryngology, Westmead Hospital, Westmead, New South Wales, Australia.

Background: The elderly represents one of the most rapidly growing subsets of the population. This population experiences a higher incidence of thyroid pathology. However, there are concerns that the elderly also experiences increased perioperative complications and are poor candidates for thyroidectomy.

Methods: Patients who underwent thyroidectomy over the age of 80 years at three tertiary head and neck units were included. Data regarding age, sex, presenting symptoms, comorbidities, preoperative investigations, type of surgery, postoperative complications, and final thyroid pathology were retrieved from hospital records.

Results: Of a total of 39 patients (69% women, mean age 83.1), the majority (40.1%) presented with a combination of symptoms: shortness of breath, feeling of pressure in the neck, dysphagia, or tiredness. Two (5.1%) were asymptomatic. Low rates of postoperative complications were encountered and were minor (n = 5). No intensive care unit admissions or mortality was experienced.

Conclusion: Thyroid surgery in octogenarians carries an acceptable complication profile.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/hed.25712DOI Listing
August 2019

A multifaceted approach to improving the quality of ENT Emergency Clinic referrals.

BMJ Qual Improv Rep 2017 20;6(1). Epub 2017 Mar 20.

Addenbrooke's Hospital, UK.

It is imperative that primary care referrals are directed to the appropriate secondary care service. Patients presenting to a primary care physician with ENT conditions may require review in an Emergency Clinic. The latter clinics provide patients with rapid access to secondary care, for urgent, yet non-life-threatening conditions. In our department, we noticed that patients with conditions inappropriate to the capabilities of the Clinic were being booked in or reviewed too late; thus causing wasted journeys for the patient. We conducted a Quality Improvement Project to improve the efficiency of the referral process. A prospective evaluation of referrals was collected continuously over a two-month period. Overall, 5 domains were deemed crucial to enable timely and accurate booking of patients to clinic: booking date, urgency, legibility, patient identification and appropriateness. Our proposed standard set for this project was 100% compliance over the 5 domains. Three separate interventions were instigated following the first cycle. The main components of the intervention were the phased development of an electronic referral system and an educational initiative for junior doctors. 20 referral forms were analysed during the initial 3-week period. No referrals met the recommended overall compliance standard of 100% (mean number of domains achieved: 3.38; standard deviation (SD): 0.637). Legibility and patient information were included in 21% and 30% of referrals, respectively. There was a trend of improvement following initiation of interventions. The mean number of domains achieved was 4.27 (SD 0.647; n=13) in the second data collection period, 4.53 (SD 0.514; n=16) in the third, and 4.75 (SD 0.452; n=24) in the fourth. Using linear regression, this change demonstrates a statistically significant improvement (p<0.001). An e-Proforma referral system represents a safe and efficient communication technology. When implementing policy change, it is crucial to acquire managerial and consultant support.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/bmjquality.u206639.w6166DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5361065PMC
March 2017

Minimally invasive surgery versus radiotherapy/chemoradiotherapy for small-volume primary oropharyngeal carcinoma.

Cochrane Database Syst Rev 2016 12 11;12:CD010963. Epub 2016 Dec 11.

Head and Neck Unit, Royal Marsden Hospital, Fulham Road, London, UK, SW3 6JJ.

Background: More than 400,000 cases of oropharyngeal squamous cell carcinoma (OPSCC) are diagnosed each year worldwide and the incidence is rising, partly as a result of human papillomavirus. Human papillomavirus-associated OPSCC affects younger patients and often presents at a higher stage; however, it is associated with a better prognosis.Until recently, first-line management of OPSCC involved chemoradiotherapy, as research had demonstrated comparable survival outcomes when compared with open surgery, with significantly decreased morbidity. However, interventions have now evolved with computerised planning and intensity-modulated radiotherapy, and the advent of endoscopic head and neck surgery, which provide the potential for decreased treatment-associated morbidity.The oropharynx plays an essential role in swallowing, speech and protecting the airway as it is situated at the bifurcation of the respiratory and digestive tracts. Treatment modality recommendations are based on survival outcomes. Given the younger patient demographic, establishing the safety of modalities that potentially have better functional outcome is becoming increasingly important.

Objectives: To assess the efficacy of endoscopic head and neck surgery (transoral robotic surgery or transoral laser microsurgery) for small-volume, primary (T1-2, N0-2) oropharyngeal squamous cell carcinoma (OPSCC) in comparison to radiotherapy/chemoradiotherapy.

Search Methods: The Cochrane ENT Information Specialist searched the ENT Trials Register; Central Register of Controlled Trials (CENTRAL 2016, Issue 10); PubMed; EMBASE; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 8 November 2016.

Selection Criteria: Randomised controlled trials in patients with carcinoma in the oropharynx subsite (as defined by the World Health Organization classification C09, C10). Cancers included were primary squamous cell carcinomas arising from the oropharyngeal mucosa. The tumours were classified as T1-T2 with or without nodal disease and with no evidence of distant metastatic spread. The intervention was transoral, minimally invasive surgery with or without adjuvant radiotherapy or adjuvant chemoradiotherapy. The comparator was primary radiotherapy with or without induction or concurrent chemotherapy for the tumour. The treatments received and compared were of curative intent and patients had not undergone prior intervention, other than diagnostic biopsy.

Data Collection And Analysis: We used the standard methodological procedures expected by Cochrane. Our primary outcomes were overall survival (disease-related mortality was to be studied where possible), locoregional control, disease-free survival and progression-free survival or time to recurrence. All outcomes were to be measured at two, three and five years after diagnosis. Our secondary outcomes included quality of life, harms associated with treatment, patient satisfaction and xerostomia score.

Main Results: No completed studies met the inclusion criteria for the review. Two ongoing trials fulfilled the selection criteria, however neither are complete.'Early-stage squamous cell carcinoma of the oropharynx: radiotherapy versus trans-oral robotic surgery (ORATOR)' is a phase II randomised controlled trial comparing primary radiation therapy with primary transoral robotic surgery for small-volume primary (T1-2, N0-2) OPSCC. It is currently in progress with an estimated completion date of June 2021.'European Organisation for Research and Treatment of Cancer 1420 (EORTC 1420-HNCG-ROG)' is a phase III, randomised study assessing the "best of" radiotherapy compared to transoral robotic surgery/transoral laser microsurgery in patients with T1-T2, N0 squamous cell carcinoma of the oropharynx and base of tongue. It was due to start accrual mid-2016.

Authors' Conclusions: The role of endoscopic head and neck surgery in the management of OPSCC is clearly expanding as evidenced by its more overt incorporation into the current National Comprehensive Cancer Network guidelines. Data are mounting regarding its outcomes both in terms of survival and lower morbidity. As confidence increases, it is being used in the management of more advanced OPSCC.Based on this review, there is currently no high-quality evidence from randomised controlled trials regarding clinical outcomes for patients with oropharyngeal cancer receiving endoscopic head and neck surgery compared with primary chemoradiotherapy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/14651858.CD010963.pub2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6463943PMC
December 2016

Lamb ingestion and odynophagia.

BMJ 2016 Nov 2;355:i5506. Epub 2016 Nov 2.

Department of Otolaryngology, Head and Neck Surgery, Addenbrooke's Hospital, Cambridge University Hospitals NHS Trust, Cambridge, UK.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/bmj.i5506DOI Listing
November 2016

CD8 T cell response to human papillomavirus 16 E7 is able to predict survival outcome in oropharyngeal cancer.

Eur J Cancer 2016 11 24;67:141-151. Epub 2016 Sep 24.

Department of Pathology, University of Cambridge, UK; Department of Dermatology, CUHT, UK.

Introduction: Immunological response to human papillomavirus (HPV) in the development and progression of HPV16+ oropharyngeal squamous cell carcinoma (OPSCC) (accounting for the majority of viral associated cases) is largely unknown and may provide important insights for new therapeutic strategies.

Methods: In this prospective clinical trial (UKCRN11945), we examined cell-mediated immune responses to HPV16 E2, E6 and E7 in peripheral blood using IFN-γ enzyme-linked immunosorbent spot assay. CD56, CD4, CD8 and regulatory T cell frequencies were also discerned by flow cytometry. Fifty-one study participants with oropharyngeal carcinoma were recruited. Control subjects were those undergoing tonsillectomy for benign disease. All patients were treated with curative intent by radiotherapy ± chemotherapy. Disease-specific survival was investigated by multivariate analysis.

Results: HPV16 DNA was detected in 41/51 of the OPSCC participants. T cell responses against HPV16 E6 or E7 peptides were detected in 33/51 evaluable patients, respectively and correlated with HPV status. Matched pre- and post-treatment T cell responses were available for 39/51 OPSCC cases. Within the whole cohort, elevated post-treatment CD8 response to HPV16 E7 correlated with longer disease free survival (multivariate DFS p < 0.03). Within the HPV + OPSCC cohort, a significant increase in regulatory T cells (p < 0.02) was noted after treatment.

Conclusions: This is the first study to provide survival data in OPSCC stratified by cell-mediated immune response to HPV16 peptides. Within the HPV16+ OPSCC cohort, enhanced immunoreactivity to antigen E7 was linked to improved survival. An increase in regulatory T cell frequencies after treatment may suggest that immunosuppression can contribute to a reduced HPV-specific cell-mediated response.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ejca.2016.08.012DOI Listing
November 2016

Ruxolitinib Associated Tuberculosis Presenting as a Neck Lump.

Case Rep Infect Dis 2015 15;2015:284168. Epub 2015 Dec 15.

ENT Department, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Road, Cambridge CB2 0QQ, UK.

Tuberculosis is an opportunistic infection with protean clinical manifestations. We describe a case of Ruxolitinib induced miliary tuberculosis presenting as a neck lump. A 78-year-old female presented with a two-month history of right-sided neck lump associated with fever, night sweats, and significant weight loss. She had a past medical history that included myelofibrosis, being treated with Ruxolitinib. Examination demonstrated 4 × 4 cm right-sided cervical lymphadenopathy. A chest radiograph showed extensive shadowing in both lungs. CT scan demonstrated perilymphatic nodes in addition to the cervical mass. An ultrasound-guided biopsy of a cervical lymph node demonstrated confirmed Mycobacterium tuberculosis infection. It was hypothesized that use of Ruxolitinib through its selective inhibition of Janus-activated kinases 1 and 2 resulted in immunosuppression and miliary tuberculosis in this patient. The medication was stopped and a 12-month regime of antituberculosis therapy commenced. She remained well at one-year follow-up with resolution of lung involvement. Clinicians should consider tuberculosis as a differential diagnosis for patients presenting with a neck lump, particularly in those taking immunosuppressant medication such as Ruxolitinib. A multidisciplinary approach is needed to promptly treat the tuberculosis and consider discontinuation of Ruxolitinib.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1155/2015/284168DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4692993PMC
January 2016

Langerhans cell sarcoma of the head and neck.

Crit Rev Oncol Hematol 2016 Mar 2;99:180-8. Epub 2016 Jan 2.

Department of Otolaryngology, Head and Neck Surgery, Addenbrooke's Hospital, Cambridge CB2 0QQ, UK.

Head and neck Langerhans cell sarcoma (HNLCS) is a rare malignant tumor carrying a poor prognosis. The aim of this work was to perform a systematic review of HNLCS cases, examine outcomes, and develop an evidence-based management algorithm. We performed a systematic literature search yielding 16 studies with 17 cases of HNLCS; 33 studies with 55 Non-HNLCS were used as a comparison. Mean disease-specific survival was 20.5 months (SE ± 5.1) for HNLCS versus 26.2 months (SE±4.2) for non-HNLCS. There was no significant difference in disease-specific (p = 0.768) or disease-free survival (p = 0.880) between the two cohorts. There was a significant difference in both disease-specific (p = 0.044) and disease-free survival (p = 0.001) between local, locoregional and disseminated disease favoring more limited disease. HNLCS appears to present later, with more disseminated disease. Surgery remains the mainstay of treatment of local disease, however clear margins do not guarantee clearance.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.critrevonc.2015.12.017DOI Listing
March 2016

Thyroid surgery for Graves' disease and Graves' ophthalmopathy.

Cochrane Database Syst Rev 2015 Nov 25(11):CD010576. Epub 2015 Nov 25.

ENT Department, Whipps Cross University Hospital, Leytonstone, London, UK, E11 1NR.

Background: Graves' disease is an autoimmune disease caused by the production of auto-antibodies against the thyroid-stimulating hormone receptor, which stimulates follicular cell production of thyroid hormone. It is the commonest cause of hyperthyroidism and may cause considerable morbidity with increased risk of cardiovascular and respiratory adverse events. Five per cent of people with Graves' disease develop moderate to severe Graves' ophthalmopathy. Thyroid surgery for Graves' disease commonly falls into one of three categories: 1) total thyroidectomy, which aims to achieve complete macroscopic removal of thyroid tissue; 2) bilateral subtotal thyroidectomy, in which bilateral thyroid remnants are left; and 3) unilateral total and contralateral subtotal thyroidectomy, or the Dunhill procedure. Recent American Thyroid Association guidelines on treatment of Graves' hyperthyroidism emphasised the role of surgery as one of the first-line treatments. Total thyroidectomy removes target tissue for the thyroid-stimulating hormone receptor antibody. It controls hyperthyroidism at the cost of lifelong thyroxine replacement. Subtotal thyroidectomy leaves a thyroid remnant and may be less likely to lead to complications, however a higher rate of recurrent hyperthyroidism is expected and revision surgery would be challenging. The choice of the thyroidectomy technique is currently largely a matter of surgeon preference, and a systematic review of the evidence base is required to determine which option offers the best outcomes for patients.

Objectives: To assess the optimal surgical technique for Graves' disease and Graves' ophthalmopathy.

Search Methods: We searched the Cochrane Library, MEDLINE and PubMed, EMBASE, ClinicalTrials.gov, and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). The date of the last search was June 2015 for all databases. We did not apply any language restrictions.

Selection Criteria: Only randomised controlled trials (RCTs) involving participants with a diagnosis of Graves' disease based on clinical features and biochemical findings of hyperthyroidism were eligible for inclusion. Trials had to directly compare at least two surgical techniques of thyroidectomy. There was no age limit to study inclusion.

Data Collection And Analysis: Two review authors independently extracted and cross-checked the data for analysis, evaluation of risk of bias and establishment of 'Summary of findings' tables using the GRADE instrument. The senior review authors reviewed the data and reconciled disagreements.

Main Results: We included five RCTs with a total of 886 participants; 172 were randomised to total thyroidectomy, 383 were randomised to bilateral subtotal thyroidectomy, 309 were randomised to the Dunhill procedure and 22 were randomised to either bilateral subtotal thyroidectomy or the Dunhill procedure. Follow-up ranged between six months and six years. One trial had three comparison arms. All five trials were conducted in university hospitals or tertiary referral centres for thyroid disease. All thyroidectomies were performed by experienced surgeons. The overall quality of the evidence ranged from low to moderate. In all trials, blinding procedures were insufficiently described. Outcome assessment for objective outcomes was blinded in one trial. Surgeons were not blinded in any of the trials. One trial blinded participants. Attrition bias was a substantial problem in one trial, with 35% losses to follow-up. In one trial the analysis was not carried out on an intention-to-treat basis.Total thyroidectomy was more effective than subtotal thyroidectomy techniques (both bilateral subtotal thyroidectomy and the Dunhill procedure) at preventing recurrent hyperthyroidism in 0/150 versus 11/200 participants (OR 0.14 (95% CI 0.04 to 0.46); P = 0.001; 2 trials; moderate quality evidence). Total thyroidectomy was also more effective than bilateral subtotal thyroidectomy at preventing recurrent hyperthyroidism in 0/150 versus 10/150 participants (odds ratio (OR) 0.13 (95% confidence interval (CI) 0.04 to 0.44); P = 0.001; 2 trials; moderate quality evidence). Compared to bilateral subtotal thyroidectomy, the Dunhill procedure was more likely to prevent recurrent hyperthyroidism in 20/283 versus 8/309 participants (OR 2.73 (95% CI 1.28 to 5.85); P = 0.01; 3 trials; low quality evidence). Total thyroidectomy compared with subtotal thyroidectomy conferred a greater risk of permanent hypocalcaemia/hypoparathyroidism in 8/172 versus 3/221 participants (OR 4.79 (95% CI 1.36 to 16.83); P = 0.01; 3 trials; low quality evidence). Effects of the various surgical techniques on permanent recurrent laryngeal nerve palsy and regression of Graves' ophthalmopathy were neutral. One death was reported in one study in year three of follow-up. No study investigated health-related quality of life or socioeconomic effects.

Authors' Conclusions: Total thyroidectomy is more effective than subtotal thyroidectomy (both bilateral subtotal thyroidectomy and the Dunhill procedure) at preventing recurrent hyperthyroidism in Graves' disease. The type of surgery performed does not affect regression of Graves' ophthalmopathy. There was some evidence that total thyroidectomy compared with subtotal thyroidectomy conferred a greater risk of permanent hypocalcaemia/hypoparathyroidism, which however, was not seen in comparison with bilateral subtotal thyroidectomy. Permanent recurrent laryngeal nerve palsy did not seem to be affected by type of thyroidectomy. Health-related quality of life as a patient-important outcome measure should form a core determinant of any future trial on the effects of thyroid surgery for Graves' disease.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/14651858.CD010576.pub2DOI Listing
November 2015

Catecholamine-secreting paraganglioma: the challenges of perioperative management.

BMJ Case Rep 2015 Nov 3;2015. Epub 2015 Nov 3.

ENT Surgery Department, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

An asymptomatic 48-year-old man presented with a right-sided neck mass. A CT scan demonstrated a lesion at the carotid bifurcation and an angiogram showed splaying of the carotid arteries. His plasma metanephrines were raised confirming a catecholamine-secreting paraganglioma. Metaiodobenzylguanidine single-photon emission CT showed focal high tracer uptake in the right of the neck. Histology revealed a tumour, arising within a nerve, composed of oval-shaped cells arranged in nested (zellballen) as well as in trabecular patterns. Immunohistochemistry was positive for neuroendocrine markers chromogranin A, synaptophysin and CD56. Preoperative management included an endocrinologist initiating α-adrenergic and β-adrenergic blockers. Intraoperatively, acute hypertension occurred whenever the tumour was manipulated. Close communication between the surgeons and the anaesthetist allowed for these episodes to be predicted and treated with fast-acting antihypertensives such as sodium nitroprusside. Postoperatively, the patient recovered well and his antihypertensives were discontinued.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2015-212737DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4654031PMC
November 2015

Deregulation of SYCP2 predicts early stage human papillomavirus-positive oropharyngeal carcinoma: A prospective whole transcriptome analysis.

Cancer Sci 2015 Nov 16;106(11):1568-75. Epub 2015 Oct 16.

Department of Pathology, University of Cambridge, Cambridge, UK.

This study was designed to identify significant differences in gene expression profiles of human papillomavirus (HPV)-positive and HPV-negative oropharyngeal squamous cell carcinomas (OPSCC) and to better understand the functional and biological effects of HPV infection in the premalignant pathway. Twenty-four consecutive patients with locally advanced primary OPSCC were included in a prospective clinical trial. Fresh tissue samples (tumor vs. matched normal epithelium) were subjected to whole transcriptome analysis and the results validated on the same cohort with RT-quantitative real-time PCR. In a separate retrospective cohort of 27 OPSCC patients, laser capture microdissection of formalin-fixed, paraffin-embedded tissue allowed RNA extraction from adjacent regions of normal epithelium, carcinoma in situ (premalignant) and invasive SCC tissue. The majority of patients showed evidence of high-risk HPV16 positivity (80.4%). Predictable fold changes of RNA expression in HPV-associated disease included multiple transcripts within the p53 oncogenic pathway (e.g. CDKN2A/CCND1). Other candidate transcripts found to have altered levels of expression in this study have not previously been established (SFRP1, CRCT1, DLG2, SYCP2, and CRNN). Of these, SYCP2 showed the most consistent fold change from baseline in premalignant tissue; aberrant expression of this protein may contribute to genetic instability during HPV-associated cancer development. If further corroborated, this data may contribute to the development of a non-invasive screening tool. This study is registered with the UK Clinical Research Network (ref.: 11945).
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/cas.12809DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4714680PMC
November 2015

Conservative or radical surgery for pediatric papillary thyroid carcinoma: A systematic review of the literature.

Int J Pediatr Otorhinolaryngol 2015 Oct 10;79(10):1620-4. Epub 2015 Aug 10.

Department of Otolaryngology, Head and Neck Surgery, Addenbrooke's Hospital, Cambridge CB2 0QQ, UK.

Background: Pediatric papillary thyroid carcinoma (PTC) is characterized by an aggressive clinical course. Early diagnosis is a challenge and treatment consists principally of partial or total thyroidectomy±neck dissection and radioactive iodine therapy. Due to the rarity of PTC in children, there is no consensus on optimal surgical treatment.

Methods And Results: A literature search was carried out using PubMed, Embase, Medline, Cochrane and Web of Science. Seven studies (489 patients) investigating the outcome of surgically managed pediatric PTC were identified. No clear advantage in survival or recurrence rate was found for total thyroidectomy compared to other surgical approaches.

Conclusion: Despite the aggressive behavior of PTC, prognosis is good, with low mortality. After removal of disease and prevention of recurrence, reduction of iatrogenic complications are a priority in this age group. Due to the paucity of available evidence, this review cannot recommend conservative or radical surgery for pediatric papillary thyroid carcinoma. To answer this question, we recommend the establishment of a randomized controlled trial with adequately matched baseline variables.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijporl.2015.08.004DOI Listing
October 2015

Multi-institutional validation of a novel textural analysis tool for preoperative stratification of suspected thyroid tumors on diffusion-weighted MRI.

Magn Reson Med 2016 Apr 20;75(4):1708-16. Epub 2015 May 20.

Cancer Research UK Cambridge Institute, University of Cambridge, Li Ka Shing Centre, Robinson Way, Cambridge, United Kingdom.

Purpose: Ultrasound-guided fine needle aspirate cytology fails to diagnose many malignant thyroid nodules; consequently, patients may undergo diagnostic lobectomy. This study assessed whether textural analysis (TA) could noninvasively stratify thyroid nodules accurately using diffusion-weighted MRI (DW-MRI).

Methods: This multi-institutional study examined 3T DW-MRI images obtained with spin echo echo planar imaging sequences. The training data set included 26 patients from Cambridge, United Kingdom, and the test data set included 18 thyroid cancer patients from Memorial Sloan Kettering Cancer Center (New York, New York, USA). Apparent diffusion coefficients (ADCs) were compared over regions of interest (ROIs) defined on thyroid nodules. TA, linear discriminant analysis (LDA), and feature reduction were performed using the 21 MaZda-generated texture parameters that best distinguished benign and malignant ROIs.

Results: Training data set mean ADC values were significantly different for benign and malignant nodules (P = 0.02) with a sensitivity and specificity of 70% and 63%, respectively, and a receiver operator characteristic (ROC) area under the curve (AUC) of 0.73. The LDA model of the top 21 textural features correctly classified 89/94 DW-MRI ROIs with 92% sensitivity, 96% specificity, and an AUC of 0.97. This algorithm correctly classified 16/18 (89%) patients in the independently obtained test set of thyroid DW-MRI scans.

Conclusion: TA classifies thyroid nodules with high sensitivity and specificity on multi-institutional DW-MRI data sets. This method requires further validation in a larger prospective study. Magnetic Resonance in Medicine published by Wiley Periodicals, Inc. on behalf of International Society for Magnetic Resonance.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/mrm.25743DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4654719PMC
April 2016

Inverted papilloma of lacrimal sac invading into the orbit: case report and review of literature.

J Cancer Res Ther 2015 Jan-Mar;11(1):238-40

Department of Otolaryngology, Head and Neck Surgery, Addenbrookes Hospital, Cambridge, United Kingdom.

Inverted papilloma (IP) is a sinonasal tumor of benign etiology with local invasion and malignant potential. IP arising in lacrimal sac invading the orbit is extremely rare with only one case reported so far. The presented case appears to be the second such case reported in the literature. A 60-year-old Caucasian male presented with a medial canthal mass and epiphora. Incisional biopsy confirmed a transitional neoplasm. The lesion was completely excised enbloc with clear margins by using a Weber-Ferguson incision; orbital clearance and nasolacrimalfossa clearance was achieved via a medial maxillectomy. Enbloc resection of orbital and nasolacrimal parts of the tumor with clear margins is recommended.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/0973-1482.155111DOI Listing
January 2016

Langerhans cell sarcoma: a systematic review.

Cancer Treat Rev 2015 Apr 9;41(4):320-31. Epub 2015 Mar 9.

Department of Otolaryngology, Head and Neck Surgery, Addenbrooke's Hospital, Cambridge, CB2 0QQ, UK.

Langerhans cell sarcoma (LCS) is a rare malignant tumour of Langerhans cells with a poor outcome. Given its rarity, there is a lack of evidence regarding the most appropriate treatment for this condition. Therefore the aim of this work was to review, compile, analyse and present clinical details and to determine the optimal treatment regimen. A search of PubMed, CINAHL, EMBASE, Cochrane, CENTRAL, clinicaltrials.gov and Google Scholar was supplemented by hand searching. Data extracted included demographics, treatment, type of LCS and clinical outcome. Of 510 citations identified by a systematic literature search, 46 case series including 66 subjects with LCS met criteria for analysis. The most common treatment modality was chemotherapy, used alone or in combination in 47 cases (71%) followed by surgery in 31 cases (47%). Overall mean (S.E.) disease specific survival and disease free survival were 27.2 (3.9) and 18.3 (3.8) months respectively. There was a significant difference in both disease specific and disease free survival between the local, loco-regional and disseminated disease cohorts (DSS p=0.014; DFS p<0.001). More localised disease confers a survival advantage. Multi-modality therapy appears to be most effective, with the addition of radiotherapy to chemotherapy appearing beneficial. Complete surgical excision with clear margins being most effective for local disease control. Any adjuvant therapy should not be delayed. Bone marrow transplant appears to be the most reliable treatment in terms of outcome especially in disseminated disease however has well known patient selection and toxicity/tolerance issues. The role of cell surface markers for prognostication remains unclear.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ctrv.2015.02.011DOI Listing
April 2015

Management of tumors arising from the parapharyngeal space: A systematic review of 1,293 cases reported over 25 years.

Laryngoscope 2015 Jun 2;125(6):1372-81. Epub 2014 Dec 2.

Department of Otolaryngology-Head and Neck Surgery, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, United Kingdom.

Objectives: We present a systematic review of 1,252 lesions published in the past 25 years, the largest to date. We also include our own experience of 41 cases.

Data Sources: Our data sources were MEDLINE and Embase databases.

Review Methods: A systematic review of the literature (1988-2014) was performed and data on histological diagnosis, presentation, surgical approach, and postoperative complications were reviewed.

Results: In total, 22 studies (including our own institution) revealed 82% of lesions were benign. The most common presentation was an intraoral mass (52%) followed by a cervical mass (48%), and the most common primary lesion was a pleomorphic adenoma (29%). Ninety-six percent of patients underwent surgery. The cervical approach was most frequently used (46%), and the most common complication was vagus nerve injury (13%). Within our institution, 5-year progression-free survival for benign and malignant disease was 93% and 61%, respectively (P = .196).

Conclusions: A parapharyngeal space mass is an uncommon tumor and requires careful preoperative assessment. Definitive treatment is usually surgery, and these patients should be counseled appropriately about the potential for permanent cranial nerve deficits.

Level Of Evidence: NA.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/lary.25077DOI Listing
June 2015

Long-term outcome of airway stenosis in granulomatosis with polyangiitis (Wegener granulomatosis): an observational study.

JAMA Otolaryngol Head Neck Surg 2014 Nov;140(11):1038-44

Department of Otolaryngology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, England.

Importance: Airway stenosis occurs in patients with granulomatosis with polyangiitis (GPA or Wegener granulomatosis). It produces significant morbidity and contributes to mortality.

Objective: To investigate the frequency and distribution of airway stenoses in GPA and evaluate the efficacy of local interventions in maintaining airway patency.

Design, Setting, And Participants: A retrospective single-center study of 44 patients with GPA and airway stenosis assessed and treated as needed by a multidisciplinary team at a university medical center between 1997 and 2012. The median duration of observation for each patient from the time of diagnosis was 146 months.

Interventions: Patients who had critical stenoses underwent either dilatation or laser radial cuts to the lesion. In some cases, intralesional administration of steroids or topical mitomycin C was used.

Main Outcomes And Measures: The main outcome measure was airway patency for at least 12 months and the number of interventions required to achieve this end point. Details of patients and interventions were recorded at baseline and at each treatment.

Results: The median age at diagnosis was 37.6 years; 73% of patients were women (n = 34). The median follow-up after the initial intervention was 62.5 months. Subglottic stenosis was found in 36 patients, lower airway stenosis in 30. There were 213 interventions in 39 patients, including balloon and bougie dilatation and laser treatment. Adjuvant local treatment was used in 71 interventions. A 12-month period of airway stability was achieved in 34 of 36 cases (97%) (5 had no procedures and 3 had follow-up shorter than 12 months). The median interval between procedures was 4.9 months, and after the last intervention recorded, patients had at least 27 months of airway stability. Fourteen adverse events were recorded (6.6%).

Conclusions And Relevance: The frequency and distribution of airway stenoses in 44 patients with GPA has been described. In the 39 patients who required intervention, multiple procedures were required, but 97% then achieved a prolonged period of airway patency. The procedures and adjuvant treatments were found to be safe. Our experience with a variety of techniques in this rare presentation has permitted design of a structured approach and an algorithm to manage and evaluate airway stenosis in GPA.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1001/jamaoto.2014.2430DOI Listing
November 2014

Intraoperative ultrasound to facilitate removal of a submucosal foreign body.

J Clin Ultrasound 2014 Nov-Dec;42(9):565-8. Epub 2014 Aug 22.

Department of ENT Surgery, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Road, Cambridge, United Kingdom, CB2 0QQ.

A 61-year-old man with a history of fish bone ingestion and poorly localized symptoms was seen. His clinical examination was unremarkable, but CT demonstrated a foreign body deeply embedded within his tongue. Intraoperative ultrasound (US) guidance facilitated identification of a bone, allowing a needle to be placed as a guide to dissection. Repeat US scanning through the incision permitted precisely targeted surgery. CT and US are the most effective imaging techniques for localizing fish bones. Intraoperative US can be used to accurately locate a submucosal fish bone in mobile tissue such as the tongue, and focused, image-guided dissection can reduce surgical tissue trauma.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/jcu.22226DOI Listing
June 2015

De-escalation treatment protocols for human papillomavirus-associated oropharyngeal squamous cell carcinoma: a systematic review and meta-analysis of current clinical trials.

Eur J Cancer 2014 Oct 1;50(15):2636-48. Epub 2014 Aug 1.

Department of Pathology, University of Cambridge, UK.

Iatrogenic complications associated with current treatment protocols for oropharyngeal squamous cell carcinoma are noted to cause high rates of acute and chronic morbidity. The aims of this study are to provide an overview of the current de-escalation trials for human papillomavirus positive (HPV+) oropharyngeal carcinoma and to evaluate the evidence supporting improved response to treatment of patients within this viral cohort. This study reviewed all completed or in progress randomised controlled trials (RCTs) assessing clinical interventions for human papillomavirus-associated locally advanced oropharyngeal squamous cell carcinoma. We utilised a validated 'risk of bias' tool to assess study quality. We identified nine RCTs that met the full inclusion criteria for this review (all of which are currently on-going and will report from 2015 onwards). Five RCTs performed a post hoc analysis by HPV status, which allowed meta-analysis of 1130 patients. The data reveal a significant difference in overall survival (hazard ratio (HR) 0.49 [95% confidence interval (CI) 0.35-0.69]), loco-regional failure (HR 0.43 [95% CI 0.17-1.11]) and disease specific survival (0.41 [95% 0.3-0.56]) in favour of the HPV+ category. In considering de-escalation treatment protocols, nine studies are currently ongoing. Our meta-analysis provides strong evidence for an improved prognosis in the viral associated cohort when treated by platinum based chemotherapy in combination with radiotherapy or primary radiotherapy. So far, one trial (with moderate to high risk of bias) suggests a reduced survival outcome for the HPV+ population when using the epidermal growth factor receptor (EGFR) inhibitor cetuximab.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ejca.2014.07.001DOI Listing
October 2014

Kaposiform hemangioendothelioma of paranasal sinus.

Laryngoscope 2014 Sep 29;124(9):2103-6. Epub 2014 Apr 29.

Department of Otolaryngology, Head and Neck Surgery, Addenbrookes Hospital, Cambridge, United Kingdom.

Kapossiform hemangioendothelioma (KHE) of the paranasal sins (PNS) is a rare cause of recurrent epistaxis. To date, only two cases of PNS KHE have been reported in the literature, both occurring in the pediatric population. The case presented here appears to be the first case of PNS KHE occurring in an adult. A 46-year-old white female presented with progressively worsening unilateral recurrent epistaxis. Diagnostic histopathology confirmed it to be KHE. After a detailed workup, the tumor was completely excised en bloc (medial maxillectomy; anterior and posterior ethmoidectomy) via a lateral-rhinotomy approach. Complete excision of the tumor with clear margins offers the best results.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/lary.24669DOI Listing
September 2014

A systematic review of 1143 parapharyngeal space tumors reported over 20 years.

Oral Oncol 2014 May 28;50(5):421-30. Epub 2014 Feb 28.

Department of Otolaryngology-Head and Neck Surgery, Cambridge University Hospitals, University of Cambridge School of Clinical Medicine, Cambridge CB2 0QQ, UK. Electronic address:

Parapharyngeal space tumours are rare and most clinicians will only see a small number during their career. We performed a systematic review of 1143 parapharyngeal space tumors published in the past 20 years to increase cumulative experience. A systematic literature review was performed and data on histological diagnosis, presentation, surgical approach and postoperative complications of cases published between 1989 and 2009 were compiled and reviewed. The systematic review identified a total of 1143 parapharyngeal space tumors presented in 17 studies. A majority (82%) were benign and 18% were malignant. The most common presentation was a cervical mass (50%) or an intraoral mass (47%). Approximately 70 different histologic subtypes of parapharyngeal space tumors were reported in the cumulative series. The most common primary lesion was a pleomorphic adenoma (34%). Ninety-five percent of patients underwent surgery. The most frequent approach and used to excise the lesions was the cervical approach (48%) and the commonest complication was the vagus nerve injury seen in 14% of the cases.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.oraloncology.2014.02.007DOI Listing
May 2014

De-escalation treatment protocols for human papillomavirus-associated oropharyngeal squamous cell carcinoma.

Cochrane Database Syst Rev 2014 Feb 15(2):CD010271. Epub 2014 Feb 15.

ENT Department, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK, CB2 0QQ.

Background: Human papillomavirus-associated oropharyngeal squamous cell carcinomas are a distinct subgroup of tumours that may have a better prognosis than traditional tobacco/alcohol-related disease. Iatrogenic complications, associated with conventional practice, are estimated to cause mortality of approximately 2% and high morbidity. As a result, clinicians are actively investigating the de-escalation of treatment protocols for disease with a proven viral aetiology.

Objectives: To summarise the available evidence regarding de-escalation treatment protocols for human papillomavirus-associated, locally advanced oropharyngeal squamous cell carcinoma.

Search Methods: We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials; PubMed; EMBASE; CINAHL; Web of Science; Cambridge Scientific Abstracts; ICTRP and additional sources for published and unpublished trials. The date of the most recent search was 25 June 2013.

Selection Criteria: Randomised controlled trials investigating de-escalation treatment protocols for human papillomavirus-associated, locally advanced oropharyngeal carcinoma. Specific de-escalation categories were: 1) bioradiotherapy (experimental) versus chemoradiotherapy (control); 2) radiotherapy (experimental) versus chemoradiotherapy (control); and 3) low-dose (experimental) versus standard-dose radiotherapy (control). The outcomes of interest were overall and disease-specific survival, treatment-related morbidity, quality of life and cost.

Data Collection And Analysis: Three authors independently selected studies from the search results and extracted data. We planned to use the Cochrane 'Risk of bias' tool to assess study quality.

Main Results: We did not identify any completed randomised controlled trials that could be included in the current version of this systematic review. We did, however, identify seven ongoing trials that will meet our inclusion criteria. These studies will report from 2014 onwards. We excluded 30 studies on methodological grounds (seven randomised trials with post hoc analysis by human papillomavirus status, 11 prospective trials and 12 ongoing studies).

Authors' Conclusions: There is currently insufficient high-quality evidence for, or against, de-escalation of treatment for human papillomavirus-associated oropharyngeal carcinoma. Future trials should be multicentre to ensure adequate power. Adverse events, morbidity associated with treatment, quality of life outcomes and cost analyses should be reported in a standard format to facilitate comparison with other studies.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/14651858.CD010271.pub2DOI Listing
February 2014

An adult with a neck lump.

BMJ 2013 Oct 28;347:f5473. Epub 2013 Oct 28.

Department of Otolaryngology and Head and Neck Surgery, Cambridge University Hospitals NHS Trust, Cambridge CB2 0QQ, UK.

A 40 year old man presents with a 1 cm tender soft swelling in the lateral neck region. This developed seven days after the onset of a tonsillitis episode that has now resolved. He has a 20 pack-year smoking history and consumes minimal alcohol. Examination is otherwise normal.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/bmj.f5473DOI Listing
October 2013

A unique case of pneumatised styloid process with cholesteatoma.

Case Rep Otolaryngol 2013 4;2013:964725. Epub 2013 Jun 4.

Department of Otolaryngology, Head and Neck Surgery, Addenbrookes Hospital, Cambridge University Teaching Hospitals NHS Foundation Trust, Cambridge CB2 0QQ, UK.

Pneumatisation of styloid process is a very rare finding and has never been reported previously. We present a unique case of a pneumatised styloid process with a cholesteatoma arising within the cavity. We describe the clinical features, associated radiological findings, and management of this lesion.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1155/2013/964725DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3687720PMC
July 2013

A rare cutaneous presentation of metastatic parotid adenocarcinoma.

Australas J Dermatol 2013 May 2;54(2):e40-2. Epub 2012 Mar 2.

Skin Cancer Institute, Dermatological Surgical Unit, Tauranga, New Zealand.

Primary adenocarcinomas of the parotid gland are rare, accounting for < 5% of all head and neck malignant neoplasms. The biological behaviour of these tumours varies considerably. Low-grade tumours are minimally invasive, whereas high-grade tumours show a high incidence of local recurrence and distant metastases. We report a case of metastatic parotid adenocarcinoma which presented with cutaneous features. This case illustrates that such salivary gland malignancies can very rarely present to the dermatologist. These potentially aggressive tumours require prompt diagnosis and management with multidisciplinary team input to ensure that the appropriate treatment is instigated.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1440-0960.2011.00865.xDOI Listing
May 2013

Radiologically predicting when a sternotomy may be required in the management of retrosternal goiters.

Ann Otol Rhinol Laryngol 2013 Jan;122(1):15-9

Department of Otolaryngology-Head and Neck Surgery, Addenbrooke's Hospital, Cambridge, England.

Objectives: Surgery remains the most effective treatment for retrosternal goiters. These commonly present as asymptomatic lesions in elderly patients, but may also cause airway and esophageal compression and, less commonly, may also be malignant. Although the majority of these goiters are amenable to transcervical thyroidectomy, in a minority of patients sternotomy is required. The ability to predict the need for sternotomy before operation would allow for safer surgery and operative counseling, as well as improved logistical efficiency if coordination with thoracic surgeons is required. In this report, we assess the radiologic factors that might be predictive of the need for sternotomy.

Methods: We performed a retrospective review of 97 retrosternal goiters for which thyroidectomy was performed within the otolaryngology department at Addenbrooke's Hospital, Cambridge, between 2001 and 2011. There were a total of 80 cervical excisions and 17 cases in which sternotomy was required. A detailed computed tomographic analysis of these 17 cases was undertaken to assess the predictive factors for the requirement of sternotomy. The factors assessed included posterior mediastinal extension, presence of an ectopic nodule, extension below the carina, extension below the aortic arch, a "conical shape" of the goiter, and tracheal compression. These were compared to the same factors in the control group of 80 patients, and Fisher's exact test was used to determine statistical significance.

Results: The significant predictive factors for sternotomy, were posterior mediastinal extension, extension below the carina, and a "conical" goiter in which the thoracic inlet becomes a ring of constriction (all p < 0.05).

Conclusions: Our results suggest that it is possible to predict on the basis of computed tomographic imaging the need for sternotomy in retrosternal goiters.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/000348941312200104DOI Listing
January 2013

Cricopharyngeal myotomy for cricopharyngeus stricture in an inclusion body myositis patient with hiatus hernia: a learning experience.

BMJ Case Rep 2013 Jan 22;2013. Epub 2013 Jan 22.

Department of ENT, Medway Maritime Hospital, London, UK.

Inclusion body myositis is a chronic progressive myopathy which tends not to respond to steroids and immunosuppressive treatments. Dysphagia is more common in this group than other inflammatory myopathies like polymyositis and dermatomyositis. Otolaryngologists are involved in the management of dysphagia in inclusion body myositis. They usually use a combination of cricopharyngeal myotomy, upper oesophageal dilation or botulinum injection to help with the symptoms. Cricopharyngeus myotomy is the preferred treatment in this group and patients tend to be discharged after a short stay in the hospital. However, our experience was completely different from what we expected as a relatively straightforward procedure led to severe morbidity and prolonged hospital admission due to continuous acid reflux and aspiration. We believe that the presence of hiatus hernia led to this problem as the patient's problem resolved completely after her hernia was treated.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2012-008058DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3604310PMC
January 2013

A disease activity score for ENT involvement in granulomatosis with polyangiitis (Wegener's).

Laryngoscope 2013 Mar 3;123(3):622-8. Epub 2013 Jan 3.

Department of Otolaryngology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

Objectives/hypothesis: Accurate assessment of disease activity in patients with otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener's) (ENT/GPA) is necessary for treatment decisions and clinical trials. We have designed a disease activity score (ENT/GPA DAS) for this purpose.

Study Design: A prospective cross-sectional study.

Methods: GPA patients seen in a tertiary center were systematically assessed for disease activity and/or infection in the ear, nose, and throat region using European Vasculitis Study Group guidelines. An ENT disease activity score was developed and validated, and compared to the ENT domain of the Birmingham Vasculitis Activity Score (ENT/BVAS).

Results: One hundred forty-four patients were studied, of whom 87% (125/144) had ENT involvement. ENT items of disease activity were correlated with expert clinical assessment. Discriminant correlation tests were performed to control for infection. Six items were retained to form the ENT/GPA DAS: reported bloody rhinorrhoea, granulation, ulcers and/or friable mucosa in the upper airway on endoscopic evaluation, objective stridor, sudden sensorineural hearing loss, and inflamed tympanic membrane/middle ear without infection. Nasal crusting was excluded. Individual items of ENT/GPA DAS had higher sensitivities and comparable specificities in predicting disease activity than ENT/BVAS items. Overall ENT/GPA DAS demonstrated higher sensitivity and lower specificity for disease activity in ENT/GPA when compared to overall ENT/BVAS.

Conclusions: A tool to assess ENT disease activity in GPA has been developed. It is potentially superior to existing tools but requires further testing for intra- and interobserver reliability.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/lary.23737DOI Listing
March 2013

Utility of an intraoperative ultrasound in lateral approach mini-parathyroidectomy with discordant pre-operative imaging.

Eur Arch Otorhinolaryngol 2013 May 27;270(6):1903-8. Epub 2012 Nov 27.

Department of Otolaryngology, Head and Neck Surgery, Addenbrooke's Hospital, Box 10, Cambridge, CB2 0QQ, UK.

Objectives of this study were to assess the utility of intra-operative ultrasound to resolve discordant pre-operative imaging prior to a lateral approach mini-parathyroidectomy, by studying prospective case series in a head and neck endocrine unit. Patients with primary hyperparathyroidism due to a single adenoma with discordant pre-operative ultrasound and sestamibi were enrolled. They underwent a further intra-operative ultrasound by a head and neck radiologist with a view to proceed with a mini-parathyroidectomy. The main outcome measure was utility of intra-operative ultrasound compared to operative findings and pre-operative imaging. Secondary measures were complications of mini-parathyroidectomy, operative and ambulatory discharge time. Twenty-two patients underwent surgery with intra-operative ultrasound in the surgical position. The intra-operative ultrasound findings correlated with the operative findings in all cases (100 %). There were 16 inferior adenomas and 6 superior adenomas. Six inferior adenomas were in a retrosternal position, eight were obscured by benign thyroid lesions and a further two reported pre-operatively as superior. Three out of six superior adenomas were reported as inferior pre-operatively as the inferior thyroid artery was inadequately visualised, two were retro-carotid and one was retro-oesophageal. All patients were discharged within 23 h of surgery. There were no unsuccessful focused explorations. Histological analysis confirmed the adenomas. No morbidity (vocal cord palsy, haematoma, hungry bones) was noted. The results indicated that intra-operative ultrasound by a dedicated radiologist is a valuable tool in resolving discordance of pre-operative imaging. Appropriate patient positioning with neck extension and muscle relaxation allows placement of the probe in the obscure retro-carotid and retro-oesophageal locations and unmasks apparent "mediastinal" parathyroids facilitating focused dissection.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00405-012-2284-0DOI Listing
May 2013

Endoscopic or external approach revision surgery for pharyngeal pouch following primary endoscopic stapling: which is the favoured approach?

Eur Arch Otorhinolaryngol 2013 May 27;270(5):1707-10. Epub 2012 Sep 27.

Department of Otorhinolaryngology and Head & Neck Surgery, Addenbrooke's Hospital, Hills Road, Cambridge CB2 0QQ, UK.

This study aimed to assess outcomes of revision endoscopic stapling and external excision of pharyngeal pouch. A 5-year prospective study was performed on all patients requiring revision pouch surgery following primary endoscopic stapling. Data were collected retrospectively. Eighteen patients underwent revision pouch surgery. In seven patients, pouch size was down-graded from 3 to 2, and these were stapled endoscopically. Two leaks resulted. Eleven patients with grade 1 or 3 pouches underwent external excision of pouch, with no post-operative complications. As per results external excision of pouch is safe for grade 1 and 3 pouches. It avoids risking redundant mucosa and recurrence of symptoms which can complicate stapling and enables a myotomy to be performed to reduce cricopharyngeal hypertonicity. The highest predictable success is with grade 2 pouches, whose size is amenable to adequate endoscopic stapling. However, the "staple over staple" effect of revision stapling leads to unpredictable fibrosis, which can contribute to risk of perforation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00405-012-2186-1DOI Listing
May 2013