The Management of Locally Invasive Primary Thyroid Paraganglioma: A Case Report and Review of the Literature.

Paragangliomas (PG) are very rare neuroendocrine tumours, arising from neural crest derived paraganglia of the autonomic nervous system. Primary thyroid paraganglioma (PTPG) is a rare site of PG and only 45 cases have been previously reported. The preoperative diagnosis of PTPGs presents a challenge as the clinical, cytological and histological features overlap with more common primary thyroid cancers. A 55 year old male was found to have significant enlargement of the left lobe of his thyroid. Following lobectomy, the thyroid lobe showed unencapsulated tumour which was positive for synaptophysin, CD56 and S100 (sustentacular cells). Post-operative imaging demonstrated incomplete resection. There was no post-operative radiotherapy and monitoring was by 6-12 monthly MRI. 48 months after his surgery he is alive and well with no evidence of disease progression. The diagnosis of PTPG was only made postoperatively, and although rare should be considered in the differential diagnosis of a hypervascular thyroid nodule.

Sudden cardiac death in adults with congenitally corrected transposition of the great arteries.

BACKGROUND: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart disease. There have been only few reports of sudden cardiac death (SCD) in patients with ccTGA and reasonable ventricular function. METHODS: A retrospective review of the medical records of all patients attending our adult congenital heart centre, with known ccTGA. RESULTS: From a database of over 3500 adult patients with congenital heart disease, we identified 39 (∼1%) with ccTGA and 'two-ventricle' circulations. 65% were male. The mean age at diagnosis was 12.4±11.4 years and the mean age at last time of review was 34.3±11.3 years. 24 patients (56%) had a history of surgical intervention. 8 (19%) had had pacemaker implantation and 2 had had a defibrillator implanted for non-sustained ventricular tachycardia (NSVT). In 544 years of patient follow-up, there had been five cases of SCD in our population; 1 death per 109 patient-years. Two of these patients had had previously documented supraventricular or NSVT. However, they were all classified as New York Heart Association (NYHA) class I or II, and systemic (right) ventricular function had been recorded as normal, mildly or mildly-moderately impaired, at most recent follow-up. CONCLUSIONS: Our experience suggests the need for improved risk stratification and/or surveillance for malignant arrhythmia in adults with ccTGA, even in those with reasonable functional class on ventricular function.

Level 5 Lymphadenopathy Warrants Heightened Suspicion for Clinically Significant Pathology.

We conclude that patients presenting with level 5 lymphadenopathy should be investigated with heightened clinical vigilance. Our results suggest that up to 80 % will harbour clinically significant pathology requiring further medical treatment, three quarters of which will be malignancy. We report an observational study of histological outcomes of level 5 lymph node biopsies from a regional histopathology department across 5 years. 184 subjects were identified as having a biopsy of a lymph node from the level 5 region within the study period. One hundred and fifty six cases (84.8 %) had clinically significant pathology on final histology requiring further medical treatment. Lymphoma accounted for the highest number of cases (n = 72, 39.1 %), followed by metastatic carcinoma (n = 65, 35.3 %) and granulomatous change (n = 17, 9.2 %). Gender and laterality were not shown to be independent predictors of pathology significance (p > 0.05).

Immunolocalisation of heme oxygenase isoforms in human nasal polyps.

BACKGROUND: Carbon monoxide is an endogenous vasodilator gas produced by the enzyme heme oxygenase (HO). HO is expressed in human nasal mucosa, but its pathophysiological role in nasal inflammatory diseases is not fully understood. The aim of this study was to detect and compare the expression of HO-1 and -2 isoforms in nasal polyps with normal nasal mucosa. METHODS: Immunohistochemical analysis using antibodies specific for HO-1 and -2 was conducted on nasal polyps from nine patients with allergic nasal polyposis, and on normal nasal mucosa from six controls. RESULTS: Intense HO-1 immunoreactivity was observed in nasal polyp epithelium but was absent in normal nasal mucosa. HO-2 staining was observed in respiratory epithelium, vascular endothelium and seromucous glands, with no difference observed between nasal polyps and normal nasal mucosa. CONCLUSIONS: HO-1 expression is up-regulated in nasal polyp epithelium, supporting the theory that respiratory epithelium plays a role in the pathogenesis of nasal polyposis.

Sino nasal assessment questionnaire, a patient focused, rhinosinusitis specific outcome measure.

It is becoming increasingly important for clinicians to be able to demonstrate the effectiveness of their interventions. We have developed a rhinosinusitis--specific outcome measure (SNAQ-11) that avoids the shortcomings of the existing tools. This paper compares its use with the widely used Sinonasal Outcome Test (SNOT-20). We carried out a prospective study that involved forty patients undergoing endoscopic sinus surgery. Their SNAQ-11 and SNOT-20 scores were compared pre and post operatively. We also recorded individual symptom scores pre and post operatively in order to study the impact of surgery. The study shows a larger change in the postoperative SNAQ score compared to that in SNOT-20 (21% c.f. 11%) Although the pre and post-op changes in SNOT-20 are significant at the p = 0.005 level, the changes in the SNAQ-11 are highly significant at the p = 0.0001 level. Furthermore we have statistically confirmed that the change seen with SNAQ-11 is larger in relation to the variation in change as compared with SNOT-20 (-1.08 c.f. -0.59). Our results show that SNAQ-11 is a valid and highly relevant rhinosinusitis outcome tool. The results also confirm that Endoscopic Sinus Surgery seems especially effective at addressing nasal obstruction, congestion and facial pain/pressure, fair at anterior nasal discharge, sneezing, hyposmia and sleep disturbance and poor for post nasal drip, cough and earache.

Guidelines for the grading of tinnitus severity: the results of a working group commissioned by the British Association of Otolaryngologists, Head and Neck Surgeons, 1999.

Tinnitus is a common experience with up to one third of the adult population experiencing it at some time in their life. Less than 1% of the adult population have tinnitus of sufficient severity to affect their quality of life seriously (although up to 8% may seek medical advice about it). Much of the severity of tinnitus relates to the individuals' psychological response to the abnormal tinnitus signal. The prevalence of tinnitus increases in association with high frequency hearing loss. There is, unfortunately, no diagnostic test that either confirms the presence of tinnitus or its severity. Currently there is no satisfactory severity grading system. A five-point severity grading scheme is therefore proposed and the entry criteria detailed. The five severity points are: slight, mild, moderate, severe and catastrophic. Categorization as 'severe' or 'catastrophic' should be, by epidemiological definition, very rare. General guidance, theory and evidential support are contained within.

Psychological morbidity following laryngectomy: a pilot study.

This study evaluated the nature and extent of problems faced by patients following discharge from hospital following laryngectomy. Semi-structured interviews investigated the practical and psychological concerns of a cohort of laryngectomy patients in a district general hospital. A number of simple practical measures can be employed to reduce early hospital re-admission following laryngectomy. Psychological morbidity is an important cause of low quality of life following laryngectomy. Formal psychological treatment could be of benefit in the management of these patients.

Partial resection of the middle turbinate at functional endoscopic sinus surgery.

The middle turbinate is often carefully preserved at Functional Endoscopic Sinus Surgery (FESS). However there is no clear understanding of its importance and its presence may prevent good access to the middle meatus to the detriment of the surgical result. In addition its bulk may cause symptoms of nasal obstruction and prevent paranasal sinus drainage. Adhesions and stenosis have been reported at the middle meatus following its preservation. We undertook a prospective study of 50 consecutive patients all of whom underwent resection of the anterior half of the middle turbinate at the time of Functional Endoscopic Sinus Surgery. The patients were asked to complete a symptom score sheet before and ten weeks after surgery. The parameters considered were nasal obstruction, nasal congestion, discharge, facial pain and headache. Non parametric analysis of the results indicated a significant improvement in all patient symptom scores and no postoperative complications were noted. We conclude that partial resection of the middle turbinate is a useful modification of accepted endoscopic sinus surgery techniques.

Establishing a new ENT-head and neck clinic.

We report our experience of the first fourteen months of a new head and neck surgical clinic established at a district general hospital. The structure of the clinic together with analysis of the patient case mix and referral patterns are considered. The perceived benefits include rapid patient access to specialist management, the development of subspecialty skills and excellent training opportunities for trainee registrars.

Extracapsular haemorrhage from a parathyroid adenoma presenting as a massive cervical haematoma.

Spontaneous massive haemorrhage from a parathyroid adenoma is rare. It can cause extensive cervical ecchymosis as well as compression of structures within the neck. We present a case, discuss the diagnostic features and review the literature.

Clinical photographs as teaching aids: how reliable are they?

A study to investigate the value and reliability of clinical photographs as teaching aids was undertaken. Twenty colour photographs were taken using the StarMed video-otoscopic system. The pictures, which were a mixture of normal and abnormal ears, were shown to 21 experienced otolaryngologists from the UK and Canada. These clinicians were asked to identify the abnormality if any. The median score for correctly identified pictures was 15 (range 12-18). This score was identical for both the UK and Canadian subgroups. Although the abnormalities were consistently well recognized with an average correct identification rate of 90 per cent (range 67-100 per cent), the 'normals' were recognized significantly less well at only 41 per cent (range 5-71 per cent) (chi-squared = 110.6; 1 df; p < 0.001). This result is probably due to failure of the camera to capture the huge variation and subtleties in the range of normal, and the clinicians' natural inclination to identify pathology, when in doubt. We would conclude that as long as this failing is recognized, clinical photographs, and specifically those from the video-otoscope, represent a useful and reliable teaching tool.