B-cell depletion with rituximab for refractory head and neck Wegener's granulomatosis: a cohort study.

OBJECTIVES: This study aimed to evaluate the response of refractory Wegener's granulomatosis affecting the ear, nose and throat and granulomatous eye disease to B-cell depletion with rituximab. DESIGN: A retrospective case note review. SETTING: Tertiary Centre. PARTICIPANTS: All patients who received rituximab for refractory Wegener's granulomatosis affecting the head and neck were included. MAIN OUTCOME MEASURES: Demographic and follow-up data at five time points were recorded. Response was measured using change in the Birmingham Vasculitis Activity Score and prednisolone dose. Secondary outcomes included changes in additional immunomodulators and anti-neutrophil cytoplasm antibodies serology. Adverse events were recorded for the duration of follow-up. RESULTS: Thirty-four patients were included in the analysis. The median age was 47, the male to female ratio was 3 : 2 and the overall median follow-up was 25.5 months. At six months, nine (26%) patients had a partial response, twenty-one (62%) were in remission and four (12%) did not respond. All four non-responders went into remission after a second course of rituximab. Total Birmingham Vasculitis Assessment score decreased after rituximab at all time points (P < 0.001). Four of five patients with retro-orbital involvement responded well to treatment. Two patients were considered secondary failures requiring alternative therapy after an initial response. Adverse events included four major chest infections, two cancers and six infusion reactions. CONCLUSIONS: Our cohort derived considerable benefit from rituximab permitting a reduction in immunosuppressive exposure and prednisolone dose with few major adverse effects. There was an 80% (4/5) response in patients with retro-orbital granulomas. The effect of rituximab was most noticeable in the first 6 months (88% response).

Near fatal haemorrhage 35 years after radiation for laryngeal cancer: emergency embolisation of a vertebral artery aneurysm.

The authors report a rare case of a 75 year old man who presented 35 years after radiotherapy for carcinoma of the larynx, with catastrophic bleeding through the mouth. Both the time and mode of presentation were unusual. The patient required emergency sacrifice of the left vertebral artery to stop the bleeding. The literature has been reviewed.

Sacrococcigeal and vertebral chordomas. Report of three cases and review of the literature.

Three cases of sacrococcygeal and vertebral chordoma are described. Histogenetic and anatomopathological aspects with particular reference to differential diagnosis from similarly distributed neoplasias are discussed. Anti-cytoskeleton monoclonal antibodies were used to this purpose. The clinical profile of sacrococcygeal and vertebral chordomas is characterized by a pronounced metastatic potential; radiation treatment can only partially counter their biological behaviour, while chemotherapy has proved little or no effectiveness. Where possible, radical surgery is currently the only treatment to guarantee long-term survival or complete cure. Palliative surgery, associate with efficient painkilling, offers a better quality of life and slows down the progress of the disease.

Presentation and initial assessment of ENT problems in patients with granulomatosis with polyangiitis (Wegener's).

BACKGROUND: Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) is a primary systemic vasculitis that affects medium to small sized vessels throughout the body. It often presents with symptoms and signs involving the ear, nose, and head and neck area. OBJECTIVE: To highlight salient features of ENT-related issues in granulomatosis with polyangiitis, and raise awareness of the condition. METHODS: A case report of a patient with limited disease and an insidious onset is presented, along with a review of the current literature. In addition, basic initial management is described. RESULTS: Eighty-six publications were used to describe salient features of ENT-related issues in granulomatosis with polyangiitis. CONCLUSION: The time to diagnosis has not reduced significantly in the last 10 years in the UK. A high index of suspicion is required for an earlier diagnosis of granulomatosis with polyangiitis.

Pneumatisation of turbinates and paranasal sinuses in children: case report.

OBJECTIVE: To describe a rare case of multiple anatomical variations in the sinonasal skeleton of an adolescent with chronic rhinosinusitis, together with its successful surgical management. CASE REPORT: A 15-year-old male adolescent was referred with a 3-year history of nasal blockage and hyposmia. His symptoms did not improve on maximal medical therapy. A pre-operative computed tomography scan revealed numerous anatomical variations in his nose and paranasal sinuses. He underwent neuronavigation-assisted endoscopic sinus surgery without complication, and with subsequent resolution of his symptoms at 10 months. CONCLUSION: Chronic rhinosinusitis in children offers its own set of unique surgical challenges. The effectiveness and safety of sinus surgery in this population can be improved through the knowledge of anatomical variants, and is aided by the use of image guidance systems.

Haemostasis in head and neck surgical procedures: Valsalva manoeuvre versus Trendelenburg tilt.

INTRODUCTION: The aim of the study was to identify whether Trendelenburg position helps detect any further bleeding points following Valsalva manoeuvre in order to achieve adequate haemostasis in head and neck surgery. PATIENTS AND METHODS: Fifty consecutive patients undergoing major head and neck surgical procedures were included. The protocol consisted in performing Valsalva manoeuvre to check haemostasis and treated any bleeding points identified. The operating table was tilted 30 degrees and haemostasis was checked again and treated accordingly. The number of vessels identified and the treatment was recorded. RESULTS: Twelve male and 38 female patients were included. The median age was 53 years and 74% had an ASA of 1. Twelve patients had complicating features such as retrosternal extensions or raised T4 levels pre-operatively. Thyroid resections were the most common operations performed. The total number of bleeding vessels identified in Trendelenburg tilt was significantly greater than when using Valsalva manoeuvre (P < 0.0001). All bleeding points found on Valsalva manoeuvre were minor (< 2 mm) and dealt with using diathermy. In Trendelenburg position, 11% of bleeding vessels required ties or stitching. The time taken during Valsalva manoeuvre was 60 s on average and 360 s in Trendelenburg position. CONCLUSIONS: The results show that the Trendelenburg position is vastly superior to the Valsalva manoeuvre in identifying bleeding vessels at haemostasis. It has become our practice to put patients in Trendelenburg tilt routinely (we have discontinued the Valsalva manoeuvre), to check its adequacy before closing the wound. We have not noticed any intracranial complications using a tilt angle of 30 degrees .

Immunoglobulin G4 related systemic sclerosing disease involving the temporal bone.

OBJECTIVE: To report a rare condition affecting the temporal bone. Immunoglobulin G4 related systemic sclerosing disease is a recently described autoimmune condition with manifestations typically involving the pancreas, biliary system, salivary glands, lungs, kidneys and prostate. Histologically, it is characterised by T-cell infiltration, fibrosis and numerous immunoglobulin G4-positive plasma cells. This condition previously fell under the umbrella diagnosis of inflammatory pseudotumour and inflammatory myofibroblastic tumour. CASE REPORT: We present the case of a 58-year-old woman with multiple inflammatory masses involving the pharynx, gall bladder, lungs, pelvis, omentum, eyes and left temporal bone, over a seven-year period. We describe this patient's unusual clinical course and pathological features, which resulted in a change of diagnosis from metastatic inflammatory myofibroblastic tumour to immunoglobulin G4 related systemic sclerosing disease. We also review the literature regarding the management of inflammatory pseudotumours of the temporal bone, and how this differs from the management of immunoglobulin G4 related systemic sclerosing disease. CONCLUSION: We would recommend a full review of all histological specimens in patients with a diagnosis of temporal bone inflammatory pseudotumour or inflammatory myofibroblastic tumour. Consideration should be given to immunohistochemical analysis for anaplastic lymphoma kinase and immunoglobulin G4, with measurement of serum levels of the latter. Management of the condition is medical, with corticosteroids and immunosuppression, rather than surgical excision.

Vasculitis of the upper and lower airway.

The pulmonary vasculitides are a heterogeneous group of rare disorders that result from an inflammatory process damaging the vessel wall and consequent impaired blood flow, ischaemia and tissue necrosis. The clinical manifestation of these vasculitides depends on the site, size, type and severity of the inflammatory process. Vasculitis involving the airways is a common feature of the anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitides (AASVs) and can predate the diagnosis by years. Secondary causes of vasculitis associated with connective tissue disorders are also capable of presenting with pulmonary features. Recognition of involvement, investigation and treatment are important to ameliorate symptoms for patients. This article concentrates on the assessment and specific management of upper and lower airway problems of AASV.

Canal wall cholesteatoma following canalplasty.

INTRODUCTION: Bony canalplasty is a common otological procedure performed to widen a narrow ear canal. The aim of this report is to describe two unusual patients who presented with a canal wall cholesteatoma many years after bony canalplasty. CASES: Two patients, aged 28 and 52 years, are presented. Both underwent canalplasty, 14 and 17 years before re-presenting with cholesteatoma evident through posterior canal wall defects. Both patients underwent exploration of the mastoid cavities and cartilage reconstruction of the canal walls. There was no recurrence at 24 and three month follow-up examinations (variously), hearing was preserved in both cases, and the patients suffered no early complications. CONCLUSIONS: The most frequent long-term complication of canalplasty is re-stenosis of the external auditory canal. The importance of sealing any inadvertently opened mastoid air cells, in order to avoid the late complication reported, is emphasised.

An unusual case of warfarin-induced pinna skin necrosis.

INTRODUCTION: Warfarin-induced skin necrosis is a rare but recognised complication of this drug. The condition predominantly affects the breasts, buttocks and thighs of obese, peri-menopausal women. We present the case of a patient with the condition in an unusual site, and we discuss the management challenges involved. CASE REPORT: An 82-year-old man presented to the ENT department with a diagnosis of pinna haematoma. There was no history of trauma or infection. The patient was taking warfarin long-term for recurrent deep vein thrombosis. Two weeks prior to admission, the patient had had a loading course of warfarin following surgery. Multiple clinical teams were involved in treatment. The only abnormal laboratory investigation was a low protein S level; biopsy showed skin necrosis. CONCLUSION: In this case, the unusual presentation created diagnostic confusion, and may have precipitated aggressive surgical debridement. However, a more conservative management strategy was used, which we would recommend in future.

Unusual presentation of temporal bone involvement in Churg-Strauss syndrome.

OBJECTIVE: To present a case of a 60-year-old male with a history of sudden onset sensorineural hearing loss due to Churg-Strauss syndrome. CASE REPORT: The patient had a 20-year history of asthma and recurrent right otitis media and a nasal polypectomy four years prior to presenting with ear symptoms. Ear, nose and throat involvement is common in Churg-Strauss syndrome, usually manifesting as allergic rhinitis and chronic rhinosinusitis with or without polyps. CONCLUSIONS: Otolaryngologists play an important role in making an early diagnosis of this disease. To our knowledge this is the first case of Churg-Strauss syndrome primarily presenting with otological pathology: left sensorineural hearing loss and right otitis media.

Presentation of first branchial cleft anomalies: the Sheffield experience.

Abnormalities of the first branchial cleft are rare. They may present with a cutaneous defect in the neck, parotid region, external auditory meatus or peri-auricular area, or with inflammatory or infective lesions at these sites. A retrospective case note review of the patients treated by the senior author is presented. This group consisted of 18 patients and represents the largest published UK series to date. Eleven patients (65 per cent) had undergone incomplete surgery prior to referral. Over half the patients had a clinically apparent lesion in relation to the external auditory meatus. There was a variable relationship between the tract and the facial nerve, which was identified at surgery in 15 cases. These findings are consistent with those of previously published series. Clinicians should keep this diagnosis in mind when assessing patients with infected lesions in the neck and parotid area. Surgeons should be familiar with parotid surgery, in children where appropriate, and be prepared to expose the facial nerve before embarking on the surgical management of these lesions.

Idiopathic maxillary antral mucocele in a child: a rare presentation.

We report a case of a large maxillary sinus mucocele in a 14-year-old girl presenting with epiphora, proptosis and dental pain. This was marsupialized endoscopically, with complete resolution of symptoms over three months' follow up. The literature is reviewed.

[Pancoast's syndrome from the analgesic viewpoint. Our current orientation]. / La sindrome di Pancoast Tobias dal punto di vista antalgico. Nostro attuale orientamento.

Based on the neuroanatomic and physiologic characteristics, the pain from the Pancoast's syndrome is distinguished in cervical-brachial and thoracic-scapular. Two new surgical methods are described for the antalgic treatment of this syndrome: release of the brachial plexus, dissection of the over-scapular nerve and removal of the stellate ganglion, in the first case; dissection of the first two (or three) intercostal nerves and subsequent removal together with their thoracic ganglions, in the second case. The advantages of the above methods and the results reached are discussed.

MFASAT: a new alphoid DNA sequence isolated from Macaca fascicularis (Cercopithecidae, Primates).

A new highly repeated DNA fragment isolated from Macaca fascicularis (MFASAT) is described. Our findings obtained by sequencing, Southern blot analysis, and fluorescent in situ hybridization (FISH) on metaphasic chromosomes strongly suggest that MFASAT can be considered as a member of the alphoid DNA family characteristic of Old World monkeys. The chromosomal localization of MFASAT, obtained by FISH, showed that this alphoid DNA is present in the peri-centromeric area of all the chromosomes. MFASAT showed a high degree of conservation when compared, by sequence alignment, to other Macaca species and Papio papio as expected for species with considerable genome conservation. A low degree of homology has been found comparing M. fascicularis alphoid DNA with a more distantly related Cercopithecidae species such as Cercopithecus aethiops.