Zenker's diverticulitis: a bitter pill to swallow.

Acute oesophageal obstruction from food bolus impaction is often triggered by underlying oesophageal pathology, both benign and malignant. These can be readily detected with standard investigations such as oesophagoscopy or computed tomography. Zenker's diverticulum (ZD) is a benign condition frequently presenting with chronic dysphagia or may be asymptomatic. We report the case of an 81-year-old man with a previously undiagnosed 1-cm ZD causing complete oesophageal obstruction secondary to localized oedema from an impacted ibuprofen tablet. Although initial clinical, endoscopic and radiological findings were equivocal and suspicious for upper oesophageal malignancy, symptoms rapidly settled in response to systemic corticosteroids. The diagnosis was later confirmed on barium swallow with no other clinical, radiological or histopathological abnormalities identified. In conclusion, ZD is an uncommon cause of acute oesophageal obstruction which may occur in diverticula of all sizes. Surgery should be performed in patients with recurrent symptoms or large diverticula.

Developing classification criteria for skin-predominant dermatomyositis: the Delphi process.

BACKGROUND: The European League Against Rheumatism/American College of Rheumatology classification criteria for inflammatory myopathies are able to classify patients with skin-predominant dermatomyositis (DM). However, approximately 25% of patients with skin-predominant DM do not meet two of the three hallmark skin signs and fail to meet the criteria. OBJECTIVES: To develop a set of skin-focused classification criteria that will distinguish cutaneous DM from mimickers and allow a more inclusive definition of skin-predominant disease. METHODS: An extensive literature review was done to generate items for the Delphi process. Items were grouped into categories of distribution, morphology, symptoms, antibodies, histology and contextual factors. Using REDCap™, participants rated these items in terms of appropriateness and distinguishing ability from mimickers. The relevance score ranged from 1 to 100, and the median score determined a rank-ordered list. A prespecified median score cut-off was decided by the steering committee and the participants. There was a pre-Delphi and two rounds of actual Delphi. RESULTS: There were 50 participating dermatologists and rheumatologists from North America, South America, Europe and Asia. After a cut-off score of 70 during the first round, 37 of the initial 54 items were retained and carried over to the next round. The cut-off was raised to 80 during round two and a list of 25 items was generated. CONCLUSIONS: This project is a key step in the development of prospectively validated classification criteria that will create a more inclusive population of patients with DM for clinical research. What's already known about this topic? Proper classification of patients with skin-predominant dermatomyositis (DM) is indispensable in the appropriate conduct of clinical/translational research in the field. The only validated European League Against Rheumatism/American College of Rheumatology criteria for idiopathic inflammatory myopathies are able to classify skin-predominant DM. However, a quarter of amyopathic patients still fail the criteria and does not meet the disease classification. What does this study add? A list of 25 potential criteria divided into categories of distribution, morphology, symptomatology, pathology and contextual factors has been generated after several rounds of consensus exercise among experts in the field of DM. This Delphi project is a prerequisite to the development of a validated classification criteria set for skin-predominant DM.

Fatigue in systemic lupus erythematosus and other autoimmune skin diseases.

BACKGROUND: Fatigue is a well-established symptom in systemic lupus erythematosus (SLE), but has not been well characterized in other skin-limited autoimmune diseases such as cutaneous lupus erythematosus (CLE), amyopathic dermatomyositis (ADM) or autoimmune blistering diseases (AIBD). OBJECTIVES: In this retrospective study, we compared fatigue in controls (n = 84) with that in patients enrolled in prospective longitudinal databases with SLE (n = 165), CLE (n = 226), ADM (n = 136) and AIBD (n = 79). METHODS: We used the 36-Item Short Form Survey (SF-36) vitality scale to analyse median scores and the percentages of patients with clinically significant fatigue (defined as a score ≤ 35) between experimental groups and controls. RESULTS: Median and interquartile range (IQR) vitality scores demonstrated greater fatigue in the experimental groups (SLE 35, IQR 20-55; CLE 50, IQR 30-70; ADM 50, IQR 30-65; AIBD 55, IQR 35-70) than in controls (73, IQR 65-85) (P < 0·05 for each experimental group vs. control). The SLE group had worse fatigue than all of the other groups (P < 0·05 SLE vs. each group), but there was no difference between the CLE, ADM or AIBD groups (all P > 0·05). In addition, the experimental groups had more clinically significant fatigue (score ≤ 35) (SLE 44·2%, CLE 25·2%, ADM 31·6%, AIBD 24·1%) than controls (2%) (P < 0·01 for each experimental group vs. control). The SLE group had more clinically significant fatigue than the CLE group (P < 0·01); however, there was no difference in clinically significant fatigue between SLE and either ADM (P = 0·17) or AIBD (P = 0·055). CONCLUSIONS: These findings demonstrate that patients with skin-limited autoimmune disease experience more fatigue than controls. Fatigue is an important symptom that negatively affects quality of life for patients. It should be addressed by clinicians and measured in future clinical trials.

Evaluation of the reliability and validity of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) in paediatric cutaneous lupus among paediatric dermatologists and rheumatologists.

BACKGROUND: The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a reliable outcome measure for cutaneous lupus erythematosus (CLE) in adults used in clinical trials. However, it has not been validated in children, limiting clinical trials for paediatric CLE. OBJECTIVES: This study aimed to validate the CLASI in paediatrics. METHODS: Eleven paediatric patients with CLE, six dermatologists and six rheumatologists participated. The physicians were trained to use the CLASI and Physician's Global Assessment (PGA), and individually rated all patients using both tools. Each physician reassessed two randomly selected patients. Within each physician group, the intraclass correlation coefficient (ICC) was calculated to assess the reliability of each measure. RESULTS: CLASI activity scores demonstrated excellent inter- and intrarater reliability (ICC > 0·90), while the PGA activity scores had good inter-rater reliability (ICC 0·73-0·77) among both specialties. PGA activity scores showed excellent (ICC 0·89) and good intrarater reliability (ICC 0·76) for dermatologists and rheumatologists, respectively. Limitations of this study include the small sample size of patients and potential recall bias during the physician rerating session. CONCLUSIONS: CLASI activity measurement showed excellent inter- and intrarater reliability in paediatric CLE and superiority over the PGA. These results demonstrate that the CLASI is a reliable and valid outcome instrument for paediatric CLE.

The diagnosis and classification of amyopathic dermatomyositis: a historical review and assessment of existing criteria.

BACKGROUND: Diagnostic criteria are used to identify a patient having a disease in a clinical setting, whereas classification criteria create a well-defined population for research purposes. The diagnosis and classification of amyopathic dermatomyositis (ADM) have not been recognized by most existing criteria for idiopathic inflammatory myopathies (IIMs). To address this, several criteria were proposed to define ADM either as a distinct disease entity or as a subset of the spectrum of IIMs. OBJECTIVES: To discuss the diagnosis and classification of ADM and to assesses the available criteria in identifying cases of ADM and/or distinguishing it from dermatological mimickers such as lupus erythematosus. METHODS: We conducted an extensive literature search using the PubMed database from June 2016 to August 2018, using the search terms 'amyopathic dermatomyositis', 'diagnosis' and 'classification'. RESULTS: The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) criteria, which are the only validated classification criteria for adult and juvenile IIM and their major subgroups, include three cutaneous items (Göttron sign, Göttron papules, heliotrope rash) to be able to classify ADM. This international and multispecialty effort is a huge step forward in the classification of skin-predominant disease in dermatomyositis. However, about 25% of the population with ADM do not meet two out of the three skin features and are misdiagnosed or classified as having a different disease entity, most commonly lupus erythematosus. CONCLUSIONS: These gaps rationalize the continuous assessment and improvement of existing criteria and/or the development of validated, separate and skin-focused criteria for DM.

Factors influencing elevated serum apolipoprotein B48 in diabetic and control participants.

Factors influencing the concentration of apolipoprotein B48 (apo B48) at fasting and post-prandial time frames are still being elucidated. This study assesses some possible contributing factors including the presence of type 2 diabetes and gender using an established enzyme-linked immunosorbent assay (ELISA) method. Apo B48 and triglyceride (TG) levels were measured before and for two, four and six hours post-prandially in 49 poorly controlled participants with type 2 diabetes and in 60 apparently healthy participants (controls). Apo B48 levels in the control participants increased post-prandially, peaking at four hours (14.81 ± 7.72 µg/mL) with similar responses demonstrated in TG concentrations. Post-prandial apo B48 levels were significantly higher in male control participants as demonstrated by apo B48 area under the curve (AUC); similar responses were also confirmed in triglyceride AUC. Post-prandial apo B48 concentrations in control participants correlated with HOMA-IR (P < 0.05). Apo B48 continued to increase throughout the six hours in participants with type 2 diabetes (17.73 ± 13.46 µg/mL), when levels were significantly greater than in the control participants (13.04 ± 7.67 µg/mL) (P < 0.05) despite a decrease in accompanying TG levels in participants with type 2 diabetes. Using an ELISA method, this study demonstrated that gender, insulin resistance (as evidenced by HOMA-IR) and diabetes status influence serum apo B48 levels. These effects were only apparent post-prandially.

Day of surgery admission--is this safe practise?

Day of surgery admission (DOSA) describes the process whereby patients are admitted to hospital and have surgery, on the same day. This is the current admission policy in our institution, for most elective Otolaryngology Head and Neck Surgery patients. We audited 75 consecutive patients admitted on the same day as surgery within our department between May 2006 and January 2007. Significant comorbidity was seen in 28 patients (37.3%). Preoperative investigations prior to surgery were conducted in 64 patients (85.3%). About 21 patients (28%) were delayed going to theatre and the average length of delay was 51 mins. Our cancellation rate was 5.3%. Hospital management have embraced the concept of DOSA in our institution without evaluating the risk to patients. If the DOSA policy is to continue it is imperative that an adequate preoperative assessment clinic is established to prevent negative outcomes for our patients.

High resolution computed tomography and magnetic resonance imaging in the pre-operative assessment of cochlear implant patients.

Optimal imaging protocols for cochlear implantation have yet to be determined. Pre-operative computed tomography (CT) and magnetic resonance image (MRI) scans are used to assess cochlear anatomy and patency, to delineate surgical access, and to aid in choice of side for implantation. However, opinion still differs as to which modality provides more information in pre-operative assessment, or if, indeed, a combination of the two is superior. The first 88 patients on the Irish National Cochlear Implant Programme (NCIP) were retrospectively studied to determine the accuracy of pre-operative CT and MRI in predicting abnormalities at the time of surgery. Correlation with surgical findings was determined in three separate groups of patients (those who had CT only, those who had MRI only, and those who had both CT and MRI performed). Of the 24 patients that had both CT and MRI performed, both modalities had a 79 per cent correlation with surgical findings. CT and MRI reports concurred in 75 per cent of cases. Specificity and negative predictive value were high (86 per cent and 90 per cent, respectively). CT alone (47 cases) correlated with surgery in 39 cases (83 per cent); MRI alone (17 cases) correlated in 15 cases (88 per cent). The findings of this study suggest that CT and MRI are effective at predicting normal inner ear anatomy, and thus at predicting the patient and the cochlea most suitable for implantation. Both modalities are useful in determining the side of implantation, thus avoiding potential surgical difficulties in cases of unilateral abnormalities. There was no significant difference between the ability of MRI and CT to detect abnormalities at the time of surgery. In this series the combination of CT and MRI has not been shown to be superior to either modality used alone, although anecdotal evidence to the contrary was noted.

Sigmoid sinus thrombosis: an old foe revisited.

Sigmoid sinus thrombosis (SST) has become increasingly uncommon. In the pre-antibiotic era this condition had a mortality rate of over 90%.1 A high index of suspicion is required to make the diagnosis. We present a rare case of sigmoid sinus thrombosis secondary to mastoiditis, which illustrates the problems of delayed diagnosis. This report highlights the importance of rapid diagnosis and early surgical intervention. We emphasis the need for scanning and otolaryngology referral in all cases of middle ear disease associated with pain or vertigo which does not resolve rapidly on appropriate antibiotic therapy.

Fine needle aspiration cytology of follicular dendritic cell sarcoma. Report of a case with cytologic detection in an extranodal site.

BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare malignancy of accessory immune cells that can present in both nodal and extranodal sites. Previous cytologic case reports of FDCS have focused on fine needle aspiration (FNA) findings in nodal sites with low grade morphology and indolent clinical courses. CASE: A 33-year-old female presented with a three-month history of abdominal distention, early satiety and nausea. Initial imaging studies showed a large abdominal mass, with subsequent studies showing lung, liver and lymph node metastases. Examination of primary and metastatic tumors by a combination of conventional histology, immunohistochemistry and FNA demonstrated an extranodal intraabdominal follicular dendritic cell sarcoma. CONCLUSION: FDCS demonstrates a characteristic cytologic picture on FNA, with important cytologic features, including both syncytial and discohesive large epithelioid to spindled malignant cells with intranuclear inclusions, nuclear grooves and a prominent, mature, lymphocytic inflammatory component. No evidence of morphologic tumor progression was noted in comparison of primary and metastatic tumors. To aid in the cytologic distinction of FDCS from other similar-appearing neoplasms, we recommend acquisition of material for immunohistochemical studies, recognition of diverse clinical presentations (including extranodal and aggressive) and acknowledgment of the range of tumor morphologic grades.

Nitinol stents: their value in tracheobronchial obstruction.

Recurrent tracheobronchial obstruction whether due to a benign or malignant cause is not always amenable to surgery and one is left with the dilemma of what to do for a patient who is slowly asphyxiating. Various modalities have been employed in the past with varying degrees of success. However due to the recent innovations in 'superelastic' biocompatible alloys we present our clinical experience with a new generation of metallic stents made from Nitinol. Over a 12-month period we inserted eight stents in six patients with malignant and benign tracheobronchial obstruction. Almost all patients had dramatic improvement in their symptoms and there was little airway reaction observed up to 18 months after insertion. However, one should exercise caution in inserting them for benign conditions as we regard them as permanent implants. Also evident is the inherent morbidity in this group of patients and this needs to be carefully considered prior to treatment.

Head and neck schwannomas--a 10 year review.

Schwannomas of the head and neck are uncommon tumours that arise from any peripheral, cranial or autonomic nerve. Twenty-five to 45 per cent of extracranial schwannomas occur in the head and neck region and thus are usually in the domain of the otolaryngologist. They usually present insidiously and thus are often diagnosed incorrectly or after lengthy delays, however, better imaging and cytological techniques have lessened this to some degree more recently. For benign lesions conservative surgical excision is the treatment of choice bearing in mind possible vagal or sympathetic chain injury. Malignant schwannomas are best treated with wide excision where possible. The role of adjuvant therapy remains uncertain and irrespective of treatment modality prognosis is poor with an overall survival of 15 per cent. However, recent advances in ras oncogene inhibitors may hold hope for the future.

Segmental colonic transit after oral 67Ga-citrate in healthy subjects and those with chronic idiopathic constipation.

UNLABELLED: Measurement of segmental colonic transit is important in the assessment of patients with severe constipation. 111In-diethylenetriamine pentaacetic acid (DTPA) has been established as the tracer of choice for these studies, but it is expensive and not readily available. 67Ga-citrate is an inexpensive tracer and when given orally is not absorbed from the bowel. It was compared with 111In-DTPA in colonic transit studies in nonconstipated control subjects and then in patients with idiopathic constipation. METHODS: Studies were performed after oral administration of 3 MBq (81 microCi) 67Ga-citrate or 4 MBq (108 microCi) 111In-DTPA in solution. Serial abdominal images were performed up to 96 h postinjection, and computer data were generated from geometric mean images of segmental retention of tracer, mean activity profiles and a colonic tracer half-clearance time. RESULTS: There were no differences in segmental retention of either tracer or in mean activity profiles between control subjects and constipated patients. Results in constipated subjects were significantly different from those in controls. The mean half-clearance times of tracer for control subjects were 28.8 h for 67Ga-citrate and 29.9 h for 111In-DTPA in control subjects and 75.0 h for 67Ga-citrate and 70.8 h for 111In-DTPA in constipated patients. CONCLUSION: Oral 67Ga-citrate can be used as a safe alternative to 111In-DTPA for accurate measurement of segmental colonic transit.

Bacteriology of normal and diseased tonsils assessed by fine-needle aspiration: Haemophilus influenzae and the pathogenesis of recurrent acute tonsillitis.

The pathogenesis of recurrent tonsillitis is largely unknown. Selection of appropriate antibiotic therapy for patients with recurrent tonsillitis is difficult because of the limitations of traditional methods of sampling tonsillar microflora and the increasing incidence of beta-lactamase producing bacteria in the tonsil. In addition, little attention has been paid to the bacteriology of normal tonsils. The tonsil core bacteria was assessed in 124 patients with recurrent acute tonsillitis. Fifty-five of these patients were randomly selected for fine-needle aspiration which revealed a similar profile of bacteria in 85%. Fine-needle aspiration of 10 normal tonsils found few pathogens; the predominant organisms being normal flora. No Haemophilus influenzae were detected in this control group. This study demonstrates the accuracy of fine-needle aspiration in identifying tonsil core bacteriology and its suitability in the clinical setting. It reports on the flora of normal healthy tonsils and it highlights the association between H. influenzae and recurrent acute tonsillitis.