bluuebunny/crossref_metadata_2025_split huggingface dataset:
630 Hailey-Hailey Disease: A Rare Presentation in the Hypopharnyx
Authors: Tan CH , Wright B , Black M , Devlin B 
Published: November 2022

Abstract

Background
Hailey-Hailey disease or familial benign pemphigus is a rare genetic blistering skin disease due to mutation of the ATP2C1 gene inherited in autosomal dominant fashion. Prevalence is around 1 in 50,000 and positive family history is observed in 75% of cases. Patients typically present with well-demarcated painful erosive and yellow-crusted rash in the skin folds, following a relapsing-remitting pattern. There are very limited known cases of extra-dermatological presentation of this disease.

Case
64-year-old gentleman presented with dysphagia, hoarseness, sore throat, dry cough, and intermittent haemoptysis. Initial flexible nasendoscopy showed mild erythema of epiglottis and false cords, post-cricoid oedema and very mild Reinke's oedema. He was treated as acid reflux with omeprazole but to little effect. Endoscopy 4 months later showed similar findings hence biopsy of the hypopharynx was taken. The sample demonstrated intraepithelial clefting, suspicious of intraepithelial vesiculobullous disorder particularly pemphigus vulgaris.
Looking into this gentleman's medical history, it was found that he is under the care of dermatology for Hailey-Hailey disease, which he was diagnosed 30 years ago. He also has a strong family history of this condition. Based on the biopsy findings and patient profile, it was concluded that this gentleman has a hypopharyngeal manifestation of Hailey-Hailey disease.

Discussion
Hailey-Hailey disease is traditionally described as a dermatological condition. This gentleman is one of the rare few cases of non-dermatological manifestation of this disease.
809 An Unusual Presentation of Left Sided Orbital Pathology- Intraconal Abscess
Authors: Mehtani N , Acharya V , Rennie C 
Published: November 2022

Abstract

Introduction
Orbital cellulitis is a severe, life-threatening infection of the orbit and its contents posterior to the septum.

Case Report
A 41-year-old admitted to hospital under ENT with complete proptosis on left eye and raising intra ocular pressure (IOP) - 26mmHg. He initially presented to Ophthalmology with worsening left eyelid swelling, pain, reduced visual acuity (VA) from 6/18 to 6/12 for the past 4 weeks. This was associated with restricted EOM, proptosis of left eye but intact colour vision and left sided headaches refractory to topical management. His ENT exam including a flexible nasoendoscopy was normal.
The CT Sinuses showed poorly enhancing retro orbital poorly enhancing mass. The differential diagnosis is wide and further assessment with MRI and tissue biopsy highly advised. He subsequently underwent MRI Head and Orbit with contrast: Appearances are suggestive of left periorbital cellulitis with an intraconal abscess.
He was started on intravenous (IV) antibiotics. His IOP had now increase to 35mmHg for which he underwent emergency lateral canthotomy and cantholysis (LCC) in clinic. This successfully reduced the IOP to 21mmHg and he subsequently had formal drainage of the abscess.
He made significant recovery and was discharged home a week after the operation. He went on to have repeat MRI- which showed almost complete resolution of the disease process. He was reviewed 4weeks postoperatively, his VA returned to 6/7.5 unaided 4 weeks but continued to have proptosis in that eye for which he is being followed up.
988 A Case of Cocaine Associated Pneumomediastinum: An Unusual Diagnosis
Authors: Jaly Y , Al-Masri K , Elamin A 
Published: November 2022

Abstract
Cocaine inhalation is an extremely rare but previously described association of spontaneous pneumomediastinum. We describe an unusual case of a previously well 20-year-old female who presented with a five-day history of central, pleuritic chest pain beginning one week after last cocaine inhalation. Blood tests were within the normal limits except for a positive d-dimer. Clinical examination was unremarkable, and a chest X-ray appeared grossly normal. A CT pulmonary angiogram revealed an extensive pneumomediastinum extending into the soft tissues of the neck and down to the peri-oesophageal region at the hiatus, without evidence of pneumothorax. The lungs and oesophagus appeared intact. The patient was admitted for observation and discharged within 48 hours. There is limited literature on this phenomenon, but almost all previous case reports suggest a benign, self-limiting course and resolution of radiological findings within 30 days. Invasive procedures are only considered if there is suspicion of oesophageal rupture or bronchial tree laceration. Spontaneous pneumomediastinum associated with cocaine inhalation is a relatively benign but uncommon condition with a predictable clinical course.
30 An Update on the Impact of Appendicectomy and Deprivation on Disease Course in Ulcerative Colitis
Authors: Sayers A , Brownson E , Nicholson G , Seenan JP 
Published: November 2022

Abstract

Aim
The impact of appendicectomy on the natural history of ulcerative colitis (UC) is unclear. We aimed to assess our cohort of patients with UC to examine the incidence of appendicectomy and association with disease severity. We also examined association of social deprivation with disease severity.

Method
Patients with UC in the health board region were identified by the Gastroenterology consultants. These were divided into moderate-severe (defined as current biologic therapy) and mild-moderate disease (no or 5-ASA therapy only). Two cohorts were sex-matched on a 1:1 basis.
Demographic data was identified by review of the patients’ Electronic Patient Record (EPR) and the Scottish Index of Multiple Deprivation 2020. The EPR was reviewed for evidence of previous appendicectomy.

Results
A total of 664 patients were identified: 328 mild-moderate and 336 moderate-severe. 145 patients (44%) in the mild-moderate group and 145 patients (43%) in the moderate-severe group were female. The majority of patients had left-sided disease. There were no significant differences in smoking status. Rates of appendicectomy in the moderate to severe group were double that of the mild to moderate group (8 v 4; p= .25). Mild-moderate disease was associated with increased social deprivation (X2 (2, N = 664) = 10.60, p = .03) (Fig. 3).

Conclusions
There were double the number of appendicectomies in the mild-moderate cohort, however the relationship between severity and previous appendicectomy was not significant. Disease severity appears to be negatively associated with social deprivation. Further collaborative work across Scotland is planned.
423 Endoscopic Balloon Dilatation for Paediatric Subglottic Stenosis: Systematic Review and Meta-Analysis
Authors: Ravikumar N 
Published: November 2022

Abstract

Aim
Subglottic stenosis (SGS) is a rare life-threatening condition that involves a narrowing of the airway. It may be congenital or acquired affecting children predominantly. Traditionally, it has been treated by surgical interventions, but in recent times a shift towards minimally invasive Endoscopic Balloon Dilatation (EBD) has been observed. This review aims to identify whether EBD is a safe approach in the treatment of SGS in the paediatric population.

Method
A systematic review was performed on EBD for paediatric SGS in compliance with the PRISMA guidelines. Studies published from 2000 onwards, with sample size greater than 5 and described EBD without adjuvant procedures were included. A meta-analysis of proportions was performed using the R software.

Results
25 studies were included, with a total of 1109 patients, of which 920 underwent EBD. The mean sample size of the studies is 43.48 (range 5–166), and the grand mean age is 3.77 years (range 0.7–17 years). Primary outcome assessed was technical success. A high overall technical success rate (successful decannulation and avoidance of tracheostomy/laryngotracheal reconstruction) was observed (83.95%, 95% CI [75.08% - 89.58%]). Similarly, low levels of mortality (3.29%, 95% CI [1.14% - 9.14%]), and high rates of symptom improvement (73.65%, 95% CI [57.81% - 85.09%]) were also observed.

Conclusion
EBD is a successful procedure in majority patients, with low levels of adverse events and marked symptom improvement. It is therefore a safe alternative to current procedures in the management of paediatric SGS.
672 Endoscopic Laser Pharyngeal Pouch Surgery: Is It Safe?
Authors: Dhar P , Maweni R , Kucheria A , Nair D , Nagala S , Corbridge R 
Published: November 2022

Abstract

Aim
Zenker diverticulum can cause significant dysphagia and surgery is the definitive management. NICE guidelines describe inadequate evidence on laser cricopharyngeal myotomy (CPM). This study aimed to assess the safety and efficacy of carbon dioxide laser and stapling CPM to treat Zenker diverticulum.

Method
Retrospective data was collected from patients who underwent endoscopic CPM at the Royal Berkshire Hospital between 2011 and 2021. Two cohorts were assigned: endoscopic laser (EL) or endoscopic stapling (ES). Primary outcomes evaluated safety. These were surgical complication rates, duration of inpatient stay, readmissions, barium swallow leak and considered case complexity (revision cases). The secondary outcomes, persistence or recurrence of symptoms evaluated efficacy.

Results
One-hundred and eight patients (mean [range] age 75 [38 – 95] yr, 71 (66%) men, 23 (21%) revision surgeries) underwent CPM. The EL (n = 76) cohort had two (2.63%) surgical complications (mucosal tear requiring no repair, mediastinitis from an oesophageal leak). The ES (n = 32) cohort had one (3.13%) surgical complication (mucosal tear requiring suture repair). The average duration of inpatient stay was 3.61 and 1.53 days for the EL and ES cohorts respectively. 65 patients (60%) underwent a post-operative barium swallow of which 2 (0.03%) patients, both from the EL cohort, had a leak. There were no readmissions. A chi-square test of independence showed no significant association between type of surgery and persistence [X2 (1) = 0.169, p = .681] or recurrence of symptoms [X2 (1) = 0.443, p = .506]

Conclusions
Treatment of pharyngeal pouches with endoscopic laser CPM is safe and efficacious.
1012 A Close Loop Audit of Acute Food Bolus Obstruction at a District General Hospital
Authors: Thakkar R , Lowe E , Dennis S , Islam M 
Published: November 2022

Abstract

Aim
The British Society of Gastroenterology (BSG) recommends biopsies from two different regions in the oesophagus. A close loop audit was undertaken to assess performance at a district general hospital, against these guidelines and following newly agreed trust guidelines after the first audit cycle of a follow up OGD within 6 weeks of the presentation.

Method
Data was collected of patients admitted with a food bolus over the first cycle (March2019 to February 2021) and the second cycle (1/10/21 to 14/10/21). Exclusion criteria were age of less than 18 and sharp food bolus.

Results
69 patients were identified. Average age was 60 years. There were 67% male patients and 33% female. 20% of patients underwent a follow up OGD in the first cycle and 45%underwent a follow up OGD within six weeks. 7% had two regions biopsy in the first cycle and21% in the second cycle. Average OGD follow up time was 92 days in the first cycle and 59days in the second cycle. Commonest findings on histology results were eosinophilic oesophagitis and Barrett's oesophagus (22% each), GORD (15%) and carcinoma (7%).

Discussion
Food bolus obstruction indicates the presence of pathology of the oesophagus and should be investigated as so, even despite spontaneous recovery from the acute event. Number of patients undergoing OGD within six weeks and a two regions biopsy improved through this audit by implementing the new trust guidelines.
58 The Unfolding Situation of Caecal Volvulus: A Review of 36 Cases
Authors: Mahendran V , Jaradat I , Reddy B 
Published: November 2022

Abstract

Aim
Caecal Volvulus is a rare cause of bowel obstruction. However, there has been a steady rise in the number of cases over the decades. The demographic profile of patients developing Caecal Volvulus has changed to a much older population. We conducted a retrospective review to find the incidence, demographic profile, management, and outcomes of Caecal Volvulus patients in our institution during the last nine years.

Method
A total of 38 patients were diagnosed with Caecal Volvulus at Worcestershire Acute Hospitals NHS Trust between 01.01.2011 to 31.03.2020.

Results
Most of our patients were females (78%) with a median age was 76 years. The majority (86%) had at least one medical comorbidity, and 36% had a previous abdominal operation. Abdominal pain was the main complaint in 94% of patients. All patients had a CT scan to confirm their diagnosis. Most of our patients (84%) underwent surgery. Open right hemicolectomy was the most commonly performed operation (87%). Out of the six patients who did not undergo surgery, three responded to bowel rest and nasogastric tube decompression; one patient underwent successful colonoscopic decompression. In contrast, two patients, unfortunately, passed away. The median length of hospital stay was 9 days, with a 30-day mortality of 3%.

Conclusion
Caecal Volvulus remains a rare cause of bowel obstruction. Most of our patients were old, frail and had medical comorbidities. More than a third of them had undergone previous abdominal surgery. Early CT scan followed by surgery was associated with low mortality.
341 Is CT Imaging Mandated for Confirming the Diagnosis of Acute Appendicitis in Patients Over the Age of 40?
Authors: Shepherd T , Farah M , Aljarad F , Bista G , El-Gaddal A , Ng P 
Published: November 2022

Abstract

Aim
Acute appendicitis remains a largely clinical diagnosis, with clinical scoring systems such as the Alvarado score calculated to give a quantitative assessment of the diagnostic probability. It remains common practice amongst surgeons to rely on CT scanning to confirm the diagnosis however, especially in older patients. Our aim was to assess the diagnostic performance of CT scanning compared with that of the Alvarado score in patients aged over 40 years old.

Method
We conducted a retrospective observational study of 149 patients greater than 40 years old who had a diagnosis of acute appendicitis and had undergone an appendicectomy with subsequent histological analysis. Data was extracted from patients’ electronic medical records. This was recorded in Microsoft Excel and analysed using NCSS.

Results
When using an Alvarado score cut-off point of ≥7, the sensitivity and specificity were 44.1% and 60.0% respectively. The sensitivity of the test improved to 80.3% when using a cut-off point of ≥ 5, however the specificity decreased to 50%. The accuracy improved to 78.1% from 45.3%, when using ≥ 5 as a cut-off point. On the other hand, the sensitivity of the CT scan was 84.9% and the specificity was 45.5%. The accuracy of CT scanning was 81.8%.

Conclusions
When comparing performance, pre-operative CT scanning did not show a statistically significant improvement in the diagnosis of acute appendicitis relative to Alvarado score. We recommend using an Alvarado score cut-off point of ≥ 5 to aid in diagnosing appendicitis in patients older than 40, as it accounts for a higher sensitivity.
574 The Successful Resurrection of NELA in a District General Hospital
Authors: Gohil B , Sarodaya V , White A , Garg A 
Published: November 2022

Abstract

Aim
The NELA requirements of data collection is known to be mandatory. Our DGH had three years of noncompliance. We present an executed plan in the collection of retrospective and prospective process to achieve national standard.

Method
A new structural setup with anaesthetic and surgical teams was formalised. A recruitment of 13 foundation doctors was made and each were induced to NELA with a teaching session. Allocation of 15–20 hospital numbers with a primary deadline of seven days to achieve full entry was expected. A formal NELA certificate and recognition for taking part from the divisional director was issued. A prospective strategy was initiated with patient ownership from the joint anaesthetic and surgical team involved. A NELA login with local access was setup, plus paper format readily available for rare access situations. A two-email reminder system with further escalation process was introduced if entry was incomplete. The escalation being contact from the clinical lead / divisional director. This information was highlighted at a local meeting plus a familiarity of the NELA database was ensured.

Results
A large proportion of the retrospective data has been collected over a short time period due to a clear incentivised scheme. Prospectively there has not been any escalation emails needed since the new setup.

Conclusion
Areas of problems were identified with a wide range of solutions being put forward. A multi collaborative approach was needed plus positive cooperation from the clinical lead, clinical director, and the divisional director to ensure the successful new era.
770 Determining the Diagnostic Value of an Abdominal Ultrasound in Women Presenting with Right Iliac Fossa Pain in an Acute Surgical Unit
Authors: Armstrong L , Sharif A , Thompson R 
Published: November 2022

Abstract

Aim
Right iliac fossa (RIF) pain is a common acute surgical presentation, with appendicitis being the main differential diagnosis. In women of childbearing age, abdominal and pelvic ultrasounds (USS) are often the imaging modality of choice and can be particularly useful when the diagnosis is unclear. We aimed to determine the diagnostic value of USS in patients of childbearing age, presenting with RIF pain.

Method
A single centre retrospective review, of women aged 14 to 45 years old, presenting with RIF pain and undergoing a subsequent USS, over a 6-month period. Investigation results, length of stay, white cell count (WCC) and C-reactive protein on admission, and treatment outcomes were all recorded.

Results
45 consecutive patients were reviewed, with a median age of 28 years (14–45). 37 USS were reported as normal and 8 illustrated gynaecological pathology. None could confirm appendicitis. 24 patients had a normal WCC and a CRP of &lt;5 on admission, none of which had radiological findings requiring surgical intervention. 3 did progress to laparoscopic appendicectomy, 66.6% of which returned with negative pathology. Conservatively managed patients, with a normal USS and normal inflammatory markers, accrued a combined hospital stay of 53 days.

Conclusion
Our results illustrate that in patients with RIF pain and normal inflammatory markers, an USS is unlikely to demonstrate pathology requiring surgical intervention and therefore is of limited positive diagnostic value. However, a negative USS may aid patient reassurance and facilitate earlier discharge in this cohort.
950 Successful Relaunching of NELA at an Inner-City District General Hospital: Need for Multidisciplinary Approach
Authors: Sarodaya V , Gohil B , White A , Garg A 
Published: November 2022

Abstract

Introduction
The national case ascertainment rate for National Emergency Laparotomy Audit (NELA) over 179 hospitals is 84.5%. Our hospital is one of the few hospitals despite being eligible, had not submitted any data to the NELA for the past three years.

Method
We retrospectively collected data for all the patients that underwent laparotomy for a period of 2 years. The mean age of patients was 58 years. We found 83 patients who fit NELA inclusion criteria from January 2020 to November 2021.

Results
The 83 patients were divided into low risk (&lt;5%) and high risk (&gt;5%) and further analysed. The overall mortality was higher than the national (24% v/s 9.3%). Areas of excellent performance against NELA standards (i.e., &gt; 80% compliance) included Consultant's involvement in theatres, Surgeons in 96.9% cases and Anaesthetist in 88.9% cases compared to National average of 96.9% and 93.9% respectively. Areas with poor compliance were Geriatrics involvement pre-operatively (14.7%) when compared to national average (24.7%). Pre-op assessment was done in 48% cases approximately half of national average of 84%. Post operatively 81.4% of the high-risk patients were directly admitted to ITU which was the same as the national average of 85%.

Conclusion
There were various factors which resulted in a low compliance rate. NELA is a useful audit for the comparison of national care. It allows for reflective practice at local level with aim to deliver a uniform standard of care.
147 Synchronous Parathyroidectomy and Extended Thymectomy in Multiple Endocrine Neoplasia Type 1- a Case Report
Authors: Robinson D , Smith A , Bille A , Simo R 
Published: November 2022

Abstract
In recent decades there has been a significant advancement in the management of neuroendocrine tumours (NETs) associated with multiple endocrine neoplasia type 1 (MEN-1). Hormone excess accounted for 75% of all MEN-1 deaths, however since the introduction of hormone blocking medication, deaths in patients with MEN-1 are now predominantly due to the malignant effects of the tumours. Half of all MEN-1 related deaths are due to tumours that are difficult to diagnose, including thymic NETs. Complete surgical resection is the mainstay of treatment for MEN-1 associated thymic NETs.
The case patient was a 49-year-old male who was being investigated for a 3-month history of symptoms consistent with hypercalcaemia. Initially a chest X-ray demonstrated a large anterior mediastinal mass. Further imaging revealed parathyroid lesions and a calcified lesion in the uncinate process of the pancreas. This combination of symptoms and radiological evidence raised the possibility of MEN-1 as the diagnosis. The lesions were biopsy-proven atypical carcinoid NETs and the patient later underwent simultaneous neck dissection for bilateral subtotal parathyroidectomy and a midline sternotomy for total thymectomy of the large mediastinal mass. Further testing revealed the pancreatic mass was another primary tumour and the patient is scheduled for excision of this. In addition, the patient is also being treated with Lanreotide alongside chemotherapy. Mutation of the MEN-1 gene was later confirmed on genetic testing.
Due to the increase in mortality from neuroendocrine malignancy, it is becoming ever more important to understand how to manage multiple NETs from a surgical perspective.
29 The Surgical Trainee Personality: Selected or Shaped?
Authors: Sayers A , Byrne M , Norman I , Jones J , Vig S 
Published: November 2022

Abstract

Aim
This study aims to describe the personality differences between trainees at different stages prior to and during surgical training.

Method
The Mental Muscle Diagram Indicator was distributed electronically to doctors in training in South West London. A total of 553 trainees completed the personality questionnaire. Specifically, there were general surgery specialty (n = 53), core surgical (n = 254), and foundation trainees (n = 246). 52% of trainees were female.

Results
Women scored significantly higher in the Extraversion, Sensing and Judging personality domains (p &lt; 0.0001), there were no significant differences between genders for ‘Thinking’. One-way ANOVA only showed a significant difference in the ‘Judging’ domain between grades. Tukey's test was performed showing the difference arose from FT to CST (p = 0.008). Two-way ANOVA showed an interaction between gender and grade for ‘Judging’ and as such simple main effects were performed, with alpha = 0.017 to control for type 1 errors. Significant differences were found in the Judging domain between genders during in FT (p=0.002) and CST (p=0.002), and there was no significant difference for SpRs (p=0.15).

Conclusions
This study demonstrated differences in personality types between trainee stages and gender. These differences appear to decrease as trainees progress through their training, although the cause of this is unclear.