Midlands Medicine - Volume 29 Issue 3

MIDLANDS MEDICINE DECEMBER 2020 VOLUME 29 - ISSUE No 3

85

88

Editor’s notes

89

Evidence-Based Medicine: Views from the Arthroscope

93

First Do No Harm

95

True Reflection

97

Saigon to Stoke-on-Trent: a Tale Between Two Cities

100

Case Report: Cranial Osteo-RadioNecrosis: Empyema Drainage and Scalp Reconstruction

104

Interrupted Aortic Arch: a Case Study

111

More Medical Ceramics

112

News

113

Research Notes on the Time of Covid

116

Quiz Night

118

Wordplay in All Our Houses

121

Interesting Image

122

Book Review: War Doctor by David Nott

123

Quiz Answers and Explanations

Midlands Medicine

Midlands Medicine is the journal of the North Staffordshire Medical Institute, whose purpose is to promote postgraduate medical education and research. The journal was first published in 1969 as the North Staffordshire Medical Institute Journal.

COVER IMAGE This is PPE 16th-19th Century style. The object was protection from infection, predominantly bubonic plague, and it would serve that purpose well today in the time of Covid. The beak was filled with flowers, herbs and spices to mitigate the stench of decay. This picture reflects PPE practice in 18th Century France. Picture courtesy of the Wellcome Collections, used under a creative commons licence.

86

Midlands Medicine

MIDLANDS MEDICINE

CONTENTS

EDITOR

EDITORIAL

Dr D de Takats

Editor’s notes

ASSISTANT EDITOR

Evidence-Based Medicine: Views from the Arthroscope Connor Henry-Blake

Mr C Bolger

First Do No Harm Paul Laszlo

EDITORIAL BOARD

True Reflection Marcus German

Mr J. Muir Dr I Smith Mr D Griffiths Helen Inwood Clive Gibson Tracy Hall Professor Divya Chari EDITORIAL ASSISTANT

ORIGINALS Saigon to Stoke-on-Trent: a Tale Between Two Cities William Osborne & Timothy Kemp Case Report: Cranial Osteo-Radio-Necrosis: Empyema Drainage and Scalp Reconstruction Pablo Suarez Benitez & Kostantinos Apostolou

Jacqueline Robinson

Interrupted Aortic Arch: a Case Study Abbie L Randall

THE NORTH STAFFORDSHIRE MEDICAL INSTITUTE

More Medical Ceramics Anthea Bond

President: Mr B Carnes Chairman: Mr J Muir Deputy Chair: Professor Murray Brunt Honorary Treasurer: Mr M Barnish

REPORTAGE News

Please forward any contributions for consideration by the Midlands Medicine Editorial Board to the Editor c/o Jacqui Robinson

Research Notes on the Time of Covid Dominic de Takats

By email: jacqui@nsmedicalinstitute.co.uk

ENDPIECES

Or by post: North Staffs Medical Institute, Hartshill Road, Hartshill, Stoke-on-Trent ST4 7NY

Quiz Night Oluseyi Ogunmekan

Views expressed in articles and papers are those of the author(s) and do not necessarily reflect the views of the Midlands Medicine Journal or the NSMI, nor imply any agreement with, nor condonement of, those views.

All material herein copyright reserved, Midlands Medicine ©2020.

Volume 29, No 3, December 2020

Wordplay in All Our Houses Dominic de Takats Interesting Image Book Review: War Doctor by David Nott Graham Samson Answers and Explanations 87

EDITOR'S NOTES It’s been a year since the last issue of Midland’s Medicine.

That is most unusual. But this has been a year in which many things have had to happen differently. You know why, and so does the whole human world. Viral global pandemics have been the stuff of novels, films and TV series for some time. All that is fantasy; the lived reality, as we are stuck with experiencing for ourselves, is less dramatic in its relentless mundanity and less constrained by any attempt to impose a neat narrative arc on its progress. Put simply, from our current perspective, there’s no clear end in sight, but the craft of history will be to impose a structure on our current experience in a way that we cannot see whilst in its midst.

I hope that you can get some time off over the Christmas season and I’m hoping, with this being a December issue, that you find the time to read through it and savour the contributions which range widely and should provide something of interest to every reader.

reconstructive surgery from Pablo Suarez Benitez and Kostantinos Apostolou. Finally, Abbie Randall takes us on a detailed guided tour of anomalies of the aortic arch.

Once again, Anthea Bond has graced our pages with medical ceramics from the Potteries Museum in Hanley.

Next comes News from the North Staffordshire Medical Institute followed by a contribution of my own which looks at how clinical researchers, epidemiologists and vaccinologists have rapidly and impressively responded to Covid-19. Our local contribution to the RECovery trial has been very significant. Hopefully the article contents lend some hope to a situation otherwise running a little short on that commodity.

We close this issue of the journal with our usual treats, an educational quiz courtesy of Oluseyi Ogunmekan, a play on words and a striking image. We also have a book review by Graham Sansom of the book, War Doctor, by the surgeon David Nott. When Covid-19 This issue starts with the pleading of a case for has long gone, I’m sorry to say that a return to ‘business evidence-based orthopaedics by Connor Henry-Blake, as usual’ for the world may mean another call to action a Keele medical student. He makes the point that for David Nott and those following him. some procedures gain widespread use and through popular momentum rather than rigorous evidence It is an ill wind… and so I’m delighted to note just how for their beneficial use, and that might sow the seeds much new language this pandemic has brought us. Some for harm. This chimes very much with the evidence of that is covered in the twentieth outing of Wordplay, presented by Baroness Cumberlege in her report issued but there is such a wealth: ‘take down services’ only to earlier this year, First do no harm. Paul Laszlo gives us ‘build back better’ as part of a ‘restoration and recovery the headlines from her report into a number of ways programme’ are but a few to conjure with. And I leave embedded medical and surgical practice have been you with the new valediction: causing harm rather than doing good, particularly to women and children. You should read his editorial and Stay safe! then take a look at her report. We should all do better.

Certainly, this is something to reflect on, and if you are Happy Reading this Christmas season. not quite sure how to go about that, Marcus German has a few handy tips.

Next in this issue we are blessed with a series of three very interesting case reports, each very different, each fascinating. We start with an infectious disease journey of great distance and duration unravelled and explained to us by William Osborne and Timothy Kemp (Stoke is not an island) we learn about complications of radiotherapy and the consequent need for heroic 88

Midlands Medicine

EVIDENCE-BASED MEDICINE: VIEWS FROM THE ARTHROSCOPE Connor Henry-Blake, Medical Student, KUSoM TREATING DEGENERATIVE KNEE DISEASE One in four adults over 55 suffer from long term knee pain due to degenerative knee disease.1 Although the only curative treatment is a total knee replacement2, knee arthroscopy (KA) rose to become one of the most commonly performed orthopaedic surgeries .3 In 2007 NICE recommended the use of arthroscopic surgery for degenerative knee disease.4 However more recent research has shown that only patients with symptoms of mechanical locking benefit from KA. This is reflected in the most recent BOA5 and NICE2 guidelines.

NICE’s original recommendation of KA wasn’t supported by strong evidence. In 2002, a randomised controlled trial (RCT) found KA to have no benefit in patients with degenerative knee disease when compared to exercise alone.6 Additionally, KA has a 2-4-week recovery time and may lead to post-operative complications including pyogenic arthritis and deep vein thrombosis.7,8 Furthermore, KA has a large financial burden, costing several billion dollars (USD) globally.9 Why then did KA become so popular, and what can be done to further drive evidence-based practice?

THE RISE OF ARTHROSCOPY Confirmation Bias Clinical experience, alongside awareness of the relevant research, should inform treatment options. Clinical experience alone does not allow for a longitudinal unbiased assessment of treatment outcomes. For instance, those patients who undergo KA report short term improvements in function and pain. However, this benefit diminishes over time, with less than 15% reporting any improvement after 2 years.10 When surgeons are presented with these short-term

Volume 29, No 3, December 2020

benefits, they can become overly emphasised due to unconscious confirmation bias. This happens when outcomes that align with a person’s pre-existing beliefs are overly emphasised, and outcomes that challenge these beliefs (for example the lack of long-term benefits following KA) are devalued. Over-reliance on our clinical experience can therefore result in patients receiving unnecessary treatment, and this has likely contributed to the rise in popularity of KA. Lack of High-Quality Evidence The implications of confirmation bias are multiplied when there is a lack of rigorous evidence, which was the case during the rising popularity of KA. Between 1966 and 2000 only 3.4% of the articles published in 5 leading surgical journals were randomised controlled trials.11 This resulted in reliance on case reports and cohort studies which are inherently open to bias. This difficulty in obtaining an unbiased assessment of KA contributed to its popularity.

What Can Be Learnt?

The popularity of KA illustrates two points. Firstly, in order to prevent the widespread use of ineffective techniques, we need to promote a strong culture of research in orthopaedic surgery. Secondly, we need to encourage the development of skills to allow surgeons to critically analyse research against their experiences to decide what treatments to offer patients.

ENCOURAGING EVIDENCE-BASED ORTHOPAEDICS Trainee-Led Research Collaboratives The key to implementing more evidence-based practice lies in the early involvement of research in careers. An effective way to do this is through trainee-led research collaboratives (TLRCs).11 TLRCs allow surgical trainees, led by experienced researchers, to get handson experience. This results in greater appreciation for

89

research methodologies and teaches trainees the tools to critically appraise papers effectively. The added benefit of TLRC’s is that it ensures high quality evidence, with multicentre projects, producing larger and more significant results, which improves the evidence base.

Research Summaries An appreciation of research methodologies is not enough, as the number and quantity of research is increasing over time, being further dispersed through more journals. It is impractical to expect all surgeons to keep completely abreast and critically analyse all the relevant research. Therefore, a filter needs to be developed that can succinctly inform surgeons of cutting-edge research in their field. Evidence based tools such as “ACCESSSS” by McMaster plus do just that.

Research is filtered, critically evaluated and directed to the relevant surgeons. This increases the utilisation of evidence12 and reduces the delay in the translation of evidence into clinical practice.

Improving Implementation Greater encompassment of the IDEAL framework will prevent unnecessary interventions becoming popularised. To improve clinical implementation, professional societies such as BOA should ensure their members are aware of the benefits of the IDEAL framework. This can be publicised through guidelines, or through the suggestion that innovative procedure must follow the IDEAL framework in order to be recognised for awards.

CONCLUSION

To prevent the implementation of procedures with little clinical utility, there is a responsibility for all of us to implement evidence-based orthopaedics.

Trainees need to be able to recognise their own biases and critically evaluate research. Researchers need to adopt transparency and staged implementation of all innovative procedures. Journals need to ensure their findings are summarised and targeted to the people Stepwise Introduction Reliance on case reports and cohort studies contributed they matter to most; and professional bodies such as to the rapid rise in arthroscopic surgery.13 Practice BOA, have the biggest challenge as they must encourage accelerated at a pace that the evidence could not keep all these individual bodies to adopt evidence-based up with. This is particularly found within surgery orthopaedics to prevent the mistakes of the past being where RCT research is often difficult. This has led to the repeated in the future.

IDEAL collaborative developing a framework to allow for the stepwise integration of innovative procedures, to ensure that clinical practice does not outpace the evidence that it should rely upon.14 IDEAL Framework The IDEAL collaborative is an international group that recognise the importance of transparent, clearly staged research to prevent the widespread implementation of procedures without evidence. It separates innovation in surgery into 5 steps, summarised in Figure 1, adapted from their paper published in the Lancet.15 The IDEAL framework aims to hit the balance between allowing unbiased rigorous evaluation of clinical procedures, without stifling innovation.

90

Midlands Medicine

Figure 1- Summary of the IDEAL framework, adapted from (McCulloch et al., 2009)8

ADDRESSES FOR CORRESPONDENCE

REFERENCES

C.J.Henry-Blake@keele.ac.uk Connor Henry-Blake 88 Cannon Hill Road Coventry CV4 7BS

1.

Volume 29, No 3, December 2020

Moseley JB, O’Malley K, Petersen NJ, Menke TJ, Brody BA, Kuykendall DH, et al.

A Controlled Trial of Arthroscopic Surgery for Osteoarthritis of the Knee.

2002 Jul 11;347(2):81–8.

Available from: http://content.nejm.org/cgi/ content/abstract/347/2/81

91

2.

Haynes RB, Holland J, Cotoi C, McKinla

y RJ, , Wilczynski NL, Walters LA, et al. Mc Master PLUS: a cluster randomized clinical trial of an intervention to accelerate clinical use of evidence-based information from digital libraries. 2006;13(6):593–600. doi: 10.1197/ jamia.M2158

9. Cullen KA, Hall MJ, Golosinskiy A. Ambulatory surgery in the United States, 2006. 2009 Jan 28;(11):1–25.

10. Wente MN, Seiler CM, Uhl W, Büchler MW. Perspectives of Evidence-Based Surgery.

2003;20(4):263–9. Available from: https://www. karger.com/Article/Abstract/71183

3.

Heidari B. Knee osteoarthritis prevalence, risk 11.

Siemieniuk RAC, Harris IA, Agoritsas T,

factors, pathogenesis and features: Part I. Poolman RW, Brignardello-Petersen R, Van 2011;2(2):205–12. de Velde S, et al. Arthroscopic surgery for 4.

MPH K Jeffrey N.MD, MSc|Brownlee, Sarah degenerative knee arthritis and meniscal tears: a

A.BA|Jones, Morgan H.MD,. The role of clinical practice guideline. 2017;357:j1982. doi:

arthroscopy in the management 10.1136/bmj.j1982

of knee osteoarthritis.

2014;28(1):143–56.

12.

Jackson RW. Debate on the use of arthroscopic

Available from: https://www.clinicalkey.es/ surgery for osteoarthritis of the knee. 2003 Jan playcontent/1-s2.0-S1521694214000096 1;16(1):27–9. [accessed 21 Feb 2019] Available 5.

National Institute for Health and Care

Excellence (NICE). Osteoarthritis: care

and management | Guidance 13.

and guidelines | NICE. NICE; [accessed 21

Feb 2019] Available from: https:// www.nice. rg.uk/guidance/cg177/ chapter/1-recommendations 6. Lubowitz JH, Ayala M, Appleby D. Return to activity after knee arthroscopy. 2008 Jan;24(1):58- 14.

61.e4. doi: 10.1016/j.arthro.2007.07.026 7.

Excellence NI for H and C, (NICE).

Arthroscopic knee washout, with or without debridement, for the treatment of osteoarthritis | Guidance and guidelines | NICE. NICE; [accessed 21 Feb 2019] Available from: https:// www.nice.org.uk/guidance/ipg230/chapter/2- 15.

The-procedure#other-comments 8.

McCulloch P, Altman DG, Campbell WB, Flum DR, Glasziou P, Marshall JC, et al. No surgical innovation without evaluation: the IDEAL recommendations.

2009 Sep 26;374(9695):1105–12. doi: 10.1016/S01406736(09)61116-8

92

from: https://www.ncbi.nlm.nih.gov/pmc/ articles/PMC1200806/

Barkun JS, Aronson JK, Feldman LS, Maddern GJ, Strasberg SM. Evaluation and stages of surgical innovations. 2009;374(9695):1089–96. [accessed 21 Feb 2019] Available from: https:// www.thelancet.com/journals/lancet/article/ PIIS0140-6736(09)61083-7/abstract Association BO, Surgeons RC of. Commissioning Guide: Painful Osteoarthritis of the Knee.

NHS; 2017 Nov p. 7–8. (British Orthopaedic Association). [accessed 2019] Available from: https://www.boa.ac.uk/wp-content/ uploads/2014/01/Painful-OA-Knee-GuideFinal-.pdf Friberger Pajalic K, Turkiewicz A, Englund M. Update on the risks of complications after knee arthroscopy. 2018 Jun 1;19(1):179. doi: 10.1186/ s12891-018-2102-y Association). [accessed 2019] Available from: https://www.boa.ac.uk/ wp-content/uploads/2014/01/Painful-OAKnee-Guide-Final-.pdf

Midlands Medicine

FIRST DO NO HARM Paul Laszlo, Consultant Physician “We have found that the healthcare system – in which I include the NHS, private providers, the regulators and professional bodies, pharmaceutical and device manufacturers and policymakers – is disjointed, siloed, unresponsive and defensive. It does not adequately recognise that patients are its raison d’etre. It has failed to listen to their concerns and when, belatedly, it has decided to act it has too often moved glacially.”1 Mention Jeremy Hunt and you will get many different responses. Many of them will be somewhat hostile.

Think of his legacy and perhaps you will think of the “new” junior doctors’ contract. But what you might not immediately think of is this report by Baroness Julia Cumberlege, First do No Harm1. But this report, commissioned by Jeremy Hunt when he was Secretary of State for Health published long after he had slid from the position to the back benches, might be his best legacy if it can reach the consciousness of all of us in a position to put into practice and influence colleagues pay it the heed it deserves.

• Hormone pregnancy tests (HPTs) – tests, such as Primodos, which were withdrawn from the market in the late 1970s and which are thought to be associated with birth defects and miscarriages; • Sodium valproate – an effective anti-epileptic drug which causes physical malformations, autism and developmental delay in many children when it is taken by their mothers during pregnancy; and

• Pelvic mesh implants – used in the surgical repair of pelvic organ prolapse and to manage stress urinary incontinence. Its use has been linked to crippling, lifechanging, complications; The report was published into potential obscurity and to make recommendations for the future.” because of its timing during the tail of the first wave of the Covid-19 pandemic in the UK. We have had so Key elements allowed these three areas to be considered much to think about and it’s so easy to move on. But together as well as individually, such as the fact that the the reason for this report was a heavy sense of not primary users were women, that they had been drawing being listened to by people who felt themselves to be attention to the problems they had faced for many years victims of the healthcare system and its regulation; not with, seemingly, little evidence of having been heard. giving this report the attention and due regard it merits Worse still, at times, not being believed. would compound the original offence of not listening to patients, which would be a terrible shame. Worse than The report is a detailed, thorough piece of work, as that, it would be a lost opportunity to do better in the comprehensive as it could be within its brief with the information available. It makes nine recommendations. future.

The first five are to do with making good what has gone As the longest serving UK Health Secretary since World before: redress. The next four consider making thigs War 2, Mr Hunt was in office long enough to be found better for the future: by a number if interest groups reaching out to be heard by the powers that be in healthcare and its regulation.

With common themes, he felt that there were sufficient grounds to commission a review, which he dis in February 2018. The remit: “… the Review was asked to investigate what had happened in respect of two medications and one medical device:

Volume 29, No 3, December 2020

Recommendation 6: The MHRA needs substantial revision, particularly in relation to adverse event reporting and medical device regulation. It needs to ensure that it engages more with patients and their outcomes. It needs to raise awareness of its public protection roles and to ensure that patients have an integral role in its work.

93

Post marketing surveillance for drugs needs to be systematic and comprehensive and medical devices all need to be entered into a registry. This is work that can be done. It needs to be both mandated and incentivised.

The commercial companies need to be made to fund it, but governments and healthcare agencies should own the data, employ their own epidemiologists to mine it and encourage academics to look to interpret it too.

Recommendation 9: The Government should immediately set up a task force to implement this Review’s recommendations. Its first task should be to set out a timeline for their implementation.

Sadly, the danger lurks that no such thing will happen due to Brexit followed by Covid-19 followed by Brexit and more Covid-19 (in 2021). If you find yourself with time on your hands during some period of isolation Recommendation 8: Transparency of payments or lockdown, you could do your patients considerable made to clinicians needs to improve. The register service by looking this report up on line and reading of the General Medical Council (GMC) should be through it. expanded to include a list of financial and nonpecuniary interests for all doctors, as well as doctors’ particular clinical interests and their recognised and accredited specialisms. In addition, there REFERENCES should be mandatory reporting for pharmaceutical First Do No Harm The report of the and medical device industries of payments made 1.

Independent Medicines and Medical Devices to teaching hospitals, research institutions and Safety Review, Baroness Julia Cumberlege individual clinicians.

(2020) Transparency allows interested parties to infer conflicts https://www.immdsreview.org.uk/downloads/ of interest when they might not be openly declared IMMDSReview_Web.pdf as such. A case to answer might be made for why a particular device appears to be favours, or promoted even, by a particular hospital or institution. It allows questions to be asked and forces answers to be formed.

That might actually cause those in the midst to consider the merit of the question and ask themselves whether working relationships with commercial sponsors have gotten a little close, swayed opinion, perhaps, or deflected scrutinising gaze a little.

Technological progress has merely provided us with more efficient means for going backwards Aldous Huxley

94

Midlands Medicine

TRUE REFLECTION Marcus German, Life Coach

It is the case that reflection written down and read by others could give the impression of being either good or bad. Good reflection gives the impression that someone has thought deeply about the subject in question, understood the implications and the relationships of the matter to professionalism, expectations of the medical profession and perhaps wider social ethical concern.

And then made some effort to relate the events, and their thoughts about them, to their medical practice.

Bad reflection gives the impression of someone going through the motions of reflection with no insight gained and no learning achieved.

A STRUCTURED APPROACH The advantage of a structured approach in reflective practice is the same as the advantage of structure in any other situation, namely that it brings a consistency of approach and a comprehensiveness that might otherwise be lacking. A good structure will allow logical flow of thoughts so that the piece makes sense as a whole. it should be easy to read and make sense to a third party reader; they should be able to come away with a genuine appreciation of the events and their INTRODUCTION circumstances, know how you felt and how you have “Medicine is a lifelong journey, immensely rich, responded to the events discussed and understand how scientifically complex and constantly developing. you have processed experiences, gaining some notion It is characterised by positive, fulfilling experiences of how you might have been influenced to respond and feedback, but also involves uncertainty and the differently in the future. What follows is a simple, yet emotional intensity of supporting colleagues and rather demanding, outline for good reflective practice. patients. Reflecting on these experiences is vital to Begin at the beginning personal wellbeing and development, and to improving Describe in outline, but in sufficient detail to allow a the quality of patient care.” is what the GMC has to say reader to fully appreciate the circumstances, the events about reflective practice on its website. But just what is and/or circumstances that provoked or inspired the true reflection? There's no use asking doctors to reflect reflection. Be careful to avoid detail that could identify when they have little idea how to go about it. After all, any specific individuals involved; create enough it has only been a few years since the idea of reflective distance between individuals involved and the account practice was thrust onto the unsuspecting medical to make any patients or colleagues unidentifiable with profession. Since its introduction there have been many any certainty. For example DO NOT state: “I saw Mr ideas about just what exactly should be going on with AW, a white man in his sixties in the COPD clinic in reflection and just what its purpose might be. Slowly February”, rather you can leave it at “I saw a middlebut surely these ideas have become a little more firmed aged man in clinic…” up and one can begin to try to capture some are concise thoughts on just how reflection might be seen.

Volume 29, No 3, December 2020

95

Don’t just give medical histories, but also give an account of the circumstances and other factors which may have influenced how you managed things on that day, for example, were you in familiar surrounds? Were you tired or well-rested? Were you distracted or hassled in any way? How familiar were you with what was being asked of you? Was the presentation an example of a common presentation masking a rare disease or a common disease presenting in an unusual way? If it was a situation you found yourself in, was it an unusual situation, rare in the extreme, or a common enough scenario with a twist that made all the difference? If so, the details may mater and an analysis of the key points that made all the difference on this occasion should be where your reflective focus fixes.

Finishing up Finally, you should look at what you have learnt and how that might amend your future practice. Are there any facts you have newly learned, pitfalls to avoid or things that would make the situation better next time around? A series of specific learnings should come towards the end of your reflection piece. Sometimes a numbered list or a series of bullet points may aid clarity of expression, thought and presentation, in other cases a more philosophical conclusion might be stated.

Sometimes both may be appropriate. When you’ve finished, read it through and make sure it reads well and is a true reflection of the process you’ve been through.

If you’re feeling brave, perhaps you could bring yourself to share the reflection with one or two colleagues, have a discussion, take some feedback and then do a little Present your distilled thoughts more cogitation and research and then incorporate this If you are writing a reflection, it is because something further reflection into your work. has happened that’s notable, memorable, has irked you and you’ve had at least one thought about it and you IT’S GOT TO BE REAL want to capture that thought, examine it, interrogate it and learn something. But it isn’t going to be just one You could fake it, but to work it's got to be real thought. There are plenty of questions you can ask (genuine, authentic, sincere). One of the dangers of a yourself, all the “What if?”s. You should put a good structured approach it will enable those of you who feel number of those down on paper and have a go at that reflective practice is just another hurdle to be got answering them. Some will be worth pursuing in more through to approach that hurdle with confidence and detail and others can be discarded. The ones you decide give a good performance, passable as the real deal to to keep, capture them fully, then do some research om the casual observer, but not in fact the genuine article. them, see if you can develop them further, make some Such a performance, and that's just what it would be, learning from the occasion. Then get this down in a merely, may get you through revalidation but will form of words that fairly reflects the distillation of that not do your patients any good at all. The path of true process. reflection may not run smoothly but if you can make the effort to deeply understand the issues and principles Reflect on your feelings at hand, perhaps seeing things from other people’s This is going to be the most difficult bit for those of a perspectives, there is genuine opportunity to grow both more traditional reserved nature. My advice for this professionally and personally. part is that it need not be particularly long, it just needs to be an honest insight. If you felt angry, say so; if you felt let down, acknowledge that, being careful not to identify individual you feel are culpable, and state what, in your view, better support would have looked like; if you feel proud, say so, then say why you feel that way; if you feel you did okay, then consider the pitfalls you managed to avoid and how you did that and what better would have looked like and how that might be achieved.

96

Midlands Medicine

SAIGON TO STOKE-ON-TRENT: A TALE BETWEEN TWO CITIES William Osborne Registrar in Infectious Diseases, UHNM Timothy Kemp, Consultant in Infectious Diseases, UHNM INTRODUCTION A travel history is sometimes a key element of the medical history. When the problem is an infectious disease, a well-taken travel history allows an epidemiological risk assessment for acquiring non-endemic infections to be made and management adjusted in its light.

Infections usually present within days-to-weeks after return however much longer incubation periods can be seen. It also provides a vignette into a patient’s life.

We present case of an infection acquired years prior to presentation and the tale between.

CASE A 66 year-old woman was referred to the Emergency Department of a tertiary hospital by the out-of-hours GP within the Midlands with abdominal pain, fevers, rigors, nausea and diarrhoea. She had returned from a three-week holiday from Fuerteventura. Her past medical history included hypertension and hypercholesterolaemia for which she took atorvastatin 20 mg at night. She was born in Vietnam and coming to the UK more than 30 years earlier. She lived with her husband, had never smoked and did not drink alcohol regularly. On examination she was febrile at 37.9 oC. Blood pressure was normal and pulse rate was 96 bpm.

The only abnormality on examination was a tender right-upper quadrant and epigastrium with no signs of peritonism. Initial blood tests revealed a raised CRP of 292 mg/L, abnormal liver function tests (ALP 311 iu/L, ALT 175 iu/L, gamma GT 647 iu/L, albumin 29 g/L) with normal bilirubin. Total white cell and neutrophil count were not raised but there was eosinophilia (1.5 × 106 cells/ml). Intravenous (IV) co-amoxiclav and fluids were started, after blood cultures were drawn, for presumed cholangitis and an ultrasound of the abdomen was requested.

multiple smaller cystic structures suggestive of hydatid disease. An infectious diseases opinion was sought; oral albendazole 400 mg twice daily was started and due to persistent fevers, antimicrobials were changed to IV meropenem due to the risk of colonizing extended spectrum beta-lactamase -producing enterobactericae (ESBL). Serum was sent to the Hospital of Tropical Diseases London for hydatid, strongyloides, fasciola and schistosoma antibody detection.

Screening rectal swab was positive ESBL-producing E. coli and serology was strongly positive for hydatid. Further imaging was acquired with computed-tomography and magnetic-resonance imaging (Figure 1). The patient was transferred to the Royal Liverpool Hospital for consideration of curative resection. After two weeks of IV meropenem the patient was discharged with the intention of elective resection after a period of albendazole therapy. The patient was re-admitted with cholangitis which was successfully treated IV meropenem for two weeks. Three months after the index presentation, the patient underwent successful complete resection of the hydatid cyst.

Abdominal ultrasonography revealed a 15 × 13cm cystic structure within the liver, within which were Figure 1

Volume 29, No 3, December 2020

97

DISCUSSION Hydatid disease, or echinococcosis, is a zoonotic disease caused by ingestion of the embryonated eggs of the Echinococcus tapeworm.1 There are two main species of Echinococcus of clinical importance, both of which produce distinct disease entities. Echinococcus granulosus, ‘Cystic Echinococcosis’, has a worldwide distribution and produces slow-growing cystic lesions in almost any organ. Further cysts develop within it, creating ‘daughter cysts’. Hydatid fluid within these cysts contain infective scolices which can create more cysts. Echinococcus multilocularis, ‘Alveolar Echinococcosis’, is confined to Northern America, Siberia, parts of Eastern Europe and central Asia. It produces rapidly-growing lesions that present mimic solid organ tumours.

The lifecycle is complex. The adult tapeworm resides within the intestine of the definitive host (e.g. dogs, foxes and other canids) and excretes infective eggs in faeces that the intermediate host (e.g. sheep, rodents and wild herbivores) ingests. The eggs hatch in the intestine of a suitable intermediate host and disseminates via the bloodstream to various organs, especially the liver and lungs; cysts then develop. If the host and its organs are consumed by another suitable definitive host, the lifecycle is completed. Humans are accidental intermediate hosts. Diagnosis can be made with characteristic imaging and a relevant travel history. Serology can be confirmatory. For single cysts, complete excision provides a curative option. Less invasive surgical options involve the ‘PAIR’ procedure which involves percutaneous puncture with a needle, aspiration of its contents, instillation of a scolicidal agent (e.g. hyptertonic saline) and re-aspiration.

Albendazole, a benzimidazole agent, has good activity against Echinococcus spp. and is an alternative option for those with multiple or inoperable cysts; treatment can be for years or lifelong. It is also used prior to surgery to reduce the risk of live scolices disseminating and producing life-threatening anaphylactoid reactions if there is accidental rupture of a cyst.

Vietnamese fled. The term ‘Vietnamese boat people’ was used for this mass migration of South Vietnamese and ethnic Chinese refugees across the South China Sea. The patient and her husband left in 1979 aboard a boat with 600 other people. After several weeks at sea, a British-registered freighter, the SS Sibonga (Figure 2) captained by Healey Martin, travelling from Bangkok to Hong Kong, spotted the boat on 21st May 1979 and took them aboard (Figure 3). The refugees had been running low on food and water, the boat had started to sink and five babies had been buried at sea the night before.2 Shortly after the rescue, another boat was spotted in distress and another 300 refugees were rescued. They were looked after by the crew and the wife of the ship’s captain, Mildred Martin.2

Figure 2

MORE HISTORY A more detailed travel history was taken from the patient. After the Fall of Saigon in 1975, many South Vietnamese were sent to ‘re-education’ camps within rural areas. The patient’s father, a chef for the South Vietnamese police, underwent re-education. As hostility intensified, the economic situation worsened and the Chinese invasion of the North, many South 98

Figure 3

Midlands Medicine

Our patient, with 1002 other rescued people, arrived in Hong Kong on 24th May. After quarantine aboard, she was brought ashore and spent several months within one of many refugee camps across the island. During an outbreak of measles across the camps, people were fed sheep’s liver for its high vitamin A content. The refugee camp is the likely source of infection with echinococcosis for our patient. From 1979 through the 1980s, hundreds of thousands of refugees arrived in Hong Kong. The recently elected Conservative government in the UK led

by Margaret Thatcher, initially refused to accept refugees contrary to most other countries fearing civil unrest. In July 1979, prior to a UN conference on the crisis, the UK Government yielded and accepted 10,000 refugees over three years who were soon settled throughout the UK.

ADDRESS FOR CORRESPONDENCE william.osborne@nhs.net REFERENCES 1. CDC - Echinococcosis. https://www. cdc. gov/ parasites/echinococcosis/index. html (accessed November 11, 2019) 2. Grant B. The Boat People: An “Age” Investigation. Penguin Books Ltd; 1979 3. UNHCR - Figures at a Glance. https://www. unhcr. org/uk/figures-at-a-glance. html (accessed November 11, 2019)

Captain Healey Martin, of Lisburn in Northern Ireland, still receives birthday cards to this day from those he rescued on 21st May 1979.

MORE DISCUSSION Tropical infections are not solely acquired during leisure travel. There are currently 70.8 million people forcibly displace worldwide, over half of whom are under the age of 18.3 With widespread conflict and climate change, this number is increasing. A refugee’s journey, both recent and distant, is more often than not, difficult and dangerous. In addition to infections such as HIV, tuberculosis and parasites, there are frequently physical and psychological injuries associated with trauma, torture and sexual assault. Listening to a patient’s tale can not only be diagnostic and therapeutic in itself, but also be enlightening and informative.

Volume 29, No 3, December 2020

99

CASE REPORT: CRANIAL OSTEO-RADIO-NECROSIS: EMPYEMA DRAINAGE AND SCALP RECONSTRUCTION Pablo Suarez Benitez, Year 2 Medical Student, KUSoM Kostantinos Apostolou, Consultant Plastic Surgeon, UHNM INTRODUCTION This case was originally the winning presentation at the last Keele University Medical School Surgical Society Case Presentation Evening before Covid-19.

Mr J is a 74-year-old Caucasian male from Shrewsbury with a myriad of co-morbidities.

of the face, giving it a characteristic “droopiness”. After a 999 call and short admission to A&E in Telford he was sent home with aspirin, as it was suspected that he had only had a TIA supported by his presenting symptoms, their rapid onset and his strong history of strokes.

He has a past medical history of spondylosis and myelopathy, transient ischaemic attacks, prostate cancer and squamous cell carcinoma. The latter is of importance to the series of events that led to the life-saving surgical procedures. It is a story of disease, treatment, complications and heroic salvage.

CASE REPORT Five years ago Mr J developed a squamous cell carcinoma on the scalp, located on the top right side of the head, positioned slightly anteriorly. In April 2015 it was surgically removed, and he then received 3 sessions of adjuvant radiotherapy. The carcinoma was successfully excised and subsequently a skin flap was grafted onto the area.

Figure 1. A photograph of Mr J’s exposed bone after the series of unsuccessful tissue regeneration attempts.

Unfortunately, it was soon thereafter rejected and had to be removed. Despite trying several different options (including hyperbaric oxygen therapy) he did not have His condition progressively worsened for the following tissue regeneration and had an exposed cranium ever 2 weeks until he had to be taken to A&E in Telford again due to a rapid deterioration in his state. The diagnosis of since the excision (Figure 1).

TIA did no longer hold and it wasn’t until after an MRI With the exception of the failure to prompt adequate was conducted that the underlying cause of his conditissue regeneration in the area, there were no major in- tion was discovered. It was determined Mr J’s bone tiscidents regarding Mr J’s health for years, until June of sue had been compromised during radiotherapy, caus2019. Mr J was out shopping and quite acutely he felt ing osteoradionecrosis progressively over the years. his whole left side go numb. The paraesthesia seemed to affect his left upper and lower extremity and the left side 100

Midlands Medicine

This in turn had caused a bilateral subdural empyema the patient had to undergo a life-saving neurosurgical which required an immediate life-saving operation, so procedure. he was transferred to UHNM promptly.

Surgical procedure I – Neurosurgical intervention He has a past medical history of spondylosis and my- In July of 2019 the neurosurgical team at UHNM in elopathy, transient ischaemic attacks, prostate cancer charge of the operation performed a right mini-craniotand squamous cell carcinoma. The latter is of impor- omy, where a skin and bone flap were retracted to access tance to the series of events that led to the life-saving the meninges, and a left frontal burr hole, was drilled. surgical procedures. It is a story of disease, treatment, complications and heroic salvage.

DISCUSSION As an overview, Mr J was admitted as a joint patient requiring of neuro and plastic surgery. He had to undergo an initial procedure to relieve the pressure in his brain, and due to the severity of his condition (sepsis had settled in, worsening his symptoms) he had to recuperate for a number of months, to then proceed to the debridement of the dead tissue and the reconstructive surgery.

Osteoradionecrosis This is a known complication of radiotherapy in which the therapeutic ionising radiation causes vascular thrombosis leading to hypovascularity within the bone tissue. This in turn causes hypoxia and ischaemic changes and effectively incurs in tissue death or necrosis. Lamentably, this makes the area highly susceptible to opportunistic infections, and that is exactly what happened. Mr J’s necrotic tissue got infected, causing osteomyelitis and the infection became systemic, incurring in sepsis. More pressingly, the dead bone was the focus of the pathogenic entities and the main site of action of his immune system. The defensive reaction of his body produced copious amounts of a pus-like exudate that accumulated in the subdural space, hence leading to bilateral subdural empyema (Figure 2).

Figure 2. Generic MRI slice showing the enhanced and diffusion restriction (red circles) consistent with subdur al empyema (note this is not Mr J’s investigation).

The necrotic bone was left in place untouched. In the case of the craniotomy, the dura was opened in a cruciate fashion (cross-shaped), applying diathermy to border to stop bleeding2. Both abscesses were drained, samples taken to lab and the area was washed as best as it could be given the brain was close to the surface before closing up using special screws and plates.

When the samples came back two bacteria were identified: Methicillin-sensitive Staphylococcus Aureus and Vancomycin-resistant Enterococci. His antibiotic regiBilateral subdural empyema Empyema is the medical term for the accumulation men was therefore changed from ceftriaxone and metof pus, normally located in the pleural cavity (pyotho- ronidazole to flucloxacillin, keeping him on metronirax)1. In this instance, said accumulation occurred in dazole. the subdural space, between the dura mater and the arachnoid mater. The empyema was compressing the brain and increasing intracranial pressure. Therefore,

Volume 29, No 3, December 2020

101

Surgical procedure 2 – Joint Neurosurgical and Plastic intervention Once the more immediate danger had been resolved and the patient condition improved greatly after treatment with antibiotics, the next step was to tackle the original source of the infection, which was the necrotic bone. This required a joint procedure of neurosurgery and plastic surgery.

The neurosurgical component included the debridement of necrotic bone. The dura appeared intact underneath the dead bone, so it was kept untouched. The area was washed and ulcerated borders removed.

The plastic component of the surgery then consisted in the obtention of a planned free-style anterolateral perforator flap from the left thigh, which was done simultaneous to the debridement. The difference between a conventional and a perforator flap resides in origin of the vasculature of said flap. Conventional flaps are connected to more superficial plexuses, musculocutaneous and fascial, whilst perforator vessels come more distally from subdermal or subcutaneous plexuses.3 It is said to be “free-style” when it is harvested based purely on the pre-operative knowledge obtained from Doppler ultrasound.4 Initially and as mentioned before, the preferred location was the anterolateral aspect. The perforator vessel would normally come from the descending branch of the lateral circumflex femoral artery but in this case, it was coursing medially intramuscularly through the rectus femoris muscle (2% anatomical variation coming from the superficial femoral system). Mr Apostolou identified this variation via Doppler ultrasound.

It is then essential that when the flaps are reattached in their new location, they are properly perfused. Firstly, the right temporal artery and vein were located and exposed (Figure 3), and the flap was then grafted onto the area. The perforator vessels were anastomosed via vascular microsurgery by the plastic surgeon. The arteries were mismatched so the flap vessel with a diameter of around 1.2 mm was anastomosed end-to-side by hand suturing to the superficial temporal artery and the veins

102

were better matched, so a vein coupler was used.

Further management Mr J was kept in observation the days following the operation in a post-surgical ward and when his condition was deemed stable, he was transferred to another long-standing ward. His management consisted of prophylactic antibiotics and mobility exercises. And for some time in November the aim was to move him back to Shrewsbury.

Figure 3. From left to right we can see Mr J 6 days after the operation (note the incision that was made to find the temporal vessels), 14 days post-operation and 17 post-operation

Psychosocial considerations It is very easy to focus solely on the clinical and surgical side of a patient’s case and lose track of the psychological aspects (there were very few notes about Mr J’s psychiatric state). Therefore, I took it upon myself to visit Mr J before he was transferred. Upon taking his history, he could not remember the period around the drainage operation, yet he explained how his mobility and sensation in the left side was none prior the life-saving surgery. After months of physiotherapy following his last operation, he was content about the progress he was making.

His wife told me how he was a bit lethargic and low in mood, to the point that doctors were considering antidepressants. She also mentioned how that very morning he had been very emotional and depressed. However, I was there when one of the nurses came in with the good news that he would be transferred that same day, and I could see his face brightening up.

Midlands Medicine

Shortly after the physiotherapists arrived to help him with his daily routine and Mr J’s wife said she had not seen that agility in months. Clearly, the prospect to go back home had worked better than any antidepressants or mobility exercises.

ACKNOWLEDGEMENTS I would like to extend my sincerest gratitude to Mr Kostantinos Apostolou for his supervision as well as Dr Joshua Pepper. Thank you as well to the Keele Surgical Society. Most of all, thank you to Mr J for his collaboration and permission to use his case.

This illustrates the paramount importance of the patient’s state of mind and attitude, considerations that should never be overlooked as it can greatly impact ADDRESS FOR CORRESPONDENCE their health and recovery.

You can contact me via my official email I will add that I have kept in contact with his wife and w9x67@students.keele.ac.uk she told me that although his recovery path is long, he is improving every day, and fortunately he could spend REFERENCES Christmas in his home with his family.

1. Martin E A (2015) Oxford Concise Medical Dictionary p248 Summary Mr J is a 74-year-old Caucasian male from Shrewsbury with past medical history of squamous cell carcinoma. 2. Cumming J, Johnson C D (1997) Surgery for Head Injury Essential Surgical Technique p131 After its excision there were complications with tissue regeneration and it was later found that he had devel3. Kim J T, Kim S W (2015) Perforator Flap Versus oped osteo-radio-necrosis, incurring in bilateral subConventional Flap Journal of Korean Medical dural empyema. He required a life-saving procedure Sciences Vol 30 pp514-22 to decompress the brain and after a suitable period of recuperation he underwent reconstructive surgery. Ne- 4.

Wei F C, Mardini S (2004) Free-Style Flaps Plastic crotic bone was debrided, and a free-style flap was used and Reconstructive Surgery Vol 114 pp910-6 to repair the area. The series of operations were successful, and Mr J is on his path to recovery from the 5.

Figure 1 was obtained from Radiopaedia. Case comfort of his house. courtesy of Associate Professor Frank Gallard, CONCLUSION The case studied in this article is a rather rare one, with many complexities that arose due to an unfortunate series of events. Several factors, from iatrogenic consequences of the treatment of cancer to misdiagnosis contributed to a very severe condition. Had it not been promptly dealt with, the situation would have very rapidly led to death. Despite it being an isolated event, the learnings from this successful management of bilateral subdural empyema could result of vital importance for other cases. Lastly, it shows the paramount importance of timely and adequate surgical interventions, liaising amongst various specialties and coordinating them to ensure the best possible outcome for the patient.

Volume 29, No 3, December 2020

rID: 5125

IMAGES Full permission to use Mr J’s anonymised photographs for the purpose of the case presentation and this case report, only. Therefore, I kindly ask these not to be further reproduced under any circumstances.

103

INTERRUPTED AORTIC ARCH: A CASE STUDY Abbie L Randall, Medical Student, KUSoM INTRODUCTION Epidemiology Congenital heart disease is defined as “a gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance”1. Any structural abnormality that results in an arrhythmia such as in Wolff-Parkinson-White syndrome is excluded from this definition. The common definition also fails to factor in the presence of these abnormalities from birth. It is estimated that the incidence of congenital heart disease ranges from 3 to 12 per 1000 live births2. This estimate is increasing as mounting numbers of specialist paediatric cardiologists emerge among western societies, increasing the rate of diagnosis as well as antenatal screening, increasing the survival rates at birth. Asia reported the highest rates of congenital heart disease worldwide at 9.3 per 1000 live births. This included relatively more pulmonary outflow obstructions than left ventricular outflow tract obstructions (LVOTO)3. This was of interest to me because of all the patients I had seen, two of the most complex cases were of Asian heritage, one of whom will be discussed in detail later in this paper.

Of these congenital heart defects, interrupted aortic arch (IAA) makes up only 1% of cases4. It is defined as the “absence of luminal continuity between the ascending and descending aorta”5. This is differentiated from aortic coarctation which is defined as “stenosis in the region of the ligamentum arteriosum”6. It is also differentiated from aortic atresia which is grossly seen as a complete occlusion but there is the presence of a fibrous band which still connects the two segments of the aorta7.

cations which can ultimately lead to shock and death4.

However, this can be avoided if the child is screened before the ductus arteriosus closes and corrective surgery can take place to bypass the interruption, avoiding systemic ischaemia.

Anatomy IAA results in an acyanotic, obstructive defect that relies on a left-to-right shunt and a patent ductus arteriosus (PDA) to supply the systemic circulation. In normal anatomy, the aortic arch gives off three main branches, from proximal to distal they are the brachiocephalic trunk (which later divides into the right subclavian and right common carotid arteries), the left common carotid artery and the left subclavian artery. Type A defects occur after all three branches have been given off therefore the upper limbs are well perfused, type B defects occur between the left common carotid and subclavian artery leaving the left arm to be less well perfused than the right, type C defects occur between the brachiocephalic trunk and the left common carotid artery.8, Fig.1

Type A

Type B

Type C

Figure 1 – Types of interrupted aortic arch9

IAA is an important disease to recognise as it can often be confused for aortic coarctation, a less severe form of the defect, as well as having life-threatening compli

104

Midlands Medicine

Embryology During embryology, the aortic arch and associated great vessels arise from the six interconnected aortic arches, each associated with a pharyngeal arch. By day 29 of development, arches I, II and V have broken down, leaving III, IV and VI bilaterally. After this process, the right-sided connection between IV and VI also breaks down to leave arch IV as the main supply of blood to the primitive descending aorta. Branch VI becomes the primitive pulmonary arteries, with the left still connected to the descending aorta to form the ductus arteriosus. However, in the case of IAA, branch IV inappropriately breaks down, leaving only branch VI, the primitive pulmonary artery, to carry the blood into the descending aorta via the ductus arteriosus.10, Fig.2

Figure 2 - Embryological development of interrupted aortic9

Physiology Because blood is unable to flow into systemic circulation via the left ventricle, the right ventricle pressure must increase and therefore it hypertrophies in order to supply the peripheries. This will also result in an increased pulmonary resistance due to the increased flow via the pulmonary circulation.11 IAA is associated with many other congenital heart defects, usually a VSD and a PDA allow for the left to right shunt to deliver to the systemic circulation. Without these associated defects, IAA is likely to be incompatible with life and although some cases have been reported. However, it is difficult to distinguish in these cases if there is a true interruption of the aorta, or if there is an aortic coarctation12. Other common defects include

Volume 29, No 3, December 2020

bicuspid aortic valve (60%), mitral valve deformity (10%), persistent truncus arteriosus (10%), subaortic stenosis (20%) and Di George syndrome (15%)8. Other non-cardiac abnormalities are also seen in 55% of patients8. Most of these defects are connected due to their link to the conotruncal development of the heart during embryology. This posterior malalignment of the conal septum leads not only to a VSD but can also contribute to the left ventricular outflow tract obstruction, similar to the mechanism seen in right ventricular outflow tract obstruction seen in tetralogy of Fallot13.

CASE REPORT This case study focuses on a 3-year-old patient, MA. His history is somewhat unclear due to a language barrier with no interpreter available. MA’s mother was initially told in an antenatal screening that there may have been a cardiac defect present, however, upon his birth nothing was done about this and he was sent home with a clean bill of health. He was born at 39+2 weeks via a caesarean as the 5th child to both his parents. He was admitted to CAU at University Hospital of North Midlands (UHNM) at 5 days old due to a history of three days of poor feeding, including one day of increased work of breathing. He was initially prescribed oral antibiotics for a presumed respiratory tract infection and sent home. He was then brought back into hospital by his parents at 10 days old due to no resolution of his symptoms despite the completion of the course of antibiotics. It was then decided he should be admitted to the ward for observation and oxygen supplementation.

It was here that a registrar noted the lack of femoral pulses and the lactate had increased to 12. The on-call paediatric cardiology consultant was therefore called to confirm the diagnosis, initially thought to be coarctation.

The urgent echocardiogram showed a greatly dilated right atrium and right ventricle. There was an 8mm VSD present with a left to right shunt, severe tricuspid regurgitation of 5 m/s and mild mitral regurgitation.

During this echo, the aortic arch could not be fully appreciated because it could not be followed past the second aortic branch.

105

However, this is actually rarely present due to the high rates of coexistent VSD and level of right ventricular hypertrophy, allowing more oxygenated blood to be shunted into the pulmonary circulation and thus reaching similar saturations to the blood present in the ascending aorta14. Another theory behind this is that the level of heart failure or pulmonary oedema present in these patients by the point of diagnosis reduces their overall saturations in the oxygenated blood, reducing the difference between the two15. A murmur is also The patient was transferred to Birmingham Children’s rarely heard initially, as the VSD and PDA present are Hospital (BCH) for more specialised care and surgery. non-restrictive and therefore the presence of a murmur However, the corrective surgery had to be postponed in this situation is more likely to indicate a left ventricuuntil his liver and kidney function improved to with- lar outflow obstruction5. stand the procedure. The echocardiogram in Birmingham confirmed multiple defects including: Type A in- Link to coarctation terrupted aortic arch, bicuspid aortic valve, moderate Differentiating between Type A IAA and coarctation perimembranous VSD and a small apical muscular remains a challenge, with many cases not being conVSD – both of which showed bidirectional flow. firmed until autopsy when histology can show a complete lack of communication between the two segments Seven days later he underwent the primary anastomosis of the aorta16. The initial symptoms are very similar for his aortic repair using a pulmonary artery homo- to coarctation of the aorta, which is defined as a stenograft patch aortoplasty. He also underwent pulmonary sis in the region of the ligamentum arteriosum. It can trunk banding, a palliative measure until he was stable occur proximal or distal to the ductus arteriosus, but enough to undergo a full VSD repair. This was done the distal form is far more common10. Coarctation is with the hope that when this time came, the muscular a fairly common congenital cardiac defect, accounting VSD would have spontaneously closed, making for a for 8% of all cases6. The presentation is therefore likesimpler repair of only the perimembranous defect. ly to be similar to that of interrupted aortic arch, with impalpable femoral pulses not being palpable. However, in coarctation, these features will likely be present DISCUSSION from birth, whereas in IAA the symptoms will worsen and become life-threatening at day five of life, due to Presentation it being a ductus dependent lesion. As the majority of Patients presenting for the first time with IAA will likely coarctations occur after the duct, the closure will have be in respiratory distress, as seen in MA's case. They will no impact on the child, and many can live into adultalso likely be poorly perfused and have absent pulses in hood with very few symptoms10. The disease is also the femorals, (or the left arm if there is a Type B IAA). likely to be less severe due to there being some degree Differential cyanosis of the lower limbs is thought to be of flow through the aorta except in very severe cases. one of the defining clinical features of interrupted aortic arch; the upper limbs are well perfused by the supply Communication of oxygenated blood from the ascending aorta. The low- One of the main issues with this patient not being idener limbs, however, receive a mixture of deoxygenated tified as having a serious congenital heart defect in a blood from the right ventricle and shunted oxygenated timely manner, was the poor communication between blood from the left ventricle via the VSD. It is therefore the parents and the health care professionals due to the expected that some level of cyanosis of the lower limbs language barrier faced. occurs.

From this echocardiogram, a preliminary diagnosis of interrupted aortic arch was made, and the patient was transferred to the PICU to be started on prostaglandins to maintain systemic flow and inotropes to increase overall cardiac function. Initial tests completed on the PICU showed severe hypoglycaemia requiring multiple boluses of 10% dextrose solution. Preliminary LFTs also showed deranged clotting with an INR of 5.4 and APTT ratio of 4.7 suggestive of ischaemic insult to the liver.

106

Midlands Medicine

NHS England’s guidance for translators in primary care states that “Interpretation and translation should be provided free at the point of delivery, be of a high quality, accessible and responsive to a patient’s linguistic needs”17. However, there is little information on the guidelines for in-hospital care and this general principle was not adhered to. In many of his follow up consultations, another member of staff was used as an interpreter, however, the use of a formal interpreter should have been initiated earlier on. Had this been implemented, the clinicians would have been able to obtain a full and coherent history, and therefore their clinical judgment would not have been impaired by incomplete information which could have been provided in the presence of an interpreter, thus avoiding delays in his care.

Management The only definitive management for interrupted aortic arch is surgical repair18, however initial medical management is imperative. Complete resuscitation over several days before surgery is due to take place is required. The use of prostaglandin E1, introduced in the late 1970s13, is also an essential step in the management.

Administration of prostaglandins maintains the patency of the ductus arteriosus, a critical feature needed in many cyanotic congenital defects (often known as ductus dependent defects) including IAA as it ensures that the blood flow leaving the pulmonary arteries is able to flow into the distal aorta and supply the peripheries.

Hence, many babies who are not picked up during antenatal screening will start to go into multi-organ failure at day five when the duct begins to close, and their systemic circulation is shut off. Low-dose dopamine infusions and assisted ventilation are also used to help with the lower body perfusion19 and reduce levels of acidosis.

Dopamine is especially advantageous as it maximises renal perfusion, limiting the level of renal ischaemia.13 However, in order to maximise pulmonary resistance to increase systemic (and not pulmonary) blood flow, the level of inspired oxygen should be kept to room air despite the signs of clear respiratory distress. The over oxygenation induced by hyperventilation can also result in respiratory alkalosis, further reducing systemic oxygenation and worsening the patients state.13 Mainstay surgical management involves a one-step repair procedure, although more complex cases with Volume 29, No 3, December 2020

multiple defects often require multistage repair19. The three main techniques used in the aortic arch repair include: direct anastomosis, patch augmentation and conduit interposition19. The one stage primary neonatal repair often involves direct aortic arch anastomosis and VSD closure via a median sternotomy. During this procedure, the neonate must be put into hypothermic circulatory arrest below 18°C. The PDA is then ligated to prevent further mixing of oxygenated and deoxygenated blood. If the VSD closure cannot be completed during the initial procedure due to the complexity of the defect, then a pulmonary artery band is considered to reduce the risk of further pulmonary hypertension.18 Although a single-stage biventricular repair has proven to be the preferred technique for repair of aortic arch defects and associated intracardiac defects, patient MA has undergone a multi-stage repair approach. It has been shown that premature neonates weighing less than 1500 g and those with multiple organ failure or very severe defects may benefit from a multistage repair. Less complex patients who are more stable would therefore benefit from a single staged approach due to the need for fewer reinterventions, avoiding pulmonary artery banding and reduced risk of requiring later arch reconstruction.20 Complications The initial complications of interrupted aortic arch include heart failure and multi-organ failure within the first few days of life. This occurs because as the PDA closes, oxygenated blood can no longer access the systemic circulation, leading to deoxygenated blood as the only supply to systemic circulation below the arms. This can quickly progress into shock and death.

Following the surgery, MA suffered from delayed speech due to left vocal cord palsy. During surgery, there is a great risk of damaging the recurrent laryngeal nerve because it loops underneath the arch of the aorta.

Damage to this nerve is the most common complication after this type of surgery, with over half of all aortic arch repairs suffering damage to the recurrent laryngeal nerve. It can result in vocal cord palsy and a hoarse voice.21 This spontaneously improved in MA but required monitoring from ENT and SALT teams.

107

A common late-stage complication of the disease is a left ventricular outflow tract obstruction (LVOTO).

This can occur through many mechanisms such as a Moulaert, wherein an associated prominent muscle bundle in the free wall of the left ventricle projects into the outflow tract. The aortic annulus itself can be hypoplastic in some cases and lead to a congenital outflow tract obstruction. The main cause of outflow tract obstruction after surgery is the formation of a fibrous subaortic membrane, usually seen one to two years after the initial repair has taken place.13 Associated conditions MA underwent full genetic assay screening due to IAA’s association with other genetic syndromes including DiGeorge syndrome. DiGeorge syndrome is a disorder caused by the deletion of part of chromosome 22 at q11.2 resulting in 22q11.2 hemizygosity.22 Some of the characteristic features of DiGeorge syndrome include complex cardiac defects, cleft palate, immunodeficiency, developmental delays (including slowed growth and speech development), ADHD, hyperthyroidism and rheumatoid arthritis.23 This gene mutation results in aplasia or hypoplasia of the thymus and parathyroid glands, conotruncal cardiac defects and minor facial dysmorphism. This is due to the mutation causing a defect within the cephalic neural crest cells which would go on to form the cardiac outflow septum, as well as the third and fourth branchial arches which give rise to the thymus, parathyroid glands, aorta and vessels of the head and neck. It has been shown that there is an association between Type B interrupted aortic arch in patients with 22q11.2 hemizygosity22, with up to 82% of patients with Type B lesions having the 22q11.2 microdeletion.6 It is therefore assumed that Type B interrupted aortic arch is more likely to be greatly associated with neural crest cell defects than the other two types and therefore the pathogenesis of Type B is distinctly different from types A and C24, consequently demonstrating why no genetic link could be found for MA’s Type A defect.

108

CONCLUSION Interrupted aortic arch is a rare but life-threatening congenital heart defect which can be easily missed in the early days of life as most cases are asymptomatic until the ductus arteriosus begins to close and the systemic circulation is shut down by this mechanism. It presents very similarly to coarctation of the aorta and it can sometimes be difficult to distinguish between the two.

Further research could go into creating defining criteria between the two to enable easier diagnosis and management pathways to be put into place. There are three main types of IAA, which have two different mechanisms of formation, with type B having a much greater genetic link and is often associated with DiGeorge syndrome and is therefore much more common than the other types. The definitive management for IAA is surgical correction, which has proven to show better outcomes when completed in a single procedure than in multiple. However, it is acknowledged that some patients have much more complex defects than others, making it impractical to complete all corrections in one procedure. It is key that during initial clerking of patients, a full and detailed history must be taken, and it is therefore imperative in patients wherein English is not their first language to employ an interpreter to see the full clinical picture. Due to the nature of this defect, it is also imperative that all young patients presenting with respiratory symptoms should undergo a full cardiovascular exam, including accurate palpation of femoral pulses in order to avoid cases like this one whereby the defect was not picked up until the neonate reached life-threatening organ failure.

Midlands Medicine

hypoplasia, and arch interruption: strategies for evaluation of the aortic arch Cardiology in the Young (2016) Vol 26 pp1553-62

ACKNOWLEDGMENTS

I would like to thank Dr P Ramesh for his support on this project. I would also like to thank Keele Surgical Society for giving me the opportunity to present this 8. Horrox F and Heaton G Manual of neonatal and paediatric heart disease London: Whurr (2004) case at their case presentation evening.

ADDRESS FOR CORRESPONDENCE W7f02@students.keele.ac.uk REFERENCES 1. Mitchell S, Korones S and Berendes H Congenital Heart Disease in 56,109 Births Incidence and Natural History Circulation (1971) Vol 43 pp323-332 2. Hoffman J Incidence of congenital heart disease: I Postnatal incidence Pediatric Cardiology (1995) Vol 16 pp103-113

9. Randall A Interrupted Aortic Arch Diagrams 2019 10. Sadler T and Langman J Langman's medical embryology 14th edition Wolters Kluwer 11. Principles of cardiac diagnosis and treatment — a surgeon's guide 2nd ed British Journal of Surgery (1992) Vol 79 p1112 12.

Akdemir R, Ozhan H, Erbilen E, Yazici M, Gündüz H and Uyan C Isolated Interrupted Aortic Arch: A Case Report and Review of the Literature The International Journal of Cardiovascular Imaging formerly The International Journal of Cardiac Imaging (2004) Vol 20 pp389-92

3. van der Linde D, Konings E, Slager M, Witsenburg M, Helbing W, Takkenberg J et al Birth Prevalence of Congenital Heart Disease Jonas R Management of Interrupted Aortic Worldwide Journal of the American College of 13.

Arch Seminars in Thoracic and Cardiovascular Cardiology (2011) Vol 58 pp2241-2247 Surgery (2015) Vol 27 pp177-88 4. MD R Interrupted Aortic Arch - PubMed NCBI [Internet] Ncbi nlm nih gov 2019 [cited 14. Roberts W, Morrow A and Braunwald E Complete Interruption of the Aortic Arch Circula30 May 2019] Available from: https://www ncbi tion (1962) Vol 26 pp39-59 nlm nih gov/pubmed/30422497 5. Sandhu SK and Pettitt T Interrupted Aortic Arch [Internet] 2002 [cited 30 May 2019] Available from: https://link springer com/content/pdf/10 1007/s11936-002-0013-5 pdf

15.

Immagoulou A, Anderson R and Moller J Interruption of the Aortic Arch: Clinical Features in 20 Patients Chest (1972) Vol 61 pp27682

6. Vriend J, Lam J and Mulder B Complete Aor- 16. Vaideeswar P, Marathe S, Singaravel S and Anderson R Discontinuity of the arch beyond the tic Arch Obstruction: Interruption or Aortic origin of the left subclavian artery in an adult: Coarctation? The International Journal of CarInterruption or coarctation? Annals of Pediatric diovascular Imaging formerly The InternationCardiology (2018) Vol 11 p92 al Journal of Cardiac Imaging (2004) Vol 20 pp393-396 7. Goudar S, Shah S and Shirali G Echocardiography of coarctation of the aorta, aortic arch Volume 29, No 3, December 2020

109

17.

18.

19.

20.

ngland N [Internet] Englandnhsuk 2019 21.

E [cited 30 May 2019] Available from: https:// wwwenglandnhsuk/wp-content/uploads/2018/09/guidance-for-commissioners-interpreting-and-translation-services-in-primary-carepdf 22.

Schreiber C The interrupted aortic arch: an overview after 20 years of surgical treatment European Journal of Cardio-Thoracic Surgery (1997) Vol 12 pp466-70

ham V, Connelly D, Wei J, Sykes K and P O’Brien J Vocal Cord Paralysis and Dysphagia after Aortic Arch Reconstruction and Norwood Procedure Otolaryngology–Head and Neck Surgery (2014) Vol 150 pp827-33

S erraf A, Lacour-Gayet F, Robotin M, Bruniaux 23.

J, Sousa-Uva M, Roussin R et al Repair of interrupted aortic arch: A ten-year experience The Journal of Thoracic and Cardiovascular 24.

Surgery (1996) Vol 112 pp1150-60

h ttps://rarediseases.info.nih.gov/diseases/10299/22q112-deletion-syndrome

ishra P Management strategies for interM rupted aortic arch with associated anomalies European Journal of Cardio-Thoracic Surgery (2009) Vol 35 pp569-76

auch A, Hofbeck M, Leipold G, Klinge J, R Trautmann U, Kirsch M et al Incidence and significance of 22q112 hemizygosity in patients with interrupted aortic arch American Journal of Medical Genetics (1998) Vol 78 pp322-31

Van Mierop L and Kutsche L Interruption of the aortic arch and coarctation of the aorta: Pathogenetic relations The American Journal of Cardiology (1984) Vol 54 pp829-34

As no two faces, so no two cases are alike in all respects, and unfortunately it is not only the disease itself which is so varied, but the subjects themselves have peculiarities which modify its action.

William Osler 110

Midlands Medicine

MORE MEDICAL CERAMICS Anthea Bond, retired Consultant Orthodontist

STORAGE JAR FOR WET MEDICINES In the Ceramics Department of the Potteries Museum and Art Gallery is this fine example of a storage jar.

It is made of Italian Majolica, the same tin-glazed earthenware is known as Delftware when made in the Netherlands. In London, this pottery is known as Lambeth ware, made by the pottery makers who migrated from Antwerp to London in the 16th Century and continued their own pottery making techniques.

These busy ports allowed the import of suitable clay and the distribution of the items made.

This pot was probably made in London in the mid1700s, earlier jars had handles. The bulbous shape was used for oils and syrups, wet medicines. It has an everted lip which could have a parchment or leather cover tied in place. The spout is placed high on the body, to allow the top of the liquid to be poured, leaving Tin-glazed earthenware was not made here in the unwanted residue. Opposite the spout is the decoration Potteries at that time. It was made in other places in with the name of the contents, “S: ZINZIB” for Syrup Britain, first in Norwich from about 1550 then from of Ginger.

1600 in London, Bristol and Liverpool. The potteries in London were along the banks of the Thames and some of them lasted until the middle of the 19th century.

Volume 29, No 3, December 2020

111

NEWS The North Staffordshire Institute has been hit hard by the Covid-19 pandemic in terms of the significant restrictions to its usual functioning that the lockdown has imposed, and the reduction to its income as a conference centre.

Nevertheless, its central purposes of supporting local medical research initiatives and continue: The research awards recipients in 2020 are as follows: The development of a tissue engineered pleural patch for repair of alveolar air leaks following surgical lung resection Dr Tina Patricia Dale, Professor Ying Yang and Prof Nicholas Robert Forsyth: £18,250 Trends, Incidence and predictors of post discharge complications, readmissions after acute myocardial infarction and their association with long term clinical outcomes Dr Muhammad Rashid: £15,220 Electrical Vagal StimulatION in Obesity – a proof of concept study – EVASION- Obesity Dr Adam Farmer, Mr Chandra Cheruvu, Prof Kirsteen Browning: £19,995 Does coloured crockery make a difference to the nutrition and hydration of older people receiving care in an acute hospital – a pilot/feasibility study?

Jo Lancaster, Prof Wilfred McSherry and Dr Simon Lea: £13,598 Testosterone Replacement Therapy: Identifying which patients with adult onset testosterone deficiency will benefit from treatment Professor S Ramachandran: £10,631

The AGM took place on 12th November virtually using Zoom. Minutes will be circulated in due course.

Many thanks go to Chris Bolger, loyal servant of Midland Medicine in the role of Assistant Editor for many years. The Covid-19 pandemic has given many, including him, pause to think and reflect. He has come to the conclusion in these uncertain times that the moment has come for him to stand down from the post he has held these many years. His support, loyalty and contributions will be much missed, and I sincerely wish him well as he moves on.

As one door closes, another opens: if there are any among you who feel they have a contribution to make, either on the editorial board, or in writing, it would be delightful to hear from you.

Many thanks and best wishes also go to another departee, John Kocierz, our highly esteemed Honorary Secretary at NSMI, who has been much appreciated for his work for the Institute. Again, these strange times have contributed to the timing. His important work and wisdom will be much missed.

112

Midlands Medicine

RESEARCH NOTES ON THE TIME OF COVID Dominic de Takats, Editor, Midlands Medicine sos MORI.” This study relies on capturing snapshots of prevalence as a surrogate for incidence in a larger deThe clinical research community were hit very hard by fined population. the Covid-19 global pandemic and almost all established clinical trials came to an abrupt halt as the pan- Taking advantage of the frustration felt about not bedemic spread globally. But those involved in clinical ing able to do anything to help, huge public awareness research are some of the brightest, most quick-witted and technology a different approach is coming out from and flexible there are. The very thing that brought a halt elsewhere in London. At King’s College, Professor Tim to what was already being done quickly became, with Spector has developed a symptom tracker app which some deft adaptation, the new object of study. And the first identified anosmia and loss of taste as key identifier breadth and depth of public concern, the notion that symptoms. The app has been made generally available rule books had been torn up and the slight tendency to and anyone interested in participating can join so long by governments to need to be seen to be doing some- as they have a suitable smartphone. This is an example thing meant that great opportunities abounded for of science outreach public science, related to, but distinct from citizen science.3 those sufficiently fleet of foot.

INTRODUCTION

I am pleased to say that the NHS came up trumps (and in the process may have saved Trump).

The three groups of researchers to quickly get their act together were epidemiologists, clinicians together with clinical trialists and vaccinologists, looking respectively at understanding the transmission of the virus, treatment of Covid-19 and its prevention.

SURVEILLANCE The REACT (Real-time Assessment of Community Transmission ) study is not to be confused with the REACT (Rescue angioplasty versus conservative treatment or repeat thrombolysis trial)1 nor with the rather similar in area and name ReACT (Randomized Evaluation of Routine Follow-up Coronary Angiography After Percutaneous Coronary Intervention Trial).2 This, after all, is an observational study, not a trial. “Imperial College London is leading a major programme of home testing for COVID-19 to track the progress of the infection across England. Called REACT, the programme was commissioned by the Department of Health and Social Care, and is being carried out in partnership with Imperial College Healthcare NHS Trust and IpVolume 29, No 3, December 2020

THERAPY In the modern world there are those who want to see results much sooner than has traditionally been the case in standard prospective, randomised placebo-controlled clinical trials, which can take years to recruit, conduct, follow-up and then analyse present and explain the results. They are not only time-consuming but, not unrelatedly, very expensive to conduct. One problem about this tried and tested but slow and laborious approach is that changes in knowledge or technology might render the results obsolete or redundant before they are fully fledged. In the last decade the US Food and Drugs Administration suggested that there was a way to gain knowledge through the clinical trials process more quickly than by sticking rigidly to pre-specified protocols. They conceptualised adaptive trials in which knowledge gained from results early in the trial could be used to focus attention differently later in the trial, changing treatment approaches, outcome measures or recruitment to answer new questions as they were thrown up by the trial results as they came in.4,5 As new knowledge emerges, the hypotheses being tested evolve so that knowledge established by 113

the research becomes rapidly either 'standard of care' or interrogated further to refine under what conditions/at what stage of the disease/in which patient subgroup the treatment works best.

cy, that is best tested where the incidence of acquisition is high, which explains why Oxford-based scientists are doing their work in Brazil. We all await the results with interest.

A further revolutionary step away from the rigid linear single investigational medical product placebo-controlled clinical trial is the platform trial. Philosophically, the approach is radically different in that the disease is at the centre of consideration rather than the therapy.

A number of candidate treatments may be considered simultaneously. Those treatments that seem effective are kept on in the trial (‘on the platform’) whilst those that seem ineffective are abandoned to make way for new candidate treatments. Those treatments that have their role established are then given to all suitable subjects whilst only newer uncertain treatments are randomised. A platform trial is a particularly extreme form of an adaptive clinical trial.

TESTING

Where there’s research, you’re sure to find innovation.

One very interesting way to speed, up testing, as transformational in approach as adaptive clinical trials, is stepping away from testing individuals as individuals and treating them as identifiable elements in a batch, using a technique called pooled testing. This is a particularly effective technique when prevalence in a population is low because when a batch made from several individuals’ sera tests negative, they all receive a negative test result on the basis of the single negative test, saving time and money. If a batch tests positive, there is a choice between then testing all the contributing individuals, or working through a protocol much like one One of the key limiting factors in getting clinical trials which allows you to find the heavy ball in a puzzle using results is the event rate. During a global pandemic of a balance. A well-worked pooled testing approach has a new disease, the event rate is very high, meaning that the potential to considerably speed up and to reduce the clinical trials should be able to identify effective treat- costs of testing for SARS-COV2. ments much more quickly than in ordinary times. The RECovery trial (randomised evaluation of Covid-19 REFERENCES therapy)6 was put together very quickly by the Nuffield Department of Population Health based at the Univer- 1. Gershlick AH, Stephens-Lloyd A, Hughes S et al Rescue angioplasty after failed thrombolytic sity of Oxford taking advantage of the large numbers therapy for acute myocardial infarction N Engl of patients presenting with the same disease at about J Med (2005) Vol 353 pp2758-68 the same time. UHNM contributed a large number of patients to the RECovery trial. It is a platform trial and Shiomi H, Morimoto T, Kitaguchi S, Nakagawa has fulfilled the promise of quickly reaching conclu- 2.

Y, Ishii K et al The ReACT Trial: Randomized sions on purported treatments: the lack of effect of hy7 8 Evaluation of Routine Follow-up Coronary Androxychloroquine and the efficacy of dexamethasone. giography After Percutaneous Coronary Intervention Trial J Am Coll Cardiol Cardiovasc PREVENTION Interv (2016) Vol 10 pp 109-17 Vaccination is like therapy but it isn’t therapy it is a https://en.wikipedia.org/wiki/Public_science means to obviate the need for therapy. More like fire- 3. proofing by using non-combustible materials than like https://www.fda.gov/regulatory-information/ incorporating a sprinkler system. Like medicines as 4. search-fda-guidance-documents/adaptive-detreatments, vaccines must undergo trials to establish sign-clinical-trials-drugs-and-biologics-guidsafety and efficacy. Safety is always at issue with someance-industry thing that is actually designed to cause an immune response, in case there’s a prospect of the wrong sort of immune response: allergy or anaphylaxis. As for effica114

Midlands Medicine

5.

https://peerj.com/preprints/1825v1.pdf

6.

www.recoverytrial.net

7.

www.recoverytrial.net/results/hydroxychloroquine-results

8.

www.recoverytrial.net/results/dexamethasone-results

Picture courtesy of the Wellcome Collections, used under a creative commons licence

Volume 29, No 3, December 2020

115

QUIZ NIGHT Oluseyi Ogunmekan, General Practitioner, Furlong Medical Centre, Stoke-on-Trent

1. Which of the following conditions is investigated using the mixed meal test?

6. Which of the following statements is true Miss Tracy's Mixture?

a) coeliac disease b) delayed gastric emptying c) food intolerances d) reactive hypoglycaemia e) small bowel bacteria overgrowth syndrome

a) it can be helpful for mucositis caused by chemotherapy b) it contains lidocaine gel 1% c) it contains 50,000 units of nystatin d) it is used in the symptomatic management of sore throats in children especially e) all of the above

2. Which of the following statements regarding empty nose syndrome is true? a) it is a form of secondary atrophic rhinitis b) it may follow a minor nasal surgical procedure c) it may follow a major nasal surgical procedure d) there are no objective physical examination findings e) all of the above 3. The Roth score is a reliable test for diagnosing Covid-19 infection: True or False?

4. Radiology: Which organ is the Bosniak scoring system is used to assess? a) brain b) heart c) kidney d) liver e) thyroid

7. Percentage carbohydrate deficient transferrin (CDT) may be used in the evaluation of which of the following conditions? a) iron deficiency anaemia b) diabetes mellitus c) alcoholism d) galactosaemia e) leukaemia 8. Which of the following is true about HBV serology? a) after immunisation the presence of a high Hepatitis B surface antibody titre indicates immunity b) after infection the presence of Hepatitis B surface antibody indicates recovery c) Hepatitis B core antibody appears at the onset of symptoms in acute Hepatitis B and persists for life d) HbSAg is present in early infection, disappears with resolution and persists in chronic infection e) all of the above

5. The Sniff Nasal Inspiratory Pressure (SNIP) may be used in assessments of patients with Amyotrophic Lateral Sclerosis: True or False?

116

Midlands Medicine

9. Which of the following statements is true about hyponatraemia?

11. Which of the following is a recognised cause of a raised serum amylase?

a) a low plasma osmolality and increased urinary sodium suggests siadh b) can occur in patients with severe burns c) can be caused by SSRIs d) if the plasma osmolality is normal, it is termed pseudohyponatraemia e) if associated with elevated plasma osmolality may suggest hyperglycaemia

a) acute pancreatitis b) alcoholism c) parotitis d) perforated peptic ulcer e) peritonitis

10. Which of the following DOACs must be kept in the original packaging and only opened when needed?

a) cirrhosis b) heart failure c) sepsis d) pulmonary embolism e) pioglitazone

a) apixaban b) dabigatran c) edoxaban d) rivaroxaban

12. Which of the following is a recognised cause of elevated N terminal pro-BNP?

CALL FOR CONTRIBUTIONS Midlands Medicine aims to fulfil a wide brief spanning from the role of a traditional medical journal to a forum for the dissemination of news and developments in local healthcare in the Midlands. We seek to reach all interested parties from patients to practitioners, from medical students to nurse Midlands Medicine’s coverage ranges from the history of medicine to ground-breaking research, from audit to audacity. Additionally, we aim to be an educational resource for students and practitioners in all healthcare disciplines.

There must be many of you out there with something to contribute for the interest of the wider healthcare, medical and surgical community. Projects or audits which were particularly informative, books you’ve read that have changed the way you look at things or matters you would like to sound off about.

If so, get writing. This journal can only thrive on your contributions. Whether you’re not sure where to start or just wish to polish up your article prior to submission, please request the ‘Guidance for Authors’. If you are thinking about writing an article but wish to discuss it first, please contact the office.

All articles are peer reviewed and subject to editorial approval and to editing

Volume 29, No 3, December 2020

117

WORDPLAY IN ALL OUR HOUSES Dominic de Takats, Consultant Nephrologist, UHNM "A plague o' both your houses," Mercutio in Romeo and Juliet by William Shakespeare PRE-FORMED LANGUAGE

Virology

As distinct from performed language that Shakespeare was most familiar with. Shakespeare was also familiar the plague in the 16th Century. This twentieth outing of Wordplay notes that there is a danger of a plague in all our houses. The plague refers, fairly specifically, to the bubonic plague, also known as the Black Death, an infectious disease caused by the bacterium Yersinia pestis. The principal vector is fleas carried by rats. A plague is more vague, and can refer to any of a number of contagious diseases, even an unknown one.

So let’s get a few things straight. SARS CoV2 (the second severe acute respiratory syndrome-causing coronavirus with the first, SARS-CoV1, being the cause of SARS first noted in Asia in 2003), is a coronavirus which causes a disease called CoViD-19 (coronavirus disease first emerging in 2019) whose original name was Wuhan novel coronavirus because it was a new disease that emerged in Wuhan in China in the autumn of 2019. So, when we say we are testing people for Covid-19, we are usually wide of the mark, because we are actually testing them for the presence of SARSThe current plague had to start somewhere. At first CoV2. Only if they have a disease associated with there was a respiratory plague in Wuhan in China in SARS-CoV2 do they have Covid-19. That ‘d’ at the end the second half of 2019. That was an epidemic (Greek: of Covid used to be a capital ‘D’ and stood for disease. epi [about or upon] + demos [people or populace]). So, the phrase asymptomatic Covid-19 is an oxymoron Then Covid-19 became endemic (Greek: en [within] + and we should really say someone has asymptomatic demos) within China. It came to the attention of the SARS-CoV2 infection/carriage. But I suspect that’s not WHO who spent weeks arguing whether or not it was a going to catch on and that in terms of that particular pandemic (Greek: pan [ubiquitous] + demos). It soon usage the equine vector has long since ‘scaped its stalls. was. Sometimes semantics are good, but then again …

Vital Coronaviruses are a family of enveloped, positive We’ve gone to town on the concept of all things key. (sense), single-stranded RNA viruses that infect certain The notion of something very important on which vertebrate groups. Transmission electron micrography we depend is long established. In arch-building the shows them to have a spherical capsule from which keystone which is centrally placed, literally, different spike proteins project. The first pictures were redolent (wedge-shaped and not flat) and larger, completes and to the scientists working on these viruses of the sun’s supports the arch. The keystone is the principal stone corona or crown, particularly as seen during an eclipse, in the arch, holding it all together. This sense is, I am sure, the one in which the term key worker has come and so they were named coronaviruses.1 to be used, meaning some very important, key, role on which society depends. Other roles of keys are to unlock LANGUAGE FOR OUR TIME things such as doors, literally, and the information on maps and charts. Unlike a keystone, key workers Come our first 21st Century plague and cue key new do not need to be wedged in at the last to take up a language to help us cope. The Covid-19 pandemic centrally important role - that’s what a politician might caused by the coronavirus SARS-CoV2 has given rise do. (BTW, are politicians key workers?) Let’s take the to new words, terms and phrases which, to use a rather next, key, step. dated term, have gone viral.

118

Midlands Medicine

The key idea, the key principle, behind key worker status, is to identify those who are allowed out during a lockdown to travel to work, because they are key workers who help to keep society functioning even when the majority of people are stuck at home not working. Of course, some key workers need not invoke their key worker status because they can do their key work at home if it mainly involves keystrokes on a keyboard (seashells on the sea shore, cadence coincidence?).

Vierzig tagen When is quarantine not quarantine? When it’s selfisolation. This enforced staying at home to prevent the spread of infectious disease to others used to be quarantine and not lockdown or self-isolation.

Lockdown used to be what you did in a school or hospital setting when an armed and violent person went on the rampage, not something mandated from Whitehall for a whole nation. Self-isolation used to run a spectrum from expected British social reserve, minding one’s own business bearing a stiff upper lip, to the amateur misanthrope and the professional exponent of hermitry as practised by a solo monk or ascetic. Now it means something that the government might ask you to do alone, or in a household or in a ‘support bubble’.*2

LANGUAGE FOR THEIR TIME Have you ever pondered the different terms used for temporary workers in different fields? Short term labourers in field might be called casual workers; doctors do locum work (Latin meaning ‘in place of ’); secretaries are “temp’s” (contraction of ‘temporary secretaries’); nurses are ‘bank nurses’ having been drawn from the nursing bank. Teachers on a stopgap basis are supply teachers, having been supplied by an agency or having supplied themselves, usually at short notice. Some work is by its very nature a series of distinct episodes.

Acting has largely always been so. In journalism selfemployment, can be even more speculative. Perhaps freelance reflects the fact that they are free much of the time. Uber drivers are the quintessential epitome of those working zero-hours contracts, a sort of semipermanent temporariness of shift work in which availability at short notice is expected but no gainful employment is guaranteed. These shifts are referred to by the same term used for musicians’ few hours of work of an evening: gig.

(UN)INTENDED

Puns may be intended, for fun or to add a certain The only reason that occurs to me for not adopting the levity to an otherwise serious situation, a deliberate term quarantine to describe the quarantine practices attempt to lighten the mood, leaven the stultifying or urged and mandated in the UK during this pandemic is season the dish, but sometimes the wordplay is entirely because of a possible association with the quarantining unintentional, forced into being by rigid formulations, of dogs that used to be operative before pet passports, dropdown lists sitting in predetermined structures. which may have left something of a bad smell around the The juxtapositions may jar or pun cringemakingly, or word. But it is a word with great history and completely amusingly. Understatement can afford a similar effect. apt to current circumstances. It is said to derive from Ponder the following which are genuine instances: the Venetian practice of denying disembarkation to sailors on inbound ships from afar for forty days (from “Heading the ball has an impact on the risk of dementia” the Venetian quarantena, a contraction from the Italian Geoff Hurst quaranta giorni), a biblically-inspired duration, during times of plague.

* A support bubble is a close support network between a household with only one adult in the home (known as a single-adult household) and one other household of any size. This is called making a ‘support bubble’. Once you’re in a support bubble, you can think of yourself as being in a single household with people from the other household. It means you can have close contact with that household as if they were members of your own household. Once you make a support bubble, you should not change who is in your bubble

Volume 29, No 3, December 2020

119

What is the scope of this endoscopy guidance?

REFERENCES

The outcome of the event ‘sudden death’ was fatal

1. Almeida JD, Berry DM, Cunningham CH, Hamre D, Hofstad MS et al

Virology: Coronaviruses Nature (1968)

Vol 220 p650 Bibcode:1968 Natur.220 650 doi:10.1038/220650b0

The event of ‘sudden death’ was assessed as serious “The subject was observed by the family members 6 hours before death and appeared ‘a bit pale.’”

Headway charity: Improving life after brain injury3 “The progress of HS2 remains on track” “Our flood helpline has been inundated with calls” “Does my mum loom big in this?”4 Pregnant then screwed*

2. www.gov.uk/guidance/making-a-supportbubble-with-another-household 3. www.headway.org.uk 4.

A touring one woman show by Arabella Weir

5. https://pregnantthenscrewed.com/

Good Grief bereavement counselling “An eye-popping display of field poppies” Car computer: “Stop immediately! Your braking system has failed.”

Diplomacy: The art of letting somebody else have your way David Frost

*Pregnant then screwed5 is a charity working to combat financial disadvantage engendered by pregnancy and motherhood.

120

Midlands Medicine

INTERESTING IMAGE

Purple Urine Bag Syndrome is well described in the literature.1 This particularly striking example is from a male patient with a long-term indwelling urinary catheter in hospital with an unrelated condition. Patients may have a clinical urinary infection or may merely be colonised with bacteria which act of tryptophan metabolites in the urine to produce indoxyl. In strongly alkaline urine, indoxyl is converted to indigo (blue colour) on the plastic surface and indirubin (red colour), which is dissolved in the plastic, and together cause the typical purple colouration.2 REFERENCES 1 https://gpnotebook.com/simplepage.cfm?ID=x20190511225343097477 2 Liolios

AC, Woess E and Lhotta K Purple urine bag syndrome (PUBS) NDT Plus (2008) Volume 1 pp365-6 https://doi.org/10.1093/ndtplus/sfn057 Volume 29, No 3, December 2020

121

BOOK REVIEW: WAR DOCTOR BY DAVID NOTT Graham Sansom, Retired Biology Teacher and Missionary

This book, full title, War Doctor - surgery on the front line, was so enthusiastically recommended to me that it became a ‘must-read’. I read it. The enthusiasm is contagious and I commend it to you.

My wonder at the working of the human body and at the advances in medical science has been deepened, while my admiration for medical practitioners and their skill has gone into orbit. However, there might be a challenge in this respect for any readers who are of a I had the time available to read in three sittings over squeamish disposition. Having expressed that concern, three days and eagerly returned on each occasion. I re- I must add that there is no sense in which David writes read it a few weeks later for the purposes of writing this with any hint of sensationalism. Sensitivity, deep review and again found it to be compelling, inspiring, personal emotion and a total commitment to save and disturbing and challenging. As someone who is serious enhance life permeates his whole being as well as every about remaining informed of world news, disasters, written word. crises, conflicts and the plights of those affected, I was shamed into recognising that my comprehension, Out of that awareness and sensitivity to others, compassion and response to the depth of suffering the accounts of his work in various situations is and need were, in fact painfully shallow. So, the interspersed with stories of his family, of his pursuit recommendation comes with a warning: this book will of interest in flying and gaining his pilot’s licence, of unforgettable drives through London in police cars, of demand a response and condemn indifference. startling discoveries in the latrines, of his journey “on David Notts writes candidly of his motivation, the conveyor belt of becoming a consultant surgeon” experiences, feelings, personal journey, failures and and then, through highs and low, of his development successes over more than two decades of volunteering into a highly respected colleague. There is humour and his services as a vascular neurosurgeon, in over twelve warmth, joy and wonder, appreciation of life, life-long countries, in “areas of pure misery and heartache … to forging of friendships and even some spiritual moments help people who, like you and I, have a right to proper as he . though “not religious”, finds strength in prayer care at this most precarious time of their lives.” He tells and solace in being blessed by a Priest in Aleppo. With us stories, true stories; briefly of his family background, disarming frankness, he tells us of his failings, struggles, the conveyor belt of becoming a consultant surgeon his adrenalin highs, his assertiveness and fixations, and of his growing conviction that he should go beyond the need to learn sensitive ways to earn the trust and the safety of London to help those people caught up in cooperation of colleagues. natural disasters but, more often, in man-made conflicts.

He takes us with him to Sarajevo, Afghanistan, Iraq, He honestly shares the cumulative cost of seeing Sierra Leone and Gaza, amongst other places, while unspeakably dreadful things that will stay with him for the rest of his life, of being in danger of his life on many majoring on three periods of relief work in Syria. occasions, notably , from ISIS fighters, of the terrible Helpfully, succinctly and with commendable clarity, consequences for children in war zones, and much David describes the background and context of each more. Exhausted and brutalised by all he had seen and conflict situation he enters. With equal skill he enables experiences, he entered “a trough of psychosis and us to visualise the injuries with which he is faced, paranoia” which professional help, love, and personal their effect upon the body and threat to life itself, the courage and strength pulled him through. challenges presented to the surgeon and the surgical techniques which are applied. As someone with a Once again, I commend this book to you. background in the biological sciences I found this enlightening and, somewhat unexpectedly gripping.

122 Midlands Medicine

ANSWERS AND EXPLANATIONS QUIZ NIGHT https://www.cebm.net/covid-19/roth-score-notrecommended-to-assess-breathlessness-over-the1. d) Reactive hypoglycaemia Reactive hypoglycaemia is a disturbance in glucose/ phone/ carbohydrate metabolism which has features opposite to impaired glucose tolerance in that relatively too much 4. c) Kidney insulin is secreted following a meal and blood sugars are The Bosniak grading system classifies renal cystic masses driven low enough to cause symptoms. A standardised into five categories based on imaging characteristics meal containing protein, complex carbohydrates and on contrast-enhanced CT scan images. It is used to sugar is a much more accurate stressor than the 75 g guide surveillance for the emergence of malignancy in glucose load used for an oral glucose tolerance test used complex cysts and to avoid unnecessary follow-up. in the diagnosis of impaired glucose tolrerance/diabetes 5. True mellitus.

It is a non-invasive test of inspiratory muscle strength.

ALS is also known as motor neurone disease.

2. e) All of the above Empty nose syndrome is not yet an established condition but a recognition that a number of patients 6. e) All of the above have disturbing and distressing symptoms following nasal surgery. It is a misnomer as it shares far more 7. c) alcoholism symptoms with a blocked nose than an empty one. The CDT may be used to detect high alcohol consumption condition is not well understood and is subject to a lot over a period of time. Healthy individuals with no or of research at the present time. There are no definitive low alcohol consumption will have a CDT less than 2%. diagnostic criteria or reliable tests for this syndrome. It The DVLA sometimes uses this to aid in the assessment for reinstating driver licences. is a diagnosis of exclusion 3. False The Roth Score is a tool for quantifying the level of breathlessness, which is assumed to correlate to the level of hypoxia. It combines maximal count reached (starting from 1 to 30 in one’s native language) during a single exhalation and the time taken to reach the maximum count (the second score is called the “counting time”).

It has been suggested that it might be useful in remotely assessing the severity of patients with Covid-19, but:

8.

e) All of the above.

9.

All are true Severe hypertriglyceridaemia and myeloma with a high serum light chain concentration are examples of causes of pseudohyponatraemia. These patients are usually euvolaemic.

10. b) Dabigatran.

Using the original manufacturing packaging will minimise product breakdown from moisture and consequent loss of potency. It can be placed in a dispenser in its unopened blister pack.

11.

All are true

12.

All are true Heart failure is far from being a specific cause of a raised NT-proBNP. You could also have hyperthyroidism and CKD as causes.

Volume 29, No 3, December 2020

123

WADE CONFERENCE CENTRE at

Now operating as Wade Conference Centre, hiring a room with us helps to fund medical research projects around the North Staffordshire area.

Contact us today to book a room for your event, meeting or conference which will assist us to fund medical research.

01782 714 888 | info@wadeconferencecentre.co.uk

124 www.wadeconferencecentre.co.uk

Midlands Medicine wadeconfcentre