Midlands Medicine - Vol 29 Issue 1 May 2019 by netinspire

MIDLANDS MEDICINE MAY 2019 VOLUME 29 - ISSUE No 1

Editor’s Notes

Manners Matter

100 Years on: World War 1 and Dual Support of Academic Medical Research

An Asymptomatic Aortic Root Abscess with no Previous History of Infective Endocarditis

A Comparison of Scoliosis Correction Surgery in Two Cases with Diﬀerent Causes

A ‘One in A Million’ Varus De-rotation Osteotomy

Management of Medial Patello-Femoral Ligament Injury

More Medical Ceramics

News

Quiz Night

Wordplay 18

Interesting Image

Quiz Answers and Explanations

Index to Volume 28

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 An eight foot high steel sculpture, one of a series of five recently installed at Royal Stoke University Hospital which commemorates many years of social healthcare provision in Staffordshire. See NEWS for more information. Picture: the Editor.

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MIDLANDS MEDICINE

CONTENTS

EDITOR Dr D de Takats

EDITORIAL Editor’s Notes

ASSISTANT EDITOR

Manners Matter Paul Laszlo

Mr C Bolger EDITORIAL BOARD Mr D Gough Dr I Smith Mr D Griffiths Helen Inwood Clive Gibson Tracy Hall Professor Divya Chari EDITORIAL ASSISTANT

100 Years on: World War 1 and Dual Support of Academic Medical Research Mark Smith ORIGINALS An Asymptomatic Aortic Root Abscess with no Previous History of Infective Endocarditis Simran Parmar, Bazegha Qamar & Qamar Abid A Comparison of Scoliosis Correction Surgery in Two Cases with Different Causes Sunna Ali

Spencer Smith

A ‘One in A Million’ Varus De-rotation Osteotomy Sophie Taylor

THE NORTH STAFFORDSHIRE MEDICAL INSTITUTE

Management of Medial Patello-Femoral Ligament Injury Taran Chaudhuri

President: Mr B Carnes Chairman: John Muir Honorary Secretary: Mr J Kocierz Honorary Treasurer: Mr M Barnish

More Medical Ceramics Anthea Bond

Please forward any contributions for consideration by the Midlands Medicine Editorial Board to the Editor c/o Spencer Smith, Editorial assistant.

REPORTAGE

By email: spencer@nsconferencecentre.co.uk Or by post: North Staffs Medical Institute, Hartshill Road, Hartshill, Stoke-on-Trent ST4 7NY

News ENDPIECES 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.

Wordplay 18 Dominic de Takats

Quiz Answers and Explanations

Volume 29, No 1, May 2019

Interesting Image

Index to Volume 28 3

EDITOR'S NOTES Taking care of your own is a concept with wide cultural support across many cultures and times. Of course, it varies in interpretation from meaning your family, your village, your tribe. Or perhaps people like you, those you can relate to for one reason or another. The army is often described as being like a family. What of us in the NHS, in the caring professions, are we together ‘people like us’ or in any sense a family? A recent BMJ essay, Doctors can’t care for patients is the NHS doesn’t care for doctors1, put me in mind of an editorial twice published previously in this journal Covenants in Healthcare by Paul Laszlo which featured in the last issue of Midlands Medicine.2 If the BMJ article interests you, why not give Paul’s article a read in the light of current debate? Enough of past issues, and indeed past volumes of Midlands Medicine, welcome to the first issue of Volume 29! This will perhaps find its way to you a little later than usual but that’s perhaps apt as this year May falls in June. The healthcare working environment can be a stressful one in which to work. Sometimes we ourselves can be the cause of some of that stress. Stress which diminishes productivity and increases errors. We don’t go to work to be on the receiving end of hassle and rudeness, nor should we be dishing it out, nor condoning it should we come across it. In our opening editorial, Paul Laszlo, drawing on the Civility Saves Lives campaign argues simply that (good) manners matter. In the second editorial in this issue, Mark Smith takes us on a journey back in time, about 100 years. The purpose is to gig around the foundations of what is known today as the MRC (medical research council). To many of us it feels as though the MRC is part of the furniture that has always been there. Not so. Mark takes us through the inception and evolution that has left us with today’s funding model for academic medical research and makes the case that the basic idea of dual funding has proved very robust and adaptable over generations. The solid backbone to academic medical research that this central funding model has given lends the opportunity

for modest grant funding, such as that given by the NSMI, to have much better yield because the skills and resources needed to pursue medical research benefit from secure long-term funding. But will this approach survive in an age of austerity? As Editor of Midlands Medicine, one of my jobs is to decide on what to include and what to leave out of each issue. Another of the jobs of an editor is to order the material chosen for inclusion. There are many ways to do this which are not arbitrary. Consideration of length, juxtaposition, section, colour page or not does need to occur, maybe even merit. Last autumn I was privileged to be invited to judge at a case presentation evening held by the Keele University Surgical Society at Keele Campus. Younger medical students had been invited and encouraged to present their cases to an audience of peers and seniors, facing questions from the judging panel at the end. They vied with each other for prestige and for prizes. The standard of presentation was high. As a physician in a subspecialty, I merely note objectively that the content was surgical in orientation and there was perhaps an orthopaedic theme. So, what to do then when faced with a series of equally excellent and interesting case reports? Well, to avoid any sense of favouring one above the other, I have ordered them according to anatomical height in the order: aortic valve, spine, hip, knee. We start, then, with medical student Simran Parmar, and surgeons Bazegha Qamar and Qamar Abid who describe a case of an asymptomatic aortic root abscess with no previous history of infective endocarditis. As incidental findings go, this may not be unprecedented, any more than hen’s teeth. It does make you wonder how on earth the case came about and rather appreciate the immunocompetent state. Next, Sunna Ali looks at scoliosis correction surgery comparing the pros and cons in a neurological origin case with pros and cons in a case of idiopathic onset scoliosis. If some of this is revision for you, I suspect you’ll find yourself most rusty. If you have a prejudice against the intellectual rigor of orthopaedic surgery, this case report could get you to change your mind.

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Sophie Taylor describes the sort of surgical approach that exemplifies the application of craft and skill and thought and effort that is sometimes necessary in paediatric orthopaedic surgery to make a success of a difficult situation. The cause of the need is a rare genetic disorder. Sophie relates the practical help that can be given to a child and his parents if the team listen and understand the problems the parents have and, in turn, explain what they can offer and how it might work. You also catch a sense of the potentially temporary nature to paediatric fixes due to subsequent development. Finally, in this run of excellent case reports we have a case report and discussion by Taran Chaudhuri of the management of medial patello-femoral ligament injury in a young woman. This, perhaps, is not so rare and something you may come across. Surgery is not always required, but when it is the chosen option, a blend of geometry and skill, surgical art and craft is called for in order to achieve the desired result. These case reports represent a huge amount of additional learning and effort by their medical student authors, they are to be commended on their efforts and I recommend these papers to you as most interesting and informative reading.

the task that producing a journal with consistently high quality content and production values requires. If there are any out there who feel they have the time to offer to write, review, edit, or copyedit this journal, I should be grateful if you could make yourselves known to Spencer. Until next time, have a good summer and Happy reading! REFERENCES 1. Elton C Doctors can’t care for patients is the NHS doesn’t care for doctors BMJ (2019) Vol 363 pp434-6 doi: https://doi.org/10.1136/ bmj.l968 2. Laszlo P Covenants in Healthcare Midlands Medicine (2018) Vol 28 pp221-3

Anthea Bond once again selects from the Potteries Museum’s collections to demonstrate another pot with a particular medical use. I’ll posit she’s onto something. Once again I am indebted to Oluseyi Ogunmekan for the quiz. After a run of very focussed quizzes, this one ranges widely and the style of questions varies. The range of difficulty is broad, which gives the opportunities to approach this as either a bit of fun or as serious CPD. As I write this, Mental health Awareness Week 2019 is coming to a close and Prince William is discussing publicly the pain of losing his mother. A week seems hardly enough. I am mindful of the fact that we could do with better coverage in this journal of mental, emotional and psychological matters. But these pages are a reflection, in large part, of the material offered for publication. So, if you feel that such themes, or any others, should be being covered on these pages, your most positive response would be to submit a contribution. Whilst in appeal mode, I note that I have now held the Editorship of this august journal for over a decade. As I become busy with other professional roles, it is becoming more difficult to lend the level of effort to

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MANNERS MATTER Paul Laszlo, Consultant Physician There are a couple of TV series that we have been bingeing on recently, one is Scorpion on Netflix (genre: fiction) and the other is Escape on Channel 4 (genre: reality TV). In the former a team has been put together by the US department of Homeland Security. This is no ordinary team; it is composed of only those of the highest IQ and skills levels. (Mainly the former, as most of the skills are founded on intelligence although also required are emotional intelligence (EQ), dexterity, physical prowess and athleticism, knowledge and so forth. Among them is a doctor, a psychiatrist and psychologist, present not only for his medical knowledge but his ability to analyse, and repair, problems in the team dynamic.) The premise is simple: this elite unit problem solves in desperate crisis situations. These vary from national security threats, to individuals in danger, to quests and tasks and scrapes they find themselves in when on an apparently mundane job. Just as interesting as the intra-episode narratives are the continuing stories of couplings-up within the group, how those are going, how they affect the moods of those in each couple and how those moods impinge on the team functioning, and how that feeds into team performance. Of course, this being fiction, the team always triumphs in the end, but with a nod to the adults in the audience, not without having to face up to a few relationship issues along the way. This drama contrasts with the reality TV series Escape which packages up groups of ‘top’ engineers from different (and rather disparate) disciplines within engineering, sets them down in the mocked-up site of a crash or a natural disaster in a remote location (desert, jungle, arctic ) and requires them to build a viable escape vehicle from scavenged parts. There is a five-day limit imposed on their endeavours. The conditions are harsh, there’s no formal authority structure and they do not know each other’s skill sets. In real time they need to assess each other’s abilities and knowledge, strengths and weaknesses, etc. They have to establish ground rules, create a team and work effectively. Predictably tantrums and tears follow, but usually, but not always, the teams meet with success and bond as a result. (A couple of observations: I guess that people generally, but not always, find themselves better able to put up with their differences of approach in a successful team:

think loss of morale and recriminations in a football team after a heavy defeat versus the same people after an unexpected victory; sometimes the team fails due to circumstances beyond their control and not because they were a dysfunctional team whilst on other occasions a team succeeds in the team objective, but at the expense of emotional damage sustained by one or more team members.) If you’ve had a bad day, some of the time you’ll be ill, at other times something will have happened in the material world that will have had the effect of annoying you, but much of the time what is most likely to have irked you, to have put you in a bad mood, or even been the cause of rage, will have been an interaction with another human being. That can happen at home, before you leave the house, some jibe or insult or slight from your, apparently, nearest and dearest, or on the way to work: either you let someone in front of you and they give no sign of appreciation or you are cut up, or perhaps when you get to work. In the former two scenarios, being at work is often the means by which anger and frustration are dissipated, worked off in the efforts and forgotten in the business of the day. But if the cause of discombobulation is at work and you are immersed in the same environment, reminded of the disharmony and unresolved nature of the situation and, worse still, anxious about the offending behaviour being repeated, then the resentment and/or anxiety will fester. How much does this matter? Potentially a great deal, according to the website Civility Saves Lives which represents the views of UK junior doctors concerned about modern manners in the working environment. (Being more honest, they are concerned to see the end of some of the less attractive, prejudicial and hierarchical mid-20th Century attitudes and practices such as high-handedness, racism, sexism arrogance, authoritarianism, incivility and dismissiveness, among others, which still pervade in some places.) Here are some of their collated observations about rudeness (from broader observational studies in working environments, but for our purposes imagine the situation as often exemplified by a senior doctor

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talking to a junior doctor or from a doctor to a type at least some of the time. Civility Saves Lives has a nurse, but including any rude behaviour between two simple model of how this may come about. They suggest professionals at work): that a stressed individual in a permissive environment will lash out. • Time is lost at work due to people worrying about having been the object of rudeness Think for a moment of the caricature that Sir Lancelot • People leave work early Spratt has become to us as we look back half a century. • Work quality suffers due to distraction of anger, upset Now imagine a similar scene closer to home, one you and resentment can relate to. At work, do you want to be known as the • A quarter of people affected end up passing it on cause of tribulation or as the author of peace? • Loyalty to the organisation and its objectives is reduced • Motivation to perform well, to finish tasks etc. is So, what’s to be done? Here is a simple three-point plan: decreased • Colleague witnesses are also affected in similar ways 1. When you see colleagues treat others badly, tackle • Service user witnesses become less trusting of the them about it, discretely, afterwards, letting them organisation know that you were upset too, that it was awkward and embarrassing, that it didn’t show them in a Sir Lancelot Spratt had his way of doing things which, good light, that their reputation will be damaged, or though bombastic, harrying and from the school of whatever will reach them in such a way that is likely education through humiliation (later shown to be to change their future behaviour; tell them that they generally ineffective) was well meaning and with at upset the object of their wrath, scorn, tirade; suggest least a trace of compassion. There may still be those they go and repair the damage, apologise. amongst us with the innate hubris he manifested, but 2. When you slip up yourself, take the earliest there is worse: there may be some professionals who are opportunity to make amends. unkind to colleagues. This ranges from active cruelty 3. Try using some of these words and phrases at work: and bullying, through unsupportiveness to apparently ‘Please’, ‘Thank you’, ‘Sorry’, ‘How are you?’ more benign disregard or indifference with no hint of malice. But if you are a struggling colleague the last is Great teamwork isn’t always going to just happen, it is only a little better than the first. likely to occur only as the result of a great deal of good leadership, clarity of roles and purpose and held together Few of us would look to try to upset patients or their by mutual consideration, compassion and kindness. If relatives, if not out of compassion then for reasons you cannot muster those qualities, you might achieve of self-preservation. Why are we less careful with at least good teamwork with civility and compromise. colleagues? Do they not matter as much? Or do we presume that they are resilient and can take their rôle as a foil to our frustrations? FURTHER INFORMATION www.civilitysaveslives.com For the sake of our patients, our colleagues and the reputation of our profession, wouldn’t it be good to put behind us the days of junior doctors having to say ADDRESS FOR CORRESPONDENCE things like “I’m sorry he was so rude in front of you and Paul Laszlo, I can see that it upset you, but he is a brilliant surgeon Consultant Physician and you’ll be glad he did the operation.”? C/o The Editor Midlands Medicine Whilst some of us may be unkind or disregarding by North Staffordshire Medical Institute nature, it is probably generally true that those in the Hartshill Road caring professions are, just that: caring. A lot more Hartshill nastiness occurs at work than can be accounted for by Stoke -on-Trent those naturally disposed to dish it out. This suggests ST4 7NY that some decent people must behave counter to their Volume 29, No 1, May 2019

100 YEARS ON: WORLD WAR 1 AND DUAL SUPPORT OF ACADEMIC MEDICAL RESEARCH Mark Smith, Formerly Research Development Manager at the Faculty of Medicine & Health Sciences, Keele University INTRODUCTION Whether we like it or not, many medical advances came about through the needs of war. Treatment of the horrific consequences of the first truly technological warfare in World War 1, between 1914 and 1918, is a centenary that is being marked by many poignant events. School pupils are taught how scientific and medical developments such as mobile x-ray units, effective wound cleaning and organised blood storage all increased the chances of survival for the wounded soldier in World War 1. Indeed Keele University has put on a whole series of public understanding of science events in Stoke around this theme. Academic researchers have another, rather obscure, reason for commemoration: December 2016 saw the centenary of what became known as the “Dual Support” system of funding research in UK universities. It is such an important underlying principle of British university research but, in general, it is so embedded and works so well that we hardly notice it. The Dual Support system can be argued to be a key foundation on which many British universities have been able to flourish as research institutions. Dual Support gives them autonomy and stability from their “block grants” as a result of the Research Excellence Framework, and it gives them tremendous responsiveness and flexibility through applying for competitive research grants.

bodies such as the Royal Society, and the wealthy amateur. Back in 1889, Queen Victoria’s Treasury had established an ad hoc University Grants Committee (UGC) to distribute £15,000 to 11 university colleges to “advance international knowledge”, which would be in excess of £1 million today. This idea that government might invest in research institutions led to one side of the Dual Support system, is known today as the “block grant” or “Quality Research (QR) grant” from Research England (recently re-organised from the Higher Education Funding Council for England, HEFCE). The key feature was, and is, that this money is spent at the universities’ discretion, being allocated following a general and retrospective assessment of the quality of each institution’s research. We still have this system, now allocating about £2 billion per year. Allocation is based on the ratings from periodic assessment through the Research Assessment Exercise (RAE) now re-branded as the Research Excellence Framework (REF), which has been held since 1986. COMPETITIVE PROJECT GRANTS

Huge changes in warfare, arising from the stalemate of the trenches of the Western Front, meant that firepower increased, distances of rifle and shell fire were short, battles became more technological, and the consequent toll of human death and suffering became immense. The charitable sector expanded rapidly to help the war effort but was very disorganised and focussed on the welfare of soldiers1, not the development of improved conditions, protective equipment and BLOCK GRANTS medical techniques. A targeted, organised approach The Dual Support system of funding research emerged was needed. In May 1915 the President of the Board from World War 1. Before 1914 the UK government of Education presented to Parliament a White Paper had very little concern with research of any kind, it urging that a permanent organisation - the Committee was the preserve of industry, the learned, through for Scientific and Industrial Research - should be set up 8

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for the promotion of scientific and industrial research. It became the Department for Scientific and Industrial Research (DSIR) in December 1916 and lasted until 1965.

to TB. The members organised visits to researchers in laboratories throughout the UK submitted the first national ‘scheme of research’ for ministerial approval. The committee initiated its own research programmes and also responded to matters of medical concern raised The DSIR’s first scheme was a lump sum of £1 million, by government departments, doing so through subknown as the “Million Fund” but worth about £30 committees and research units specialising in specific million today, to encourage groups of firms to set up areas. It also provided funding for research by outside co-operative industrial research associations. The bodies or individuals, complementing the research DSIR encouraged and supported scientific research in resources of universities and hospitals. universities and other institutions by means of grants for special research projects, research fellowships, studentships. It is a format we still recognise today ARM’S LENGTH FUNDING FOR HIGH QUALITY through the schemes run by the UK Research Councils RELEVANT STUDIES and charities. A key feature of the project grants side of Dual Support is that it takes a prospective view of likely Narrowly avoiding being merged with a Ministry of research quality, as well as likely impact, in response to Health for England and Wales which was responsible specific, costed, competitive proposals. for National Health Insurance, in 1918-19, the Medical Research Council retained its independence and became the royal chartered organisation we know today. THE FIRST RESEARCH COUNCIL FOR MEDICAL Its independence from direct government intervention RESEARCH was first proposed in a 1918 report by a committee led by Lord Richard Haldane. The report said: “Although In parallel, the Medical Research Council (MRC) came the operations of the Medical Research Committee into being just after WW1. In 1911, Parliament passed are within the province of the Minister responsible for the National Insurance Act, introduced by David Lloyd Health Insurance…the Minister relies…upon the MRC George as Chancellor of the Exchequer, which put in to select the objects upon which they will spend their place schemes for health and unemployment insurance. income, and to frame schemes for the efficient and One provision – paid for with a penny per working economical performance of their work.” person per year - was sanatorium treatment for cases of tuberculosis and for ‘purposes of research’. This created Independence of bodies such as the UK Research a national fund for medical research and amounted to Councils from direct government control has since £57,000 a year (equivalent to nearly £4 million today). became known as the ‘Haldane Principle’. The royalThe executive committee decided that the money chartered Research Councils now spend about £3 billion should be spent on research carried out by investigators of taxpayers’ money on university research per year with that it would employ and who would work in approved relative autonomy. The MRC focussed initially on its institutions. It also thought that some ‘exceptional’ own institutes, the first being the National Institute for researchers should be given a salary and pension so that Medical Research in London in 1920, but now supports they could devote their whole time to research. “Efforts a wide range of projects in universities throughout the should also be made to retain for research young and UK. The first MRC grants came to North Staffordshire talented investigators who would otherwise tend to via Keele University’s School of Postgraduate Medicine drift into other lines,” the committee said. in the late 1980s. Keele currently holds a portfolio of at least 15 MRC research projects simultaneously, which The Medical Research Committee and Advisory receive over £650,000 per year in MRC funds. Most of Council (now simply MRC) was set up in 1913. It was, the projects involve partners at University Hospitals in effect, a single research organisation for the whole of North Midlands NHS Trust and the Robert Jones & of the UK, with funds provided under the National Agnes Hunt Orthopaedic Hospital at Oswestry. Insurance Act for medical research, and not limited

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CHANGES TO THE DUAL SUPPORT SYSTEM Despite some periodic calls for radical change2 the only changes to Dual Support in over 100 years have amounted to using it as a policy driver, or tinkering around the edges of its formulas. From the mid-1980s, and the advent of the Research Assessment Exercise3 there has been an effective drive to use the block grant aspect to drive selectivity, i.e. reducing the number of departments and universities that receive QR funding, to reduce the â&#x20AC;&#x153;tailâ&#x20AC;? of the funding graph and concentrate resources in the highest quality research environments (figure 1).

Figure 1: Distribution of HEFCE research funding between institutions from 1992 & 1996 RAEs. Each point on the X-axis represents a separate institution.4

There have been alterations in the balance of funding through the two arms of Dual Support. In response to a gradual increase in project grants awarded by the Research Councils and many other bodies, the QR element was increasingly stretched and unable to provide high quality infrastructure for all the research that was going on. In the early 1990s some of the money paid as QR was transferred to project grants, initially to fund overheads at 46% of staff costs. This was resisted by many large institutions but was of particular value to universities such as Keele which were rapidly expanding their research and able to adequately fund each project.

Figure 2: The changing balance of dual support: the disaggregation of UK higher education research support funds into core and project sources, underpinning the transfer of some core funding to grants, and the eventual need for Full Economic Costing.5

This balance was refined in the mid-2000s with REFERENCES Full Economic Costing (FEC) which calculated all 1. Grant P (2014) Philanthropy and Voluntary the resources needed for a high quality research Action in the First World War: Mobilizing Charity, environment in a given year, and apportioned them Routledge as overheads in bids made the following year, thus enabling strength and growth to be maintained. 2. Royal Society (2003) Supporting basic research in science and engineering: a call for a radical review of the UK’s dual support system RS Policy Document 25/03 CONCLUSION AND PARALLELS Dual Support is a foundation stone of the vibrant research carried out in Britain today. As a nation we have tinkered with it only a little over the years, and it has been imitated by other developed nations seeking to build their research culture. It is interesting to note that the efforts to fund clinical research in the UK National Health Service have also followed its pattern, moving a decade ago from an almost entirely block grant based system – Culyer Funds – to a projectgrant driven system – the National Institute for Health Research (NIHR) – using FEC and underpinned by infrastructure funded through the Research Capability Funding (RCF). A crucial difference is that CCF is allocated based on volume of relevant clinical research activity, not through a periodic quality exercise like REF. Although it came about almost by accident, Dual Support serves us well and 100 years on it is still fit for purpose, despite the core funding in most universities being very over-stretched to support the demands for more research, especially in biomedicine and health. Adams & Bekhradnia, when asking in (2004) What future for dual support?5, stated “The key issue is not one of changing the way funds are allocated, but substantially restoring the core funding stream and building back the characteristics that have enabled the UK research base to be so effective and so efficient for so long.” In other words: the funding model works well, but the amount of funding matters too; you get what you pay for.

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3. UGC A strategy for higher education into the 1990s the University Grants Committee's advice. HMSO (1984) 4. Harris M The role of selectivity and the characteristics of excellence Higher Education Funding Council for England 1999 5. Adams J & Bekhradnia B What future for dual support? Higher Education Policy Institute, February (2004)

FURTHER INFORMATION Medical Research Committee, First Annual Report, 1914-15 https://mrc.ukri.org/news-events/ publications/annual-report-1914/ “Records created or inherited by the Department of Scientific and Industrial Research, and related bodies". DSIR53/1 Annual Reports. The National Archives (2018)

ADDRESS FOR CORRESPONDENCE marksmith@rvc.ac.uk

AN ASYMPTOMATIC AORTIC ROOT ABSCESS WITH NO PREVIOUS HISTORY OF INFECTIVE ENDOCARDITIS Simran Parmar, Medical Student, KUSoM; Bazegha Qamar, Leicester Medical School and Qamar Abid, Consultant Cardiothoracic Surgeon, Royal Stoke University Hospital SUMMARY

BACKGROUND

A 48-year-old male with a type 1 bicuspid aortic valve presented to general practice with chest tightness, dyspnoea, and a six-month history of syncope and extreme fatigue. The patient had had pericarditis at the age of 35 years but no history of infective endocarditis. A transthoracic echocardiogram revealed moderate aortic stenosis (peak gradient 48 mmHg, aortic valve area 1.2cm2) and aortic regurgitation. A CT aortogram showed dilatation of the aortic root (diameter 4.6cm) and ascending aorta (diameter 4.8cm). The patient underwent an elective replacement of the ascending aorta and aortic valve (mechanical prosthesis). During the procedure an annular abscess was incidentally discovered. The annulus was debrided and washed thoroughly with gentamicin solution. The operation and recovery were without complication. This case highlights the complications associated with bicuspid aortic valve including asymptomatic aortic root abscesses.

Bicuspid aortic valve (BAV) is the commonest congenital cardiac variant in adults.1 It is associated with many cardiac conditions including aortic stenosis and regurgitation, aortic aneurysms and infective endocarditis.2 BAV has an incidence of 0.9% - 2% in the general population and has a strong familial association (9% of first degree relatives of people with BAV also have the anomaly).3 Normally the aortic valve is comprised of three individual cusps (the right and left coronary cusps anteriorly and the non-coronary cusp posteriorly). In BAV there is abnormal fusion of one or more of the cusps during development. This increases the risk of disease to the aortic valve and surrounding structures.4 There are three different subtypes of BAV, as illustrated in Figure 1. They are called type 1, 2 or 3, based on the number of raphes (false commissures) that are present.5

Fig 1. The different morphologies of the aortic valve5

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The condition of BAV is associated with significant morbidity and mortality. A major reason for this is because of its association with aortic stenosis (AS). AS is defined as a chronic and progressive narrowing of the left-ventricular outflow tract due to a disease process similar to atherosclerosis.2,6 Nearly all patients with BAV show calcification of the aortic valve by the age of 30.4 As the disease progresses the aortic valve area decreases. This causes an increase in enddiastolic volume (EDV).6 Invoking the Frank-Starling mechanism (EDV is proportional to stroke volume), this in turn causes an increase in stroke volume. This increased cardiac output causes the left ventricle to undergo hypertrophy, increasing its oxygen demand and resulting in ischemia. This combined with pulmonary hypertension from increased EDV and the inability to raise mean arterial pressure during exercise are responsible for the cardinal symptoms of AS: angina, syncope, breathlessness, and fatigue.2 However these

symptoms only present after a prolonged latent period.6 Symptomatic AS has a very poor clinical outcome; as shown in Figure 2, the average life expectancy of symptomatic AS without surgical intervention is 2 â&#x20AC;&#x201C; 3 years.6 The medical management options for aortic stenosis are limited, and are primarily used to maintain an optimal blood pressure and treat complications of the disease such as heart failure.7 The best treatment of symptomatic AS is an open aortic valve replacement (AVR). AVR should also be considered in patients with asymptomatic disease if there is a high likelihood of rapid progression.6,8 Patients who are considered to be too high risk for open AVR may be eligible for a trans-catheter aortic valve implantation (TAVI).8 This determination is made by clinical judgement, the size and shape of the aortic root, and by the use of validated scoring systems such as EuroSCORE (European System for Cardiac Operative Risk Evaluation).

Fig 2. The natural history of aortic stenosis without surgical intervention. A prolonged asymptomatic period is followed by the onset of symptoms and a steep decline in survival6

Infective endocarditis (IE) is another major complication experienced by up to 30% of patients with BAV.9 IE is a life-threatening infection of the cardiac endothelium, native valves, or iatrogenic structures such as mechanical valves or pacing wires. IE often presents as an acute or a sub-acute illness, and is predisposed by valvular or endocardial lesions (including BAV). Pathognomonic features include peripheral signs of septic emboli,

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including Janeway lesions, splinter haemorrhages, and Oslerâ&#x20AC;&#x2122;s nodes. Other clinical features include new murmurs, haematuria, renal infarction, and pyrexia.9 The management of IE includes a prolonged course of antibiotics and surgical removal of infected tissue. Despite this, the outcomes of IE are poor. Patients have an in-hospital mortality rate of between 15-20%.10

CASE PRESENTATION A 48-year-old male broadcast engineer manager with a known type 1 bicuspid aortic valve (rightleft commissure) presented to general practice with chest tightness, dyspnoea, and a six-month history of episodes of syncope and extreme fatigue. The patient also experienced chest pain radiating to his neck and back. His past medical history included a tonsillectomy at 18 years-old, pericarditis at 35 years-old, and a history of gout. The patient had been regularly monitored for his BAV up until 4 years previously. He had no known allergies and his only regular medication was allopurinol for gout prophylaxis. His father had ischemic heart disease and had undergone coronary artery bypass graft surgery and both his siblings were not known to have any congenital heart defects. He had never smoked but drank 24-36 units of alcohol per week. INVESTIGATIONS After presenting to GP, he was referred to A&E for further investigations. All blood results including troponin levels were normal, and his chest radiograph was clear. He was classified as grade 2 according to the Canadian Cardiovascular Society (CCS) grading of angina pectoris and class 2 according to the New York Heart Association (NYHA) classification of heart failure. A CT aortogram revealed a dilated aortic root (4.6cm) and ascending aorta (4.8cm) (Figure 3a) as well as a heavily calcified aortic valve (Figure 3b). The aorta returned to a normal calibre just proximal to the brachiocephalic vein. A trans-thoracic echocardiogram revealed mixed aortic stenosis (moderate severity, peak gradient 48 mmHg, aortic valve area 1.2cm2) and aortic regurgitation. A coronary angiogram was also performed, and showed clear, unobstructed coronary arteries.

Fig 3a. CT scan showing the patients dilated (4.8cm diameter) ascending aorta (red arrow)

Fig 3b. CT scan showing heavily calcification of the aortic root (red arrow)

The patient was started on bisoprolol after presenting to A&E because of a raised systolic blood pressure (150 mmHg). After a work-up the patient consented to an elective AVR and an aortic root and ascending aortic aneurysm repair. He was informed of the risks of surgery which included arrhythmias (1-in-3), cerebrovascular accident (1-in-50), permanent pacemaker (1-in-70), and sternal infection (1-in-100). The patients EuroSCORE II risk of mortality was 2 - 3%. The operation started with a midline sternotomy followed by careful dissection down to the pericardium. The right atrium and aorta were cannulated, and the heart was placed on a cardiopulmonary bypass machine. The patient was cooled to 34Â°C, and a high potassium cardioplegia solution was delivered to the coronary arteries. The surgeon clamped the aorta and then dissected the aortic root to reveal an abscess in the annulus which occupied approximately half of the length of the left coronary cusp. The area around the abscess was heavily calcified. The pus inside the abscess was a creamy-white colour, it was sent for microbiological evaluation. The aortic root was thoroughly debrided and washed with a gentamicin solution. A mechanical 23mm Sorin fitline aortic valve was stitched into the aorta, and a 28mm Gelwave tube graft was used to repair the dilated ascending aorta. Pacing wires were placed on the atria and ventricles, and drains were left in the mediastinum and both hemithoraces. After the operation, the patient was started on dalteparin, warfarin, paracetamol, oxycontin, laxatives and omeprazole, in addition to his regularly prescribed bisoprolol and allopurinol. He was also prescribed a six-week course of rifampicin and vancomycin as a prophylactic measure to reduce the risk of infection of his new prosthetic valve. Midlands Medicine

microbiological cultures taken from the abscess at the time of surgery showed no growth. There are perhaps The patient was in significant discomfort in the first few two explanations for this. The first is that the patient’s days following the operation. He experienced sharp, immune system had successfully cleared the infection shooting pains in his chest, abdomen, and neck. He at some point in time before the operation, which is slowly began to mobilise more as the pain subsided, why there was no growth on the culture. The other and his pacing wires were removed on the fourth day. explanation is that the growth of the organism was Two weeks after surgery his sternum was still very sore inhibited by pre-operatively administered antibiotics however he was now able to walk independently with which all cardiac surgical patients receive. ease. He was discharged from hospital 19 days after the The other interesting aspect of this case is that the operation, and went on to have a normal recovery. patient developed a cardiac abscess without any history of IE. All previously published reports on cardiac abscesses were preceded by symptomatic IE except DISCUSSION for one report from a team of surgeons in Germany. This case highlights the importance of regular They describe an asymptomatic myocardial abscess in monitoring in patients with BAV. The adverse effects of a patient with no history of endocarditis.12 The abscess BAV can be mitigated by regularly screening patients for was initially mistaken for a cardiac tumour and the associated conditions such as AS and aortic aneurysms. patient was operated on. Apart from this case, no others It is also important that healthcare providers know that have mimicked this patient’s natural history of having their patient has BAV because otherwise rare and life- unrecognized endocarditis followed by late asymptomatic threatening conditions (such as aortic dissections and abscess formation. IE) present much more commonly in this patient group. This knowledge will encourage providers to think about these rare conditions during acute presentations and LEARNING POINTS make them less likely to rule these conditions out based • Bicuspid aortic valve is associated with significant on the infrequency of these diseases. morbidity and mortality, including aortic stenosis and infective endocarditis The unique aspect of this case is that the patient’s incidentally discovered annular abscess was completely • Regular monitoring for people with bicuspid aortic valve is essential to help prevent serious complications asymptomatic. The patient presented with no signs of acute or sub-acute illness. Cardiac abscesses usually • Asymptomatic cardiac abscesses may develop without being an evident complication of previous infection present with systemic features including fever, lethargy, chest pain, arthralgia, rashes and headaches and have high mortality rates.11 The lack of systemic symptoms suggests that the body had isolated and sterilised the ADDRESS FOR CORRESPONDENCE abscess and prevented it from entering the blood and causing septicaemia. The fact that the annulus and Simran Parmar the adjacent structures surrounding the abscess were C/o The Editor heavily calcified suggests that it was not a new or active Midlands Medicine abscess, and it was most likely the result of an old bout North Staffordshire Medical Institute of infective endocarditis. This means that we cannot Hartshill Road be sure exactly when the abscess had formed. The pus Hartshill was a creamy-white colour, which could be suggestive Stoke-on-Trent of a staphylococcus aureus infection, however ST4 7NY OUTCOME

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REFERENCES 1. Tzemos N, Therrien J, Yip J, Thanassoulis G, Tremblay S et al Outcomes in adults with bicuspid aortic valves JAMA (2008) Vol 300 pp1317-25 2. Kumar, P and Clark, M (2012) Kumar & Clark's clinical medicine 8th ed London: Elsevier, pp 746748 3. Yener N, Oktar GL, Erer D, Yardimci MM, Yener A Bicuspid aortic valve Annals of thoracic and cardiovascular surgery (2002) Vol 8 pp264-7 4. Adamo L, Braverman AC Surgical threshold for bicuspid aortic valve aneurysm: a case for individual decision-making Heart (2015) Vol 101 pp1361-7 5. Sievers HH, Schmidtke C A classification system for the bicuspid aortic valve from 304 surgical specimens The Journal of thoracic and cardiovascular surgery (2007) Vol 133 pp1226-33 6. Carabello BA, Paulus WJ Aortic stenosis The lancet (2009) Mar Vol 373 pp956-66 7. Siu SC, Silversides CK Bicuspid aortic valve disease Journal of the American College of Cardiology (2010) Vol 55 pp2789-800

8. Chacko M, Weinberg L Aortic valve stenosis: perioperative anaesthetic implications of surgical replacement and minimally invasive interventions Continuing Education in Anaesthesia, Critical Care and Pain (2012) Vol 12 pp295-301 9. Fedak PW, Verma S, David TE, Leask RL, Weisel RD, Butany J Clinical and pathophysiological implications of a bicuspid aortic valve Circulation (2002) Vol 106 pp900-4 10. Murdoch DR, Corey GR, Hoen B, MirĂł JM, Fowler VG, et al Clinical presentation, etiology, and outcome of infective endocarditis in the 21st Century: the International Collaboration on Endocarditisâ&#x20AC;&#x201C;Prospective Cohort Study Archives of internal medicine (2009) Vol 169 pp463-73 11. Choussat R, Thomas D, Isnard R, Michel PL, Iung B, Hanania G, Mathieu P et al Perivalvular abscesses associated with endocarditis: clinical features and prognostic factors of overall survival in a series of 233 cases European heart journal (1999) Vol 20 pp232-41 12. Borowski A, Korb H, Voth E, De Vivie ER Asymptomatic myocardial abscess The Thoracic and cardiovascular surgeon (1988) Vol 36 pp338-40

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A COMPARISON OF SCOLIOSIS CORRECTION SURGERY IN TWO CASES WITH DIFFERENT CAUSES Sunna Ali, Medical Student, KUSoM BACKGROUND Treatment for NMS is focussed not on curing the primary condition but on preventing the progression of the deformity. The underlying pathology also amplifies the complexity of surgical intervention in NMS. Medical therapy includes custom moulded thoracolumbosacral orthosis (TLSO) and moulded seating Adolescent Idiopathic Scoliosis (AIS), is the most supports.4 Principles of surgery in NMS differ from common cause of scoliosis accounting for 85% of cases, those in AIS, as deformity is identified at a younger with no identifiable underlying pathologies causing age, and the fused portion of the spine is longer. this deformity and with an, as yet, unknown aetiology. Fusion to the sacrum is fairly common because many An increased incidence is seen with a positive family of these children do not have sitting balance or have history of scoliosis, suggesting a genetic predisposition.2 pelvic obliquity).5 Indications for corrective surgery Gender also makes difference in the time of onset are overall improvement in functional level, cosmesis, because girls begin their adolescent growth spurt and respiratory status, pain, and overall quality of life of the patient. However, it is also associated with a high risk of reach skeletal maturity earlier than boys. peri- and postoperative complications.6 Management for AIS uses a multidisciplinary approach with Scoliosis In-patient Rehabilitation (SIR), corrective bracing and Spinal Correction Surgery with or without CASE PRESENTATION spinal fusion. The indications for surgery have been set to a curve measurement of 50Â° Cobb angle indicating Patient A was a 15-year-old female presenting with a a significance that may lead to the patient developing curvature in the spine accompanied by back pain in health risks. Other indications for surgery in AIS the mid-thoracic region, initially noticed in her hot patients are cosmetic or psychological, to correct the tub during the summer, there had been a degree of progression since. She was 2 years postmenarchal and alignment of the trunk to improve self-esteem. has no other medical problems, including no significant In comparison, Neuromuscular Scoliosis (NMS) family history. She engaged in activities including is secondary to other neuromuscular pathologies, basketball and football. Her mid-thoracic back pain whereby the deformity of the spine arises due to was exacerbated by her sporting activities. She was abnormalities of the myo-neural pathways of the body. unhappy with her body alignment and complains of Loss of muscular support of the spinal column causes shoulder protrusion and truncal rotation (rib cage and mal-alignment with muscles failing to supply the breast asymmetry). On examination, the patient had normal supportive tensioning and pulling in abnormal undergone puberty. Her left shoulder was higher and directions. NMS usually develops before the age of 10, more prominent and her left hip was higher with the left thus early-onset. In neuromuscular spinal deformities, leg presenting slightly shorter. She had normal vesicular progression occurs much more frequently than in breathing with no added sounds. A full neurological idiopathic scoliosis.3 Scoliosis often presents itself, or examination excluded any neurological cause. worsens, during an adolescent's growth spurt. Scoliosis has been defined as a lateral curvature of the spine greater than 10Â° in the coronal plane.1 This three-dimensional spinal structural deformity is fairly common. It develops mainly during puberty related to a growth spurt. A number of onsets are recognised.

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Patient B was a 16-year-old male with cerebral palsy and dystonia who presented to the hospital with sitting balance problems, due to his progressive curvature. Developing since the age of 10, it was more noticeable after weight gain with per-endoscopic feeding gastrostomy (PEG) insertion three years prior. In addition, the patients father described a complaint of severe gagging and constipation. An examination revealed patient 45° lean to the right side and pelvic asymmetry with left hip protrusion with a C-shape spine typical of neuromuscular scoliosis. These are both examples of structural deformities where the curve is fixed. In both cases, it had either developed or worsened after a growth spurt in puberty.

No associated vertebral abnormalities were noted. Figure 1a shows the Cobb angle measures approximately 47.1°, with the apex of the curve at the T9 vertebral body. Figure 1b shows the iliac apophyses are complete along the iliac crests, but have not yet fused with the ilium, indicating that the patient has not yet reached skeletal maturity (Risser’s Sign). In comparison, patient B had a sitting AP radiograph of the entire spine (as unable to stand) which demonstrated an arcuate thoraco-lumbar scoliosis with a rightward convexity (C-shape). Figure 2 shows the measurement 52° Cobb angle, with an apex of the curve at the L2 vertebral body.

Standing radiographs were used to monitor severity and progression and magnitude of the curves preoperatively for Patient A. The antero-posterior (AP) standing radiograph of the entire spine demonstrated an arcuate thoracic scoliosis with a rightward convexity.

Fig. 1b: iliac apophyses are complete along the iliac crests, but have not yet fused with the ilium, indicating that the patient has not yet reached skeletal maturity.

Fig. 1a: Standing AP of the entire spine demonstrate an arcuate thoracic scoliosis with a rightward convexity. Cobb angle is 47.1 degrees measured from top of T5 and the bottom of the T12 vertebral bodies.

Specialised lateral bending films allow assessment of the flexibility of the curve, this is important assessment to decide if which segment of the curve should be included in the surgical fixation. Patient A shows a S- shape curve, thus you have a primary curve and a compensatory curve. Figure 3a shows a lateral bend to the left, assessing the flexibility of this compensatory curve, as this is straight it wasn’t included in the fixation. Figure 3b assesses the primary curve, within the thoracic area showing no flexibility, thus included in the spinal correction fixation.7 Figure 4 demonstrates the fulcrum bend radiographs, with application of force in different positions, further assessing the flexibility of the compensatory curve. It straightens out when force applied onto it, and the thoracic curve doesn’t change. With this curve in the surgical range and the patient concerned over the appearance of her back, surgical treatment is a reasonable undertaking. Midlands Medicine

Fig. 4: Fulcrum bend views, with application of force in different positions, further assessing the flexibility of the compensatory curve.

The surgical procedure used was a posterior spinal fusion with instrumentation and bone graft. The term "instrumentation" refers to a variety of devices such as rods and screws, which are used to hold the correction of the spine in as normal an alignment as possible while the bone fusion heals. The instrumentation is rarely removed. Fig. 2: Sitting AP of the entire spine demonstrate an arcuate thoracolumbar scoliosis with a rightward convexity. Cobb angle is 52 degrees.

Fig. 5a (left): shows lateral bend to the left (same direction patient sits in his wheelchair). Fig. 3a (above left): shows a lateral bend to the left, assessing the flexibility of this compensatory curve. Fig. 3b (above right): shows the bend to the right assessing the primary curve.

Patient Bâ&#x20AC;&#x2122;s specialised lateral bending films are shown in Figure 5a with a left lateral bend, which is the same direction the patient leans in their wheelchair, shows the extent of the curve. The right lateral bending film in Figure 5b shows that the curve does straighten up, thus flexible and a good indication for surgery for an improvement of posture in the wheelchair. Volume 29, No 1, May 2019

Fig. 5b (right): right lateral bending film.

For patient A, surgery completed was from T2-T12. Figure 6A shows the patientâ&#x20AC;&#x2122;s shoulders and balance is nicely restored with very minimal truncal deformity. Radiographically, there is persistent coronal tilt on the lower instrumented vertebra which is required to maintain the balance of the two curves. There has been significant improvement in the apical vertebral translation of both curves and levelling of the rib cage and shoulders. Figure 6B shows the sagittal section showing improved thoracic kyphosis and lumbar lordosis. 19

For patient B, surgery completed was from T2- S1 fixating the whole spine. Surgical stabilization constitutes the mainstay of treatment for neuromuscular scoliosis. Progressive curves require surgical correction and stabilization, including the pelvis.

Fig. 6a (above): coronal standing X-rays before and after the surgical procedure.

Fig. 6b (above): sagittal standing X-rays before and after the surgical procedure.

Fig. 7a: coronal sitting X-rays before (above left) coronal views (above right).

Fig. 7b: post-surgical sagittal sitting radiographs (above left) and coronal view of extent of the pelvic fixation (above right).

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DISCUSSION According to the Society on Scoliosis Orthopaedic and Rehabilitation Treatment measurement of these curves are necessary to qualify a patient for surgery. Conservative management usually includes observation of curve progression indicated for patients with a curve less than 20°. Monitoring occurs with 6 monthly/ yearly basis with whole spine PA radiographs.8 Patients that warrant discharge are those who reach skeletal maturity with scoliosis curve under 40°.9 Bracing is another treatment option, which has shown to be effective to prevent progression of high-risk curves but has poor compliance due to the long hours wearing and the nature of the brace.10 For curves with a high likelihood of progression (greater than 45 to 50° of magnitude), these would be cosmetically unacceptable as an adult. For patients with spina bifida and cerebral palsy, progressive curves would interfere with sitting posture and care, which may also affect physiological functions such as breathing.3 Surgical management is thus indicated improve the deformity and to prevent progression of deformity in the future. Advancing procedures have come a long way recently, with new "instrumentation" procedures consisting of a rod and screw combination, used to hold the spinal correction. To reduce the risks of neurological injuries, intra-operative neuro-monitoring with evoked motor potentials is completed during the procedure. Outcomes for surgery ideally would be to correct the deformity in the coronal and rotational planes, whilst maintaining sagittal balance in thoracic kyphosis and lumbar lordosis.9 Although both patients presented had progressive curves greater than 45°, the different onsets of scoliosis for these patients meant indications for surgery, potential risks, complications and outcomes of correction surgery differed. For patient A with AIS, indication for surgery was cosmetic to correct the visual presentation of the deformity. The Scoliosis Research Society modified SRS-22 questionnaire is a valid instrument to assess the quality of life of patients with AIS.11 With domains in function, pain, mental health, self-image and satisfaction/dissatisfaction, this contributed to indication for surgery for Patient A. Unhappy with

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her body alignment and interference of functioning of playing sports, the surgery would have good consequences on her social and phycological wellbeing. The patient was satisfied with the surgery, excited to resume normal social activities and level of function. In comparison, the SRS-22 wouldn’t be applicable in the case of Patient B with neuromuscular scoliosis, as only the pain and function domains could be assessed. The surgical indications for this case would mainly be increased level of function, with posture realignment and rotational correction, overall improving this patient’s chair balance. With social support, the correction surgery would improve caring responsibilities for the patient’s father as the patient would be easier to move into and out of his chair. In addition to his body alignment, the patient’s quality of life would greatly improve, with ease of gagging and constipation, with better ventilation to help the patient feel less uncomfortable. More attention would be needed for this patient’s postoperative plan, as the surgical correction procedure would have been a duration of 10 hours instead of Patient A’s 6-hour procedure. Further, it comes with higher risk of complications as a longer segment of spine was operated on. Being PEG fed, this patient would be immunocompromised having needs for nutritional support, and there may be a higher risk of infection with a larger incision, and involvement of pelvic fixation. There are also more chance of bleeding with a neuromuscular scoliosis than the AIS procedure. These were highlighted as the patient did need a blood transfusion and became respiratory acidotic, which was corrected. The implications of certain post-operative complications for Patient A differed however. With intensive work on the spine, the spinal correction surgery came with a risk of paralysis. This would be a catastrophic outcome for Patient A, as an otherwise healthy individual, who went for surgery for improved functioning and cosmesis. In Patient B’s case, whom is wheelchair bound and already completely dependent on his carer, this complication would be less of detriment. In essence, intra-operative motor evoked potential neuro-monitoring significantly reduces the risk of paralysis.9 This has now been made mandatory by the Scoliosis Research Society in surgical correction as it maintains good practice.

CONCLUSION

REFERENCES

Patients reported outcomes of improved level of function and satisfaction with surgery is what drives surgical results which can be applied to future patients with similar clinical presentations. Although each case presented with different onsets, indications of surgery and complication risks, all patients will undergo an extensive functional, social and psychological assessment. In addition to this, a full examination of body alignment, flexibility and central function is used to decide on surgical management and the degree of scoliosis correction. The main aim of surgery is to achieve a balanced spine with level shoulders and pelvis with normal sagittal balance regardless of the aetiology of scoliosis. Ultimately, restoring level of function and satisfaction for patients, with care taken to minimise complications, will improve short and long-term quality of life.

1. Weiss HR Adolescent Idiopathic Scoliosis–case report of a patient with clinical deterioration after surgery Patient safety in surgery (2007) Vol 1 p7

ACKNOWLEDGEMENTS This work was supported by Mr E Ahmed (UHNM), who provided expertise that greatly guidance the work. In addition, many thanks to Mr E Ahmed and Mr N Rouholamin (UHNM) for and for allowing observation of the operations, and to the patients and their families. ADDRESS FOR CORRESPONDENCE Sunna Ali C/o The Editor Midlands Medicine North Staffordshire Medical Institute Hartshill Road Hartshill Stoke-on-Trent ST4 7NY

2. Czeizel A, Bellyei A, Barta O, Magda and T Molnar L Genetics of adolescent idiopathic scoliosis J Med Genet; (1978) Vol 15 p424e7 3. Nordon DG, Lugão AF, Machado LCC, Marcon RM, Cristante AF at al Correlation between the degree of correction of neuromuscular scoliosis and patient quality of life Clinics (2017) Vol 72 pp71-80 4. Asher MA and Burton DC Adolescent idiopathic scoliosis: natural history and long term treatment effects Scoliosis 2006 (1) p2 doi.org/10.1186/17487161-1-2 5. Cotton LA Unit rod segmental spinal instrumentation for the treatment of neuromuscular scoliosis Orthopedic nursing (1991) Vol 10 pp1723 6. Sharma S Wu C Andersen T Wang Y Hansen ES and Bünger CE Prevalence of complications in neuromuscular scoliosis surgery: a literature metaanalysis from the past 15 years European Spine Journal (2013) Vol 22 pp1230-1249 7. King HA Moe JH Bradford DS and Winter RB The selection of fusion levels in thoracic idiopathic scoliosis J Bone Joint Surg Am (1983) Vol 65 pp1302-13 8. Dimeglio A, Canavese F and Charles YP Growth and adolescent idiopathic scoliosis: when and how much? Journal of Pediatric Orthopaedics (2011) Vol 31ppS28-S36

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9. Hamad A Ahmed EB and Tsirikos AI Adolescent idiopathic scoliosis: a comprehensive approach to aetiology diagnostic assessment and treatment Orthopaedics and Trauma (2017) Vol 31(6) pp343-349 10. Weinstein SL Dolan LA Wright JG and Dobbs MB Effects of bracing in adolescents with idiopathic scoliosis New England Journal of Medicine (2013) Vol 369 pp1512-1521 11. Glattes RC Burton DC Lai SM Frasier E and Asher MA The reliability and concurrent validity of the Scoliosis Research Society-22r patient questionnaire compared with the Child Health QuestionnaireCF87 patient questionnaire for adolescent spinal deformity Spine (2007) Vol 32 pp1778-1784 FURTHER INFORMATION Scoliosis Research Society (2018) Patient Outcome Questionnaires: SRS-22 https://wwwsrsorg/professionals/onlineeducation-and-resources/patient-outcomequestionnaires

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A ‘ONE IN A MILLION’ VARUS DE-ROTATION OSTEOTOMY Sophie Taylor, Medical Student, KUSoM SUMMARY

CASE REPORT

Radiographic investigation of the pelvis of a four-yearold showed a subluxed left hip. This appeared to be a complication of his cerebral palsy and a very rare genetic condition, resulting in short stature. This patient’s condition is called Microcephalic Osteodysplastic Primordial Dwarfism Type 1 (MOPD1). For management of this complication, the patient was initially fitted with a camp abduction brace. However, subsequent dislocation occurred upon its removal. Orthopaedic surgical intervention was therefore required. A varus de-rotation osteotomy was done to realign the femur into the acetabulum of the innominate bone. Due to the abnormally small size of the patient’s femoral bone, a consequence of his conditions, a metatarsal plate had to be used. Upon removal of the plate, the joint had successfully stabilised. However, it is likely, due to contractures, that dislocation may reoccur later in the patient’s life.

This case report follows the journey of Patient X from birth to aged four years, focusing on the treatments used and consider their benefits but also the limitations imposed by his genetic condition and co-morbidities and how they have affected his development, life and ability to be cared for. During his mother’s pregnancy, it was found that patient X displayed intrauterine growth retardation, a characteristic feature of his rare condition, MOPD1. This failure to thrive continued after birth. Now, at four years of age, Patient X weighs a mere four kilograms and will remain the size of a neonate. As can be seen in Figure 1, this is well below even the lowest percentile.

Fig. 1: WHO boys’ growth chart showing the percentiles for weight against age4

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It was discovered later that Patient X has a genetic condition known as proportional Microcephalic Osteodysplastic Primordial Dwarfism Type 1 (MOPD1). This condition is sometimes known as Taybi-Linder syndrome. It means that his growth is very limited, but his limbs and torso are in proportion with each other. This disease is the consequence of an autosomal recessive mutation in the RNU4ATAC gene.1 This gene codes for a U12-dependent minor spliceosome, involved in catalysing splicing after transcription.2 Identifying these mutations is critical for a definitive diagnosis. Typical manifestations of this disease include a flat nasal bridge, dry skin, lissencephaly and agenesis of the corpus collosum amongst other brain abnormalities.3 With a prevalence of less than one in a million it is extremely rare.4 In addition to MOPD1, Patient X has other disabling comorbidities such as bilateral cerebral palsy, hypertonic lower limbs and global development delay. As cerebral palsy and MOPD1 are both likely to cause lower limb spasticity and hip dysplasia, having both conditions

greatly exacerbates the patient’s risk of spasticity.5 He also has visual impairment due to bilateral optic disc coloboma (congenital malformation of the eye) and optic nerve compression due to the size of the skull being abnormally small. Moreover, he is on levetiracetam to control epilepsy. The combination of his impairments unfortunately leaves Patient X non-mobile, non-verbal and reduces his life expectancy to his early twenties. Due to the complexity of Patient X’s combined conditions and the specificity that his treatment and management requirements, he is closely monitored across many specialist departments within the University Hospitals of North Midlands NHS Trust (UHNM). In 2016 the patient’s mother brought him to the Orthopaedic clinic at UHNM complaining of difficulty when changing his undergarments. Physical examination identified that this was due to limited abduction in the patient’s left leg at the hip. Upon further investigation, a radiograph revealed subluxation of the left hip (Figure 2).

Fig. 2. Pelvic radiograph indicating left hip subluxation as highlighted by circle

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The initial treatment was a conservative, less invasive approach consisting of a camp abduction brace, which was fitted to fix the hip joint at ninety degrees of flexion and sixty degrees of abduction. During this time, the femoral head relocated successfully back into the acetabulum of the pelvis. These braces are usually recommended to be worn for 6-12 weeks.6 An example of the brace can be seen in Figure 3.

Fig. 4. Subsequent subluxation of the left hip upon removal of the abduction brace.

Fig. 3: Camp abduction brace.

Due to the rarity of this case, there are no previous reports of a camp abduction brace being fitted in patients with both MOPD1 and cerebral palsy. However, a prospective longitudinal cohort study has been performed in children under 18-monthsold with bilateral cerebral palsy, whereby Chailey postural management equipment was used to prevent subluxation.7 The study concluded that there was a positive correlation between equipment use and chance of not experiencing hip subluxation after 60 months.7 Another study showed that use of a Pavlik harness in children aged 0-6 months with developmental dysplasia of the hip had a success rate of more than 90% in patients that were previously positive for Barlowâ&#x20AC;&#x2122;s test.8,9 The importance of screening high risk new-borns using the proper physical examinations in order to detect such problems as early as possible for appropriate management is also highlighted.8

There is thus clear evidence for the benefits of this type of conservative management, particularly in congenital paediatric dysplastic conditions, and has been shown to help in preventing deformity and increasing muscle length.10 In Patient Xâ&#x20AC;&#x2122;s case, whilst it was worth trialling this non-invasive approach in order to avoid surgical complications such as infection and anaesthesia risk, his dysplasia was too severe for any true benefit. Upon removal of the brace, dislocation of the left femur reoccurred (Figure 4). Surgery was therefore required to help reduce pain, improve function and avoid any future osteoarthritis or full dislocation. A varus de-rotation osteotomy was undertaken to better align the femoral head within the acetabulum. This involves rotating the femur anteriorly and toward the midline. Additionally, a percutaneous adductor longus tenotomy was performed to further improve the restricted abduction. This means surgically cutting the adductor longus tendon through the skin without needing to make a full incision. A range of different sized plates were prepared for the surgery, as it was initially unclear which would be most suitable for the procedure, due to the femur being smaller and thinner than normal. During the operation, surgeons used tried a series of plates to see which was the most appropriate. Ultimately, a metatarsal plate was used instead of the typical femoral plate.

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The hip surgery was closed reduction as opposed to open because there was nothing blocking the acetabulum and thus, no need to perform an open reduction by going into the joint capsule. A straight lateral incision was made at the left thigh and the vastus lateralis and tensor fasciae latae muscles were reflected to reveal the femur and hip joint. The operation was radiologically-guided, including using an arthrogram to attach the three-hole locking plate to the proximal diaphysis of the femur. K wires (1.6 mm) were inserted into the femoral neck (Figure 5). The femur was de-rotated forwards and, at a fixed thirty-degree varus angle, toward the midline. The rotation and movement of the joint was assessed while the patient was still anaesthetised in the theatre.

A notable complication that arose during the surgery was the difficulty during the anaesthetising of Patient X. Due to Patient X having the physiology of a four-yearold boy but the anatomy of a neonate, it took ninety minutes to anesthetise him, longer than would be expected. Moreover, when questioned, the orthopaedic surgeon stated she found it a challenge working on an unusually anatomically small scale.

CONCLUDING NOTES Whilst holistically (regarding diagnosis, co-morbidities and treatment) this case was extremely rare, the required operation that was performed is somewhat more common as it is often done on patients with persistent dysplasia and congenital dislocation of the hip.11 Following the removal of the plate from Patient X, the femoral head successfully and stably relocated into the acetabulum of the innominate bone. However, as previously mentioned, Patient X is immobile and so his immobility combined with his skeletal dysplasia unfortunately increases the likelihood of further dislocation later in life. It is possible that the psoas tendon could tighten, forcing the head of the femur out of its acetabulum once again. In which case, another operation may be required, bringing new risks and challenges for the patient and his family.

Fig. 5. Completed surgery showing successful insertion of metatarsal plate and locking screws.

A neonatal Spica cast was used post-operatively to help fix the femur at ninety degrees of flexion and forty degrees of abduction.

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For this reason, frequent imaging and physiotherapy should be undertaken to monitor the anatomy and range of movement at the hip. The current management plan for Patient X is to have semi-annual reviews. This will ensure that the patientâ&#x20AC;&#x2122;s quality of life and independence can be maximised and also that his pain can be reduced. In turn, this will hopefully ease the care work for the mother, especially with her being the sole carer for Patient X, it is important for her own wellbeing too.

ACKNOWLEDGEMENTS

resources/uk-who-growth-charts-2-18-years

Ms Belen Carsi. Consultant Orthopaedic paediatrician, 5. Cornell MS The hip in cerebral palsy Dev Med Child Neurol (1995) Vol 37 pp3–18 Royal Stoke University Hospital Melissa Bowerman, Ph.D. Lecturer in Bioscience, 6. Hip Abduction Brace - Infant Hip Dysplasia | International Hip Dysplasia Institute [Internet] School of Medicine, Keele University Available from: httpspp//hipdysplasiaorg/ developmental-dysplasia-of-the-hip/childADDRESS FOR CORRESPONDENCE treatment-methods/hip-abduction-brace/ Sophie Taylor 7. Pountney TE, Mandy A, Green E, Gard PR Hip C/o The Editor subluxation and dislocation in cerebral palsy - a Midlands Medicine prospective study on the effectiveness of postural North Staffordshire Medical Institute management programmes Physiother Res Int J Res Hartshill Road Clin Phys Ther 2009 Jun;14(2)pp116–27 Hartshill Stoke-on-Trent 8. Roof AC, Jinguji TM and White KK ST4 7NY Musculoskeletal screeningpp developmental dysplasia of the hip Pediatr Ann (2013) Vol 42 pp229–35 REFERENCES 1. Ferrell S, Johnson A and Pearson W Microcephalic osteodysplastic primordial dwarfism type 1 BMJ Case Reports (2016) Jun 16;2016. bcr2016215502

9. Barlow TG Early diagnosis and treatment of congenital dislocation of the hip Proc R Soc Med (1963) Vol 56 pp804–6

2. Shelihan I, Ehresmann S, Magnani C, Forzano F, Baldo C et al Lowry-Wood syndromepp further evidence of association with RNU4ATAC, and correlation between genotype and phenotype Hum Genet 2018 Dec;137(11–12)pp905–9

10. Picciolini O, Albisetti W, Cozzaglio M, Spreafico F, Mosca F and Gasparroni V “Postural Management” to prevent hip dislocation in children with cerebral palsy Hip Int J Clin Exp Res Hip Pathol Ther 2009 Mar;19 Suppl 6ppS56-62

3. Putoux A, Alqahtani A, Pinson L, Paulussen ADC, Michel J, Besson A et al Refining the phenotypical and mutational spectrum of Taybi-Linder syndrome Clin Genet (2016) Vol 90 pp550–5

11. Kasser JR, Bowen JR and MacEwen GD Varus derotation osteotomy in the treatment of persistent dysplasia in congenital dislocation of the hip J Bone Joint Surg Am (1985) Vol 67 pp195–202

4. Figure 1. UK-WHO growth charts - 2-18 years Available from: https://www.rcpch.ac.uk/

Midlands Medicine

CASE REPORT AND RETROSPECTIVE ANALYSIS OF MEDIAL PATELLO-FEMORAL LIGAMENT INJURY, TREATMENT AND MANAGEMENT Taran Chaudhuri, Medical Student, KUSoM SUMMARY A 28-year-old female, Ms Y, called 999, having dislocated her right patella. Paramedics repositioned the patella, but Ms Y remained in pain and was brought to A&E. A radiograph was taken, and she was sent home with conservative management. The following day, Ms Y was assessed by the orthopaedic registrar who noted trauma to the knee, but no notable fractures on the radiograph. He therefore requested an MRI scan

of her knee, and scheduled physiotherapy. One month later, Ms Y returned after three more dislocations. The orthopaedic consultant described a ruptured medial patellofemoral ligament, and discussed the possibility of surgery with Ms Y. After assessing the risks and benefits of the procedure, Ms Y decided to go through with the surgery, which was carried out six months later. Follow up appointments with both her GP and the surgeon were booked to review Ms Yâ&#x20AC;&#x2122;s health.

Fig. 1: (A) Diagrammatic anterior view of the right knee joint, demonstrating presence of musculature, cartilaginous tissue (both meniscal and hyaline), and structural ligaments. (Adapted and modified from Hoffman, 20172); (B) Diagrammatic medial view of the knee, with medial patellofemoral ligament arising from medial femoral condyle and inserting into superomedial surface of the patella. (Adapted and modified from Elrashidy et al, 20163)

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BACKGROUND The knee is a complex hinge joint, comprising numerous muscular, cartilaginous and ligamentous tissues, which are responsible for its structural integrity (Figure 1A). Injuries of the knee are common, with Bollen reporting that approximately 500 individuals in a population of 400,000 experience “significant knee injuries” per annum, equating to just over 1/1000.1 The case presented describes the rupture of the medial patellofemoral ligament (MPFL), the subsequent lateral patellofemoral dislocation (LPD), and the surgery performed (MPFL reconstruction) to re-establish stability of the joint. The MPFL originates from the surface of the medial femoral condyle and inserts into the superomedial surface of the patella. Its main function is to keep the patella within the trochlear groove of the femur during flexion and extension of the knee.4 LPD tends to occur between the ages of 20-40 years and can be predisposed to by several factors. These include any previous injuries causing patellar instability, an increased Q angle, and other various anatomical factors such as patella alta or patella-femoral dysplasia. Patellar instability is defined as the loss of the MPFLs ability to guide the patella into the trochlear groove when displacing force is applied to the joint.4 These forces may be due to sporting activities, such as rugby, football or dancing, where change in position of the knee occurs at high intensity; or could be applied simply from falling and placing one’s foot in the wrong position, resulting in overwhelming lateral forces on the patella, causing LPD. The Q angle is an important clinical marker of patellar instability and is calculated by drawing a straight line from the anterior superior iliac spine (ASIS) to the mid-patella, and then drawing a straight line up from the tibial tubercle. The acute angle this creates is typically 10-20°, with men at the lower end, and women at the higher end; this is thought to be due women’s naturally wider hips, tendency to have a relatively shorter femur length, and the relation of the two.6,7

Fig. 2 The Q angle: A straight line is drawn from the ASIS to the mid-patella, and another is drawn through the midline of the tibial tubercle. The angle created is measured. (Adapted and modified from Pagare et al, 20195).

CASE REPORT A 28-year-old female, Ms Y, called 999 after stepping off her landing and misplacing her foot, thereby dislocating her patella. Paramedics were able to relocate the kneecap but brought Ms Y to A&E as there was persistent pain and visible contusion of the medial knee. Entonox was given to manage the pain, a plain radiograph was requested. Ms Y was sent home with crutches and a cricket pad splint. Ms Y attended the trauma and orthopaedic clinic the next day where a past medical history was taken: she described three previous LPDs (all in her teens) with the first dislocation occurring at the age of 14. She was found to have lax joints (hypermobility) with a Beighton Score of 8/9.8 Pain, bruising and effusion of the knee were noted again upon examination. The radiographs showed no signs of a fracture and a normal Q angle, but

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4), and a disrupted MPFL, with chondral damage due to the recurrent dislocations. When discussing potential management options (continuing physiotherapy, caution and supportive splints or strapping, or opting for surgery), Ms Y stated that the recurrent dislocations were causing her a substantial problem with her daily life, as well as looking after her children and mother. The following month, Ms Y returned to clinic having Although unemployed at the time of consultation, she suffered three more dislocations, which she had expressed her wishes to start working soon and that managed to relocate herself. Her MRI scans showed the cumulative problems with her knee were enough to mild patella alta (high riding patella), as well as a laterally consider operative intervention. With this in mind, the sitting patella and contusion of both the medial patella surgeon recommended an MPFL reconstruction to Ms and lateral femoral condyle (Figure 3). The surgeon Y and chose the MPFL reconstruction surgery. described a normal TT-TG distance (10.7mm) (Figure an MRI scan was requested. The orthopaedic registrar also referred Ms Y for physiotherapy to gradually wean her out of the cricket pad splint and strengthen the vastus medialis oblique muscles. A follow-up with the Consultant Orthopaedic Surgeon was booked to review the MRI scan and discuss further management.

Fig. 3: MRI scan of Ms Yâ&#x20AC;&#x2122;s right knee showing ruptured MPFL and significant contusion of the medial patella and lateral femoral condyle (Consent to use MRI image was acquired from the patient.)

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Six months later Ms Y attended a pre-operative clinic and spoke with the orthopaedic Consultant who would be performing the surgery. The surgeon examined Ms Y for mobility of the knee, confirming the collateral, anterior and posterior cruciate ligaments were all intact, although patella-femoral irritability was noted. The surgeon explained to Ms Y the damaging effect of recurrent dislocations, and why he thought surgery was appropriate despite potential secondary degenerative changes to the joint. He also discussed the various nonsurgical management options that were possible, but Ms Y was keen to have her patella stabilised and decided to proceed with the MPFL reconstruction.

DISCUSSION LPDs are commonly due to sporting injuries, such as Ms Yâ&#x20AC;&#x2122;s initial dancing injury at the age of 14, or sudden changes in movement at the knee, resulting in abnormal lateral force on the patella, which describes Ms Yâ&#x20AC;&#x2122;s presenting complaint.

The significant pain that Ms Y was in after these dislocations can be attributed to the degree of tissue damage caused, both to the ligament as it ruptured increasingly with each dislocation, as well as the bone bruising and cartilaginous damage caused from the direct impact of the medial patella into the lateral One week later, Ms Y underwent a right knee MPFL femoral condyle. Conservative management using reconstruction with gracilis tendon autograft from the a cricket pad splint and crutches were used for quick same leg (right side). Post-operatively, Ms Y was given and effective relief, but only temporarily to allow the a hinged knee brace (0-40) and told she could partially relevant scans (X-ray, MRI) to be done. bear weight on the right leg for four weeks, and then fully bear weight with full range of motion after that. Follow-up appointments were made with both the GP (for two weeks after surgery) to have her sutures removed, and with the surgeon for a review (for eight weeks after surgey).

Figure 4 MRI scans at the Trochlear Groove (TG) and the Tibial Tubercle (TT). (A) A line, perpendicular to the posterior intercondylar line, is drawn through the trochlear groove at its deepest part. (B) Another line is drawn perpendicular to the intercondylar line, through the tibial tubercle where the patella tendon inserts. The distance between these two lines is known as the TT-TG distance. A value <15 m = normal, 15-20mm = borderline, >20mm = abnormal. (Adapted and modified from LaPrade et al, 20149).

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The initial post A&E follow-up appointment allowed Ms Y to give a full and detailed history of her previous dislocations, as well as explore her concerns, mainly the problems in caring for her children and mother, as well as not being able to work. These are important social aspects of the history that add weight to the argument for surgery. If Ms Y was not able to cook or drive because of the strain on her knee, this would directly impact on her ability to care for her family. The pain had become persistent, whereas after previous dislocations it had not been, and that was also one of the reasons she wanted to have surgery. The assessment of hypermobility gave further evidence to Ms Y’s predisposition for LPD, with her Beighton score of 8/9 indicating lax joints (>4 = hypermobility likely). The radiograph taken in the A&E department was done to rule out any trauma to the underlying bone, and showed a small avulsion fragment, but a normal Q angle. A normal value here prompted further investigation as to why she might be suffering these recurrent dislocations. The referral for physiotherapy was also an important inclusion, as strengthening of the quadriceps muscles greatly improves patella tracking (alignment) and reduces dependency of and disruption caused by the cricket pad splint in daily life. Ms Y’s MRI scan showed a laterally subluxed patella, with injury to the MPFL, medial patellar facet and lateral femoral condyle (Figure 3), which correlated well with the history of recurrent dislocations. The reporting radiologist also noted mild patella alta and a very shallow trochlear groove, both of which predispose mal-tracking of the patella, and so were almost certainly influencing factors in Ms Y’s dislocations. During one consultation, the surgeon discussed options of conservative management, such as the splint she was using, or surgery with Ms Y. Although MPFL reconstruction was decided upon, the surgeon did offer a tibial tubercle osteotomy. The open and honest discussion of treatment and management options, as

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well as potential risks and complications of surgery is crucial in a mutualistic doctor-patient relationship; this allowed Ms Y to make a well balanced and informed decision. The surgeon explained to Ms Y that she might experience some anterior knee pain due to the repositioning of her normal cartilage into a more load bearing position. Alongside this, he explained the risks of bleeding, infection, scarring, blood clots, neurovascular injury, fracture to the patella, failure to resolve symptoms, pain, stiffness and swelling. Although it might seem like a long and daunting list to a patient, it was crucial that the surgeon explain these possible complications to Ms Y, as only then could she make a fully informed decision. Ms Y’s MPFL reconstruction involved the harvesting of the gracilis tendon from its insertion point at the pes anserine (at the tibial tubercle); the semi-tendinosus tendon is sometimes used instead. Two holes were drilled into the medial facet of the patella and one drilled into the medial femoral condyle, roughly between the adductor tubercle and the medial epicondyle; a tunnel, for the graft to pass, was made under the skin but over the joint capsule. The graft ends were fixed into the patellar holes, and the loop of the graft was passed through the tunnel and fixed into the femoral hole. The graft is designed to mimic the MPFL structurally and functionally. The management after the operation aimed to get Ms Y moving and weight bearing on her right knee as quickly as possible. In this case she was instructed to partially weight bear for the first four weeks, and then fully weight bear after four weeks. Early mobilisation is necessary to minimise the chances of any deep vein thrombosis developing, which could lead to pulmonary embolism. Another reason is to maintain muscle mass and joint health in the affected leg, specifically the quadriceps in this instance, as the strength of the muscle directly impacts on the stability of the patellofemoral joint; physiotherapy was scheduled for this reason.

ACKNOWLEDGEMENTS Mr Amit Patel, Consultant Orthopaedic Surgeon (Royal Stoke University Hospital), and Mr Sachin Kumar, Orthopaedic Specialty Registrar (Royal Stoke University Hospital), for helping me with this presentation.

3. Elrashidy H, Carney J, Khan N and Fithian D Schematic diagram of the medial knee [image] (2016) Available at: https://musculoskeletalkey. com/medial-patellofemoral-ligamentreconstruction-2/

I would like to thank Ms Y for her consent for the 4. Post W and Fithian D Patello-femoral Instability: A Consensus Statement From the AOSSM/PFF publishing of this case report Patellofemoral Instability Workshop Orthopaedic Journal of Sports Medicine (2018) doi org/10.1177/2325967117750352 ADDRESS FOR CORRESPONDENCE Taran Chaudhuri C/o The Editor Midlands Medicine North Staffordshire Medical Institute Hartshill Road Hartshill Stoke-on-Trent ST4 7NY

5. Pagare V, Hafeez A, Ritchie, L, Kasehagen B and Knott C 'Q' Angle Physiopedia (2019) www. physio-pedia.com/index.php?title=%27Q%27_ Angle&oldid=203078

REFERENCES

7. Horton M and Hall T Quadriceps Femoris Muscle Angle: Normal Values and Relationships with Gender and Selected Skeletal Measures Physical Therap (1989) Vol 69 pp897-901

1. Bollen S Epidemiology of knee injuries: diagnosis and triage British Journal of Sports Medicine (2000) Vol 34 pp227-8 2. Hoffman M Knee (Human Anatomy): Images, Function, Ligaments, Muscles (2017) www.webmd. com/pain-management/knee-pain/picture-of-theknee#

6. Outerbridge R Further studies on the etiology of chondromalacia patellae The Journal of Bone and Joint Surgery (1964) British volume 46-B(2) pp179-190

8. www.physio-pedia.com/Beighton_score 9. LaPrade R, Cram T, James E and Rasmussen M Trochlear Dysplasia and the Role of Trochleoplasty Clinics in Sports Medicine (2014) Vol 33 pp531-45

Midlands Medicine

MORE MEDICAL CERAMICS Anthea Bond, retired Consultant Orthodontist This posset pot can be found in the section of the Potteries Museum and Art Gallery showing tin-glazed earthenware. It has a lid and two handles, there are two spouts arising lower down the pot for pouring the whey. It was made in Bristol in the mid-18th Century.

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Posset was a warm drink made of milk curdled with alcohol or lemon juice, often sweetened and spiced. It was first described in the 16th century when it was thought to be a cure for coughs and colds. Modern recipes for posset give a cold dessert made with cream, sugar and lemon.

NEWS NEW FACILITIES AT NSMI A significant benefaction by Jeremy Wade has enabled plans to be drawn up with confidence of fruition for a grand refurbishment of the lecture theatre facilities at NSMI. Plans are firming up, and it may be that work can start over the Summer months. More news on this is bound to follow. THE ORGAN DONOR REGISTER IS CHANGING Daily people die in the UK, who could have benefited from a transplant because there aren’t enough organ donors. As things stand in England, the Organ Donor Register is a register of those who have expressed a wish to make their organs (and tissue) available for donation if they die in such a way that makes this possible. To help increase the number of transplants, the Government has announced plans for a presumed, or deemed, consent system of organ donation to take effect from Spring 2020. Under the system, you would be a donor unless you register a decision not to donate, so the Organ Donor Register becomes a register of those who not wish to donate their organs after death and those not on that register will be deemed to be at best supportive, and at least not against the idea of donating their organs after death. Families will still be able to object to donation, but their objections will come under greater scrutiny in the absence of a registered objection to organ donation. This increased willingness to examine families’ objections is thought likely to lead to a modest increase in donors but since

one donor can save or transform several lives through organ donation, and even more lives by donating tissue, this multiplier effect means that a significantly larger number of donations is likely to result. What does this mean for you? UK Blood and Transplant would like organ donation to be a topic that is in the public arena, discussed in popular conversation and in families and couples so that if the tragic time comes when you might be in a position to donate, your family are not racked by a need for soul searching but already know what your thoughts were. NEW SCULPTURAL ART HOSPITAL UNVEILLED

ROYAL

STOKE

A new display of artwork celebrating the history of the Potteries has been installed at Royal Stoke thanks to UHNM Charity Funding. Five steel discs depicting the region’s traditional mining, steel-working, canal digging, pottery and healthcare industries have been erected at the entrance to the hospital’s new multimillion pound modular wards. The 8ft artworks, made from 8mm-thick mild steel were designed by renowned local artist Andrew Edwards and made in Chesterton by training provider PM Training*. *Project Management (Staffordshire) Limited, Kingsley, The Brampton, Newcastle-under-Lyme, Staffordshire, ST5 0QW.

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QUIZ NIGHT Oluseyi Ogunmekan, General Practitioner, Furlong Medical Centre, Stoke-on-Trent 1. Which of the following is/are a recognized cause of burning mouth syndrome?

6. MELD score is used to estimate relative disease severity of which of the following?

a) diabetes mellitus c) Low vitamin B12 e) all of the above

a) Glomerulonephritis b) Interstitial lung disease c) Liver cirrhosis d) Measles e) Psoriasis

b) Sjogren's syndrome d) oral candidiasis f) None of the above

2. All babies under 1 year of age should have a daily 8.5 to 10 microgram vitamin D supplement

7. For which of the following conditions is PEST a validated screening tool? a) pulmonary fibrosis c) pulmonary embolism e) cardiac failure

True or False?

b) psoriatic arthritis d) parasite infestation

3. All pregnant and breastfeeding women should take a vitamin D supplement of 10 micrograms (400units) daily

8. Proton pump inhibitors are a common cause of hypomagnesaemia

True or False?

4. What section of the Mental Health Act gives the police the power to remove a person from a public place â&#x20AC;&#x153;to a place of safetyâ&#x20AC;? when they appear to be suffering from a mental disorder?

9. Which of the following is NOT a cause of a high urine osmolality?

a) 5(e) d) 136

b) 17 e) 174

c) 117(Annexe 2)

5. Boerhaave's syndrome is spontaneous perforation of the oesophagus most commonly caused by straining or vomiting

a) acute kidney injury b) congestive cardiac failure c) dehydration d) excessive fluid intake e) high glucose 10. Rhesus anti-D immunoglobulin should be given to rhesus negative women in all cases of threatened miscarriage with a viable fetus and cessation of bleeding before 12 weeks gestation.

True or False? True or False?

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11. Who has a clinical frailty score named after him? a) Rappaport d) Rothwell

b) Rockall e) Routlidge

c) Rockwood

12. Which of the following drugs may be used in the management of gastroparesis ? a) domperidone c) metoclopramide e) None of the above

e) Stop warfarin, give vitamin K1 and give fresh frozen plasma both intravenously 15. In Acute Kidney Injury (AKI) which of the following statements is false?

b) erythromycin d) All of the above

13. In which of the following conditions is the STOP BANG score is used? a) Acute Kidney Injury b) Intussusception c) Obstructive Sleep Apnoea d) Subarachnoid haemorrhage e) Trauma Severity assessment

a) Stage 1 is diagnosed when there is a rise in creatinine of 50-90% from the baseline within 7 days b) Stage 3 is diagnosed when there is a rise in creatinine of 200% or more from the baseline within 7 days c) All patients with Stage 1 require hospital admission d) Stage 2 is diagnosed when there is a rise in creatinine of 100-199% from the baseline within 7 days e) Patients recovering from AKI Stage 3 should have a urinary albumin/creatinine ratio checked about 3 months after hospital discharge 16.Which of the following patients should not receive pneumococcal vaccination according to the Green Book? Patients with: a) Homozygous sickle cell disease b) Severe asthma c) Diet controlled type 2 diabetes mellitus d) Cochlear implants e) Nephrotic syndrome f) Kidney transplants

14. How do you manage a patient taking warfarin with an INR of 8.2 who is not bleeding? a) Stop warfarin and monitor b) Stop warfarin and give intravenous vitamin K1 c) Stop warfarin and give the intravenous preparation of vitamin K1 orally d) Stop warfarin, give vitamin K by injection and give dried prothrombin complex Factors II, VII, IX and X

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WORDPLAY 18 Here are some names which suggest characteristics which those so called may live up to aptronyms), live Here are a few observations on names just to get you into down to (antaptronyms) or hope any comparison this 18th outing of the Wordplay series. Surnames were between them and their name is just avoided: Joy, Large, originally derived at the earliest from characteristics, Small, Hope, Cope, Goodheart, Young (true for a while later certainly from place or occupation. Surnames but hopefully progressively less so), Lively, Longfellow, soon became fixed and for centuries in Britain we have Short, Brighthead, Thick and Nicholas. handed them on through a traditionally patronymic system (conferatur Iceland). To this day many local place names also serve as surnames. Examples are Alsager, PREGNANCY IS SO FULL OF POTENTIAL Crewe (also Crew or Croux), Leighton, Newcastle, Stoke, Sandbach and Twemlow. Occupational examples Obviously. And possible portent and certainly include Barber, Butcher, Clark, Smith, Tailor, and preoccupation. But here the reference is to the word and not the state. Weaver. NOMINAL DETERMINISM, POSSIBLY MAYBE

Nominative determinism is quite the reverse of surnames being given to people as a result of their business, craft or trade rather it is the hypothesis that people might get drawn to areas of work that suit their names. It The term was first used in the magazine New Scientist in 1994, after the magazine's humorous Feedback column noted several studies carried out by researchers with remarkably fitting surnames. These included a book on polar explorations by Daniel Snowman and an article on urology by researchers named Splatt and Weedon.1 One may come across solid, or possible all around. For example, I am sad, for the purposes of this discussion, that professor Roger Kneebone, who directs the Imperial College Centre for Engagement and Simulation Science (ICCESS), based within the Division Surgery on the Chelsea & Westminster campus, is principally a medical educator and no longer a trauma surgeon. A related concept is that of the aptronym2 which notes that a (sur)name works particularly well with someone’s state, situation, job, personality or whatever without speculating on any determinism at play.

Though outside the body you can freeze an embryo, one thing not possible to do, is to pause a pregnancy. (Owning an embryo is not the same as being pregnant which has the dictionary definition: (of a woman or female animal) having a child or young developing in the uterus. Though paused pregnancies may not exist, pregnant pauses do. There are two schools of thought on pregnant pauses: One that it is used to build up emphasis in a monologue or dialogue, the emphasis coming from active anticipation, or expectancy, of what is about to be stated or revealed, and a particular use is in comedy, just before the delivery of a punchline. In this school of thought, the distinction is between a pause used to allow time for the audience to reflect versus a pause used to cause anticipation of what is about to be declared; the second school relates to the heavy belly of pregnancy, the weight gained, the gravity of the situation: a pregnant pause is one burgeoning with significance, staggering under the weight of its own importance. In this view, the pause both gives rise to and lends weight to what follows. Legitimately, the thought goes, the pause itself may be considered gestatory and finally, when the words awaited follow, those words matter, they change things. The pause not only causes anticipation but suggests and lends weight to what follows.

So far as I know, to date there doesn’t exist a word that serves as an antonym to aptronym. I have a suggestion which might serve: antaptronym. (You might think contronym but that is already taken to signify words which may be used as their own opposites such as cleave “Ontogeny recapitulates phylogeny" is a catchy or oversight.) phrase coined by Ernst Haeckel, a 19th century German biologist and philosopher to mean that the development of an organism (ontogeny) expresses all Volume 29, No 1, May 2019

the intermediate forms of its ancestors throughout evolution (phylogeny), so time lapse photography of the development of a chick embryo would show, the theory goes, all the evolutionary steps from first vertebrates to birds. There is something in that, but not everything; a sketch perhaps rather than a picture. Pregnancy has given birth to a lot of language. In English we have very many informal ways of referring to the state: in the family way, expecting, eating for two, up the duff, duffed, a bun in the oven, gravid, with child, carrying, preggars are a few examples. We might use slightly different language when referring to non-human animals, such as the phrase: carrying young. Foreign languages are also instructive: Spanish: embarasada; Italian: incinta (swollen); Irish: ag iompar (carrying [a child]); German: schwanger (pregnant); Hungarian: terhes (onerous, encumbered), állapotos (pregnant), hasas (paunchy) vemhes (with young). LOST IN TRANSLATION AND MISHEARD LYRICS Alan Ayckbourn once said “I love the English language: people can misunderstand each other for ever.” I have no idea what he meant but perhaps clues lie in phrases such as “If I said you had a beautiful body, would you hold it against me?” (Bellamy Brothers), either a shy compliment or a carnal invitation; “We need concrete policies.” either a soviet command economy’s diktat on construction resources or a western politician’s metaphorical imploring; “He’s academic”, either praising the high intellect of an individual or condemning him as irrelevant. Audiotyping is a skilled task, sometimes given to a machine to do. A machine may apply voice recognition software and artificial intelligence (AI), but may not apply artificial general intelligence; it may not be able to sense check. Whether a human or machine is performing, just like misheard song lyrics or Chinese whispers, what goes in is not always what comes out. Here are a few examples: Basel bolus; necrotic syndrome; diuretic nephropathy (some people might wish to coin this term and others argue strenuously against its existence, but all along diabetic nephropathy

was intended. After all, common things are common.); “He continues to require a huge amount of vesus plesus to maintain his blood pressure.” (resusc’ measures); “On examination today a pulse at almas was felt in the left paraumbilical region.” (pulsatile mass); drug alerting stent (drug-eluting); modern aortic stenosis (moderate); bowel duct; Stencil (Stem cell). LET US DIGRAPH OUR STEELY GAZE British English is a beast of a language, easy to speak well enough to be understood, but nigh on impossible to master. Pronunciation doesn’t always follow sensible rules but seems rather to be a matter of habit. (consider scon[e] es the split digraph come into play for you?) You’ll probably be aware of the silent letters that are often found in English such as the initial k in knowledge or knight or the penultimate letter in debt. Or the final letter, usually, in damn, though it is recovered in damnation. Surgical examples include sign, knife, stitch, knot, scissors. Private practice might consider the silent h in charisma and the sonically redundant p in receipt. (The word receipt has a number of senses and a number of related roots and a deliberate re-insertion of a lost p as an attempt to recapitulate its etymology. These roots go back to two Latin words, re-[Latin, back] and capio [Latin, I take]. Not too distant in sound from recap meaning to go over again, short for recapitulate. Also related to receptacle and reception. These involve the verb to receive, which has lost the p. Well, the ‘p’ was put back by well-meaning philologists but the pronunciation stuck with receive thread rather than the receptacle one.) But perhaps you have not already considered the invisible letters that litter this language of British English. For example, there is an h in sugar that follows the initial s and makes the digraph sh, except for the fact that it doesn’t appear on the page. Then we have the y in news that follows the initial n to create the nye sound. Americans don’t bother, they speak as they find the letters so they pronounce news as noos [or nooze not noose](unlike mews or muse which both also have an invisible ‘y’ after the initial ‘m’ and should both really be, and from the mouths of Americans almost certainly are, more like what a cow does: moos). News as pronounced nooze by an American sounds

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very odd to a British ear (possibly less so north of the border?). But as written, working from only the letters on the page, they’re right. (How it galls me to say that!) Another digraph in English is ‘gn’, most commonly seen in words of Greek origin such as diagnosis, prognosis and so forth but it also occurs in gnash. In the words such as diagnosis the g and n are most usually pronounced very distinctly, with perhaps the tiniest of pauses twixt ‘em but if it were muted, you’d still be understood. In the gnash words, is the ‘g’ silent? Usually, almost and more so when speaking at speed, but if you if you placed a muted short joined hard ‘g’ at the beginning, you’d readily be understood and not sound silly. The word gnash (Middle English gnasten, Old Norse gnasta) means grinding teeth in rage, bemoaning or threat (and, by extension, informally, gnashers has come to mean both teeth and dentures). There is another word that describes a medical condition of teeth clenching and teeth grinding as a deleterious habit possibly as a response to stress, often when asleep: bruxism. This has an entirely different root from the Greek ebryxa meaning to gnash teeth.

Gimlet is not a letter, it is a word in which no silent nor invisible letters occur and each letter sticks pretty well to its basic sound. It is a tool not unlike an awl or bradawl. These are like pointed nails with a handle. They are used for making indentations or small holes in wood or similar materials whereas a gimlet is like a screw attached to a handle and is used for boring a small hole without splitting the wood. The point is that it is sharp-pointed. This helps to explain the phrase gimlet eye. One may have a gimlet eye or run a gimlet eye over a contract. It refers to a sharp or piercing gaze which all lend to a look an ability to cut which is felt keenly but which cannot have a physical counterpart. Eagle-eyed is a metaphor which is literalistic by comparison. Steel may be keen or dull. When it comes to appearances, a steely gaze is the latter, unimpressed and disapproving. ADDRESS FOR CORRESPONDENCE

Dr D de Takats Consultant Nephrologist The Kidney Unit Back to unpronounced letters. The word ophthalmology Royal Stoke University Hospital is often misspelt because people miss the conjunction of Newcastle Road two digraphs at the beginning of the word. For those Stoke-on-Trent who learn language by hearing rather than reading, ST4 6QG they will commonly write opthalmology because what they have often heard spoken is op-thalmology not off-thal-mology. Those who know the correct spelling FURTHER INFORMATION should take care to stress the correct pronunciation to help hearers to spell correctly. The etymology of the www.etymonline.com word is simple, it is from the Greek words οφθαλμοσ www.synonyms.com (ophthalmos = eye) where the second Greek letter, phi, becomes ph in Roman and the third letter, theta, becomes th + ology, so ‘study of the eye’. REFERENCES Gamma is the third letter of the Greek alphabet and 1. Nominative determinism entry in Wikipedia Gimel the third letter of the Hebrew alphabet. Lots of hard ‘g’s there. Once soft sounds g and hard c sounds 2. Aptronym entry in Wikipedia were apt to be confused. Rather than giving k all the hard c work, g was invented deliberately not spuriously (well, actually, a Roman called Spurius Cavilius Ruga did invent the letter g, so in the sense that Spurius invented g, it was spuriously invented).

Volume 29, No 1, May 2019

INTERESTING IMAGE

You’ve heard of the trigeminal nerve, the fifth cranial nerve, which splits into three divisions: Ophthalmic, Maxillary and Mandibular, and of the pain that can occur within their sensory distribution, trigeminal neuralgia. You may not have considered that the term trigeminal means three sets of twins referring to left and right Ophthalmic, Maxillary and Mandibular branches. But there is another use for the term trigeminal…

paired with an ectopic beat occurs repeatedly, the rhythm is referred to as bigeminal. Similarly, runs of three ectopic beats are called triplets, but when a pattern of three beats together, usually two sinus and then an associated premature ventricular beat followed by a ‘compensatory’ pause, then you have a trigeminal rhythm (see image).

Quadrigeminal rhythms are recognised, but not higher When two similar abnormal beats occur together on orders which are simply classified as occasional ectopic the ECG, they are termed a couplet, and if they occur beats, regardless of regularity. Such is the clarity and repeatedly, couplets, but if a pattern of a sinus beat the arbitrariness of convention and received wisdom.

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QUIZ ANSWERS & EXPLANATIONS 1. e) All of the above Burning mouth syndrome is a name given to discomfort or pain in the mouth. It often affects the tongue, lips and cheeks but other parts of the skin lining inside the mouth can also feel uncomfortable. Most people with the condition complain of a burning or scalded feeling. Burning mouth syndrome is a common condition. It often affects women, particularly after the menopause, but men can sometimes get it too. Up to one in three older women report noticing a burning sensation in their mouth. 2. False Children who have more than 500mls of infant formula a day do not need additional vitamin D as formula is already fortified 3. True These are recommendations from NICE and the SACN and endorsed by Public Health England 4. d) Section 136 If the police find you in a public place and you appear to have a mental disorder and are in need of immediate care or control, they can take you to a place of safety (usually a hospital or sometimes the police station) and detain you there under Section 136 of the 1983 Mental health Act. 5. True Sometimes referred to as “effort rupture of the oesophagus”. Boerhaave’s syndrome differs from Mallory-Weiss which is only a mucosal tear. 6. c) Liver cirrhosis MELD stands for Model for End-stage Liver Disease. It is not appropriate for patients under the age of 12 years. It can be used to estimate likely survival of patients awaiting liver transplantation 7. b) Psoriatic arthritis PEST stands for Psoriatic Epidemiology Screening Tool. It is recommended that patients with psoriasis who do not have a diagnosis of psoriatic arthritis complete an annual PEST questionnaire. A score of 3 or more indicates referral to rheumatology should be considered. 8. a) True Be very careful reading questions of this type. PPIs do not cause hypomagnesaemia commonly; Volume 29, No 1, May 2019

Hypomagnesaemia is uncommon. But amongst those cases found, PPIs are a well attested cause. Other causes include diuretics, especially loop diuretics, aminoglycosides and theophylline. 9. d) Excessive fluid intake (Just checking you’re awake!) 10. b) False It is rare for a woman to be sensitized after uterine bleeding in the first 12 weeks of pregnancy where the fetus is viable and the pregnancy continues. That said, this is a complex area and advice is always reasonably sought e.g. https://bnf.nice.org.uk/drug/anti-d-rh0immunoglobulin.html 11. c) Rockwood The scale is numbered is numbered from 1 to 9. Ray Rappaport was an American cell biologist. The Rockall score attempts to identify patients at risk of adverse outcome following acute upper gastrointestinal bleeding. The Rothwell score is used to determine the risk for stroke in the days following a transient ischemic attack. 12. d) All of the above 13. c) Obstructive sleep apnoea STOP BANG is an acronym for Snoring Tired Observed Pressure BMI Age Neck and Gender. These are elements that determine likelihood of obstructive sleep apnoea 14. b) & c) Are both acceptable Giving Vitamin K1 using the IV preparation orally is an unlicensed use. If the INR is repeated after 24 hours is still high, the dose of the vitamin K can be repeated. Warfarin may be re-started when the INR is < 5.0. 15. c) AKI Stage 1 is very common during acute illness. Small rises in creatinine often improve in line with recovery from the underlying condition and competent clinical management. Routine referral to Nephrology is not necessary for people with AKI Stage 1. 16. c) Only those with diabetes mellitus requiring insulin or oral hypoglycaemic drugs are recommended to have immunisation against pneumococcal infection. 43

Original Paper Original Paper

Laszlo P Tabinor M Sherman SM, Estacio E, Nailer E, Cohen C, Taylor J & Redman CWE Mamas MA, Jordan K & Kadam U Dale TP, Osman W, Spiteri MA, Haris MF, El Haj AJ, Yang Y & Forsyth NR Cartlin V Ogunmekan O de Takats D Crews W Thornflesh I Gray C Alabbad B, Priest H, Hawkins C & Hunter SM AL-Shallawi A, Blana D & Pandyan A Aries A Begum S, Povey R, Gidlow C, Ellis N, Duval L & Riley V Behforootan S, Chatzistergos P, Chockalingam N & Naemi R

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