APPENDICITIS

Appendicitis in children and young people

Robert A Wheeler
Abstract
The incidence of appendicitis has its peak between 12 and 18 years of age. It is one of the commonest, and familiar, emergency surgical conditions in this age group, but it appears to be difcult to diagnose, since it is one of the most frequent causes of litigation on the basis of missed diagnosis. This paper explores why appendicitis should pose such difculties to clinicians.

Introduction
Appendicitis is sufciently common for most people to have encountered it; either by suffering it personally, or by knowing of someone who has. For a ubiquitous disease, surprisingly, appendicitis is often diagnosed only after a delay of days or weeks of ill-health. For children and young people who suffer this variety of presentation, the illness can be serious, and sometimes fatal. This begs the question as to how can a common disease fail to be identied in a timely way? Is a delay in diagnosis indicative of substandard clinical care? From the national perspective, this is demonstrably a question that is frequently posed. Appendicitis ranks second (only superseded by meningitis) in the allegations of diagnostic errors relating to paediatric malpractice in the United States1; where failure to diagnose appendicitis resulted in 39% of all claims2. In France, appendicitis and testicular torsion were equally ranked, as the commonest surgical misdiagnoses3.

Anatomy and pathophysiology

The appendix usually lies in the right iliac fossa, since its base is attached to the caecum, and the caecum occupies this site. In 65% of 10,000 subjects4, the appendix lay posterior to either the caecum or the ileocaecal junction. A further 30% lie in the pelvis, and only a few percent sit either anterior to or below the caecum. This distribution, based originally on post mortem studies, has been partially validated by laparoscopic appendicetomy, which enables the operator to view the appendix site in detail; a luxury not available to the open surgeon, who uses a blind nger to mobilise the appendix, and thus cannot necessarily identify the original appendicular site. The appendix is between 2-20 cm long, mean length 9 cm. The effect of these measurements is that the appendicular tip is usually located outside the right iliac fossa,

Robert A Wheeler, Consultant Paediatric Surgeon, Southampton University Hospitals Trust, Tremona Road, Southampton, SO16 6YD, UK Email: robert.wheeler@suht.swest.nhs.uk

either lying in the right mid zone of the abdomen or in the pelvis. Furthermore, the appendix is usually separated from the examining hand not only by the thickness of the abdominal wall, but also by the caecum. The caecum is often full of gas, thus acting as a poor transmitter of the pressure applied by the hand on the abdominal wall. In patients with a pelvic appendix, the organ is largely inaccessible to the examining hand on the abdominal wall. Inammation of the appendix probably occurs as a consequence of luminal blockage. This may occur due to the swelling of the lymphoid tissue that is incorporated in the appendix wall, or intraluminal obstruction with faecal matter, which may contain concretions (forming a faecolith). The appendix distal to the blockage becomes full of the mucus which is continually secreted from the mucosal lining, since this is now unable to drain into the colon. The pressure in the distended segment begins to rise as more uid accumulates, and consequentially, its wall begins to distend. This distension stretches the muscle coat, which in turn causes pain, increasing incrementally with the rising pressure. The elevated pressure also impinges on the blood owing into and out of the appendix. Initially, this affects the low pressure venous return, and the appendix becomes engorged with venous blood that cannot escape. This has the effect of raising the pressure within the distended segment still further, and eventually, this exceeds the arterial inow pressure, resulting in ischaemia. This is followed by perforation of the appendix secondary to ischaemic necrosis, and usually occurs within 2-4 days from the start of the illness. In young children, the natural history is quicker, and perforation may occur within 48 hours of presentation. Perforation occurs in 20-40% of children who have a nal diagnosis of appendicitis; the perforation rate in the under 5s is 80%; in the under 1 year olds, almost universal, perhaps due to their inability to report their symptoms. Perforation is rarely associated with macroscopic faecal soiling. Instead, the distension and ischaemia cause the serosa of the appendix to become inamed. This, in turn, leads to secretion of inammatory uid into the tissues surrounding the appendix. In some patients this may become walled off as a peri-appendicular collection, conned to the
DOI: 10.1258/cr.2011.011a18

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area of the appendix. In others, the uid may be widespread, collecting in the pelvis, or spreading to the four quadrants of the abdomen. It seems likely that the original site of the appendix has some part to play in determining whether the distribution is localised or widespread. However, the most signicant determinant is age. Children of less than 2 years are more likely to have diffuse peritonitis, possibly because of their as yet undeveloped omentum, an anatomical structure playing an important part in the localisation of inammation within the abdominal cavity. The ischaemia has the effect of reducing the viability of the appendix wall, hours or days before perforation occurs. This allows bacteria to cross the wall from the lumen to the appendicular serosa, a process known as bacterial translocation. Once translocated onto the serosa, the bacteria are able rapidly to colonise the inammatory exudate, which is an ideal medium for bacterial proliferation. This rapidly turns the inammatory exudate into pus; in some patients, the formation of the exudate and the pus is effectively synchronous. Pus in contact with the lining of the abdominal cavity, induces a generalised inammatory reaction, manifesting as the clinical signs and symptoms of peritonitis. However, localised collections of pus, particularly in the pelvic cavity, may become walled off by adjacent tissues, preventing the clinical picture of peritonitis developing. These collections incorporate the pus, comprising an abscess component; together with the residual appendix; any faecolith that was contained within it, and bowel loops.

The atypical presentation

If the appendix tip (which is inevitably affected by proximal obstruction) is located in the retrocaecal or pelvic positions, the signs in the right iliac fossa may be absent, or take so long to develop that by the time they present, perforation and abscess formation has already occurred. During medical training thirty ve years ago, students were invariably taught that a rectal examination was compulsory to exclude appendicitis, if the diagnosis was in doubt. But this approach is now regarded as outdated, considered both undignied and invasive. In children, the procedure has been largely abandoned. Notwithstanding this, many surgeons believed the rectal examination to be effective in diagnosing pelvic appendicitis. Thus a patient (with a pelvic appendix) may present with diarrhoea or urinary symptoms days or weeks after some rather vague periumbilical abdominal pain and nausea, the history of which becomes detached from that of the presenting illness. The clinician will thus be faced with a patient who is apparently presenting with these new symptoms, and claims to have been previously well. It is unsurprising that such patients are investigated for gastroenteritis, colitis and urinary infection in the rst instance. Furthermore, bacterial and viral infections of the gut may be the cause of the appendicitis; but the discovery of these pathogens in the stool of a patient purportedly presenting with gastroenteritis or colitis is likely to reinforce that diagnosis, rather than guide the clinician away to an alternative pathology. Bacteria may translocate from an inamed pelvic appendix through the wall of the adjacent urinary bladder. If the patient is apparently presenting only with urinary symptoms, the presence of urinary bacteria and pus will only reinforce this diagnosis.

The classical presentation

Fifty percent of patients present with a classical presentation, learnt as part of basic medical training. The initial distension of the appendix causes a referred pain. Both the appendix and the periumbilical region are served by the same nerve roots, T8-10. Neural messages from the distended appendix are initially perceived as emanating from the periumbilical region, since the brain does not recognise the appendix as a source of pain. However, once the disease progresses to the stage where inammation spreads to the peritoneal covering of the appendicular surface, the source of the neural impulses are far better dened by the brain, and the patient will have localised tenderness in the right iliac fossa. As the illness progresses, rebound tenderness and guarding will develop, as the peritoneal inammation becomes established. At this point, the patients coughing and sudden movement will exacerbate the pain. If the collection is walled off, these signs will be localised. But if it is not, then generalised peritoneal signs will be present. In either case, systemic symptoms of anorexia and nausea are frequent, together with vomiting and fever. If the inammatory mass is adjacent to the bladder or bowel, urinary symptoms or diarrhoea may occur. An elevated temperature and heart rate are usual.

The clinical course, if diagnosed as the disease develops

In a patient presenting with the classical picture, it is likely that the rst doctor who reviews the patient will include appendicitis within her differential diagnosis. Notwithstanding this, the surgeon will be duty-bound to conrm the diagnosis before operating, and a period of assessment will usually be required to ensure that the history and physical signs are consistent with appendicitis. Further delay may be required for imaging, most usually an ultrasound examination, although some clinicians advocate a CT scan as the most effective investigation. In general, blood tests rarely change a decision to operate, or to wait and watch. Observation may have an important role in the diagnosis. In a patient with the classical presentation of peri umbilical pain moving to the right iliac fossa, with nausea and fever, the clinical examination may still be equivocal, the examiner unconvinced by colleagues ndings of tenderness or peritoneal irritation.
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Wheeler

The patient should be reviewed by a surgeon of sufcient experience to make the decision; but a period of 12-24 hours observation is likely to clarify whether the clinical picture is transient, or whether the signs will become established, and an appendicectomy will be warranted. Providing they are, an appendicectomy will be performed, either via an open incision in a skin crease in the right iliac fossa, or laparoscopically. At the time or writing, both methods are common. The surgery will ideally be performed in normal working hours, between 0800-2200 in the United Kingdom. If the patient is seriously unwell, with signs of peritonitis, this may be an indication to perform surgery in the middle of the night. But it is recognised that the risks associated with operating at this time are signicantly higher than those that pertain in the daytime; thus overnight surgery is usually restricted to saving life and limb. Surgical units vary as to their use of antibiotics for appendicitis. Usually, patients receive a single perioperative dose of antibiotics, which may then be converted into a 3-7 day course depending on the operative ndings. In patients who have a diagnosis of appendicitis where it is planned to defer surgery until the next day, preoperative antibiotics are sometimes administered. Hospital policies vary considerably, and no single postoperative antibiotic regimen has yet gained widespread acceptance. In some cases, it may be immediately clear from the history that the appendicitis has been present for many days. The physical examination reveals a mass arising from the pelvis, and this is conrmed on ultrasound as consistent with an appendix mass, often containing an abscess. Opinions still differ on the correct approach to this situation, but in childrens surgery, a conservative approach is usually adopted, since the risk of surgical intervention at this stage is high. Bowel loops may be damaged during the dissection of a mass; and infection (which is otherwise walled off within the mass) may unavoidably be spread widely by operation. For this reason, a prolonged course of intravenous and then oral antibiotics is employed to cure the patient, and is usually effective. If the clinical picture of pain, fever and anorexia fails to resolve, intervention may be required, but it should be avoided if possible. Traditionally, the remnants of the appendix have then been removed during an interval appendicectomy 6-8 weeks later, although there is evidence emerging that this may be unnecessary.

The clinical course if the diagnosis is not initially made

There is no doubt that evidence from litigation is a testament to the number of patients who fall into this group. The diagnosis may have been made by a GP , (and shared with the parents); only to be doubted by the hospital clinicians. Investigations, and possibly prevarications, follow. . .. And to everyones distress; the diagnosis is conrmed after a long delay, associated with harm that may well have been
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avoided, if only the general practitioner had been listened to in the rst place. Alternatively, a child is taken back and fro to the GP , who steadfastly diagnoses an illness requiring no intervention, despite the parents admonishment that this is appendicitis. An assertion that the clinicians in the hospital roundly endorse, having provided a desperately sick child with an emergency appendicetomy on the night of admission; and handed the parents a diagnostic label of missed appendicitis. It is accepted that these characterisations are crude, but they nevertheless represent recurring themes in the commonest cause for instruction in clinical surgical negligence in children. In either situation, there are undoubtedly instances when an elementary process of history-taking and physical examination, properly performed, would have clinched the diagnosis and resulted in a timely appendicetomy. However, from a surgeons perspective, it is inescapable that the diagnosis of appendicitis may be difcult. In patients where the diagnosis is suspected, but cannot be proved, the process is somewhat easier. Rightly, surgeons are reluctant to operate on a patient who may not need surgery, and both ultrasound and cross sectional imaging may assist with conrming the diagnosis, although will rarely exclude it. Faced with a patient in whom no diagnostic progress has been made after 24-36 hours of investigation, the surgical question is simple, if stark. Does the clinical risk of missing appendicitis exceed the risk of the operative intervention necessary to exclude it? The answer, almost invariably, will be that you need to have a look. The far more difcult situation is where the diagnosis is not initially considered, for the reasons given above. This is usually when the characteristic signs in the right iliac fossa are persistently absent, and the patient presents with the end results of the appendicitis, (such as a pelvic abscess) rather than the early stages of appendicular inammation. Days or weeks of investigation and observation have yielded either no diagnosis, or more usually, an alternative (but incorrect) diagnosis. Commonly, the patient is in hospital, managed by non-surgical teams. The anorexia and nausea has resulted in substantial weight loss, and nutrition is often augmented by intravenous (parenteral) feeding, which may have its own complications. The weight loss is usually associated both with muscle loss, and the lack of mobility that ows from this, so pressure sores are not unusual on dependent areas of the body. Prolonged courses of antibiotics may have controlled the persistent fever, but rarely cure it. However, these may have altered the bowels bacterial ora, and the patient may have longstanding diarrhoea due to this, in addition to that caused by a pelvic appendix pressing on (and irritating) the rectum. In addition to all this, the relationship between the patient, her parents and the clinicians are likely to be very strained, fuelled by the frustration of making no progress with the diagnosis, and continuing constitutional deterioration. This will do nothing to ameliorate the misery, and perhaps depression, of the child who is stuck in this situation.

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Eventually, the appendicitis is diagnosed when a second opinion is requested, offered or demanded. By this stage, the surgical problem is substantial. The option of long term antibiotics alone will have been limited by this stage, and the surgical alternative will be required, although very hazardous. Holes are likely to be made in the bowel during a very difcult dissection of friable oedematous bowel which has a consistency of blotting paper, so stulas between the bowel and skin can be anticipated. The appendix will be fragmented, and thus nigh on impossible to remove completely. A faecolith, commonly contained within an inamed appendix, may not be identiable. Secondary surgery during the patients admission may be necessary because of continuing infection, and the faecolith may be encountered and retrieved at this procedure. Up to 20% of children with a perforated appendicitis have been reported to develop a wound infection, although this is reducing to 3-6%, with postoperative abscess formation in only 1-3%. The wound may break down due to the patients poor nutrition, requiring either resuturing or the application of specialised dressings to manage the wound whilst it heals into a safe, but ugly, scar. The entire episode may be followed by adhesion obstruction, although it may be difcult to assess whether an enhanced risk of adhesions can be attributed to the perceived delay in diagnosis. Thankfully, most patients who suffer this ordeal recover, to be discharged from hospital. Nevertheless, it

seems likely that many bear the mental scars of this experience into their foreseeable futures. In summary, appendicitis in children is usually easy to diagnose. However, although this is a common disease, it may present without its common clinical features. It can be seen that some cases of missed appendicitis will represent substandard clinical care. But faced with a child with vomiting and diarrhoea, in the absence of any pain or tenderness in the right iliac fossa, gastroenteritis could be construed as a reasonable diagnosis, which does not merit further referral. This may go some way to explain why the diagnosis of appendicitis may take longer than is expected.

References
1 McAbee GN, Donn SM, Mendleson RA, et al. Medical diagnoses commonly associated with pediatric malpractice suits in the United States. Pediatrics 2008; 122 : e 1282 6 2 Selbst SM, Freidman MJ, Singh SB. Epidemiology and etiology of malpractice lawsuits involving children in US Emergency Departments and urgent care centers. Pediatric Emergency Care 2005; 21: 165 169 3 Najaf-Kadeh A, Dubos F , Pruvost I, et al. Epidemiology and aetiology of paediatric malpractice claims in France. Archives of Disease in Childhood 2011; 96: 127130 4 Wakeley CP . The position of the vermiform appendix as ascertained by the analysis of 10,000 cases Journal of Anatomy 1933;67:277

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