- Email: [email protected]
The Child with Recurrent Abdominal Pain
The Child with Recurrent Abdominal Pain JOHN APLEY, M.D.
PAIN IN DIAGNOSIS
To most of us pain is no stranger, and pain is what brings many children to the physician. Yet so much about pain is unknown and unexplained. It cannot be measured. It can hardly be described except by analogy-children have spoken of abdominal pain as an ache, or colicky, or ''brown,'' or "my tummy feels pale," '1ike a lump of food," "sharp as knives," or "a headache in my tummy." An identical stimulus produces pain which does not seem identical in different people, or in the same person at different times and in different moods. Pain is associated with varying reactions, which can be physical (e.g. pallor) or emotional (e.g. irritability). It may respond differently in different people to the same drugs. Feeling pain may become almost a habit or a way of life. When a child keeps complaining of abdominal pain, when the customary examinations and investigations fail to reveal the cause, when drugs and diets have no real effect, the patient and his parents may lose confidence in their physician and the baffied physician lose confidence in himself. In these circumstances one might sympathise if he is tempted to call the patient "a little bellyacher," and to resort to cliches, which are unconvincing and often untrue, like "There's nothing wrong," and "He will grow out of it" (see p. 69). But the correct diagnosis can nearly always be made quite simply, and rational treatment based on it usually leads to a gratifying response. With a suitable comprehensive approach the clinical exercise in diagnosis and treatment of recurrent abdominal pain becomes one of the most fascinating in medicine. It is also one of the most rewarding, for recurrent abdominal pain is among the commonest disturbances of childhood. 'i> " In a survey of unselected school children 2 it was found that one in nine had had at least three attacks of recurrent abdominal pain, severe enough to affect their activities, over a period longer than three months. The frequency may vary in different parts of the world, but my impression from discussions with colleagues in many countries is that it might tum out to be proportional to each country's level of sophisticationl
63
64
JOHN ApLEY
In attempting to make a precise diagnosis, how much can be learnt from the presenting symptom, pain? In a series of children with recurrent abdominal pain I have analysed the characteristics of the pain: 1 what it feels like, its site, severity, time of occurrence, duration, frequency and periodicity. The common associations of attacks of pain were also scrutinised: they were vomiting, pain in the head or limbs, elevated temperature, sleepiness after attacks, pallor. In most cases the features described are characteristic of visceral pain, 5 but for differential diagnosis this is not enough. It is important to appreciate that the pain is not an isolated occurrence: it is part of a pattern of reaction. So often we are asked to see a child who complains, as he has done before, of abdominal pain, and who points to the umbilicus as the place where it hurts. He may have vomited. His temperature may be slightly elevated. Usually he looks pale, and on examination there is vague abdominal tenderness. In a few hours he has recovered. In each child there tends to be a "pattern" of attacks, with slight variations over a limited period of time; but over a period of years there may be pronounced changes of pattern. The child who is "a little bellyacher" is likely to have been a vomiter in infancy and to suffer from headaches, migraine and other disturbances when he becomes an adult (see p. 69). If we limit our enquiries to the main presenting symptom, pain alone, we usually get nowhere. It is essential to extend them further than the symptom or the abdomen, and further than the present attack. Pain is an experience in which the whole child is concerned. The diagnostic approach must be broadened to include the whole child as he is-during and between attacks-and as he was before the attacks commenced. To enter and explore the child's world we need a key; when he complains of pain he may be asking for help and offering us the key. (>
RECURRENT ABDOMINAL PAIN WITH ORGANIC DISEASES
"Organic or non-organic?" The question is over-simplified, for organic symptoms have an emotional component and emotional symptoms have an organic component. The whole patient is involved. But the practising physician is rightly concerned not to overlook an organic disorder which he hopes may be quickly cured and which, if undiagnosed, might prove lethal. So, in practice, the physician who considers organic disorders first, if only to exclude them, is prudent, so long as this approach does not limit his perspective or warp his medical philosophy . ., In the most severe pains, with the child screaming and writhing in agony, no organic cause could be found.
THE CHILD WITH RECURRENT ABDOMINAL PAIN
65
It has been generally agreed in several large surveys that in children with abdominal pain which has recurred over a period of months or years, an organic cause can be demonstrated in only a small proportion. Since the practice of medicine is largely based on probabilities, it is important to appreciate how small this proportion is. In the first 100 cases which were extensively studied for research purposes in Bristoll an organic cause was demonstrated in eight. In the next 100 cases, similarly studied, six causative organic causes were found. In several hundred cases seen since, in which, as a result of the lessons learned, investigations were deliberately restricted, the proportion with an organic cause has been of the same order. Doubtful and unacceptable causes will be considered shortly, and the overwhelmingly more common cases with no organic disorder can then be considered; but an undisputed organic cause for recurrent abdominal pain is to be found in little more than one case of every 20. Organic Causes Among these no single disorder is common, and most are extremely rare. Renal tract causes together are roughly equal in number to gastro-intestinal causes. Extra-abdominal causes are extremely uncommon as a cause of pain over an extended period of time. They include spinal disease (e.g. tuberculous), intra-cranial neoplasm, hyperthyroidism and hypoglycaemia. Porphyria may present with pain over a period of years, and I suspect that some other rare metabolic disturbances may come to be added to the list. Castro-intestinal tract causes include partial malrotation or duplication of intestine, recurrent intussusception, regional ileitis, tumours, Meckel's diverticulum and Chilaiditi's syndrome, but all these are rare. Peptic ulcer is less uncommon, but even so, with strict criteria for diagnosis, I assess its frequency as about 1 per cent of all cases with recurrent abdominal pain. The usual radiological findings, variable changes in peristalsis and altered mucosal patterns with an "irritable stomach,"3 are liable to be misinterpreted. I mention here excessive air-swallowing, which I find is occasionally a cause of recurrent abdominal distension and pain in children, long after infancy, and is associated with severe emotional disturbances. Other intra-abdominal causes include Henoch-Schoenlein purpura, sickle cell anaemia, lead poisoning, splenomegaly, haemochromatosis, cirrhosis of liver, hepatomegaly with heart failure, cholecystitis, calcification of the pancreas. In children with severe asthma abdominal pain (presumably muscular) is sometimes a secondary feature of the attacks. Renal tract causes include recurrent renal infection, pyelonephritis,
66
JOHN APLEY
hydronephrosis and, much more uncommon, calculi, bladder neck obstruction, urethral cyst. The tally, even if incomplete, is a formidable one. But it must be re-emphasised that all the foregoing causes together explain only about one case in 20. In this small fraction with organic disorder there should be definite indications to suggest the need for the appropriate investigations. Bulldozing through all the remote possibilities is unnecessary and unjustifiable. Indeed, it may be actively harmful, for the physician runs the risk of himself becoming "a pathogenic agent in perpetuating the illness by his well-meaning but never-ending efforts to find a physical cause."6 Unacceptable Causes Many so-called causes do not stand up to critical and objective scrutiny. The evidence from controlled studies in Bristol and from other sources has been detailed elsewhere, 1 and here only a few of the conclusions are summarised. Worms. The bed-pan is a happy hunting-ground, not only for worms, but also for theories. In some tropical countries heavy infestation with worms may cause abdominal pain, but in Western countries neither statistical evidence nor the results of treatment support the notion that recurrent pains are caused by pinworms or any other worms. Non-specific Mesenteric Lymphadenitis. Because acute non-specific mesenteric adenitis occurs in association with acute upper respiratory tract infections it has been assumed that it has a counterpart, chronic non-specific mesenteric adenitis (presumed to be due to virus infections ), which may arise in the same way and cause pains recurring over a long period. It has not been shown that mesenteric adenitis is commoner in children with recurrent abdominal pain than in others; virus studies have been inconclusive; and the results of treatment (described later) are not consistent with the hypothesis. Chronic Appendicitis. Though "today every appendix is condemned by some pathologist somewhere," there is no validated pathology for a continuing low-grade appendicitis which persists indefinitely. If a "lily-white appendix" is removed by the surgeon and the pains cease, it may indicate that he has successfully removed not a source of infection, but of anxiety. In some children the pains do, in fact, continue to recur after appendicectomy, but the surgeon is unlikely to have been told about them. In other children the onset of recurrent abdominal pain can be dated back to appendicectomy. In either event, an incorrect diagnosis and consequent operation may make subsequent management more difficult. Children with recurrent abdominal pain, whatever the cause, cannot of course be guaranteed against developing an acutely inflamed appendix at some time. If there is real doubt about acute appendicitis,
THE CIllLD WITH RECURRENT ABDOMINAL PAIN
67
it is obviously prudent to remove the appendix; but the symptoms and signs in the attack should satisfy the physician. If acute appendicitis develops in a child with a history of previous abdominal pains, the features and the pattern of the attack are different from his usual pattern. This important diagnostic point has repeatedly been confirmed in my cases. Food Sensitivity. Milk, fat and other items in the diet have been postulated as causes of recurrent abdominal pain. A degree of proof sufficiently rigorous to be scientifically convincing is difficult to achieve, and the element of suggestion is often inseparable from treatment. In children with recurrent abdominal pains even strongly suggestive histories of food sensitivity or allergy can so often be disproved, and the pains come and go irrespective of diet. Moreover, even when the diet is not modified, the symptoms are improved by attention to other factors, Epilepsy. It has been claimed that recurrent abdominal pain is a common manifestation of epilepsy without loss of consciousness; but in my experience it is an inordinately rare one. In my series of children with abdominal pains epileptiform electroencephalograms were no commoner than in a control series. What is more, anti-convulsant treatment has no specific effect on the pains, but they respond satisfactorily to other measures.
RECURRENT ABDOMINAL PAIN WITH EMOTIONAL DISTURBANCES
Agreement on the precise proportion of children in whom an organic cause can be found may not be complete; what is agreed is that they form only a small fraction of the total. Most have no organic disorder, and it is they who pose the real and most interesting problems of diagnosis and management. The problems can nearly always be solved without recourse to a vast paraphernalia of diagnostic apparatus. Nobody will dispute that the physician needs to learn and practise the appropriate techniques, if he is to elicit and interpret accurately the signs of physical disorder. It is just as necessary to learn and practise the appropriate techniques if he is to elicit and interpret accurately the signs of emotional disorder. Some of the evidence of emotional disturbance is obtained by observation of the child, and the child-parent relationship; mostly it is derived from the history of his developmental progress and his inter-personal relationships. Observation and history-taking are essential skills which can be developed into highly efficient tools of diagnosis. In the presumptive diagnosis of an emotional cause for abdominal pains, the failure to demonstrate an organic cause is not itself enough; there· should be, in addition, acceptable evidence of emotional disturbance.
;;~---------------68
JOHN ApLEY
A series of children with recurrent abdominal pain, but no organic disease, were compared with a control series, l and a helpful and characteristic clinical profile can be outlined. The intelligence was no higher and no lower than in unaffected children. As regards personality, they were much more often described as highly-strung, fussy, excitable, anxious, timid or apprehensive (a very few, on the contrary, were aggressive and quarrelsome). Some were bad mixers. Many were extraordinarily over-conscientious. What I refer to as the "persistent umbilical cord syndrome" (maternal over-protection) was often obvious. Expressions of emotional disturbance were much commoner than in the controls. They included undue fears, nocturnal enuresis and sleeping difficulties; but the most common and significant were appetite problems and food fussiness, and difficulties at school. In most "little bellyachers" several of these items occur together. No one of them is specifically diagnostic; but the combination of characteristic personality traits and emotional disturbances is usually convincing and diagnostically reliable. Evidence of an emotional association with the pains can be obtained in many cases from the history of a time relationship between emotional stress and the onset of pain, or from enquiries about precipitating factors. "Monday morning colic" is one example of many which imply school difficulties. Domestic factors, such as parental disharmony, paternal over ambition for the child, maternal illness, are even commoner, though they may be harder to unearth. I recall a child who was admitted to hospital for observation and, as usually happens, had no pains in hospital-until one day, when she was looking out of the window, she saw her mother approaching and promptly became pale and lay down with her usual "tummy" pain. The slender clue was followed successfully. Unacceptable Causes We must be no less critical in accepting emotional disorders than in accepting physical disorders to explain recurrent abdominal pain. There can be few families which have not recently moved house, or had another child, or been involved with some illness, accident or change of routine. On the basis of such occurrences alone it is certainly not justifiable to accept emotional stress as a cause of abdominal pain. Which Comes First? Even with definite evidence of emotional disturbances, one should consider which came first-the pain or the disturbances? Continuing pain due to any cause may provoke emotional disorders. In children with recurrent abdominal pain due to an organic cause the degree of emotional disturbance is not often convincing; but, and this is more
THE CHILD WITH RECURRENT ABDOMINAL PAIN
69
important, if it is present, it is recent and does not antedate the onset of pains. On the other hand, when no organic cause is demonstrable, the evidence of emotional disturbance can, as a rule, be traced back, often long before the onset of the pains. The Family Indeed, the evidence of emotional disturbance can usually be followed back into the rest of the family. One of the most helpful diagnostic aids in a child with recurrent abdominal pain is a full family history." When the parents and siblings of children with abdominal pain were compared with those of controls, l the incidence of similar pains (past or present) in the immediate family was nearly six times higher (a positive family history can be obtained in at least half such cases). The comparative frequency of other family disorders is also helpful: migraine, frequent bad headaches and other pains, and nervous disorders are very much commoner in the families of "little bellyachers." A negative family history is exceptional in the families of children with abdominal pains. In a small proportion of cases neither organic nor emotional disorder can be diagnosed, even if one goes to unjustifiable extremes. In nearly half of these it may be found that there is a positive family history of abdominal pains and related symptoms. With appropriate management (see p. 71 ) these children almost invariably do well; it may be that a minor emotional disturbance was undiagnosed, or (as I prefer to think) that an acquired awareness of pain in healthy children had been abolished.
Recurrent abdominal pain is usually part of a pattern of reaction to emotional stress, with the child's pattern of reaction reflecting a family pattern.
THE PRACTICAL APPROACH
Does it matter if recurrent abdominal pain is not correctly diagnosed, and accordingly is not correctly treated? With an organic disorder the answer is self-evident, but what of the children without organic disease? A follow-up survey has shown how poor is the long-term prognosis without appropriate treatment.! In brief, by the time they reach adolescence or early adult life only one third of the patients are completely well and free from abdominal pains. The remaining two thirds, though half have lost their abdominal pains, continue to suffer from recurrent bodily and nervous complaints. In other words, neglected "little
bellyachers" tend to become "big bellyachers." "The early death of grandparents in an undue proportion of cases has been decribed by Sibinga and Barbero. 4
70
JOHN APLEY
A logical and helpful strategy of approach is based on the diagnostic probabilities. A small proportion of patients have an organic disorder, which is promptly diagnosable and should be promptly diagnosed. As a corollary, the large proportion of patients have no organic disease. Their future will be jeopardised if they are investigated pointlessly and interminably, or treated superficially with drugs, while an underlying emotional disorder is ignored. History In most cases it is the history which provides the only positive evidence on which the cause of recurrent abdominal pain should be diagnosed, but the history must be comprehensive, and it must go much further than the attacks of pain. The severity and other characteristics of the pain itself are not often diagnostic. Some points from the history in favour of an organic disorder might be that the pain is not central, or that it persists unchanged for 24 hours or more; that there is no history of headaches and limb pains, and no evidence of emotional disorder in the child; and that there is no history of nervous disorders, recurrent abdominal or other pains or migraine in the immediate family. A time-relationship, of pain associated with a stressful situation, is strongly in favour of a psychogenic cause. Details of the first attack may reveal a precipitating factor, which mayor may not continue to be operative later. It is important to obtain information about the child's behaviour and reactions before the pains began. ~ His behaviour and progress between attacks may give a clear indication of the cause of the pains. The question, "What sort of child is he?" often allows the mother to bring out crucial evidence which has been submerged and forgotten. In most cases, to understand the symptom it is necessary to understand the child as a person, in the varied circumstances of school and play, home and family. Time and again it is found that an intimate knowledge of the family, including its illnesses and inter-relationships, its attitudes and way of life, provides the only positive clues to diagnosis. Clinical Examination
When the clinical examination is carried out, its thoroughness, which should be apparent to everyone concerned, may itself prove therapeutic. Rarely will it reveal a physical disorder causing the pains; otherwise its main purpose is usually to confirm and emphasise the child's good physical condition. To satisfy himself, as well as the family, the physician will need to examine the child at least once between attacks and during an .. Sibinga and Barbero4 found that 40 per cent of children with recurrent abdominal pain had had neonatal difficulties such as prolonged colic, diarrhoea and vomiting or were hospitalized for other reasons.
THE CHILD WITH RECURRENT ABDOMINAL PAIN
71
attack. The examination should be repeated during an attack if the pattern of that attack differs from the child's usual pattern, because some new disorder (e.g. acute appendicitis) may have developed. But unnecessarily frequent abdominal examination may be actively harmful, and undermine all future management, by ingraining fears of serious organic disorder and by permanently focussing attention on a symptom rather than on the child. Investigations It is mandatory to examine the urine microscopically, if renal tract disorders are not to be overlooked. But, to use William Penn's phrase, a 'Wantonness in inquiry" should be avoided, because indiscriminate and continued investigations undermine confidence and make a logical plan of management impossible. Additional investigations should be based on positive indications derived from the history and the clinical evidence. A legitimate exception, however, may be an investigation done to help convince parents who have some specific fear (e.g. of leukaemia or tuberculosis) nagging in their minds. If the child's condition is deteriorating and he is losing weight (he rarely is), infection and neoplastic disease must promptly be excluded. Pallor which is persistent (not just occurring in short bouts) calls for haematological investigations and examination of the stools for blood. Abdominal distension in attacks, or localised pain persisting unabated for 24 hours or more, will indicate the need for barium meal studies. Pains in the loin, or any urinary symptoms, demand a full urogenital investigation. With generalised adenitis or splenomegaly the appropriate blood examinations are necessary. If there is a history of loss of consciousness, an electroencephalogram may be helpful. The physician may accept the axiom that his first duty is to exclude organic disease, but this should not make him lose perspective. His immediate duty is to make reasonably sure that no serious organic disorder is being missed; but he should also be reasonably sure that no serious emotional disorder is being missed or even made worse. Usually the child's good general physique is confirmatory evidence that he has no serious and urgent organic disorder, and the physician can then safely assess the situation at leisure, remembering that all diagnosis is provisional. If all does not go well, he can explore further, bearing in mind that the positive indications for further study or for specialist advice may be physical, but may (much more frequently) be emotional.
Management When an organic cause has been demonstrated, the treatment is usually clear-cut. But, whether the cause is organic or non-organic,
72
JOHN ApLEY
treatment does not apply only to the symptom-it applies to the person with the symptom. The physician is interested not only in the presenting complaint or the abdomen, not only in x-ray or laboratory reports, but also in the child. This implies management rather than treatment, and management starts with the Rrst words spoken at the Rrst diagnostic consultation. When organic disease has been reasonably excluded, the parents (and patient) should not be left in a diagnostic vacuum with a statement like "There's nothing wrong." First, it is necessary to explain to them how and why the possibilities of an organic disorder have been ruled out: "No reassurance without explanation" is a useful rule in building up their conRdence, whether they are vaguely anxious or fear some particular disease. Second, it helps if parents and child are encouraged to "blow off steam" and so lower the emotional tension . Third, down-to-earth guidance on modifying harmful aspects in the child's environment should be offered. The aim is to ease the pain, but to do more than that alone: it is to help the child (and his family) to develop a more resilient and robust attitude to the problems of growing up. Drug treatment has not been discussed here. Drug treatment is at best superRcial, for it is aimed at a symptom, and the use of drugs tends to obscure the underlying cause. Osler once complained of those who came to him "asking not wisdom, but drugs to charm with."
REFERENCES 1. Apley, J.: The Child with Abdominal Pains. Oxford, Blackwell Scientific Publications, 1964. 2. Apley, J., and Naish, N.: Recurrent Abdominal Pains: A Field Survey of 1,000 Schoolchildren. Arch. Dis. Childhood, 33: 165, 1958. 3. Heinild, S. V., Malver, E., Roelsgaard, G., and Worning, B.: A Psychosomatic Approach to Recurrent Abdominal Pain in Childhood. Acta Paediat., 48:361, 1959. 4. Sibinga, M. S., and Barbero, G.: Natural History of Abdominal Pain in Childhood; in Psychosomatic Aspects of Castro-intestinal Illness in Childhood. Columbus, Ohio, Ross Laboratories, 1963. 5. Smith, L. A., and others: An Atlas of Pain Patterns. Springfield, Ill., Charles C Thomas, 1961. 6. Weiss, E.: Psychogenic Rheumatism. Ann. Int. Med., 26:890, 1947. Bristol Royal Hospital for Sick Children St. Michael's Hill Bristol, 2 England
PAIN IN DIAGNOSIS
To most of us pain is no stranger, and pain is what brings many children to the physician. Yet so much about pain is unknown and unexplained. It cannot be measured. It can hardly be described except by analogy-children have spoken of abdominal pain as an ache, or colicky, or ''brown,'' or "my tummy feels pale," '1ike a lump of food," "sharp as knives," or "a headache in my tummy." An identical stimulus produces pain which does not seem identical in different people, or in the same person at different times and in different moods. Pain is associated with varying reactions, which can be physical (e.g. pallor) or emotional (e.g. irritability). It may respond differently in different people to the same drugs. Feeling pain may become almost a habit or a way of life. When a child keeps complaining of abdominal pain, when the customary examinations and investigations fail to reveal the cause, when drugs and diets have no real effect, the patient and his parents may lose confidence in their physician and the baffied physician lose confidence in himself. In these circumstances one might sympathise if he is tempted to call the patient "a little bellyacher," and to resort to cliches, which are unconvincing and often untrue, like "There's nothing wrong," and "He will grow out of it" (see p. 69). But the correct diagnosis can nearly always be made quite simply, and rational treatment based on it usually leads to a gratifying response. With a suitable comprehensive approach the clinical exercise in diagnosis and treatment of recurrent abdominal pain becomes one of the most fascinating in medicine. It is also one of the most rewarding, for recurrent abdominal pain is among the commonest disturbances of childhood. 'i> " In a survey of unselected school children 2 it was found that one in nine had had at least three attacks of recurrent abdominal pain, severe enough to affect their activities, over a period longer than three months. The frequency may vary in different parts of the world, but my impression from discussions with colleagues in many countries is that it might tum out to be proportional to each country's level of sophisticationl
63
64
JOHN ApLEY
In attempting to make a precise diagnosis, how much can be learnt from the presenting symptom, pain? In a series of children with recurrent abdominal pain I have analysed the characteristics of the pain: 1 what it feels like, its site, severity, time of occurrence, duration, frequency and periodicity. The common associations of attacks of pain were also scrutinised: they were vomiting, pain in the head or limbs, elevated temperature, sleepiness after attacks, pallor. In most cases the features described are characteristic of visceral pain, 5 but for differential diagnosis this is not enough. It is important to appreciate that the pain is not an isolated occurrence: it is part of a pattern of reaction. So often we are asked to see a child who complains, as he has done before, of abdominal pain, and who points to the umbilicus as the place where it hurts. He may have vomited. His temperature may be slightly elevated. Usually he looks pale, and on examination there is vague abdominal tenderness. In a few hours he has recovered. In each child there tends to be a "pattern" of attacks, with slight variations over a limited period of time; but over a period of years there may be pronounced changes of pattern. The child who is "a little bellyacher" is likely to have been a vomiter in infancy and to suffer from headaches, migraine and other disturbances when he becomes an adult (see p. 69). If we limit our enquiries to the main presenting symptom, pain alone, we usually get nowhere. It is essential to extend them further than the symptom or the abdomen, and further than the present attack. Pain is an experience in which the whole child is concerned. The diagnostic approach must be broadened to include the whole child as he is-during and between attacks-and as he was before the attacks commenced. To enter and explore the child's world we need a key; when he complains of pain he may be asking for help and offering us the key. (>
RECURRENT ABDOMINAL PAIN WITH ORGANIC DISEASES
"Organic or non-organic?" The question is over-simplified, for organic symptoms have an emotional component and emotional symptoms have an organic component. The whole patient is involved. But the practising physician is rightly concerned not to overlook an organic disorder which he hopes may be quickly cured and which, if undiagnosed, might prove lethal. So, in practice, the physician who considers organic disorders first, if only to exclude them, is prudent, so long as this approach does not limit his perspective or warp his medical philosophy . ., In the most severe pains, with the child screaming and writhing in agony, no organic cause could be found.
THE CHILD WITH RECURRENT ABDOMINAL PAIN
65
It has been generally agreed in several large surveys that in children with abdominal pain which has recurred over a period of months or years, an organic cause can be demonstrated in only a small proportion. Since the practice of medicine is largely based on probabilities, it is important to appreciate how small this proportion is. In the first 100 cases which were extensively studied for research purposes in Bristoll an organic cause was demonstrated in eight. In the next 100 cases, similarly studied, six causative organic causes were found. In several hundred cases seen since, in which, as a result of the lessons learned, investigations were deliberately restricted, the proportion with an organic cause has been of the same order. Doubtful and unacceptable causes will be considered shortly, and the overwhelmingly more common cases with no organic disorder can then be considered; but an undisputed organic cause for recurrent abdominal pain is to be found in little more than one case of every 20. Organic Causes Among these no single disorder is common, and most are extremely rare. Renal tract causes together are roughly equal in number to gastro-intestinal causes. Extra-abdominal causes are extremely uncommon as a cause of pain over an extended period of time. They include spinal disease (e.g. tuberculous), intra-cranial neoplasm, hyperthyroidism and hypoglycaemia. Porphyria may present with pain over a period of years, and I suspect that some other rare metabolic disturbances may come to be added to the list. Castro-intestinal tract causes include partial malrotation or duplication of intestine, recurrent intussusception, regional ileitis, tumours, Meckel's diverticulum and Chilaiditi's syndrome, but all these are rare. Peptic ulcer is less uncommon, but even so, with strict criteria for diagnosis, I assess its frequency as about 1 per cent of all cases with recurrent abdominal pain. The usual radiological findings, variable changes in peristalsis and altered mucosal patterns with an "irritable stomach,"3 are liable to be misinterpreted. I mention here excessive air-swallowing, which I find is occasionally a cause of recurrent abdominal distension and pain in children, long after infancy, and is associated with severe emotional disturbances. Other intra-abdominal causes include Henoch-Schoenlein purpura, sickle cell anaemia, lead poisoning, splenomegaly, haemochromatosis, cirrhosis of liver, hepatomegaly with heart failure, cholecystitis, calcification of the pancreas. In children with severe asthma abdominal pain (presumably muscular) is sometimes a secondary feature of the attacks. Renal tract causes include recurrent renal infection, pyelonephritis,
66
JOHN APLEY
hydronephrosis and, much more uncommon, calculi, bladder neck obstruction, urethral cyst. The tally, even if incomplete, is a formidable one. But it must be re-emphasised that all the foregoing causes together explain only about one case in 20. In this small fraction with organic disorder there should be definite indications to suggest the need for the appropriate investigations. Bulldozing through all the remote possibilities is unnecessary and unjustifiable. Indeed, it may be actively harmful, for the physician runs the risk of himself becoming "a pathogenic agent in perpetuating the illness by his well-meaning but never-ending efforts to find a physical cause."6 Unacceptable Causes Many so-called causes do not stand up to critical and objective scrutiny. The evidence from controlled studies in Bristol and from other sources has been detailed elsewhere, 1 and here only a few of the conclusions are summarised. Worms. The bed-pan is a happy hunting-ground, not only for worms, but also for theories. In some tropical countries heavy infestation with worms may cause abdominal pain, but in Western countries neither statistical evidence nor the results of treatment support the notion that recurrent pains are caused by pinworms or any other worms. Non-specific Mesenteric Lymphadenitis. Because acute non-specific mesenteric adenitis occurs in association with acute upper respiratory tract infections it has been assumed that it has a counterpart, chronic non-specific mesenteric adenitis (presumed to be due to virus infections ), which may arise in the same way and cause pains recurring over a long period. It has not been shown that mesenteric adenitis is commoner in children with recurrent abdominal pain than in others; virus studies have been inconclusive; and the results of treatment (described later) are not consistent with the hypothesis. Chronic Appendicitis. Though "today every appendix is condemned by some pathologist somewhere," there is no validated pathology for a continuing low-grade appendicitis which persists indefinitely. If a "lily-white appendix" is removed by the surgeon and the pains cease, it may indicate that he has successfully removed not a source of infection, but of anxiety. In some children the pains do, in fact, continue to recur after appendicectomy, but the surgeon is unlikely to have been told about them. In other children the onset of recurrent abdominal pain can be dated back to appendicectomy. In either event, an incorrect diagnosis and consequent operation may make subsequent management more difficult. Children with recurrent abdominal pain, whatever the cause, cannot of course be guaranteed against developing an acutely inflamed appendix at some time. If there is real doubt about acute appendicitis,
THE CIllLD WITH RECURRENT ABDOMINAL PAIN
67
it is obviously prudent to remove the appendix; but the symptoms and signs in the attack should satisfy the physician. If acute appendicitis develops in a child with a history of previous abdominal pains, the features and the pattern of the attack are different from his usual pattern. This important diagnostic point has repeatedly been confirmed in my cases. Food Sensitivity. Milk, fat and other items in the diet have been postulated as causes of recurrent abdominal pain. A degree of proof sufficiently rigorous to be scientifically convincing is difficult to achieve, and the element of suggestion is often inseparable from treatment. In children with recurrent abdominal pains even strongly suggestive histories of food sensitivity or allergy can so often be disproved, and the pains come and go irrespective of diet. Moreover, even when the diet is not modified, the symptoms are improved by attention to other factors, Epilepsy. It has been claimed that recurrent abdominal pain is a common manifestation of epilepsy without loss of consciousness; but in my experience it is an inordinately rare one. In my series of children with abdominal pains epileptiform electroencephalograms were no commoner than in a control series. What is more, anti-convulsant treatment has no specific effect on the pains, but they respond satisfactorily to other measures.
RECURRENT ABDOMINAL PAIN WITH EMOTIONAL DISTURBANCES
Agreement on the precise proportion of children in whom an organic cause can be found may not be complete; what is agreed is that they form only a small fraction of the total. Most have no organic disorder, and it is they who pose the real and most interesting problems of diagnosis and management. The problems can nearly always be solved without recourse to a vast paraphernalia of diagnostic apparatus. Nobody will dispute that the physician needs to learn and practise the appropriate techniques, if he is to elicit and interpret accurately the signs of physical disorder. It is just as necessary to learn and practise the appropriate techniques if he is to elicit and interpret accurately the signs of emotional disorder. Some of the evidence of emotional disturbance is obtained by observation of the child, and the child-parent relationship; mostly it is derived from the history of his developmental progress and his inter-personal relationships. Observation and history-taking are essential skills which can be developed into highly efficient tools of diagnosis. In the presumptive diagnosis of an emotional cause for abdominal pains, the failure to demonstrate an organic cause is not itself enough; there· should be, in addition, acceptable evidence of emotional disturbance.
;;~---------------68
JOHN ApLEY
A series of children with recurrent abdominal pain, but no organic disease, were compared with a control series, l and a helpful and characteristic clinical profile can be outlined. The intelligence was no higher and no lower than in unaffected children. As regards personality, they were much more often described as highly-strung, fussy, excitable, anxious, timid or apprehensive (a very few, on the contrary, were aggressive and quarrelsome). Some were bad mixers. Many were extraordinarily over-conscientious. What I refer to as the "persistent umbilical cord syndrome" (maternal over-protection) was often obvious. Expressions of emotional disturbance were much commoner than in the controls. They included undue fears, nocturnal enuresis and sleeping difficulties; but the most common and significant were appetite problems and food fussiness, and difficulties at school. In most "little bellyachers" several of these items occur together. No one of them is specifically diagnostic; but the combination of characteristic personality traits and emotional disturbances is usually convincing and diagnostically reliable. Evidence of an emotional association with the pains can be obtained in many cases from the history of a time relationship between emotional stress and the onset of pain, or from enquiries about precipitating factors. "Monday morning colic" is one example of many which imply school difficulties. Domestic factors, such as parental disharmony, paternal over ambition for the child, maternal illness, are even commoner, though they may be harder to unearth. I recall a child who was admitted to hospital for observation and, as usually happens, had no pains in hospital-until one day, when she was looking out of the window, she saw her mother approaching and promptly became pale and lay down with her usual "tummy" pain. The slender clue was followed successfully. Unacceptable Causes We must be no less critical in accepting emotional disorders than in accepting physical disorders to explain recurrent abdominal pain. There can be few families which have not recently moved house, or had another child, or been involved with some illness, accident or change of routine. On the basis of such occurrences alone it is certainly not justifiable to accept emotional stress as a cause of abdominal pain. Which Comes First? Even with definite evidence of emotional disturbances, one should consider which came first-the pain or the disturbances? Continuing pain due to any cause may provoke emotional disorders. In children with recurrent abdominal pain due to an organic cause the degree of emotional disturbance is not often convincing; but, and this is more
THE CHILD WITH RECURRENT ABDOMINAL PAIN
69
important, if it is present, it is recent and does not antedate the onset of pains. On the other hand, when no organic cause is demonstrable, the evidence of emotional disturbance can, as a rule, be traced back, often long before the onset of the pains. The Family Indeed, the evidence of emotional disturbance can usually be followed back into the rest of the family. One of the most helpful diagnostic aids in a child with recurrent abdominal pain is a full family history." When the parents and siblings of children with abdominal pain were compared with those of controls, l the incidence of similar pains (past or present) in the immediate family was nearly six times higher (a positive family history can be obtained in at least half such cases). The comparative frequency of other family disorders is also helpful: migraine, frequent bad headaches and other pains, and nervous disorders are very much commoner in the families of "little bellyachers." A negative family history is exceptional in the families of children with abdominal pains. In a small proportion of cases neither organic nor emotional disorder can be diagnosed, even if one goes to unjustifiable extremes. In nearly half of these it may be found that there is a positive family history of abdominal pains and related symptoms. With appropriate management (see p. 71 ) these children almost invariably do well; it may be that a minor emotional disturbance was undiagnosed, or (as I prefer to think) that an acquired awareness of pain in healthy children had been abolished.
Recurrent abdominal pain is usually part of a pattern of reaction to emotional stress, with the child's pattern of reaction reflecting a family pattern.
THE PRACTICAL APPROACH
Does it matter if recurrent abdominal pain is not correctly diagnosed, and accordingly is not correctly treated? With an organic disorder the answer is self-evident, but what of the children without organic disease? A follow-up survey has shown how poor is the long-term prognosis without appropriate treatment.! In brief, by the time they reach adolescence or early adult life only one third of the patients are completely well and free from abdominal pains. The remaining two thirds, though half have lost their abdominal pains, continue to suffer from recurrent bodily and nervous complaints. In other words, neglected "little
bellyachers" tend to become "big bellyachers." "The early death of grandparents in an undue proportion of cases has been decribed by Sibinga and Barbero. 4
70
JOHN APLEY
A logical and helpful strategy of approach is based on the diagnostic probabilities. A small proportion of patients have an organic disorder, which is promptly diagnosable and should be promptly diagnosed. As a corollary, the large proportion of patients have no organic disease. Their future will be jeopardised if they are investigated pointlessly and interminably, or treated superficially with drugs, while an underlying emotional disorder is ignored. History In most cases it is the history which provides the only positive evidence on which the cause of recurrent abdominal pain should be diagnosed, but the history must be comprehensive, and it must go much further than the attacks of pain. The severity and other characteristics of the pain itself are not often diagnostic. Some points from the history in favour of an organic disorder might be that the pain is not central, or that it persists unchanged for 24 hours or more; that there is no history of headaches and limb pains, and no evidence of emotional disorder in the child; and that there is no history of nervous disorders, recurrent abdominal or other pains or migraine in the immediate family. A time-relationship, of pain associated with a stressful situation, is strongly in favour of a psychogenic cause. Details of the first attack may reveal a precipitating factor, which mayor may not continue to be operative later. It is important to obtain information about the child's behaviour and reactions before the pains began. ~ His behaviour and progress between attacks may give a clear indication of the cause of the pains. The question, "What sort of child is he?" often allows the mother to bring out crucial evidence which has been submerged and forgotten. In most cases, to understand the symptom it is necessary to understand the child as a person, in the varied circumstances of school and play, home and family. Time and again it is found that an intimate knowledge of the family, including its illnesses and inter-relationships, its attitudes and way of life, provides the only positive clues to diagnosis. Clinical Examination
When the clinical examination is carried out, its thoroughness, which should be apparent to everyone concerned, may itself prove therapeutic. Rarely will it reveal a physical disorder causing the pains; otherwise its main purpose is usually to confirm and emphasise the child's good physical condition. To satisfy himself, as well as the family, the physician will need to examine the child at least once between attacks and during an .. Sibinga and Barbero4 found that 40 per cent of children with recurrent abdominal pain had had neonatal difficulties such as prolonged colic, diarrhoea and vomiting or were hospitalized for other reasons.
THE CHILD WITH RECURRENT ABDOMINAL PAIN
71
attack. The examination should be repeated during an attack if the pattern of that attack differs from the child's usual pattern, because some new disorder (e.g. acute appendicitis) may have developed. But unnecessarily frequent abdominal examination may be actively harmful, and undermine all future management, by ingraining fears of serious organic disorder and by permanently focussing attention on a symptom rather than on the child. Investigations It is mandatory to examine the urine microscopically, if renal tract disorders are not to be overlooked. But, to use William Penn's phrase, a 'Wantonness in inquiry" should be avoided, because indiscriminate and continued investigations undermine confidence and make a logical plan of management impossible. Additional investigations should be based on positive indications derived from the history and the clinical evidence. A legitimate exception, however, may be an investigation done to help convince parents who have some specific fear (e.g. of leukaemia or tuberculosis) nagging in their minds. If the child's condition is deteriorating and he is losing weight (he rarely is), infection and neoplastic disease must promptly be excluded. Pallor which is persistent (not just occurring in short bouts) calls for haematological investigations and examination of the stools for blood. Abdominal distension in attacks, or localised pain persisting unabated for 24 hours or more, will indicate the need for barium meal studies. Pains in the loin, or any urinary symptoms, demand a full urogenital investigation. With generalised adenitis or splenomegaly the appropriate blood examinations are necessary. If there is a history of loss of consciousness, an electroencephalogram may be helpful. The physician may accept the axiom that his first duty is to exclude organic disease, but this should not make him lose perspective. His immediate duty is to make reasonably sure that no serious organic disorder is being missed; but he should also be reasonably sure that no serious emotional disorder is being missed or even made worse. Usually the child's good general physique is confirmatory evidence that he has no serious and urgent organic disorder, and the physician can then safely assess the situation at leisure, remembering that all diagnosis is provisional. If all does not go well, he can explore further, bearing in mind that the positive indications for further study or for specialist advice may be physical, but may (much more frequently) be emotional.
Management When an organic cause has been demonstrated, the treatment is usually clear-cut. But, whether the cause is organic or non-organic,
72
JOHN ApLEY
treatment does not apply only to the symptom-it applies to the person with the symptom. The physician is interested not only in the presenting complaint or the abdomen, not only in x-ray or laboratory reports, but also in the child. This implies management rather than treatment, and management starts with the Rrst words spoken at the Rrst diagnostic consultation. When organic disease has been reasonably excluded, the parents (and patient) should not be left in a diagnostic vacuum with a statement like "There's nothing wrong." First, it is necessary to explain to them how and why the possibilities of an organic disorder have been ruled out: "No reassurance without explanation" is a useful rule in building up their conRdence, whether they are vaguely anxious or fear some particular disease. Second, it helps if parents and child are encouraged to "blow off steam" and so lower the emotional tension . Third, down-to-earth guidance on modifying harmful aspects in the child's environment should be offered. The aim is to ease the pain, but to do more than that alone: it is to help the child (and his family) to develop a more resilient and robust attitude to the problems of growing up. Drug treatment has not been discussed here. Drug treatment is at best superRcial, for it is aimed at a symptom, and the use of drugs tends to obscure the underlying cause. Osler once complained of those who came to him "asking not wisdom, but drugs to charm with."
REFERENCES 1. Apley, J.: The Child with Abdominal Pains. Oxford, Blackwell Scientific Publications, 1964. 2. Apley, J., and Naish, N.: Recurrent Abdominal Pains: A Field Survey of 1,000 Schoolchildren. Arch. Dis. Childhood, 33: 165, 1958. 3. Heinild, S. V., Malver, E., Roelsgaard, G., and Worning, B.: A Psychosomatic Approach to Recurrent Abdominal Pain in Childhood. Acta Paediat., 48:361, 1959. 4. Sibinga, M. S., and Barbero, G.: Natural History of Abdominal Pain in Childhood; in Psychosomatic Aspects of Castro-intestinal Illness in Childhood. Columbus, Ohio, Ross Laboratories, 1963. 5. Smith, L. A., and others: An Atlas of Pain Patterns. Springfield, Ill., Charles C Thomas, 1961. 6. Weiss, E.: Psychogenic Rheumatism. Ann. Int. Med., 26:890, 1947. Bristol Royal Hospital for Sick Children St. Michael's Hill Bristol, 2 England