1064 COT DEATH AND RESPIRATORY DRIVE SMALL babies
are rather bad at breathing. This the just applies very premature, but also to babies well beyond term, and is almost certainly a major factor in the production of cot deaths. Naeyel suggests that chronic hypoxia is a frequent antecedent to cot death. Necropsies on such babies have disclosed hypertrophy of pulmonary arteries2 and right ventricle,3 persistence of brown fat,4 lipid-laden cells in the cerebrospinal fluid,s astroglial proliferation in the brainstem,6 and postnatal growth retardatiohl-all of which occur in babies with chronic hypoxia. The poor response of sleeping babies to upper respiratory obstruction was accidentally demonstrated by Cross and Lewis,8 and Swift and Emery9 found that six-week-old babies were even more likely than the newborn to respond inadequately to deliberate nasal obstruction, especially during rapid-eye-movement (R.E.M.) sleep. Upper-respiratory infection in a small baby, though apparently trivial, may thus bear sinister
not
to
implications. Babies may, however, stop breathing without any airway obstruction. Normal infants, and even adults, have striking variations in respiration during R.E.M. sleep,’o and an extension of this irregularity may account for the "near-miss-cot-death" syndrome, in which sleeping babies are found cyanbsed or pale and apnoeic but can be resuscitated. Investigations of this syndrome, which may have a strong familial tendency, indicate that such babies may have frequent and prolonged apnoeic attacks
during sleep, especially
R.E.M.
sleep." Nasopharyngitis
may increase the attacks, but they occur in its absence. The syndrome is strikingly similar to apnoea of prematurity, and the response of the two groups of babies to changes in blood gases bears comparison. Very premature babies have a greatly reduced ventilatory response to rising Pco2, related to gestation (probably due to neurological immaturity) and to age (perhaps due to stiff lungs from retained fluid).12,13 They also have a
characteristic response to hypoxia, which, paradoxically, depresses ventilation. 14 Shannon et al. 15 suggest that nearmiss-cot-death babies also respond poorly to the stimulus of CO2, and showed that 11 such infants had significantly’higher alveolar CO2 and less hyperventilation on breathing 5% CO2 than normal controls, although the response to hypoxia was not measured. This provides strong supportive evidence for the association between chronic hypoxaemia and cot death, as well as a terminal mechanism of death in these babies. It is, however, difficult to rule out the small possibility that their findings were due to acute anoxic brain damage, despite clinical and electroencephalographic normality at the time of the investigations: all the babies had had neuro1. Naeye, R. L. Archs Path. Lab. Med. 1977, 101, 165. 2. Naeye, R. L. New Engl. J. Med. 1973, 289, 1167. 3. Naeye, R. L. Science, 1974, 186, 837. 4. Naeye, R. L., et al. Am. J. Path. 1976, 82, 1. 5. Gadsdon, D. R., Emery, J. L. Archs Dis. Childh. 1976, 51, 42. 6. Naeye, R. L. Am. J. clin. Path. 1976, 66, 526. 7. Peterson, D. R., et al. Am. J. Epidem. 1974, 99, 389. 8. Cross, K. W., Lewis, S. R. Archs Dis. Childh. 1971, 46, 211 9. Swift, P. G. F., Emery, J. L. ibid. 1973, 48, 947. 10. Aserinsky, E. Science, 1965, 150, 763. 11. Steinschneider, A. Pediatrics, 1972, 50, 646. 12. Rigatto, H., Brady, J. P., Verduzco, R. ibid. 1975, 55, 614. 13. Frantz, I. D., et al. J. appl. Physiol. 1976, 41, 41. 14. Rigatto, H., Verduzco, R., Cates, D. B. ibid. 1975, 39, 896. 15. Shannon, D. C, Kelly, D. H., O’Connell, K. New Engl. J. Med. 1977, 297, 747
logical abnormalities for up to 48 hours after an apnceic attack. One interesting investigation would be to compare their responses with those of babies known to have had acute anoxia, such as birth asphyxia (who also frequently have apnoeic episodes). We can also compare these patients with infants with Ondine’s curse, who have an inadequate (or even paradoxical) response to CO2, and become apnoeic during non-R.E.M. sleep.’s These babies breathe normally during R.E.M. sleep, indicating that CO2 drive is not necessary at this time, whereas this is the phase in which near-miss infants tend to have their apnoeic attacks."I Cot death is a "symptom" rather than a disease, and there is at present no way of knowing how often it is due to primary failure of ventilation: even a large prospective study of ventilatory responses in newborn might not give the answer, since respiratory drive may decrease during the first few weeks of life.9 Even when babies at risk are identified, death cannot always be prevented : 5 of 16 such babies in two series subsequently died at home. CHRONIC CONSTIPATION IN CHILDHOOD CHILDREN’S bowels have been an anxious topic for many a year. In the lst century A.D., the aptly named Soranus recommended digital anal dilatation in the newborn infant: "With the little finger whose nail has first been cut short one must for the unhindered passing of the excrements dilate the anus and divide the thin membranous body which is often grown around it".’ Purging was the standard remedy for all ills, and many cultures insisted on a clean-out once a month: Herodotus says that the Egyptians took aperients for three consecutive days in each month. The modern view is more relaxed, but chronic constipation is a condition which wreaks havoc in a child’s life, especially when (as is often the case) it is associated with soiling. In published work on the subject, lack of agreement on nomenclature makes facts hard to distinguish from fancy. The terms encopresis and faecal incontinence tend to be used synonymously; what one mother regards as constipation is of no concern to another. Clayden2 recommends a convention to smooth the passage of ideas: he defines constipation as difficulty or delay in passage of faeces; soiling as the frequent passage of loose or semisolid stools in clothing; and encopresis as the passage of normal stools in abnormal places (including the clothes). The anatomy and physiology3-6 of the anorectal region is complex, and the precise mechanisms for defzecation and faecal continence are not fully understood. The initial desire to defaecate arises from distension of the rectum when faeces descend from the colon. As the rectum is distended the tone of the internal anal sphincter decreases and immediate continence is achieved by contraction of the external sphincter and levator ani muscles. With small rectal volumes the inhibition of the internal sphincter is temporary, but, as rectal distension increases, the internal sphincter remains 16. Shannon, D C, et al. Pediatrics, 1976, 57, 342 1 Soranus, Gynæcology Book 2 (translated by Johannes
Ilberg); p. 83. Balti-
more, 1956. 2. Clayden, G. S. Br. med. J. 1976, i, 515. 3. Lawson, J. O. N.Ann. R. Coll. Surg. 1974, 54, 244, 288. 4. Gowers, W. R. Proc. R. Soc. 1877, 26, 77. 5. Denny-Brown, D., Robertson, E. G. Brain, 1935, 58, 256. 6. Gaston, E. A. Surgery. Gynec. Obstet. 1948, 87, 280.
1065 inhibited and the external sphincter is either relaxed voluntarily or tires and the stool is passed. When rectal distension ceases the internal-sphincter tone returns. In a child, acquisition of the social skill of defalcation takes about two years.7-9 The bowels are not open in utero unless the fetus becomes distressed, but the reason for this is unknown. Reflex defaecation occurs soon after birth, within 36 hours in most babies, but it may be delayed in preterm infants by a mucus plug and in neonates by ileus, an anatomical anomaly of the anus, or Hirschsprung’s disease. Constipation is unusual in breast-fed babies, symptom-free delays of up to 10 days being normal in this group. Bottle-fed babies on mildly hyperosmolar feeds will dehydrate their faeces to provide enough water for urine solute clearance. These babies pass stools with considerable strain. Their reflex response to distension of the anal canal by these hard stools produces such grunting and facial plethora as to alarm the most placid of mothers. Tranquillity may be restored by ensuring adequate water intake and adding, if necessary, some sugar to the feeds. As the child approaches 2 years of age he becomes progressively aware of his ability to control the stool, and normally experiences some pleasure in defaecating in the pot, especially if he is rewarded with parental acclaim. However, this skill may also become a weapon to use against his parents. The negativism of the 2-year-old is frequently shown in pot training, especially if the parents are too aggressive or too effusive about this skill. Delay in acquiring stool control will be expected in children with other evidence of developmental delay. Disturbance may also arise if there are physical abnormalities making defalcation difficult or painful, or if sensation is impaired. True constipation in infancy must be taken seriously, especially if the abdomen is distended. When the difficulty in defalcation is unrelieved by simple measures such as adjusting the milk formula or adding a little milk of magnesia, and hypothyroidism and hypercalcaemia are excluded, an organic cause should be suspected. Rectal examination may detect a stricture, a covered anus, or reveal a gush as the finger is withdrawn characteristic of Hirschsprung’s disease. Sacral anomalies may also be found. Acute constipation often follows a dietary or environmental change, or a febrile illness. Sometimes an anal fissure is found. Most acute constipation settles spontaneously when the child returns to normal diet and fluid intake, but failing this the treatment is to soften the stool and gently stimulate a return to regular defaecation. It is much easier to treat a 7-10-day delay in this way than to evacuate manually under anxsthesia when acute impaction ensues after 2-3 weeks. A suitable stool softener is dioctyl sodium sulphosuccinate; this is best used on its own.lO Standardised senna and danthron are gentle laxatives suitable for children. Chronic constipation, with its frequently accompanying soiling, is more of a problem. Soiling is common; 1-32% of children over age 4 soiled by day with a ratio of 3/1 boys to girls." When Berg and Jonesl2 studied 78
children with functional faecal incontinence
(soiling and were able to definitions) they Clayden’s encopresis by divide the children into four major groups-16 with training problems without severe constipation; 16 with pot-refusal retention syndrome; 16 with severe constipation with overflow; and 30 with uncomplicated functional faecal incontinence passing normal stools (encopresis). They found that the two groups with associated constipation responded to combined oral laxative therapy and simple outpatient psychotherapy. Clayden and Lawson13 studied 106 children with longstanding chronic constipation who had failed to respond to standard medical treatment. Anorectal manometry in these children revealed that 10 had Hirschsprung’s disease (later confirmed by rectal biopsy) and 10 had Hirschsprung’s-like anorectal activity (failure of anal sphincter to relax in response to rectal distension) but normal biopsy appearances with standard histochemical techniques. Of the non-Hirschsprung’s group most showed exaggerated rhythmic activity in the anal canal, a higherthan-normal volume required in the rectum to produce internal-sphincter relaxation, and a reduced sensation of the call to stool. The last two observations would be expected with a megacolon, but the increased rhythmic activity suggested hypertrophy of the internal sphincter. With this in mind, in the non-Hirschsprung’s group they proceeded to dilate the anus forcefully (under ketamine anaesthesia) and were able to show striking improvement in most cases; and, as defaecation became easier, behavioural troubles also tended to abate. The prevalence of previously undiagnosed Hirschsprung’s disease was surprisingly high. Since 4 of the 10 Hirschsprung’s patients had a history of soiling, soiling cannot be used to exclude Hirschsprung’s disease. There have been several cases of Hirschsprung’s disease presenting in adult life and nearly always the trouble started in infancy. 11-20 One reason why such cases are missed in childhood is the mistaken view that chronic constipation should not be interfered with, but that Nature should be allowed to take its course. A complication of Hirschsprung’s disease which can be fatal is necrotising enterocolitis; Hirschsprung’s disease should, therefore, be considered in every case of persistent constipation. Anal manometry is the best screening method, with biopsy in likely cases.14 Fhe research trends are encouraging: improvements in histochemistry have made the diagnosis of ultrashortsegment Hirschsprung’s disease more accurate, since fibres which aganglionic areas have excess stain strongly for acetylcholinesterase;21 work on the nonadrenergic non-cholinergic (purinergic) system will probably shed more light on and possibly lead to more rational pharmacological approaches to the deranged internal sphincter;22 and anorectal manometry is now a safe and reliable investigation. The most encouraging trend is the multidisciplinary approach to the child with constipation. Whatever the original cause of the consti-
cholinergic
13.
7. Isaact, S. in On the Bringing up of Children (edited by J. Rickman); pp. 123, 135, 147, 160, and 165. London, 1936. 8 MacFarlane, J. W., Allen, L., Hanzig, M. A Developmental Study of Behaviour Problems of Normal Children; vol. II, p. 85. Los Angeles, 1954. 9. Douglas, J. W. B., Blomfield, J. M. Children under 5; p. 127. London, 1958. 10. Godding, E. W. Br. med.J. 1976, iii, 838. 11 From Birth to Seven: report from National Child Development Survey; p. 399. London, 1972. 12. Berg, I., Jones, K. V. Archs Dis Childh. 1964, 39, 465.
Clayden, G. S., Lawson, J. O. N. ibid. 1976, 51, 918.
Lawson, J. O. N., Nixon, H. H. J. pediat. Surg. 1967, 2, 544. 15. Whitehouse, F., Bargen, J. A., Dixon, C. F. Gastroenterology, 1943, 1, 922. 16. Rosin, J. D., Bargen, J. A., Waugh, J. M. Mayo Clin. Proc. 1950, 25, 710. 17. Riker, W. L. Archs Surg. 1957, 75, 362. 18. Jennings, P. J. Proc. R. Soc. Med. 1967, 60, 805. 19. Gertz, T. C., Hanson, L. K. Nord. Med. 1966, 75, 325. 20. Todd, I. P. Br. J. Surg. 1977, 64, 311. 21. Meier-Ruge, W. Curr. Topics Path. 1974, 59, 131. 22. Burnstock, G. Pharmac. Rev. 1972, 24, 509. 14.
1066
pation, the emotional factors
must be taken into Work such as the combined St. Thomas’/Great Ormond Street study, designed to assess the psychological and physiological factors, will define more accurately the aetiology and ideal management of this unpleasant problem of childhood. account.
PREMEDICATION TRADITION PREMEDICATION is dominated by tradition. The principles of our use of preoperative drugs survive from the days of ether anxsthesia when the euphoria and sedation of morphine were invaluable in helping the patient
the distress and excitement of the early induction stages and atropine or hyoscine was needed to control the salivary and bronchial secretions induced by ether. Barbiturates, phenothiazines, and tranquillisers were subsequently introduced over the years to replace or supplement the opiates. Now that induction is intravenous in most cases, is any form of sedative premedication necessary, unless a patient is particularly anxious? A preoperative visit by a sympathetic anaesthetist is often the best way of allaying the natural fear that most people have of losing consciousness. Though opinion is divided, injection of an antisialogogue is probably still a useful precaution about an hour preoperatively to avoid the complications of laryngeal irritation by oral secretions. In an effort to promote more rational choice of premedicant drugs, Forrest and others’ have investigated six commonly used premedications-two opiates, two barbiturates, an antihistamine, and a minor tranquilliser. Drugs were administered randomly from identical syringes to 509 patients, in whom subjective responses were scored on 0-9 scales 30 and 60 minutes after premedication. Independent assessments were made by nurse-observers and overall evaluations were recorded by the anxsthetists themselves. The observers tended to credit the antihistamine and the minor tranquilliser with an ability to suppress apprehension, but the patients thought otherwise. The anxsthetists went one better and rated the placebo as satisfactory in facilitating induction. This surely confirms that the induction of anwsthesia nowadays causes the patient little distress. How do you evaluate tradition?
through
SUPPORT FOR DISABLED HOUSEWIVES WOMEN are not fighting hard enough for their rights, and they have only themselves to blame if they are discriminated against and regarded merely as the dependants of their husbands. Thus, Dr Shirley Summerskill, Under-Secretary at the Home Office, told a conference on discrimination in employment on Oct. 19.2 Her words must indeed have seemed very provoking to one group of women-the disabled housewives, who have had to wait 2 years for the non-contributory invalidity pension accorded them under the Social Security Act of 1975. This Act provides for a non-contributory invalidity pension (N.C.I.p.) for men and women of working age who have been incapable of work for at least 28 consecutive weeks and who have not satisfied the contribution condi1. 2.
Forrest, W. H., Brown, C. R , Brown, B. W. Anesthesiology, 1977, 47, 241. Times, Oct. 20, p.6.
tions for sickness and invalidity benefit. Payment of N.C.I.P. for men and single women started on Nov. 20, 1975, and meant little extra charge on Treasury funds, since most claimants were already receiving the money
supplementary benefit. Housewives were originally excluded altogether from the draft legislation considered by Parliament: for them extra money would be needed, since they previously received nothing. Under pressure the (Labour) Government gave way on this point, but the difficulties of identifying the housewives entitled to benefit, and of verifying their claims, were given as reasons why payment of the housewives’ pension (H.N.C.I.P.) should be deferred. The scheme started on Nov. 17, and it is inequitable. The married woman will lose out if her husband is not working, for the value of her pension will be deducted from any sickness or other benefit he receives; and the 2-year delay has meant that some women have been cut out of the scheme altogether, since claimants must be under 60 to qualify (though benefit may continue beyond 60 if the claim is made before reaching that age). But its worst aspect is the claims procedure. Men and single women claim their N.C.I.P. on a short form which asks only for the essential information such as the date when incapacity for work started and the number of dependants being claimed for. On her form the housewife applicant is required to supply information on, among other things, the kind of accommodation she lives in, what adaptations have been made for her, what appliances are available and what help she needs to use them, how far the family or others contribute to doing the household jobs, and whether she is fit to do any paid work and if so to suggest what kind of work this might be. The discrimination does not end with the form, for the Department also requires special evidence to support the claim. A circular to general practitioners which went out in September explains that, while evidence on claims from men and single women normally takes the form of a doctor’s statement on form Med 3, for married women "the need to cover in some detail the physical and mental functions involved in household duties makes it impossible to rely on the use of form Med 3". Doctors will be invited to provide special medical reports, covering the housewife’s fitness for paid work, and her ability to perform household duties; moreover, completion of the form, the circular says, will normally involve a medical examination in the claimant’s home. A fee of 00 for these reports has been agreed with the B.M.A. Originally the Department intended that its own appointed doctors should carry out these- examinations, but it has now conceded that the patient’s own G.P. shall do them. Claims for mobility and attendance allowance have always had to be supported by evidence from a medical examination. But it is difficult to understand why a doctor who is thought capable of judging a person’s fitness to work without observing him actually doing it is yet incapable of similarly judging a woman’s fitness to carry out normal household duties. The Department of Health must have some means of checking dubious claims, but the procedures already in operation for N.C.I.P. would surely do. The system for claiming H.N.C.I.P. is cumbersome and expensive; it wastes medical time and it discriminates against married women. The housewives could have been incorporated into the existing scheme simply by amending the claim form and doctor’s statement. as
are rather bad at breathing. This the just applies very premature, but also to babies well beyond term, and is almost certainly a major factor in the production of cot deaths. Naeyel suggests that chronic hypoxia is a frequent antecedent to cot death. Necropsies on such babies have disclosed hypertrophy of pulmonary arteries2 and right ventricle,3 persistence of brown fat,4 lipid-laden cells in the cerebrospinal fluid,s astroglial proliferation in the brainstem,6 and postnatal growth retardatiohl-all of which occur in babies with chronic hypoxia. The poor response of sleeping babies to upper respiratory obstruction was accidentally demonstrated by Cross and Lewis,8 and Swift and Emery9 found that six-week-old babies were even more likely than the newborn to respond inadequately to deliberate nasal obstruction, especially during rapid-eye-movement (R.E.M.) sleep. Upper-respiratory infection in a small baby, though apparently trivial, may thus bear sinister
not
to
implications. Babies may, however, stop breathing without any airway obstruction. Normal infants, and even adults, have striking variations in respiration during R.E.M. sleep,’o and an extension of this irregularity may account for the "near-miss-cot-death" syndrome, in which sleeping babies are found cyanbsed or pale and apnoeic but can be resuscitated. Investigations of this syndrome, which may have a strong familial tendency, indicate that such babies may have frequent and prolonged apnoeic attacks
during sleep, especially
R.E.M.
sleep." Nasopharyngitis
may increase the attacks, but they occur in its absence. The syndrome is strikingly similar to apnoea of prematurity, and the response of the two groups of babies to changes in blood gases bears comparison. Very premature babies have a greatly reduced ventilatory response to rising Pco2, related to gestation (probably due to neurological immaturity) and to age (perhaps due to stiff lungs from retained fluid).12,13 They also have a
characteristic response to hypoxia, which, paradoxically, depresses ventilation. 14 Shannon et al. 15 suggest that nearmiss-cot-death babies also respond poorly to the stimulus of CO2, and showed that 11 such infants had significantly’higher alveolar CO2 and less hyperventilation on breathing 5% CO2 than normal controls, although the response to hypoxia was not measured. This provides strong supportive evidence for the association between chronic hypoxaemia and cot death, as well as a terminal mechanism of death in these babies. It is, however, difficult to rule out the small possibility that their findings were due to acute anoxic brain damage, despite clinical and electroencephalographic normality at the time of the investigations: all the babies had had neuro1. Naeye, R. L. Archs Path. Lab. Med. 1977, 101, 165. 2. Naeye, R. L. New Engl. J. Med. 1973, 289, 1167. 3. Naeye, R. L. Science, 1974, 186, 837. 4. Naeye, R. L., et al. Am. J. Path. 1976, 82, 1. 5. Gadsdon, D. R., Emery, J. L. Archs Dis. Childh. 1976, 51, 42. 6. Naeye, R. L. Am. J. clin. Path. 1976, 66, 526. 7. Peterson, D. R., et al. Am. J. Epidem. 1974, 99, 389. 8. Cross, K. W., Lewis, S. R. Archs Dis. Childh. 1971, 46, 211 9. Swift, P. G. F., Emery, J. L. ibid. 1973, 48, 947. 10. Aserinsky, E. Science, 1965, 150, 763. 11. Steinschneider, A. Pediatrics, 1972, 50, 646. 12. Rigatto, H., Brady, J. P., Verduzco, R. ibid. 1975, 55, 614. 13. Frantz, I. D., et al. J. appl. Physiol. 1976, 41, 41. 14. Rigatto, H., Verduzco, R., Cates, D. B. ibid. 1975, 39, 896. 15. Shannon, D. C, Kelly, D. H., O’Connell, K. New Engl. J. Med. 1977, 297, 747
logical abnormalities for up to 48 hours after an apnceic attack. One interesting investigation would be to compare their responses with those of babies known to have had acute anoxia, such as birth asphyxia (who also frequently have apnoeic episodes). We can also compare these patients with infants with Ondine’s curse, who have an inadequate (or even paradoxical) response to CO2, and become apnoeic during non-R.E.M. sleep.’s These babies breathe normally during R.E.M. sleep, indicating that CO2 drive is not necessary at this time, whereas this is the phase in which near-miss infants tend to have their apnoeic attacks."I Cot death is a "symptom" rather than a disease, and there is at present no way of knowing how often it is due to primary failure of ventilation: even a large prospective study of ventilatory responses in newborn might not give the answer, since respiratory drive may decrease during the first few weeks of life.9 Even when babies at risk are identified, death cannot always be prevented : 5 of 16 such babies in two series subsequently died at home. CHRONIC CONSTIPATION IN CHILDHOOD CHILDREN’S bowels have been an anxious topic for many a year. In the lst century A.D., the aptly named Soranus recommended digital anal dilatation in the newborn infant: "With the little finger whose nail has first been cut short one must for the unhindered passing of the excrements dilate the anus and divide the thin membranous body which is often grown around it".’ Purging was the standard remedy for all ills, and many cultures insisted on a clean-out once a month: Herodotus says that the Egyptians took aperients for three consecutive days in each month. The modern view is more relaxed, but chronic constipation is a condition which wreaks havoc in a child’s life, especially when (as is often the case) it is associated with soiling. In published work on the subject, lack of agreement on nomenclature makes facts hard to distinguish from fancy. The terms encopresis and faecal incontinence tend to be used synonymously; what one mother regards as constipation is of no concern to another. Clayden2 recommends a convention to smooth the passage of ideas: he defines constipation as difficulty or delay in passage of faeces; soiling as the frequent passage of loose or semisolid stools in clothing; and encopresis as the passage of normal stools in abnormal places (including the clothes). The anatomy and physiology3-6 of the anorectal region is complex, and the precise mechanisms for defzecation and faecal continence are not fully understood. The initial desire to defaecate arises from distension of the rectum when faeces descend from the colon. As the rectum is distended the tone of the internal anal sphincter decreases and immediate continence is achieved by contraction of the external sphincter and levator ani muscles. With small rectal volumes the inhibition of the internal sphincter is temporary, but, as rectal distension increases, the internal sphincter remains 16. Shannon, D C, et al. Pediatrics, 1976, 57, 342 1 Soranus, Gynæcology Book 2 (translated by Johannes
Ilberg); p. 83. Balti-
more, 1956. 2. Clayden, G. S. Br. med. J. 1976, i, 515. 3. Lawson, J. O. N.Ann. R. Coll. Surg. 1974, 54, 244, 288. 4. Gowers, W. R. Proc. R. Soc. 1877, 26, 77. 5. Denny-Brown, D., Robertson, E. G. Brain, 1935, 58, 256. 6. Gaston, E. A. Surgery. Gynec. Obstet. 1948, 87, 280.
1065 inhibited and the external sphincter is either relaxed voluntarily or tires and the stool is passed. When rectal distension ceases the internal-sphincter tone returns. In a child, acquisition of the social skill of defalcation takes about two years.7-9 The bowels are not open in utero unless the fetus becomes distressed, but the reason for this is unknown. Reflex defaecation occurs soon after birth, within 36 hours in most babies, but it may be delayed in preterm infants by a mucus plug and in neonates by ileus, an anatomical anomaly of the anus, or Hirschsprung’s disease. Constipation is unusual in breast-fed babies, symptom-free delays of up to 10 days being normal in this group. Bottle-fed babies on mildly hyperosmolar feeds will dehydrate their faeces to provide enough water for urine solute clearance. These babies pass stools with considerable strain. Their reflex response to distension of the anal canal by these hard stools produces such grunting and facial plethora as to alarm the most placid of mothers. Tranquillity may be restored by ensuring adequate water intake and adding, if necessary, some sugar to the feeds. As the child approaches 2 years of age he becomes progressively aware of his ability to control the stool, and normally experiences some pleasure in defaecating in the pot, especially if he is rewarded with parental acclaim. However, this skill may also become a weapon to use against his parents. The negativism of the 2-year-old is frequently shown in pot training, especially if the parents are too aggressive or too effusive about this skill. Delay in acquiring stool control will be expected in children with other evidence of developmental delay. Disturbance may also arise if there are physical abnormalities making defalcation difficult or painful, or if sensation is impaired. True constipation in infancy must be taken seriously, especially if the abdomen is distended. When the difficulty in defalcation is unrelieved by simple measures such as adjusting the milk formula or adding a little milk of magnesia, and hypothyroidism and hypercalcaemia are excluded, an organic cause should be suspected. Rectal examination may detect a stricture, a covered anus, or reveal a gush as the finger is withdrawn characteristic of Hirschsprung’s disease. Sacral anomalies may also be found. Acute constipation often follows a dietary or environmental change, or a febrile illness. Sometimes an anal fissure is found. Most acute constipation settles spontaneously when the child returns to normal diet and fluid intake, but failing this the treatment is to soften the stool and gently stimulate a return to regular defaecation. It is much easier to treat a 7-10-day delay in this way than to evacuate manually under anxsthesia when acute impaction ensues after 2-3 weeks. A suitable stool softener is dioctyl sodium sulphosuccinate; this is best used on its own.lO Standardised senna and danthron are gentle laxatives suitable for children. Chronic constipation, with its frequently accompanying soiling, is more of a problem. Soiling is common; 1-32% of children over age 4 soiled by day with a ratio of 3/1 boys to girls." When Berg and Jonesl2 studied 78
children with functional faecal incontinence
(soiling and were able to definitions) they Clayden’s encopresis by divide the children into four major groups-16 with training problems without severe constipation; 16 with pot-refusal retention syndrome; 16 with severe constipation with overflow; and 30 with uncomplicated functional faecal incontinence passing normal stools (encopresis). They found that the two groups with associated constipation responded to combined oral laxative therapy and simple outpatient psychotherapy. Clayden and Lawson13 studied 106 children with longstanding chronic constipation who had failed to respond to standard medical treatment. Anorectal manometry in these children revealed that 10 had Hirschsprung’s disease (later confirmed by rectal biopsy) and 10 had Hirschsprung’s-like anorectal activity (failure of anal sphincter to relax in response to rectal distension) but normal biopsy appearances with standard histochemical techniques. Of the non-Hirschsprung’s group most showed exaggerated rhythmic activity in the anal canal, a higherthan-normal volume required in the rectum to produce internal-sphincter relaxation, and a reduced sensation of the call to stool. The last two observations would be expected with a megacolon, but the increased rhythmic activity suggested hypertrophy of the internal sphincter. With this in mind, in the non-Hirschsprung’s group they proceeded to dilate the anus forcefully (under ketamine anaesthesia) and were able to show striking improvement in most cases; and, as defaecation became easier, behavioural troubles also tended to abate. The prevalence of previously undiagnosed Hirschsprung’s disease was surprisingly high. Since 4 of the 10 Hirschsprung’s patients had a history of soiling, soiling cannot be used to exclude Hirschsprung’s disease. There have been several cases of Hirschsprung’s disease presenting in adult life and nearly always the trouble started in infancy. 11-20 One reason why such cases are missed in childhood is the mistaken view that chronic constipation should not be interfered with, but that Nature should be allowed to take its course. A complication of Hirschsprung’s disease which can be fatal is necrotising enterocolitis; Hirschsprung’s disease should, therefore, be considered in every case of persistent constipation. Anal manometry is the best screening method, with biopsy in likely cases.14 Fhe research trends are encouraging: improvements in histochemistry have made the diagnosis of ultrashortsegment Hirschsprung’s disease more accurate, since fibres which aganglionic areas have excess stain strongly for acetylcholinesterase;21 work on the nonadrenergic non-cholinergic (purinergic) system will probably shed more light on and possibly lead to more rational pharmacological approaches to the deranged internal sphincter;22 and anorectal manometry is now a safe and reliable investigation. The most encouraging trend is the multidisciplinary approach to the child with constipation. Whatever the original cause of the consti-
cholinergic
13.
7. Isaact, S. in On the Bringing up of Children (edited by J. Rickman); pp. 123, 135, 147, 160, and 165. London, 1936. 8 MacFarlane, J. W., Allen, L., Hanzig, M. A Developmental Study of Behaviour Problems of Normal Children; vol. II, p. 85. Los Angeles, 1954. 9. Douglas, J. W. B., Blomfield, J. M. Children under 5; p. 127. London, 1958. 10. Godding, E. W. Br. med.J. 1976, iii, 838. 11 From Birth to Seven: report from National Child Development Survey; p. 399. London, 1972. 12. Berg, I., Jones, K. V. Archs Dis Childh. 1964, 39, 465.
Clayden, G. S., Lawson, J. O. N. ibid. 1976, 51, 918.
Lawson, J. O. N., Nixon, H. H. J. pediat. Surg. 1967, 2, 544. 15. Whitehouse, F., Bargen, J. A., Dixon, C. F. Gastroenterology, 1943, 1, 922. 16. Rosin, J. D., Bargen, J. A., Waugh, J. M. Mayo Clin. Proc. 1950, 25, 710. 17. Riker, W. L. Archs Surg. 1957, 75, 362. 18. Jennings, P. J. Proc. R. Soc. Med. 1967, 60, 805. 19. Gertz, T. C., Hanson, L. K. Nord. Med. 1966, 75, 325. 20. Todd, I. P. Br. J. Surg. 1977, 64, 311. 21. Meier-Ruge, W. Curr. Topics Path. 1974, 59, 131. 22. Burnstock, G. Pharmac. Rev. 1972, 24, 509. 14.
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pation, the emotional factors
must be taken into Work such as the combined St. Thomas’/Great Ormond Street study, designed to assess the psychological and physiological factors, will define more accurately the aetiology and ideal management of this unpleasant problem of childhood. account.
PREMEDICATION TRADITION PREMEDICATION is dominated by tradition. The principles of our use of preoperative drugs survive from the days of ether anxsthesia when the euphoria and sedation of morphine were invaluable in helping the patient
the distress and excitement of the early induction stages and atropine or hyoscine was needed to control the salivary and bronchial secretions induced by ether. Barbiturates, phenothiazines, and tranquillisers were subsequently introduced over the years to replace or supplement the opiates. Now that induction is intravenous in most cases, is any form of sedative premedication necessary, unless a patient is particularly anxious? A preoperative visit by a sympathetic anaesthetist is often the best way of allaying the natural fear that most people have of losing consciousness. Though opinion is divided, injection of an antisialogogue is probably still a useful precaution about an hour preoperatively to avoid the complications of laryngeal irritation by oral secretions. In an effort to promote more rational choice of premedicant drugs, Forrest and others’ have investigated six commonly used premedications-two opiates, two barbiturates, an antihistamine, and a minor tranquilliser. Drugs were administered randomly from identical syringes to 509 patients, in whom subjective responses were scored on 0-9 scales 30 and 60 minutes after premedication. Independent assessments were made by nurse-observers and overall evaluations were recorded by the anxsthetists themselves. The observers tended to credit the antihistamine and the minor tranquilliser with an ability to suppress apprehension, but the patients thought otherwise. The anxsthetists went one better and rated the placebo as satisfactory in facilitating induction. This surely confirms that the induction of anwsthesia nowadays causes the patient little distress. How do you evaluate tradition?
through
SUPPORT FOR DISABLED HOUSEWIVES WOMEN are not fighting hard enough for their rights, and they have only themselves to blame if they are discriminated against and regarded merely as the dependants of their husbands. Thus, Dr Shirley Summerskill, Under-Secretary at the Home Office, told a conference on discrimination in employment on Oct. 19.2 Her words must indeed have seemed very provoking to one group of women-the disabled housewives, who have had to wait 2 years for the non-contributory invalidity pension accorded them under the Social Security Act of 1975. This Act provides for a non-contributory invalidity pension (N.C.I.p.) for men and women of working age who have been incapable of work for at least 28 consecutive weeks and who have not satisfied the contribution condi1. 2.
Forrest, W. H., Brown, C. R , Brown, B. W. Anesthesiology, 1977, 47, 241. Times, Oct. 20, p.6.
tions for sickness and invalidity benefit. Payment of N.C.I.P. for men and single women started on Nov. 20, 1975, and meant little extra charge on Treasury funds, since most claimants were already receiving the money
supplementary benefit. Housewives were originally excluded altogether from the draft legislation considered by Parliament: for them extra money would be needed, since they previously received nothing. Under pressure the (Labour) Government gave way on this point, but the difficulties of identifying the housewives entitled to benefit, and of verifying their claims, were given as reasons why payment of the housewives’ pension (H.N.C.I.P.) should be deferred. The scheme started on Nov. 17, and it is inequitable. The married woman will lose out if her husband is not working, for the value of her pension will be deducted from any sickness or other benefit he receives; and the 2-year delay has meant that some women have been cut out of the scheme altogether, since claimants must be under 60 to qualify (though benefit may continue beyond 60 if the claim is made before reaching that age). But its worst aspect is the claims procedure. Men and single women claim their N.C.I.P. on a short form which asks only for the essential information such as the date when incapacity for work started and the number of dependants being claimed for. On her form the housewife applicant is required to supply information on, among other things, the kind of accommodation she lives in, what adaptations have been made for her, what appliances are available and what help she needs to use them, how far the family or others contribute to doing the household jobs, and whether she is fit to do any paid work and if so to suggest what kind of work this might be. The discrimination does not end with the form, for the Department also requires special evidence to support the claim. A circular to general practitioners which went out in September explains that, while evidence on claims from men and single women normally takes the form of a doctor’s statement on form Med 3, for married women "the need to cover in some detail the physical and mental functions involved in household duties makes it impossible to rely on the use of form Med 3". Doctors will be invited to provide special medical reports, covering the housewife’s fitness for paid work, and her ability to perform household duties; moreover, completion of the form, the circular says, will normally involve a medical examination in the claimant’s home. A fee of 00 for these reports has been agreed with the B.M.A. Originally the Department intended that its own appointed doctors should carry out these- examinations, but it has now conceded that the patient’s own G.P. shall do them. Claims for mobility and attendance allowance have always had to be supported by evidence from a medical examination. But it is difficult to understand why a doctor who is thought capable of judging a person’s fitness to work without observing him actually doing it is yet incapable of similarly judging a woman’s fitness to carry out normal household duties. The Department of Health must have some means of checking dubious claims, but the procedures already in operation for N.C.I.P. would surely do. The system for claiming H.N.C.I.P. is cumbersome and expensive; it wastes medical time and it discriminates against married women. The housewives could have been incorporated into the existing scheme simply by amending the claim form and doctor’s statement. as
