PROGNOSIS FOR BABIES WITH MENINGOMYELOCELE AND HIGH LUMBAR PARAPLEGIA AT BIRTH

993 had intestinal obstruction. Terminal intestinal obstruction occurred in some 10% of hospice patients with primary tumours of the large bowel. It is thus a fairly common terminal event, but the findings reported here indicate that the distressing symptoms of intestinal obstruction due to far-advanced malignant disease can be

3% of all

patients

controlled by drugs.

4.

5. 6. 7.

We thank Mr A. E. Young, consultant surgeon at St Thomas’ Hospital, London, for help with the preparation of this paper; and the Wellcome Foundation for financial support.

Correspondence should be addressed to M. B., St Lawne Park Road, Sydenham, London SE26 6DZ.

Christopher’s Hospice,

8. 9. 10. 11.

Surg 1973; 125: 316. pathological aspects

12. Carter RL. Some

REFERENCES 1 Sise JG, Crichlow RW. Obstruction due to malignant tumours. Sem Oncol 1978; 5: 213-24. 2 Osteen RT, Guyton S, Steele G, Wilson RE Malignant intestinal obstruction Surgery 1980; 67: 611-15.

Child Health PROGNOSIS FOR BABIES WITH MENINGOMYELOCELE AND HIGH LUMBAR PARAPLEGIA AT BIRTH R. G. MENZIES

J.

M. PARKIN

E. N. HEY Princess Mary Maternity

Hospital,

Newcastle upon

Tyne

life

expectation of babies with lumbar meningomyelocele not paralytic offered immediate surgery at birth appears to be influenced by the extent to which parents are involved in the child’s early care. 8 of the 27 children offered family-centred care in one hospital in 1971-80 and not offered immediate surgery survived to school entry and none of these children has since died. All are chairbound and incontinent, but none is intellectually retarded and many are no more handicapped than the children offered immediate surgical treatment at birth. The choice before the family at birth does not have to be presented as an urgent and immediate choice between life and death. Summary

The

JC, Buchler DA, Mack EA, Ruzicka FF, Crowley JJ, Carr WR. The management of ovarian-cancer-caused bowel obstruction. Gynecol Oncol 1981; 12: 186-92. Oliver DJ. The use of methotrimeprazine in terminal care. Br J Clin Pract 1985; 39: 339-40 Baines MJ. Control of other symptoms. In: Saunders CM, ed. The management of terminal malignant disease. London: Edward Arnold, 1984. Regnard C, Newbury A. Pain and the portable syringe pump. Nursing Times 1983; 79: 25-28. Walsh HPJ, Schofield PF. Is laparotomy for small bowel obstruction justified in patients with previously treated malignancy? Br J Surg 1984; 71: 933-35. Ketcham AS, Hoye RC, Pilch YH, Morton DL. Delayed intestinal obstruction following treatment for cancer. Cancer 1970; 25: 406-10. Piver MS, Barlow JJ, Lele SB, Frank A. Survival after ovarian cancer induced intestinal obstruction. Gynecol Oncol 1982; 12: 44-49. Aranha GV, Folk FA, Greenlee HB. Surgical palliation of small bowel obstruction due to metastatic carcinoma. Am Surg 1981; 47: 99-102. Glass RL, LeDuc RJ Small intestinal obstruction for peritoneal carcinomatosis. Am J

3 Tunca

INTRODUCTION

BEFORE the introduction of the Holter valve twenty-five years ago, the prognosis for babies with serious spina bifida was extremely bleak. A policy of immediate back closure with subsequent shunt surgery for the hydrocephalus then developed, but this soon led to the survival of large numbers of very handicapped children. In turn, therefore, a policy of more selective treatment was widely adopted in the United Kingdom after 1971,’ in the belief that children with gross

of advanced malignant disease. In: Saunders CM The management of terminal malignant disease. London: Edward Arnold, 1984. 13. Carter RL, Pittam MR, Tanner NSB. Pain and dysphagia m patients with squamous carcinomas ofthe head and neck-the role of perineural spread. J Roy Soc Med 1982; 75: 598-606.

in several other countries.7-9 A working party for the Northern RHA believed it was possible to reach a consensus on such issues when it reviewed the ethical guidelines involved and published its views in The Lancet ten years ago,1O but this conclusion was based on the assumption that babies not offered surgery would always die within weeks or months if not offered surgery at birth. Evidence, however, from cases selected for referral to a regional surgical centre after delivery"’’shows that this is not always true. Our own experience (based on an unselected group of children) suggests that 30% of all children not offered surgery at birth may survive to enter school 5 years later, and that they enter school with a range of handicap (other than the handicap of wheelchair dependence) little different from that ofa group of children without "adverse criteria"’offered surgery at birth.

SURVEY

Families who are told that their baby is unlikely to survive very long, and that skilled nursing care (with or without medication) may be necessary to keep the baby comfortable, readily accept the idea that their child should remain in hospital. This was not the way the first few babies were managed conservatively in this hospital, however, when a policy of selective treatment for early surgery was introduced in 1971. Three of the first families offered conservative care chose to take their babies home, and nursery staff rapidly came to see from the outset that this could be a valid alternative to continued hospital care. Parents have always, therefore, been encouraged to involve themselves in the child’s care in hospital from birth and have had the alternatives explained to them without pressure to conform to any particular pattern of care, and we soon

hydrocephalus, paralysed legs, or kyphosis at birth would die anyway if not offered immediate surgery; and this is, indeed, what normally happens when such children are cared for in

hospital.2-6 This was not, however, what happened to a group of such children cared for in a family setting in Newcastle between 1971 and 1980. The ethical dilemma facing those responsible for the care of

baby with high paraplegia at birth remains as acute as ever: clinicians in the UK advise against surgical treatment for such children, but this runs counter to the prevailing view a

most

Prognosis for babies with a meningomyelocele not offered surgery at birth between 1971 and 1980 because of adverse factors.!

994 not take their baby home early round to requesting discharge home nearly always (sometimes, initially, on a trial basis) if the baby was still making satisfactory progress 2-4 weeks after delivery. We have now, therefore, ascertained the outcome of every pregnancy since 1971 that resulted in the birth of a liveborn baby with meningomyelocele at this hospital. 1 baby with proven trisomy 18 and 1 baby with renal agenesis were excluded from further consideration. All the long-term survivors and their families were then reviewed by a doctor and were seen by a medical social worker not previously known to the family. Particular attention was paid to the outcome for those children not offered surgical treatment at birth, and comparable data were obtained from six of other maternity units in the Midlands3and North of England (none of which offered early surgery to more than 40% of the liveborn babies with meningomyelocele in the years in question) (figure). We report here the outcome for babies born between 1971 and 1980.

found that those families that did came

have been no deaths, old.

as

yet, in children more than three years

Outcome In the light of these findings an assessment was undertaken of all the long-term survivors (table). 3 children had behaviour problems requiring psychiatric treatment, and cortical blindness developed in 2 after their shunts became blocked. The Ichildren who were offered early surgery are all still alive, but 8 are incontinent and 7 can walk only with calipers or crutches. The 8 children who were not offered surgery at birth and who survived are all chairbound. 1 has OUTCOME FOR LIVEBORN BABIES WITH MENINGOMYELOCELE

RESULTS

Survival 11 of the 38 babies born at this hospital were offered early surgery and all survived. 10 had no severe adverse criteria at birth but 1 was operated on (in 1971) at the request of the

parents despite the presence of overt hydrocephalus and a high lumbar paraplegia. The 8 babies with hydrocephalus were all offered shunt surgery within two weeks of birth. 27 babies were not offered early surgery. All these babies (with one exception) had a high lumbar paraplegia (higher than the 2nd lumbar vertebra, L2) and several also had other adverse criteria. The exception was a baby with satisfactory motor function down to L3 whose parents declined surgery despite advice and who died with bronchopneumonia at six months. None of the babies managed conservatively in the units where it was traditional to offer long-term hospital care survived six months, and most were dead within a month, but more than a third of the babies not offered surgery at birth born at this hospital over the same period survived for at least two years, and 30% survived to enter school (figure). In the absence of any alternative evidence we attribute the difference to the active involvement of the family in the child’s care from the outset, and to possible differences in the use of analgesia and sedation. It might have been thought that the high survival rate among the babies treated conservatively was due to an early change of expectation amongst those caring for the baby. One baby whose lumbar lesion closed particularly quickly was given a Rickham reservoir at 6 weeks because of symptomatic hydrocephalus, but no baby was offered shunt surgery within the first 10 weeks of life, and 85% of the babies born in units that do not normally proffer home care were already dead by then. The spinal lesion was left to close spontaneously over the first few months of life, and shunt surgery was only undertaken an average of 38 (range 10-130) weeks after birth after cerebrospinal fluid loss from the back had virtually ceased. Increased pain and distress from raised intracranial pressure and progressive unsightly hydrocephalus were the usual indications for surgery. Antibiotics were never used before surgery; neither were any other drugs used in the first few months of life (except to relieve pain in a few dying children). 10 of the children conservatively managed at birth were finally offered surgery, and 5 of these were offered cosmetic surgery for the lumbar lesion as well as a shunt. 2 died sixteen and twenty-seven months later when it was decided to withhold further surgery after shunt complications developed, but the remainder survived. There

*Naturally continent without bladder catheterisation. CNS = central nervous system;

recourse to

ureteric diversion

or recurrent

CSF = cerebrospinal fluid.

severe scoliosis, and 2 have renal disorders as well as incontinence, but in other respects they are not really any

than the children who were offered birth. surgery On their own assessment, the parents of the surviving children who had been treated conservatively at first were coping as adequately as the parents of the children offered early surgery. Most had transport of their own (which was considered a necessity), and most lived in accommodation that was purpose-built or well adapted to their child’s needs. Their only comment in this respect was that it had always taken a very long time to get the adaptations made. 2 mothers commented that they had taken many weeks to come to terms with the birth of their handicapped child, and 2 fathers commented that the scale of the task before them had only really dawned when their child was about two years old. All the parents readily accepted at interview that further practical and emotional problems would arise at some stage, but none seemed to feel that these would prove unsurmountable. more

handicapped at

995

Similar comments were made by the parents of some of the children offered immediate surgery. The children themselves showed no obvious signs of frustration or anger at their disability, and the siblings all seemed well adjusted and There was some some of the children were secure.

parental concern, however, that reaching their full educational

not

potential.

-

COMMENT

Survival among children initially treated conservatively in

study was much higher than in most other published studies2-6 other than in the selective series reported from the surgical unit in Liverpool in 1975." Overall survival at two years was comparable with that found in Sheffield in 1971 at a time when every baby was offered surgery at birth (50%),’and higher than in the studies reported from Edinburgh (46%)5 this

and Sheffield (34%)6 after the introduction of a more selective

approach

to

surgical

intervention. We

were

concerned,

determine how much additional handicap was associated with this increased survival. 84% of our surviving children were incontinent-a similar proportion to that found in Edinburgh (85070)5 but more than that found in the Sheffield study (45%)—and a much higher proportion of the surviving children were wheelchair dependent. Some differences in continence, and striking differences in mobility, were seen between those offered immediate surgery at birth and those managed conservatively in the present study, but there were few other differences between the two 2,13 groups. Recently published data from Cardiff’ point in the same direction, but only half the surviving children not offered early surgery in Cardiff were of normal intelligence. None of the children treated conservatively at birth in the present study were educationally handicapped, but none of the survivors had been born with any of the adverse criteria listed by Lorber other than a high lumbar paraplegia, and this may account for some of the difference in outcome. Comparisons are hampered by the fact that the Cardiff results relate to patients selected for referral to a specialist centre for management, and by the inclusion of children with meningocele and encephalocele in the group offered immediate surgery. All the families in this study seemed to have adapted and responded positively to the challenges presented to them. There will be those who say nevertheless that, however acceptable the immediate outcome, the long-term prognosis for these children when they enter their teens is likely to be so grim that our current management policy must be wrong. Yet it is questionable whether the long-term outlook is any grimmer than that facing children with progressive neuromuscular diseases such as muscular dystrophy. With these conditions society seems to have agreed that a happy and purposeful childhood is an end worth striving for in its own right, however bleak the long-term prognosis. The eventual outlook for children with spina bifida must depend, in any case, on the extent to which the necessary services are made available to patients beyond school leaving age.14 The measure of life’s value is not made in years. It is best expressed in the fostering of caring human relationships. 15 In our experience children with meningomyelocele not offered surgery do not always die within a matter of weeks if cared for in a family setting. The general belief that life will be short’" has, we believe, generated pressure that tends to ensure that reality matches expectation. Parents who are led to believe that death is inevitable within a few weeks certainly

therefore,

to

find it very difficult to readjust when this belief turns out to be ill-founded. The belief that decisions regarding surgery must be made within a matter of hours to prevent further neurological deterioration is not supported by the evidence’2,16 but it still tends to result in options being presented in a polarised way-any decisions then being treated as irrevocable. The equanimity with which clinicians in the UK have decided against offering any treatment to some children is not always matched elsewhere.’-9 In addition, the prognosis for children today cannot necessarily be adduced from data collected on children born fifteen to twenty years ago, and the long-term prognosis for children with adverse criterial who survive despite conservative management at birth may not be adducable from data collected at a time when all children received active surgical treatment from birth. Close family involvement seems to help, rather than hinder, the grieving process:" if such involvement results in the family then asking to have the child home, who are we to discourage such a move? The idea that every child with a meningomyelocele should be cared for according to one of two quite distinct and divergent management plans could lead all too readily to the idea that, since some children are going to die anyway, their death, too, should be actively "managed". Situations still exist, however, where medical activity has to give way to medical inactivity. We are grateful to medical colleagues for access to case records; to the staff of the Spina Bifida Clinic at Newcastle General Hospital, where all the surviving children were eventually referred for continuing skilled care and support; and to Mrs Fiona Southern for her skilled and sympathetic assessment of the social problems encountered by the long-term survivors and their families.

Correspondence should be addressed to J. M. P., Children’s Clinic, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP. Reprints will not be available. REFERENCES 1 Lorber J Results of treatment of myelomeningocele Devel Med Child Neurol 1971; 13: 279-303. 2 Gross RH, Cox A, Tatyrek R, Pollay M, Barnes WA Early management and decision making for the treatment of myelomeningocele Pediatrics 1983; 72: 450-58 (see also correspondence vol 74, pp 162-67). 3 Richardson SA, Goodall J. Trends in the management of spina bifida babies 1971-1981: home care for non-surgical group Matern Child Health 1984; 9: 252-57 4 Hide DW, Parry Williams H, Ellis HL The outlook for the child with meningomyelocele for whom early surgery was considered inadvisable. Devel Med Child Neurol 1972; 14: 304-07. 5 Stark GD, Drummond M Results of selective early operation in myelomeningocele Arch Dis Child 1973; 48: 676-83. 6. Lorber J, Salfield SAW Results of selective treatment of spina bifida cystica Arch Dis Child 1981; 56: 822-30 (see also correspondence vol 57, p 321). 7. American Academy of Pediatrics. Committee on Bioethics Treatment of critically ill newborns. Pediatrics 1983; 72: 565-66. 8. Freeman JM. Early management and decision making for the treatment of myelomeningocele: a critique. Pediatrics 1984; 73: 564-66. 9. Australian College of Paediatrics. Working Party Report. Non-intervention in children with major handicaps. Aust Paediatr J 1983; 19: 217-22 (see also pp 208-16). 10. Newcastle Regional Health Authority. Working Party Report Ethics of selective treatment of spina bifida Lancet 1975, i: 85-88. 11. Robards MF, Thomas GG, Rosenbloom L. Survival of infants with unoperated myeloceles. Br Med J 1975; iv: 12-13. 12. Evans RC, Tew B, Thomas MD, Ford J. Selective surgical management of neural tube malformations. Arch Dis Child 1985; 60: 415-19. 13. Tew B, Evans R, Thomas M, Ford J. The results of a selective surgical policy on the cognitive abilities of children with spina bifida. Devel Med Child Neurol 1985; 27: 606-14. 14. Rickwood AMK, Hodgson J, Lonton AP, Thomas DG. Medical and surgical complications in adolescent and young adult patients with spina bifida. Health Trends 1984; 16: 91-95. 15. Goodall J. Ethical dilemmas in neonatal care. Mater Child Health 1984; 9: 276-79. 16. Charney EB, Weller SC, Sutton LN, Bruce DA, Schut LB. Management of the newborn with myelomeningocele: time for a decision making process. Pediatrics 1985; 75: 58-64. 17. Dorner S, Atwell JD Family adjustment to the early loss of a baby born with spina bifida. Devel Med Child Neurol 1985, 27: 461-66