Further Follow-up Study of Early Operation for

I 196062

Total number born 203 Perinatal deaths 73 Not operated, died later 30 Operated 100 Long-term survivors 62.

Open Myelomeningocele

1963-65 Total

147 350 40 113 10 40 97 197 71 133.

T. MA WDSLEY and P. P. RICKHAM

Early Surgery The percentage of children who were referred for surgery has considerably increased

and in the second three-year period only 10 children (just over 7 per cent) of those surviving the first few hours of life were not operated upon. So although the total number of children reported in this second period has decreased, the total number of cases operated upon has remained approximately the same for the two three-year periods.

Although a larger proportion of the children were operated upon in the second period, the physical and mental abilities of the survivors have not diminished; they have remained virtually the same as in the first group, or may even be a little better (Table 11).

It is interesting to note that only one of the operated children previously reported as living

Alder Hey Children’s Hospital, West Derby, Liverpool. 8

TABLE 11 Physical and mental abilities of 133 survivors

I 1960-62

Normal or near normal 26 Major handicaps (Tab. 111) 29 Physically normal, E.S.N. 1 ‘Vegetative’ 5*

196365 Total

30 56 34 63 2 3 6 11

*1 died since 1965.

in the 1960-62 series has since died, and this child was one of the very severely mentally handicapped.

It appears, therefore, that the last column in Table I1 indicates the true proportions of handicaps in a given population if early operation is employed in most children with myelomeningocele.

TABLE 111 Major handicaps

1960-62 1963-65 Total

Bladder only Limbs only Limbs and bladder 15 22 37 Physical Handicap and E.S.N. 6 6 12

TOTALS 1 29 I 34 1 63 1 Types of Handicap

A breakdown of the severe handicaps is in Table 111. The follow-up study of the first series revealed that there had been no change in the type of handicap previously reported. There is no significant difference in the types of major handicaps between the first and second studies. It can therefore be accepted that some 63, or almost 50 per cent, of the long-term survivors will suffer from severe physical handicaps and that the types of handicaps will follow the pattern shown in the last column of Table 111.

In an attempt to study the degree of physical handicap in mentally normal children, we have, as in the previous study, examined locomotion and continence handicaps separately.

Locomotion Table IV breaks down the major locomotion handicaps according to the PULTIBEC

system (Lindon 1963), which we still find a most useful method of classification. A com- parison of the first two columns in this table shows that there has been a very noticeable improvement in the degree of handicapping in the 1960-62 children when they were re- examined three years later. This reflects on the efficiency of orthopaedic management and further training. It is striking that the children born in the 1963-65 period are already less handicapped than the children in the first group, and our past experience with the first group makes it likely that their handicaps will further improve during the coming years.

Continence As can be seen in Table V, there has been a considerable improvement in the degree of

continence of the first group of children since the first report three years ago. This indicates 9

TABLE 1V %]or handicaps: locomotion

Able to use limbs normally Able to walk and run Walk reasonably with crutches Walk 20-200 yards with aids Stand only with support Chair or bed only

I 1960-62

0 0 0 1 1 1 7 14 9 6 6 1

1 1st Report 1 Now

TOTALS 1 23 23

1963-65

0 1

12 10 4 1

28

TABLE V Major handicaps: continence

I 1960-62

I 1st Report

Continent Continent but enuretic Continent with appliances or

special training Manipulative difficulty, other-

wise continent Intermittent incontinence Double incontinence

I 23 TOTALS

Now

6 1

10

5 0 1

23

1963-65

3 1

2

11 4 7

28 - that with training, operation and appliances, most of these children should ultimately become continent (i.e. Grade 3).

The present state of continence of the 1963-65 children is not much different from that of the earlier group when first reported, but our policy is to provide appliances earlier and this accounts for the larger number of children in the ‘manipulative difficulty’ group in the second series.

I.Q. Levels and School Placement A histogram of all the 133 children discussed in this paper is shown in Figure 1. Our

investigations showed that, on re-examination of the first group of children, there had been no great change in the I.Q. The combined histogram is not, therefore, radically altered from that of the first group which we published three years ago; the only difference is that there were slightly more children in the previous histogram with 1.Q.s between 100 and 119 than between 75 and 99, whereas this position is now reversed. I t appears, therefore, that estima- tion of the I.Q., imperfect as it may be, remains broadly the same as it was in our first report.

Table VI shows the school placement of these children. There are no changes in the placement of the 1960-62 group in the various schools, reflecting the accuracy of the evaluation of these children by the school authorities. The school placement of the second group of children has virtually been the same as the first group, therefore the Table shows only the combined school placement of both groups.

10

TABLE VI Schooling (no change in 1960-62 children)

Normal school

‘Vegetable’ Ineducable

50-74 E - W O O - ~ ~ ~ 1 2 0

Discussion In our previous paper we stated that, with early operation, 49 per cent of the children who

had survived for the first 24 hours would survive for long periods and we predicted that these results could be improved to about 60 per cent. This has been confirmed by our 1963-65 series; 71 of the 107 children who survived the perinatal period are still living. Of the operated cases, 70 per cent have survived; approximately half of the deaths occurred shortly after operation and the other half occurred after discharge from hospital.

It appears that advances in the paediatric and surgical care of these infants have resulted in an improvement in the quality of survival. Although the actual percentage of handicapped children remains unchanged at about 57 per cent, a more detailed analysis of the handicaps reveals that, because of more efficient orthopaedic care, the children with locomotor handicaps in the second series are not so severely affected as those in the first series and that even the latter have improved considerably during the last three years. There is good reason to expect further improvements, although not on the same scale.

The picture is rather similar with incontinence handicaps. We feel that the earlier use of more sophisticated appliances will result in still further improvements and that it should ultimately be possible to control faecal and urinary incontinence in virtually all children of normal intelligence.

Only about 7 per cent of the children have such deformed or damaged brains that they must lead a ‘vegetable’ existence. Just over 10 per cent are educationally subnormal. In the remainder, the I.Q. is not much different from that of the general population. However, intelligence tests on the children in the first series confirmed an observation by Laurence and Coates (1962). Intelligence in this group was first tested between 3 and 5 years of age by the Terman-Merrill method. Much more detailed examinations, mainly by the Wechsler method, were undertaken after the children had been in school for periods of between two and four years. These have revealed that although the total I.Q. has changed very little,

11

there is a group of children in whom a considerable discrepancy is observable between verbal and perceptual quotients. These findings will be reported in greater detail by our colleague Dr. R. Burns, in a future paper, as will the results of the attempt by the Liverpool school authorities to overcome this handicap by special teaching methods.

Conclusions Guthkelch (1965) predicted that 50 to 60 per cent of all live-born spina bifida infants will

survive to adult life and our figures seem to confirm this prediction for children born with open myelomeningocele. At present this success rate will only be possible in the large conurbations, because of the need for concentrated services. We admit children from a population nearly five times as big as that of Liverpool itself but the admissions from the outlying areas are not, as yet, as concentrated as those from within the city.

When he predicted that only 25 per cent of the survivors would be educable by normal methuds, Guthkelch was perhaps being unduly pessimistic; our figures indicate that this number is now approximately 40 per cent. There will be a group of some 7 per cent for whom society must provide special accommodation on a life-long basis.

The important point is that just over 50 per cent of the survivors will need special schooling facilities and as the large majority of these are of virtually normal intelligence, the community

,must betmade aware of its responsibility to provide adequate facilities to allow these children to develop their abilities to the full. In many parts of this country these provisions are, as yet, not fully established.

SUMMARY A survey of all children born with open myelomeningoceles in the city of Liverpool

between the years 1960 and 1965 is presented. Over 80 per cent of the children who survived the first 24 hours came to surgery and 70 per cent of these children are now surviving to school age and beyond. An analysis is made of their physical and mental handicaps.

RESUMI~ Les auteurs prksentent une Ctude de tous les enfants nCs avec un myClomCningocde ouvert,

A Liverpool, durant les annCes 60 il65. Plus de 80 pour cent des enfants ayant survCcu au deld des 24 premibres heures ont CtC opCrCs et 70 pour cent d’antre eux ont atteint rage scolaire ou au-delli. Les auteurs prksentent une analyse de leurs handicaps physique et mental.

ZUSAMMENFASSUNG Eine Verlaufsstudie aller in Liverpool zwischen 1960 und 1965 geborenen Kinder mit

offener Myelomeningocele wird vorgelegt. Uber 80 prozent derjenigen Kinder, welche die ersten 24 Stunden uberlebten, wurden operiert, und 70 prozent dieser Kinder uberlebten bisher bis ins Schulalter und darunter. Ihre physischen und geistigen Beschrankungen werden ausgewertet.

REFERENCES Guthkelch, A. N. (1965) ‘Thoughts on the surgical management of spina bifida cystica.’ Actu neurochir.

Laurence, K. M., Coates, S. (1962) ‘The natural history of hydrocephalus.’ Arch. Dls. Childh., 37,345. Lindon, R. L. (1963) ‘Pultibec system for medical assessment of handicapped children.’ Develop. Med.

Mawdsley, T., Rickham. P. P., Roberts, I. R. (1967) ‘Long-term results of early operation of open myelo-

12

( Wien), 13,407.

Child Neurol., 5 , 125.

meningoceles and encephaloceles.’ Brit. med. J., i, 663.