RESULTS OF TREATMENT OF 171 CONSECUTIVE MYELOMENINGOCELES—1963 TO 1968

(1)

RESULTS

OF

TREATMENT

OF

171 MYELOMENINGOCELES-

I 963

CONSECUTIVE

TO

1968

Supported in part by Children’s Bureau, Department of Health, Education and Welfare, Washington,

D.C., and the Division of Maternal and Child Health, Pennsylvania Department of Heabth,

Harris-burg, Pa.

ADDRESS FOR REPRINTS: (M.D.A. ) 1740 Bainbridge Street, Philadelphia, Pennsylvania 19146.

PEDIATRICS, Vol. 50, No. 3, September 1972

DIAGNOSIS

AND

TREATMENT

Mary D. Ames, M.D., and Luis Schut, M.D.

From the Departments of Pediatrics and Neurosurgery, Children’s Hospital of Philadelphia

and the University of Pennaylvania School of Medicine

ABSTRACT. Of the 171 children with

myebo-meningocele referred to the Children’s Hospital of Philadelphia in a six-year period, 33 died, 23

were not available for follow-up, and 115 have

been followed for three to eight years. In the 79

children whose hydrocephalus required shunt

operation, 42% with thoracal lumbar lesion and

80% with lumbar and lumbosacral lesions are

am-bulatory and competitive with a Developmental

or Intelligent Quotient of 80 or higher. In the

35 children not requiring such surgery, 90% are

ambulatory and competitive. These results have

been obtained by employing a simple but

com-prehensive and coordinated plan of treatment

shared by the neurosurgeon and pediatrician with

extensive use of paramedical personnel in the

actual care of the child. Other doctors are used

only as consultants and not in primary

decision-making. Because no infallible criteria for deter-mining potential at birth have evolved, all

chil-dren are operated upon to close the defects and relieve the hydrocephalus.

Pediatrics, 50 :466, 1972, MYELOMENINGOCELE

MANAGEMENT.

EDITOR’S NoTE: Although not a problem

o1c diagnods nor, fortunately, a common one

of treatment, the newborn infant with a

myelomeningocele confronts the

pediatri-cian or hospital wit/i serious problems in

management, not onl/ immediately but

of-ten for years thereafter. Therefore, this

ac-count of what can be done for such babies

and their families fullq deserved

appear-ance under the DiagnosLs and Treatment

heading.

C

HILDREN with myelomeningocele may have many physical problenis con-nected with the defect. The primary diffi-culties are hydrocephalus, loss of bladder and sphincter control, and varying degrees of decreased sensation and motor activity of the lower extremities. Complications of the primary disabilities include eye muscle im-balance, blocked shunts, infections of the blood stream and/ or of the meninges, in-fections of the urinary tract, deformities of the lower extremities, fractures, trophic ul-cers, and obesity. Mental retardation may

also occur.

It is a formidable task to provide cane for

a child threatened by these many problems and question has been raised whether or not all children born with

myelomeningo-cele should be treated. Most authorities agree that myelomeningoceles in children with minor difficulties should be operated upon soon after birth.13 But what criteria should determine the exact dividing line between those suitable for surgery and the others?

This paper describes a simple but com-prehensive and coordinated plan of

treat-ment for all children with niyelomeningo-cele, regardless of the extent of the lesion, and reports results of such a plan in 171 consecutive admissions. Only two doctors are directly intimately involved in the care of the children. The primary doctor is the neurosurgeon. The coordinating doctor is the pediatrician. They are assisted by a

so-cial worker, Public Health Nurse, physical

therapist, onthotist, and psychologist. We believe the success of the program depends

on total interaction of this group of inter-ested professional people.

(2)

TABLE I

FOLLOW-UP OF 171 CHILDREN ADMITTED TO THE MYELOMENINGOCELE PROGRAM 1963 TO 1968

* Three institutionalized, twenty moved out of area.

TABLE II

467

at The Children’s Hospital of Philadelphia

for definitive surgical treatment. In all, the myelomeningocele is repaired as soon as possible but not as an emergency proce-dure. Subsequently, the head is measured regularly and, if it enlarges rapidly, studies are instituted to determine the type of

hy-drocephalus. Within a week of the diagrio-sis, the hydrocephalus is treated with yen-triculoatrial shunting and a low pressure Holter valve. If the myelomeningocele is so

large as to make repair difficult the

shunt-ing procedure precedes the repair.

From the time of admission to the

hospi-tal the pediatrician oversees the child’s

gen-eral health, with particular attention to the

genitourinary system. The social worker

in-terprets and explains the diagnostic and

therapeutic procedures to the family and

becomes their confidant regarding fears and tension relative to the birth of a child with

a congenital defect. The Public Health Nurse instructs them in the care of the baby, including the manual expression of urine from the bladder in a manner similar to the method Cred#{233}described for empty-ing the uterus of the placenta. She also con-tacts the Public Health Nurse in the child’s district to outline a plan of continuing care

including nutrition and weight control. The

physical therapist devotes herself to the problems of muscle impairment and

possi-ble deformities of lower extremities. She shows the hospital nurses and parents how

to swaddle the baby with his legs in neutral

I,evel ofLe.rion Ae!ie

Follow-up Lost’ Died Total

Thoracoluinbar 34 (.54%) 8 (11%) (35%) 64

Lumbosacral 75 (7.5%) 15 (15%) 11 (11%) 101

Sacral 6(100%) 0 0 6

Totals 115(67.%) S(1S.4%) 38(19.4%) 171

position and also institutes range of motion exercises for all the joints of the lower cx-tremities. Proper swaddling overcomes or prevents contractures when there is muscle impairment.4 Incidentally, a baby with

floppy legs is easier and safer to handle for

feeding and general care when he is swad-dled.

Following discharge from the hospital the child is seen in the Myelomeningocele

Clinic monthly for six months, bimonthly for six months, every four months the see-ond year, and at least every six months thereafter. All children with shunts are seen at every clinic visit by the neurosurgeon. All are seen at every clinic visit by the pedi-atnician, social worker, nurse, and physical

therapist. All children at every visit are evaluated for growth and development, general health and nutrition, urinary tract infection, and status of muscles and joints. Group sessions moderated by the social

A. AGE AT DEATH

Level of Lesion 1 irk. 1 mo. 1-6 mo. 6-12 mo. 1-2 yr. 2-3 yr. 3- yr. 5-6 yr.

Thoracolumbar (2Q) Lumbar and lumbosacral (11)

4 4

3 0

5 4 0

3 2 0

2 2

3 0 0

.

Level of Lesion

B.

.

Pneumonia

CAUSE OF DEATH

‘ Blocked shunt

Lncontrolled

.

n’ithovt

hydroceplialus

. .

rerzsion

Vent rico! itis and

_.

seplzcemla

.

Seizures

Thoracolumbar

Lumbar and lumbosacral

3

C4

6 2

4 1

9 4

(3)

SNo nouwalker is competitive. With 33 deaths and 23 children

unavail-TABLE III

SURGICAL ‘FREATMENT OF IIYDROCEPIIALUS IN

115 CIIIIu1lEN IN ACTIVE FoLiow-uP

.

Level of Lesion Shunted Non-shunted

Thoracolurnbar 31 (91) 3

Lumbar and lumbosacral 47 (62) 28

Sacral I (16w) 5

Totals 79 (68

.

7) 36

worker are held with parents for discussion and solution of mutual problems.

Ambulation is most important in the

mo-ton development of any child, and interest in walking is more variable in those with myelomeningocele. Therefore, the child with myelomeningocele is given braces and crutches when by the Gesell test he reaches a developmental age of 8 to 10 months, which is the usual age for standing in nor-mal children. Early bracing allows the child to become accustomed to the braces and to the upright position and to be ready for walking when the desire to walk occurs.

When the child is not in his braces he is swaddled. Swaddling and early bracing prevent fractures. When a fracture occurs, the braces may be used as a splint. No or-thopedic surgery is carried out from the

time the child is braced until he is walking well. Children living within 50 miles are

in-structed in walking by the physical

thera-TABLE IV

LOCOMOTION AND COMPETITIVE POTENTIAL IN

79 CHILDREN WITH SHUNTS

.

I.evei of Lwon 11.(llktlS

Non-walkers

Competitive Braces

(md Crutches

Braces No .1 p-7111(iflCCs

Tboraeoiumbar

Lumboaacral

Sacral

13(4%) 4(30%)

I

l

8

0 0

8 3

1

9

14

0

Totals 38 (48%) 50 3 3 3

pist in weekly group sessions at the Center.

Those living in more distant areas of

Penn-sylvania are instructed in satellite centers

operated by Easter Seal Societies or

corn-munity hospitals.

To prevent urinary infection children weighing 2,000 gm or more are given acetyl sulfisoxazole

(

50 mg per kilo in two divided doses

)

daily from 36 hours of life. This drug is changed only if urinary infection

occurs with resistant organism. The bladder is emptied by the Cred#{233}method which is

carefully taught to the parents prior to the baby’s discharge from the hospital. Their techniques are routinely checked during

the early clinic visits and whenever a un-nary infection occurs. Intravenous urogra-phy is performed during the initial

hospital-ization, at one year, and every two years thereafter, and more frequently if urinary infection persists. The urologist, consulted

when the urogram shows evidence of stasis and! or infection in the upper tracts, has usually elected treatment with a tubeless cutaneous cystostomy.

The Gesell Developmental Test is admin-istered every 12 months beginning at 6 months of age until 48 months of age; at 48 months of age psychological evaluations are

obtained and are administered every two to three years to determine intellectual status and clarify educational needs.

RESULTS

One hundred and seventy-one children with spinal myelomeningocele were admit-ted to the program in the six years from January 1, 1963, through December 13,

1968. Sixty-four

(

37%) had thoracolumbar

lesions; 101

(

59%) had lumbar and

lumbo-sacral lesions; and 5

(

4%) had sacral

Ic-sions.

As noted by others thoracolumbar lesions were associated with the highest mortality,

sacral with the lowest

(

Table I

)

#{149}56 Overall

mortality was 19.4%. Table II confirms the

findings of other Centers that death occurs

niost often during the first year as a result

(4)

TABLE V

LOCOMOTION AND COMPETITIVE POTENTIAL IN 36 CHILDREN WIThoUT SHrNTS

* No nonwalker is competitive.

TABLE VI

UROLOGICAL STATUS IN 115 CHILDREN

IN AC-FIVE FoLww-uP

A. SPIIINC-FER CONTROL

Thoracolumbar

Lumbosacral Sacral

6 6 0

64 8 1

6 0 0 0

Totals 96(83’) 14(1) 4(4) 1(1)

469

aAs (letermirled by intravenous urography.

able, 115 children have been in active follow-up for three years or longer. Of these two-thirds had required a shunting procedure

(

Table III

),

more frequently needed for le-sions in the thoracolumban area.

Of the 79 children with shunts, 56

(

Table

IV), are ambulatory although 50 require braces and crutches at the present time. The term “competitive” refers to the results of the developmental and!or intellectual assessment. Thirty-eight (48%) are consid-ered competitive with Developmental or Intellectual Quotients of 80 on better. None

of those with shunts but unable to walk has a developmental and!or intellectual level

above 60. Thirty-one of the 79 children with shunts had thonacolumbar def#{128}cts, in the presence of which some have recom-mended that no therapy be instituted.2’3 However, it should be noted that 13 (42%) of this group fall in the competitive range

and that 22

(

71%) are walking with braces

and crutches. Thus, only nine (29%) of the thoracolumban group are nonwalkers. While it is true that more than 50% of the

children with thoracolumbar defects are noncompetitive, one half of these are at least able to walk with the aid of crutches and braces. All but one of the 36 children

who did not require a shunt (Table V) are

walking, 14 (40%) with braces and

crutches, 7

(

20%

)

with braces alone, and 14 (40%) independently.

In spite of the rarity of normal bladder control, the upper-urinary tract can be

pro-tected as shown in Table VI. The policy of the Clinic has been to employ a simple

re-vensible tubeless cutaneous cystostomy to effect drainage in the presence of stasis and! or infection. This allows the patient to share in the decision concerning permanent diversion at a later date.7

Hospital admissions are limited to those for surgery except for an occasional admis-sion for intensive urinary tract antibiotic

therapy. In the 115 surviving children

followed more than three years there have

been 90 orthopedic procedures in 61, 47

urologic procedures in 29, and 119 shunt

re-visions in 52 children. The length of stay on

the Orthopedic Service, the Genitouninary

.

LevetofLesion it (ZILt-Ts.

Non-walkers

Corn peli- Braces and

tire Crutche’ Braces

No .4 p-pliances

Thoracic

Lumbar

Sacral

I

5 13

5 0

I

6

0 0

9

5 1

0

Totals 3 (,90’) 14 7 14 1

Service, and Neurosurgical Service when shunts are revised electively, averages three

days pen admission. Blocked shunts,

in-fected shunts, and ventriculitis obviously require having longer periods of hospitaliza-tion.

DISCUSSION

Among the increasing number of

multi-ply handicapped children who do not die in

early life, it is not always possible to deter-mine potential at birth. Therefore, this

Cen-ten has developed a conservative approach

to the care of the child with

myelomeningo-Normal Tubeless

.4bnormal Sphincter

Level of Lesion Sphincter Cutaneous

Control

Control Ieaieoatomy

Dry with Cred#{234} Wet

Thoracolumbar 1 16 15

Lumbosacral 6 18 37 14

Sacral 0 2 3 1

Totals 7 (6%) t (18’) 56 (50%) 30 (6%)

B. UPPER-URINARY TRACT DETERIORATION’

(5)

TREATMENT OF MYLOMENINGOCELES

cele in which prevention of complications is emphasized, orthopedic surgery is delayed,

and rehabilitation is based on the child’s de-velopmental level. Ambulation is stressed. The child who can walk, even though in the noncompetitive area of mental achieve-ment, is able to do things for himself which the child in the wheel chair cannot do, and the great psychological benefit of this ac-complishment to the patient and his family should not be minimized.

The entire program is home-oriented, with the Myelomeningocele Center work-ing with the family physician who car-nies on longitudinal health supervision. Throughout the child’s development the

Center supports him as an integral member of his family regardless of his age or the cx-tent of his disability. Decisions are individ-ualized to each child and his problem. This relatively conservative program has produced about the same percentage of children who are competitive as have those more aggressive programs in which the

early months and years are given oven to

surgical procedures.2

Bladder control for esthetic reasons is not emphasized until the child becomes con-cerned. It has been the experience of this Center that until school age incontinence need cause no social problem. For the school age child, regular routine emptying of the bladder by mother, school nurse, on the child himself can keep him moderately dry and thereby eliminate the odor which is the primary objection of the school authoni-ties. Through the approach of the Public Health Nurse in the Center to the School

Nurse the identification of these children to their school districts at an early age has made their acceptance in regular school

more assured than waiting until the child reaches school age.

In summary, it has been demonstrated that with the program of conservative,

lim-ited surgical care of 171 consecutive admis-sions to the Myelomeningocele Clinic 33

(

19%) children died; 20

(

11%) children are lost to follow-up; 3 ( 3%) children have been

institutionalized for mental retardation; and

1 15

(57%)

children have been in follow-up

for three to eight years.

We have been unable to determine at

birth which of the children will be

competi-tive and ambulatory. We have, therefore, adopted the program of operating on all children to close the defect and relieve the

hydrocephalus, individualizing the time of the procedure to each patient. The goals of

this approach are to determine the child’s assets and help him realize his full poten-tial. Every effort is made to make the child an ambulatory contributing individual. Our

experience shows that the child with myelo-meningocele can be handled on a develop-mental, preventive, “total child” basis with no more hardship and disappointment to

his parents than results in children with central nervous system deficits from neona-tal insults by anoxia, hyperbilirubinemia, or infection. Parental disappointments are

certainly no greater for the parents of these

children than for those children with Down’s Syndrome, muscular dystrophy, congenital rubella, or other neurological and/or special sensory deficits. Through a

developmental, preventive, total child ap-proach it is possible to teach parents how to provide appropriately for each of their

chil-dren without regard to physical and/or mental disabilities.

REFERENCES

1. Ford, F. R. : Diseases of the Nervous System in

Infancy, Childhood and Adolescence, ed. 5.,

Springfield, Illinois: Charles C Thomas, 1966. 2. Lorber, J.: Results of treatment of

myelomenin-gocele. Develop. Med. Child Neurol., 13:277, 1971.

3. Matson, D. D. : Surgical treatment of

myelo-meningocele. PicmAmics, 42 :225, 1968. 4. Wilison, M. A. : Multidisciplinary problems of

myelomeningocele and hydrocephalus. J.

Amer. Phys. Ther. Ass., 45: 1139, 1965.

5. Smith, E. D. : Spina Bifida and the Total Care

of Spinal Myelomeningocele. Springfield,

Illi-nois: Charles C Thomas, 1965.

6. Lawrence, K. M. : The natural history of spina bifida cystica: Detailed analysis of 407 cases.

Arch. Dis. Child., 39:14, 1966.

7. Michie, A. J., Boms, P., and Ames, M. D. :

Im-provement following tubeless cystostomy of

myelomeningocele patients with hydrone-phrosis and recurrent acute pyelonephritis. J.

(6)

1972;50;466

Pediatrics

Mary D. Ames and Luis Schut

1963 TO 1968

−−