Urological Complications of Spina Bifida in Children

THE JOURNAL OF UROLOGY

Vol. 75, No. 3, March 1956 Printed in U.S.A.

UROLOGICAL COMPLICATIONS OF SPINA BIFIDA IN CHILDREN PABLO A. MORALES, GEORGE G. DEAVER

AND

ROBERTS. HOTCHKISS

From the Department of Urology and Institute of Physical Medicine and Rehabilitation, New York University-Bellevue Medical Center, New York, N. Y.

The future of the child born with spina bifida has improved considerably. In 1918, Frazier1 wrote that eighty to ninety per cent of such children died during their first year and of the small percentage remaining, the majority were mentally defective and physically crippled. The operative mortality was forty to sixty per cent. 2- 5 , Except in the simplest forms of spina bifida, the tendency had been to defer operation, thus dooming these children to almost certain death. However, with improvements in neurosurgical methods and the advent of antibiotics, the majority of these children are now able to survive the critical first year of life. 6 , 7 Rapid strides in the field of orthopedic surgery have made possible the correction of practically all associated deformities in the growing child. As a result of recent advances in rehabilitative therapy, the spina bifida paraplegic child can now be taught to care for his daily needs within the limits of his disability, ambulate with braces and crutches, and have the opportunity to take his place eventually as a self-dependent and productive member of society. 8 Total rehabilitation in this group of patients has, however, been seriously impeded by two factors, namely, 1) urinary incontinence and 2) progressive renal damage. Unsatisfactory control of urination has made these children malodorous, uncomfortable and socially unacceptable. The development of destructive lesions in the kidneys undoubtedly is the major cause of decreased life expectancy. The number of spina bifida children with paraplegia who survive early adulthood, although not accurately known, is probably small. The solution to these problems obviously devolves on the urological member of the team of specialists concerned with the care of these children. A survey of the nature and frequency of the changes occurring in the urinary system of the spina bifida child is therefore warranted; a better understanding of the pathophysiology of such changes may lead to improved methods of therapy. Accepted for publication October 24, 1955. This investigation was supported by a research grant (B-374 C) of the National Institutes of Health, United States Public Health Service. 1 Frazier, C.H.: Surgery of the Spine and Spinal Cord. New York; D. Appleton and Co., 1918, pp. 265-324. 2 Moore, J. E.: Spina bifida with report of three hundred and eighty-five cases treated by excision. Surg. Gynec. & Obst., 1: 137-140, 1905. 3 Cutler, G. D.: End-results in sixty-two cases of spina bifida and cephalocele. Arch. Neurol. & Psychiat., 12: 149-166, 1924. 4 Craig, W. M.: Spina bifida: Preoperative, surgical, and postoperative trea,tment. S. Clin. North Amer., 9: 219-229, 1929. 5 Harmer, T. W.: Spina bifida and allied malformations. Based on operative experience of 34 cases. Boston Med. & Surg. J., 177: 353-357, 1917. 6 Ingraham, F. D. and Swan, H.: Spina bifida and cranium bifidum: A survey of five hundred and forty-six cases. New Eng. J. Med., 228: 559-563, 1943. 7 Schwidde, J. T.: Spina bifida: A survey of two hundred twenty-five encepha1oceles, meningocele, and myelomeningoceles. Am. J. Dis. Child., 84: 35-51, 1952. 8 Deaver, G. G., Buck, B. and McCarthy, J.: Spina bifida. Yearbook of Physical Medicine and Rehabilitation. 1951, pp. 9-24. 537

538

P. A. MORALES, G. G. DEAVER AND R. S. HOTCHKISS

This paper is a preliminary report dealing with observations on the urologic status and our experience in the treatment and care of thirty-four spina bifida children. VARIETIES OF SPINA BIFIDA

Spina bifida is a congenital anomaly in which there is a defect in the bony wall of the neural canal, through which the meninges and spinal cord may protrude. The resultant conditions are classified as follows: 9 A. Spina bifida anterior B. Spina bifida posterior 1. Spina bifida occulta 2. Spina bifida cystica a. Meningocele b. Meningomyelocele c. Myelocele. Spina bifida anterior. The defect involves the anterior portion of the spinal canal and is a relatively rare condition. Spina bifida occulta. The defect is covered by skin and may present on physical examination no external malformations. However, the presence of an area of localized hypertrichosis, a dimple on the skin, a fibroma or lipoma, or a pigmented depression over the lumbosacral area may suggest its presence. The lesion may be of such a mild nature that there are no symptoms or neurologic manifestations of any kind. Occasionally, as the child grows, symptoms may appear. Extensive defects cause conspicuous symptoms and findings. Spina bifida cystica. Spina bifida cystica includes all of the cases of spina bifida with an external malformation of the cord and/ or meninges such as meningocele, meningomyelocele and myelocele. A meningocele is characterized by a cystic protrusion in the midline of the back at the site of the vertebral defect and is composed of a sac formed by the meninges, filled with cerebrospinal fluid and covered with skin. The spinal cord and its nerve roots may be perfectly normal in some cases of meningocele, and not so in others. M eningomyelocele differs from meningocele in that the spinal cord or its nerves are involved in the formation of the sac. Myelocele is the most serious type and is characterized by a flat, inflamed ulcerated area on the back which corresponds to the embryonic medullary plate. CLINICAL MATERIAL

This report is based on a urologic investigation of 34 patients with spina bifida undergoing habilitative training at the Children's Division, New York University-Bellevue Medical Center Institute of Physical Medicine and Rehabilitation. The age and sex of patients are given in table 1. Their ages ranged from 3 to 18 years, the median age being 8 years. There were 16 boys and 18 girls. The site and variety of the lesions are shown in table 2. The vertebral defect 9 Bucy, P. C.: Spina bifida and associated malformations. In: Brenneman's Practice of Pediatrics, Hagerstown, Md. W. F. Prior Co., vol. 4 chap. 15, 1953, pp. 1-30.

COMPLICATIONS OF SPINA BIFIDA TABLE

539

1. Age and sex (34 cases)

Ages of Patients 0-5 .. 6-10. 11-15 ..

12 12 7 3

16-20. Sex of Patients

16 18

Males. Females. TABLE

2. Site and variety of spina bifida (34 cases) Site

Cervical. Thoracolumbar. Lumbar. Lumbosacral. Sacral.

1 2

3

26 2

Variety

21

Meningomyelocele .. Meningocele. Spina bifida occulta.

11 2 TABLE

3. Associated congenital anomalies 15

Arrested hydrocephalus Club feet. Dislocation of hips. Erb's palsy.

20 7 1 TABLE

Paraplegia Complete Incomplete Quadriplegia Complete .. Incomplete . No motor disturbances.

4. Motor disturbances (34 cases)

23 9

0 1

1

was located in the cervical region in one case, thoracolumbar in two, lumbar in three, lumbosacral in twenty-six, and sacral in two. There were twenty-one instances of meningomyeloceles, eleven meningoceles, and two spina bifida occulta. The following associated congenital anomalies (table 3) were encountered: arrested hydrocephalus, 15 patients; club feet, twenty; dislocation of the hips, seven; and Erb's palsy, one. Table 4 lists the motor disturbances involving the patients in this series. Com-

540

P. A. MORALES, G. G. DEAVER AND H. S. HOTCHKISS

TABLE

5. Urinary symptoms (34. cases)

Recognition of desire to void Complete absence. Vague sensations. Ability to initiate micturition Absent. Present. Initiation of micturition All cases were incontinent

26 8 2

32

plete paralysis of the lower extremities was present in 23 children and partial paralysis in nine. One patient with cervical meningocele had spastic quadriparesis. No motor deficiency was noted in one patient ·whose only manifestation of the disease aside from radiographic findings was inability to control urination. It is noteworthy that largely as a result of the habilitation program to which the children in this series have been subjected, nineteen of them are now able to ambulate with braces and/or crutches. The remaining fourteen, excluding the 1 case without motor disability, are now capable of wheel chair activities and should be able to achieve ambulation eventually when they are older and develop more strength in their arms. ANALYSIS OF CASES

Symptoms. Inquiries were made into the three essential component8 of voiding, namely, the desire to void, initiation of micturition, and its inhibition. The information was obtained from the child, parent, or nurse. Urinary control was unsatisfactory in all the cases (table 5). Twenty-six children could not recognize the desire for urination. The other eight claimed to have a more or less vague sensation of fulness, the significance of which they were not cognizant. No aurae of impending micturition such as flushing of the faee, headaches, goose pimples or sweating were experienced by any of the patients. Practically all the patients could initiate micturition when requested to do so by straining and applying Crede pressure. Urine escaped in a stream which was continued only so long as the pressure was constantly maintained. One case of cervical spina bifida voided spontaneously, while another with seYere scoliosis and lordosis could not expel her urine voluntarily despite straining and manual compress10n. Prior to the institution of a voiding schedule, the majority of the children made no conscious efforts to urinate. Micturition occurred by overflmy and emptying was obviously incomplete. In spite of adherence to a voiding schedule, inhibition of micturition could not be accomplished. Constant dribbling or intermittent spurts of urinary leakage occurred shortly after each voluntary urination and were exacerbated by movements or straining efforts. Varying degrees of rectal incontinence were present in practically all the eases, although this could usually be handled fairly ·well by establishing a scheduled time for bowel evacuation and ·with the aid of glycerine suppositories. CystmLrethrography. Cystograms were done to determine the degree of trabecu-

CO:\Il'LlC.\T!Ol\iS OF SPDL\ BlFIJJ.\

T,\BLE

541

ti. Cys/ugraphic ji11!linr;s (84 ccrncs)

Smooth bladrkr wall Trnbeeub, i0110 ;\Jockrnte J\larkf,d \1·ith ecllule, Ye,ico11rctt'rni nd1m,.

13

1:2 [l

lation <1.nd cellule formation, and to reveal the presence of diwn-ticulum and ve:,;icoureternl reflux. Crcthrograms may show 1,he condition of the Ycsical 1wck llut are of limitt>d usefulue:,,s. The: bladder 1n1:o filled \Yith approximately 7D cc of contrast medium, iujeded through a urr,Hual (;atheter, The catheter wa:;; tlwn removed and an anteroposterior x-ray film taken llrethrograms were alr:,0 seeured in male pa1ients h~, into the urethra 1vith an a:,;epto syringe. A second film was taken oblique , and a third or delayed film 20 minute:,; later. tiudings are given in table 6. The bladder ,ntf'. smooth in \J casc.s, tra.beeula1ed in thirteen, and markedly trabeculatcd \1'ith cellnlc funnation. iH twe] vc No large-sized bladder diveriiculum was dernonVesicouret.cral reflux ,Yas present in 9 childrnn, s1rated m ,rny of che in sev<,u and hilaternlly in t.,rn. The reflux was arosociawd with masin 5 cases_, rnoderate in three, am! normal pelvir-; and had reflux demonstrable only in the delayed film, but this ,nt:s n:prn(hwihlc (i rnonths later in all the films togetlwr 11-ith tasis and udicctasi::;, Cystosr:u7Ju. A Axamination is of ,-alue in as:,;cs:,;ing the sm·erity of bladder i11frctio11, rcYealing the presence of bladder caleuli. ancl aud detenmninµ: the arnou111, and degree of tra.beculatio11, The pre:,;ence of 8pasticit)· m contra(:1 ioll of the vesical neck is hard to dPtermine by oscopy 11 as in 1D patients (table 7). 'fhe bladder nmcosa ex· hihited a mild degree of eongu,tion in ,111 eases, indicatillg slight urinary infection No inten;;e inffamrnatory process \\'a::: observed in any of the bladder;, exarniued. Unlike ad111t paraplegics, not a single patient had hlaclder calr:uli, nor 11as any large divm-ticulum visualizecL The amount and degree of trabeculations ol),:;erved during conformed to the cystographfo findingR. urogranrn reveal both the anatomy and function ln order to endnate the renal changes, the follm,·ing classification was used: onnal · .'.\' orrnal excretory urogra.rn Grndc 1: 1\iinirnal hydronephrosis Grade 2: Definite hydronephrosi:,; Ora de ;->;. l\:IassiYe hydronephrosis Crnde ·±. Noufunctiouing kidney by intrn,·euom, pyelogram TAHU:

ModPratY ('.Oagestion Calenlns hrmatiol1 Divf'rl.i1,nlum (large)

7. Cysloscopic findinys (Ji) cases) 1U 0 0

542

P. A. MORALES, G. G. DEAVER AND R. S. HOTCHKISS TABLE

Normal. ..... Grade 1 .. Grade 2. Grade 3. Grade 4.

8. Classification of pyelographic findings No. of Kidneys

Percentage

51 4 4 8 1

75.0 5.9 5.9 11.8 1.4

...........

TABLE

9. Relation of pyelographic findings to variety of spin a bifida

Occulta ...... . . . . .......... Meningocele. . . . . . . ......... Meningomyelocele ... ..... . . .

No. of Kidneys

Normal

Grade 1

Grade 2

4 22 42

4 (100%) 17 ( 77%) 30 ( 71%)

0 (0%) 2 (9%) 2 (5%)

0 (0%) 2 (9%) 2 (5%)

Grade 4

Grade 3

0 ( 0%) 0 (0%) 0 ( 0%) 1 (5%) 8 (19%) 0 (0%)

In table 8, the excretory urograms obtained on the 34 patients are classified according to the preceding criteria. The pyelograms are tabulated in terms of individual kidneys. It will be noted that 51 kidneys (75 per cent) were graded as normal, and the remaining 17 kidneys (25 per cent) showed pathological changes. Of these, 4 kidneys (5.9 per cent) were considered as grade 1, four kidneys (5.9 per cent) were grade 2, eight kidneys (11.8 per cent) were grade 3, and 1 kidney (1.4 per cent) was grade 4. The relation between the incidence of renal damage to the variety of neural lesion is shown in table 9. Renal damage occurred in approximately the same percentage in meningoceles and meningomyeloceles. Although there was only a limited number of cases of spina bifida occulta in our series, it is reasonably certain that associated destructive lesions in the kidney would be infrequent. The incidence of pathological kidney changes in relation to age is tabulated in table 10. Renal damage was greatest in incidence and severity between the ages of 6 and 10 years, after which a decline occurred in the succeeding age groups. It is improbable that an improvement in renal status was responsible for the decrease in the frequency and intensity of destructive lesions in the older age groups because the majority did not have any planned urologic care prior to their inclusion in this series. Rather, it suggests that most of the deaths arising from kidney damage occur between the ages of 6 and 10 years, which should therefore be regarded now as the critical period in the life of a child with spina bifida. Cystometry. A cystometrogram is a graphic record of vesical activity. The folTABLE

10. Relation of pyelographic findings to age of patient

Age Group

No. of Kidneys

0-5 6-10 10-15 16-20

24 24 14 6

Normal

21 14 11 5

(88%) (58%) (79%) (83%)

Grade 1

0 2 2 0

( 0%) ( 8%) (14%) ( 0%)

Grade 1

1 3 0 0

( 4%) (13%) ( 0%) ( 0%)

Grade 2

2 4 1 1

( 8%) (17%) ( 7%) (17%)

Grade 3

0 1 0 0

(0%) (4%) (0%) (0%)

COMPLICATIONS OF SPINA BIFIDA

543

lowing data may be obtained during the procedure: 1) intracystic pressure in relation to bladder volume, 2) presence or absence of reflex contractions of the bladder muscle and measurement of their amplitude, 3) maximum voluntary intravesical pressure during abdominal straining, 4) presence or absenre of vesical sensations, 5) bladder capacity. In this investigation, a Lewis aneroid cystometer and a simple water manometer were connected by a Y-tube to an indwelling catheter. This arrangement furnished a continuous recording of pressure changes and allowed detection of small contraction waves or slight changes in intravesical pressure. Fluid was introduced into the bladder from a reservoir equipped with a Murphy drip and calibrated so that the number of cubic centimeters of fluid introduced would be easily read. The rate of flow was controlled by a Hoffman screw clamp. Pressure changes were observed 1) after each addition of 25 cc fluid increments, 2) during introduction of fluid at a slow constant rate (11 drops/min.), and 3) while allowing the bladder to fill by excreted urine. Figure 1, A depicts vesical activity in an 8-year-old spina bifida paraplegic boy during introduction of 25 cc increments of fluid at 1 minute time intervals through a suprapubic cystostomy tube. It will be noted that with each additional quantity of fluid, there was a steep rise in intravesical pressure which slowly diminished to a resting level. An initial rise in the resting pressure occurred, which, however, was sustained at about 30 mm. Hg. after the third injection of fluid, at which time urine started to escape from the urethra with each further addition of fluid. This pressure curve demonstrates the tendency of the spina bifida bladder to resist suddenly injected fluid, indicating poor adaptation to stretch. Figure 1, B shows the cystometric graph from the same patient when fluid was introduced at a slow steady drip (100 drops/min.). The pressure curve was flat and contained no forceful sustained contraction. Superimposed were small rhythmical waves indicating minor ineffectual contractions. Although the patient dribbled urine continually during the determination, bladder tonus was sustained at 10-15 mm. Hg. A similar type of recording was obtained when the bladder was allowed to fill by excreted urine (fig. 1, C). During the early part of this investigation, several of our recordings obtained from a water manometer erroneously showed "hypertonic" pressure curves. It can now be readily seen that such a record can be expected to occur when inadequate time is allowed between adding increments of fluid or when fluid is steadily but rapidly run into the bladder. Residual urine determination. Residual urine is the result of the inability of the bladder to empty completely, because of inefficient detrusor action or increased resistance at the vesical neck, or a combination of both. It has been generally accepted that this is the primary disturbance responsible for most of the problems associated with neurogenic vesical dysfunction among adult paraplegics, and its elimination will minimize such problems. The presence of more than 100 cc residual urine has been regarded arbitrarily as an indication for definitive treatment.

544

P. A. MORALES, G. G. DEAVER AND R. S. HOTCHKISS

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Residual urine was measured after voiding with the aid of intrinsic (strain) and extrinsic (Crede) pressures. Only 5 cases had more than 60 cc residual urine, 24 cases had less than 60 cc, and five had no residuum (table 11). Of the 5 cases with large volumes of residual urine, three had moderate hydro-

COMPLIC,'I_TION8 OF 8PlXA HIFlDA

TABLE

lJ. Res·idual 1/rine delenninalion (34 cases)

:\Jore t ban 60 ce .. r,es~ than 60 cc ·:, o rnsidunm

11ephrosis without Jemoustrable reflux, and the other two had normal pe!vi:c; and calyces . .Nine cases of hydroncphrosis, including the most adva11ced lesions i11 ihi:-; seri0s, ,Yere encountered among patim1t;; 1Yho had little residual urine ur 1101 w ,diat;;oev0r, Hefiux was demon;;trable in all belonging to the latter group. TREA'l'ME:'\T

There are two phaioes in the management of the child ·with nenrogt>nic veflical dysfunction due to spina bifida, namely, l) correction of urinary inrontinruce, and 2) treatment of progreRsive renal damage. <'orrcct?:on of urinary incontinence. The long list of prrn,edures achocatcd for correction of neurogenic bladder i.11conti11encc, suggec1ts that no 011e of tlwrn ha~ tx,en rornpletely adequate. a. Bladder training: As part of their training in self care, the children m thic" series were placed routinely on a bladder training program/· 10 except whm1 vcsicoureteral reflux \\"as demonstrated by cystography. They learned to use Lhe toilet or cornmode rather than a bedpan, Lltilizecl different methods of i11itiati11g micturition, followed a ,·rnding :-,chedule regulated fluid intake, observed genir.a[ cleanlines.s, c:hanged their pad;; aucl rubber pants, and latPr became familiar 1Yith ihe use and care of a minal bag and other rubber appliances. Contrary to prm·ious clinical impressions,8 no child in thi;; group ,ms able 11, achieve urinary ccmtinencc. Bladder training haR, hmrnYer, brought ahout increased independence in the child's daily needs and lessened the amomit of wetting. Urinary diversion cithPr with an inlying catheter or supra.pubic: cystostorny iH indicated when iHcontimmce remains intolerable despite all efforts Rubber urinals hc1ve slightly improYed the situation among the older boys, bnt no satisfactory appliance ha:,, been de,·ised for girls. b. Lmninectomy: Exploration of the spinal cord ,rais fonJred by Chute 11 am! .'.\Iertz, 12 who reported successful results follmYing relief of traction or pre,,surc from corcl structures. Despite reports of improyement or cure in an occusiomLI case, the ,mccesses follm,·ing this proredure are fmL Alexander and his asc'ociatcs13 reported one: case ,,-here bladder continence ,yas gained a pparcn tly as fa result of the crushing of ibe S-:l nerve roots. c. Sphincter operations: T\umerous operations haxc been performed in whid1 ic Munro, D.: The rnlrnbilitat.ion oI patients t,otally paralyzed below the waiRt, withe,, p1_,ciri.l reference to making them mnbufa.tory and capable of earning their living. 111. Tidai drain:1ge, cy~tometry and bladder t,rnining. ?sie11" Eng . .J. Med., 236: 22:)..23fi, 1947. u Chu Le, A. L : The relation bc,!.ween spin:, bifida occulta and cerkiin eases oI retention. J. -Ciro!., 5-: 317-324, Hl21. 1·1 1\Jertz. H. 0. and l'
546

P. A. MORALES, G. G. DEAVER AND R. S. HOTCHKISS

muscle or fascial strips were placed in a sling or pseudosphincter fashion about the bladder neck or posterior urethra. Varying degrees of success have been reported, but in general, they have not been encouraging. Smith and Engel1 4 used the gracilis muscle, Miller15 employed the pyramidalis muscle, and Ewert and Flint16 passed a flap of rectus muscle and fascia around the posterior urethra to form an artificial sphincter. We utilized the last procedure in 2 cases with no success. d. Transurethral resection: Workers from the Mayo Clinic have reported relief of incontinence following transurethral resection of the vesical neck. 17 • 18 This form of therapy would be applicable only for the minority whose urinary incontinence is associated with retention. e. Urinary diversion: The use of an indwelling Foley catheter connected to a rubber urinal appliance affords the simplest way of diverting the urinary stream. This arrangement, however, is at best only a temporary measure. The frequent irrigations and changes of the catheter which must be carried out are inconvenient to the child and his parents. Protracted use of the urethral catheter is complicated by urethral sepsis and vesical calculus formation. In our experience, a suprapubic cystostomy has represented the best method for urinary diversion. Of 5 children in this series who have cystostomy drainage, four are able to remain dry and comfortable. We have observed that when the suprapubic tube is connected to open drainage, the bladder can usually be kept sufficiently empty so that the small remaining sphincteric tonicity is sufficient to retain the small amount of urine constantly present in the cystostomized bladder. Leakage will, of course, occur when the tube is clamped, but this occasion will rarely arise when a rubber collecting appliance is available. We do not believe that surgical closure of the urethra should be done at the time of cystostomy as advised by others,19 although it may have to be resorted to later in the cases where simple cystostomy fails. Once cystostomy is properly established, the child's parents rarely encounter difficulty in caring for the tube. Daily saline irrigations can easily be done by the child or parents and the catheter is usually changed once a month. Other workers have advocated more radical procedures to relieve incontinence. The so-called "wet colostomy" which consists of a ureterosigmoid anastomosis and an end sigmoidostomy has been tried and found unsatisfactory because of the foul odor from intermixture of urine and feces, and the high incidence of ascending urinary infection. 19 Artificial bladders using the cecum20 or sigmoid19 to 14 Smith, C. K. and Engel, L. P.: Neurogenic vesical dysfunction in children. J. Urol., 28: 675-715, 1932. 15 Miller, N. F.: The surgical treatment of urinary incontinence in the female. J.A.M.A., 98: 628-632, 1932. 16 Ewert, E. E. and Flint, F. D.: Rectus muscle sling for neurogenic incontinence. Surgery, 27: 688-698, 1950. 17 Thompson, G. J. and Jacobson, C. E.: Neurogenic vesical dysfunction due to spina bifida and myelodysplasia. Treatment by transurethral resection of the vesical neck. Am. J. Surg., 61: 223-238, 1943. 18 Emmett, J. L. and Helmholz, H. F.: Transurethral resection of the vesical neck in infants and children. J. Urol., 61: 463-478, 1948. 19 Gross, R. E., Holcomb, G. W. and Swan, H.: Treatment of neurogenic urinary and fecal incontinence in children. Arch. Surg., 66: 143-154, 1953. 20 Gilchrist, R. K., Merricks, J. W., Hamlin, H. H. and Rieger, I. T.: Construction of a substitute bladder and urethra. Surg., Gynec. & Obst., 90: 752-760, 1950.

COMPLICATIONS OF SPINA BIFIDA

547

act as reservoirs with a small opening on the anterior abdomen have been constructed. The following complications have been encountered after such bladder substitutions: 1) leakage or retention of urine from too large or too small an opening, 2) spasms of the isolated segment of bowel causing abdominal pain, 3) calculus formation, 4) ulceration and bleeding, and 5) rupture of the pouch. The Bricker procedure21 which utilizes an isolated piece of the terminal ileum to convey the urine from both ureters to an external stoma has been tried by Bill and his co-workers 22 on a case of spina bifida with encouraging results. The endresults following such extensive operations, however, do not justify the risks involved, especially when one considers that just as much can be accomplished with a simple suprapubic cystostomy. Treatment of progressive renal damage. The progressive deterioration of the upper urinary tracts has been a very vexing problem and represents the most serious complication of spina bifida in children. In the past, various surgical procedures such as nephrostomy, pyelostomy, cutaneous ureterostomy and cystostomy have been tried, yielding inconclusive clinical benefits. Our present policy is to institute continuous drainage either with an inlying catheter or suprapubic cystostomy whenever there is evidence of renal damage. These procedures afford the least interference to the spina bifida child's activities of daily living. In the presence of a large residual urine, a transurethral resection of the vesical neck may be performed and bladder decompression deferred, provided the kidney lesion is not severe. Ureteral plastic procedures may be employed in cases of advanced hydronephrosis with extensive ureteral dilation and angularity. The tortuous ureters are freed throughout their entirety from the bladder to the kidney pelvis and the straightened proximal segments are reimplanted into the bladder after resection of the redundant distal portions. This procedure is designed to reduce urinary stasis and promote dependent renal drainage. Improvements in the upper urinary tract status were observed in 4 patients treated in this manner. We are not certain, however, whether simple cystostomy drainage alone might suffice to relieve the ureteral dilation and tortuosity and result in reduction of the hydronephrosis present in advanced cases. Our studies are still incomplete and further observations will be necessary before definitive rules can be set. In extreme cases characterized by continued septic temperature elevations despite bladder drainage, nephrostomy maybe necessary and life-saving. We had to resort to this procedure, however, in only 1 case. Followup care. These patients must be followed with the utmost vigilance as long as they live. Excretory urography, cystography, and estimation of residual urine should be made every 6 months even in the absence of symptoms. It should be borne in mind that dangerous renal lesions may develop insidiously and a bladder that empties well is no guarantee for the permanency of good renal function. 21 Bricker, E. M.: Bladder substitution after pelvic evisceration. S. Clin. N. Amer., 30: 1511-1521, 1950. 22 Bill, A.H., Dillard, D. H., Eggers, H. E. and Jensen, 0.: Urinary and fecal incontinence due to congenital abnormalities in children. Management by implantation of the ureters into an isolated ileostomy. Surg. Gynec. & Obst., 98: 575-580, 1954.

548

P. A. MORALES, G. G. DEAVER AND R. S. HOTCHKISS

DISCUSSION

The neurophysiology of the bladder disturbances occurring in spina bifida have been discussed by various authors. 13 • 14 • 23 • 24 Most workers now agree that they are comparable to those observed in patients who have been rendered paraplegic or paraparetic by trauma, tumor or infection. The nature of the neurogenic vesical dysfunction will depend on the level and extent of involvement of the spinal cord and its nerve roots. Inasmuch as the majority of spina bifida are situated in the lumbosacral vertebrae, the resulting vesical changes bear many points of resemblance to those seen with the acquired lesions of the sacral portion of the spinal cord and the cauda equina. This type of disturbance is referred to as the autonomous type of neurogenic vesical dysfunction. The parasympathetic innervation of the bladder which comes from the sacral segment of the spinal cord has been partially or completely destroyed. The bladder, except for its sympathetic innervation, is completely divorced from the central nervous system. Any activity in the detrusor muscle and internal sphincter must apparently occur as the result of impulses transmitted through a reflex arc that lies wholly within the vesical wall. Such a bladder, because of its self-governing or autonomous character, is called an autonomous bladder. The following features may be observed: 1) A powerful sustained contraction of the detrusor muscle characteristic of reflex micturition is not possible, 2) abdominal straining has replaced detrusor contraction as the expulsive force in voiding, 3) spontaneous contraction waves of small amplitude occur during bladder filling, 4) the detrusor muscle resists rapid filling of the bladder. It is obvious that the incontinence in spina bifida is the consequence of flaccid urinary sphincters. A more or less constant urinary leakage increased by bodily movements is the usual finding. Although this type of incontinence has been described or recognized by others in connection with cauda equina or low spinal cord injuries, 25 • 26 it is well known that its occurrence in traumatic lesions of the spinal cord is relatively infrequent. Neither does it occur, except in few cases, following subarachnoid alcohol block, 27 sacral rhizotomy, 28 or cordectomy, 29 all of which cause paralysis of the urinary sphincters. Watkins believes that urine does not escape from the bladder in lesions of the conus and cauda equina despite paralysis of the external sphincter and incompetence of the internal sphincter 23 Mertz, H. 0.: The relation of spina bifida occulta to neuromuscular dysfunction of the urinary tract. J. Urol., 29: 521-530, 1933. 24 Langworthy, 0. R., Kolb, L. C. and Lewis, L.: Physiology of Micturition. Baltimore: Williams & Wilkins Co., 1940, pp. 80-87. 25 Munro, D.: Rehabilitation of veterans paralyzed as the result of injury to the spinal cord and cauda equina. Am. J. Surg., 76: 3-18, 1948. 26 Denny-Brown, D. and Robertson, E.G.: The state of the bladder and its sphincters in complete transverse lesions of the spinal cord and cauda equina. Brain, 56: 397-462, 1933. 27 Bors, E.: Bladder disturbances and the management of patients with injury to the spinal cord. J. Internat. Coll. Surg., 21: 513-527, 1954. 28 Brendler, H., Krueger, E.G., Lerman, P., Harper, J. G., Bradley, D., Berman, M. H., Hertzberg, F., Lerman, F. and Dean, A. L.: Spinal root section in treatment of advanced paraplegic bladder. J. Urol., 70: 223-229, 1953. 29 McCarthy, C. S.: The treatment of spastic paraplegia by selective spinal cordectomy. J. Neurosurg., 11: 539-545, 1954.

COMPLICATIONS OF SPINA BIFIDA

549

because of the existence of considerable mechanical resistance to the outflow of urine, brought about by the pressure and elasticity of the tissues surrounding the urethra in the region of the triangular ligament. 30 Such a resistance to the urine outflow is not expected in spina bifida lesions in view of the flaccid paralysis and atrophy of the perinea! muscles. Certain bladder complications like calculosis, urinary stasis and severe cystitis are, however, infrequent in spina bifida. Not a single calculus was observed in this series, in contrast to the 15 to 70 per cent incidence among adults with traumatic paraplegia. 25 • 31 • 32 • 33 Although other workers have reported a higher percentage of spina bifida patients who carry substantial amounts of residual urine, 17 • 18 · 34 the majority of our cases had little residual urine or none whatsoever. Cystitis characterized by an intense inflammatory reaction of the bladder and foul turbid urine were not encountered in any of the patients. Unlike his adult counterpart, the child who has spina bifida does not go through any prolonged period of recumbency and immobilization which is a major precipitating factor in calculus formation. Bladder emptying is facilitated by the relaxed sphincters. In the absence of calculi and urinary stasis, urinary infection is expectedly minimal. It has been commonly believed in the treatment of paraplegics that the health of the upper urinary tract is dependent upon the health of the bladder. Hydronephrosis is usually traceable to stasis and infection in the bladder. The frequency of occurrence of advanced hydronephrosis and ureterectasis among pa tients in this study who carried moderate amounts of residual urine or none whatsoever, and with mild urinary infection, indicates that one cannot prognosticate the future of the kidneys by judging bladder function. It is more probable that vesicoureteral reflux was largely responsible for the renal complications in the majority of our patients. The etiology of reflux is still poorly understood, but it is apparent from our study that stasis of urine in the bladder, if at all, plays only a minor role. After an extensive study, Graves and Davidoff35 came to the conclusion that a well sustained tonus of the bladder musculature as it resists filling plays an important role in the causation of reflux. Our cystometric data and the findings of advanced trabeculation substantiate this hypothesis. SUMMARY

As a result of advances in the fields of neurosurgery, orthopedics, and rehabilitative therapy, the spina bifida paraplegic child now has an opportunity to take Watkins, K. H.: The bladder function in spinal injury. Brit. J. Surg., 23: 734-759, 1936. Bumpus, H. C., Nourse, M. H. and Thompson, G. J.: Urologic complications in injury of the spinal cord. J.A.M.A., 133: 366-369, 1947. 32 Petroff, B.: The paralyzed patient. Urologic aspects. J.A.M.A., 129: 154-155, 1945. 33 Bors, E.: Urological aspects of rehabilitation in spinal cord injuries. J .A.M.A., 146: 225-229, 1951. 34 McCarroll, H. R.: Spina bifida urinary incontinence: A report of cystometric studies in a series of 30 cases with some suggestions regarding their clinical management. Surg. Gynec. & Obst., 64: 721-737, 1937. 35 Graves, R. C. and Davidoff, L. M.: Studies on the ureter and bladder with especial reference to regurgitation of the vesical contents. J. Urol., 10: 185-231, 1923. 30 31

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P. A. MORALES, G. G. DEAVER AND R. S. HOTCHKISS

his place eventually as a self-dependent and productive member of society. Total rehabilitation has, however, been seriously impeded by two factors, namely, 1) urinary incontinence and 2) progressive renal damage. Data obtained in a survey of the nature and frequency of the changes occurring in the urinary system of a group of thirty-four spina bifida children are presented. Our experience in their urological treatment and care is also discussed. Unsatisfactory control of urination characterized by a more or less constant urinary leakage increased by bodily movements was the usual finding. Cystoscopy revealed the presence of trabeculations in the majority of bladders examined. Cystography demonstrated reflux in 9 children (26 per cent), unilateral in seven and bilateral in two. Cystometric study showed that the spina bifida bladder has the characteristic features of an autonomous type of neurogenic vesical dysfunction. Evidence of renal destructive lesions was noted in seventeen (25 per cent) of 68 kidneys studied during excretory urography. Renal damage was greatest in incidence and severity between the ages of 6 and 10 years, after which a decline occurred in the succeeding age groups. It is probable that most of the deaths from kidney complications occur between the ages of 6 and 10 years, which should therefore be regarded now as the critical period in the life of the spina bifida child. Bladder training was instituted routinely as part of the self-care program, except when reflux was demonstrable. Although continence was not achieved in any of the cases, it has brought about increased independence in the child's daily needs and lessened the amount of wetting. Urinary diversion either with an inlying catheter or suprapubic cystostomy is advisable when incontinence remains intolerable despite all efforts. Continuous bladder drainage, either with an indwelling urethral catheter or suprapubic cystostomy, should be established whenever there is evidence of renal damage. If the residual urine is large, transurethral resection of the vesical neck may be performed and bladder decompression deferred, provided the kidney lesion is not severe. Four patients with advanced hydronephrosis and extensive ureteral dilation and angularity were treated by freeing the ureter, reimplanting the straightened proximal segment into the bladder, and resecting the redundant distal portion, thus promoting free dependent drainage of the kidney. Improvement in the upper urinary tract status followed. Studies are, however, being conducted with other patients to determine whether simple cystostomy alone will suffice in such cases. N ephrostomy may be necessary and life-saving in extreme cases of urinary sepsis not responding to bladder drainage. Followup examinations consisting of excretory urograms, cystograms, and residual urine determinations should be done every 6 months even in the absence of symptoms. Dangerous renal lesions may develop insidiously and a wellemptying bladder is no guarantee for the permanency of good renal function. The authors are indebted to Mrs. Jean McCarthy Lichtenstein (nurse supervisor, Children's Division, Institute of Physical Medicine and Rehabilitation) and Mr. James Ramseur (research technician) for their assistance in this work.