Current Trends in Pediatric Urology

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MEETING REPORT

Editorial Committee of the Urology Section of the American Academy of Pediatrics

Current

Trends

in Pediatric

Urology

John R. Woodard, M.D., F.A.A.P., Chairman, A. Barry Belman, M.D., F.A.A.P., and

Harry C. Miller, M.D., F.A.A.P.

EDITOR’S NOTE: This is a small experiment to

determine if it is worthwhile for our /ournal to carry brief reports on pediatric subspecialty

meet-ings which hare been written by a physician

attending the meeting. Reports of these meetings

are now widely covered and headlined by the free th row-away medical newspapers.

The accuracy of the reporting is quite variable.

We belieUe a physician is better qualified to

interpret and judge the value of the presentations.

if you find this section useful and interesting, we

would appreciate hearing from you.

With 29 papers presented before the Urology

Section (Washington, D. C., October 18 to 23,

1975), the annual meeting of the American

Academy of Pediatrics is rapidly becoming the

major forum for pediatric urology in this country.

The content of this year’s papers highlights much

of what is current in this growing subspecialty.

The topics discussed largely fell into three major categories, i. e., genitouninary anomalies,

prob-lenis relating to or resulting from obstructive

uropathy, and genitourinary neoplasms.

ANOMALIES OF THE GENITOURINARY

TRACT

Hypospadias and cryptorchidism are two of

the most common genital anomalies. Following

his excellent movie on embryology of the male

external genitalia, C.

J.

Devine presented a series

of 200 consecutive patients with hypospadias

surveyed for associated anomalies. Over one

fourth of these patients had other birth defects,

some multiple. Hernia, hydrocele, and

crypton-chism were commonly associated anomalies. Of

particular importance, he found that 16 of 160

(10%) of the patients so examined had abnormal

excretory urograms (IVP). Over one half of the

pyelographic abnormalities were surgical lesions

such as hydronephrosis and vesicoureteral reflux.

On the basis of this extensive experience Dr.

Devine recommended that all patients with

hypospadias undergo excretory urography.

In managing the child with DO palpable testes,

S. D. Levitt and his associates pointed out that

bilateral cryptorchism is much more common

than anorchia but that surgical exploration, unless

extensive, has not proven completely reliable in

making the differentiation. They proposed a test

whereby the presence of a functioning testis

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972 TRENDS IN UROLOGY

thereby possibly avoiding the need for such

surgery. They reported that chonionic

gonado-trophin (HCG) stimulation with measurement of

basal and peak testosterone levels was accurate in

differentiating anorchia from cryptorchism.

Micropenis has been a therapeutic challenge

and in recent years the trend has been towards

converting such individuals to female at an early

age. The alternative therapeutic approach has

been hormonal and surgical attempts at

augment-ing the size of the inadequate phallus. S.

J.

Kogan

and D. I. Williams, on the basis of experience

with 34 patients, suggest that each patient be

assessed individually and that two parameters,

i.e., absolute penile size and the presence of

palpable gonads, are helpful in deciding upon

therapy. The absence of palpable gonadal tissue

indicates poor potential. However, of those with

palpable testes, 64% had adequate penile growth

with testosterone therapy or at puberty. R. K.

Danish et a!. reviewed 43 patients with

micrope-nis, many of whom fell into other syndromes.

They suggested that absolute penile

measure-inents be used in making a diagnosis. The

stretched penile length in a normal male neonate

should exceed 2 cm. Their data suggested a poor

prognosis for normal sexual function in males

with micropenis. They questioned the ultimate

benefit of testosterone therapy. Their experience

with 1 1 patients reared as females indicated that

this (sex reassignment) is likely to be the best plan

of management.

In presenting a patient with penile agenesis,

J.

Y. Dwoskin discussed the techniques of

converting the scrotal tissues into a vulva. He

stressed the importance of a team approach to the

management of this type of problem. The more

commonly done reconstruction of the vinilized

female infant was discussed by A. D. Perimutter

and E. T. Gonzales, Jr. By their technique a good

cosmetic result is usually possible by recessing

rather than amputating the clitoris.

Urethral duplication is an unusual anomaly. As

pointed out by

J.

R. Woodard and S. Ambrose it

usually presents with an epispadic appearance to

the penis but normal pubic bones. In this type of

duplication, where the ventral channel is the

more normal, the functional and cosmetic results

were excellent in six boys treated by excision of

the accessory dorsal channel back to the

urogen-ital diaphragm and closure of the defect on the

dorsum of the penis.

That fused and ectopic kidneys are likely to

result in the need for urological intervention was

confirmed by P. Donahoe who presented the

experience of H. Hendren et al. with nine cases of

crossed renal ectopia. The urological anomalies

occurring in eight of these patients included

refluxing megaloureter, ectopic megaloureter,

urethral valves, periureteral diverticuli, and

ureteral pelvic obstruction. Since eight of their nine cases had surgically correctable associated

genitourinary anomalies, the authors advocated

complete urological investigation of any child

with crossed fused renal ectopia.

OBSTRUCTIVE UROPATHY

That urological obstructions remain a major

problem in pediatrics was evident by the two

panels and numerous papers dealing with various

aspects of this problem.

Perhaps because of an overly enthusiastic

surgical attack on the bladder necks of children in previous years, there is recent evidence of reti-cence in I1aking a diagnosis of primary bladder

neck obstruction. In questioning whether bladder

neck obstruction in children is fact or fantasy

L. King reports that, though uncommon, this

entity is real. Objective findings such as residual

urine and bladder trabeculation must confirm the

diagnosis. This disorder, correctable by anterior

vesical neck revision, must be distinguished from

sphincter dysenergia. The latter is treated

nonsur-gically with smooth muscle relaxants and

phe-noxybenzaline.

Infants with absent abdominal muscles (the

prune belly syndrome) present a unique type of

obstructive or urostasis problem where the cause

of death has frequently been renal.

J.

W. Duckett,

J

r., suggests this syndrome be viewed as a

spec-trum. Those infants, he says, born with good

kidneys will, if urinary tract infection is

prevented, do well despite the grossly abnormal

appearance of their urinary tracts radiographi-cally. Conversely, those born with poor kidneys, dysplasia, are likely to do poorly even if total

surgical reconstruction of their urinary drainage

systeni is undertaken . Duckett recommended

minimal surgical intervention with cutaneous

vesicostomy as a temporizing maneuver should

infection occur.

In discussing the historical perspective on

management of posterior urethral valves

F. Fuqua reviewed the past 16 years’ experience

at Children’s Medical Center in Dallas. His data

indicated that a conservative approach, valve

resection and infection control, might in general

be preferable to either early reconstruction or

temporary high diversion.

Froni a panel discussion on the management of

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ureteroceles chaired by

J.

W. Dwoskin came a qualified recommendation that the typical

ectop-ic ureterocele be treated by heminephrectomy or

pyelostomy alone. This recommendation was

based on the assumption that the remaining intact

ureterocele may cause no further problem.

Though there was relatively little discussion of

vesicoureteral reflux at this year’s meeting, A.

Colodny, M.D. presented seven patients with

reflux (grades 2 and 3) in association with a

paraureteral (Hutch) diverticulum. In these seven

patients the diverticulae disappeared in six and

the reflux in all seven simply by clearing infection or relieving obstruction if present. Dr. Colodny

suggested that such a diverticulum no longer be

considered an indication for correcting associated

reflux.

Previous reports have shown that children with

reflux were likely to show some deterioration in

renal concentrating ability. Of 23 patients

undergoing reimplantation for reflux, D. T.

Ueh-ling and

J.

B. Wear, Jr., reported improvement in

renal concentrating ability in 1 1. They advocated

the use of this measurement of tubular function as

a more sensitive means of following such patients.

In their report on abnormalities of renal tubular

function secondary to obstruction, L. Hill and

E. T.

Gonzales, Jr., also observed that changes in

renal tubular function may be out of proportion to the changes in the level of glomerular function. Nephrogenic diabetes insipidus and renal tubular

acidosis are distinct disorders of the distal

nephron associated with obstruction. When these

disorders are present, the degree of acidosis may

be more severe and the management of water

balance more difficult than the level of glomer-ular function would suggest.

D. Walker reported data collected with his

associates indicating generally less than adequate surveillance of renal function in children having

urinary diversions. They recommended greater

use of such tests as creatinine or urea clearance, studies of acid-base balance, fasting concentrating ability, and free water clearances in an effort to

detect earlier evidence of renal deterioration.

After pointing out that urinary incontinence

has been a surgical complication in up to 50% of boys with posterior urethral valves and the sole

reason for urinary diversion in one third of the

patients with myelodysplasia, A. Colodny

pre-sented his experience with an implantable

artifi-cial urinary sphincter. He has implanted the

hydraulic cuff (developed by B. Scott and

W. Bradley in Houston) in seven patients, two

with previous urethral valves and five with

myelodysplasia. Four patients appeared to have

good results at the time of his presentation.

A report by C. Firlit on the role of ileal

conduits and defunctionalized bladders in

child-hood renal transplantation indicated that (1) ileal conduits have proven acceptable receptacles for

renal grafts and (2) bladders previously

defunc-tionalized for long periods of time regained useful

function in two to three weeks.

GENITOURINARY NEOPLASMS

In a fascinating paper based upon a neonate

followed prospectively with open renal biopsies

from the stage of nodular renal blastema through

nephroblastomatosis, S. N. Rous compared the

relationships between nodular renal blastema,

nephroblastomatosis, and Wilms’ tumor. He

concluded that these three entities were different

points on the same disease spectrum and

suggested that his study also demonstrated

malig-nant potential of nephroblastomatosis.

In discussing the current treatment of sarcomas

of the lower urinary tract which comprise 5% to

8% of childhood malignancies, B. Belman

confirmed recent evidence that surgical

extirpa-tion in combination with chemotherapy and

radi-ation has improved survival. The ideal

combina-tion between the surgical and nonsurgical

thera-pies is still undetermined. Dr. Belman also

suggested that consideration be given towards

avoiding debilitating surgical procedures in these patients in favor of chemotherapy.

Several conclusions might be drawn from the

discussion of postpubertal testis tumors by H. C.

Ridlon et al. and the survey on prepubertal testis

tumors reported by E. S. Tank. Testis tumors in

boys beyond puberty should be considered of the

adult type and managed accordingly. It appears

wise to also place those in the 10- to 12-year

group in that category. In the young child most

mortalities occur in those under the age of 2

years. It has been difficult to correlate clearly retroperitoneal node dissection with increased

survival. In Tank’s review all patients with

nongerminal tumors survived and node dissection

did not appear necessary.

With the wide use of cyclophosphamide in

treating childhood neoplasms, bladder

hemor-rhage from this agent is not an uncommon

problem. It can be a serious complication. A

report from A. P. M. Kumar and associates from

the St. Jude Children’s Research Hospital gives

their experience with intractable bladder

hemor-rhage from cyclophosphamide therapy and pelvic

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974 TRENDS IN UROLOGY

by cystoscopic evacuation of clots followed by

cauterization with silver nitrate (0.5% to 1.0%

solution).

MISCELLANEOUS

There has been general urologic interest in the

evidence that certain families have an increased

incidence of vesicoureteral reflux.

J.

Y. Dwoskin

reported that in 61 families where one child had

reflux 20 families were found to have one or more

additional children with reflux. Many families

also had a second symptomatic child evaluated

only to find more serious disease. It is becoming

increasingly apparent that siblings of children

with vesicoureteral reflux should undergo

screen-ing urograms.

Though urinary calculous disease is uncommon

in children in this country, it remains important.

In a report from Liverpool by F. Bartone and

J.

H.

Johnston 19 children with staghorn renal calculi

were discussed. Most had urinary infection,

usually Proteus. None had hypercalcemia,

hyper-calciuria, or amino aciduria. The results of their

surgery-usually a complete kidney split-were

excellent with normal renal growth and clearing

of the infection occurring after complete stone

removal.

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1976;57;971

Pediatrics

John R. Woodard, A. Barry Belman and Harry C. Miller