- Email: [email protected]
Lower Urinary Tract Problems Related to Infection: Diagnosis and Treatment
Lower Urinary Tract Problems Related to Infection: Diagnosis and Treatment HENRY BURKO, M.D.* ROBERT K. RHAMY, M.D.**
During the past decade, the significance and sequelae of urinary tract infections in children have evoked considerable interest. Sensitive bacteriologic criteria and sophisticated radiographic techniques have aided in diagnosing and evaluating urinary tract infections more frequently. In presenting this review of lower urinary tract problems related to infection, our responsibility to the pediatrician and to his patient necessitates an evaluation of the accuracy and limitations of the diagnostic methods employed and an appraisal of the treatment of these problems, based on our present beliefs resulting from considerable personal experience.
INCIDENCE OF URINARY TRACT INFECTIONS IN CHILDHOOD Asymptomatic bacilluria has been reported by Kunin et al. 14 in 1.2 per cent of 16,000 school children. Michie l6 found that 5 per cent of all children on pediatric services had significant urinary tract infection (U.S.A.); and Burke4 reported that 3 to 4 per cent of all pediatric admissions to the Wittington Hospital in London were due to this condition. Bugbee and Wollstein,3 in reporting 4903 autopsies, found an incidence of 4.5 per cent of pyelonephritis in children. It is evident that a significant segment of pediatric practice is related to urinary tract infections and their sequelae (Table 1). During the first 3 months of life, the incidence of urinary tract infection is approximately equal in males and females. However, over From the Departments of Radiology and Urology, Vanderbilt University School of Medicine, Nashville, Tennessee ·Professor of Radiology ··Professor and Chairman of the Division of Urology Pediatric Clinics of North America- VoL 17, No.2, May, 1970
233
234
HENRY BURKO, ROBERT
K.
RHAMY
Table 1. Clinical Symptoms and Signs of Children Referred for Roentgen Examination of the Lower Urinary Tract (410 Patients) Infection Infection, repeated, with previous reflux Neurogenic abnormality'" Dysuria Enuresis Oiliff
40% 20% 13% 12% 12% 3%
"'The high percentage of neurogenic abnormality reflects the activities of the Birth Defect Center at Vanderbilt.
the age of 3 months, urinary tract infection in females is three times as frequent as in males. 24
ROUTES OF INFECTION Much has been written concerning the etiology of urinary tract infections. Hematogenous dissemination of microorganisms to the urinary tract has been proposed for many years; and, indeed, it is probably more common in children than in adults. Nevertheless, a hematogenous source for urinary tract infection is unusual. Spread of infection to the urinary tract via lymphatics from adjacent organs or by direct extension from adjacent organs is rare. The most common pathway for the introduction of microorganisms is ascent to the upper urinary tract via the urethra and lower urinary tract. The frequency of ascending infection in the urinary tract necessitates a complete evaluation of the lower urinary tract in any child who exhibits one or more bouts of urinary tract infection.
ORGANISMS In the uncomplicated, noninstrumented lower urinary tract infection, the occurrence of multiple bacterial species is infrequent. Laurell and Lindbom15 stated that 80 to 100 per cent of uncomplicated infections were due to a single species. The most common organism encountered in uncomplicated urinary tract infections in children is Escherichia coli. The frequency of various organisms involved in urinary tract infection is outlined in Table 2. Through the years, the percentages of uncomplicated infections caused by E. coli have diminished, and higher percentages of more virulent organisms, such as Klebsiella, Pseudomonas, and Proteus, have been reported from many clinics. In our experience, the child who has been instrumented has a three times greater chance of developing a urinary tract infection due to a resistant organism, compared to a child who has not been instrumented. The increasing incidence of urinary tract infections and an alarming increase in the detection of virulent organisms, with greater chance of drug resistance, provides a stimulus
235
URINARY TRACT INFECTION
Table 2. Organisms Involved in Urinary Tract Infections (2000 Patients)
\
'I
I
~
UNCOMPLICATED
COMPLICATED
Single Organisms Mixed Organisms
80% 20%
20% 80%
E. coli Paracolon Klebsiella Proteus Pseudomonas Enterococci Staphylococci
82% 5% 2% 5% 0 14% 4%
18% 5% 45% 43% 7% 31% 9%
No significant differences in organisms were found between pediatric and adult age groups.
for critical evaluation of methods of investigation and aggressive therapeutic management.
METHODS OF INVESTIGATION OF THE URINARY TRACT Investigation of the urinary tract requires a survey of the functional and bacteriologic status of the urinary system from the glomerulus to the meatus. Therefore, evaluation should consist of: 1. A good history and physical examination.
2. 3. 4. 5. 6. 7.
Urine culture, bacterial counts, and sensitivities. Renal functional assessment. Excretory urography. Voiding cystourethrography. Endoscopic examination. Flow and pressure measurements, when feasible.
ROENTGENOGRAPHIC METHODS OF PERFORMING VOIDING CYSTOURETHROGRAPHY Mode of Filling Excretory micturition cystourethrography is the performance of a voiding cystourethrogram after the urinary bladder has been filled with contrast material injected intravenously.17 Catheterization of the urethra is thereby avoided, but the concentration within the urinary bladder is often inadequate to obtain a diagnostic study. Cooperation on the part of the patient is required so that voiding will not occur prior to roentgen recording. Generally, this is not feasible in the first 2 or 3 years of life. Although this method may provide a diagnostic examination in many instances (572 studies out of 728 performed), its reliability is inadequate for general application. Filling the bladder through a urethral catheter is accomplished preferentially by gravity. The catheter is connected to a container of contrast
236
HENRY BURKO, ROBERT
K.
RHAMY
material placed 1 meter above the table. Manual injection with a syringe attached to the urethral catheter is an alternate method of filling the bladder, which should be performed under fluoroscopic control by an experienced examiner. The size and configuration of the urinary bladder, as observed fluoroscopically, recognized discomfort to the patient, and the detection of spontaneous voiding around the catheter provide clues to the adequacy of bladder filling and the imminence of voiding. Contrast Material Sodium or meglumine diatrizoate or iothalamate diluted to a 25 to 30 per cent solution are easily visi;"l.e, nonirritating contrast media. 21 Method of Recording The simplest, and possibly the crudest, method of recording voiding cystourethrography is the exposure of a single roentgenogram beneath the patient, during the act of micturition. It is evident that by utilizing this method, even the simplest radiographic equipment is suitable for performance of the examination. A classic paper, describing posterior urethral valves, was published, utilizing this crude methodY Serial radiography, utilizing either manual or automatic film changing, can provide four or more exposures during voiding. B• 27 As in the single exposure method described above, this method of filming does not allow fluoroscopic observation and is, therefore, less desirable than the methods to be described subsequently.
Figure 1. Normal female urethra demonstrated by spot film during voiding. Note the funneling of the base plate of the bladder.
URINARY TRACT INFECTION
237
Figure 2. Normal female urethra demonstrated by spot film during voiding. Note extreme funneling of the base plate of the bladder into the urethra. Vaginal filling posterior to the urethra is a normal occurrence of voiding during recumbency.
Conventional spot filming under fluoroscopic control, preferably with image intensification, is a widely used method for recording micturition. 5 Four to eight exposures with excellent film detail can be obtained. A further refinement is the use of 70 mm. image-intensifier fluorography, allowing up to 45 exposures and providing excellent image
Figure 3. Normal male urethra demonstrated by spot film during voiding. Complete funneling of the base plate of the bladder into the prostatic urethra is seen. Anterior indentation in the prostatic urethra is caused by descent of the bladder during high velocity voiding. Posterior indentations in the bladder wall represent normal detrusor contractions.
238
HENRY BURKO, ROBERT
K.
RHAMY
Table 3. Analysis of 410 Voiding Cinecystourethrograms (July 1968 to June 1969) ROENTGEN INTERPRETATION
NUMBER OF CASES
Normal Vesicoureteral reflux, all causes Neurogenic bladder Unsuccessful or inconclusive examinations External sphincter spasm, etiology unknown Periureteral diverticulum Cystourethritis, without reflux Posterior urethral valve Megacystis-megatrigone syndrome Vesical outlet obstruction Meatal stenosis
282 67 27 13 7 7 5 1 1
o o
quality.u The gonadal dose utilizing 70 mm. film is lower than with conventional spot filming and cinefluorography. Cinecystourethrography enables the recording of the entire micturition event under fluoroscopic observation and is especially useful in studying details of physiologic events and alterations in the voiding pattern, such as occur in neurogenic disorders of the bladder. Image detail is not as good as with conventional spot filming or 70 mm. fluorography. Gonadal dose is within an acceptable range, varying with the duration of filming. At the present time, conventional spot filming, 70 mm. fluorography, and cinefluorography are the most commonly utilized methods of examination, and 70 mm. fluorography is the preferred method because of excellent image quality, facility for recording a considerable number of events, and minimizing gonadal dose12 (Figs. 1 to 3). Table 3 provides an analysis of roentgen findings of voiding cinecystourethrograms performed in a recent year. For the most part, the symptoms and signs were those of urinary tract infection (Table 1).
POSTERIOR URETHRAL VALVES Waterhouse28 has described 27 examples of posterior urethral valves in the examination of 2500 children. The examination of 1461 children at the Karolinska Institute, by Kjellberg, Ericsson, and Rudhe,13 demonstrated posterior urethral valves in 52 patients. It is noteworthy that the diagnosis of posterior urethral valves was rarely made ante mortem, prior to the utilization of voiding cystourethrography. The Young classification31 of types of valves is generally used. In type I, single or double folds on the posterior urethral wall run downward and laterally from the verumontanum. Type III valve is an iris diaphragm, above or below the verumontanum (Fig. 4). Type II valves are thought by Stephens25 not to exist: none have been seen in our series. The embryologic origin of these valves remains in dispute. Bazy'sl suggestion of the persistence of the urogenital membrane would account for type III valves. Stephens' explanation for type I valves indicates that
239
URINARY TRACT INFECTION
TYPE I VALVES
TYPE III VALVES Figure 4. Posterior urethral valves. Type I valves are embryologic remnants extending downward and laterally from the verumontanum. Type III valves are persistent membranes located either proximal or distal to the verumontanum.
the valve results from "deficient integration of the Wolffian ducts into the walls of the urethra, to abnormal locations of the original orifice into the cloaca, and to an abnormal course of their distal ends."25 (Fig. 4). Clinically, the child with posterior urethral valves presents with the sequelae of high-grade intravesical obstruction, namely infection and renal deterioration. The posterior urethra becomes markedly dilated and may manifest itself, on physical examination, as an enlarged prostate. The marked degree of lower urinary tract obstruction may result in the development of a urachal fistula in the newborn child. Instances of transudation of urine through the bladder, or perforation of small bladder saccules causing ascites in newborn infants, have also been reported. 28 Unfortunately, the upper urinary tract in these children is almost always dilated significantly. The presence of large, tortuous, dilated ureters with associated hydronephrosis is the rule. Cystic changes in the kidney, compatible with type IV polycystic disease, as described by Potter,20 may result from these chronic obstructions. Although the changes present within the urinary tract are usually quite marked, the incidence of associated congenital anomalies is low. Most patients with posterior urethral valves will have symptoms during the first year of life. In a series reported by Williams,29 one half of the children with posterior urethral valves were seen within the first 3 months of life.
240
HENRY BURKO, ROBERT
K.
RHAMY
The presenting symptoms are failure to thrive, vomiting, recurring infection, and an abnormal voiding pattern. Physical examination may reveal palpable kidneys and bladder. The prostate may also feel enlarged. Tests of renal function demonstrate a rapid deterioration during the first 3 weeks of life. The diagnosis of posterior urethral valve is established or confirmed by voiding cystourethrography. During voiding, contrast material outlines the valves and demonstrates their obstructive nature (Fig. 5). The valves appear as transverse or curvilinear filling defects extending from the region of the verumontanum, usually ballooning downward and causing obstructive dilatation of the prostatic urethra. The distention of the prostatic urethra may attain such proportions that the valves actually present at the level of the membranous urethra. The presence of the obstructing valves causes secondary hypertrophy of the bladder wall, manifesting trabeculation and diverticula formation, with the thickened bladder wall indenting the column of opaque contrast medium at the level of the vesical neck. Congenital bladder neck obstruction and posterior urethral valves do not occur concomitantly, and the apparent constriction at the vesical neck is a manifestation of detrusor muscle hypertrophy, as outlined by contrast medium in the bladder proximally and in the dilated prostatic urethra distally. Commonly, there is reflux into the seminal vesicles and vas deferens during micturition. Approximately one third of these children demonstrate ureteral reflux V':hich augments upper tract dilatation caused by chronic obstruction. Intravenous urography usually demonstrates dilated upper tract with tortuous, dilated ureters.
Figure 5. Posterior urethral valves demonstrated by a spot film during voiding. The irregular outline of the bladder and the deep indentations at the vesical neck are manifestations of severe bladder wall hypertrophy secondary to chronic 0 bs truction. The prostatic urethra is markedly distended proximal to type I valves which balloon downward into the membranous urethra. A diminutive stream of opacified urine is seen within the anterior urethra. (Courtesy of Dr. Reginald Keith Waterhouse.)
URINARY TRACT INFECTION
241
Endoscopic examination is essential for confirmation of the presence of posterior urethral valve. The appearance of the bladder and bladder neck will reflect changes secondary to the chronic obstruction. The posterior urethra will be dilated with elongation of the veru in a longitudinal plane. Folds will be seen extending downward and outward from the verumontanum or, on occasion, a diaphragm-like membrane may be present in the region of the verumontanum. Treatment of posterior urethral valves and their complications may tax the resources of the pediatrician and the urologist. Correction of dehydration, electrolyte imbalances, and consequences of supressed renal function may require attention prior to the initiation of definitive treatment of the prostatic urethral valves. In some instances, peritoneal dialysis may be necessary to improve the child's condition sufficiently to allow operative intervention. 28 In the most severe cases, the condition of the child may limit the operative procedure to that of urinary diversion. The use of a urethral catheter is mentioned only to be condemned because it does not provide adequate drainage and inevitably results in the formation of urethral strictures. The use of a suprapubic cystostomy is also condemned because markedly hypertrophied bladders, in many instances, will contract down into a sphere around the catheter or suprapubic tube with resultant reduction in bladder capacity. In the seriously ill child, drainage proximal to the urinary bladder is often essential. Tube drainage has undesirable side effects, and bilateral nephrostomy drainage has serious long-range drawbacks. The use of a loop ureterostomy, as described by Williams and Eckstein,30 has been extremely valuable in decompressing the upper urinary tract and improving renal functional status. When the patient's condition allows surgical correction of urethral valve, removal by endoscopy or open operative resection may be performed. Transurethral resection through a perineal urethrostomy has been frequently employed, but we favor the retropubic approach with resection of the prostatic valvular leaflets, under direct vision. The relatively high position of the prostatic urethra in the young male allows this operation to be easily performed. In most instances, resection of the vesical neck hypertrophy is not required. The hypertrophy usually recedes spontaneously after relief of the distal urethral obstruction. If ureteral reflux is present, conservative management of the reflux should be employed initially and reimplantation of the ureters should be contemplated only if persistence of reflux results in deterioration of upper urinary tract function. Approximately one half of the children with concomitant reflux will have spontaneous regression in the first 6 months after valve resection. Williams 29 has reported that children presenting with posterior urethral valves before the age of 3 months have a mortality rate of almost 50 per cent, while those presenting after the age of 3 months have a mortality rate of approximately 12 per cent. The prognosis is dependent upon the degree of upper urinary tract obstruction initially demonstrated.
242
HENRY BURKO, ROBERT
K. RHAMY
BLADDER NECK OBSTRUCTION During voiding, we see a disproportion of the forces of expulsion and the forces of resistance. During contraction of the detrusor, the intravesical pressure rises and concomitantly with this, there must be a reduction in the resistance to the outflow of urine. When these two events occur in the proper fashion, voiding is the result. Thus, without proper decrease in the resistance to the flow of urine, residual urine may result. This may be produced at the vesical neck by dysfunction or actual anatomic contracture. The most flagrant abuse of the observations gained from voiding cystourethrography has been in the interpretation of rings, lips, and indentations in the region of the bladder outlet. In a carefully documented study of patients, Shopfner22 has demonstrated that indentations in the region of the bladder neck did not form passages of a smaller diameter than those found at the level of the external sphincter or the distal urethra and therefore could not be considered sites of obstruction to bladder emptying. Considerable variation in the diameter of the bladder neck is found in different individuals of the same age and size, and in the same individuals during various stages of voiding. No higher incidence of secondary signs of obstruction, such as vesicoureteral reflux, bladder trabeculation, or significant residual urine, was found in patients with and without indentations in the region of the bladder neck. Analysis of thousands of voiding cystourethrograms has demonstrated that such structures as rings, lips, and indentations found in the vast majority of patients younger than 2 years, represent manifestations of normal anatomic structures undergoing physiologic change during the act of voiding. These rings and indentations represent corresponding positions of the elements of the base plate of the urinary bladder. The movements of the base plate observed during micturition assume a more vertical orientation as a child gets older (somewhere between the ages of 2 and 5 years), whereafter the indentations in the region of the bladder outlet are no longer evident on voiding cystourethrography.22 The fact that these indentations do not form the narrowest diameter in the lower urinary tract, that they are seen in equal frequency in normal and ill patients, and that they are not associated with secondary signs of obstruction more frequently than in other children, makes it difficult to utilize these structures as roentgenologic evidence of obstruction. Bladder neck obstruction has been the subject of wide differences of opinion and extensive re-evaluation. The frequency with which this lesion is encountered varies considerably: bladder neck obstruction is described as the most common cause of urinary tract infection by some observers, and its existence is denied by others. Generally, the diagnosis has been made less frequently in recent years than in the past (Table 4). Obstructive lower urinary tract disorders causing residual urine, persistent or recurrent infection, deterioration of the upper urinary tracts, and vesicoureteral reflux require the critical evaluation of all methods of lower urinary tract examination. Radiographic assessment of the vesical neck as a cause of lower urinary tract obstruction is contradicted
243
URINARY TRACT INFECTION
Table 4. Incidence of Clinical Diagnosis of Bladder Neck Obstruction (Vanderbilt University Medical Center) YEAR
NUMBER OF PATIENTS
1965 1966 1967 1968 1969
16 7 4 5 1
by the appearance of identical rings, lips, and indentations in normal voiding patterns. The voiding cystogram, however, is useful for documenting changes attributed to obstruction and for eliminating other causes of lower urinary tract obstruction. The accuracy of endoscopic diagnosis of bladder neck obstruction has been subjected to considerable criticism. The use of voiding pressures and measurement of flow rates have been confusing because of the marked overlap between normal values and those values indicative of obstruction. In order to restore adequate voiding, the urologist may resort to surgical efforts to reduce the resistance to voiding. The most commonly employed method of bladder neck resection at the present time is the Y-V plasty.32 An attitude of conservatism prevails regarding the reflux. Under the circumstances described, reflux often disappears after bladder neck resection. The procedure has not been attended by sexual dysfunction later in life.
MEATAL STENOSIS In congenital meatal stenosis, the orifice has a circular "pin hole" instead of a normal longitudinal slit. The diagnosis is easily made by inspection of the urethral meatus. Meatotomy is the treatment of choice. When hypospadias is present (particularly the coronal variety), the incidence of congenital meatal stenosis is considerably greater. The size of the meatus must be determined by grasping the skin behind the meatus and determining the distensibility of the orifice. Again, the diagnosis is made by simple inspection and meatotomy is the therapy of choice. Acquired meatal stenosis is a common condition in boys aged 8 to 10 months and older. It is more common in uncircumcized males. The etiology is usually a meatal ulcer developing in an enure tic child with a redundant foreskin. Subsequent scarring at the healed ulcer site results in meatal stenosis. Inspection of the meatus will demonstrate the healed scar tissue about the narrowed meatus. The roentgen diagnosis of distal urethral or meatal obstruction is fallacious. In the female, the distal urethral segment corresponds anatomically to the male membranous urethra. The distal urethral wall is composed largely of collagenous tissue with limitation in distensibility, as compared to the more proximal portion of the urethra. During voiding,
244
HENRY BURKO, ROBERT
K.
RHAMY
Figure 6. Normal female urethra demonstrated by cinefluorography. The proximal and mid portions of the urethra are dis tended by a large volume, high velocity flow of opacified urine. The bladder neck, distal urethra, and meatal orifice are sites of less marked distention because of their inherent anatomic characteristics. The diameter at each of these sites is well above average.
the proximal and mid portions of the urethra distend considerably in the presence of a large-volume, high-velocity flow (Fig. 6). Shopfner:l has demonstrated that the size of the proximal and mid portions of the urethra is well correlated with the size of the distal urethra-i.e., the largest diameter distal urethral segments were related to the largest mid and proximal urethral diameters. It is evident that a diagnosis of meatal stenosis rests entirely upon physical examination, and is not dependent upon the size of the proximal or distal urethral segments demonstrated during voiding cystourethrography.
ECTOPIC URETEROCELE Ureterocele is a congenital cystic dilatation at the distal end of the ureter. It may be either simple or ectopic, and approximately 75 per cent of ureteroceles are associated with ureteral duplication. The ectopic ureterocele, first described by Ericsson13 in 1954, arises in the ureter that has an ectopic orifice situated in or extending into the urethra. The ectopic ureterocele is always proximal to the external urethral sphincter; thus, incontinence does not occur. Ectopic ureterocele is almost always diagnosed in infants and small children and is more common in females than in males by an incidence of 13: 1, in the series reported by Ericsson. 13 Recurrent urinary tract infection, with or without urinary calculi, is the most common presenting symptom. The diagnosis of ectopic ureterocele is usually established by excretory urography. Retrograde cystography will also demonstrate a filling defect in the bladder which represents an ectopic ureterocele. The voiding cystourethrogram may demonstrate reflux into the ectopic ureterocele.
URINARY TRACT INFECTION
245
Figure 7. Bilateral ectopic ureteroceles demonstrated by intravenous urography. Note the depression and lateral deviation of the lower pole of each kidney by dilated ureters filled with unopacified urine. The enlarged submucosal portions of both ureteroceles cause filling defects in the opacified urinary bladder. (Courtesy of Dr. Walter E. Berdon.)
On intravenous urography, the lower pole calyces are displaced downward and laterally by the urine-filled ureteral structure extending to the upper pole of the kidney. Shortly after the intravenous injection of contrast medium, when the vascular spaces throughout all organs are filled with the contrast medium, the dilated ureter extending to the upper pole will have a relatively radiolucent appearance. The opacified urinary bladder will usually demonstrate a filling defect representing the submucosal portion of the ectopic ureter. There may be signs of obstruction or reflux into the ipsilateral or contralateral ureters. The filling defect may also obstruct the bladder outlet and urethral orifice2 (Fig. 7). Treatment of the ectopic ureterocele will depend upon the degree of obstruction of the proximal kidney. Total excision is performed for dilated, nonfunctioning systems. When appropriate, ureteroneocystoto my is performed to preserve the functioning segment of the kidney.
NEUROGENIC BLADDER Neurogenic bladder in children is almost always associated with myelodysplasia of one type or another. Bladder disorders are rarely seen in children with cerebral palsy, poliomyelitis, or various myopathies. The incidence of traumatic paraplegia in children is practically nil. The diagnosis of neurogenic bladder is best established by voiding cystourethrography.
246
HENRY BURKO, ROBERT
K. RHAMY
Figure 8. Neurogenic bladder demonstrated by cinecystourethrography. The bladder outline is grossly irregular, indicating hypertrophy secondary to chronic obstruction. The bladder neck is widely funneled. Obstruction to voiding is caused by spasm of the external sphincter. Reflux into the left ureter is also noted.
The contour of the filled urinary bladder may be smooth and elongated, irregular secondary to trabeculations or diverticula or both, or triangular with irregular outline. In a majority of neurogenic bladders, the bladder outlet is widely patent and the external sphincter is either spastic or intermittently or incompletelyrelaxed7 (Fig. 8). A large residual volume of contrast material is usually present following micturition. In instances in which detrusor tone is poor, residual contrast material can be expressed from the bladder by the Crede maneuver. Substantiation of neurogenic bladder disorder can be made by roentgen examination of the vertebral column. The following bony anomalies and deformities are associated with neurogenic bladder: spina bifida, sacral agenesis, sacral hypoplasia, hemisacrum, sacral scoliosis, congenital block vertebra, and diastematomyelia. Bony deformities secondary to neurofibromatosis, inflammatory diseases, and arteriovenous malformations are also causes of neurogenic disorder of the bladder. 10 The radiographic demonstration of external sphincter spasm is attributed to neurogenic disorder of the bladder when bony anomalies or deformities are detected, or when clinical or electromyographic evidence of neuromuscular disorder is elicited. External sphincter spasm in the absence of other findings may be due to the following causes: (1) neurogenic disorder of the bladder in a patient who may subsequently demonstrate other manifestations of neurologic disorder; (2) spasm secondary to urethritis; (3) spasm secondary to irritation of the introduced urethral catheter; or (4) voluntary contraction of the sphincter in an otherwise normal patient.
URINARY TRACT INFECTION
247
Therapy for these lesions will depend upon whether the bladder is flaccid or spastic, whether the child is male or female, whether there is sensation present or not, and whether upper tracts are normal or not. Initially, most of these children do well with the Crede maneuver in an attempt to adequately empty the bladder. Sometimes, with a spastic component, the maneuver will worsen the upper tract dilatation and its use must be discontinued. In children with adequate emptying, the social aspects of the disease may dictate operative intervention. Whatever the mode of therapy of this lesion, the goal should be a tube type of drainage, free from infection: one which will render the child acceptable so that he may attend school and perform in a normal fashion, insofar as possible. In many instances, urinary diversion into an ileal conduit will be required. These children ordinarily excrete sterile urine without the use of antibiotics.
THE TRIAD SYNDROME Congenital absence of the abdominal wall musculature associated with dilated ureters and undescended testes represents a maldevelopment of mesenchymal tissue. 25 This disorder affects males almost exclusively, although three controversial cases have been reported in females. The muscular deficiency involves the lower part of the rectus muscles and the lower and medial part of the oblique musculature. Only fat separates the skin from the peritoneum. The diagnosis can be made on the
Figure 9. Voiding cystoureth. rogram of a child with triad syndrome. The urinary bladder is oriented horizontally because of umbilical attachment. The bladder neck and prostatic urethra are markedly dilated. A persistent utriculus masculinis extends posteriorly from the dilated prostatic urethra. The anterior urethra is narrowed. Massive reflux into dilated, tortuous. and flaccid ureters is seen.
248
HENRY BURKO, ROBERT
K.
RHAMY
Figure 10. Triad syndrome. Close-up of the urethra. Note the dilated prostatic urethra with persistent utriculus masculinis. The caliber of the anterior urethra is smaller than normal.
basis of the classical appearance of the anterior abdominal wall and can be demonstrated with radiographic findings. Radiography demonstrates anterior and lateral bulging of the abdominal wall by gas-filled intestinal loops. On voiding cystourethrography, the bladder outlet is widely patent with elongation and dilatation of the prostatic urethra. The anterior urethra may be narrowed or may be of normal caliber. The bladder capacity is increased, with the bladder elongated and cone-shaped anterosuperiorly because of persistent urachal attachment. The ureters are dilated, elongated, and tortuous. Vesicoureteral reflux usually occurs. Peristaltic activity in the ureters is absent. On intravenous urography, the renal collecting structures are usually dilated and poorly functioning. Treatment should be directed toward providing adequate drainage. In severe cases, urinary diversion, either by cutaneous ureterostomies or into an ileal segment, will be necessary. The milder cases may be managed conservatively. Since all of the males demonstrate cryptorchism, orchiopexy may be required (Figs. 9 and 10).
CYSTOURETHRITIS Normally, in the resting phase, the bladder is smoothly outlined. Irregularity of the bladder outline may be due to chronic obstruction with cellule and diverticulum formation. In the absence of organic or
249
URINARY TRACT INFECTION
physiologic obstruction, irregularity in bladder outline may be the result of edema or mucosal hyperplasia secondary to chronic or recurrent infection. During voiding, inflammatory granulomas within the urethra may be demonstrated as linear or nodular filling defects in the opacified urethra. Good image detail, obtained by spot filming or 70 mm. fluorography, is usually necessary to demonstrate these findings.
REFLUX Is reflux abnormal? Vesicoureteral reflux is considered to be abnormal. The length of the intramural portion of the ureter as well as the angle of insertion of the ureter into the bladder wall, and the muscular investments of the lower ureter extending into the trigone of the bladder, are considered to be responsible for maintenance of competency of the ureterovesical junction. In animal experiments, intravesical pressures have been raised to the point of rupture before reflux could be produced. Voiding cystourethrography performed in newborn infants has failed to demonstrate reflux. How prevalent is reflux? The exact incidence of reflux in the general population is not known. Edwards 6 found reflux in 34.5 per cent of children investigated for urinary tract infection. The incidence of reflux detected during investigation of lower urinary tract infection varies from 12 to 48 per cent (Table 5). Approximately 48 per cent of children with reflux will demonstrate an abnormal intravenous urogram. Admittedly, some of the changes are reversible and represent an indication of altered dynamics during the period of reflux. However, many of the changes reflect irreversible pyelonephritis. The frequency of lower urinary tract infection in females is reflected in the higher incidence of upper urinary tract infection in girls and in women. How is reflux detected? The diagnosis of reflux is established by voiding cystourethrography. Reflux usually occurs during the period of voiding when there is a maximal increase in intravesical pressure. However, reflux may occasionally occur during low-volume filling of the urinary bladder and may only be transiently evident. For these reasons, fluoroscopic control during filling of the urinary bladder and during filming of the micturition event is necessary to detect fleeting episodes of reflux. What is the mechanism of reflux? Several causes for reflux are known and can be classified as follows: 1. The most common cause of reflux is lower urinary tract infection. The inflammatory edema of the bladder caused by infection renders the ureterovesical junction incompetent temporarily. Reflux disappears when infection subsides and the inflammatory edema no longer alters the competent ureterovesical mechanism. 2. Primary reflux is seen predominantly in females with thin-walled bladders and congenitally deficient ureterovesical junction mechanisms. The diagnosis is considered when reflux is noted to persist after subsidence of infection. Primary reflux is seen less commonly than in-
250 Table 5.
HENRY BURKO, ROBERT
Incidence of Reflux in Children with
SOURCE AND DATE
NUMBER PER CENT OF CASES REFLUX
RHAMY
Pyelonephritis~'
TYPE OF CYSTOGRAM
12
Children with pyuria and enuresis
Immediate and after voiding
13.5
Children with bladder neck obstruction
Immediate, voiding, and after voiding
290
34
Children with urinary tract infection withou t obstruction or neurologic lesion
Voiding cystogram (multiple films during voiding)
51
39
Children with recurring urinary tract infection
Voiding cystogram (multiple films during voiding)
McGovern et al.,' 1960
152
35
Symptomatic children
Multiple films, some during voiding
Palken, Kennelly," 1960
53
37.5
Girls with recurring urinary tract infection
Voiding cystourethrography and cine· fluoroscopy
Gross, Sanderson,' 1961
83
47
Children with recurring urinary tract infection
Cinefluoroscopy
190
48
Children and adults with urinary tract infection
Cinefluoroscopy
Campbell,' 1951 St. Martin et. al.,' 1956 Kjellberg et al.,' 1958
Forsythe, Wallace,' 1958
Hutch et al.," 1962
722
TYPE OF PATIENTS
K.
74
"From Hutch, Miller, and Hinman (1963a). 'Campbell, M.: Clinical Pediatric Urology. Philadelphia, W. B. Saunders Co., 1951. 'St. Martin, E. C., Campbell, J. H., and Pasquier, C. M.: J. Urol. (Baltimore),75:151, 1956. "Kjellberg, S. R, Ericsson, N. D., and Rudhe, U.: The Lower Urinary Tract in Childhood. Chicago, Yearbook Medical Publishers, 1957. 'Forsythe, W. J., and Wallace, 1. R: Brit. J. Urol., 30:297,1958. "McGovern, J. H., Marshall, V. F., and Paquin, A. J., Jr.: J. Urol. (Baltimore), 83: 122,1960. "Palken, M., and Kennelly, J., Jr.: J. Urol. (Baltimore), 83:745,1960. 'Gross, K. E., and Sanderson, S. S.: Radiology, 77:573, 1961. "Hutch, J. A., Hinman, F., Jr., and Miller, E. R, unpublished data.
flammatory reflux. Surgical intervention is necessary to cure primary reflux, when recurrent urinary tract infection is detected. 3. Chronic obstruction causing anatomic changes in the hypertrophied bladder wall may result in reflux. Posterior urethral valves and neurogenic bladder disorders are largely responsible for bladder wall hypertrophy. However, concomitant infection often is present in these conditions. 4. Recently, attention has been directed to the neurogenic origin of reflux, demonstrated by sectioning the lumbar sympathetic chain, both in animals and humans. 26 Many patients with previous lumbar sympathectomies for arteriosclerotic vascular insuffiCiency have subsequently developed reflux.
URINARY TRACT INFECTION
251
5. Congenital types of reflux are also seen in duplication of the urinary tract and ectopically placed ureteral orifices. 6. Iatrogenic causes of reflux include ureteral meatotomy, transurethral resection, and simple non-tunneling techniques of ureteroneocystotomy. What is the prognosis in a child who has reflux? The incidence of reflux detected in lower urinary tract infection constitutes a major problem. Many children have repeated lower urinary tract infections, some of which are unrecognized. The growing kidney is considerably more sensitive to infection than is the adult kidney. The result of chronic or recurrent reflux of infected urine is a development of chronic pyelonephritis by the route of ascending infection. In the series of patients with chronic reflux, 12 per cent developed an atrophic kidney.24 The changes usually started before the age of 5 and in most instances prior to the age of 2 years. What course of management is suggested in the child with urinary tract infection? Following detection of urinary tract infection, a urine culture is obtained and appropriate antibiotic therapy is undertaken. An intravenous urogram at this time is advised to determine the presence of upper urinary tract obstruction and other serious renal disorders. Most investigators believe that, following an initial lower urinary tract infection in the male and an initial or second lower urinary tract infection in the female, a thorough investigation of the entire urinary tract should be undertaken, as outlined previously. The voiding cystourethrogram should be performed 6 weeks after subsidence of the lower urinary tract infection, in order to detect primary reflux unrelated to inflammatory changes. Approximately two thirds of children having ureteral reflux during infection will be free of reflux after successful antibiotic treatment. If the voiding cystourethrogram is abnormal, cystoscopy is advised. If the ureteral orifices are normal cystoscopic8;lly, no specific treatment is required at this time. However, if the ureteral orifices appear markedly abnormal cystoscopically, consideration should be given to early surgical intervention. If a child has recurrent urinary tract infection, a repeat intravenous urogram and voiding cystourethrogram are necessary to assess renal deterioration and the persistence or recurrence of reflux. When should reflux be treated surgically? Those children who harbor infection while on medication, who have recurrence of infection following adequate medical management, or who have evidence of deterioration of the upper urinary tract, require surgical intervention to eliminate reflux and prevent progressive renal deterioration. The techniques of surgery used to prevent reflux are excellent when dealing with the normal caliber ureter. The Politano-Leadbetter procedure l9 has been effective in preventing recurrences of reflux in 89 per cent of cases. In only 2 per cent of cases has it been necessary to reimplant the ureter a second time. The dilated ureter represents a more serious problem and the success rate is considerably less than with the normalsized ureter. In the group with dilated ureters, the success rate has been 60 per cent, using the Paquin technique. IS
252
HENRY BURKO, ROBERT
K.
RHAMY
SUMMARY Lower urinary tract infection in children is a serious problem whose incidence and sequelae have been fully appreciated in the past decade. The morbidity and mortality attributed to adult pyelonephritis demands an awareness of the role of ascending infection from the lower urinary tract, often beginning in infancy and childhood. An increasing incidence of virulent organisms adds to the necessity of comprehensive examination of the entire urinary tract. Voiding cystourethrography is an essential method of examining the lower urinary tract. The examination can be performed with the crudest radiographic equipment, although refinements, such as 70mm. image-intensifier fluorography, can provide the most accurate data, excellent image quality, and minimal gonadal dose. Children with posterior urethral valves present with sequelae of high grade obstruction: infection and renal deterioration. Voiding cystourethrography establishes the diagnosis and demonstrates the secondary changes of obstruction. Therapy is directed to improvement of the child's general condition, and often upper tract decompression must be accomplished before the valves can be resected. The existence and incidence of bladder neck obstruction'are controversial. The demonstration of obstructive phenomena: residual urine, bladder hypertrophy, persistent infection, reflux, and renal deterioration, necessitate an empiric reduction of resistance to voiding. This is accomplished by V-V plasty, often curing reflux and obviating reimplantation procedures. Meatal stenosis is diagnosed by physical examination and is cured by meatotomy. Ectopic ureterocele, a cystic dilation of the distal ureter, is diagnosed by intravenous urography or voiding cystourethrography, and is treated by resection or diversion to relieve ureteral obstruction. Neurogenic bladder disorders in children are physiologic obstructions usually caused by external sphincter spasm. Associated anomalies elicited on pbysical examination or radiographic demonstration of bony deformities, provide confirmatory evidence. Augmented voiding techniques or urinary diversion into an ileal conduit are satisfactory modes of therapy. The triad syndrome is a mesenchymal disorder characterized by absent abdominal wall musculature and dilatation and atony of the lower urinary tract structures, resulting in stasis and upper tract deterioration. Urinary diversion by cutaneous ureterostomies or ileal loop construction is required in severe cases. Vesicoureteral reflux is abnormal and most commonly is the result of lower urinary tract infection. Many children with reflux will respond to long-term, appropriate antibiotic therapy. If response to medical management fails, especially when upper tract deterioration is evident, ureteral reimplantation is necessary. With a normal-sized ureter, surgical reimplantation is usually successful. REFERENCES 1. Bazy, P.: A propos du diagnostic des lesions renal unilaterales. Bull. Soc. Chir., 29:32,
1903.
URINARY TRACT INFECTION
253
2. Berdon, W. E., Baker, T. H., Becker, J. A., and Uson, A. C.: Ectopic ureterocele. Radiol. Clin. N. Amer., 6:205-214, 1968. 3. Bugbee, H. G., and Wollstein, M.: Surgical pathology of the urinary tract in infants. J.A.M.A., 83:1887,1924. 4. Burke, J. B.: Pyelonephritis in infancy and childhood. Lancet, 2: 1116, 1961. 5. Davis, L. A., Lich, R., Howerton, L., and Joule, W.: The lower urinary tract in infants and children. Radiology, 77:445-451, 1961. 6. Edwards, D.: The lower urinary tract. Proc. World Soc. of Me d., 59:417,1966. 7. Edwards, R. H., Burko, H., Rhamy, R. K., and Trapp, J. D.: Cine-radiographic analysis of neurogenic bladder in children. J. Urol., 100:290-292, 1968. 8. Gould, H. R., and Peterson, C. G.: VOiding cystourethrography in children. The use of rapid mm changer. Amer. J. Roentgenol., 98:192-199,1966. 9. Griesbach, W. A., Waterhouse, R. K., and Mellins, H. Z.: Voiding cystourethrography in the diagnosis of congenital posterior urethral valve. Amer. J. Roentgenol., 82:521-529, 1959. 10. Grossman, H., Winchester, P. H., and Colston, W. C.: Neurogenic bladder in childhood. Radiol. Clin. N. Amer. 6:155-163, 1968. 11. Kaude, J. V., and Reed, J. M.: VOiding urethro-cystography by means of 70 mm. image intensifier fluorography. Radiology, 92:768-770,1969. 12. Kaude, J. V., Lorenz, E., and Reed, J. M.: Gonad dose to children in voiding urethrocystography performed with 70 mm. image intensifier fluorography. Radiology, 92: 771-774,1969. 13. Kjellberg, S. R., Ericsson, N. E., and Rudhe, U.: The Lower Urinary Tract in Children. Chicago, Yearbook Medical Publishers, Inc., 1957. 14. Kunin, C. M., and Paquin, A. J.: Frequency and natural history of urinary tract infection in school children. In Kass, E. H., ed.: Progress in Pyelonephritis. Philadelphia, F. A. Davis Co., 1965, p. 33. 15. Laurell, G., and Lindbom, G.: Urinary tract infections. 1. Drugs and bugs in urinary tract infection from the bacteriologist's point of view. Acta Paediat., 49: 189, 1960. 16. Michie, A. J.: Pediatric urology. Summary of a round table. Pediatrics, 24:1118, 1959. 17. N ogrady, M. D., and Dunbar, J. S.: The value of excretory micturition cystourethrography (EMCU) in the pediatric age group. J. Canad. Assoc. Radiologists, 16:181-189, 1965. 18. Paquin, A. J.: Ureterovesical anastomosis: Description and evaluation of a technique. J. Urol., 82:573, 1959. 19. Politano, V. A., and Leadbetter, W. F.: An operative technique for correction of vesicoureteral reflux. J. Urol., 79:932, 1958. 20. Potter, E. L., and Osathanondh, B.: Normal and abnormal lievelopment of the kidney. In Mostofi, F. K., and Smith, D. E., eds.: The Kidney. Baltimore, The Williams and Wilkins Co., 1966. 21. Shopfner, C. E.: Clinical evaluation of cystourethrographic contrast media. Radiology, 88:491-497, 1967. 22. Shopfner, C. E.: Roentgenologic evaluation of bladder neck obstruction. Amer. J. Roentgenol.,100:162-176,1967. 23. Shopfner, C. E.: Roentgen evaluation of distal urethral obstruction. Radiology, 88:222231, 1967. 24. Smallpiece, V.: Urinary Tract Infection in Childhood and its Relevance to Disease in Adult Life. St. Louis, C. V. Mosby Co., 1969. 25. Stephens, F. D.: Congenital Malformations of the Rectum, Anus, and Genitourinary Tracts. Edinburgh and London, E. & S. Livingstone, Ltd., 1963. 26. Tanagho, E. A.. Hutch, J. A., Meyers, R. H., and Rambo, O. N.: Primary vesicoureteral reflux: Experimental studies of its etiology. J. Urol., 93:165-176, 1965. 27. Thornbury, J. R., and Immergut, M. A.: Poly-view voiding cystourethrography in child. reno Amer. J. Roentgenol., 95:475-478,1965. 28. Waterhouse, K.: Anomalies of the urethra. In Encyclopedia of Urology. Berlin, SpringerVerlag, 1968. 29. Williams, D.: Grand rounds, Southwestern Medical School, 1964. 30. Williams, D. I., and Eckstein, H. B.: Obstructive valves in the posterior urethra: Case. J. Urol., 35:524, 1936. 31. Young, H. H., Frontz, Z. A., and Baldwin, K. C.: Congenital obstruction of the posterior urethra. J. Urol., 3:289, 1919. 32. Young, B. W., and Niebel, J. D.: Vesico-urethroplasty for congenital vesical neck obstruc. tion in children. J. Urol., 79:838, 1958. Vanderbilt University School of Medicine Nashville, Tennessee 37203
During the past decade, the significance and sequelae of urinary tract infections in children have evoked considerable interest. Sensitive bacteriologic criteria and sophisticated radiographic techniques have aided in diagnosing and evaluating urinary tract infections more frequently. In presenting this review of lower urinary tract problems related to infection, our responsibility to the pediatrician and to his patient necessitates an evaluation of the accuracy and limitations of the diagnostic methods employed and an appraisal of the treatment of these problems, based on our present beliefs resulting from considerable personal experience.
INCIDENCE OF URINARY TRACT INFECTIONS IN CHILDHOOD Asymptomatic bacilluria has been reported by Kunin et al. 14 in 1.2 per cent of 16,000 school children. Michie l6 found that 5 per cent of all children on pediatric services had significant urinary tract infection (U.S.A.); and Burke4 reported that 3 to 4 per cent of all pediatric admissions to the Wittington Hospital in London were due to this condition. Bugbee and Wollstein,3 in reporting 4903 autopsies, found an incidence of 4.5 per cent of pyelonephritis in children. It is evident that a significant segment of pediatric practice is related to urinary tract infections and their sequelae (Table 1). During the first 3 months of life, the incidence of urinary tract infection is approximately equal in males and females. However, over From the Departments of Radiology and Urology, Vanderbilt University School of Medicine, Nashville, Tennessee ·Professor of Radiology ··Professor and Chairman of the Division of Urology Pediatric Clinics of North America- VoL 17, No.2, May, 1970
233
234
HENRY BURKO, ROBERT
K.
RHAMY
Table 1. Clinical Symptoms and Signs of Children Referred for Roentgen Examination of the Lower Urinary Tract (410 Patients) Infection Infection, repeated, with previous reflux Neurogenic abnormality'" Dysuria Enuresis Oiliff
40% 20% 13% 12% 12% 3%
"'The high percentage of neurogenic abnormality reflects the activities of the Birth Defect Center at Vanderbilt.
the age of 3 months, urinary tract infection in females is three times as frequent as in males. 24
ROUTES OF INFECTION Much has been written concerning the etiology of urinary tract infections. Hematogenous dissemination of microorganisms to the urinary tract has been proposed for many years; and, indeed, it is probably more common in children than in adults. Nevertheless, a hematogenous source for urinary tract infection is unusual. Spread of infection to the urinary tract via lymphatics from adjacent organs or by direct extension from adjacent organs is rare. The most common pathway for the introduction of microorganisms is ascent to the upper urinary tract via the urethra and lower urinary tract. The frequency of ascending infection in the urinary tract necessitates a complete evaluation of the lower urinary tract in any child who exhibits one or more bouts of urinary tract infection.
ORGANISMS In the uncomplicated, noninstrumented lower urinary tract infection, the occurrence of multiple bacterial species is infrequent. Laurell and Lindbom15 stated that 80 to 100 per cent of uncomplicated infections were due to a single species. The most common organism encountered in uncomplicated urinary tract infections in children is Escherichia coli. The frequency of various organisms involved in urinary tract infection is outlined in Table 2. Through the years, the percentages of uncomplicated infections caused by E. coli have diminished, and higher percentages of more virulent organisms, such as Klebsiella, Pseudomonas, and Proteus, have been reported from many clinics. In our experience, the child who has been instrumented has a three times greater chance of developing a urinary tract infection due to a resistant organism, compared to a child who has not been instrumented. The increasing incidence of urinary tract infections and an alarming increase in the detection of virulent organisms, with greater chance of drug resistance, provides a stimulus
235
URINARY TRACT INFECTION
Table 2. Organisms Involved in Urinary Tract Infections (2000 Patients)
\
'I
I
~
UNCOMPLICATED
COMPLICATED
Single Organisms Mixed Organisms
80% 20%
20% 80%
E. coli Paracolon Klebsiella Proteus Pseudomonas Enterococci Staphylococci
82% 5% 2% 5% 0 14% 4%
18% 5% 45% 43% 7% 31% 9%
No significant differences in organisms were found between pediatric and adult age groups.
for critical evaluation of methods of investigation and aggressive therapeutic management.
METHODS OF INVESTIGATION OF THE URINARY TRACT Investigation of the urinary tract requires a survey of the functional and bacteriologic status of the urinary system from the glomerulus to the meatus. Therefore, evaluation should consist of: 1. A good history and physical examination.
2. 3. 4. 5. 6. 7.
Urine culture, bacterial counts, and sensitivities. Renal functional assessment. Excretory urography. Voiding cystourethrography. Endoscopic examination. Flow and pressure measurements, when feasible.
ROENTGENOGRAPHIC METHODS OF PERFORMING VOIDING CYSTOURETHROGRAPHY Mode of Filling Excretory micturition cystourethrography is the performance of a voiding cystourethrogram after the urinary bladder has been filled with contrast material injected intravenously.17 Catheterization of the urethra is thereby avoided, but the concentration within the urinary bladder is often inadequate to obtain a diagnostic study. Cooperation on the part of the patient is required so that voiding will not occur prior to roentgen recording. Generally, this is not feasible in the first 2 or 3 years of life. Although this method may provide a diagnostic examination in many instances (572 studies out of 728 performed), its reliability is inadequate for general application. Filling the bladder through a urethral catheter is accomplished preferentially by gravity. The catheter is connected to a container of contrast
236
HENRY BURKO, ROBERT
K.
RHAMY
material placed 1 meter above the table. Manual injection with a syringe attached to the urethral catheter is an alternate method of filling the bladder, which should be performed under fluoroscopic control by an experienced examiner. The size and configuration of the urinary bladder, as observed fluoroscopically, recognized discomfort to the patient, and the detection of spontaneous voiding around the catheter provide clues to the adequacy of bladder filling and the imminence of voiding. Contrast Material Sodium or meglumine diatrizoate or iothalamate diluted to a 25 to 30 per cent solution are easily visi;"l.e, nonirritating contrast media. 21 Method of Recording The simplest, and possibly the crudest, method of recording voiding cystourethrography is the exposure of a single roentgenogram beneath the patient, during the act of micturition. It is evident that by utilizing this method, even the simplest radiographic equipment is suitable for performance of the examination. A classic paper, describing posterior urethral valves, was published, utilizing this crude methodY Serial radiography, utilizing either manual or automatic film changing, can provide four or more exposures during voiding. B• 27 As in the single exposure method described above, this method of filming does not allow fluoroscopic observation and is, therefore, less desirable than the methods to be described subsequently.
Figure 1. Normal female urethra demonstrated by spot film during voiding. Note the funneling of the base plate of the bladder.
URINARY TRACT INFECTION
237
Figure 2. Normal female urethra demonstrated by spot film during voiding. Note extreme funneling of the base plate of the bladder into the urethra. Vaginal filling posterior to the urethra is a normal occurrence of voiding during recumbency.
Conventional spot filming under fluoroscopic control, preferably with image intensification, is a widely used method for recording micturition. 5 Four to eight exposures with excellent film detail can be obtained. A further refinement is the use of 70 mm. image-intensifier fluorography, allowing up to 45 exposures and providing excellent image
Figure 3. Normal male urethra demonstrated by spot film during voiding. Complete funneling of the base plate of the bladder into the prostatic urethra is seen. Anterior indentation in the prostatic urethra is caused by descent of the bladder during high velocity voiding. Posterior indentations in the bladder wall represent normal detrusor contractions.
238
HENRY BURKO, ROBERT
K.
RHAMY
Table 3. Analysis of 410 Voiding Cinecystourethrograms (July 1968 to June 1969) ROENTGEN INTERPRETATION
NUMBER OF CASES
Normal Vesicoureteral reflux, all causes Neurogenic bladder Unsuccessful or inconclusive examinations External sphincter spasm, etiology unknown Periureteral diverticulum Cystourethritis, without reflux Posterior urethral valve Megacystis-megatrigone syndrome Vesical outlet obstruction Meatal stenosis
282 67 27 13 7 7 5 1 1
o o
quality.u The gonadal dose utilizing 70 mm. film is lower than with conventional spot filming and cinefluorography. Cinecystourethrography enables the recording of the entire micturition event under fluoroscopic observation and is especially useful in studying details of physiologic events and alterations in the voiding pattern, such as occur in neurogenic disorders of the bladder. Image detail is not as good as with conventional spot filming or 70 mm. fluorography. Gonadal dose is within an acceptable range, varying with the duration of filming. At the present time, conventional spot filming, 70 mm. fluorography, and cinefluorography are the most commonly utilized methods of examination, and 70 mm. fluorography is the preferred method because of excellent image quality, facility for recording a considerable number of events, and minimizing gonadal dose12 (Figs. 1 to 3). Table 3 provides an analysis of roentgen findings of voiding cinecystourethrograms performed in a recent year. For the most part, the symptoms and signs were those of urinary tract infection (Table 1).
POSTERIOR URETHRAL VALVES Waterhouse28 has described 27 examples of posterior urethral valves in the examination of 2500 children. The examination of 1461 children at the Karolinska Institute, by Kjellberg, Ericsson, and Rudhe,13 demonstrated posterior urethral valves in 52 patients. It is noteworthy that the diagnosis of posterior urethral valves was rarely made ante mortem, prior to the utilization of voiding cystourethrography. The Young classification31 of types of valves is generally used. In type I, single or double folds on the posterior urethral wall run downward and laterally from the verumontanum. Type III valve is an iris diaphragm, above or below the verumontanum (Fig. 4). Type II valves are thought by Stephens25 not to exist: none have been seen in our series. The embryologic origin of these valves remains in dispute. Bazy'sl suggestion of the persistence of the urogenital membrane would account for type III valves. Stephens' explanation for type I valves indicates that
239
URINARY TRACT INFECTION
TYPE I VALVES
TYPE III VALVES Figure 4. Posterior urethral valves. Type I valves are embryologic remnants extending downward and laterally from the verumontanum. Type III valves are persistent membranes located either proximal or distal to the verumontanum.
the valve results from "deficient integration of the Wolffian ducts into the walls of the urethra, to abnormal locations of the original orifice into the cloaca, and to an abnormal course of their distal ends."25 (Fig. 4). Clinically, the child with posterior urethral valves presents with the sequelae of high-grade intravesical obstruction, namely infection and renal deterioration. The posterior urethra becomes markedly dilated and may manifest itself, on physical examination, as an enlarged prostate. The marked degree of lower urinary tract obstruction may result in the development of a urachal fistula in the newborn child. Instances of transudation of urine through the bladder, or perforation of small bladder saccules causing ascites in newborn infants, have also been reported. 28 Unfortunately, the upper urinary tract in these children is almost always dilated significantly. The presence of large, tortuous, dilated ureters with associated hydronephrosis is the rule. Cystic changes in the kidney, compatible with type IV polycystic disease, as described by Potter,20 may result from these chronic obstructions. Although the changes present within the urinary tract are usually quite marked, the incidence of associated congenital anomalies is low. Most patients with posterior urethral valves will have symptoms during the first year of life. In a series reported by Williams,29 one half of the children with posterior urethral valves were seen within the first 3 months of life.
240
HENRY BURKO, ROBERT
K.
RHAMY
The presenting symptoms are failure to thrive, vomiting, recurring infection, and an abnormal voiding pattern. Physical examination may reveal palpable kidneys and bladder. The prostate may also feel enlarged. Tests of renal function demonstrate a rapid deterioration during the first 3 weeks of life. The diagnosis of posterior urethral valve is established or confirmed by voiding cystourethrography. During voiding, contrast material outlines the valves and demonstrates their obstructive nature (Fig. 5). The valves appear as transverse or curvilinear filling defects extending from the region of the verumontanum, usually ballooning downward and causing obstructive dilatation of the prostatic urethra. The distention of the prostatic urethra may attain such proportions that the valves actually present at the level of the membranous urethra. The presence of the obstructing valves causes secondary hypertrophy of the bladder wall, manifesting trabeculation and diverticula formation, with the thickened bladder wall indenting the column of opaque contrast medium at the level of the vesical neck. Congenital bladder neck obstruction and posterior urethral valves do not occur concomitantly, and the apparent constriction at the vesical neck is a manifestation of detrusor muscle hypertrophy, as outlined by contrast medium in the bladder proximally and in the dilated prostatic urethra distally. Commonly, there is reflux into the seminal vesicles and vas deferens during micturition. Approximately one third of these children demonstrate ureteral reflux V':hich augments upper tract dilatation caused by chronic obstruction. Intravenous urography usually demonstrates dilated upper tract with tortuous, dilated ureters.
Figure 5. Posterior urethral valves demonstrated by a spot film during voiding. The irregular outline of the bladder and the deep indentations at the vesical neck are manifestations of severe bladder wall hypertrophy secondary to chronic 0 bs truction. The prostatic urethra is markedly distended proximal to type I valves which balloon downward into the membranous urethra. A diminutive stream of opacified urine is seen within the anterior urethra. (Courtesy of Dr. Reginald Keith Waterhouse.)
URINARY TRACT INFECTION
241
Endoscopic examination is essential for confirmation of the presence of posterior urethral valve. The appearance of the bladder and bladder neck will reflect changes secondary to the chronic obstruction. The posterior urethra will be dilated with elongation of the veru in a longitudinal plane. Folds will be seen extending downward and outward from the verumontanum or, on occasion, a diaphragm-like membrane may be present in the region of the verumontanum. Treatment of posterior urethral valves and their complications may tax the resources of the pediatrician and the urologist. Correction of dehydration, electrolyte imbalances, and consequences of supressed renal function may require attention prior to the initiation of definitive treatment of the prostatic urethral valves. In some instances, peritoneal dialysis may be necessary to improve the child's condition sufficiently to allow operative intervention. 28 In the most severe cases, the condition of the child may limit the operative procedure to that of urinary diversion. The use of a urethral catheter is mentioned only to be condemned because it does not provide adequate drainage and inevitably results in the formation of urethral strictures. The use of a suprapubic cystostomy is also condemned because markedly hypertrophied bladders, in many instances, will contract down into a sphere around the catheter or suprapubic tube with resultant reduction in bladder capacity. In the seriously ill child, drainage proximal to the urinary bladder is often essential. Tube drainage has undesirable side effects, and bilateral nephrostomy drainage has serious long-range drawbacks. The use of a loop ureterostomy, as described by Williams and Eckstein,30 has been extremely valuable in decompressing the upper urinary tract and improving renal functional status. When the patient's condition allows surgical correction of urethral valve, removal by endoscopy or open operative resection may be performed. Transurethral resection through a perineal urethrostomy has been frequently employed, but we favor the retropubic approach with resection of the prostatic valvular leaflets, under direct vision. The relatively high position of the prostatic urethra in the young male allows this operation to be easily performed. In most instances, resection of the vesical neck hypertrophy is not required. The hypertrophy usually recedes spontaneously after relief of the distal urethral obstruction. If ureteral reflux is present, conservative management of the reflux should be employed initially and reimplantation of the ureters should be contemplated only if persistence of reflux results in deterioration of upper urinary tract function. Approximately one half of the children with concomitant reflux will have spontaneous regression in the first 6 months after valve resection. Williams 29 has reported that children presenting with posterior urethral valves before the age of 3 months have a mortality rate of almost 50 per cent, while those presenting after the age of 3 months have a mortality rate of approximately 12 per cent. The prognosis is dependent upon the degree of upper urinary tract obstruction initially demonstrated.
242
HENRY BURKO, ROBERT
K. RHAMY
BLADDER NECK OBSTRUCTION During voiding, we see a disproportion of the forces of expulsion and the forces of resistance. During contraction of the detrusor, the intravesical pressure rises and concomitantly with this, there must be a reduction in the resistance to the outflow of urine. When these two events occur in the proper fashion, voiding is the result. Thus, without proper decrease in the resistance to the flow of urine, residual urine may result. This may be produced at the vesical neck by dysfunction or actual anatomic contracture. The most flagrant abuse of the observations gained from voiding cystourethrography has been in the interpretation of rings, lips, and indentations in the region of the bladder outlet. In a carefully documented study of patients, Shopfner22 has demonstrated that indentations in the region of the bladder neck did not form passages of a smaller diameter than those found at the level of the external sphincter or the distal urethra and therefore could not be considered sites of obstruction to bladder emptying. Considerable variation in the diameter of the bladder neck is found in different individuals of the same age and size, and in the same individuals during various stages of voiding. No higher incidence of secondary signs of obstruction, such as vesicoureteral reflux, bladder trabeculation, or significant residual urine, was found in patients with and without indentations in the region of the bladder neck. Analysis of thousands of voiding cystourethrograms has demonstrated that such structures as rings, lips, and indentations found in the vast majority of patients younger than 2 years, represent manifestations of normal anatomic structures undergoing physiologic change during the act of voiding. These rings and indentations represent corresponding positions of the elements of the base plate of the urinary bladder. The movements of the base plate observed during micturition assume a more vertical orientation as a child gets older (somewhere between the ages of 2 and 5 years), whereafter the indentations in the region of the bladder outlet are no longer evident on voiding cystourethrography.22 The fact that these indentations do not form the narrowest diameter in the lower urinary tract, that they are seen in equal frequency in normal and ill patients, and that they are not associated with secondary signs of obstruction more frequently than in other children, makes it difficult to utilize these structures as roentgenologic evidence of obstruction. Bladder neck obstruction has been the subject of wide differences of opinion and extensive re-evaluation. The frequency with which this lesion is encountered varies considerably: bladder neck obstruction is described as the most common cause of urinary tract infection by some observers, and its existence is denied by others. Generally, the diagnosis has been made less frequently in recent years than in the past (Table 4). Obstructive lower urinary tract disorders causing residual urine, persistent or recurrent infection, deterioration of the upper urinary tracts, and vesicoureteral reflux require the critical evaluation of all methods of lower urinary tract examination. Radiographic assessment of the vesical neck as a cause of lower urinary tract obstruction is contradicted
243
URINARY TRACT INFECTION
Table 4. Incidence of Clinical Diagnosis of Bladder Neck Obstruction (Vanderbilt University Medical Center) YEAR
NUMBER OF PATIENTS
1965 1966 1967 1968 1969
16 7 4 5 1
by the appearance of identical rings, lips, and indentations in normal voiding patterns. The voiding cystogram, however, is useful for documenting changes attributed to obstruction and for eliminating other causes of lower urinary tract obstruction. The accuracy of endoscopic diagnosis of bladder neck obstruction has been subjected to considerable criticism. The use of voiding pressures and measurement of flow rates have been confusing because of the marked overlap between normal values and those values indicative of obstruction. In order to restore adequate voiding, the urologist may resort to surgical efforts to reduce the resistance to voiding. The most commonly employed method of bladder neck resection at the present time is the Y-V plasty.32 An attitude of conservatism prevails regarding the reflux. Under the circumstances described, reflux often disappears after bladder neck resection. The procedure has not been attended by sexual dysfunction later in life.
MEATAL STENOSIS In congenital meatal stenosis, the orifice has a circular "pin hole" instead of a normal longitudinal slit. The diagnosis is easily made by inspection of the urethral meatus. Meatotomy is the treatment of choice. When hypospadias is present (particularly the coronal variety), the incidence of congenital meatal stenosis is considerably greater. The size of the meatus must be determined by grasping the skin behind the meatus and determining the distensibility of the orifice. Again, the diagnosis is made by simple inspection and meatotomy is the therapy of choice. Acquired meatal stenosis is a common condition in boys aged 8 to 10 months and older. It is more common in uncircumcized males. The etiology is usually a meatal ulcer developing in an enure tic child with a redundant foreskin. Subsequent scarring at the healed ulcer site results in meatal stenosis. Inspection of the meatus will demonstrate the healed scar tissue about the narrowed meatus. The roentgen diagnosis of distal urethral or meatal obstruction is fallacious. In the female, the distal urethral segment corresponds anatomically to the male membranous urethra. The distal urethral wall is composed largely of collagenous tissue with limitation in distensibility, as compared to the more proximal portion of the urethra. During voiding,
244
HENRY BURKO, ROBERT
K.
RHAMY
Figure 6. Normal female urethra demonstrated by cinefluorography. The proximal and mid portions of the urethra are dis tended by a large volume, high velocity flow of opacified urine. The bladder neck, distal urethra, and meatal orifice are sites of less marked distention because of their inherent anatomic characteristics. The diameter at each of these sites is well above average.
the proximal and mid portions of the urethra distend considerably in the presence of a large-volume, high-velocity flow (Fig. 6). Shopfner:l has demonstrated that the size of the proximal and mid portions of the urethra is well correlated with the size of the distal urethra-i.e., the largest diameter distal urethral segments were related to the largest mid and proximal urethral diameters. It is evident that a diagnosis of meatal stenosis rests entirely upon physical examination, and is not dependent upon the size of the proximal or distal urethral segments demonstrated during voiding cystourethrography.
ECTOPIC URETEROCELE Ureterocele is a congenital cystic dilatation at the distal end of the ureter. It may be either simple or ectopic, and approximately 75 per cent of ureteroceles are associated with ureteral duplication. The ectopic ureterocele, first described by Ericsson13 in 1954, arises in the ureter that has an ectopic orifice situated in or extending into the urethra. The ectopic ureterocele is always proximal to the external urethral sphincter; thus, incontinence does not occur. Ectopic ureterocele is almost always diagnosed in infants and small children and is more common in females than in males by an incidence of 13: 1, in the series reported by Ericsson. 13 Recurrent urinary tract infection, with or without urinary calculi, is the most common presenting symptom. The diagnosis of ectopic ureterocele is usually established by excretory urography. Retrograde cystography will also demonstrate a filling defect in the bladder which represents an ectopic ureterocele. The voiding cystourethrogram may demonstrate reflux into the ectopic ureterocele.
URINARY TRACT INFECTION
245
Figure 7. Bilateral ectopic ureteroceles demonstrated by intravenous urography. Note the depression and lateral deviation of the lower pole of each kidney by dilated ureters filled with unopacified urine. The enlarged submucosal portions of both ureteroceles cause filling defects in the opacified urinary bladder. (Courtesy of Dr. Walter E. Berdon.)
On intravenous urography, the lower pole calyces are displaced downward and laterally by the urine-filled ureteral structure extending to the upper pole of the kidney. Shortly after the intravenous injection of contrast medium, when the vascular spaces throughout all organs are filled with the contrast medium, the dilated ureter extending to the upper pole will have a relatively radiolucent appearance. The opacified urinary bladder will usually demonstrate a filling defect representing the submucosal portion of the ectopic ureter. There may be signs of obstruction or reflux into the ipsilateral or contralateral ureters. The filling defect may also obstruct the bladder outlet and urethral orifice2 (Fig. 7). Treatment of the ectopic ureterocele will depend upon the degree of obstruction of the proximal kidney. Total excision is performed for dilated, nonfunctioning systems. When appropriate, ureteroneocystoto my is performed to preserve the functioning segment of the kidney.
NEUROGENIC BLADDER Neurogenic bladder in children is almost always associated with myelodysplasia of one type or another. Bladder disorders are rarely seen in children with cerebral palsy, poliomyelitis, or various myopathies. The incidence of traumatic paraplegia in children is practically nil. The diagnosis of neurogenic bladder is best established by voiding cystourethrography.
246
HENRY BURKO, ROBERT
K. RHAMY
Figure 8. Neurogenic bladder demonstrated by cinecystourethrography. The bladder outline is grossly irregular, indicating hypertrophy secondary to chronic obstruction. The bladder neck is widely funneled. Obstruction to voiding is caused by spasm of the external sphincter. Reflux into the left ureter is also noted.
The contour of the filled urinary bladder may be smooth and elongated, irregular secondary to trabeculations or diverticula or both, or triangular with irregular outline. In a majority of neurogenic bladders, the bladder outlet is widely patent and the external sphincter is either spastic or intermittently or incompletelyrelaxed7 (Fig. 8). A large residual volume of contrast material is usually present following micturition. In instances in which detrusor tone is poor, residual contrast material can be expressed from the bladder by the Crede maneuver. Substantiation of neurogenic bladder disorder can be made by roentgen examination of the vertebral column. The following bony anomalies and deformities are associated with neurogenic bladder: spina bifida, sacral agenesis, sacral hypoplasia, hemisacrum, sacral scoliosis, congenital block vertebra, and diastematomyelia. Bony deformities secondary to neurofibromatosis, inflammatory diseases, and arteriovenous malformations are also causes of neurogenic disorder of the bladder. 10 The radiographic demonstration of external sphincter spasm is attributed to neurogenic disorder of the bladder when bony anomalies or deformities are detected, or when clinical or electromyographic evidence of neuromuscular disorder is elicited. External sphincter spasm in the absence of other findings may be due to the following causes: (1) neurogenic disorder of the bladder in a patient who may subsequently demonstrate other manifestations of neurologic disorder; (2) spasm secondary to urethritis; (3) spasm secondary to irritation of the introduced urethral catheter; or (4) voluntary contraction of the sphincter in an otherwise normal patient.
URINARY TRACT INFECTION
247
Therapy for these lesions will depend upon whether the bladder is flaccid or spastic, whether the child is male or female, whether there is sensation present or not, and whether upper tracts are normal or not. Initially, most of these children do well with the Crede maneuver in an attempt to adequately empty the bladder. Sometimes, with a spastic component, the maneuver will worsen the upper tract dilatation and its use must be discontinued. In children with adequate emptying, the social aspects of the disease may dictate operative intervention. Whatever the mode of therapy of this lesion, the goal should be a tube type of drainage, free from infection: one which will render the child acceptable so that he may attend school and perform in a normal fashion, insofar as possible. In many instances, urinary diversion into an ileal conduit will be required. These children ordinarily excrete sterile urine without the use of antibiotics.
THE TRIAD SYNDROME Congenital absence of the abdominal wall musculature associated with dilated ureters and undescended testes represents a maldevelopment of mesenchymal tissue. 25 This disorder affects males almost exclusively, although three controversial cases have been reported in females. The muscular deficiency involves the lower part of the rectus muscles and the lower and medial part of the oblique musculature. Only fat separates the skin from the peritoneum. The diagnosis can be made on the
Figure 9. Voiding cystoureth. rogram of a child with triad syndrome. The urinary bladder is oriented horizontally because of umbilical attachment. The bladder neck and prostatic urethra are markedly dilated. A persistent utriculus masculinis extends posteriorly from the dilated prostatic urethra. The anterior urethra is narrowed. Massive reflux into dilated, tortuous. and flaccid ureters is seen.
248
HENRY BURKO, ROBERT
K.
RHAMY
Figure 10. Triad syndrome. Close-up of the urethra. Note the dilated prostatic urethra with persistent utriculus masculinis. The caliber of the anterior urethra is smaller than normal.
basis of the classical appearance of the anterior abdominal wall and can be demonstrated with radiographic findings. Radiography demonstrates anterior and lateral bulging of the abdominal wall by gas-filled intestinal loops. On voiding cystourethrography, the bladder outlet is widely patent with elongation and dilatation of the prostatic urethra. The anterior urethra may be narrowed or may be of normal caliber. The bladder capacity is increased, with the bladder elongated and cone-shaped anterosuperiorly because of persistent urachal attachment. The ureters are dilated, elongated, and tortuous. Vesicoureteral reflux usually occurs. Peristaltic activity in the ureters is absent. On intravenous urography, the renal collecting structures are usually dilated and poorly functioning. Treatment should be directed toward providing adequate drainage. In severe cases, urinary diversion, either by cutaneous ureterostomies or into an ileal segment, will be necessary. The milder cases may be managed conservatively. Since all of the males demonstrate cryptorchism, orchiopexy may be required (Figs. 9 and 10).
CYSTOURETHRITIS Normally, in the resting phase, the bladder is smoothly outlined. Irregularity of the bladder outline may be due to chronic obstruction with cellule and diverticulum formation. In the absence of organic or
249
URINARY TRACT INFECTION
physiologic obstruction, irregularity in bladder outline may be the result of edema or mucosal hyperplasia secondary to chronic or recurrent infection. During voiding, inflammatory granulomas within the urethra may be demonstrated as linear or nodular filling defects in the opacified urethra. Good image detail, obtained by spot filming or 70 mm. fluorography, is usually necessary to demonstrate these findings.
REFLUX Is reflux abnormal? Vesicoureteral reflux is considered to be abnormal. The length of the intramural portion of the ureter as well as the angle of insertion of the ureter into the bladder wall, and the muscular investments of the lower ureter extending into the trigone of the bladder, are considered to be responsible for maintenance of competency of the ureterovesical junction. In animal experiments, intravesical pressures have been raised to the point of rupture before reflux could be produced. Voiding cystourethrography performed in newborn infants has failed to demonstrate reflux. How prevalent is reflux? The exact incidence of reflux in the general population is not known. Edwards 6 found reflux in 34.5 per cent of children investigated for urinary tract infection. The incidence of reflux detected during investigation of lower urinary tract infection varies from 12 to 48 per cent (Table 5). Approximately 48 per cent of children with reflux will demonstrate an abnormal intravenous urogram. Admittedly, some of the changes are reversible and represent an indication of altered dynamics during the period of reflux. However, many of the changes reflect irreversible pyelonephritis. The frequency of lower urinary tract infection in females is reflected in the higher incidence of upper urinary tract infection in girls and in women. How is reflux detected? The diagnosis of reflux is established by voiding cystourethrography. Reflux usually occurs during the period of voiding when there is a maximal increase in intravesical pressure. However, reflux may occasionally occur during low-volume filling of the urinary bladder and may only be transiently evident. For these reasons, fluoroscopic control during filling of the urinary bladder and during filming of the micturition event is necessary to detect fleeting episodes of reflux. What is the mechanism of reflux? Several causes for reflux are known and can be classified as follows: 1. The most common cause of reflux is lower urinary tract infection. The inflammatory edema of the bladder caused by infection renders the ureterovesical junction incompetent temporarily. Reflux disappears when infection subsides and the inflammatory edema no longer alters the competent ureterovesical mechanism. 2. Primary reflux is seen predominantly in females with thin-walled bladders and congenitally deficient ureterovesical junction mechanisms. The diagnosis is considered when reflux is noted to persist after subsidence of infection. Primary reflux is seen less commonly than in-
250 Table 5.
HENRY BURKO, ROBERT
Incidence of Reflux in Children with
SOURCE AND DATE
NUMBER PER CENT OF CASES REFLUX
RHAMY
Pyelonephritis~'
TYPE OF CYSTOGRAM
12
Children with pyuria and enuresis
Immediate and after voiding
13.5
Children with bladder neck obstruction
Immediate, voiding, and after voiding
290
34
Children with urinary tract infection withou t obstruction or neurologic lesion
Voiding cystogram (multiple films during voiding)
51
39
Children with recurring urinary tract infection
Voiding cystogram (multiple films during voiding)
McGovern et al.,' 1960
152
35
Symptomatic children
Multiple films, some during voiding
Palken, Kennelly," 1960
53
37.5
Girls with recurring urinary tract infection
Voiding cystourethrography and cine· fluoroscopy
Gross, Sanderson,' 1961
83
47
Children with recurring urinary tract infection
Cinefluoroscopy
190
48
Children and adults with urinary tract infection
Cinefluoroscopy
Campbell,' 1951 St. Martin et. al.,' 1956 Kjellberg et al.,' 1958
Forsythe, Wallace,' 1958
Hutch et al.," 1962
722
TYPE OF PATIENTS
K.
74
"From Hutch, Miller, and Hinman (1963a). 'Campbell, M.: Clinical Pediatric Urology. Philadelphia, W. B. Saunders Co., 1951. 'St. Martin, E. C., Campbell, J. H., and Pasquier, C. M.: J. Urol. (Baltimore),75:151, 1956. "Kjellberg, S. R, Ericsson, N. D., and Rudhe, U.: The Lower Urinary Tract in Childhood. Chicago, Yearbook Medical Publishers, 1957. 'Forsythe, W. J., and Wallace, 1. R: Brit. J. Urol., 30:297,1958. "McGovern, J. H., Marshall, V. F., and Paquin, A. J., Jr.: J. Urol. (Baltimore), 83: 122,1960. "Palken, M., and Kennelly, J., Jr.: J. Urol. (Baltimore), 83:745,1960. 'Gross, K. E., and Sanderson, S. S.: Radiology, 77:573, 1961. "Hutch, J. A., Hinman, F., Jr., and Miller, E. R, unpublished data.
flammatory reflux. Surgical intervention is necessary to cure primary reflux, when recurrent urinary tract infection is detected. 3. Chronic obstruction causing anatomic changes in the hypertrophied bladder wall may result in reflux. Posterior urethral valves and neurogenic bladder disorders are largely responsible for bladder wall hypertrophy. However, concomitant infection often is present in these conditions. 4. Recently, attention has been directed to the neurogenic origin of reflux, demonstrated by sectioning the lumbar sympathetic chain, both in animals and humans. 26 Many patients with previous lumbar sympathectomies for arteriosclerotic vascular insuffiCiency have subsequently developed reflux.
URINARY TRACT INFECTION
251
5. Congenital types of reflux are also seen in duplication of the urinary tract and ectopically placed ureteral orifices. 6. Iatrogenic causes of reflux include ureteral meatotomy, transurethral resection, and simple non-tunneling techniques of ureteroneocystotomy. What is the prognosis in a child who has reflux? The incidence of reflux detected in lower urinary tract infection constitutes a major problem. Many children have repeated lower urinary tract infections, some of which are unrecognized. The growing kidney is considerably more sensitive to infection than is the adult kidney. The result of chronic or recurrent reflux of infected urine is a development of chronic pyelonephritis by the route of ascending infection. In the series of patients with chronic reflux, 12 per cent developed an atrophic kidney.24 The changes usually started before the age of 5 and in most instances prior to the age of 2 years. What course of management is suggested in the child with urinary tract infection? Following detection of urinary tract infection, a urine culture is obtained and appropriate antibiotic therapy is undertaken. An intravenous urogram at this time is advised to determine the presence of upper urinary tract obstruction and other serious renal disorders. Most investigators believe that, following an initial lower urinary tract infection in the male and an initial or second lower urinary tract infection in the female, a thorough investigation of the entire urinary tract should be undertaken, as outlined previously. The voiding cystourethrogram should be performed 6 weeks after subsidence of the lower urinary tract infection, in order to detect primary reflux unrelated to inflammatory changes. Approximately two thirds of children having ureteral reflux during infection will be free of reflux after successful antibiotic treatment. If the voiding cystourethrogram is abnormal, cystoscopy is advised. If the ureteral orifices are normal cystoscopic8;lly, no specific treatment is required at this time. However, if the ureteral orifices appear markedly abnormal cystoscopically, consideration should be given to early surgical intervention. If a child has recurrent urinary tract infection, a repeat intravenous urogram and voiding cystourethrogram are necessary to assess renal deterioration and the persistence or recurrence of reflux. When should reflux be treated surgically? Those children who harbor infection while on medication, who have recurrence of infection following adequate medical management, or who have evidence of deterioration of the upper urinary tract, require surgical intervention to eliminate reflux and prevent progressive renal deterioration. The techniques of surgery used to prevent reflux are excellent when dealing with the normal caliber ureter. The Politano-Leadbetter procedure l9 has been effective in preventing recurrences of reflux in 89 per cent of cases. In only 2 per cent of cases has it been necessary to reimplant the ureter a second time. The dilated ureter represents a more serious problem and the success rate is considerably less than with the normalsized ureter. In the group with dilated ureters, the success rate has been 60 per cent, using the Paquin technique. IS
252
HENRY BURKO, ROBERT
K.
RHAMY
SUMMARY Lower urinary tract infection in children is a serious problem whose incidence and sequelae have been fully appreciated in the past decade. The morbidity and mortality attributed to adult pyelonephritis demands an awareness of the role of ascending infection from the lower urinary tract, often beginning in infancy and childhood. An increasing incidence of virulent organisms adds to the necessity of comprehensive examination of the entire urinary tract. Voiding cystourethrography is an essential method of examining the lower urinary tract. The examination can be performed with the crudest radiographic equipment, although refinements, such as 70mm. image-intensifier fluorography, can provide the most accurate data, excellent image quality, and minimal gonadal dose. Children with posterior urethral valves present with sequelae of high grade obstruction: infection and renal deterioration. Voiding cystourethrography establishes the diagnosis and demonstrates the secondary changes of obstruction. Therapy is directed to improvement of the child's general condition, and often upper tract decompression must be accomplished before the valves can be resected. The existence and incidence of bladder neck obstruction'are controversial. The demonstration of obstructive phenomena: residual urine, bladder hypertrophy, persistent infection, reflux, and renal deterioration, necessitate an empiric reduction of resistance to voiding. This is accomplished by V-V plasty, often curing reflux and obviating reimplantation procedures. Meatal stenosis is diagnosed by physical examination and is cured by meatotomy. Ectopic ureterocele, a cystic dilation of the distal ureter, is diagnosed by intravenous urography or voiding cystourethrography, and is treated by resection or diversion to relieve ureteral obstruction. Neurogenic bladder disorders in children are physiologic obstructions usually caused by external sphincter spasm. Associated anomalies elicited on pbysical examination or radiographic demonstration of bony deformities, provide confirmatory evidence. Augmented voiding techniques or urinary diversion into an ileal conduit are satisfactory modes of therapy. The triad syndrome is a mesenchymal disorder characterized by absent abdominal wall musculature and dilatation and atony of the lower urinary tract structures, resulting in stasis and upper tract deterioration. Urinary diversion by cutaneous ureterostomies or ileal loop construction is required in severe cases. Vesicoureteral reflux is abnormal and most commonly is the result of lower urinary tract infection. Many children with reflux will respond to long-term, appropriate antibiotic therapy. If response to medical management fails, especially when upper tract deterioration is evident, ureteral reimplantation is necessary. With a normal-sized ureter, surgical reimplantation is usually successful. REFERENCES 1. Bazy, P.: A propos du diagnostic des lesions renal unilaterales. Bull. Soc. Chir., 29:32,
1903.
URINARY TRACT INFECTION
253
2. Berdon, W. E., Baker, T. H., Becker, J. A., and Uson, A. C.: Ectopic ureterocele. Radiol. Clin. N. Amer., 6:205-214, 1968. 3. Bugbee, H. G., and Wollstein, M.: Surgical pathology of the urinary tract in infants. J.A.M.A., 83:1887,1924. 4. Burke, J. B.: Pyelonephritis in infancy and childhood. Lancet, 2: 1116, 1961. 5. Davis, L. A., Lich, R., Howerton, L., and Joule, W.: The lower urinary tract in infants and children. Radiology, 77:445-451, 1961. 6. Edwards, D.: The lower urinary tract. Proc. World Soc. of Me d., 59:417,1966. 7. Edwards, R. H., Burko, H., Rhamy, R. K., and Trapp, J. D.: Cine-radiographic analysis of neurogenic bladder in children. J. Urol., 100:290-292, 1968. 8. Gould, H. R., and Peterson, C. G.: VOiding cystourethrography in children. The use of rapid mm changer. Amer. J. Roentgenol., 98:192-199,1966. 9. Griesbach, W. A., Waterhouse, R. K., and Mellins, H. Z.: Voiding cystourethrography in the diagnosis of congenital posterior urethral valve. Amer. J. Roentgenol., 82:521-529, 1959. 10. Grossman, H., Winchester, P. H., and Colston, W. C.: Neurogenic bladder in childhood. Radiol. Clin. N. Amer. 6:155-163, 1968. 11. Kaude, J. V., and Reed, J. M.: VOiding urethro-cystography by means of 70 mm. image intensifier fluorography. Radiology, 92:768-770,1969. 12. Kaude, J. V., Lorenz, E., and Reed, J. M.: Gonad dose to children in voiding urethrocystography performed with 70 mm. image intensifier fluorography. Radiology, 92: 771-774,1969. 13. Kjellberg, S. R., Ericsson, N. E., and Rudhe, U.: The Lower Urinary Tract in Children. Chicago, Yearbook Medical Publishers, Inc., 1957. 14. Kunin, C. M., and Paquin, A. J.: Frequency and natural history of urinary tract infection in school children. In Kass, E. H., ed.: Progress in Pyelonephritis. Philadelphia, F. A. Davis Co., 1965, p. 33. 15. Laurell, G., and Lindbom, G.: Urinary tract infections. 1. Drugs and bugs in urinary tract infection from the bacteriologist's point of view. Acta Paediat., 49: 189, 1960. 16. Michie, A. J.: Pediatric urology. Summary of a round table. Pediatrics, 24:1118, 1959. 17. N ogrady, M. D., and Dunbar, J. S.: The value of excretory micturition cystourethrography (EMCU) in the pediatric age group. J. Canad. Assoc. Radiologists, 16:181-189, 1965. 18. Paquin, A. J.: Ureterovesical anastomosis: Description and evaluation of a technique. J. Urol., 82:573, 1959. 19. Politano, V. A., and Leadbetter, W. F.: An operative technique for correction of vesicoureteral reflux. J. Urol., 79:932, 1958. 20. Potter, E. L., and Osathanondh, B.: Normal and abnormal lievelopment of the kidney. In Mostofi, F. K., and Smith, D. E., eds.: The Kidney. Baltimore, The Williams and Wilkins Co., 1966. 21. Shopfner, C. E.: Clinical evaluation of cystourethrographic contrast media. Radiology, 88:491-497, 1967. 22. Shopfner, C. E.: Roentgenologic evaluation of bladder neck obstruction. Amer. J. Roentgenol.,100:162-176,1967. 23. Shopfner, C. E.: Roentgen evaluation of distal urethral obstruction. Radiology, 88:222231, 1967. 24. Smallpiece, V.: Urinary Tract Infection in Childhood and its Relevance to Disease in Adult Life. St. Louis, C. V. Mosby Co., 1969. 25. Stephens, F. D.: Congenital Malformations of the Rectum, Anus, and Genitourinary Tracts. Edinburgh and London, E. & S. Livingstone, Ltd., 1963. 26. Tanagho, E. A.. Hutch, J. A., Meyers, R. H., and Rambo, O. N.: Primary vesicoureteral reflux: Experimental studies of its etiology. J. Urol., 93:165-176, 1965. 27. Thornbury, J. R., and Immergut, M. A.: Poly-view voiding cystourethrography in child. reno Amer. J. Roentgenol., 95:475-478,1965. 28. Waterhouse, K.: Anomalies of the urethra. In Encyclopedia of Urology. Berlin, SpringerVerlag, 1968. 29. Williams, D.: Grand rounds, Southwestern Medical School, 1964. 30. Williams, D. I., and Eckstein, H. B.: Obstructive valves in the posterior urethra: Case. J. Urol., 35:524, 1936. 31. Young, H. H., Frontz, Z. A., and Baldwin, K. C.: Congenital obstruction of the posterior urethra. J. Urol., 3:289, 1919. 32. Young, B. W., and Niebel, J. D.: Vesico-urethroplasty for congenital vesical neck obstruc. tion in children. J. Urol., 79:838, 1958. Vanderbilt University School of Medicine Nashville, Tennessee 37203