Venkata R. Jayanthi

THE RELATIONSHIP AMONG DYSFUNCTIONAL ELIMINATION SYNDROMES, PRIMARY VESICOURETERAL REFLUX AND URINARY TRACT INFECTIONS IN CHILDREN

PURPOSE We determine whether functional bladder and/or bowel disorders influence the natural history or treatment of children with primary vesicoureteral reflux. MATERIALS AND METHODS We assessed 143 children with primary vesicoureteral reflux that stopped spontaneously or was surgically corrected for functional bowel and/or bladder disorders, including bladder instability, constipation and infrequent voiding, termed the dysfunctional elimination syndromes. RESULTS Dysfunctional elimination syndromes were present in 66 of 143 children (43%) thought to have primary vesicoureteral reflux. Of these 66 patients 54 (82%) had a breakthrough urinary tract infection and underwent reimplantation compared to only 18% without the syndromes. Of 70 children who had a breakthrough urinary tract infection dysfunctional elimination syndromes were present in 54 (77%) and absent in 16 (23%). Of the remaining 73 patients who did not have a breakthrough infection dysfunctional elimination syndromes were present in 12 (16%) and absent in 61 (84%). In children with dysfunctional elimination syndromes the resolution of reflux that was 1 grade less severe required an average of 1.6 years longer. After the disappearance of reflux, urinary tract infection developed in 18 children, including 14 (78%) with dysfunctional elimination syndromes. Unsuccessful surgical outcomes involving persistent, recurrent and contralateral reflux occurred only in children with dysfunctional elimination syndromes. CONCLUSIONS Dysfunctional elimination syndromes are common and are often unrecognized in children with primary reflux. These syndromes are associated with delayed reflux resolution and an increased rate of breakthrough urinary tract infection, which leads to reimplantation surgery. Dysfunctional elimination syndromes also adversely affect the results of reimplantation and represent a risk for recurrent urinary tract infection after reflux resolves. The evaluation and management of dysfunctional elimination syndromes should be an integral part of the treatment of every child with vesicoureteral reflux. Effective evaluation and treatment may be made cost-effective by decreasing the followup, the number of breakthrough urinary tract infections and the number of children requiring reimplantation.

THE LONG-TERM FOLLOWUP OF NEWBORNS WITH SEVERE UNILATERAL HYDRONEPHROSIS INITIALLY TREATED NONOPERATIVELY

PURPOSE During the last decade it has become apparent that prenatally detected, unilateral severe hydronephrosis does not necessarily represent obstruction and may spontaneously improve or resolve postnatally. To define its natural history better we performed a long-term (mean 78 months) followup study of infants with hydronephrosis. MATERIALS AND METHODS A total of 104 newborns with antenatally diagnosed, primary, unilateral severe hydronephrosis were followed nonoperatively unless evidence of renal deterioration occurred for which pyeloplasty was performed. RESULTS All 23 infants (22%) who required pyeloplasty were younger than 18 months and had progressive hydronephrosis and/or reduction in differential renal function. Differential function exceeded predeterioration levels in all kidneys postoperatively. Of those cases followed nonoperatively hydronephrosis resolved in 69% and improved in 31%. Mean time to maximum improvement of hydronephrosis was 2.5 years. In 76% of those cases followed nonoperatively initial differential function was greater than 40% and final function averaged 49%. In the remaining 24% of cases differential function was less than 40% (mean 23%), and in an average of 18 months differential function increased to a mean of 47%. Initial half-time in nonoperative cases was greater than 30 minutes in 37%, 20 to 30 in 21% and less than 20 in 42%. Final half-time was greater than 30 minutes in 16%, 20 to 30 in 17% and less than 20 in 67%. Half-time was greater than 30 minutes in 87% of the patients and 20 to 30 in 4% before, and greater than 30 in 10%, 20 to 30 in 27% and less than 20 in 63% after pyeloplasty. CONCLUSIONS Unilateral newborn hydronephrosis appears to be relatively benign and in most instances dilatation and renal function improve with time. However, close followup is necessary to identify the subgroup of less than 25% of infants with obstruction because prompt pyeloplasty will prevent permanent loss of renal function. Standard tests for assessing obstruction in older patients appear to be invalid in infants because prolonged half-time and/or high grade hydronephrosis is neither an indicator of obstruction or surgery. Nonoperative treatment with close followup especially during the first 2 years is safe and recommended for these children.

STRATEGIES FOR MANAGING UPPER TRACT CALCULI IN YOUNG CHILDREN

PURPOSE Pediatric urolithiasis is relatively uncommon and there is little information on the application of modern surgical procedures in young children. We present a single center experience with the surgical management of upper tract calculi in this age group. MATERIALS AND METHODS We reviewed presentation, co-morbidity, treatment, outcome and complications in all prepubertal patients who required surgical treatment for ureteral or renal calculi during a 4-year period. The series consists of 24 girls and 17 boys 17 months to 14 years old (mean age 7.5 years). A total of 26 children were anatomically normal, and 4 had myelomeningocele, 4 had ureteropelvic junction obstruction (in a pelvic kidney in 1), 2 had cloacal anomalies, 2 had vesicoureteral reflux, and 1 each had nonrefluxing megaureter, orthotopic ureterocele and a functioning renal transplant. RESULTS Extracorporeal shock wave lithotripsy was performed in 24 patients. Stents or nephrostomy tubes were only used in the 4 patients who presented with pyonephrosis. Of the 41 cases 17 were rendered stone-free, 3 had a decreased stone burden and 4 were failures. Ureteroscopic extraction of distal ureteral calculi was successful in 11 of 12 children, of whom the youngest was 2.5 years old. No child had postoperative infection or evidence of ureteral obstruction. Stent placement facilitated stone passage or dissolution in 2 patients, a renal calculus was percutaneously extracted in 2 and 7 required open surgery, mostly for correcting simultaneous anatomical abnormalities or after minimally invasive surgery failed. Some metabolic abnormality was detected in 80% of the children tested. CONCLUSIONS The surgical management of upper urinary tract calculi in young children parallels that in adults. Minimally invasive surgical methods may be safely used even in young infants. Most children do not need elective stenting before lithotripsy. Open procedures are still required in 17% of cases. The majority of children have definable metabolic abnormalities.

Long-term Followup of Prenatally Detected Severe Bilateral Newborn Hydronephrosis Initially Managed Nonoperatively

PURPOSE We determine the outcome of severe bilateral primary ureteropelvic junction type hydronephrosis detected prenatally and managed postnatally with an initially nonoperative protocol. MATERIALS AND METHODS A total of 19 newborns (38 kidneys) with prenatally diagnosed primary grade 3 to 4 bilateral hydronephrosis were followed nonoperatively for a mean of 54 months (range 14 to 187). If urinary obstruction with evidence of renal deterioration (decreased differential function and/or progressive hydronephrosis) occurred pyeloplasty was performed. RESULTS Pyeloplasty was required in 13 kidneys (35%) in 9 patients (bilateral 4, unilateral 5). Age at pyeloplasty ranged from 2 to 22 months (mean 6.5) in 12 patients and 64 months in 1. The remaining 25 kidneys were followed nonoperatively (bilateral 20, unilateral 5). At last followup the Society for Fetal Urology grade of hydronephrosis in kidneys followed nonoperatively was 0 to 2 in 21 and 3 in 4, compared to 0 to 2 in 9 and Society for Fetal Urology 3 in 4 kidneys treated with pyeloplasty. Mean followup required for the most severely hydronephrotic kidney to achieve maximum ultrasound improvement was 10 months (range 3 to 34) for kidneys followed nonoperatively and 14 months (4-31) for kidneys after pyeloplasty. Differential renal function was measured in each kidney pair and compared using the difference in percent function between the 2 kidneys. In the nonoperative group mean initial difference in percent function was 8% (range 6% to 20%) and mean final difference was 5% (2% to 8%). In the pyeloplasty group mean initial difference in percent function was 16% (range 8% to 30%) and mean final difference was 7% (2% to 16%). With close followup and prompt pyeloplasty renal function improved to greater than pre-deterioration levels in all kidneys. CONCLUSIONS These data represent the natural history of severe bilateral newborn hydronephrosis. Renal dilatation and function improve with time in most kidneys. Close followup is required in the first 2 years of life to identify the subgroup (35%) of children with obstruction that requires prompt surgery. Such an approach prevented permanent loss of renal function. Nonoperative management with close followup during the first 2 years appears to be a safe and recommended approach for neonates with primary bilateral ureteropelvic junction type hydronephrosis.

Loss of Elasticity in Dysfunctional Bladders: Urodynamic and Histochemical Correlation

To store adequate volumes of urine at low safe pressures an elastic bladder wall is required. We developed 2 new techniques to measure this ability in our urodynamic laboratory: pressure specific bladder volume, which measures the bladder capacity at a given pressure, and dynamic analysis of bladder compliance. Recently, morphometric and histochemical techniques have been used to determine the relative volume of connective tissue in the bladder wall and to measure the 2 major types (I and III) of collagen within the bladder wall. These methods quantitate 3 parameters of bladder ultrastructure: 1) relative volume of per cent connective tissue, 2) ratio of connective tissue to smooth muscle and 3) ratio of type III to type I collagen. These parameters have been shown to be abnormally elevated in patients with dysfunctional bladders compared to normals. The purpose of the study was to describe the ultrastructural changes that occur in the wall of dysfunctional bladders and to determine the ability of these new urodynamic techniques to detect reliably the clinical effect of these histological changes. The study included 29 consecutive patients with dysfunctional bladders necessitating bladder augmentation. All patients had upper tract changes and/or were incontinent despite treatment with clean intermittent catheterization and pharmacotherapy. Preoperative urodynamic evaluation included measurement of the total bladder capacity, pressure specific bladder volume and dynamic analysis of bladder compliance. Full thickness bladder biopsies were obtained from the dome of the bladders during augmentation. The per cent connective tissue and the ratio of connective tissue to smooth muscle were determined for all patients, and 4 unselected patients from this group had the ratio of type III to type I collagen determined. These histological results were compared to previously established normal values. All 29 patients had a decreased pressure specific bladder volume and dynamic analysis of bladder compliance, whereas 9 had a normal total bladder capacity. The per cent connective tissue was 35.19 +/- 2.84 and ratio of connective tissue to smooth muscle was 0.60 +/- 0.08 compared to normal values of 10.6 +/- 0.020 and 0.131 +/- 0.021, respectively (p < 0.05). Ratio of type III to type I collagen was also significantly elevated in the 4 samples analyzed (30.53 +/- 1.37 versus 24.00 +/- 2.50, p < 0.05). We conclude that poor storage function of poorly compliant bladders is secondary to an alteration in the connective tissue content of the bladder wall. Furthermore, these pathological ultrastructural changes are universally reflected by an abnormally low pressure specific bladder volume and dynamic analysis of bladder compliance. This strong association validates the use of these parameters and suggests that they are urodynamic indicators of a loss of elasticity in bladder wall.

THE VALVE BLADDER SYNDROME: PATHOPHYSIOLOGY AND TREATMENT WITH NOCTURNAL BLADDER EMPTYING

PURPOSE We determine the etiology and treat the specific pathophysiology of the valve bladder syndrome. MATERIALS AND METHODS Defined as persisting or progressive severe hydroureteronephrosis without residual or recurrent obstruction, the valve bladder syndrome developed in 18 boys who underwent successful ablation of the posterior urethral valve. Serial radiographic, renal function, renographic, urodynamic and perfusion studies were performed for a mean time of 11 years. RESULTS The cause of the valve bladder syndrome proved to be sustained bladder over distention due to a combination of polyuria with 24-hour urine volume greater than 2 l. in 10 boys, impaired bladder sensation in 18 and residual urine volume in 14. Treatment of over distention during the daytime alone was unsuccessful. Nocturnal bladder emptying was performed with an indwelling nighttime catheter, intermittent nocturnal catheterization and/or frequent nocturnal double voiding. Hydronephrosis markedly improved once nocturnal bladder emptying was started and was comparable to the results after urinary diversion. CONCLUSIONS The valve bladder syndrome is not due to a permanent prenatal alteration in bladder anatomy and function. Instead, it appears to result from sustained postnatal bladder over distention due to a combination of polyuria, impaired bladder sensation and residual urine volume, which represent sequelae of prenatal valve injury. These factors synergize to prevent bladder normalization after valve ablation and progressively reduce functional bladder capacity to maintain bladder over distention. Bladder decompensation, upper tract dilation, and renal injury develop and characterize the valve bladder syndrome. Because current therapy, including intermittent catheterization, leaves the bladder full throughout the night, it remains markedly over distended. Nocturnal bladder emptying is the specific antidote for this pathophysiological situation, and results in prompt and impressive improvement or elimination of hydronephrosis in these and similar groups of patients. This response to nocturnal bladder emptying suggests that the bladder is not the primary cause for the valve bladder syndrome.

Bladder Augmentation: Ureterocystoplasty Versus Ileocystoplasty

The primary advantages of augmentation ureterocystoplasty include the absence of mucus, lack of electrolyte absorption from the augmenting segment and the avoidance of gastrointestinal complications. We tested whether the ureteral patch offers sufficient biomaterial to increase adequately the storage efficiency of dysfunctional bladders. Between April 1989 and November 1992, 8 children with unilaterally dilated and tortuous ureters underwent bladder augmentation using detubularized reconfigured megaureter. Clinical and urodynamic outcomes were compared between these patients and a control group of 8 children matched in age and diagnosis who had undergone ileocystoplasty during the same time. Total bladder capacity, pressure specific bladder volume at pressure less than 30 cm. water, dynamic analysis of bladder compliance, continence and upper tract status were compared between the 2 groups before and after augmentation. Preoperatively, all 16 patients were incontinent with high pressure, small capacity bladders, and all had upper tract changes. Postoperatively, the mean total bladder capacity was 417 ml. in the ureterocystoplasty group and 381 ml. in the ileocystoplasty group (p > 0.05), while the mean pressure specific bladder volume was 413 and 380 ml. (p > 0.05), respectively. Pressure specific bladder volume and dynamic bladder compliance were normal in 7 of 8 patients (87.5%) in the ureterocystoplasty group. All patients in the ileocystoplasty group had normal postoperative urodynamics. We conclude that megaureters subtending effete kidneys may be used to improve the storage function of dysfunctional bladders to the same extent as that achieved with ileum without the complications pursuant to ileocystoplasty, and that the improvement is maintained long term.

LONG-TERM OUTCOME OF TRANSURETHRAL PUNCTURE OF ECTOPIC URETEROCELES: INITIAL SUCCESS AND LATE PROBLEMS

PURPOSE We studied the long-term outcome of transurethral puncture of ectopic ureteroceles specifically associated with duplex systems. MATERIALS AND METHODS We retrospectively reviewed the records of patients who underwent transurethral puncture of an ectopic ureterocele. Study exclusion criteria were orthotopic, bilateral and prolapsing ureteroceles. RESULTS We identified 19 girls and 2 boys, of whom 11 presented with prenatal hydronephrosis and 10 presented with urinary tract infection. Mean age at puncture was 5 months (range 0.5 to 60). Preoperatively voiding cystourethrography revealed no reflux in 7 patients, isolated ipsilateral lower pole reflux in 8, and bilateral and/or contralateral reflux in 6. Postoperatively studies initially showed no reflux in 8 cases but in 4 of the 8 reflux recurred up to 4 years after puncture. In 10 patients (48%) reflux developed into the ureterocele and upper pole segment. Repeat puncture was required 1 to 13 months after the initial procedure in 4 patients for persistent or recurrent upper pole hydroureteronephrosis. Subsequent open surgery was required in 15 of the 21 cases (71%), including ureterocele excision with ureteral reimplantation in 14. Of the children 10 and 4 underwent open surgery for recurrent urinary tract infection and progressive reflux, respectively, while 1 underwent ureteroureterostomy for progressive upper pole reflux. No patient underwent upper pole nephrectomy. Of the remaining 6 patients 4 have low grade reflux. CONCLUSIONS Transurethral puncture of ectopic ureteroceles provides effective short-term correction of upper pole obstruction but it is not definitive therapy in the majority of cases. Most children still require open surgery. In patients without reflux after the puncture procedure new onset, recurrent or progressive reflux may later develop with extended followup. Repeat puncture may be required to ensure adequate decompression in a minority of cases, as in the 20% in our series.

PREOPERATIVE TREATMENT WITH HUMAN CHORIONIC GONADOTROPIN IN INFANCY DECREASES THE SEVERITY OF PROXIMAL HYPOSPADIAS AND CHORDEE

PURPOSE We determined whether human chorionic gonadotropin (HCG) pretreatment of severe proximal penoscrotal hypospadias and chordee causes sufficient penile shaft or skin enlargement to enhance surgical repair and improve patient outcome. MATERIALS AND METHODS A total of 12 boys 6 to 12 months old with proximal hypospadias and severe chordee received a course of HCG for 5 weeks immediately preceding hypospadias repair. RESULTS Chordee decreased and penile length increased in all cases (mean increase 94%). Penile length gain was disproportional. Most of the increase in length was proximal to the urethral meatus, which moved the meatus distally an average of 11.4 mm. (range 6.0 to 19.0), producing a mean increase of 586% in the distance between the penoscrotal junction and meatus. In contrast, there was no statistically significant increase in penile shaft length distal to the urethral meatus. Surgical treatment was facilitated by HCG pretreatment. Three meatal based repairs were performed, only 1 urethral fistula developed and chordee was corrected by penile degloving only in 8 cases. CONCLUSIONS HCG pretreatment in infancy produces disproportional penile enlargement, which advances the meatus distally to decrease the severity of hypospadias and chordee. This response pattern simplifies the required surgical procedure and appears to improve surgical results. It may benefit select patients, and provide insights into the endocrinopathy of hypospadias and the embryopathy of the hypospadias-chordee complex.

CONCOMITANT BLADDER NECK CLOSURE AND MITROFANOFF DIVERSION FOR THE MANAGEMENT OF INTRACTABLE URINARY INCONTINENCE

In a 7-year period 28 patients 1 to 20 years old have undergone bladder neck closure in conjunction with Mitrofanoff diversion for the management of severe incontinence. Surgery was performed as a salvage procedure in 19 patients and as a primary anti-incontinence procedure in 9. At a mean followup of 29 months 27 of 28 patients (96%) were totally continent, requiring no pads. Bladder neck closure was primarily successful in 24 of 28 patients (86%) and 25 (89%) had stable upper tracts. Five patients had bladder calculi and 5 required stomal revisions. One child had a bladder perforation associated with blunt trauma. Bladder neck closure and Mitrofanoff diversion were done without bladder augmentation in 11 cases and augmentation was performed previously or concurrently in the remainder. Four patients who did not initially undergo augmentation required later augmentation (2 for hydronephrosis and 2 for persistent incontinence). We conclude that bladder neck closure in conjunction with Mitrofanoff diversion is highly efficacious in achieving continence in a highly complex subgroup of patients with intractable urinary leakage. With careful patient selection and diligent followup total continence can be achieved in this most difficult patient population.