Robert E. Steckler

Ureteral Bladder Augmentation

Virtually all segments of the gastrointestinal tract have been used successfully in augmentation cystoplasty. The complications inherent in enterocystoplasty are well described. Megaureters subtending effete kidneys (poorly or nonfunctioning) provide a novel and excellent source of augmentation material with urothelium and muscular backing, free of the electrolyte and acid base disturbances, and mucus production that plague enterocystoplasty. Augmentation cystoplasty using detubularized, reconfigured, otherwise disposable megaureter, with or without ipsilateral total or partial nephrectomy, was performed in 16 patients (mean age 8.8 years, range 1 to 25) with inadequate and dysfunctional bladders. Postoperative followup varied between 8 and 38 months (mean 22). The overall renal function and radiographic appearance of the remaining upper tracts have remained stable or improved in all patients. Of the 16 patients 15 require intermittent catheterization and 1 voids spontaneously. Ten patients are continent day and night, 5 have improved continence (4 damp at night and 1 stress incontinence) and 1 has failed to gain continence despite good capacity and compliance. Complete postoperative urodynamic evaluations in 12 of 13 patients show good capacity, low pressure bladders with no instability. Complications occurred in 5 patients, including transient urine extravasation in 2, contralateral ureterovesical obstruction in 2 and Mitrofanoff stomal stenosis in 1. Augmentation ureterocystoplasty combines the benefits common to all enterocystoplasties without adding any of the untoward complications or risks associated with nonurothelial augmentations.

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.

Stent-Free Thiersch-Duplay Hypospadias Repair With the Snodgrass Modification

PURPOSE Incision of the glanular urethral plate (the Snodgrass modification) permits tubularization of the neourethra in the Thiersch-Duplay fashion when anatomy would otherwise preclude a tension-free anastomosis and necessitate another operative technique for hypospadias repair. To take advantage of these cosmetic benefits without the potential morbidity associated with indwelling catheters, we performed a stent-free Thiersch-Duplay repair incorporating the Snodgrass modification. MATERIALS AND METHODS The Thiersch-Duplay hypospadias repair was combined with the Snodgrass modification. Hinging the urethral plate was necessary when the glanular groove was too shallow to perform a standard Thiersch-Duplay repair. RESULTS Stent-free repairs were performed in 33 children 0.47 to 2.66 years old (mean age plus of minus standard deviation 0.98 +/- 0.47). Followup was obtained in 31 children. There was no postoperative urinary retention, fistulas or meatal stenosis. No unusual or prolonged discomfort distinguished these children from those who underwent a standard Thiersch-Duplay repair. CONCLUSIONS Excellent cosmetic results can be anticipated irrespective of the preoperative glans configuration. Incision of the glanular urethral plate can be performed safely as an adjunct to a modified Thiersch-Duplay hypospadias repair without postoperative indwelling catheters.

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.

Intrarenal Resistive Index Correlates with Renal Pelvis Pressure

Elevation in the intrarenal resistive index has been suggested by many to be a physiological parameter useful for detecting functionally significant hydronephrosis. It is currently unknown whether the intrarenal resistive index changes truly reflect the changes in collecting system pressure or whether they are simply a coincidental epiphenomenon. The purpose of this study is to establish the relationship between intrarenal resistive index and collecting system pressure. Between August 1992 and October 1993, 9 patients younger than 1 year underwent a percutaneous pressure-flow study as part of hydronephrosis evaluation. During the pressure-flow study intrarenal resistive index was measured serially with simultaneous renal pelvis pressure readings. In all patients the index increased as the renal pelvis pressure increased. Furthermore, using the experimentally derived proximal tubular pressure of 14 cm. water as the probable threshold for functionally normal collecting system pressure, it was found that all intrarenal indexes of 82% or less corresponded to renal pelvis pressures of less than 14 cm. water, while all of those greater than 82% corresponded to renal pelvis pressures greater than 14 cm. water. By combining several lines of evidence, it appears probable that as maximal diuresis induced by physiological and pharmacological means leads to acute transient elevation in renal pelvis pressure in a functionally obstructed collecting system, the intrarenal resistive index is capable of reflecting this dynamic elevation in renal pelvis pressure and potentially able to distinguish physiologically significant upper urinary tract obstruction from nonobstructive dilatation.

THE SENSITIVITY OF PRESSURE SPECIFIC BLADDER VOLUME VERSUS TOTAL BLADDER CAPACITY AS A MEASURE OF BLADDER STORAGE DYSFUNCTION

Assessment of bladder storage function requires an accurate measure of bladder capacity and pressure. Pressure specific bladder volume is the volume that a bladder can accommodate at a specific pressure. A total of 21 consecutive children with neurogenic bladders who were candidates for bladder augmentation based on standard clinical criteria (upper urinary tract deterioration, incontinence and infection) was studied to determine the efficacy of pressure specific bladder volume as a measure of bladder dysfunction. Urodynamic indexes were compared to previously established nomograms. All 21 patients had bladder volumes at pressures of 30 cm. water or less, which decreased below the 5th percentile as determined by the nomogram. In 7 patients (33%) normal total bladder capacity was achieved at the expense of elevated storage pressures. Pressure specific bladder volume provides a better measure of bladder storage function than total bladder capacity because it relates volume to intravesical pressure, does not rely on a subjective end point to bladder filling, and is objective and reproducible.

Cryptorchidism, pediatricians, and family practitioners: Patterns of practice and referral

A multicenter study was undertaken to study cryptorchidism and the timing of orchidopexy. A total of 329 children underwent surgery at a mean age of 4.2 years; 17% of the surgery was performed between 6 and 12 months of age, 25% between 5 and 10 years of age, and 9% during or after puberty. Only 30% of the pediatricians and 14% of the family practitioners recommended orchidopexy between 6 and 12 months of age, and 17% of these referring physicians recommended waiting until 3 to 10 years of age. Improved education is needed if current recommendations for early orchidopexy are to be achieved.

Contradictory Supranormal Differential Renal Function During Nuclear Renographic Investigation of Hydroureteronephrosis

We selected a group of children with unilateral hydroureteronephrosis to assess the significance of the differential renal function as determined by nuclear renography as a factor in defining obstructive dilatation. All children who presented to our institution during a 21-month period with unilateral hydroureteronephrosis and a normal contralateral kidney were reviewed. Patients with reflux or bladder pathology were excluded, resulting in 13 evaluable patients younger than age 2 years. Diuretic renography was performed using 99mtechnetium-diethylenetriaminepentaacetic acid in accordance with the well tempered renogram. Ancillary studies included voiding cystourethrography, ultrasonography and/or excretory urography. Of the 13 patients 5 had a differential function greater than 55% in the hydronephrotic kidney. Ten of the 13 patients, including the 5 with supranormal function, underwent surgical correction in the form of ureteral reimplantation for recurrent infections, increasing hydronephrosis, decreasing or decreased function and loss of parenchyma. Stenotic distal adynamic ureteral segments with proximal dilatation were confirmed in all cases. The implication by nuclear renography that a dilated kidney has better function than its morphologically normal counterpart is at variance with any available clinical or experimental data. The etiology of this apparently elevated function is unclear and its presence on a renographic study should not be interpreted as suggestive that the kidney is free of risk of nephron loss. Management decisions should not be based solely on the differential function as determined by nuclear renography.

A new technique for dynamic analysis of bladder compliance

We propose an alternative method of measuring compliance that takes into account the multiple phases of bladder filling. We describe our new technique, dynamic compliance analysis, and evaluate its clinical applicability. To perform the analysis we digitized a cystometrogram curve at a sampling rate of 2 samples per second using an MS-DOS computer system. A program designed to retrieve the stored data was used to analyze the subtracted bladder pressure. The result yielded a value of compliance every half second that was then plotted on an x-y graph, with instantaneous compliance as the dependent variable and per cent of total volume infused as the independent variable. To determine the clinical applicability of this technique we chose 63 curves from clinically normal patients. The results of the dynamic compliance analyses were predictable. The dynamic compliance values for the normal group had a minimum that was always greater than 10 ml./cm. water throughout the tonus limb (phase 2) of the cystometrogram. We conclude that dynamic compliance analysis yields more information about bladder response during filling, similar to the stress-strain curve used in the study of solid mechanics.

Xanthogranulomatous Pyelonephritis in Pediatric Patients

We observed xanthogranulomatous pyelonephritis in 5 of 27 nephrectomies (18.5%) performed for end stage pyelonephritis. Two patients were 5 months old or younger, of normal birth weight and the products of uncomplicated full-term pregnancies. In all cases clinical histories and findings were consistent with xanthogranulomatous pyelonephritis yet this diagnosis was suspected prospectively in only 1 case. Our observations, supported by recent literature, suggest that xanthogranulomatous pyelonephritis may occur more commonly than previously reported. In addition, the clinical and pathological expression of this process in the pediatric population appears to be no different from that in adults. The true incidence and presumed chronicity of this process need to be reevaluated in light of recent data.