Hrair-George J. Mesrobian

The malone antegrade colonic enema enhances the quality of life in children undergoing urological incontinence procedures

PURPOSE Functional alterations of the gastrointestinal and genitourinary tracts, and physical limitations in children with spina bifida, imperforate anus and spinal cord injury challenge the ability to have independent fecal and urinary continence. Urologists have successfully helped these patients achieve urinary continence. We report our experience with the antegrade colonic enema procedure, which allows select individuals to achieve continence of stool, enhancing quality of life. MATERIALS AND METHODS Since December 1992, 18 antegrade colonic enema procedures were performed in 12 female and 6 male patients 5 to 31 years old of whom 14 had spina bifida, 2 had imperforate anus and 2 had spinal cord injury. Simultaneous urological continence procedures were performed in 8 patients, including appendicovesicostomy in 4, augmentation cystoplasty in 2 and augmentation cystoplasty plus an ileal Mitrofanoff procedure in 2. Four patients previously underwent urological reconstruction. RESULTS In 24 months of followup (average 6.6) all patients with a functioning stoma remained continent of stool and 17 were continent of urine. Complications related to the antegrade colonic enema procedure occurred in 4 children (22%) of whom 3 required further surgery. Three patients (17%) had minor stomal stenosis. CONCLUSIONS The antegrade colonic enema procedure is easily performed and it should be considered for any child with significant physical limitations and/or refractory fecal incontinence before urological continence promoting procedures are done.

Unilateral renal agenesis may result from in utero regression of multicystic renal dysplasia.

Renal agenesis is generally thought to result from a lack of induction of the metanephric blastema by the ureteral bud, which may be secondary to ureteral bud maldevelopment and/or to a problem with the formation of the mesonephric duct. Multicystic dysplasia is thought to result from early ureteral obstruction as evidenced by the high frequency of associated proximal ureteral atresia. The recent obstetrical practice of large scale screening of large numbers of fetuses with sonography has resulted in a significant increase in the detection of this and other genitourinary anomalies. These findings have contributed to our understanding of the natural history of many of these malformations, resulting in ongoing reassessment and refinement of current management modalities. Thus, it is now well established that a significant number of multicystic dysplastic kidneys, initially detected in utero and followed postnatally, involute and disappear with time. We report the unequivocal presence of unilateral multicystic dysplasia detected during maternal sonography in 3 fetuses, which could not be seen on subsequent studies during the fetal or immediate postnatal period. To our knowledge, these cases constitute the first report of complete in utero regression of multicystic kidneys. This novel observation indicates that some cases of unilateral renal agenesis result from in utero regression of multicystic dysplastic kidneys. This phenomenon may explain the presence of an ipsilateral blind ending ureter found in some patients with unilateral renal agenesis. However, this infrequent observation does not explain all cases of renal agenesis. Thus, the etiology of renal agenesis remains multifactorial.

Long-Term Followup of 103 Patients with Bladder Exstrophy

We reviewed 103 patients with exstrophy of the bladder. Followup was more than 15 years in 51 patients. Initial management consisted of primary bladder closure in 32 patients and urinary diversion in 71. Urinary continence, renal function, urinary tract infections and development of malignant lesions were evaluated. Factors leading to success or failure were analyzed. Although the highest continence rate (83 per cent) was achieved in 40 patients with ureterosigmoidostomy, renal functional deterioration was highest in this group, with 70 per cent of the evaluable renal units being abnormal. Furthermore, 10 per cent of this group died of renal failure and 23 per cent lost 1 kidney each. In 26 patients with an ileal conduit 69 per cent of the renal units evaluated were abnormal. Only 1 patient died of renal failure but 27 per cent lost 1 kidney each. Of 32 patients with primary bladder closure 31 had preservation of renal function. Twelve of 18 patients (67 per cent) in whom vesical neck reconstruction had been completed had total urinary continence and 3 (17 per cent) had partial continence. The incidence of significant urinary tract infections was highest in the ureterosigmoidostomy group (63 per cent) and next to the highest in the ileal conduit group (48 per cent). Malignant lesions developed in 8 patients (8 per cent).

Management of Children with Myelodysplasia: Urological Alternatives

Recent reports have suggested that the prophylactic use of clean intermittent catheterization in selected infants with myelodysplasia and with normal upper tracts may prevent their deterioration. The selection of these patients at risk depends on urodynamic criteria. We reviewed our experience with clean intermittent catheterization instituted when upper tract deterioration was found. Of 130 patients with myelodysplasia followed at our clinic 25 (19%) had or presented with radiological upper urinary tract deterioration. A total of 21 patients had vesicoureteral reflux and 4 had hydronephrosis without reflux. Treatment consisted of clean intermittent catheterization alone in 5 patients and combined with anticholinergic medication in 16. Four patients with no post-void residual urine were carefully observed without intervention. Urodynamic evaluation was performed selectively in 11 of these 25 patients for persistence of upper urinary tract deterioration or urinary incontinence after institution of treatment. Of the 21 children with reflux 19 (90%) had resolution or improvement for an average followup of 47 months. Hydronephrosis completely resolved in the 4 patients without reflux. Thus, the over-all resolution or improvement rate was 92% for an average of 41 months. Although these excellent results may not be superior to the prophylactic use of clean intermittent catheterization, they validate the assumption that upper tract deterioration can be reversed. The application of current urodynamic selection criteria may result in overtreating a significant number of infants who otherwise may not have upper tract deterioration. Further refinements in these criteria may be necessary to select better not only patients at risk but also to determine the frequency of imaging necessary in this urologically heterogeneous population. In addition, careful consideration should be given to urethral dilation as a third and equally viable alternative to prevent upper tract deterioration.

Wilms Tumor in Horseshoe Kidneys: A Report from the National Wilms Tumor Study

An analysis of information on 2,961 patients with Wilms tumor entered in the National Wilms Tumor Study up to July 1983 revealed 13 tumors occurring in horseshoe kidneys. The correct preoperative diagnosis was made in 6 of the 13 patients. At presentation 5 patients had stage 1, 3 stage 2 and 5 stage 3 disease. Major preoperative or intraoperative tumor spillage occurred in 3 patients. The over-all survival rate was 85 per cent after a mean followup of 45 months. The surgical, pathological and therapeutic aspects of Wilms tumor in horseshoe kidneys were analyzed carefully. The frequency of horseshoe kidneys in patients with Wilms tumor registered with the National Wilms Tumor Study was determined and was used in conjunction with population data to estimate the relative risk of Wilms tumor occurrence in children with horseshoe kidneys.

Long-Term Followup of Cosmetic Appearance and Genital Function in Boys with Exstrophy: Review of 53 Patients

Long-term results of genital function and cosmetic appearance were analyzed in 53 patients with bladder exstrophy. Satisfactory cosmetic appearance of the external genitalia, with a straight penis angulated downward in the standing position, was achieved in 55 per cent of the patients. Normal erectile function was preserved in all patients and 61 per cent of the postpubertal patients had experienced satisfactory intercourse. The recent use of formal penile elongation by partial mobilization of the crura of the corpora cavernosa from the pubic rami combined with single-stage or multistage urethroplasty has produced a significant improvement in results. Of the patients 12 are married and 5 have fathered children.

Pediatric Urology: The Impact of Prenatal Sonography on the Morbidity and Outcome of Patients With Renal Duplication Anomalies

Historically, most patients with a renal duplication anomaly associated with upper pole hydronephrosis underwent upper pole nephrectomy and partial ureterectomy. Prenatal sonography has resulted in increased recognition of these anomalies and, therefore, earlier urological referral and evaluation. We investigated whether as a result more upper pole moieties were preserved by performing alternative procedures, such as ureteroureterostomy. In addition, the impact on overall morbidity of the disease and its treatment is assessed. Since 1984, of 29 patients treated for ectopic ureter or ureterocele 13 presented initially with a diagnosis of prenatal hydronephrosis. Of these patients, 5 (38.5%) underwent a renal sparing procedure consisting of ureteroureterostomy in 4 and excision of ureterocele with common sheath ureteral reimplantation in 1. The remaining 8 patients underwent upper pole nephrectomy and partial ureterectomy for a nonfunctioning upper pole moiety. In contrast, of 16 patients who presented without having undergone prenatal sonography only 2 (12.5%) underwent a renal preserving procedure. Initial treatment in the remaining 14 patients was upper pole nephrectomy and partial ureterectomy. The average age at initial treatment in the prenatally detected group was 3 months, compared with 5 years in the other group. Only 1 of the 13 patients (8%) in the prenatally detected group had symptoms, whereas 14 of 16 in the other group (87.5%) presented with significant symptoms consisting of sepsis in 12, an abdominal mass in 1, urinary incontinence in 1, and an incidental diagnosis made during evaluation of an associated anomaly in 2. Although there is still controversy regarding the impact of prenatal detection of hydronephrosis on the preservation of renal function in patients with a single system, our results show a beneficial effect in patients with duplication anomalies. This was reflected in our ability to salvage the upper pole moieties 3 times more frequently than was previously possible with minimal morbidity. More importantly, the potential serious clinical manifestations of these duplication anomalies in later life were eliminated by virtue of early treatment.

NONOBSTRUCTIVE DILATATION OF UPPER URINARY TRACT MAY LATER CONVERT TO OBSTRUCTION

We report on 5 babies found to have pyelocaliectasis or hydroureteronephrosis antenatally who were evaluated soon after birth because of persistent mild or moderate upper urinary tract dilatation. In each instance, vesicoureteral reflux was absent and the technetium-99m diethylenetriaminepentaacetic acid renal scan revealed good function in the ipsilateral kidney. Renogram curves, after furosemide, did not show an obstructed pattern. Indeed, allowing for the dilatation, the drainage patterns were thought to be normal. In each instance the patient represented or was found on follow-up to have increased dilatation. Renograms were then repeated, using the same radiopharmaceutical and dose of diuretic. An obstructive pattern was seen in each instance. These significant observations demonstrate that nonobstructive hydronephrosis, diagnosed prenatally, may later convert to obstruction at the ureteropelvic junction (UPJ) or in the juxtavesical ureter (UVJ). Thus, careful follow-up, for a period yet to be determined, is desirable in patients in whom the initial postnatal evaluation does not demonstrate obstruction by current criteria.

Long-term Followup of Cosmetic Appearance and Genital Function in Male Epispadias: Review of 70 Patients

In male patients who undergo surgery for epispadias the major postoperative complaint often concerns the cosmetic appearance of the penis and the failure to correct the dorsal chordee rather than the urinary incontinence. In the past straightening and lengthening of the penis were not given adequate consideration, and penile elongation was limited to release of dorsal skin chordee only. Recently, however, additional penile length has been achieved by partial mobilization of the crura of the corpora cavernosa from the pubic rami. In a series of 70 patients penile lengthening and urethroplasty were performed separately or were combined as a 1-stage procedure with full thickness grafts or preputial flaps. The recent use of hair-bearing groin flaps has provided excellent skin coverage for the large dorsal defect at the penoabdominal angle. A satisfactory cosmetic appearance of the external genitalia, with a straight penis angulated downward in the standing position, was achieved in 67 per cent of the patients. Normal erectile function was preserved in all patients, 80 per cent of whom have had a straight penis with erection and satisfactory intercourse. Of the patients 29 are married and 19 have fathered children. Meticulous attention to the technical aspects of reconstructive surgery usually can result in a gratifying cosmetic appearance, normal genital function and preservation of fertility potential in most patients.

Pyeloureterostomy with Appendiceal Interposition

We report a case of renal salvage after total right ureteral loss using the isolated vermiform appendix with preservation of the blood supply. The appendix was used to bridge the gap between the right renal pelvis and left ureter. Followup revealed satisfactory function of the appendiceal conduit with preservation of renal function. The appendix, used in this fashion, is an attractive alternative to other surgical modalities after total or near total ureteral loss. The excellent contractility combined with the relatively small size eliminates the problems associated with urinary stasis and electrolyte reabsorption. However, its use is limited by its availability and length.