Cadaveric renal transplants in children under 6 years of age.

Thirty-nine cadaveric renal allografts were performed in 28 children under 6 years of age. Common primary renal diseases were glomerulonephritis, dysplasia/hypoplasia, and reflux/obstructive nephropathy. After a mean follow-up of 40 months of patients with surviving grafts, 19 patients had functioning grafts, 3 had returned to dialysis, and 6 had died. These children required an extraordinary amount of care postoperatively because of anorexia, diarrhea, and ileus. Their psychomotor and physical development was retarded prior to transplant; this reversed dramatically after transplant, but catch-up growth occurred in only 4 patients. Many patients were noticeably more active and distractible for 1 to 2 years post-transplant. Major causes of graft failure were primary nonfunction of 5 donor kidneys (4 from donors under 1 year old) and renal vessel thrombosis in 5 recipients (3 with native kidneys in place who received kidneys from donors over 10 years old). Other causes were recurrence of hemolytic uremic syndrome and Wilms tumor, rejection, and sepsis. Kidneys from donors under 1 year old proved unsatisfactory, and large donor kidneys in small children tended to thrombose, especially when native kidneys with high urine output were left in situ.

Surgical management of traumatic posterior urethral strictures in children.

We review our recent experience with the treatment of traumatic strictures of the posterior urethra in children. Five males, ages six to seventeen years with dense posterior urethral strictures, have required open reconstructive procedures. Four patients had injury secondary to pelvic fractures, and 1 patient had an iatrogenic injury from surgery for imperforate anus. Two patients were repaired perineally, 2 with a combination retropubic-perineal approach, and 1 patient required a transpubic approach. Excision and direct anastomosis was achieved in 3 patients, and a foreskin interposition tube graft was used in 2 patients. Excellent results were achieved with return of urethral voiding and preservation of continence in all patients. Complications were seen in 3 patients. One secondary internal urethrotomy was required. Erectile capability was preserved in all patients who were potent before surgery. Posterior urethral strictures in children can be successfully managed with a variety of surgical approaches. This experience demonstrates that the surgical procedure must be individualized depending on the anatomy of the injury.

Gender assignment in male pseudohermaphrodite children.

Experience with male pseudohermaphrodites at The Hospital for Sick Children, Toronto, from 1976 to 1981 was reviewed to identify factors that had influenced gender assignment. Seventeen children with microphallus, ambiguous genitalia, and 46 XY karoytype were studied. Five had been raised as females and 12 as males. Of those raised as females, 3 were from families with prior experience of androgen insensitivity. The other 2 had gross perineal abnormalities in addition to genital ambiguity. Of those raised as males 6 were not considered for gender reassignment, 4 because they were too old at presentation, and the other 2 because of parental refusal. In this series the parents were not prepared to accept gender reassignment on the basis of a small phallus alone. Androgen stimulation so far has been a reliable indicator of phallic growth potential, but long-term follow-up is necessary to determine its true value.

Acute appendicitis mimicking pelvic neoplasm.

Acute appendicitis can be difficult to diagnose when it presents in an atypical manner. We report a case of perforated appendicitis mimicking a pelvic neoplasm.

Neoplastic potential in patients with disorders of sexual differentiation.

The occurrence of tumors was determined in 157 patients with disorders of sexual differentiation at a major pediatric hospital from 1960 to 1980. Gonadoblastoma developed in 2 of the 4 patients with mixed gonadal dysgenesis (MGD) who had not had bilateral gonadectomy; this indicates the need for early gonadectomy in such cases. Gonadal embryonal carcinoma developed in 1 child with Turner's syndrome (1 of 71). Four cases of nephroblastoma occurred, 1 in a child with MGD (1 of 18) and 3 in male pseudohermaphrodites (3 of 35). The nature of the defect linking male pseudohermaphroditism with Wilms tumor remains to be established.

External genital abnormalities associated with Wilms tumor.

The records of 170 patients with unilateral and 18 patients with bilateral Wilms tumor and 6 patients with congenital mesoblastic nephroma were reviewed for abnormalities of the external genitalia. There were 4 patients with cryptorchism, 1 with hypospadias, 1 with mixed gonadal dysgenesis, and 3 with male pseudohermaphroditism. Of the group, these 9 patients had earlier symptoms; 6 of them were under two years old. Five patients (27.7%) with bilateral Wilms tumors had external genital anomalies in contrast to only 4 (2.3%) of those with unilateral tumors. One of the 9 children with genital anomalies had unilateral congenital mesoblastic nephroma; the others had Wilms tumor. In all cases the histologic subtypes were unusual. These observations substantiate the previously reported association between external genital abnormalities and Wilms tumor and also represent a somewhat higher than expected incidence. The children with this association are remarkable for the high frequency of a particular histologic type of tumor and for the high incidence of bilaterality. The high incidence of Wilms tumors in the male pseudohermaphrodite population presented suggests that such patients should be monitored for development of renal tumors.

Blunt renal trauma in the pediatric population: indications for radiographic evaluation.

OBJECTIVES: The purpose of this study was to define more clearly the clinical indications for radiographic evaluation of blunt renal injury in the pediatric population. METHODS: Children evaluated for blunt abdominal trauma at the Children's Hospital of Los Angeles and Los Angeles County/University of Southern California Medical Center undergo routine physical examination, laboratory analysis, and computed tomography (CT) scan of the abdomen and pelvis regardless of urinalysis results. We retrospectively evaluated the abdominal and pelvic CT scans of 412 children sustaining blunt abdominal trauma between June 1985 and June 1990. A total of 48 children, ages 6 months to 14 years (mean 5.6 years), with CT-documented renal injuries secondary to blunt trauma were identified. The radiographic findings were correlated with clinical presentation in this group of patients. RESULTS: Of the 48 children sustaining renal injuries (12% of the group), 23 (48%) had renal contusions and 25 children (52%) sustained more serious (significant) renal injuries. Of the children with significant renal injuries, 17 (68%) had minor renal lacerations and 8 (32%) had major renal lacerations. No child sustained a renal pedicle injury. All 25 children sustaining significant renal injuries presented with hematuria: 17 (68%) had microscopic (more than 3 red blood cells per high-power field) and 8 (32%) had gross hematuria. In the 23 children with renal contusions, 4 (17%) had no hematuria, 13 (57%) had microscopic hematuria, and 6 (26%) presented with gross hematuria. Hypotension occurred in 2 of the 25 children with significant renal injuries and in 2 of 23 children with renal contusions. Fifteen of the 25 patients (60%) with significant renal injuries had associated organ injuries, and 17 of the 23 children (74%) with renal contusions had associated organ injuries. CONCLUSIONS: In adults, gross hematuria and microscopic hematuria with hypertension following blunt trauma have been correlated with significant renal injuries requiring radiographic investigation. We conclude that these clinical criteria proposed to guide the radiographic evaluation of the adult population with blunt trauma do not apply to children. In our study, the degree of hematuria did not correlate with the degree of renal injury, and significant renal injury did occur with microhematuria in the absence of hypotension. We suggest that any child with a history of blunt abdominal trauma and any evidence of hematuria should undergo abdominal and pelvic CT scanning for the proper diagnosis and staging of renal and other associated intra-abdominal injuries.

Growth in boys and posterior urethral valves. Primary valve resection vs upper tract diversion.

Seventy-four patients with posterior urethral valves were evaluated for long-term growth potential and renal function. In those boy diagnosed and treated within the first year of life, initial treatment by temporary supravesical diversion (prior to valve resection) was associated with better growth potential and renal function than was treatment by primary valve resection alone.

Complications of posterior urethral valve surgery and their prevention.

The treatment of patients with posterior urethral valves has improved dramatically in recent times. Our personal experience with 207 patients with posterior urethral valves between 1957 and 1978 reveals a striking reduction in both mortality and morbidity during the latter decade. We have attempted to identify clearly the incidence, etiologic factors, preventive measures, and surgical treatment of complications arising in these patients. The overall improvement in management is certainly attributable to a growing experience and the constant advancements in the medical and surgical armamentarium. Our next goal at this time is to maximize long-term renal function and growth potential in these patients.

Surgery for anomalies of the urachus.

Thirty-five children with anomalies of the urachus which have required surgical management have been encountered in this institution over a 20-year period. Of these 35, 19 were classified as patent urachus, 12 as urachal cyst, and 4 as urachal sinus. All cases were treated by excision or drainage. There was one death. Gastrointestinal and other genitourinary anomalies were commonly seen in these patients. Intravenous pyelography was helpful in identifying unsuspected associated genitourinary anomalies while cystography was not. Investigation of the gastrointestinal tract does not appear to be warranted in the absence of symptoms.

Renal transplantation in patients with bilateral Wilm's tumor.

Review of cases and of published reports of patients with bilateral Wilms' tumor revealed a significantly higher mortality rate in those who had received a renal transplant than in those who had not. The increased mortality is attributed to overwhelming sepsis resulting from chemotherapy, radiotherapy and immunosuppression. Growth of the tumor did not appear to be accelerated by immunosuppression and transplantation.

Endoscopic treatment of pediatric urolithiasis.

Children have been slow to benefit from the improvements in stone therapy. Despite the success of less invasive techniques, open surgery for pediatric urolithiasis is still commonly practiced. The authors' goal was to evaluate the indications, efficacy, and complications of endourologic surgery in the treatment of pediatric urolithiasis. They reviewed the treatment of 53 children who had upper tract calculi. Open nephro- and ureterolithotomy were avoided in all cases. The management of 25 children whose stones were not amenable to extracorporeal shock wave lithotriopsy is presented. Seventeen children (19 stones) were treated with miniature ureteroscopes and lasertripsy. Only one (6%) of the patients required ureteral dilation, and only five (29%) needed a postoperative internal stent. Eight children with large renal calculi were treated percutaneously, without transfusion, complication, or evidence of persistent renal damage. All 25 patients were rendered stone-free. The hospital stay was shorter and secondary procedures were less common in comparison to other series of endoscopic lithotripsy. With significant experience in adult endourology and proper instrumentation, one can apply the same techniques to children and achieve equal if not greater benefit. Nephrolithiasis in children is uncommon and should be managed in an institution where endourologists and multiple treatment modalities are available.

Surgical treatment of testicular trauma: effects on fertility and testicular histology.

Unilateral testicular trauma in the postpubertal male can lead to alterations in semen analysis, but it is not clear what effect this has on fertility. To better understand how surgical treatment of testicular trauma affects both fertility and testicular histology the following study was performed. Eighty postpubertal Lewis rats were divided into eight equal groups with one group serving as a control. In the 70 remaining rats the left testicle was subjected to blunt or penetrating injury. The testicles were either left untreated, were removed, or were repaired with sutures or mesh before being returned to the scrotum. Following recovery, each male was allowed to mate to determine fertility. Fertility rates were significantly lower in all postinjury groups except the postinjury orchiectomy group. Histological analyses showed nonspecific inflammation, smaller tubules, and impaired spermatogenesis in all postinjury testicles regardless of the type of treatment. Contralateral testicles had no evidence of autoimmune injury and were essentially identical to the control group. In the postpubertal Lewis rat, unilateral testicular trauma leads to impaired fertility unless the injured testicle is removed soon after the injury. Various methods of repairing the injury did not improve fertility. In spite of the impaired fertility, the contralateral testicle appears histologically normal.

Neurogenic bladder in infants born with anorectal malformations: comparison with spinal and urologic status.

BACKGROUND/PURPOSE: Spinal dysraphism and neurovesical dysfunction (NVD) frequently are associated in children with anorectal malformations (ARM). This study compares the urodynamic data from a selected group of patients with the results of their spinal and urologic imaging studies. METHODS: Twenty-six children (20 with isolated imperforate anus and six with persistent cloacal malformations) were investigated. All patients were evaluated with leak point pressures (LPP), renal ultrasound scan, and voiding cystourethrography. Eight children had urodynamics performed before and after posterior sagittal anorectoplasty (PSARP). The spinal cord was assessed using ultrasonography or magnetic resonance imaging. Current urologic status was obtained to provide long-term follow-up. RESULTS: Twenty-one of 26 children demonstrated elevated LPPs above the established normal value of 40 cm H2O, and 15 of these children had normal spinal imaging study findings. Uroradiographic studies findings showed that 12 of 21 children with elevated LPPs had hydronephrosis or vesicoureteral reflux with seven of these patients having normal spinal cords. LPPs in the eight patients with pre- and postoperative studies were 74 +/- 14.7 cm H2O and 68 +/- 31.8 cm H2O (mean +/- SD), respectively. CONCLUSIONS: These urodynamic and radiographic data confirm that NVD (elevated LPP) is common in patients with anorectal malformations despite normal spinal cords. Bladder dysfunction does not appear to be a sequelae of a properly performed PSARP. Patients with ARM and any uroradiographic or clinical urologic abnormality should undergo urodynamic testing even though the spinal studies are normal.

Infant pyeloplasty is a low-risk procedure.

Early pyeloplasty for the treatment of congenital ureteropelvic junction obstruction to maximize nephron salvage is only justified if the potential hazards of operating on small infants are avoided. The records of all infants who underwent pyeloplasty by the authors over a 7-year period were analyzed. The outcome of surgery in the younger infant group (YIG; patients less than 2 months of age) was compared with that of the older infant group (OIG; patients more than 2 months of age). Preoperative evaluation in cases of mild or moderate hydronephrosis was directed toward ruling out a nonobstructed collecting system and included voiding cysto-urethrography and serial ultrasonography and/or dual isotope diuretic renography. Open pyeloplasty was performed if collecting systems had deteriorated or were demonstrated to be obstructed; it was also performed for severe cases of hydronephrosis. Postoperative assessment consisted of serial ultrasonography and/or nuclear imaging to confirm decompression and relief of obstruction. Thirty three pyeloplasties were performed in 31 patients in the YIG (two bilateral), and 33 were performed in 32 infants in the OIG (one bilateral). The only significant differences between the groups were as follows. Patients in the YIG were more likely to present in utero (87% v 53%; P < .01), whereas those in the OIG were more likely to present with a urinary tract infection (44% v 0%; P < .01). The YIG was more likely to have nephrostomy drainage postoperatively (67% v 45%; P < .01). In the overall series, there were five complications, all of which were postoperative infections requiring intravenous antibiotic therapy. Two occurred in the YIG and three in the OIG.(ABSTRACT TRUNCATED AT 250 WORDS)

Use of tubularized peritoneal free grafts as urethral substitutes in the rabbit.

As an alternative to bladder mucosa, free grafts of tubularized peritoneum were used as urethral substitutes in a rabbit model of hypospadias. In group 1, six mature rabbits underwent partial penile urethrectomy followed by interposition of a 2-cm-long peritoneal-lined tube graft. These animals had urethrograms performed at 3 months and were killed at 6 (n = 4) or 12 (n = 2) months. In group 2, six rabbits underwent total penile urethrectomy with placement of 3-cm-long grafts. These animals were killed 1 to 4 weeks after surgery. Clinical assessment and gross examination of the 12 rabbits showed no urinary retention, two small fistulas at the proximal anastomosis, and no strictures or diverticular. At 6 and 12 months (group 1) the urethra had healed completely and the graft edges were not visible. In group 2, 1 to 4 weeks after surgery the graft was intact and the interface between the graft and native urethra was visible. Histological studies of the grafts were compared with control peritoneum. At 1 week, a high-density single-cell layer was present. Beginning at 2 weeks, a multilayered epithelium was present, which became more organized in the older grafts. Neovascularity became visible in the subepithelial layer at 2 weeks. Acute inflammatory cells were present early and were replaced by a palisading layer of lymphocytes and plasma cells in the older grafts. Minimal fibrosis was observed. Tubularized peritoneal free grafts are a promising new urethral substitute. Graft placement is technically simple. Replacement of the peritoneum by a multilayered epithelium resembling transitional epithelium occurs early after graft placement. As much as 1 year later, minimal scarring is present.

Venous ischemia as a cause of ureteral necrosis in transplanted ureters.

BACKGROUND: Urologic complications after pediatric renal transplantation can adversely effect the outcome and may result in decreased graft survival. Efforts to prevent these complications are worthwhile. This study investigates the incidence of these complications in a clinical transplant program and reports on an animal model used to investigate one possible cause. METHODS: In the clinical study, the results of a pediatric renal transplant program at a large children's hospital for a 5(1/2)-year period were reviewed with special attention paid to patients suffering ureteral necrosis. In the experimental study, 9 swine underwent laparotomy, bilateral complete infrahilar ureteric dissection, and extravesical ureteroneocystostomy. On the left side only, the renal and adrenal veins were ligated. The arterial supply remained intact. The right side did not undergo vessel ligation and served as the control. Three pigs each were killed at 3, 8, and 15 days. Kidneys, ureters and a cuff of bladder were examined histologically. RESULTS: In the clinical study 75 renal transplants were performed with a total of 5 cases of early ureteral necrosis. Two of these 5 displayed venous congestion and ischemia, and 2 were associated with kidneys displaying primary nonfunction of the graft. Seventy-one of 75 grafts are continuing to function. One of the 4 early graft losses also had an ischemic ureter. In the experimental study all right kidneys and ureters were normal. All left kidneys had complete hemorrhagic necrosis. Necrosis also was found in 5 of 9 proximal left ureters and in 7 of 9 distal left ureters. Viable left ureters displayed moderate to severe submucosal and periureteric hemorrhage. Four of 9 ureters displayed more damage distally than proximally. The extent of necrosis was similar at 3, 8, and 15 days. CONCLUSION: In both clinical and experimental studies, venous congestion and subsequent ischemia have been shown to be important causes of ureteral necrosis after renal transplantation.