Biomedical subjects
Publications and source records attributed to S A Kramer.
In ten young males with the epispadias-exstrophy complex, a new technique of bilateral rhomboid flaps was used for penile elongation and genital reconstruction. This approach offers less chance of injury to the verumontanum and ejaculatory ducts and accurately defines the penopubic angle. Cosmetic and functional results were satisfactory in all patients.
Many cases of Wilms' tumor developing in a horseshoe kidney have been previously reported. We present a case of Wilms' tumor developing in a "lump" kidney, associated with sacral agenesis. We discuss the implications for staging and make recommendations for follow-up in these cancer-prone children.
Testicular torsion is the most common cause of acute scrotal pain in prepubertal and adolescent boys and should be foremost in the minds of primary care physicians evaluating these children. Intermittent testicular torsion is a separate entity that should be considered in all young males with a history of scrotal pain and swelling. Acute and intermittent sharp testicular pain and scrotal swelling, interspersed with long intervals without symptoms, are characteristic. Physical findings may include horizontal or very mobile testes, an anteriorly located epididymis, or bulkiness of the spermatic cord from partial twisting. Awareness of this entity and early elective orchiopexy will improve testicular salvage in patients with intermittent testicular torsion.
The importance of several pathogenetic factors in the development of reflux nephropathy was evaluated in 25 piglets with complete unilateral vesicoureteral reflux and urinary tract infection. Three independent risk factors were studied: roentgenographic intrarenal reflux, P-fimbriation of the bacterial strain, absence of previous immunization. E. coli with P-fimbriae produce mannose-resistant agglutination of pig red blood cells and are more adherent to pig uroepithelial cells than E. coli without P-fimbriae. Vesicoureteral reflux was surgically induced at 2 weeks, urinary tract infection introduced at 6 weeks and the animals killed at 12 weeks of age. Independently, the 3 risk factors had a borderline or insignificant effect on renal scarring. Animals with none or only 1 risk factor, however, had significantly less scarring, fewer glomerular lesions and lower serum creatinines than those with 2 or 3 factors present. Several independent pathogenetic factors seem to have a synergistic effect on the development of reflux nephropathy.
Reoperative ureteroneocystostomy in 69 patients with primary vesicoureteral reflux was reviewed. Of the renal units 49 were obstructed and 48 had reflux. Postoperative obstruction was caused by mechanical factors in 61 per cent of the renal units, distal ureteral scarring in 31 per cent and a previously unrecognized functionally neurogenic bladder (without a neurological lesion) in 8 per cent. Persistent postoperative reflux was secondary to a short submucosal tunnel in 94 per cent of the renal units and occult neurogenic bladder in 6 per cent. Reoperative ureteroneocystostomy was successful in 79 per cent of the renal units, with a mean followup of 33 months. The modified Paquin technique (omitting the ureteral cuff) yielded consistently superior results in children undergoing reoperation for ureterovesical junction obstruction. In patients with postoperative vesicoureteral reflux a variety of techniques produced similar and gratifying results. That 52 per cent of our patients had no symptoms indicates clearly the absolute necessity of careful followup after ureteral reimplantation. Furthermore, 20 per cent of our patients had late failure (4 to 10 years after initial ureteroneocystostomy), which suggests the need for careful monitoring of the reimplanted ureter past puberty.
Effective chemotherapy combined with surgery has produced excellent disease-free survival rates in most children with genitourinary malignancy. For patients with Wilms' tumor, the overall 2-year survival rate with no evidence of disease is greater than 90 per cent, and the interval of chemotherapy continues to be reduced for those children with low-stage lesions and favorable histologic patterns. In children with pelvic rhabdomyosarcoma, primary cytoreductive chemotherapy has proved to be an effective alternative to exenterative surgery. Patients with yolk-sac tumor who have localized disease need not be subjected to the potential morbidity of retroperitoneal lymph-adenectomy or the toxicity of adjuvant chemotherapy. Conversely, patients with advanced disease can be salvaged with recently developed combination chemotherapeutic regimens.
The records of 14 children with yolk sac carcinoma were reviewed to determine if the production of specific proteins by the tumor could be used to predict the biologic potential of the neoplasm. Several marker proteins were identified by immunohistochemical staining of tissue specimens. The presence or absence of these proteins (periodic acid, Schiff-positive and diastase-resistant globules, alpha-1-antitrypsin, alpha-fetoprotein, beta-subunit of human chorionic gonadotropin and albumin) was of no prognostic significance when survivals free of disease were compared. Six patients underwent retroperitoneal node dissection as part of initial staging (group A), 1 (17 per cent) of whom had retroperitoneal lymphatic metastasis. Eight patients were not subjected to lymphadenectomy initially (group B). Retroperitoneal node dissection did not produce an increase in survival free of disease. Our findings suggest that retroperitoneal node dissection should not be used routinely in all children with yolk sac tumors. Adjunctive chemotherapy has proved extremely effective in salvaging patients in whom metastatic disease develops.
This study was designed to test the hypothesis that young animals with vesicoureteral reflux might be more vulnerable to renal parenchymal infection by bacteria to which they had not been previously exposed. Forty-four crossbred male piglets had surgical induction of vesicoureteral reflux at 2 weeks of age and introduction of urinary tract infection at 6 weeks. They were sacrificed at 12 weeks of age. Between the ages of 2 and 6 weeks, 22 piglets received subcutaneous injections of formalin-killed Escherichia coli in incomplete Freund's adjuvant as described. The remaining 22 piglets received incomplete Freund's adjuvant and vehicle alone. The antibody responses to antigenic challenge were weak to moderate. Immunized animals tended to have less renal scarring and better renal tubular uptake of dimercaptosuccinic acid, in addition to significantly lower serum creatinine values (p less than 0.001) and less mesangial cell proliferation in glomeruli (p = 0.05). We conclude that previous exposure to a specific bacterial strain and bacterial immunization have at least a mild protective effect on the development of reflux nephropathy.
Pediatric urologic evaluation by endoscopy is one of the most precise techniques in medicine. Recently developed endoscopic instrument permit an accurate and detailed examination of the child's urethra and bladder. Anomalies of the urethra and bladder in children may be divided into congenital, iatrogenic, infectious or inflammatory, and neoplastic groups. This review discusses the applicability of endoscopy for the diagnosis and treatment of urologic abnormalities in children.
Twelve Mayo Clinic patients less than 17 years of age were identified who had yolk sac tumors of the testis. Seven additional pediatric patients were identified who had embryonal carcinoma of the testis. These 19 patients form the basis for this study correlating histopathology and immunocytochemical patterns with clinical outcome. Immunoperoxidase studies were done with antibodies to alpha-fetoprotein, beta-subunit of human chorionic gonadotropin, alpha-1-antitrypsin, and human albumin. The immunocytochemical staining pattern in these neoplasms did not appear to correlate with prognosis.
Recent technological advances in prenatal ultrasonography have been responsible for the detection of a variety of congenital abnormalities in utero. The widespread use of this study during pregnancy has led to an increased recognition of foetal hydronephrosis. It has been postulated that unrelieved, high-grade urinary obstruction in utero may produce progressive renal damage. This supposition has resulted in the development of techniques for foetal intervention in attempts to correct obstructive uropathy and arrest renal dysplasia. Herein we review the techniques for assessment of foetal renal function and treatment options available for the foetus with hydronephrosis in utero. Most foetuses with hydronephrosis have dilated, low pressure systems with adequate urinary output and amniotic fluid volume and can be followed safely to term delivery. The accumulated experience to date indicates that the foetus with congenital hydronephrosis detected in utero should be managed expectantly with serial ultrasound examinations after delivery to establish an accurate diagnosis and to evaluate the status of the contralateral kidney. Functional tests after birth, such as renal scans and excretory urography, are mandatory to establish the proper diagnosis and assess the need for operative intervention.
Of 49 children with renal ectopy, 25 (51%) had hydronephrosis on excretory urography. Dilatation of the renal pelvis was the result of primary ureteropelvic junction obstruction (17 children), significant vesicoureteral reflux (6 children), or extrarenal pelves and calices with renal malrotation producing apparent ureteropelvic junction obstruction (2 children). Vesicoureteral reflux was a common finding in these patients, occurring in 52% of those with crossed renal ectopy and in 70% of those with pelvic kidney. The need for surgical intervention results from either (1) ureteropelvic junction obstruction secondary to high insertion of the ureter on the renal pelvis or (2) vesicoureteral reflux secondary to aberrant migration of the lower ureter. Most children with renal ectopy and ureteropelvic dilatation require surgical reconstruction at the renal level. In selected patients, ureterocalicostomy is an attractive alternative to conventional pyeloplasty and appears to afford improved drainage with superior results.
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Between 1946 and 1980, 82 male and 12 female patients with epispadias were evaluated at our institution. Factors critical to the achievement of complete continence include deferral of an operation until the patient is at least 3 years old, a well developed bladder with adequate capacity and musculature, and maturation of the prostate at puberty in boys. Complete continence was obtained in 10 of the 12 female patients (83 per cent). Of 53 incontinent male patients undergoing an operation 18 (34 per cent) obtained complete urinary continence in the initial postoperative period but control was delayed until puberty in 19 additional male patients, for an over-all success rate in 37 of 53 patients (70 per cent). The significant increase in continence with the onset of puberty demonstrates the importance of expectant management of progress in a boy in relation to the potential contribution of prostatic maturation in the development of urinary control.
We discuss 20 children who have undergone surgical correction of chordee without hypospadias. In 9 patients ventral curvature was demonstrated secondary to corporeal disproportion, 8 had chordee secondary to deficiency of the dartos fascia (skin chordee) and 3 had deficiency of the dartos and Buck's fasciae. Eight patients underwent excision of dorsal ellipses according to the Nesbit principle, 8 had lysis of the dartos fascia using the Allen-Spence technique, 2 underwent mobilization of the urethra with excision of underlying fibrous tissue and 2 had division of the urethra with a staged urethroplasty. The majority of cases of chordee without hypospadias are a result of anomalies of the tissue layers of the penis, and adequate straightening can be achieved by excision of underlying abnormal fascial tissue. All patients have achieved a satisfactory cosmetic and functional result with a straight penis and a urethral meatus positioned distally at the glans tip.
Simple renal cysts in childhood are uncommon and often are confused with duplication anomalies or renal malignancy. A review of 20 children with simple renal cysts at our institution has documented the spectrum of clinical presentations and radiographic findings of this unusual renal mass. Surgical exploration and marsupialization of the cyst wall were done in 12 patients, 4 underwent nephrectomy earlier in our series and 4 have been followed conservatively by ultrasonography or computerized tomography scanning. Although equivocal cases necessitate surgical intervention, we advocate conservative therapy with diagnostic ultrasonography with or without cyst puncture in asymptomatic and normotensive children with simple renal cysts.
Single-stage reconstruction of female epispadias produced gratifying functional and cosmetic results and complete urinary control in 10 out of 12 patients (83%). Factors critical to the achievement of complete continence in these children include deferment of surgery until the patient is at least 3 years of age and a well-developed bladder with adequate capacity and musculature. Because complete continence is often delayed several weeks to months postoperatively in these children, expectant management and observation are necessary to allow enlargement of bladder capacity, which enhances successful results.