Prenatal diagnosis of cloacal malformation.

Cloacal malformation is a rare abnormality, occurring only in females and characterized by a direct communication between the gastrointestinal, urinary, and genital structures resulting in a single perineal opening. We report 2 cases of prenatal diagnosis of this condition with a varied ultrasonic appearance including fetal ascites, cystic retrovesical mass, ambiguous genitalia, nonvisualization of the bladder, and oligohydramnios.

Urachal anomalies: defining the best diagnostic modality.

OBJECTIVES: Urachal abnormalities are uncommon and the literature is primarily comprised of case reports. Conclusions regarding the presentation and diagnosis of these abnormalities may be elucidated by reviewing a large experience. METHODS: The records of 45 patients with urachal abnormalities in the pediatric age group were reviewed from 1970 to 1997. This included 24 boys and 21 girls with an age range from 1 day to 20 years (average 4.0 years). The presenting complaint was periumbilical discharge in 19 patients (42%), umbilical cyst or mass in 15 (33%), abdominal or periumbilical pain in 10 (22%), and dysuria in 1 (2%). The diagnosis consisted of a urachal sinus in 22 children (49%), a urachal cyst in 16 (36%), and a patent urachus in 7 (15%). Various radiographic studies were used to establish the diagnosis. RESULTS: Patients with a urachal sinus had 16 voiding cystourethrograms performed (only 1 diagnostic), 9 sinograms (all diagnostic), 8 ultrasounds (4 diagnostic), and 1 excretory urogram (normal). Those with a urachal cyst had 8 voiding cystourethrograms (1 diagnostic), 5 excretory urograms (all normal), 4 ultrasounds (all diagnostic), and 1 computed tomography scan (diagnostic). Children with a patent urachus had 2 excretory urograms (both diagnostic), 1 voiding cystourethrogram (diagnostic), and 2 ultrasounds (normal). One baby with a patent urachus was diagnosed prenatally during ultrasound screening. The diagnosis was made by history and physical examination alone in 5 children and at the time of surgery in 1. Treatment consisted of surgical excision of the urachal abnormality with a cuff of bladder in 22 children, surgical excision without a bladder cuff in 22, incision and drainage of a urachal cyst (1%), and laparoscopic excision of a patent urachus with a bladder cuff in another (1%). There were three wound infections postoperatively. None developed any long-term sequelae. CONCLUSIONS: The diagnosis of urachal abnormalities can be made with certainty if a good physical examination and the appropriate radiographic test are performed. A patient who presents with periumbilical drainage should have a sinogram performed, which should be diagnostic for both a urachal sinus and a patent urachus. Any child who presents with a periumbilical mass should have an ultrasound performed, which should be diagnostic for a urachal cyst.

Hematuria in children. A practical approach.

The investigation of hematuria in children should never be allowed to pose a greater risk to the child's health than does the problem itself. For this reason, angiography and cystoscopy are rarely employed in the evaluation. The evaluation of the child should proceed methodically and rest firmly on a well-taken history and results of physical examination. The microscopic examination of the urinary sediment is the cornerstone of the evaluation process and can itself suggest the origin of the hematuria. Red cell casts, if present, indicate glomerulonephritis and obviate further urologic evaluation. Genitourinary imaging should be tailored to each clinical situation.

Cryptorchidism and testicular torsion.

The cause of cryptorchidism remains obscure. Hormonal therapy is ineffective for most patients and initial response may be temporary. Surgery remains the most effective treatment for cryptorchidism. Testicular torsion is a surgical emergency in the postnatal infant and the pubertal male. Controversy surrounds the management of prenatal torsion.

Progressive ureteral dilation for subsequent ureterocystoplasty.

PURPOSE: The complications associated with bladder augmentation using the gastrointestinal tract are well known. A bladder based ureteral flap is a viable alternative for augmentation. However, its use is limited to cases in which a dilated ureter is already present due to associated pathological conditions, such as reflux. We designed a system for performing progressive dilation in a normal ureter, which can subsequently be used for augmentation. MATERIALS AND METHODS: Ten rabbits underwent unilateral ureteral ligation at the ureterovesical junction and ipsilateral nephrectomy. A silicone catheter was threaded into the proximal ipsilateral ureter and connected to an injection port, which was secured subcutaneously. Ten days to 2 weeks after surgery a saline-antibiotic solution was injected subcutaneously daily into the injection port. Cystography and cystometrography were performed with an accurate measurement of bladder capacity. Ureterography was done initially and then weekly by injecting contrast medium into the ureter. After ureteral dilation ureterocystoplasty was performed in each animal. RESULTS: After 1 month of daily saline-antibiotic solution injections the ureteral units were dilated at least 10-fold, as measured by radiography. The dilated ureteral diameter exceeded that of adjacent colon in each instance. Augmentation cystoplasty was performed with the reconfigured dilated ureteral segment. Repeat cystography and cystometrography showed an average 260% increase in bladder capacity (range 190 to 380%). CONCLUSIONS: Ureteral dilation can be accomplished using our system. At least a 10-fold increase in ureteral size is possible. The dilated ureteral segment can be used effectively for bladder augmentation, avoiding the use of bowel segments.

Pantaloon spica cast: an effective method for postoperative immobilization after free graft hypospadias repair.

PURPOSE: We used a postoperative dressing of silicone foam in conjunction with a pantaloon spica cast to optimize the chances for successful graft take in hypospadias repairs with grafts. We compared the results to those of inpatient bed restriction. MATERIALS AND METHODS: A total of 15 patients underwent graft urethroplasty, and application of a penile silicone foam dressing and fiberglass pantaloon spica cast. Patients were discharged home the following morning. RESULTS: Two fistulas developed. Results are no different from those of our previously reported cases which required 6 or 7 days of hospitalization. CONCLUSIONS: We believe that the pantaloon spica cast provides necessary immobilization for facilitating imbibition and inosculation, which are required for graft survival. Also, the spica cast allows early discharge home, obviating the need for prolonged hospitalization and bed rest.

Prenatal and postnatal findings in monochorionic, monoamniotic twins discordant for bilateral renal agenesis-dysgenesis (perinatal lethal renal disease).

We report on a male twin born with no functional renal tissue and without the extrarenal manifestations of Potter facies, skin changes, club feet and pulmonary hypoplasia. The monoamniotic co-twin had normal renal function, thereby maintaining sufficient amniotic fluid to avoid the classic presentation of Potter's syndrome. Prenatal diagnosis of this condition allowed prompt confirmation of these findings and support for the parents without unnecessary intervention. This experiment of nature demonstrates the necessity of normal renal function in the maintenance of amniotic fluid and its relationship to the proper development of the pulmonary and integumentary systems.

Lidocaine toxicity secondary to postoperative bladder instillation in a pediatric patient.

The intravesical use of local anesthetics has been described for a variety of urologic procedures with no previous reports of toxicity. We recently took care of a 2.5- year-old girl with systemic lidocaine toxicity following treatment for postoperative bladder spasms. The patient developed a generalized seizure requiring endotracheal intubation but recovered fully with supportive care. We report the clinical details of this case as well as a brief review of lidocaine toxicity.

Phenotypic and cytogenetic characterization of human bladder urothelia expanded in vitro.

A simple method for the harvest of bladder cell types from surgical specimens was used to generate strains of normal human urothelial cells that could be reproducibly cultivated, passaged and extensively expanded in serum-free medium. Immunostaining of the bladder epithelial cells with broadly reacting anti-cytokeratin antibodies and with an anti-cytokeratin antibody specific to cytokeratin 7, a transitional cell marker, indicated that they expressed a stable epithelial phenotype with serial passage. Low levels of immunostaining for E-cadherin and low levels of E-cadherin messenger ribonucleic acid, as determined by Northern blot analysis, and strongly positive immunostaining with an anti-vimentin antibody indicated collectively that the uroepithelial cells express a nonbarrier-forming phenotype under these culture conditions. However, when the urothelial cells were implanted subcutaneously into athymic mice on biodegradable synthetic polymers, they formed multilayered structures, suggesting that they retain the capability to differentiate in a living host. The urothelial cells proliferated in an epidermal growth factor independent manner and expressed high levels of transforming growth factor-alpha and amphiregulin messanger ribonucleic acids, suggesting the possibility of autocrine regulation of growth by epidermal growth factor-like factors. Cytogenetic analysis indicated that urothelial cells cultured for 6 passages possessed a normal chromosomal complement. These results demonstrate that primary cultures of autologous human bladder epithelial cells can be extensively expanded in vitro and, consequently, might be used in cell transplantation strategies for genitourinary reconstruction.

Refined microscopic urinalysis for red blood cell morphology in the evaluation of asymptomatic microscopic hematuria in a pediatric population.

PURPOSE: The use of refined microscopic urinalysis for the presence of dysmorphic red blood cells (RBCs) has been evaluated in children and adults with a known source of hematuria. We examined the clinical usefulness of this study in a pediatric population with an unknown source of hematuria. MATERIALS AND METHODS: Children 12 years old or younger referred for evaluation of asymptomatic microscopic hematuria exhibiting 4 or more RBCs per high power field were enrolled in this study. Patients provided a first morning urine sample subjected to refined urinalysis for RBC morphology. Standard evaluation of patients was performed until a final diagnosis of the hematuria source was identified. RESULTS: A total of 44 patients completed the study. Refined urinalysis revealed pure dysmorphic RBCs in 22 patients, pure isomorphic RBCs in 8 and mixed isomorphic/dysmorphic RBCs in 14. The presence of dysmorphic RBCs correctly predicted a glomerulotubular source of hematuria in 29 of 36 patients (sensitivity 83%, specificity 81%), while the presence of isomorphic RBCs predicted a uroepithelial source of hematuria in 2 of 8 patients (sensitivity 25%, specificity 22%). Hematuria and 2+ proteinuria (100 mg./dl.) were more sensitive (100%) and specific (83%) than the presence of dysmorphic RBCs in predicting glomerulotubular hematuria. CONCLUSIONS: We believe that this is a costly test offering little additional information to the evaluation of microscopic hematuria in children. A thoughtful history and physical examination with microscopic urinalysis and dipstick for proteinuria provide an equal amount of diagnostic information. We do not recommend its routine use in the evaluation of microscopic hematuria in children.

A noninvasive test for vesico-ureteric reflux in children.

OBJECTIVE: To report the development and testing of a device for the noninvasive diagnosis of vesico-ureteric reflux (VUR) which avoids the need for urethral catheterization (currently required to reliably determine the presence of VUR), and which thus avoids the anxiety of parents and patients that causes many families to refuse such evaluation. PATIENTS AND METHODS: Fifty-four children (49 girls and five boys, mean age 7.2 years, range 4-14) previously evaluated as having VUR volunteered to participate; no child was symptomatic at the time of the study. Refluxing units were known to be present by voiding cysto-urethrography (within 1 year, mean 7 months) in 45 and absent in 16. The device developed acquires electronically processed acoustic signals from the child during an observed urination. The signals are then analysed 'off-line' to determine the presence or absence of VUR. The initial preparation for the test included: (i) a full bladder [at least 0.80 x ((2 + age) x 30 mL)] measured by ultrasonography; and (ii) localization of the pelvi-ureteric junction by ultrasonography to accurately place the device's sensors on the child's back. The children were then positioned at a commode after placing the sensors; the recording was started and continued until voiding occurred. The children were tested with the recording and analysis team unaware of the presence and/or degree of VUR. The first 47 studies were single-kidney examinations and the remaining seven included simultaneous monitoring of both kidneys. RESULTS: Sixty-one renal units were assessed and interpretable signals were obtained from 54 (89%). There were seven episodes of 'system failure' when no interpretable data were obtained. One unit with no VUR had a 'reflux' signal; in four kidneys, spontaneous (two) and postsurgical (two) resolution of reflux was predicted by the testing and subsequently verified by cyclic radionuclide cystography. CONCLUSIONS: This noninvasive diagnostic technique detected VUR in 35 of 37 refluxing units and verified no reflux in 16 of 17 units without VUR. Further refinements may allow this technology to be used in all children with suspected VUR.

The ultrasonic description of postpubertal testicles in men who have undergone prepubertal orchiopexy for cryptorchidism.

PURPOSE: We provide a sonographic description of postpubertal testicles in patients who have undergone prepubertal orchiopexy with placement of a suture through the tunica albuginea. MATERIALS AND METHODS: Testicular ultrasound was performed in men who had undergone prepubertal testicular fixation for cryptorchidism using a suture passed through the tunica albuginea. Comparison was made to the sonographic appearance of cryptorchid testicles not secured with a fixating suture through the tunica albuginea. The operative report for each patient was reviewed with specific attention to use and type of suture material, and age at orchiopexy. RESULTS: Ultrasonography was performed on 22 men in whom a tunica albuginea fixating suture was placed during orchiopexy for cryptorchidism. Average patient age at orchiopexy was 5.6 years and median time from orchiopexy to this examination was 14.8 years. Right cryptorchidism existed in 64% of the men. No significant difference was noted in size between the 2 testicles in any patient. Ultrasonography did not identify any parenchymal abnormalities. A tunica albuginea calcification with posterior shadowing on the side of fixation was noted in 32% of patients, of whom 3 had calcified areas evident on physical examination. An area of subtunical hypoechogenicity was noted in 14% of patients. All tunical abnormalities were associated with the use of chromic suture. The remaining 12 patients (54%) had normal sonographic and physical examinations. The control group comprised 6 men who underwent orchiopexy without a fixating suture through the tunica albuginea. These men were examined identically and no sonographic abnormalities were noted in the tunica albuginea or testicular parenchyma. At a median of 16 months no patient had been diagnosed with testicular cancer or had a change in testicular self-examination. CONCLUSIONS: Tunica albuginea calcifications and hypoechogenic cysts are common ultrasonic findings in men who have undergone orchiopexy using a suture passed through the tunica albuginea. Parenchymal lesions should not be considered secondary to this procedure and must be treated as de novo abnormalities. These changes seem to be induced by the use of a fixating suture through the tunica albuginea.

Laparoscopically assisted ureterocystoplasty.

OBJECTIVE: To assess the efficacy and safety of laparoscopically assisted ureterocystoplasty (LAU) in children. PATIENTS AND METHODS: From 1999 to 2001, five patients (mean age 7 years, range 3.5-13) from four centres underwent LAU with laparoscopic mobilization of the small kidney and upper ureter combined with ureterocystoplasty, with exposure of the bladder through a Pfannenstiel incision. The details and outcomes are reviewed. RESULTS: The LAU was successful in all five patients; there were no complications. A large midline incision was avoided and the LAU carried out through the better tolerated and less painful Pfannenstiel incision. CONCLUSION: LAU is an appealing technique that is safe with the added benefit of a reduced abdominal incision and acceptable operative duration. This represents the first published report of LAU.

Tubularized incised plate urethroplasty: expanded use in primary and repeat surgery for hypospadias.

PURPOSE: We evaluated the impact of tubularized incised plate urethroplasty on primary and repeat hypospadias repair. MATERIALS AND METHODS: We retrospectively reviewed the medical records of all boys who underwent hypospadias repair at our institution during a recent 3-year period. The level of the hypospadias defect, technique of repair, primary repair versus reoperation, age at surgery and complications were recorded. RESULTS: A total of 520 hypospadias repairs were done from May 1996 through June 1999. We began to perform tubularized incised plate urethroplasty in November 1996. During the ensuing consecutive 32 months 181 primary and 25 repeat hypospadias repairs were done using this technique. Mean patient age at surgery was 22 months (range 3 months to 30 years). During the 6 months immediately before we began to use this method the Mathieu flip-flap procedure was the most commonly performed technique, accounting for 38% of all hypospadias repairs. In contrast, during the last 6 months reviewed tubularized incised plate urethroplasty accounted for 63% of all repairs, including 41 of 65 primary operations (63%) and 4 of 6 reoperations (67%), while no Mathieu procedures were performed. Postoperative followup was 6 to 38 months for tubularized incised plate repair. Overall meatal stenosis and a urethrocutaneous fistula developed in 1 and 14 boys, respectively (7% complication rate). CONCLUSIONS: Tubularized incised plate urethroplasty has become the preferred technique of primary and repeat hypospadias repair at our institution. The technique has few complications as well as proved success and versatility that continues to expand its applicability and popularity.

Natural history of vesicoureteral reflux in girls after age 5 years.

PURPOSE: Expectations concerning the likelihood that vesicoureteral reflux will resolve during a given interval are predominantly based on experience with children younger than 5 years. We assess the natural course of vesicoureteral reflux in girls older than 5 years. MATERIALS AND METHODS: We reviewed the diagnostic and followup cystograms, medical records and renal imaging studies of 200 girls with vesicoureteral reflux, of whom 97 were diagnosed before age 60 months and 103 were diagnosed at or after age 60 months. Vesicoureteral reflux was considered to have resolved when a followup radionuclide cystogram demonstrated no reflux. RESULTS: Vesicoureteral reflux demonstrated at or after age 60 months by a radionuclide or radiographic examination (index study) resolved in 43% of cases during a mean followup interval of 41 months. The yearly percent chance of resolution approached or exceeded 20% through age 11 years. For girls with moderate vesicoureteral reflux on the index study unilateral moderate vesicoureteral reflux was associated with a higher overall percent chance of resolution and a shorter time from index study to resolution. Evidence of new or progressive parenchymal injury was not indicated in any of 92 girls who underwent serial renal ultrasonograms. CONCLUSIONS: Vesicoureteral reflux resolution continues after age 5 years at a rate similar to that in younger children. Continued medical management in the anticipation of spontaneous resolution is safe and appropriate for most patients.

Neonatal scrotal haematoma: mimicker of neonatal testicular torsion.

OBJECTIVE: To describe the clinical features of neonatal scrotal haematoma and distinguish them from those of neonatal testicular torsion. PATIENTS AND METHODS: Five neonates presenting with an acute scrotum and initial diagnosis of neonatal testicular torsion were found to have neonatal scrotal haematoma. In one case the diagnosis was surgical and in four subsequent cases the diagnosis was by colour Doppler ultrasonography, and surgery was avoided. Four of the five children had risk factors associated with neonatal scrotal haematoma, including bleeding diathesis, birth trauma and high birth weight. CONCLUSIONS: The importance of including haematoma in the differential diagnosis of the acute neonatal scrotum is emphasized, as is the value of contemporary Doppler ultrasonography in making this diagnosis.