Edwin A. Smith

Increased incidence of hypospadias in small‐for‐gestational age infants in a neonatal intensive‐care unit

Objective To identify the incidence of hypospadias in children born prematurely and small‐for‐gestational age (SGA), and to compare this subgroup with infants of similar age and weight without hypospadias.

ACUTE URINARY RETENTION IN CHILDREN

PURPOSE Acute urinary retention is relatively infrequent in children. There are a variety of causes that are poorly defined in the literature, and they differ greatly from those seen most frequently in adults. We review our experience with pediatric patients presenting with urinary retention to 2 major metropolitan childrens hospitals. MATERIALS AND METHODS Records from Egleston and Scottish Rite Hospitals for Children were reviewed for a 6-year period for the diagnosis of urinary retention. Urinary retention was defined as inability to empty the bladder volitionally for greater than 12 hours with a volume of urine greater than expected for age ([age in years + 2] x 30 cc) or a palpably distended bladder. All cases resulting postoperatively and believed secondary to surgical dissection, narcotic use or immobility, and children previously diagnosed with chronic neurological disorders and voiding dysfunction or with reduced mental status were excluded from study. The majority of these encounters were emergency department visits specifically for acute urinary retention. RESULTS We identified 53 children meeting these criteria, including 37 boys 6 months to 17 years old and 16 girls 1 to 17 years old. Etiologies included neurological processes in 17%, severe voiding dysfunction in 15%, urinary tract infection in 13%, constipation in 13%, adverse drug effect in 13%, local inflammatory causes in 7%, locally invading neoplasms in 6%, benign obstructing lesions in 6%, idiopathic in 6%, combined urinary tract infection and constipation in 2%, and incarcerated inguinal hernia in 2% of cases. Adverse drug effects and dysfunctional voiding were implicated 3 times as often in males than females (16% versus 6% and 19% versus 6%, respectively). Urinary tract infections were 6 times more common in females (31% versus 5%). Constipation and local inflammatory processes were twice as common in females than males (19% versus 11% and 12% versus 5%, respectively). Local neoplasms, benign obstruction and idiopathic causes were found exclusively in males. CONCLUSIONS Urinary retention in children is a relatively rare entity but there is a significant incidence of neurological abnormalities in this population. If a clear reason for this condition cannot be delineated based on history, physical examination and laboratory assessment, these patients should undergo prompt neurological evaluation and appropriate imaging studies.

Blunt traumatic hematuria in children. Is a simplified algorithm justified

PURPOSE We determined whether radiographic evaluation is indicated in all children with traumatic hematuria. MATERIALS AND METHODS We retrospectively reviewed the records of 110 children from 1992 to 1999 diagnosed with blunt trauma and hematuria. It is routine practice at our emergency department to perform radiographic evaluation in all children with hematuria regardless of the degree. Each chart was evaluated for the mechanism of injury, degree of hematuria, hypotension, imaging studies, renal injury, renal anomalies, associated injuries and outcome. RESULTS A total of 110 patients 1 to 18 years old (mean age 9) were identified. The most common mechanism of injury was motor vehicle accident in 37 children (34%), followed by a fall in 32 (29%). Grades I to V renal injury was present in 5, 6, 6, 6 and 1 cases, respectively (22%), while 1 (0.9%) involved ureteropelvic junction avulsion. No child had renal pedicle injury. In 9 patients renal anomalies were detected incidentally. Of the 110 patients 101 underwent radiographic evaluation, including computerized tomography in 97 (88%). The 24 patients (22%) with significant renal injury and all with incidentally diagnosed renal anomalies had 50 or greater red blood cells per high power field on urinalysis, while 1 with ureteropelvic junction avulsion presented without hematuria. Hypotension was present in only 3 patients (2.7%), who also had associated injuries, including 2 who presented with renal injury. All 3 with associated injuries. Associated injuries were identified in 11 of 25 patients (44%). The 9 patients (8%) who did not undergo radiographic imaging had negative results on repeat urinalysis with an excellent outcome. CONCLUSIONS We recommend that radiological evaluation consisting of abdominal and pelvic computerized tomography should be performed only in patients with 50 or greater red blood cells on urinalysis, hypotension at presentation to the emergency room or based on the severity of mechanism of injury, for example high speed motor vehicle accident deceleration injuries. The patient who presented with ureteropelvic junction avulsion without hematuria would have undergone imaging considering the mechanism of injury and number of associated injuries.

METABOLIC RISK FACTORS FOR STONE FORMATION IN PATIENTS WITH CYSTIC FIBROSIS

PURPOSE Cystic fibrosis is characterized by chronic pulmonary disease, insufficient pancreatic and digestive function, and abnormal sweat concentrations. Patients with cystic fibrosis also have an increased incidence of nephrolithiasis. We compared the results of metabolic evaluation in patients with cystic fibrosis with and without nephrolithiasis. MATERIALS AND METHODS A total of 496 patients were evaluated at our center, including 98 with a mean age of 25 years who had cystic fibrosis and complete metabolic evaluation available between 1996 and 2000. Of these patients 13 (13%) had a history of nephrolithiasis. The records were reviewed for clinical characteristics and all patients underwent metabolic evaluation, including serum electrolyte measurement and 24-hour urine collection. Statistical analysis was done to compare the stone versus nonstone groups. RESULTS The incidence of nephrolithiasis in our study was 3%. We identified 13 patients 16 to 41 years old (mean age 27) with nephrolithiasis, of whom 62% had had multiple episodes. Flank pain was the presenting symptom in 9 of the 13 cases (69%). Renal ultrasound and computerized tomography were the most common imaging modalities. In 9 cases stones were passed without intervention, extracorporeal shock wave lithotripsy was required in 2 and ureteroscopy with stone extraction was done in 2. Calcium oxalate was the dominant stone composition in the 9 patients in whom stone analysis was performed. Metabolic evaluation of the stone versus no stone groups showed elevated urinary oxalate (45.5 versus 42.5 mg./24 hours), relative calcium oxalate supersaturation (5.3 versus 7.2) and decreased urinary citrate in the 2 groups. There was a statistical difference in citrate excretion with lower levels in stone formers (102 versus 218 mg./24 hours, p = 0.0007). CONCLUSIONS Patients with cystic fibrosis have an increased incidence of nephrolithiasis and are at high risk for recurrence. Metabolic evaluation is indicated in all patients with cystic fibrosis and urolithiasis since most have hyperoxaluria, urinary calcium oxalate supersaturation and decreased urinary citrate. Correcting detected stone risk factors in these cases may decrease stone recurrence.

Pyelonephritis, renal scarring, and reflux nephropathy: a pediatric urologist's perspective

Imaging of children with a clinical diagnosis of pyelonephritis is performed to characterize the extent of the infection, to identify associated renal injury and to uncover risk factors for future infections and renal damage. Although there is general agreement regarding the need for parenchymal imaging and the need to exclude processes that are either functionally or anatomically obstructive, there is controversy regarding the need for routine cystography, especially when parenchymal involvement has not been documented. A protocol that limits the use of cystography for evaluation of urinary tract infections must assume that the diagnosis of reflux is at least of variable clinical significance. It is now clear that vesicoureteral reflux and reflux nephropathy represent a diverse population that includes both congenital and acquired processes. MR imaging will improve our understanding of vesicoureteral reflux, pyelonephritis and renal scarring and might help us to identify and manage those patients most at risk for recurrent infections and renal injury. To recognize the potential contributions of this newer imaging technique it is helpful to look at our understanding of the pathophysiology of pyelonephritis, reflux and reflux nephropathy.

Clinical Manifestations and Management of Prune-belly Syndrome in a Large Contemporary Pediatric Population

OBJECTIVE To review the clinical manifestations and operative management of a large contemporary pediatric cohort of patients with prune-belly syndrome (PBS). METHODS PBS patients aged <21 years followed up in our pediatric urology clinic were identified by the International Classification of Diseases, Ninth Revision code (756.71). Demographics, concomitant diagnoses, surgical history, imaging studies, and renal or bladder function were evaluated. RESULTS Data were available for 46 pediatric patients (44 boys and 2 girls). Mean age was 7.6 ± 4.7 years (range, 0.9-20 years). Average length of clinical follow-up was 6.8 ± 5 years. Forty-five children (97.8%) had hydroureteronephrosis, and 36 of them (78.3%) had vesicoureteral reflux. Five patients (10.9%) had significant pulmonary insufficiency, and 2 patients (4.3%) were oxygen dependent. Eighteen children (39.1%) had other congenital malformations, including cardiac in 4 patients (8.7%) and musculoskeletal anomalies in 10 patients (21.7%). Orchidopexy was the most common surgery, with all boys aged ≥3 years having undergone the procedure. Twenty-two patients (47.8%) had a history of ureteral surgery, 22 (47.8%) had bladder surgery, 11 (23.9%) had renal surgery, and 6 (13%) had urethral procedures. Nineteen patients (41.3%) underwent abdominoplasty. Eighteen children (39.1%) had documented chronic kidney disease, and 8 children (17.4%) underwent renal transplantation. Average age at transplantation was 5.1 ± 2.9 years. The mean nadir creatinine level for patients with end-stage renal disease was 1.4 mg/dL compared with 0.4 mg/dL for those not requiring transplantation (P <.001). CONCLUSION Children with PBS have significant comorbidities and require frequent operative intervention, with disease heterogeneity necessitating an individualized management approach. Early end-stage renal disease is prevalent, with approximately 15% of children requiring kidney transplantation.

Renal cell carcinoma in a 2-year-old child with tuberous sclerosis.

Tuberous sclerosis is an autosomal dominant neurocutaneous disease that affects 1 in 6,000 individuals, with up to two-thirds of the cases being sporadic.1 The tuberous sclerosis complex frequently manifests as renal lesions, the most common being angiomyolipoma, which is found in up to 80% of the cases.2 Patients are also at increased risk for renal cell carcinoma, with an onset 20 to 30 years earlier than in the general population and bilateral disease in up to 43% of the cases.3 To our knowledge we report on the youngest individual to have renal cell carcinoma in the setting of the tuberous sclerosis complex.

The GMS hypospadias score: Assessment of inter-observer reliability and correlation with post-operative complications

OBJECTIVE An agreed upon method for describing the severity of hypospadias has not been established. Herein we assess the inter-observer reliability of the GMS hypospadias score and correlate it with the risk of a post-operative complication. METHODS A 3-component method for grading the severity of hypospadias was developed (GMS). Eighty-five consecutive patients presenting for hypospadias repair were graded independently by at least 2 surgeons using the GMS criteria. Scores were compared statistically to determine agreement between the observers. The outcomes of these patients were then reviewed to determine how the GMS score correlates to the risk of a surgical complication. RESULTS The G, M, and S scores had excellent agreement between observers. The GMS total score was exactly the same or differed by one point in 79/85 (93%) of patients. The complication rate was 5.6% for patients with a GMS score of 6 or less, but was 25.0% for patients with a GMS score greater than 6. CONCLUSIONS The GMS score provides a concise method for describing the severity of hypospadias and appears to have high inter-observer reliability. The GMS score also appears to correlate with the risk of a surgical complication.

Further analysis of the Glans-Urethral Meatus-Shaft (GMS) hypospadias score: Correlation with postoperative complications

INTRODUCTION AND OBJECTIVE The Glans-Urethral Meatus-Shaft (GMS) score is a concise and reproducible way to describe hypospadias severity. We classified boys undergoing primary hypospadias repair to determine the correlation between GMS score and postoperative complications. STUDY DESIGN Between February 2011 and August 2013, patients undergoing primary hypospadias repair were prospectively scored using the GMS classification. GMS scoring included a 1-4 scale for each component: G - glans size/urethral plate quality, M - meatal location, and S - degree of shaft curvature, with more unfavorable characteristics assigned higher scores [Figure]. Demographics, repair type, and complications (urethrocutaneous fistula, meatal stenosis, glans dehiscence, phimosis, recurrent chordee and stricture) were assessed. Total and individual component scores were tested in uni- and multivariate analysis. RESULTS Two-hundred and sixty-two boys (mean age 12.3 ± 13.7 months) undergoing primary hypospadias repair had a GMS score assigned. Mean GMS score was 7 ± 2.5 (G 2.1 ± 0.9, M 2.4 ± 1, S 2.4 ± 1). Mean clinical follow-up was 17.7 ± 9.3 months. Thirty-seven children (14.1%) had 45 complications. A significant relationship between the total GMS score and presence of any complication (p < 0.001) was observed; for every unit increase in GMS score the odds of any postoperative complication increased 1.44 times (95% CI, 1.24-1.68). Urethrocutaneuous fistula was the most common complication, occurring in 21 of 239 (8.8%) of single-stage repairs. Patients with mild hypospadias (GMS 3-6) had a 2.4% fistula rate vs. 11.1% for moderate (GMS 7-9) and 22.6% for severe (GMS 10-12) hypospadias (p < 0.001). Degree of chordee was an independent predictor of fistula on multivariate analysis; S4 (>60° ventral curvature) patients were 27 times more likely to develop a fistula than S1 (no curvature) boys (95% CI, 3.2-229). DISCUSSION The GMS score is based on anatomic features (i.e. glans size/urethral plate quality, location of meatus, and degree of chordee) felt to most likely impact functional and cosmetic outcomes following hypospadias repair. We demonstrated a statistically significant increase in the likelihood of any postoperative complication with every unit increase in total GMS score. The concept that factors aside from meatal location affect hypospadias repair and outcomes is not novel, and degree of ventral curvature and urethral plate quality are often cited as important factors. In our series, boys with greater than 60° of ventral curvature undergoing a single-stage repair were 27 times more likely to develop a fistula than those without chordee on multivariate analysis, making severe curvature an independent predictor of urethrocutaneous fistula formation. That meatal location did not retain significance on multivariate analysis highlights the importance of considering the entire hypospadias complex when determining severity, rather than just evaluating the position of the meatus. Our study has several limitations that warrant consideration. While GMS scores were assigned prospectively, the data was collected retrospectively, subjecting it to flaws inherent with such study design. Furthermore, type of repair is influenced by surgeon preference and subjective assessment of hypospadias characteristics not incorporated in our scoring system (i.e. tissue quality, urethral hypoplasia, penoscrotal transposition). Despite these limitations, our study demonstrates a strong correlation between the GMS classification and surgical complications, furthering supporting its potential as a tool to standardize hypospadias severity and gauge postoperative complications. CONCLUSION The Glans-Urethral Meatus-Shaft (GMS) classification provides a means by which hypospadias severity and reporting can be standardized, which may improve inter-study comparison of reconstructive outcomes. There is a strong correlation between complication risk and total GMS score. Degree of chordee (S score) is independently predictive of fistula rate.

Use of Rectus Abdominis Muscle Flap as Adjunct to Bladder Neck Closure in Patients With Neurogenic Incontinence: Preliminary Experience

PURPOSE Vesicoureteral fistula is a well-known potential complication following bladder neck closure for neurogenic incontinence. Various maneuvers, including omental interposition, have been described to prevent this problem. Unfortunately omentum is not always available or feasible for use. We describe the surgical anatomy and use of a rectus abdominis muscle flap as an adjunctive maneuver during bladder neck closure to correct or prevent development of bladder neck fistula. MATERIALS AND METHODS We performed a retrospective chart review of all patients at our institution undergoing rectus abdominis muscle flap by a single surgeon (EAS). Patient demographics, indications for surgery, intraoperative and postoperative complications, and long-term efficacy were assessed. Cadaveric dissection was also performed to gain a greater understanding of the surgical anatomy relevant to this procedure. RESULTS In 6 patients with neurogenic bladder dysfunction a rectus abdominis muscle flap was interposed between the bladder neck and urethral stump at bladder neck closure. There were no intraoperative or postoperative complications associated with this procedure. At a mean followup of 45.5 months (range 18 to 120) all 6 patients were continent of urine. There have been no urinary fistulas related to use of the rectus abdominis muscle flap. Cadaveric dissections confirmed the inferior epigastric artery to be the dominant and readily mobile blood supply of the rectus abdominis muscle flap. CONCLUSIONS The rectus abdominis muscle flap is easily harvested without significant risk of morbidity and offers a well vascularized tissue for coverage of a bladder neck closure when an omental flap is not available.