Parent perceptions of psychosocial care for children with differences of sex development.

SHORT INTRODUCTION/BACKGROUND: Children affected by differences/disorders of sex development (DSDs) and their families are vulnerable to significant risks across developmental stages that threaten quality of life and psychosocial functioning. Accordingly, both experts in DSD treatment and patient advocacy groups have endorsed the incorporation of psychosocial care into interdisciplinary management of DSD conditions. OBJECTIVE: This study assessed psychosocial needs and received services reported by parents of children with DSD treated at two large US academic medical centers. Specifically, differences in parents' perceptions of psychosocial service needs were compared between those who received or did not receive interdisciplinary care that included psychology/social work professionals. STUDY DESIGN: In a cross-sectional study, sixty-four parents of children with DSD aged 0-19 years attending two major academic centers with interdisciplinary teams completed a questionnaire about their receipt and perception of 12 individual psychosocial services throughout their child's DSD treatment. RESULTS: Receipt of individual psychosocial services ranged from 27 to 81%. Most commonly, parents reported having a psychosocial provider explain medical terms and answer questions after talking with a doctor (81%), assist with words and terms to describe the condition and treatment (69%), and help navigate the hospital system (63%). Families positively endorsed psychosocial services, with 91-100% of services received rated as helpful. Parents of children who received care as part of an interdisciplinary team were significantly more likely to receive psychosocial services than those treated by single providers (e.g., urologists). Specific gaps in psychosocial care were noted in regard to access to mental health providers familiar with DSD, fertility counseling, and support with community advocacy (e.g., arranging for accommodations at the school or advocating on patient's behalf with the insurance company). Among families who had not received them, services most desired were assistance with words and terms to describe condition or treatment; explanation of medical terms and answering questions after meeting with a doctor; connection to resources such as books, pamphlets, websites, and support groups; and a central care coordinator for the medical team. DISCUSSION AND CONCLUSION: Families value psychosocial services but are far less likely to receive services if they are not seen in an interdisciplinary clinic visit that includes a psychosocial provider. Families desire but often lack mental health, advocacy, and fertility-related support. This study highlights the need for sustained psychosocial follow-up across development, even in the absence of pressing medical concerns, to provide support and anticipatory guidance as needs and issues evolve.

Combined spinal/caudal catheter anesthesia: extending the boundaries of regional anesthesia for complex pediatric urological surgery.

BACKGROUND: Spinal anesthesia (SA) is an established anesthetic technique for short outpatient pediatric urological cases. To avoid general anesthesia (GA) and expand regional anesthetics to longer and more complex pediatric surgeries, the authors began a program using a combined spinal/caudal catheter (SCC) technique. STUDY DESIGN: The authors retrospectively reviewed the charts of all patients scheduled for surgery under SCC between December 2016 and April 2018 and recorded age, gender, diagnosis, procedure, conversion to GA/airway intervention, operative time, neuraxial and intravenous medications administered, complications, and outcomes. The SCC technique typically involved an initial intrathecal injection of 0.5% isobaric bupivacaine followed by placement of a caudal epidural catheter. At the discretion of the anesthesiologist, patients received 0.5 mg per kilogram of oral midazolam approximately 30 min prior to entering the operating room. One hour after the intrathecal injection, 3% chloroprocaine was administered via the caudal catheter to prolong the duration of surgical block. Intra-operative management included either continuous infusion or bolus dosing of dexmedetomidine, as needed, for patient comfort and to optimize surgical conditions. Prior to removal of caudal catheter in the post-anesthesia care unit, a supplemental bolus dose of local anesthesia was given through the catheter to provide prolonged post-operative analgesia. RESULTS: Overall, 23 children underwent attempted SCC. SA was unsuccessful in three patients, and surgery was performed under GA. The remaining 20 children all had successful SCC placement. There were 11 girls and nine boys, with a mean age of 16.5 months (3.3-43.8). Surgeries performed under SCC included seven ureteral reimplantations, two ureterocele excisions/reimplantations, two megaureter repairs, four first-stage hypospadias repairs, one distal hypospadias repair, one second-stage hypospadias repair, two feminizing genitoplasties, and one open pyeloplasty. Average length of surgery was 109 min (range 63-172 min). Pre-operative midazolam was given in 13/20 (65%). All SCC patients were spontaneously breathing room air during the operation, and there were no airway interventions. Only one SCC patient received opioids intra-operatively. There were no intra-operative or perioperative complications. DISCUSSION: This pilot study shows that the technique of SCC allows one to do more complex urologic surgery under regional anesthesia than what would be possible under pure SA alone. The main limitations of the study include the relatively small number of patients and the small median length of the operative procedures. As a proof of concept, however, this does show that complex genital surgery bladder level procedures such as ureteral reimplantation can be performed under regional anesthesia. CONCLUSION: SCC allows for more complex surgeries to be performed exclusively under regional anesthesia, thus obviating the need for airway intervention, minimizing or eliminating the use of opioids, and thus avoiding known and potential risks associated with GA. The latter is of particular importance given current concerns regarding hypothetical neurocognitive effects of GA on children aged below 3 years.

Benefits of spinal anesthesia for urologic surgery in the youngest of patients.

INTRODUCTION: Increasing concerns regarding potential negative effects of early use of inhalational and intravenous anesthetics on neurocognitive development have led to a growing interest in alternative forms of anesthesia in infants. The study institution's outcomes with spinal anesthesia (SA) for urologic surgery in infants aged less than 90 days are reported and their outcomes with a matched cohort of patients who underwent general anesthesia (GA) are compared. METHODS: This is a retrospective single-center analysis. Patients aged less than 90 days who underwent SA for four urologic surgeries (inguinal hernia repair, scrotal exploration, posterior urethral valve ablation, and ureterocele puncture) were identified from the study institution's SA database. An age- and procedure-matched control cohort was identified from a list of patients who underwent the aforementioned four procedures under GA since 2013. Outcomes of interest included success rate of SA, complications from spinal placement, narcotic use, need for supplemental medications and oxygen, and length of hospital stay. RESULTS: Forty patients were identified; 20 in the SA and 20 in the GA group. Mean patient age was 54 (standard deviation, 35) days. There were no significant differences between the groups in age, gender, weight, history of prematurity, or presence of comorbidities. Eighty percent of SA patients had successful SA; reasons for conversion to GA included failure of spinal needle placement (75%) and agitation during operative procedure (25%). Ninety-six percent of patients who received GA (primarily or converted) had an endotracheal tube (ETT) placed. No patient in the SA group had a complication from spinal needle placement. Patients in the SA group were less likely to receive narcotics during the operative procedure (P = 0.001) and also had a lower mean morphine equivalent dose/kilogram (P = 0.002). Patients in the SA group were also less likely to receive any supplemental medications during the operative procedure (P = 0.001), particularly intravenous corticosteroids (P < 0.001). There were no significant differences in the length of hospital stay. CONCLUSIONS: The use of SA has clear advantages for this medically vulnerable population. For the majority of patients, it obviates the need for ETT placement and airway management and avoids the potential negative effects of GA on neurocognitive development. It also decreases the use of narcotics and other supplemental medications. In scenarios in which the benefit of surgery must be weighed against the risk of GA, such as neonatal torsion, SA may allow a paradigm shift in the timing of surgery.

Urinary antimicrobial peptides: Potential novel biomarkers of obstructive uropathy.

INTRODUCTION: Antimicrobial peptides (AMPs) have historically been evaluated for their role in protecting against uropathogens. However, there is mounting evidence to support their expression in noninfectious injury, with unclear meaning as to their function. It is possible that AMPs represent urothelial injury. Urinary tract obstruction is known to alter the urothelium; however, AMPs have not been evaluated for expression in this noninfectious injury. OBJECTIVE: A pilot study to compare urinary AMP expression in children undergoing surgical intervention for ureteropelvic junction obstruction (UPJO) with nonobstructed controls. STUDY DESIGN: Bladder urine was collected from consenting/assenting pediatric patients with UPJO at intervention. Control bladder urines were obtained from age-matched and sex-matched healthy children without known obstruction or infection. Enzyme-linked immunosorbent assays were run for the following AMPs: ß defense 1 (BD-1), neutrophil gelatinase-associated lipocalin (NGAL), cathelicidin (LL-37), hepatocarcinoma-intestine-pancreas/pancreatitis-associated protein (HIP/PAP), and human α defensin 5 (HD-5); and normalized to urine creatinine. Results were analyzed with Student's t-test or Mann-Whitney U test, when appropriate, and receiver operating characteristic curves. A P-value of <0.05 was considered significant. RESULTS: Thirty bladder urine samples were obtained from children with UPJO at the time of decompressive intervention. Mean patient age was 4.7 years (range 0.3-18.4); 20 (67%) patients were male. Fifteen bladder urine samples were obtained from age-matched and sex-matched controls. Urinary AMP levels were significantly higher in UPJO patients than controls for BD-1 (P = 0.015), NGAL (P < 0.001), LL-37 (P < 0.001), and HIP/PAP (P = 0.046). Optimal threshold values of these AMPs were determined, with each demonstrating significant odds ratios of predicting urinary obstruction. DISCUSSION: Certain urinary AMPs are altered even in noninfectious urinary tract pathology. This represents a novel induction of AMP expression, as the current study is the first to report elevations in BD-1 and HIP/PAP in urinary tract obstruction. This suggests other roles for these AMPs outside of their antimicrobial properties, and likely is a reflection of the urothelial and tubular stress resulting from obstructive uropathy. CONCLUSIONS: Induction of AMPs BD-1, NGAL, LL-37, and HIP/PAP was found to occur in urinary tract obstruction. Further evaluation of AMP expression as a biomarker of uroepithelial injury outside of infection is indicated.

Vesicoscopic cross-trigonal ureteral reimplantation: High success rate for elimination of primary reflux.

INTRODUCTION: Open ureteral reimplantation (UR) is the gold standard for the surgical management of vesicoureteral reflux (VUR). There have been increasing reports on robot-assisted UR, but this approach remains controversial due to reports suggesting increased complications and reduced success compared with open repair. This study presented extensive experience with vesicoscopic ureteral reimplantation (VR) for primary reflux. In this procedure, cross-trigonal reimplantation was performed in a manner analogous to open repair under carbon dioxide 'pneumovesicum'. OBJECTIVE: To review outcomes of vesicoscopic cross-trigonal ureteral reimplantation and compare them with other minimally invasive approaches for the correction of reflux. STUDY DESIGN: All patients who underwent VR at the current institution were retrospectively reviewed. The following were recorded for each patient: age, sex, grade of reflux, operative time, outcome, and complications. RESULTS: The series consisted of 182 consecutive patients who underwent VR. There were 165 girls and 17 boys. Mean age was 7.03 years (range 16 months-38.2 years). Fifteen had failed prior injection therapy. Of the 182 patients, 135 underwent bilateral repairs and 47 unilateral. Mean operative time for bilateral repairs was 197 (112-284) minutes and 169 (99-288) for unilateral. Major complications included two ureteral obstructions: one resolved with stent placement and the other underwent re-operative reimplantation. Postoperative voiding cystourethrogram (VCUG) was obtained in 100 patients and was normal in 93 (93%). Four of these failures occurred in the first 30 patients. Of the last 49 patients tested, 48 were normal, suggesting a 98% effective success rate after the learning curve. DISCUSSION: Vesicoscopic ureteral reimplantation is an approach that completely recreates all aspects of open cross-trigonal repair. Complications were uncommon and success rates were very high in the current study. CONCLUSIONS: Vesicoscopic ureteral reimplantation is a minimally invasive procedure for the definitive repair of primary reflux. After the learning curve at the current institution, success rates were found to be equivalent to open repair and were higher than published reports of robot-assisted procedures.

The modified Ulaanbaatar procedure: Reduced complications and enhanced cosmetic outcome for the most severe cases of hypospadias.

INTRODUCTION/OBJECTIVE: Proximal hypospadias is one of the most challenging conditions that pediatric urologists have to deal with. Many procedures have been devised over the years, but nothing has been proven to be the best option. Although there have been some attempts at correcting severe hypospadias in one procedure, most have advocated a staged approach. The classic approach - laying penile skin or a graft within a split glans followed by glanuloplasty at the second stage - by definition requires two operations on the glans. In the Ulaanbaatar procedure the distal glanular urethra is constructed at the first stage, allowing for a single glans procedure and thus potentially better cosmetic outcomes. The present study discusses experience with the Ulaanbaatar procedure for severe hypospadias. STUDY DESIGN: The study retrospectively reviewed every child who underwent both stages of this procedure at the present institution. It reviewed age, associated diagnoses, surgical technique and outcomes. SURGICAL TECHNIQUE: The first stage was analogous to a classic first-stage procedure with regard to division of the urethral plate and correction of penile curvature. However, an island flap of preputial skin was mobilized and tubularized to create the glanular urethra. No attempt was made to bridge the native meatus and this reconstructed urethra, and the remaining penile skin was placed between the two. The second stage was performed 6 months later by tubularizing the penile skin between the two meatuses. RESULTS: The series consisted of 34 boys. Mean age at surgery was 18.3 months (range 6-118). Nineteen underwent evaluation for genital ambiguity at birth (56%). Thirty (88%) received pre-operative testosterone or human chorionic gonadotropin (HCG). After urethral plate transection, persistent curvature was addressed during the first stage, with dorsal plication in 12 (35%), urethral plate transection alone in six (18%) or ventral grafting with small intestinal submucosa in 16 (47%). Twenty-three boys (67%) had the neourethra tunneled through the glans, and 11 (33%) had the glans split followed by glanuloplasty. Average time between the two stages was 7 months (range 4.0-13.9). Four patients (12%) developed urethral diverticula that required repair. One developed recurrent epididymitis related to an abnormal ejaculatory duct (no stricture) and underwent vasectomy. No patient developed a fistula. Mean length of follow-up was 15.2 months (range 0.3-55.5). DISCUSSION: This modification of the classic staged hypospadias repair may allow for better cosmetic outcome, since the majority of boys required no formal glanuloplasty. There were reduced complications, perhaps because the urethral defect acted like a controlled fistula, allowing for better tissue healing prior to final urethral reconstruction.

Ureteroureterostomy irrespective of ureteral size or upper pole function: a single center experience.

OBJECTIVE: Although ureteroureterostomy (UU) is an established procedure for the treatment of duplex anomalies, there may be a reluctance to apply this approach to patients with poor upper pole function and/or marked degrees of ureteral dilation. METHODS: An institutional review board (IRB)-approved retrospective analysis of all patients undergoing UU between 2006 and present was performed. All patients underwent an end-to-side anastomosis with a double-J stent left in the lower pole ureter. Laparoscopic repairs were done 'high' and open repairs were done 'low'. If the upper pole ureter remained massively dilated after transection, the ureter was partially closed to reduce the length of the anastomosis. Data collected included demographics, diagnosis, surgical interventions, imaging studies and outcomes. RESULTS: A total of 41 patients (43 renal units) were identified. There were 35 females and six males with an average age at surgery of 2.3 years (range 55 days to 15.9 years) and an average follow up of 2.8 years. Diagnosis included ureterocele (17), ectopic duplex ureter (25) and ureteral triplication (1). Thirty-six patients underwent UU only and five underwent UU with simultaneous lower pole reimplantation. Twelve of the 41 patients (29%) underwent laparoscopic repair. Twelve of the 43 renal units (28%) required ureteral tapering, of which three were performed laparoscopically. Preoperative median upper pole function was 17% (0-35%). Six patients had no measurable function and ten had < 15%. No patient developed lower pole hydronephrosis in the follow-up period. There were two complications: one patient was found to have a post-operative ureterovesical junction (UVJ) stricture and the second had an anastomotic stricture. CONCLUSION: Ureteroureterostomy is a safe and effective technique for the reconstruction of duplex anomalies, even with a massively dilated and poorly functioning upper pole moiety. With no identifiable negative effect on the lower pole system, the concept of automatically removing 'dysplastic' upper pole segments can be challenged.

Is voiding cystourethrogram necessary in all cases of antenatal hydronephrosis?

Hydronephrosis is the most common genitourinary anomaly as detected on obstetric ultrasonography and the incidence of associated vesicoureteral reflux is around 10-12%. There is inconsistency in the literature regarding which child should under go a voiding cystourethrogram (VCUG) in cases of antenatal hydronephrosis (AHN). Besides, there is a scarcity of prospective studies to demonstrate the risk of varying degree of AHN, associated reflux and their long-term impact on the kidneys. The present analysis suggests that children with AHN should undergo an ultrasound within the first month of life and further course of action should be decided on the basis of the individual case. Children with persistent moderate to severe AHN should undergo a VCUG and a functional study.

Renal pelvis volume during diuresis in children with hydronephrosis: implications for diagnosing obstruction with diuretic renography.

PURPOSE: We measured the volume of the renal pelvis during diuretic renography (DR) in children with normal and hydronephrotic kidneys to determine if changes in pelvic volume could affect the accuracy of DR in diagnosing obstruction. MATERIALS AND METHODS: We studied 18 patients 1 month to 10 years old with unilateral hydronephrosis ultimately proved to be either obstructive or nonobstructive. Simultaneous DR and ultrasound were performed with patients supine using the gamma camera. Ultrasound measurements of the renal pelvis in 3 dimensions, obtained before and at intervals after diuretic injection, were used to calculate renal pelvic volume. The contralateral normal kidneys were used as controls. RESULTS: Between 15 and 60 minutes after diuretic injection the renal pelvis enlarged to a maximum volume in all hydronephrotic and normal kidneys and then gradually decreased in size. Mean average increase in volume for hydronephrotic kidneys ranged from 46% in obstructed kidneys to 88% in nonobstructed kidneys. Volume expansion caused dilution of isotope within the renal pelvis, which resulted in prolongation of elimination half-time (T1/2) in 42% of nonobstructed hydronephrotic kidneys sufficient to register an obstructed washout pattern. However, there were no differences in the initial pelvic volume or the rate or extent of increases or decreases in pelvic volume that would permit nonobstructed hydronephrotic kidneys to be distinguished from obstructed ones. CONCLUSIONS: The renal pelvis enlarges during diuresis in children with hydronephrosis. This enlargement causes dilution of isotope within the renal pelvis during DR, which prolonged the isotope washout rate or T1/2 sufficiently to produce an obstructed washout pattern in more than 40% of hydronephrotic kidneys that were ultimately proved to be nonobstructed. This misdiagnosis of obstruction is particularly likely to occur in children younger than 2 years because pelvic volume expansion is so exaggerated. Consequently, T1/2 appears to be particularly vulnerable to inaccuracy in diagnosing obstruction in this age group, and, therefore, it should not be relied on as an operative determinant.

Long-term followup of prenatally detected severe bilateral newborn hydronephrosis initially managed nonoperatively.

PURPOSE: We determine the outcome of severe bilateral primary ureteropelvic junction type hydronephrosis detected prenatally and managed postnatally with an initially nonoperative protocol. MATERIALS AND METHODS: A total of 19 newborns (38 kidneys) with prenatally diagnosed primary grade 3 to 4 bilateral hydronephrosis were followed nonoperatively for a mean of 54 months (range 14 to 187). If urinary obstruction with evidence of renal deterioration (decreased differential function and/or progressive hydronephrosis) occurred pyeloplasty was performed. RESULTS: Pyeloplasty was required in 13 kidneys (35%) in 9 patients (bilateral 4, unilateral 5). Age at pyeloplasty ranged from 2 to 22 months (mean 6.5) in 12 patients and 64 months in 1. The remaining 25 kidneys were followed nonoperatively (bilateral 20, unilateral 5). At last followup the Society for Fetal Urology grade of hydronephrosis in kidneys followed nonoperatively was 0 to 2 in 21 and 3 in 4, compared to 0 to 2 in 9 and Society for Fetal Urology 3 in 4 kidneys treated with pyeloplasty. Mean followup required for the most severely hydronephrotic kidney to achieve maximum ultrasound improvement was 10 months (range 3 to 34) for kidneys followed nonoperatively and 14 months (4-31) for kidneys after pyeloplasty. Differential renal function was measured in each kidney pair and compared using the difference in percent function between the 2 kidneys. In the nonoperative group mean initial difference in percent function was 8% (range 6% to 20%) and mean final difference was 5% (2% to 8%). In the pyeloplasty group mean initial difference in percent function was 16% (range 8% to 30%) and mean final difference was 7% (2% to 16%). With close followup and prompt pyeloplasty renal function improved to greater than pre-deterioration levels in all kidneys. CONCLUSIONS: These data represent the natural history of severe bilateral newborn hydronephrosis. Renal dilatation and function improve with time in most kidneys. Close followup is required in the first 2 years of life to identify the subgroup (35%) of children with obstruction that requires prompt surgery. Such an approach prevented permanent loss of renal function. Nonoperative management with close followup during the first 2 years appears to be a safe and recommended approach for neonates with primary bilateral ureteropelvic junction type hydronephrosis.

Percutaneous nephrostomy in children and adolescents: outpatient management.

PURPOSE: To determine if percutaneous nephrostomy can be performed safely as an outpatient procedure in children and adolescents. MATERIALS AND METHODS: Percutaneous nephrostomy was performed in 102 kidneys in 87 patients at 93 separate encounters. Patients were excluded from outpatient treatment if they presented with signs of infection, were hospitalized for other reasons, were undergoing additional endourologic stone procedures, had solitary kidneys or poor renal function, had social problems precluding outpatient care, or had a procedural complication. Follow-up was performed by means of direct communication and/or chart review. RESULTS: Successful outpatient percutaneous nephrostomy was performed in 39 (42%) of the 93 encounters. Reasons for exclusion included infection (n = 23), concomitant problems requiring hospitalization (n = 11), stone therapy (n = 7), solitary kidney with renal failure (n = 3), and social reasons (n = 10). No procedure-related complication occurred. No patient required readmission within 3 weeks for a tube- or procedure-related problem. CONCLUSION: Outpatient percutaneous nephrostomy can be safely performed in a selected group of patients.

Modification of the Koyanagi technique for the single stage repair of proximal hypospadias.

PURPOSE: We describe a modification of the Koyanagi technique for hypospadias. Use of opposing parameatal-based skin flaps that extend distally to incorporate the inner layer of the prepuce was modified to preserve blood supply to the flaps in an attempt to reduce complications and improve results. MATERIALS AND METHODS: During the last 7 years 20 boys underwent treatment of proximal hypospadias using the modified hypospadias repair. RESULTS: Cosmetic and functional, long-term (mean 34 months) results were excellent. Complications consisted of 4 urethrocutaneous fistulas (20%). There were no instances of meatal stenosis, diverticulum or urethral stricture. CONCLUSIONS: The modified technique permits 1-stage repair of proximal hypospadias with a low complication rate.

The long-term followup of newborns with severe unilateral hydronephrosis initially treated nonoperatively.

PURPOSE: During the last decade it has become apparent that prenatally detected, unilateral severe hydronephrosis does not necessarily represent obstruction and may spontaneously improve or resolve postnatally. To define its natural history better we performed a long-term (mean 78 months) followup study of infants with hydronephrosis. MATERIALS AND METHODS: A total of 104 newborns with antenatally diagnosed, primary, unilateral severe hydronephrosis were followed nonoperatively unless evidence of renal deterioration occurred for which pyeloplasty was performed. RESULTS: All 23 infants (22%) who required pyeloplasty were younger than 18 months and had progressive hydronephrosis and/or reduction in differential renal function. Differential function exceeded predeterioration levels in all kidneys postoperatively. Of those cases followed nonoperatively hydronephrosis resolved in 69% and improved in 31%. Mean time to maximum improvement of hydronephrosis was 2.5 years. In 76% of those cases followed nonoperatively initial differential function was greater than 40% and final function averaged 49%. In the remaining 24% of cases differential function was less than 40% (mean 23%), and in an average of 18 months differential function increased to a mean of 47%. Initial half-time in nonoperative cases was greater than 30 minutes in 37%, 20 to 30 in 21% and less than 20 in 42%. Final half-time was greater than 30 minutes in 16%, 20 to 30 in 17% and less than 20 in 67%. Half-time was greater than 30 minutes in 87% of the patients and 20 to 30 in 4% before, and greater than 30 in 10%, 20 to 30 in 27% and less than 20 in 63% after pyeloplasty. CONCLUSIONS: Unilateral newborn hydronephrosis appears to be relatively benign and in most instances dilatation and renal function improve with time. However, close followup is necessary to identify the subgroup of less than 25% of infants with obstruction because prompt pyeloplasty will prevent permanent loss of renal function. Standard tests for assessing obstruction in older patients appear to be invalid in infants because prolonged half-time and/or high grade hydronephrosis is neither an indicator of obstruction or surgery. Nonoperative treatment with close followup especially during the first 2 years is safe and recommended for these children.

Functional urinary tract obstruction developing in fetuses with isolated gastroschisis.

OBJECTIVE: To evaluate the frequency and natural history of urinary tract abnormalities developing in fetuses presenting with initially isolated gastroschisis. METHODS: Serial ultrasounds were performed prospectively on fetuses identified by our prenatal diagnosis program as having a gastroschisis. When abnormalities in the urinary tract were identified prenatally, newborns were evaluated by a pediatric urologist. RESULTS: Over a 1-year period four out of 12 fetuses with gastroschisis developed deformations of the urinary tract. In three fetuses the bladder herniated through the abdominal wall defect. Two also had upper tract dilatation. A fourth fetus developed bilateral hydronephrosis with a normally situated bladder. Once the gastroschisis was repaired none of the newborns had evidence of structural obstruction of the urinary tract, however, hydronephrosis with or without reflux persisted for several months. CONCLUSIONS: Deformations of the fetal urinary tract can develop secondary to gastroschisis. They do not appear to represent separate malformations and evaluation with fetal karyotyping may not be indicated. When hydronephrosis is present ongoing urologic evaluation of the neonate is indicated.

Preoperative treatment with human chorionic gonadotropin in infancy decreases the severity of proximal hypospadias and chordee.

PURPOSE: We determined whether human chorionic gonadotropin (HCG) pretreatment of severe proximal penoscrotal hypospadias and chordee causes sufficient penile shaft or skin enlargement to enhance surgical repair and improve patient outcome. MATERIALS AND METHODS: A total of 12 boys 6 to 12 months old with proximal hypospadias and severe chordee received a course of HCG for 5 weeks immediately preceding hypospadias repair. RESULTS: Chordee decreased and penile length increased in all cases (mean increase 94%). Penile length gain was disproportional. Most of the increase in length was proximal to the urethral meatus, which moved the meatus distally an average of 11.4 mm. (range 6.0 to 19.0), producing a mean increase of 586% in the distance between the penoscrotal junction and meatus. In contrast, there was no statistically significant increase in penile shaft length distal to the urethral meatus. Surgical treatment was facilitated by HCG pretreatment. Three meatal based repairs were performed, only 1 urethral fistula developed and chordee was corrected by penile degloving only in 8 cases. CONCLUSIONS: HCG pretreatment in infancy produces disproportional penile enlargement, which advances the meatus distally to decrease the severity of hypospadias and chordee. This response pattern simplifies the required surgical procedure and appears to improve surgical results. It may benefit select patients, and provide insights into the endocrinopathy of hypospadias and the embryopathy of the hypospadias-chordee complex.

Long-term outcome of transurethral puncture of ectopic ureteroceles: initial success and late problems.

PURPOSE: We studied the long-term outcome of transurethral puncture of ectopic ureteroceles specifically associated with duplex systems. MATERIALS AND METHODS: We retrospectively reviewed the records of patients who underwent transurethral puncture of an ectopic ureterocele. Study exclusion criteria were orthotopic, bilateral and prolapsing ureteroceles. RESULTS: We identified 19 girls and 2 boys, of whom 11 presented with prenatal hydronephrosis and 10 presented with urinary tract infection. Mean age at puncture was 5 months (range 0.5 to 60). Preoperatively voiding cystourethrography revealed no reflux in 7 patients, isolated ipsilateral lower pole reflux in 8, and bilateral and/or contralateral reflux in 6. Postoperatively studies initially showed no reflux in 8 cases but in 4 of the 8 reflux recurred up to 4 years after puncture. In 10 patients (48%) reflux developed into the ureterocele and upper pole segment. Repeat puncture was required 1 to 13 months after the initial procedure in 4 patients for persistent or recurrent upper pole hydroureteronephrosis. Subsequent open surgery was required in 15 of the 21 cases (71%), including ureterocele excision with ureteral reimplantation in 14. Of the children 10 and 4 underwent open surgery for recurrent urinary tract infection and progressive reflux, respectively, while 1 underwent ureteroureterostomy for progressive upper pole reflux. No patient underwent upper pole nephrectomy. Of the remaining 6 patients 4 have low grade reflux. CONCLUSIONS: Transurethral puncture of ectopic ureteroceles provides effective short-term correction of upper pole obstruction but it is not definitive therapy in the majority of cases. Most children still require open surgery. In patients without reflux after the puncture procedure new onset, recurrent or progressive reflux may later develop with extended followup. Repeat puncture may be required to ensure adequate decompression in a minority of cases, as in the 20% in our series.

Strategies for managing upper tract calculi in young children.

PURPOSE: Pediatric urolithiasis is relatively uncommon and there is little information on the application of modern surgical procedures in young children. We present a single center experience with the surgical management of upper tract calculi in this age group. MATERIALS AND METHODS: We reviewed presentation, co-morbidity, treatment, outcome and complications in all prepubertal patients who required surgical treatment for ureteral or renal calculi during a 4-year period. The series consists of 24 girls and 17 boys 17 months to 14 years old (mean age 7.5 years). A total of 26 children were anatomically normal, and 4 had myelomeningocele, 4 had ureteropelvic junction obstruction (in a pelvic kidney in 1), 2 had cloacal anomalies, 2 had vesicoureteral reflux, and 1 each had nonrefluxing megaureter, orthotopic ureterocele and a functioning renal transplant. RESULTS: Extracorporeal shock wave lithotripsy was performed in 24 patients. Stents or nephrostomy tubes were only used in the 4 patients who presented with pyonephrosis. Of the 41 cases 17 were rendered stone-free, 3 had a decreased stone burden and 4 were failures. Ureteroscopic extraction of distal ureteral calculi was successful in 11 of 12 children, of whom the youngest was 2.5 years old. No child had postoperative infection or evidence of ureteral obstruction. Stent placement facilitated stone passage or dissolution in 2 patients, a renal calculus was percutaneously extracted in 2 and 7 required open surgery, mostly for correcting simultaneous anatomical abnormalities or after minimally invasive surgery failed. Some metabolic abnormality was detected in 80% of the children tested. CONCLUSIONS: The surgical management of upper urinary tract calculi in young children parallels that in adults. Minimally invasive surgical methods may be safely used even in young infants. Most children do not need elective stenting before lithotripsy. Open procedures are still required in 17% of cases. The majority of children have definable metabolic abnormalities.

Postneonatal circumcision with local anesthesia: a cost-effective alternative.

PURPOSE: Despite the controversy regarding the need for routine neonatal circumcision, most boys in the United States are circumcised. Physicians are commonly asked to perform circumcision after the neonatal period and are often unaware of the cost factors related to the timing and location of postneonatal circumcision. MATERIALS AND METHODS: We describe the medical and financial advantages of postneonatal circumcision with local versus general anesthesia. RESULTS: During a 30-month period 245 boys 6 months to 15 years old underwent circumcision under general anesthesia in the operating room. Hospital charges (facility and equipment) averaged $1,555 and anesthesia charges averaged $250. Therefore, the average cost for circumcision in the operating room was $1,805. During the same time period 287 infants 3 days to 9 months old (20% older than 3 months) underwent circumcision under local anesthesia in an office setting. The facility and equipment charge for these office procedures averaged $196. Overall, approximately $461,783 were saved in this 30-month period ($184,713 annually) by performing circumcision with local anesthesia in an office setting rather then in the operating room with general anesthesia. There was no significant difference in complication rates between the local and general anesthesia groups (1.4 versus 1.6%). CONCLUSIONS: Circumcision with local anesthesia can be performed easily and safely during the first several months of life and has many advantages. Parents prefer this method because it is more convenient and eliminates the risk of general anesthesia. The enormous cost savings using local as opposed to general anesthesia should prompt a reexamination of the location and timing of postneonatal circumcision.

The relationship among dysfunctional elimination syndromes, primary vesicoureteral reflux and urinary tract infections in children.

PURPOSE: We determine whether functional bladder and/or bowel disorders influence the natural history or treatment of children with primary vesicoureteral reflux. MATERIALS AND METHODS: We assessed 143 children with primary vesicoureteral reflux that stopped spontaneously or was surgically corrected for functional bowel and/or bladder disorders, including bladder instability, constipation and infrequent voiding, termed the dysfunctional elimination syndromes. RESULTS: Dysfunctional elimination syndromes were present in 66 of 143 children (43%) thought to have primary vesicoureteral reflux. Of these 66 patients 54 (82%) had a breakthrough urinary tract infection and underwent reimplantation compared to only 18% without the syndromes. Of 70 children who had a breakthrough urinary tract infection dysfunctional elimination syndromes were present in 54 (77%) and absent in 16 (23%). Of the remaining 73 patients who did not have a breakthrough infection dysfunctional elimination syndromes were present in 12 (16%) and absent in 61 (84%). In children with dysfunctional elimination syndromes the resolution of reflux that was 1 grade less severe required an average of 1.6 years longer. After the disappearance of reflux, urinary tract infection developed in 18 children, including 14 (78%) with dysfunctional elimination syndromes. Unsuccessful surgical outcomes involving persistent, recurrent and contralateral reflux occurred only in children with dysfunctional elimination syndromes. CONCLUSIONS: Dysfunctional elimination syndromes are common and are often unrecognized in children with primary reflux. These syndromes are associated with delayed reflux resolution and an increased rate of breakthrough urinary tract infection, which leads to reimplantation surgery. Dysfunctional elimination syndromes also adversely affect the results of reimplantation and represent a risk for recurrent urinary tract infection after reflux resolves. The evaluation and management of dysfunctional elimination syndromes should be an integral part of the treatment of every child with vesicoureteral reflux. Effective evaluation and treatment may be made cost-effective by decreasing the followup, the number of breakthrough urinary tract infections and the number of children requiring reimplantation.

Reconstructive surgery of the upper urinary tract.

This paper reviews the major publications over the past year regarding upper urinary tract reconstruction. Controversies in the diagnosis of ureteropelvic junction obstruction and in the surgical repair of the obstructed upper urinary tract are discussed. Special emphasis is placed on issues surrounding minimally invasive techniques.