Sonographically Determined Fecal Width: An Objective Indicator of Management of Bladder and Bowel Dysfunction in Children.

OBJECTIVE: To test whether sonographically determined fecal width (SDFW) correlates with symptom improvement in a population of children with bladder and bowel dysfunction (BBD) managed with standard urotherapy (SU), even for those patients lacking initial bowel complaints. METHODS: We retrospectively analyzed 200 pediatric BBD patients managed with SU for at least 3 months. Self-reported symptom improvement (complete, partial, no response) following International Children's Continence Society guidelines was tabulated. Patients with complex urologic diagnoses other than vesicoureteral reflux (VUR) were excluded. Pharmacotherapy choice, physical therapy (PT), urinary tract infection (UTI) occurrence, and VUR status were tabulated. SDFW was recorded. Non-parametric analysis of variants (ANOVA) and parametric/non-parametric t testing were used for analysis. RESULTS: Patients had a mean age of 9.5 years (4-12). Forty-eight patients had no gastrointestinal complaints at presentation. Urotherapy yielded complete, partial, and no responses in 14% (n = 27), 33% (n = 67), and 53% (n = 106) of patients, respectively. The average SDFW for those patients with complete response (2.6 cm) was smaller than the SDFW of those with a partial response (3.1 cm) or no response (3.3 cm) (P = .0001). Non-compliance led to greater SDFW compared to compliant patients (3.7 cm and 3.1 cm, respectively, P = .0001). Fecal width was unaffected by VUR, UTI, PT, or pharmacotherapy. CONCLUSION: SDFW correlates well with symptom improvement in pediatric patients managed for BBD, confirming our hypothesis. SDFW is reasonable as single objective parameter to identify successful management in patients with BBD, extending to those without bowel complaints at presentation.

Dermoid Cyst in a Multicystic Dysplastic Kidney: A Case Report and Literature Review.

Most multicystic dysplastic kidneys (MCDKs) are discovered prenatally. There is no consensus regarding initial workup and appropriate follow-up. We present a 9-year-old female who was fetally diagnosed with a MCDK and without follow-up returned with an 18-cm multicystic mass. The patient underwent laparoscopic nephrectomy. Final pathology revealed a dermoid cyst arising in a pediatric kidney, which to our knowledge has not been previously described. Patients with MCDK have hypertension as a possible sequela and possible reflux to their functioning kidney. Voiding cystourethrogram seems reasonable initially, and renal ultrasound is ideally noninvasive. Focused clinical awareness of the solitary kidney is important.

En Bloc Retroperitoneoscopic Removal of Horseshoe Kidney for End-stage Renal Disease.

Horseshoe kidney (HSK) is the most common renal fusion anomaly. There have been reports of an association of HSKs with medical renal disease. We report a case of a child with nephrotic-range proteinuria and a HSK. As the patient was on peritoneal dialysis, the entire HSK was removed en bloc via a retroperitoneoscopic approach with early postoperative reinitiation of peritoneal dialysis.

Cross Talk Between GH-Regulated Transcription Factors HNF6 and CUX2 in Adult Mouse Liver.

Hepatocyte-enriched nuclear factor (HNF)6 and CUX2 are GH and STAT5-regulated homeobox transcription factors. CUX2 shows female-specific expression and contributes to liver sex differences by repressing many male-biased genes and inducing many female-biased genes, whereas HNF6 is expressed at similar levels in male and female liver. In cell-based transfection studies, CUX2 inhibited HNF6 transcriptional regulation of the sex-specific gene promoters CYP2C11 and CYP2C12, blocking HNF6 repression of CYP2C11 and HNF6 activation of CYP2C12. These inhibitory actions of CUX2 can be explained by competition for HNF6 DNA binding, as demonstrated by in vitro EMSA analysis and validated in vivo by global analysis of the HNF6 cistrome. Approximately 40 000 HNF6-binding sites were identified in mouse liver chromatin, including several thousand sites showing significant sex differences in HNF6 binding. These sex-biased HNF6-binding sites showed strong enrichment for correspondingly sex-biased DNase hypersensitive sites and for proximity to genes showing local sex-biased chromatin marks and a corresponding sex-biased expression. Further, approximately 90% of the genome-wide binding sites for CUX2 were also bound by HNF6. These HNF6/CUX2 common binding sites were enriched for genomic regions more accessible in male than in female mouse liver chromatin and showed strongest enrichment for male-biased genes, suggesting CUX2 displacement of HNF6 as a mechanism to explain the observed CUX2 repression of male-biased genes in female liver. HNF6 binding was sex independent at a majority of its binding sites, and HNF6 peaks were frequently associated with cobinding by multiple other liver transcription factors, consistent with HNF6 playing a global regulatory role in both male and female liver.

Duration of urinary leakage after open non-stented dismembered pyeloplasty in pediatric patients.

OBJECTIVE: We aimed to determine the duration and associated complications of postoperative urinary leakage in pediatric patients undergoing open, non-stented dismembered pyeloplasty for ureteropelvic junction obstruction. METHODS: A retrospective review of 100 patients who underwent an open non-stented dismembered pyeloplasty between 2003 and 2008 was performed. Duration of urinary leakage and postoperative complications were tabulated. Patients were considered to have a dry anastomosis if the Penrose drain was removed within one week of surgery. RESULTS: Duration of leakage ranged from 0 to 27 days. 86% had Penrose drain removal within 7 days of surgery and were considered dry.14 patients demonstrated a persistent urinary leakage (PUL) ranging from 7 to 27 days. Complications of any type were significantly more likely in the group with prolonged drainage (p = .0126). UTI and obstruction were not significantly more likely to occur in patients with PUL (p = .0931 and p = .2616 respectively). Only one patient with PUL required placement of a ureteral stent. CONCLUSION: We demonstrate that stentless dismembered pyeloplasty is feasible with a low rate of urinary drainage beyond one week. The character and quality of the slightly increased complications in those that demonstrated PUL were not great and not bothersome enough to warrant routine stenting.

Is the comparison of a left varicocele testis to its contralateral normal testis sufficient in determining its well-being?

OBJECTIVE: To determine whether a size difference between a left testis involved with a varicocele and the contralateral normal testis is sufficient in its size assessment. METHODS: We reviewed all pediatric scrotal ultrasounds at Helen DeVos Children's Hospital between 2001 and 2008. Sonographic testicular measurements were recorded for patients with clinically diagnosed left varicocele (n = 81 for "varicocele" group) and for patients with no specific pathologic findings (n = 184 for "normal" group). We first compared the sizes between left and right testes for all patients, then between the "varicocele" group and the "normal" group separately for left and right. RESULTS: There were no significant size differences between left and right testes for the "normal" group, whereas the left was significantly smaller than the contralateral right for the "varicocele" group (P = .0048 for length; P = .012 for volume) for all ages. For boys 16 years or older, both the left and contralateral right testes of "varicocele" boys were smaller than those of "normal" boys, adjusting for age (P for left = .026 for length and .059 for volume; for right P = .033 for length and .031 for volume). CONCLUSION: Our study confirms that the left testis in boys with varicocele is most often smaller than the contralateral right testis no matter the age. Compared with normal boys in late adolescence, the contralateral right testis is smaller in boys with varicocele.

Rate and associations of epididymal cysts on pediatric scrotal ultrasound.

PURPOSE: We established the baseline occurrence of epididymal cysts, and the correlation between epididymal cysts and testicular size. MATERIALS AND METHODS: We retrospectively reviewed all pediatric scrotal ultrasounds done at our institution in 8 years. We analyzed the proportion of cysts by patient age and compared testicular size in boys with vs without epididymal cysts. RESULTS: Of all patients 14.4% had epididymal cysts. The cyst incidence increased with age, ie 35.3% of boys older than 15 years had cysts. Boys with epididymal cysts had larger testes than boys without cysts regardless of side or age (p <0.001). CONCLUSIONS: Epididymal cysts are more common in older boys. Boys with epididymal cysts had larger testes than boys without cysts.

Is age at toilet training associated with the presence of vesicoureteral reflux or the occurrence of urinary tract infection?

PURPOSE: We sought to determine whether age at toilet training is influenced by a history of vesicoureteral reflux or urinary tract infection. MATERIALS AND METHODS: We reviewed records on 1,184 patients treated at a pediatric urology practice. All patients had information available regarding age at toilet training, renal sonography and voiding cystourethrography, and presence or absence of urinary tract infection. We evaluated possible associations between vesicoureteral reflux and urinary tract infection, and age at toilet training. RESULTS: Of 1,184 patients 280 had unilateral reflux, 339 had bilateral reflux and 565 had normal anatomy. Also, 926 patients had urinary tract infections. Girls tended to be toilet trained 3 months earlier than boys (p <0.001) in all subgroups (normal anatomy, unilateral reflux, bilateral reflux). Children with and without urinary tract infections were toilet trained at similar ages. However, timing of the first urinary tract infection seemed to be associated with age at toilet training. For girls a urinary tract infection occurring earlier tended to delay toilet training, while earlier toilet training seemed to be associated with a later urinary tract infection (p <0.001). The patterns were similar for boys but were not statistically significant. CONCLUSIONS: Age at toilet training seems to be independent of the presence of vesicoureteral reflux. Urinary tract infection itself is not necessarily associated with age at toilet training. However, timing of the first urinary tract infection seems to be related to age at toilet training.

Correlation of hydronephrosis index to society of fetal urology hydronephrosis scale.

Purpose. We seek to correlate conventional hydronephrosis (HN) grade and hydronephrosis index (HI). Methods. We examined 1207 hydronephrotic kidneys by ultrasound. HN was classified by Society of Fetal Urology guidelines. HN was then gauged using HI, a reproducible, standardized, and dimensionless measurement of renal area. We then calculated average HI for each HN grade. Results. Comparing HI to standard SFU HN grade, average HI is 89.3 for grade I; average HI is 83.9 for grade II; average HI is 73.0 for grade III; average HI is 54.6 for SFU grade IV. Conclusions. HI correlates well with SFU HN grade. The HI serves as a quantitative measure of HN. HI can be used to track HN over time. Versus conventional grading, HI may be more sensitive in defining severe (grades III and IV) HN, and in indicating resolving, stable, or worsening HN, thus providing more information for clinical decision-making and HN management.

An approach for using full thickness skin grafts for complex penile surgeries in children.

PURPOSE: Many problems in pediatric urology derive from a paucity of penile skin resulting from prior surgical interventions. While hypospadias surgery is most often responsible for creating this problem, excessive circumcision also can leave a patient with too little skin to cover the penis. To our knowledge we describe the first series of pediatric patients in whom FTSGs were used in a variety of difficult circumstances where penile skin was lacking. MATERIALS AND METHODS: We retrospectively studied a cohort of 11 children 2 to 13 years old who underwent urethral repair and adjunctive skin grafting due to circumcision injuries (4 patients), traumatic urethral injury (1) or congenital lymphangiectasis (1), or for congenital hypospadias with previous failed surgery (5). In our patients available penile skin was used to reconstruct the urethra, while full thickness inguinal skin grafts were fashioned to resurface the denuded penis following reconstruction. RESULTS: All patients underwent successful reconstruction and grafting. There were no intraoperative complications. There was 100% take of the grafts. Average followup was 23 months (range 3 weeks to 8.6 years). One patient had slight chordee at 6 years postoperatively, and 1 had development of a urethrocutaneous fistula at 8.6 years. All patients reported normal caliber urinary streams. CONCLUSIONS: Use of full thickness inguinal skin grafts to resurface the penis provided patients with an esthetically acceptable result, and where necessary allowed penile skin to be used for urethroplasty. This technique is useful and justifies consideration in appropriately selected patients.

MrNomogram: a web-based multivariable pediatric renal nomogram.

UNLABELLED: Multivariable Pediatric Renal Nomogram, or MrNomogram, is a web-based clinical tool for the evaluation of pediatric renal length. Unlike other available age-adjusted renal nomograms, MrNomogram takes into consideration the fact that the renal length is influenced by multiple demographic variables. It provides a more accurate prediction of pediatric renal length, given the patient's demographic characteristics, such as age, gender, height, etc. ( AVAILABILITY: https://www.prevmed.sunysb.edu/jjc/MrNomogram).

Voiding dysfunction: outcome in infants with congenital vesicoureteral reflux.

OBJECTIVES: To assess the rate of development of voiding dysfunction as an outcome in patients with congenital vesicoureteral reflux. METHODS: A computerized database was used to review the medical records of all patients with congenital reflux diagnosed before 6 months of age from 1988 to 2002. Only those infants with reflux who were followed up for at least 6 months past the age of toilet training were included. The parameters tabulated in this group included the presence of frequency, daytime wetting, urge incontinence, and infrequent voiding. The variables were summarized using contingency tables, and associations were evaluated using chi-square analysis and Fisher's exact test. RESULTS: Of 342 patients (145 boys and 197 girls) with reflux, 67 (19.6%) developed voiding dysfunction (17.9% boys and 20.8% girls). No statistically significant difference was found between high grade (IV and V) and low grade (I and II) or grade III reflux, or between unilateral and bilateral reflux for the development of voiding dysfunction. Of the patients with reflux who had renal damage on dimercaptosuccinic acid scan, 24.3% of girls and 30.4% of boys developed voiding dysfunction, which was not significantly different from those without renal damage. CONCLUSIONS: Approximately 20% of patients with vesicoureteral reflux diagnosed before 6 months of age demonstrated dysfunctional voiding after the age of toilet training. Voiding dysfunction occurred independent of sex, laterality, or severity of vesicoureteral reflux. Infants with renal damage on dimercaptosuccinic acid scan developed dysfunctional voiding only at a slightly greater, but not significantly different, rate than those with normal kidneys.

A multivariable assessment of renal size and growth of scarred kidneys in children.

PURPOSE: We applied a recently developed multivariable renal size nomogram to sonographic measurements of kidneys with known scarring to assess systematically their sizes and growth patterns compared to normal kidneys. MATERIALS AND METHODS: We retrospectively reviewed renal sonograms of 138 kidneys (55 right and 83 left) with known scarring. The sizes of these scarred kidneys were compared to the 95% prediction limits calculated according to the multivariable renal size nomogram, adjusting for patient age, gender, race, weight and height. The growth of scarred kidneys was evaluated by fitting individual linear regression lines using serial sonographic measurements and comparing the results with normal predicted values. RESULTS: The sizes of 89.1% of the right and 81.9% of the left scarred kidneys were within the 95% normal prediction limits. Only 17 of 138 of the scarred renal units showed smaller kidney sizes compared to the normal prediction limits. Serial sonographic measurements revealed that compared to normal predicted values, 24 of 60 left and 16 of 38 right scarred kidneys grew within +/- 1.0% annually of the boundaries of normal predicted values. Additionally, 8 left and 7 right scarred kidneys indicated a growth rate of more than 1% annually higher than the normal predicted values. CONCLUSIONS: Multivariable analyses of renal sonographic measurements provided a dynamic picture of kidney well-being in children with renal scarring. We found that the majority of scarred kidneys were within the normal predicted limits, and a large proportion of these kidneys grew equally well compared to those with normal anatomy.

Endocrine disruption and hypospadias.

The complexity of human biology makes it impossible to know for certain if endocrine disruption accounts for human penile deformities. Toxicologists point out that an overall assessment of risk must include other factors in addition to exposure including absorption, metabolism, excretion, bioaccumulation and other chemical interactions (Harrison et al., 1997). Many skeptics observe lack of analytic ability to document contaminant levels during critical windows of exposure (Safe, 2000). Further, the environmental estrogens studied (DDT, PCB and bis-phenol A) are quite weak compared to the well studied potent estrogen DES which did not cause penile deformities (Joffe, 2001). While environmental estrogens may be unlikely in contributing to penile deformities, the antiandrogens (phthalates, vinclozolin and DDE) are more plausible is this regard, as maleness is critically dependent upon androgen action. Observers note that, in general, the environmental concentrations of persistent organochlorine compounds have been decreasing over the past two decades. Some feel that our current levels of exposure are too low and the potency of the anti-androgens too weak to account for any significant developmental genital effect (Williams et al., 2001). Caution and restraint are always reasonable in matters of data intrepretation. Past researchers were reassured that pthtalate esters were quite safe when they first were assessed for possible harmful effects on male fertility. Unfortunately it took different models, analyzing transgenerational effects, before it became crystal clear that these compounds can dramatically affect male genital development following experimental maternal exposure at dosages and concentrations currently present in most women. We can not now be so reassured that our male development is unaffected by any of the over 65,000 manmade organochlorine compounds on the planet. Multiple observations from diverse disciplines provide credible evidence that proliferation of xenobiotic chemicals can cause potentially disastrous unintended consequences for the male gender, and upon reflection, our species.

A multivariate analysis of dysfunctional elimination syndrome, and its relationships with gender, urinary tract infection and vesicoureteral reflux in children.

PURPOSE: We explored and quantified the relationships between dysfunctional elimination syndrome (DES), and gender, urinary tract infection (UTI) and vesicoureteral reflux (VUR) in children. MATERIALS AND METHODS: Data on 2,759 pediatric patients treated at a referral practice who underwent renal sonography and voiding cystourethrography were summarized. The patients were children with VUR or normal genitourinary anatomy who presented with UTI or dysfunctional voiding and children screened for genitourinary problems such as hematuria, sibling reflux or bedwetting. A multivariate logistic regression approach was used to model and quantify the associations between DES and other pediatric urology factors. RESULTS: Of the girls 36.0% with unilateral VUR had DES, while 36.1% with bilateral VUR had DES. The corresponding rates for boys were 20.5% and 21.2%. The higher rate of DES in girls was independent of UTI and VUR status. While UTI was not associated with DES in boys or girls without VUR, in patients with VUR and UTI the risk of DES almost doubled (OR 1.97). Reflux alone without UTI was negatively associated with DES in boys (OR 0.50, 95% CI 0.34, 0.73) and girls (OR 0.26, 95% CI 0.19, 0.36). CONCLUSIONS: Girls had a significantly higher rate of DES than boys in all UTI and VUR subgroups in the current data. UTI significantly impacts the DES occurrence in patients with VUR. No statistically significant difference was detected in the DES rate between the unilateral and bilateral VUR groups, and the reflux group as a whole did not seem to have a higher rate of DES in boys or girls.

Epididymal cysts in children: natural history.

PURPOSE: We reviewed the outcome of conservative management of epididymal cyst in children at our institution. MATERIALS AND METHODS: We retrospectively reviewed the records of all patients with epididymal cyst from 1991 to 2002. Age and mode of presentation as well as time to complete involution of the cysts were studied. Diagnosis of epididymal cyst was confirmed by scrotal ultrasound in all cases. RESULTS: A total of 20 patients were identified with epididymal cyst. Average patient age at presentation was 10.5 years. Fifteen patients presented with scrotal mass and 4 with scrotal pain. Cysts were between 3 and 30 mm. Only 1 patient required surgical excision due to persistent pain. Epididymal cysts resolved in 10 patients who completed followup. Average time to complete regression was 17 months. None of our patients had a history of exposure to diethylstilbestrol, cryptorchidism, cystic fibrosis or von Hippel-Lindau disease. CONCLUSIONS: Our findings imply that most epididymal cysts involute with time.