Imaging characteristics predicting elevated detrusor pressures in patients with spina bifida.

PURPOSE: Patients with spina bifida require urologic follow up with assessments of bladder and renal function. The ideal screening protocol for bladder compliance remains controversial. This study assessed associations between bladder end filling pressure (EFP) and detrusor leak point pressure (DLPP) on urodynamic studies (UDS) and findings from renal bladder ultrasounds (RBUS) and voiding cystourethrograms (VCUG). METHODS: Pediatric spina bifida patients who underwent RBUS and/or VCUG within six months of UDS at a single institution from 2015 to 2021 were retrospectively reviewed. Demographics, VCUG and RBUS results, and UDS EFP and DLPP were recorded. Multiple linear regression analyses and Pearson chi-square tests were conducted to correlate RBUS and VCUG findings with DLPP/EFP. RESULTS: 129 patients were included. In adjusted analyses, moderate to severe hydronephrosis on RBUS was significantly associated with elevations in mean DLPP/EFP (p = 0.006). Patients with round bladders had significantly lower mean DLPP/EFP than patients with elongated or conical bladders (p < 0.0001). The presence of trabeculations was associated with increased mean DLPP/EFP (p < 0.0001) and increases in severity of trabeculations were associated with higher DLPP/EFP (p < 0.0001). The presence of vesicoureteral reflux (VUR) on VCUG was not associated with significant differences in mean DLPP/EFP (p = 0.20). CONCLUSIONS: Abnormal bladder shape and trabeculations on VCUGs in spina bifida patients had significant associations with increased mean bladder pressures on UDS. Assessment of bladder shape and contour by VCUG may be a valuable screening tool in conjunction with routine RBUS in determining the need for further assessment of bladder pressures in patients with spina bifida.

False positive pregnancy tests in pediatric patients with augmentation enterocystoplasty.

PURPOSE: Our primary aim was to determine the prevalence of positive over-the-counter (OTC) pregnancy tests among pediatric female and male patients who had undergone augmentation enterocystoplasty. A secondary aim was to determine whether patient factors or urinalysis (UA) results were associated with false positive OTC pregnancy test results in patients with a history of augmentation enterocystoplasty. METHODS: Patients at a tertiary pediatric hospital who had previously undergone augmentation enterocystoplasty, Mitrofanoff appendicovesicostomy only, and age- and sex-matched controls were prospectively recruited. Urine samples were obtained, and two OTC pregnancy tests were conducted - OTC Test A reported the ability to detect pregnancy at hCG levels as low as 9.6 mIU/mL, while OTC Test B reported positive pregnancy test results at hCG levels ≥25 mIU/mL. A point of care (POC) pregnancy test and UA were also completed. Mann-Whitney U tests and Fisher's exact tests were used to assess whether patient factors or UA results were associated with false positive pregnancy test results. RESULTS: Fifty pediatric patients were enrolled, including 20 with augmentation enterocystoplasty, 20 age- and sex-matched controls, and 10 with Mitrofanoff appendicovesicostomy only. Among the 20 patients who had undergone augmentation cystoplasty, 15 (75.0%) had positive pregnancy test results on OTC Test A, including 11/13 (84.6%) females and 4/7 (57.1%) males. No control patients or patients with Mitrofanoff appendicovesicostomy only had a positive test. No patients had positive pregnancy test results on OTC Test B or the POC test. While patients with augmentation enterocystoplasty were significantly more likely to test positive for blood (p = 0.01), nitrate (p = 0.03), and leukocytes (p < 0.0001), these factors were not significantly associated with false positive pregnancy results. No patient factors or UA results were associated with increased likelihood of false positive OTC pregnancy test results. CONCLUSIONS: These findings indicate that sensitive OTC pregnancy tests that detect low quantities of hCG in urine may result in false positive results among patients who have undergone augmentation enterocystoplasty. Female patients of reproductive age with bladder augmentations should be counseled regarding appropriate pregnancy testing options. Additional research is needed to determine the mechanism responsible for false positive pregnancy tests in this patient population.

Utility of renal scintigraphy in diagnosis of multicystic dysplastic kidney.

PURPOSE: To compare the prevalence of vesicoureteral reflux (VUR), febrile urinary tract infection (fUTI), and chronic kidney disease (CKD) among patients with multicystic dysplastic kidney (MCDK) diagnosed by renal scintigraphy (RS) versus follow-up renal ultrasound (RUS) alone. METHODS: This was a retrospective review of patients seen at a tertiary care center from 2010 to 2020 with MCDK diagnosed by RS or follow-up RUS. Differences in the prevalence of VUR, fUTI, and CKD by cohort were assessed using logistic regression analysis, Pearson X2 , and Fisher's Exact tests. Temporal trends in diagnostic methods used (RUS versus RUS + RS) were evaluated using the Cochran-Armitage trend test. RESULTS: One-hundred seventy-two patients were included: 50% (n = 86) underwent RUS + RS and 50% (n = 86) underwent RUS alone to diagnose MCDK. Prevalence of VUR, fUTI, and CKD did not significantly vary between groups. Among patients who had a VCUG, 4.4% had contralateral VUR (1.7% RUS + RS group; 7.4% RUS group; p = 0.19) and 14.5% had at least one fUTI (16.3% RUS + RS group; 12.8% RUS group; p = 0.52). Females were significantly more likely to have at least one fUTI (p = 0.04). Four patients (2.3%) developed CKD, all in the RUS + RS cohort (p = 0.12). Diagnosis of MCDK by RUS versus RUS + RS did not significantly vary over time (p = 0.17). CONCLUSION: Patients with unilateral MCDK confirmed by RS versus RUS alone do not significantly vary in the prevalence of VUR, fUTI, or CKD. Renal scintigraphy studies may not be necessary in unilateral MCDK diagnosis but continue to be used.

Association between severity of prenatally diagnosed hydronephrosis and receipt of surgical intervention postnatally among patients seen at a fetal-maternal center.

BACKGROUND: Hydronephrosis (HN) is the most common abnormality detected on prenatal ultrasound. This study sought to stratify outcomes of patients by severity of prenatal HN with postnatal outcomes. METHODS: This was a retrospective review of patients referred to a tertiary care fetal-maternal clinic with diagnosis of prenatal HN from 2004 to 2019. HN severity was categorized as mild, moderate, or severe. Data were analyzed to determine the association between HN severity and surgical intervention. Decision for surgery was based on factors including history of multiple urinary tract infections, evidence of renal scarring, and/or reduced renal function. Surgery-free survival time was represented by the Kaplan-Meier method, and hazard ratios were calculated using the log-rank test. RESULTS: 131 kidneys among 101 infants were prenatally diagnosed with hydronephrosis; 35.9% had mild HN, 29.0% had moderate HN, and 35.1% had severe HN. 8.5% of patients with mild HN, 26.3% of patients with moderate HN, and 65.2% of patients with severe HN required surgery. Patients with severe HN were 12.2 (95% CI 6.1-24.4; p < 0.001) times more likely to undergo surgery for HN than patients with mild HN and 2.9 (95% CI 1.5-5.3; p = 0.003) times more likely to undergo surgery than patients with moderate HN. Patients with moderate HN were 4.3 times more likely to require surgery than patients with mild HN (95% CI 1.5-12.9; p = 0.01). Median age at surgery was 11.8 months among patients with mild HN (IQR 11.7-14.1 months), 6.6 months among patients with moderate HN (IQR 4.2-16.4 months), and 5.4 months among patients with severe HN (3.7-12.4 months). CONCLUSION: Among this cohort of referrals from a fetal-maternal clinic, severity of HN correlated with increased likelihood of surgical intervention. Continued assessment of patients with prenatal HN should be evaluated to best determine the role of the pediatric urologist in cases of prenatal HN.

Mature Ovarian Cystic Teratoma Mimicking a Bladder Diverticulum in a 16-year-old Female.

A 16-year-old female was incidentally found to have an abnormal pelvic ultrasound while undergoing evaluation for mild scoliosis. A large, thick-walled, lobular, fluid-filled structure was found at midline in the pelvis that initially resembled bladder diverticula due to its anatomical position and sonographic appearance. Voiding cystourethrogram and computed tomography later revealed a large cystic mass originating from the right ovary. This report outlines a case of a mature cystic teratoma convincingly mimicking bladder anomaly on sonography.

Missed anterior sacral meningomyelocele presenting with obstructive uropathy.

A 2-month-old full-term female presented with a large anterior sacral meningomyelocele resulting in transient obstructive uropathy with bilateral hydronephrosis and acute kidney injury. After initial bladder decompression and surgical resection of the meningomyelocele, there was spontaneous resolution of bladder function confirmed with urodynamics. Anterior spinal meningomyelocele (ASM) is a rare neural tube defect that may present with urinary dysfunction secondary to compression of the bladder and sacral nerve roots or congenital defects to the bladder nervous supply. Obstructive uropathy due to ASM may spontaneously resolve after surgical resection.

Robotic adrenalectomy in the pediatric population: initial experience case series from a tertiary center.

BACKGROUND: Laparoscopic resection is the most well described minimally-invasive approach for adrenalectomy. While it allows for improved cosmesis, faster recovery and decreased length of hospital stay compared with the open approach, instrument articulation limitations can hamper surgical dexterity in pediatric patients. Use of robotic assistance can greatly enhance operative field visualization and instrument control, and is in the early stages of adoption in academic centers for pediatric populations. CASE PRESENTATION: We present a single-institution series of pediatric adrenalectomy cases. The da Vinci Xi surgical system was used to perform adrenalectomies on three consecutive patients (ages, 2-13 years) at our center. Final pathology revealed ganglioneuroblastoma (n = 2) and pheochromocytoma (n = 1). Median operating time was 244 min (range, 244-265 min); median blood loss was estimated at 100 ml (range, 15-175 ml). Specimens were delivered intact and all margins were negative. Median post-operative hospital stay was 2 days (range, 1-6 days). All patients remain disease-free at median follow-up of 19 months (range, 12-30 months). CONCLUSION: Our experience continues to evolve, and suggests that robotic surgery is safe, feasible and oncologically effective for resection of adrenal masses in well-selected pediatric patients.

Severe ureteropelvic junction obstruction with massive hydronephrosis causing compression of the inferior vena cava.

A 7-week-old girl presented with tachypnea and abdominal distension. Abdominal ultrasonography demonstrated a 16 cm cystic mass in the right abdomen. Contrast-enhanced T2-weighted magnetic resonance imaging revealed severe hydronephrosis of the right kidney with thinning of the renal parenchyma consistent with a massive ureteropelvic junction (UPJ) obstruction. A percutaneous nephrostomy tube was placed for decompression of her right kidney and 1270 ml of fluid was drained. The patient underwent an open pyeloplasty. Her follow-up renal ultrasonography demonstrated resolution of her severe hydronephrosis.

Forniceal Rupture in Neonate Masquerading as a Cystic Renal Mass.

A 5-week-old full-term female with high-risk right hydronephrosis was transferred to the emergency department (ED) for tachypnea. A contrast-enhanced computed tomography (CT) study demonstrated a 9.0 cm cystic lesion in the region of the previously seen hydronephrosis (Fig. 1). Urology was consulted to rule out a cystic neoplasm, which on review of imaging was more suggestive of a contained forniceal rupture. An ultrasound-guided percutaneous nephrostomy tube drained 200 cc of urine. Culture and cytology were negative. Respiratory status markedly improved. The patient was discharged with outpatient follow to evaluate for ureteropelvic junction obstruction.

Pelvic floor laxity: A not so rare but unrecognized form of daytime urinary incontinence in peripubertal and adolescent girls.

INTRODUCTION: Stress urinary incontinence (SUI) is common among older multiparous females but rarely reported in active, young girls. OBJECTIVES: Our hypothesis is: physically active adolescent females develop pelvic floor laxity demonstrable on upright VCUG. Our objectives are to (1) increase awareness of SUI in young females, (2) test our hypothesis with an upright VCUG, and (3) report effectiveness of step-wise management. STUDY DESIGN: A retrospective review was performed of nulliparous girls with only SUI seen from 2000 to 2015, who were evaluated with upright voiding cystourethrography (VCUG) (bladder descent defined as ≥2 cm drop of bladder neck below pubic ramus at capacity). Data collection included level of physical activity, physical examination, BMI and Z-scores, urodynamics, management, and treatment response. Standard urotherapy (SUT) (timed voiding, proper diet, adequate fluids, bowel management) and biofeedback therapy (BFT) was initiated. Fisher exact test was used to calculate 'p' values. RESULTS: Thirty-three females (median age 15.1 years, range 5.5-20.3) were identified who underwent an upright VCUG; 20 had bladder neck descent (Fig.). Of these 20, 15 (75%) were involved in strenuous activity, whereas only three of 13 (23%) without descent engaged in intense athletics. No differences were noted in median BMI and Z-score with strenuous activity (21.1 (15.2-26.7) and 0.31 (-0.9-1.94)), respectively, versus patients without (21.3 (15.8-33.5) and 0.62 (-0.0-2.38)). Average follow-up for all was 16.6 months (range 0.4-102.2). Of 20 demonstrating bladder neck descent, three did not complete therapy and were lost to follow-up. Only six of these 17 became dry. Of the remaining 11, eight underwent surgery: Burch colposuspension (5), fascial sling (2), Coaptite to the bladder neck (1), and an artificial urinary sphincter (1). This latter girl had a failed Burch colposuspension 1 year previously. All surgical patients are dry. Of 13 without bladder descent on VCUG, five did not complete therapy and were lost to follow-up. The remaining eight were managed non-surgically; seven were fully dry at last follow-up. Overall, 13 of 25 (52%) achieved dryness. SUT and BFT were more effective in those without, than in those with bladder descent (87.5% vs. 35.3%, p = 0.0302, Fisher exact test). DISCUSSION AND CONCLUSIONS: Physically active, nulligravid girls with SUI can be efficaciously diagnosed on upright VCUG. They should be considered for non-surgical therapy but will likely require bladder neck elevating surgery. Non-surgical therapy works for those with minimal bladder descent on cystography.

Deletion of neuropilin 2 enhances detrusor contractility following bladder outlet obstruction.

Chronic urethral obstruction and the ensuing bladder wall remodeling can lead to diminished bladder smooth muscle (BSM) contractility and debilitating lower urinary tract symptoms. No effective pharmacotherapy exists to restore BSM contractile function. Neuropilin 2 (Nrp2) is a transmembrane protein that is highly expressed in BSM. Nrp2 deletion in mice leads to increased BSM contraction. We determined whether genetic ablation of Nrp2 could restore BSM contractility following obstruction. Partial bladder outlet obstruction (pBOO) was created by urethral occlusion in mice with either constitutive and ubiquitous, or inducible smooth muscle-specific deletion of Nrp2, and Nrp2-intact littermates. Mice without obstruction served as additional controls. Contractility was measured by isometric tension testing. Nrp2 deletion prior to pBOO increased force generation in BSM 4 weeks following surgery. Deletion of Nrp2 in mice already subjected to pBOO for 4 weeks showed increased contractility of tissues tested 6 weeks after surgery compared with nondeleted controls. Assessment of tissues from patients with urodynamically defined bladder outlet obstruction revealed reduced NRP2 levels in obstructed bladders with compensated compared with decompensated function, relative to asymptomatic controls. We conclude that downregulation of Nrp2 promotes BSM force generation. Neuropilin 2 may represent a novel target to restore contractility following obstruction.

An initial report of a novel multi-institutional bladder exstrophy consortium: a collaboration focused on primary surgery and subsequent care.

PURPOSE: For bladder exstrophy repair it is universally accepted that successful initial surgery is paramount to achieve the optimal outcome. Gaining the necessary surgical experience is challenging due to the rarity of bladder exstrophy. We report preliminary findings of a multi-institutional collaboration created to increase experience and proficiency with the care of bladder exstrophy. MATERIALS AND METHODS: Our 3 institutions alternatively served as the host site for scheduled surgeries with observation, commentary and critique by visitors from the other sites. The technique was complete primary repair with bilateral iliac osteotomy. The timing of complete primary repair at age 1 to 3 months facilitated collaboration. We recorded patient demographics and outcomes, and the impact of this collaboration on our technique and experience. Video recording was used for real-time observation and teaching, and future analysis, editing and review. RESULTS: A total of 16 site visits occurred from February 2013 through May 2014. Complete primary repair was performed in 9 males and 7 females with bladder exstrophy. Median age at complete primary repair was 2 months (range 0.1 to 28.8). Median followup was 8.9 months (range 2.8 to 18.2). All closures were successful with no dehiscence. Complications included urethrocutaneous fistula in 2 patients, 1 episode of pyelonephritis in 3 each and urethral obstruction in 2 females, of whom 1 required clean intermittent catheterization. CONCLUSIONS: We report a multi-institutional collaboration to standardize the surgical management of bladder exstrophy. This effort increased the annual experience of each institution involved from threefold to ninefold and it has accelerated the physician knowledge base to ultimately benefit patient care.

Where do we place the sacrocolpopexy stitch? A magnetic resonance imaging investigation.

OBJECTIVES: Sacrocolpopexy presacral sutures are placed at or slightly above the sacral promontory without knowledge of the location of intervertebral discs. We used magnetic resonance imaging to assess the anatomic relationship of the sacral promontory to intervertebral discs. METHODS: We reviewed spinal magnetic resonance images of women imaged at Loyola University Medical Center between January 2010 and February 2012. Sagittal T1 fluid-attenuated inversion recovery sequence images of the lumbosacral spine were used to identify the promontory as the most prominent point where S1 intersected with the superior anatomic structures. All measurements were obtained at the midline of the spinal cord. RESULTS: The mean age of 73 study subjects was 59 years (range, 22-89 years). The promontory was an intervertebral disc in many women (53 [73%]); the remaining images confirmed a nondisc promontory at the superior aspect of S1 in 20 patients (27%). The distance between the promontory and the next bony structure (L5) was 13 mm (25th-75th interquartile range, 11-16). In women without disc at the promontory, the median distance between the promontory and the base of L5 disc was 1.29 mm (interquartile range, 1.1-2.2). The mean height of the disc was 13.3 mm (4.4-20.6 mm). Age was not associated with the most prominent structure (P = 0.2), nor was it correlated to disc height (P = 0.27, r = 0.13) or distance to L5 (P = 0.75, r = 0.04). CONCLUSIONS: Given the high proportion of women with an intervertebral disc at the promontory, suture placement strategies that avoid this location may avoid-reduce disc-related sequelae after sacrocolpopexy.

Published surgical success rates in pediatric urology--fact or fiction?

PURPOSE: Parents counseled for surgery are quoted operative risks based on published results in the literature. However, outcomes from single surgeon or single institution retrospective studies are not generalizable. We assessed whether published outcomes were perceived to be representative of personal practice by pediatric urologists. We also correlated patterns of perceptions with surgical volumes and demographic variables. MATERIALS AND METHODS: A survey of 26 questions on 8 topics was e-mailed to 269 members of the American Academy of Pediatrics Section on Urology. Topics studied included distal and single stage proximal hypospadias, pyeloplasty, ureteral reimplantation with or without tapering, bladder neck reconstruction, and single and 2-stage bladder exstrophy repair. Participants were asked whether their rates of results were lower, the same or higher than specific published outcomes on a 5-point Likert scale. Study participants were also requested to provide demographic data and to estimate their annual case volume for each condition. RESULTS: Of the 269 section members who were contacted 110 (40.9%) completed the survey. More than 80% of respondents practice pediatric urology exclusively with 50.9% reporting operative volumes of more than 400 cases per year. A greater proportion of participants reported worse outcomes than published reports for hypospadias fistula rates (distal p = 0.001, proximal p = 0.023), bladder neck repair (p = 0.018) and exstrophy repair continence rates (single and 2-stage p <0.001). Improved outcomes compared to published data were reported for ureteral reimplantation (p = 0.013) and pyeloplasty (p = 0.003). However, these findings did not correlate consistently with case volume or other demographic characteristics. CONCLUSIONS: A significant proportion of pediatric urologists perceive their personal outcomes to be different than those in the published literature, regardless of practice setting, operative volume or time in practice. In an era of pay for performance and quality improvement, publication bias can have implications for patient care, reimbursement and malpractice.

Pediatric renal lymphangiectasia: importance of recognition and accurate renal imaging.

Renal lymphangiectasia is a rare diagnosis. It is also referred to as renal lymphangioma, renal lymphangiomatosis, peripelvic lymphangiectasia, renal peripelvic multicystic lymphangiectasia, and hygroma renale. The presentation varies, and the diagnosis depends on accurate radiologic interpretation. We present the radiologic images of a pediatric patient who was diagnosed with renal lymphangiectasia after being evaluated for suspected nonaccidental trauma to emphasize the importance of identifying this entity by the characteristic radiologic findings.

Suprapubic cystostomy: a bizarre complication of catheter migration causing ureteric obstruction.

Suprapubic catheters are used routinely for bladder drainage; however, complications are commonly reported. These complications could be attributed to surgical technique or the catheter itself. Complications related to surgical technique can be controlled and reduced, but catheter-related complications are unavoidable and unpredictable. We report a rare catheter-related complication leading to obstruction of the ureter in a solitary kidney in a patient with neurogenic bladder and voiding dysfunction managed with suprapubic catheter drainage.