Double Trouble: A Case Report of Caudal Duplication Syndrome.

Caudal duplication syndrome is a rare congenital anomaly with various duplications of structures derived from the embryonic cloaca and notochord. A male neonate was born with diphallia, bifid scrotum, and duplicated anorectal malformation. Diagnostic and operative evaluation identified a partially duplicated right kidney with left-to-right crossed fused ectopia, bilateral hydronephrosis, 2 separate hemi-bladders, left ectopic ureter with vesicoureteral reflux, and a left rectourethral fistula. To our knowledge, this is the first reported caudal duplication anomaly with complete genitourinary duplication, with diphallia, duplicated bladder, bifid scrotum, extra-numerary kidneys, and a duplicated rectum.

The conundrum of high-grade hydronephrosis with non-obstructive drainage on diuretic renography.

BACKGROUND: Patients with high grade hydronephrosis (HN) and non-obstructive drainage on mercaptoacetyltriglycine (MAG-3) diuretic renography (renal scans) can pose a dilemma for clinicians. Some patients may progress and require pyeloplasty; however, more clarity is needed on outcomes among these patients. OBJECTIVE: Our primary objective was to predict which patients with high-grade HN and non-obstructive renal scan, (defined as T ½ time <20 min) would experience resolution of HN. Our secondary objective was to determine predictors for surgical intervention. STUDY DESIGN: Patients with prenatally detected HN were prospectively enrolled from 7 centers from 2007 to 2022. Included patients had a renal scan with T ½<20 min and Society for Fetal Urology (SFU) grade 3 or 4 at last ultrasound (RBUS) prior to renal scan. Primary outcome was resolution of HN defined as SFU grade 1 and anterior posterior diameter of the renal pelvis (APD) < 10 mm on follow-up RBUS. Secondary outcome was pyeloplasty, comparing patients undergoing pyeloplasty with patients followed with serial imaging without resolution. Multivariable logistic regression was used for analysis. RESULTS: Of the total 2228 patients, 1311 had isolated HN, 338 patients had a renal scan and 129 met inclusion criteria. Median age at renal scan was 3.1 months, 77% were male and median follow-up was 35 months (IQR 20-49). We found that 22% (29/129) resolved, 42% of patients had pyeloplasty (54/129) and 36% had persistent HN that required follow-up (46/129). Univariate predictors of resolution were age≥3 months at time of renal scan (p = 0.05), T ½ time≤5 min (p = 0.09), SFU grade 3 (p = 0.0009), and APD<20 mm (p = 0.005). Upon multivariable analysis, SFU grade 3 (OR = 4.14, 95% CI: 1.30-13.4, p = 0.02) and APD<20 mm (OR = 6.62, 95% CI: 1.41-31.0, p = 0.02) were significant predictors of resolution. In the analysis of decision for pyeloplasty, SFU grade 4 (OR = 2.40, 95% CI: 1.01-5.71, p = 0.04) and T ½ time on subsequent renal scan of ≥20 min (OR = 5.14, 95% CI: 1.54-17.1, p = 0.008) were the significant predictors. CONCLUSIONS: Patients with high grade HN and reassuring renal scan can pose a significant challenge to clinical management. Our results help identify a specific candidate for observation with little risk for progression: the patient with SFU grade 3, APD under 20 mm, T ½ of 5 min or less who was 3 months or older at the time of renal scan. However, many patients may progress to surgery or do not fully resolve and require continued follow-up.

Cloacal Dysgenesis Sequence in a Preterm Neonate.

BACKGROUND Cloaca malformations result from a disproportion of apoptosis, cell growth, and maturation. The range of cloacal malformations is extensive and diverse, with a lack of a straightforward classification system. Cloacal dysgenesis sequence (CDS), also known as urorectal septum malformation sequence, is a rare cloaca variant described as the absence of a perineal orifice. Prenatal magnetic resonance imaging and antenatal ultrasounds may reveal a cloacal malformation; however, many patients are not diagnosed with cloacal malformation until birth. CASE REPORT We present a case of a female neonate delivered by a 23-year-old G2P1T1A0L0 mother who had received comprehensive prenatal care. During pregnancy, bilateral multicystic dysplastic kidneys were identified prenatally, leading to the in utero placement of a vesicoamniotic shunt. The physical exam revealed a distended abdomen with reduced abdominal musculature and laxity, ascites, a vesicoamniotic shunt in place, absent urethra, ambiguous genitalia with no vaginal opening, no perineal opening, and clubfoot. Abdominal radiograph showed findings consistent with significant abdominal ascites. An exploratory laparotomy was performed that included diverting colostomy, mucous fistula creation, tube vaginostomy, removal of the vesicoamniotic shunt, and suprapubic tube placement. The patient recovered well from this operation with no complications. CONCLUSIONS CDS is an uncommon condition in pediatric patients, and although sonographic findings can reveal urinary tract abnormalities, prenatal imaging might not always identify CDS. Our case underscores the uniqueness of the case and the significance of early detection and immediate medical and surgical intervention.

Echidna Splenule: A Case Study of Continuous Splenogonadal Fusion.

Splenogonadal fusion (SGF) is a rare congenital anomaly of an aberrant accessory spleen-gonad connection. We present a rare case of continuous splenogonadal fusion in a full-term male with a left undescended testis, multiple congenital limb anomalies, and syndromic facies. Diagnostic laparoscopy revealed the "Echidna Splenule," a snake-like intraperitoneal splenule coursing from the spleen along the left paracolic region and engulfing an atrophic intra-abdominal testis preventing spontaneous descent and distally herniating into the left open internal inguinal ring. The atrophic testis and Echidna Splenule were resected. Splenogonadal fusion should be considered in children with left undescended testis and concomitant limb and facial anomalies.

Endoscopic treatment of primary obstructive megaureter with high pressure balloon dilation in infants.

INTRODUCTION AND OBJECTIVE: Ureteral reimplantation of the dilated ureter in infants is challenging; however, some patients with primary obstructive megaureter (POM) in this age group require intervention due to clinical or radiological progression. We sought to determine if high pressure balloon dilation (HPBD) can serve as a definitive treatment for POM in children under one year of age, or as a temporizing measure until later reimplantation. MATERIALS AND METHODS: All patients from a single institution who underwent HPBD between October 2009 and May 2022 were retrospectively reviewed. Patients were excluded if older than 12 months or diagnosed with neurogenic bladder, posterior urethral valves, or obstructed refluxing megaureter. Patients with prior surgical intervention at the ureterovesical junction were excluded. Indications for surgery included progressive hydroureteronephrosis or urinary tract infection (UTI). Balloon dilation was performed via cystoscopy with fluoroscopic guidance, followed by placement of two temporary ureteral stents. Primary outcomes were improvement or resolution of megaureter and rates of subsequent reimplantation. Secondary outcomes included total number of anesthetics and postoperative UTIs. RESULTS: Fifteen infants with median age of 7.6 months (IQR 3.8-9.7) underwent HPBD. Twelve (80%) patients were detected prenatally and 3 (20%) after a UTI. Indication for surgery was progressive hydroureteronephrosis in 10 patients (67%) and UTI in five (33%). All had SFU grade 3 or 4 hydronephrosis on preoperative ultrasound and median distal ureteral diameter was 13 mm. Median follow up was 2.9 years. Twelve (80%) succeeded with endoscopic treatment: 7 patients had an undetectable distal ureter on ultrasound at last follow-up, 5 were improved with median distal ureteral diameter of 7 mm. Three patients (20%) required ureteral reimplantation due to progressive dilation, all with grade 4 hydronephrosis and distal ureteral diameters were 11, 15, and 21 mm. Six patients (40%) required two anesthetics to complete endoscopic treatment. Among those, 4 patients required initial stent placement for passive dilation followed by a second anesthetic for HPBD weeks later. Two patients underwent repeat HPBD following postoperative proximal migration of the ureteral stents. All 15 patients had an additional anesthetic for removal of stents. Five patients (33%) were treated for a symptomatic UTI (4 febrile, 1 afebrile) with the stents indwelling but there were no UTIs in the group following stent removal. CONCLUSION: Balloon dilation is a practical option for treatment of POM in infants, and in most cases (80%) avoids subsequent open surgery (over median 2.9 years of follow-up).

The double-face onlay-tube-onlay transverse preputial flap: An advantageous alternative to the two-stage hypospadias repair?

OBJECTIVE: To compare the surgical outcomes and complications of boys who underwent double-face onlay-tube-onlay transverse preputial island flap (DFOTO) one-stage repair vs. two-stage repair for proximal hypospadias. STUDY DESIGN: Males with proximal hypospadias who underwent DFOTO or two-stage repair at a single institution from 2008 to 2021 were identified. Patients who had prior hypospadias surgery were excluded. Outcomes were surgical complications, number of surgical procedures, operative time, and post-operative uroflowmetry results. RESULTS: Fifty-three males who underwent DFOTO and 39 who underwent two-stage repair were included. Median age at surgery was 1.1 years (IQR 0.83-1.6) and median follow-up was 3.0 years (IQR 1.2-6.8). Although not statistically significant, the DFOTO group had higher rates of urethrocutaneous fistula (30% vs. 15%, p = 0.10), urethral stricture (15% vs. 3%, p = 0.07) and urethral diverticulum (8% vs. 3%, p = 0.39). Although the unplanned re-operation rate was higher in DFOTO (58% vs. 33%, p = 0.02), the mean number of procedures and median total surgical time were lower in DFOTO (1.8 ± 0.9 vs. 2.4 ± 0.8, p = 0.0004; 337 min [IQR 278-460] vs. 468 min [IQR 400-563], p = 0.008). There were no significant differences between groups for mean peak flow rates and post void residuals. CONCLUSIONS: In males who underwent DFOTO, 42% achieved completion of their proximal hypospadias repair with one operation, while the remainder had largely minor complications. Accounting for reoperation rates, the mean number of procedures per patient was lower in the DFOTO group. Comparable results can be achieved with both techniques; the risks of higher unplanned operation rates in the DFOTO group should be considered with the benefit of fewer total procedures.

The Pseudo-Bladder: A Case of the Prostatic Urethra Masquerading as the Bladder.

Congenital bladder underdevelopment has detrimental genitourinary effects. We present a male infant with declining renal function, recurrent urinary tract infections, and epididymo-orchitis, in the setting of bilateral high grade hydronephrosis and vesicoureteral reflux. Cystoscopic evaluation revealed the presumed "bladder" to be a massively dilated prostatic urethra. The small dysplastic "true bladder" was found at the anterior surface of the prostatic urethra with massively dilated ureteral orifices. Temporizing diversion was performed in the form of bilateral cutaneous ureterostomies, with an ultimate plan for reconstructive bladder surgery. This case presents diagnostic challenges utilizing current modalities due to a massively dilated prostatic urethra in the rare case of bladder dysplasia.

The secret life of baby turtles: A novel system to predict hatchling emergence, detect infertile nests, and remotely monitor sea turtle nest events.

Current understanding of sea turtle nesting, hatching, and emergence events has been largely limited to observable events on the surface of the sand, though recent approaches using audio or visual equipment have allowed scientists to better understand some underground nest phenomena. We used a technology-based approach to define motion-related Caretta caretta hatching and emergence nest events. We describe a novel low-cost, accelerometer-based system called TurtleSense that can detect movement and temperature within sea turtle nests remotely. TurtleSense is successfully able to specifically detect motion within sea turtle nests over the entire course of incubation. This system allows for the identification of infertile nests and the detection of four predictable sequential developmental activity patterns in viable nests, including a hatch and posthatch period, the timing of which can be used to tightly predict hatchling emergence events almost to the day. TurtleSense provides a much better understanding about what is happening in the nest before emergence and allows for the generation of a theory of the mechanism that triggers mass emergence. Our results suggest that motion plays a large role in hatchling communication and that the timing of emergence events may be related to the cessation of movement within the nest. Current management of sea turtle nesting events is primarily driven by counting the number of days since the nest was laid, with further safeguards placed at the nest upon subsequent visual observation of depression or emergence events. Use of TurtleSense technology can impact nest management and conservation efforts, allowing organizations to use this motion data to more tightly predict emergence dates for sea turtle hatchlings and to use viability data to inform nest management decisions.

Home bladder pressure measurements correlate with urodynamic storage pressures and high-grade hydronephrosis in children with spina bifida.

BACKGROUND: Patients with spina bifida are at risk for developing bladder and renal deterioration secondary to increased bladder storage pressures. OBJECTIVES: To determine the association of home bladder volume and pressure measurements (home manometry) to: 1) detrusor storage pressures on urodynamics (UDS); and 2) the presence of Society of Fetal Urology (SFU) grades 3-4 hydronephrosis on renal bladder ultrasound in patients with spina bifida. METHODS: Data were prospectively collected on patients with spina bifida and neurogenic bladder requiring clean intermittent catheterization. Patients used a ruler and typical catheterization equipment to measure bladder pressures and volumes at home. Home measurements were compared to UDS detrusor pressures and SFU hydronephrosis grade. Detrusor pressure <20 cm H2O at 50% maximal cystometric capacity (MCC) on UDS was used as a measure of safe storage pressures on UDS; conversely, detrusor pressure >20 cm H2O was used a measure to capture both unsafe storage pressures and those with potential for unsafe storage pressures. Receiver-operator characteristic curves and area under curve (AUC) were calculated to depict the association between home manometry variables with detrusor pressures on UDS and SFU grades 3-4 hydronephrosis. RESULTS: Included were 52 patients with a median age of 10.3 years (interquartile range 6.3-14.4 years). Three home manometry measurements (maximum bladder pressure, bladder pressure at maximum catheterized volume, and mean bladder pressure) > 20 cm H2O were sensitive for Pdet >20 cm H2O at 50% MCC. Maximal bladder pressure >20 cm H2O was the most sensitive among home manometry measures (sensitivity 100%, specificity 70%, AUC 0.92 for Pdet >20 cm H2O at 50% MCC on UDS; sensitivity 100%, specificity 62%, AUC 0.89 for SFU grade 3-4 hydronephrosis). None of the patients who had maximum home bladder pressure <20 cm H2O had SFU grades 3-4 hydronephrosis; conversely, individuals with maximal home bladder pressure >20 cm had a wide range of hydronephrosis grades. CONCLUSION: None of the patients with maximal home bladder pressure <20 cm H2O had grade 3-4 hydronephrosis. Home measurements of maximal bladder pressure, bladder pressure at maximum catheterized volume and mean bladder pressure of >20 cm H2O were all sensitive for Pdet >20 cm H2O at 50% MCC on UDS. Home manometry is an inexpensive and simple technique to identify patients at risk for and to monitor individuals at high risk of upper tract dilation, without incurring significant cost or morbidity.