Vesicostomy is a temporary solution for patients with bladder outlet challenger who are not eligible for catheterization. A 73 year-old man who has successfully managed his bladder using a vesicostomy for 59 years. Complications of prolapse and dermatitis led to vesicostomy closure and cystostomy placement at age 73. Vesicostomy can be safely used to preserve bladder and upper urinary tract health for a lifetime.

UNASSIGNED: Lower urinary tract dysfunction (LUTD) in cerebral palsy (CP) and other neuromuscular diseases can present with chronic retention that leads to hydronephrosis, recurrent urinary tract infections (UTI), and stone formation. Whenever the conservative treatment of LUTD fails for any reason, it is considered to be complicated LUTD, in which a surgical approach is warranted. Cutaneous vesicostomy (CV) is a simple, well-tolerated, and potentially reversible procedure that protects the upper tracts. We describe our experience using CV for this complex population.
UNASSIGNED: Children with CP and other neuromuscular diseases admitted to pediatric long-term care units for palliative care between 2015 and 2019 were included in the study. They present multi-system involvement, polypharmacy, and Gross Motor Function Classification System levels of 4 or 5. We retrospectively studied this population\'s indications and results of CV.
UNASSIGNED: Of the 52 admitted patients, 18 presented LUTD with UTI (n:18; 100%), stones (n:5; 28%), progressive hydroureteronephrosis (n:3; 17%), or stones (n:2; 11%). Conservative initial management (catheterizations, prophylaxis antibiotics) was effective in half the cases. The remaining nine were defined as complicated LUTD and underwent CV. After a mean follow-up of 11.3 months, the follow-up showed improved hydronephrosis in all nine (100%) patients. Recurrent UTIs were no longer seen in eight of nine patients, although three patients required bladder irrigations; bladder stones did not recur after CV; the kidney stones needed further intervention. Revision of the CV was required in two (11%) cases at 12 and 24 months postoperatively due to stoma stenosis.
UNASSIGNED: CV is a relatively simple and effective procedure representing a pragmatic solution for managing complicated LUTD in complex long-term institutionalized pediatric palliative care patients with neuropathic bladders.

BACKGROUND: Multiple factors impact ability to achieve urinary continence in cloacal malformation including common channel (CC) and urethral length and presence of spinal cord abnormalities. Few publications describe continence rates and bladder management in this population. We evaluated our cohort of patients with cloacal malformation to describe the bladder management and continence outcomes.
METHODS: We reviewed a prospectively collected database of patients with cloacal malformation managed at our institution. We included girls ≥3 years (y) of age and evaluated their bladder management methods and continence. Dryness was defined as <1 daytime accident per week. Incontinent diversions with both vesicostomy and enterovesicostomy were considered wet.
RESULTS: A total of 152 patients were included. Overall, 93 (61.2%) are dry. Nearly half (47%) voided via urethra, 65% of whom were dry. Twenty patients (13.1%) had incontinent diversions. Over 40% of the cohort performed clean intermittent catheterization (CIC), approximately half via urethra and half via abdominal channel. Over 80% of those performing CIC were dry. In total, 12.5% (n = 19) required bladder augmentation (BA). CC length was not associated with dryness (p = 0.076), need for CIC (p = 0.253), or need for abdominal channel (p = 0.497). The presence of a spinal cord abnormality was associated with need for CIC (p = 0.0117) and normal spine associated with ability to void and be dry (p = 0.004) CONCLUSIONS: In girls ≥ 3 y of age with cloacal malformation, 61.2% are dry, 65% by voiding via urethra and 82% with CIC. 12.5% require BA. Further investigation is needed to determine anatomic findings associated with urinary outcomes.
METHODS: IV.

BACKGROUND: Urinary drainage for posterior urethral valves can be achieved with valve ablation (VA) or diversion by vesicostomy (VES) or cutaneous ureterostomy (CU). The effect of these interventions on long-term bladder function remains debated, and voiding symptomatology after VES or CU reversal has been poorly characterized.
OBJECTIVE: The objective of this study was to examine the prevalence and scope of physician treatment patterns as a surrogate for retention or incontinence symptomatology among PUV patients undergoing primary VA or diversion by VES/CU and determine rates of progression to augmentation.
METHODS: This is a single-institution retrospective cohort study. Retention Scores (R) were calculated 1 point for: retention behavior (double/timed void), alpha-blocker, intermittent catheterization, or overnight indwelling catheter. Incontinence Scores (I) were calculated 1 point for: incontinence behavior (double/timed void), oral medication, or botulinum toxin. Patients with R score above 3 or I score above 2 were deemed to have severe retention or incontinence symptomatology respectively. End stage bladder (ESB) was defined as need for bladder augmentation.
RESULTS: We identified 76 patients between 5 and 40 years old with median follow-up of 14.6 [5.0-40.4) years. There was no difference in the rates of severe retention or incontinence treatment pattern scoring between VA versus VES/CU (Figure). Rates of achieving R(1) status are similar between VA and VES/CU groups, though age of reaching R(1) was younger for those with VES/CU (4.8 years) compared to VA (6.6 years). There was no significant difference in rate of ESB by intervention category VA (9.4%) versus VES/CU (17.4%; p = 0.323).
CONCLUSIONS: Treatment of retention symptomatology was more common than treatment of incontinence symptomatology regardless of primary management, VA or VES/CU. This study also indicates that VES/CU patients were just as responsive as VA patients to conservative treatments (behavioral changes, pharmacotherapy) for any type of bladder symptomatology as the progression to treatment of severe symptomatology and ESB were similar between cohorts. In this cohort, bladder outcomes were not associated with type of urinary diversion (VA or VES/CU).
CONCLUSIONS: Long term bladder outcomes for valve patients demonstrated similar treatment patterns and progression to end-stage bladder regardless of diversion status. Patients went on to ESB approximately 4.4 years after diagnosis at similar rates between groups.

BACKGROUND: Aphallia is a rare congenital anomaly often associated with other urogenital anomalies. The management of aphallia cases for both the immediate and long-term treatment of patients with aphallia pose a major dilemma. Patients are at risk for psychosocial and psychosexual challenges throughout life.
METHODS: A systematic review was conducted on aphallia cases. We searched online databases until March 2023 for relevant articles and performed according to the PRISMA-P guidelines.
RESULTS: Of the 43 articles screened, there were 33 articles included. A total of 41 patients were analyzed qualitatively. Asia is the region with the most aphallia cases with 53% (n:22), while the United States is the country with the most most reported aphallia cases 31% (n:13). Most cases were identified as male sex (n: 40), and most cases were neonate with 68% (n:28) cases. Physical examination generally found 85% (N = 35) with normal scrotal development and palpable testes. The most affected system with anomalies is the genitourinary system with fistulas in 80% (n:29) cases. Initial management in 39% (n:16) of patients involved vesicostomy. Further management of 31% (n:13) included phalloplasty or penile reconstruction, and 12% (n:5) chose female sex. 17% (n:7) of patients refused medical treatment or were lost to follow-up, and 12% (n = 5) patients deceased.
CONCLUSIONS: Aphallia is a rare condition and is often associated with other inherited genitourinary disorders. In most cases, physical examinations are normal except for the absence of a phallus, and laboratory testing shows normal results. The initial management typically involves the vesicostomy procedure. Subsequent management focuses on gender determination. Currently, male sex is preferred over female. Due to the significant variability, the rarity of cases, and the lack of long-term effect reporting in many studies on aphallia, further research is needed to minimize bias.

Resolution of underlying urinary tract anomalies prior to kidney transplantation in patients with end stage renal disease (ESRD) secondary to uropathy, has been historically supported under the argument that this would help prevent infectious complications and graft loss. We propose to perform earlier kidney transplantation with a transient vesicostomy, deferring resolution of the uropathy to the post-transplantation period. The aim of this study was to evaluate the outcomes of kidney transplantation in children with a vesicostomy.
A retrospective, multicenter study was performed including all patients under 18 years of age who underwent kidney transplantation with a vesicostomy, between January 2005 and December 2020 and had at least one year of follow up. Data related with the indication and timing of vesicostomy, time until transplantation, post-transplantation complications, urinary tract infections (UTI) and graft survival rate were collected.
Of the 758 transplantations performed in the study period, 16 patients met the inclusion criteria. Mean age at transplantation was 58 months (range 20-151), and mean weight was 13.5 Kg (range 8.4-20). Mean time from vesicostomy to kidney transplantation was 30 months (range 0-70). There were 2 (12.5%) ureteral complications that required reoperation. Eighteen episodes of UTI were identified in 8 patients (50%), accounting for 0.4 UTIs per patient-year of follow-up. UTIs did not lead to graft loss in any of the cases. Urinary tract reconstruction was performed in 5 patients (31.3%) at an interval of 1-91 months post-transplantation. After a mean follow-up of 44.8 months (range 13-200) from transplantation, patients with vesicostomy had a mean creatinine clearance of 86.6 ml/min/1.73 m2, with a mean serum creatinine level of 0.6 mg/dl. Graft survival rate was 100%.
Early kidney transplantation into a vesicostomy permits a resolution of the ESRD, avoiding deleterious effects related to dialysis. With a low rate of UTIs, we found no graft loss due to infectious complications. This strategy permits careful planning and better timing for the urinary tract reconstruction without delaying kidney transplantation.
Kidney transplantation in pediatric patients with vesicostomy seems to be a safe and effective strategy. UTI rate was similar to that reported in the literature of patients with corrected urinary anomalies undergoing kidney transplantation without urinary diversion.

UNASSIGNED: There are conflicting reports for the management of severe posterior urethral valve (PUV) after ablation. The primary objective was to assess the renal outcomes using the estimated glomerular filtration rate (eGFR) and secondary outcomes in severe PUVs who underwent early partially diverting reduction ureterostomy (PDRU) and a delayed undiversion protocol.
UNASSIGNED: This 10-year retrospective study reviewed the records of 1094 boys with PUV, where severe PUV cases were treated with early PDRU (324 surgeries). We then analyzed those patients who completed the early diversion and delayed undiversion protocol. The long-term renal outcomes using eGFR and antero-posterior diameter (APD) were compared at various time points using appropriate statistical methods.
UNASSIGNED: Of the 171 severe PUV patients who underwent PDRU, 31 completed undiversion and 26 (47 renal units) were analyzed after exclusions. The mean age (standard deviation) at presentation was 1.46 ± 4.1 months. Thirty-two units were refluxing and 15 were nonrefluxing megaureters. PDRU was closed at a mean age of 3.9 years and had a mean duration of follow-up of 6.4 years. The mean eGFR increased from a minimum of 10.78 ± 10.25 at baseline and remained stable at 28.69 ± 18.89 after closure of both stoma. Similarly, mean APD decreased from 12.07 ± 6.79 at the diagnosis to 7.00 ± 6.20. Three patients (3 renal units) required revision of the stoma for stenosis and 1 patient had a parastomal hernia that was repaired at the time of undiversion.
UNASSIGNED: In severe PUVs, early PDRU with delayed undiversion is a reliable surgical option that may ensure better renal outcomes in the long-term. Nonrefluxing renal units recover better than the refluxing. APD measurements also are shown to improve favorably.

Cloaca is a rare, complex malformation encompassing the genitourinary and anorectal tract of the female in which these tracts fail to separate in utero, resulting in a single perineal orifice. Prenatal sonography detects a few cases with findings such as renal and urinary tract malformations, intraluminal calcifications, dilated bowel, ambiguous genitalia, a cystic pelvic mass, or identification of other associated anomalies prompting further imaging. Multi-disciplinary collaboration between neonatology, pediatric surgery, urology, and gynecology is paramount to achieving safe outcomes. Perinatal evaluation and management may include treatment of cardiopulmonary and renal anomalies, administration of prophylactic antibiotics, ensuring egress of urine and evaluation of hydronephrosis, drainage of a hydrocolpos, and creation of a colostomy for stool diversion. Additional imaging of the spinal cord and sacrum are obtained to plan possible neurosurgical intervention as well as prognostication of future bladder and bowel control. Endoscopic evaluation and cloacagram, followed by primary reconstruction, are performed by a multidisciplinary team outside of the neonatal period. Long-term multidisciplinary follow-up is essential given the increased rates of renal disease, neuropathic bladder, tethered cord syndrome, and stooling issues. Patients and families will also require support through the functional and psychosocial changes in puberty, adolescence, and young adulthood.

In boys with posterior urethral valves (PUVs) the main treatment aim is to preserve long-term bladder and renal function. To determine the effectiveness of secondary vesicostomy in boys with PUVs, the medical records of 21 patients with PUV (2010-2019), divided into two groups (group I: valve ablation; group II: secondary vesicostomy), were reviewed regarding the course of serum creatinine, renal ultrasound, voiding cystourethrogram, urodynamics, postoperative complications, need of further surgery, and long-term solution. The median age of all patients at first follow-up was 11 (9-13) months and at last follow-up 64.5 (39.5-102.5) months. Despite a significant difference of the SWDR score (shape, wall, reflux, and diverticula) (p = 0.014), both groups showed no significant differences preoperatively. Postoperatively, serum creatinine (p = 0.024), grade of vesicoureteral reflux (p = 0.003), side of upper tract dilatation (p = 0.006), side of megaureter (p = 0.004), and SWDR score (p = 0.002) were significantly decreased in group II. Postoperative urodynamic measurements showed comparable results in both groups. Stoma complications were found in three (20%) patients (group II). Eight (53.3%) patients already received a closure of the vesicostomy. Seven out of eight (87.5%) patients were able to micturate spontaneously. Vesicostomy remains a reliable treatment option for boys with PUV to improve bladder function and avoid further damage to the urinary tract.

Persistent cloaca involves fusion of the bladder, vagina, and rectum into a single duct called the common duct. Although its pathogenesis remains unclear, it has been associated with hyperchloremic metabolic acidosis. Herein, we present the case of a neonatal girl with high-confluence type variant of persistent cloaca treated with vesicostomy (Blocksom) for refractory metabolic acidosis. She was diagnosed with persistent cloaca before birth; colostomy was performed and a urinary catheter was placed in the bladder. Voiding cystourethrography on day 19 after birth showed that most of the contrast material leaked into the rectum; hence, the urinary catheter was removed. On day 27, hyperchloremic metabolic acidosis was detected and treatment with oral sodium bicarbonate was initiated; however, the infant showed no response. Because hyperchloremia occurred after removal of the urinary catheter, continuous urine retention in the colon through the common duct was believed to have caused the progression of hyperchloremic metabolic acidosis through transporters in the intestinal mucosa. As reinstallation of a urinary catheter was technically difficult, vesicostomy was performed on day 29, after which the metabolic acidosis improved. This report suggests vesicostomy as an effective treatment for refractory hyperchloremic metabolic acidosis associated with high-confluence type persistent cloaca.