Long-term safety and efficacy of psoas bladder hitch in infants aged <12 months with unilateral obstructive megaureter.

OBJECTIVE: To determine the long-term safety and efficacy of ureteric reimplantation with psoas bladder hitch (PBH) in patients aged <12 months with unilateral obstructive megaureter (OM). PATIENTS AND METHODS: We retrospectively compared a group of patients aged <12 months (study group) with an group of patients aged ≥12 months (comparison group), who underwent PBH for OM between September 2007 and April 2017, in terms of preoperative patient characteristics, intra- and peri-operative results, and postoperative results. RESULTS: The study group comprised seven infants, five boys and two girls. The median (range) age at the time of PBH was 3 (2-8) months; OM was detected by ultrasonography during the fetal period. The left side was affected in four infants and the right side in three. Four infants had primary OM (POM). In all three infants who had ectopic OM in the complete double renal pelvis and ureter, the OM involved the ureter from the upper half of the kidney. The median (range) follow-up period after PBH was 45 (33-129) months. Comparison of the two groups showed no significant difference in terms of surgical time (P = 0.948) and length of hospital stay (P = 0.125). In both groups, hydroureteronephrosis improved postoperatively in all patients. There was no significant difference between the two groups in terms of postoperative complications, such as vesico-ureteric reflux, febrile urinary tract infection and deterioration of ipsilateral renal function. Notably, no patient underwent reoperation in either group. CONCLUSION: Psoas bladder hitch for unilateral OM including POM appears to be safe and effective in the long term for patients aged <12 months and for those aged ≥12 months. Although not routinely recommended, PBH appears to be a viable option for selected infants with POM.

The discrepancy between the uroflowmetry parameters, curve patterns, and lower urinary tract symptoms of children with nocturnal enuresis according to the presence or absence of daytime urinary incontinence.

AIM: To evaluate the uroflowmetry (UFM), which reflects the urinary symptoms of children with nocturnal enuresis (NE). METHODS: This study retrospectively investigated 93 children who underwent UFM three times on the same day (≥8 years old; mean age: 9 years and 10 months; 66 boys and 27 girls). Children with the combination of daytime incontinence (DI) and NE (DI+NE group: n = 45) and NE alone (isolated NE group: n = 48) were compared. UFM curve patterns were classified into five types: bell (B), tower (T), staccato (S), plateau (P), and interrupted (I). An unclassified (U) curve pattern was designated when all three curve patterns differed. Patterns other than the B pattern were considered abnormal (A). Each score in the dysfunctional voiding symptom score (DVSS) was also compared between the two groups. RESULTS: The UFM curve pattern was B (n = 21), T (n = 15), S (n = 4), P (n = 1), U (n = 4) in the DI+NE group and B (n = 25), T (n = 10), S (n = 7), P (n = 2), U (n = 4) in the isolated NE group, indicating no significant difference (B vs A; P = .680). The two items of DVSS questionnaire were significantly greater in the DI+NE group than in the isolated NE group (voiding postponement: 0.93 ± 1.18 vs 0.23 ± 0.56, P = .001, urgency: 2.09 ± 1.06 vs 0.56 ± 0.85, P < .001). CONCLUSION: The abnormal UFM curve patterns did not differ significantly between NE children with and without DI. However, the DVSS questionnaire scores differed significantly. UFM did not reflect the urinary symptoms of children with NE.

Prevention of recurrent febrile urinary tract infection in infants: Ultrasonography-oriented approach is more practical than a top-down approach.

BACKGROUND: We previously reported that the top-down approach (TDA) for infants with febrile urinary tract infections (fUTI) could prevent recurrent fUTI (r-fUTI) but produced a high number of false-positives on acute-phase 99m Tc dimercaptosuccinic acid (DMSA) renal scintigraphy. Therefore we compared the ultrasonography-oriented approach (USOA) with TDA from the viewpoint of prevention of r-fUTI. METHODS: The TDA was applied between July 2010 and February 2014 and the USOA was applied between March 2014 and April 2017 in infants with first fUTI. In the USOA group, voiding cystourethrography (VCUG) was performed in the case of abnormality on acute-phase renal bladder ultrasonography (RBUS) or on chronic- phase DMSA, which were performed in all cases. The frequency of r-fUTI was compared between the TDA group and USOA group retrospectively. RESULTS: Seventy-four infants (52 male) and 79 infants (60 male) received TDA or USOA, respectively. No significant differences were found between the TDA and USOA groups in male : female ratio, age in months at initial onset of fUTI, observation period, or number of cases of r-fUTI (TDA group, n = 4; USOA group, n = 5). Seventy-four DMSA scintigraphy and 25 VCUG were carried out in the USOA group, and 111 DMSA scintigraphy and 34 VCUG in the TDA group. CONCLUSIONS: Both USOA and TDA were valid for prevention of r-fUTI, but USOA was superior to TDA with regard to the reduced number of patients undergoing VCUG and DMSA.

The changes of urethral morphology recognized in voiding cystourethrography after endoscopic transurethral incision for posterior urethral valve in boys with intractable daytime urinary incontinence and nocturnal enuresis.

PURPOSE: Endoscopic transurethral incision (TUI) of posterior urethral valve (PUV) can improve daytime urinary incontinence (DUI) and nocturnal enuresis (NE). However, the underlying mechanism has not been elucidated. In this study, we retrospectively examined the mobility of the urethra before and after TUI by measuring the urethral angle with voiding cystourethrography (VCUG), to clarify the effects of TUI on the morphology of the urethra during voiding. METHODS: Between July 2010 and December 2014, 29 boys with intractable DUI and/or NE were diagnosed as PUV and underwent endoscopic TUI. VCUG during voiding phase was performed at sequential radiographic spot images (1 image per second) at a 45° angle in oblique standing position. The point at which the angle of the urethra was the smallest during urination was regarded as the minimum urethral angle. The maximum urethral angle during early voiding phase was compared with the minimum urethral angle, and the percentage by which this angle changed was calculated as the flexion rate. Then changes in minimum urethral angle and flexion rate were analyzed before and 3-4 months after TUI. RESULTS: After TUI, the minimum urethral angle on VCUG became more obtuse (before vs. after TUI, respectively: 112.7 vs. 124.5°, p < 0.001), the flexion rate decreased (before vs. after TUI, respectively: 11.8 vs. 4.1%, p < 0.001). CONCLUSIONS: This study demonstrated a significant difference in the degree of change. The findings may contribute to understanding of the mechanism of improvement in symptoms after TUI in patients with PUV.

Top-down approach is possible strategy for predicting breakthrough fUTIs and renal scars in infants.

BACKGROUND: Acute-phase technetium-99 m dimercaptosuccinic acid (DMSA) scintigraphy is recommended for initial imaging in children with febrile urinary tract infection (fUTI). Recently, the importance of identifying patients at risk of recurrent fUTI (r-fUTI) has been emphasized. To clarify the effectiveness of DMSA scintigraphy for predicting r-fUTI in infants, we investigated the relationship between defects on DMSA scintigraphy and r-fUTI. METHODS: Seventy-nine consecutive infants (male: female, 60:19) with fUTI were enrolled in this study. DMSA scintigraphy was performed in the acute phase, and patients with defect underwent voiding cystourethrography and chronic-phase (6 months later) DMSA scintigraphy. Patients were followed on continuous antibiotic prophylaxis (CAP). RESULTS: Defects on acute-phase DMSA scintigraphy were observed in 32 children (40.5%) of 79. The mean follow-up observation period was 17.0 ± 10.1 months. Four patients had r-fUTI (5%). Two of them had defects on DMSA scintigraphy in both the acute phase and chronic phase, and had bilateral vesicoureteral reflux (VUR) grade IV. Two others had r-fUTI without defects on DMSA and did not have VUR. Twelve patients had defect on chronic-phase DMSA scintigraphy and four of them had no VUR. CONCLUSIONS: The top-down approach is a possible method for predicting r-fUTI in infants and does not miss clinically significant VUR. Also, given that the prevalence of r-fUTI was 5% regardless of the presence of defects on acute-phase DMSA, then, in conjunction with genital hygiene and CAP, acute-phase DMSA might be unnecessary if chronic-phase DMSA is performed for all patients to detect renal scar.

Long-Term Outcome of Low Scrotal Approach Orchiopexy without Ligation of the Processus Vaginalis.

PURPOSE: We performed low scrotal approach orchiopexy in patients with prescrotal cryptorchidism. The processus vaginalis was not ligated if it was not widely patent. We retrospectively evaluated the long-term outcomes of low scrotal approach orchiopexy without processus vaginalis ligation. MATERIALS AND METHODS: A total of 137 patients (227 testes) were diagnosed with prescrotal cryptorchidism between October 2009 and April 2014. All patients underwent low scrotal approach orchiopexy. Mean age at surgery was 34.9 months. The processus vaginalis was deemed to be not widely patent when a sound could not be passed into the abdominal cavity through the internal inguinal ring, and the processus vaginalis was not ligated in such cases. RESULTS: Intraoperative findings revealed that the processus vaginalis was widely patent in 10 testes and was not widely patent in 217. A widely patent processus vaginalis was closed via scrotal approach in 5 testes, while an inguinal approach was necessary in 5. Median followup was 44 months (range 20 to 73). Postoperative complications included reascending testis in 1 case where an inguinal approach was necessary. No patient manifested testicular atrophy or inguinal hernia. CONCLUSIONS: Low scrotal approach orchiopexy is a useful and safe procedure for treating patients with prescrotal cryptorchidism. Ligation is unnecessary when the processus vaginalis is not widely patent.

Long-Term Outcome of the Pippi Salle Procedure for Intractable Urinary Incontinence in Patients with Severe Intrinsic Urethral Sphincter Deficiency.

PURPOSE: We evaluated the long-term outcome of the Pippi Salle procedure in patients with severe intrinsic urethral sphincter deficiency. MATERIALS AND METHODS: We performed the Pippi Salle procedure in 6 males and 6 females with severe intrinsic sphincter deficiency between March 2003 and August 2013. Median patient age was 15 years (range 6 to 45). Mean followup was 75 months (range 17 to 142). Six males and 3 females had neurogenic intrinsic sphincter deficiency (spina bifida in 8 and spinal cord injury in 1). Three females had anatomical intrinsic sphincter deficiency (idiopathic bladder hypoplasia in 2 and pseudo-ureterocele in 1). Four patients had previously undergone bladder neck surgery, 3 had been treated with endoscopic injection of collagen, 2 had undergone fascial sling and 1 had been treated with tension-free vaginal tape surgery. The Pippi Salle procedure was performed alone (2 patients), or in combination with bladder augmentation (4) or catheterizable abdominal stoma (1), or both (5). RESULTS: Complete dryness was achieved in 7 patients (58%). Of 9 patients with neurogenic intrinsic sphincter deficiency 7 (78%) achieved complete dryness. Eight patients experienced complications, including continued urinary incontinence (5), difficulty catheterizing per urethra (3) and urinary calculi (1). These 8 patients were successfully treated with additional endoscopic interventions, including injection of collagen in 4, injection of dextranomer-hyaluronic acid in 1, transurethral incision of urethral kink in 3 and vesicolithotripsy in 1. After these simple interventions complete dryness was achieved in all 12 patients. CONCLUSIONS: Although we experienced some minor complications in the short term, most patients were simply and successfully treated with endoscopic surgery. The long-term results of the Pippi Salle procedure are promising.

Labial adhesion causing voiding but not sexual problems in a married woman.

A married woman of reproductive age had labial adhesion with voiding difficulty. She and her husband had not been bothered by their inability to engage in sexual intercourse for the 10 years of their marriage. Surgical incision and reconstruction disclosed the normal vaginal vestibule and urethral meatus. Six months after surgery, her labium was fully open without recurrence. We must be aware that labial adhesion may occur and be hidden in a woman of reproductive age, even when the patient does not notice any 'abnormality' in her genitalia.

Retroperitoneoscopy-Assisted Dismembered Pyeloplasty With Single-Site Plus One Port in Older Children With Congenital Hydronephrosis.

INTRODUCTION: This study aimed to clarify the advantage of retroperitoneoscopy-assisted dismembered pyeloplasty with single-site plus one port (RPSPO) for pediatric congenital hydronephrosis in patients aged ≥7 years. METHODS: We retrospectively compared a group of patients aged ≥7 years (study group) with patients aged <7 years (comparison group), who underwent RPSPO between August 2015 and August 2018, in terms of preoperative patient characteristics, intra- and perioperative results, and postoperative results. RESULTS: The study group consisted of eight patients. The median body weight at surgery was 27 kg (20-38 kg). The median age at surgery was 102.5 m (87-139 m). The severity of hydronephrosis on the affected side graded by the Society for Fetal Urology grade was grade 3 in one case and grade 2 or less in all other cases. All patients underwent a 99m Tc-MAG3 renogram. Comparison between the two groups showed significant differences in body weight at surgery (p = 0.003), age (p < 0.001), and preoperative hydronephrosis grade (p = 0.007), but the median length of the skin incision was 20 mm in both groups, with no significant difference (p = 1.000). Redo pyeloplasty was not required in any patient in either group. CONCLUSION: RPSPO is an advantageous procedure for older children because it allows precise ureteropelvic neoanastomosis under direct vision and the same wound size as in younger children.

Transurethral incision of congenital obstructive lesions in the posterior urethra in boys and its effect on urinary incontinence and urodynamic study.

UNLABELLED: Study Type--Therapy (case series) Level of Evidence 4. What's known on the subject? and What does the study add? We have clarified that there exist two types of voiding urodynamics (pressure-flow-study) for congenital urethral obstruction in boys; one is synergic pattern (SP) and the other is dyssynergic pattern (DP). In terms of daytime incontinence and nocturnal enuresis, the transurethral endoscopic incision of these obstructive lesions is only effective in the SP type, while never effective in the DP type. The synergic pattern (SP) seems to represent simple anatomical obstruction, while the dyssynergic pattern (DP) may represent anatomical obstruction complicated with functional obstruction. The efficacy of endoscopic incision to mild forms of congenital urethral obstruction has been controversial, especially in terms of nocturnal enuresis. One of the reasons for the controversy is due to the lack of pre-and post-operative urodynamic assessment with its linkage to symptomatic change. We have, for the first time in the world, systematically conducted voiding urodynamic study for those elusive lesions seen in enuretic boys. Conclusively, for simple mechanical obstruction (SP), we confirmed that some voiding urodynamic parameters improve after the endoscopic incision, parallel to symptomatic improvement, while in the rest (DP) endoscopic incision is never effective. The cause of this ineffectiveness seemed to be due to persistent functional obstruction having superimposed on mechanical obstruction. The result of the study urges us to be more keen to diagnose and treat the mild congenial urethral obstruction as well as the concomitant functional obstruction in boys with nocturnal enuresis. OBJECTIVE: • To evaluate the clinical significance of congenital obstructive lesions of the posterior urethra in boys with refractory primary nocturnal enuresis. PATIENTS AND METHODS: • VCUG was performed in 43 consecutive boys who visited our department from April 2004 to April 2009 who were unresponsive to conservative treatment. 20 patients of the 43 patients, underwent TUI. VCUG and UDS were performed before and 3-4 months after TUI. • In UDS, the maximum flow rate (Qmax), maximum bladder capacity, and post-voiding residual urine volume were determined using uroflowmetry (UFM), and the detrusor pressure (Pdet) at Qmax was determined in a pressure flow study (PFS). • Clinical outcome was evaluated 3-4 months and 6 months after TUI. RESULTS: • In VCUG performed 3-4 months after TUI, improvement was observed in urethral morphology in all patients. In preoperative PFS, two patterns were observed: 13 patients (65%) had a synergic pattern (SP) in which the Pdet increased with increasing urinary flow rate simultaneously with the initiation of voiding and seven (35%) had a dyssynergic pattern (DP) in which the Pdet was not coincident with the initiation of voiding, but was higher immediately before voiding than at Qmax. TUI was effective only in the SP group: symptomatic improvement was observed in 87.5% of patients with daytime incontinence and 77% of patients with nocturnal enuresis 6 months after TUI. • In the DP group, no effect was observed (0%). With regard to changes in UDS parameters, a significant decrease (P= 0.0004) was observed in the Pdet at Qmax and a significant increase (P= 0.036) was observed in the maximum bladder capacity in the SP group, whereas no significant differences were noted in any parameters in the DP group. CONCLUSION: • Two voiding urodynamic patterns with different clinical outcomes of TUI were detected among patients with congenital posterior urethral obstruction, the underlying disease of refractory primary nocturnal enuresis in boys.

Mid-Term Safety and Efficacy of the Modified Double Hydrodistention Implantation Technique (HIT), Termed Systematic Multi-Site HIT (SMHIT), for Patients with Primary Vesicoureteral Reflux.

PURPOSE: To evaluate the treatment outcomes and postoperative complications associated with the systematic multi-site hydrodistention implantation technique (SMHIT) for primary vesicoureteral reflux (VUR) and to determine its mid-term efficacy and safety. PATIENTS AND METHODS: We retrospectively reviewed the data for 17 ureters from 12 consecutive children, aged ≥1 year, with grade II-IV reflux and a history of febrile urinary tract infections (FUTI), who underwent a single-session of SMHIT. The primary outcome was the absence of postoperative FUTI (clinical success). The secondary outcome was improvement in reflux to grade 0-I on postoperative voiding cystourethrography (radiographic success). RESULTS: Five and 7 children had bilateral and unilateral reflux, respectively. Reflux was categorized as grade II, III, and IV reflux in 2, 12, and 3 ureters, respectively. Seven of 10 (70%) toilet-trained children had bladder-bowel dysfunction (BBD) preoperatively. The SMHIT was performed for all patients, after which BBD improved. The mean postoperative follow-up period was 6 years and 9 months. The clinical success rate was 100%. Radiographic success was achieved in 16/17 ureters (94%) at 3-4 months, 17/17 (100%) ureters at 1 year, and 17/17 (100%) ureters at 3 years postoperatively. Major complications did not develop postoperatively. CONCLUSION: When prioritizing treatment of concomitant BBD in children with primary VUR and avoiding dextranomer/hyaluronic acid injection therapy in contraindicated children according to the Food and Drug Administration recommendations, a single-session of SMHIT may be as effective and safe in the mid-term as performing open anti-reflux surgery.

Distal Urethroplasty and Glanuloplasty Procedure Can be Suitable for All Types of Glanular/Subcoronal Hypospadias.

OBJECTIVE: To correct all types of glanular/subcoronal hypospadias, we performed surgery named the distal urethroplasty and glanuloplasty procedure (DUG procedure). We analyzed cases that we have experienced. METHODS: A vertical incision with the meatal margin was made in the 12 o'clock direction, with the margin of the external urethral meatus as the center. By this meatoplasty according to Heineke-Mikulicz principle, we changed all type of glanular/subcoronal hypospadias to the hypospadias with wide meatus and wide glans. And then Thiersh-Duplay procedure was performed. RESULTS: Consecutive 24 underwent modified DUG procedure. The mean age at the time of surgery was 19.0 ± 11.9 months and the mean preoperative glans width was 13 ± 1.5 mm. The external urethral meatus was located glanular in 13 and subcoronal in 11. Hypoplastic urethra (HU) was observed in 7. Skin chordee in 10, penile torsion in 6 and meatal stenosis in 10 were observed. The mean surgical duration was 106 ± 25.4 minutes and the mean postoperative observation period was 40.5 ± 26.2 months. All patients with preoperative skin chordee, penile torsion, and meatal stenosis were improved postoperatively, and in all cases, apart from the patient with meatal regression with longest HU from glanular to distal penile, the slit-like shape of the external urethral meatus was achieved. CONCLUSION: DUG procedure can be used for any type of glanular/subcoronal hypospadias but care should be taken not to indicate too aggressively for glanular/subcoronal hypospadias accompanying long HU to distal penile shaft.

Does small voided volume influence uroflowmetry curve patterns in Japanese children with daytime urinary incontinence?

BACKGROUND: A voided volume (VV) of <50% of the expected bladder capacity for age is considered small VV. It was recommended that a VV ≥50% of expected bladder capacity for age is required to assess uroflowmetry (UFM) curves because a small VV causes changes in UFM curve characteristics. However, no clear consensus has been reached on the criterion for evaluating UFM curve patterns. OBJECTIVE: The aim of the study was to evaluate the reproducibility and characteristics of UFM curve patterns in children with daytime urinary incontinence (DUI) and with a variety of VVs. METHODS: This study investigated 119 children (79 boys and 40 girls) with primary DUI who underwent UFM 3 times on the same day and were classified into two groups: small VV (<50% of expected bladder capacity for age) in 0-1 of the 3 UFM measurements (group 1; normal VV) or in 2-3 of the 3 UFM measurements (group 2; small VV). The authors then evaluated the agreement of UFM curve patterns among the 3 measurements, classifying complete, partial, or no agreement according to the number of identical curve patterns. The authors also evaluated the most characteristic patterns of UFM curve patterns for each group. RESULTS: Group 1 comprised 45 children, and group 2 comprised 74 children. Rates of complete agreement (group 1, 24/45; group 2, 30/74), partial agreement (group 1, 19/45; group 2, 35/74), and no agreement (group 1, 2/45; group 2, 9/74) did not differ significantly between groups (p = 0.226). Bell curve patterns were significantly more common in group 1 than in group 2 (p = 0.025). Frequency of the tower pattern was significantly higher in group 2 than in group 1 (p = 0.006) (Summary table). DISCUSSION: No differences in agreement rates of UFM curve patterns were seen between two groups (small and normal VV). The authors thus suggest that UFM curve patterns can be validly assessed in children with DUI and with small VV. It was found that the bell pattern was significantly more common among children with normal VV, whereas the tower pattern was significantly more common among children with small VV. The tower pattern reflects an overactive bladder. The present results suggest that some children have DUI that is not attributable to urgency. CONCLUSION: Reproducibility of UFM curve patterns might be properly assessed even in children with DUI and with small VV. This result suggests the presence of various pathological conditions other than the conditions with urgency underlying DUI.

Pediatric Primary Alveolar Soft Part Sarcoma of the Bladder.

A 9-year-old girl was diagnosed with primary alveolar soft part sarcoma of the bladder after imaging examinations and transurethral resection (TUR) of the bladder tumor. As a positive surgical margin of the TUR indicated residual tumor cells, we performed a cystourethrectomy to remove the tumor. A continent urinary reservoir for self-catheterization was constructed using the Mainz pouch technique, and an abdominal (umbilical) continent catheterizable stoma using the appendix was performed. For 2.5 years postoperatively, the patient remained free of local recurrence and distant metastasis. The patient's clinical course has been favorable, with good management of clean intermittent self-catheterization.

Urethral polyp in a young girl: a case report.

Urethral polyps are rarely found in young girls. A total of 12 urethral polyps have been described in young girls in the English literature to date. Here we present a case of urethral polyp that was detected in the distal urethra of a 12-year-girl. Her chief complaint was a sudden blood discharge. On examination, a 15 x 9 mm polypoid mass with a 7 mm pedicle was seen protruding from the urethral meatus. The mass was excised under general anesthesia. Histopathologically, the polyp was covered with urothelium and squamous epithelium, and was composed of congested blood vessels and inflammatory infiltrates. These findings were similar to those of urethral caruncles in postmenopausal female. She has been free from recurrence and has had no complications, as of 12 months after excision.

Evaluation of the Effectiveness of a Short-term Treatment and Repeat Treatment of Nocturnal Enuresis Using an Enuresis Alarm.

OBJECTIVE: To evaluate the effectiveness of a 3-month enuresis alarm (EA) treatment and repeat EA treatment among pediatric patients with nocturnal enuresis, and to compare patient characteristics among "responders" and "nonresponders" to treatment. MATERIALS AND METHODS: Clinical outcomes were retrospectively evaluated for 137 children (94 boys and 43 girls, mean age, 10.1 years). Effectiveness was evaluated after an initial 3-month treatment, using the International Children's Continence Society criteria. Among children in the no-response group at 3 months, those who continued the EA treatment for ≥4 months were subclassified into group 1, whereas children who repeated the EA treatment at an interval ≥6 months were subclassified into group 2. RESULTS: Among our 137 cases, 19 achieved complete response and 47 achieved partial response at 3 months, for an overall treatment effectiveness rate of 48%. Among the no-response group, treatment was extended in 17 cases (group 1), with 3 (18%) achieving a successful outcome. Treatment was repeated in 18 cases (group 2). In group 2, 8 (44%) achieved successful outcome at 3-month time point. Daytime urinary incontinence did not modify treatment effectiveness. CONCLUSION: EA treatment should be given for a short period of time and should not be continued without a definite purpose or clear response. Suspending and then repeating this treatment after an appropriate interval is effective for patients who do not respond to the initial course of treatment.

Aggressive diagnosis and treatment for posterior urethral valve as an etiology for vesicoureteral reflux or urge incontinence in children.

Vesicoureteral reflux (VUR) is one of the most common diseases in pediatric urology and classified into primary and secondary VUR. Although posterior urethral valve (PUV) is well known as a cause of the secondary VUR, it is controversial that minor urethral deformity recognized in voiding cystourethrography represents mild end of PUV spectrum and contributes to the secondary VUR. We have been studying for these ten years congenital urethral obstructive lesions with special attention to its urethrographic and endoscopic morphology as well as therapeutic response with transurethral incision. Our conclusion to date is that congenital obstructive lesion in the postero-membranous urethra is exclusively PUV (types 1 and 3) and that severity of obstruction depends on broad spectrum of morphological features recognized in PUV. Endoscopic diagnostic criteria for PUV are being consolidated.

Renal dysplasia characterized by prominent cartilaginous metaplasia lesions in VACTERL association: A case report.

BACKGROUND: Renal dysplasia is the most important cause of end-stage renal disease in children. The histopathological characteristic of dysplasia is primitive tubules with fibromuscular disorganization. Renal dysplasia often includes metaplastic cartilage. Metaplastic cartilage in renal dysplasia has been explained as occurring secondary to vesicoureteral reflux (VUR). Additionally, renal dysplasia is observed in renal dysplasia-associated syndromes, which are combinations of multiple developmental malformations and include VACTERL association. CASE PRESENTATION: We observed the following multiple developmental malformations in a 108-day-old male infant during a nephrectomy: a nonfunctioning right kidney with VUR, hemidiaphragmatic eventration, a ventricular septal defect (VSD) with tetralogy of Fallot in the heart, cryptorchidism, and hyperdactylia. These developmental anomalies satisfied the diagnostic criteria for VACTERL association. A surgical specimen of the right nonfunctioning kidney revealed prominent cartilaginous metaplasia in the renal dysplasia with VUR. The densities of the ectopic cartilaginous lesions in this nonfunctioning kidney were extraordinarily high compared with other renal dysplasia cases. Giemsa banding of his genome produced normal results. The patient has not undergone further detailed genomic investigation. CONCLUSION: This case might be a novel type of VACTERL association, that is, renal dysplasia combined with prominent cartilaginous metaplasia, tetralogy of Fallot and VSD of the heart, hemidiaphragmatic eventration, and hyperdactylia.

The Endoscopic Morphological Features of Congenital Posterior Urethral Obstructions in Boys with Refractory Daytime Urinary Incontinence and Nocturnal Enuresis.

Purpose This study aims to evaluate the endoscopic morphological features of congenital posterior urethral obstructions in boys with refractory daytime urinary incontinence and/or nocturnal enuresis. Patients and Methods A total of 54 consecutive patients underwent endoscopy and were diagnosed with a posterior urethral valve (PUV) (types 1-4). PUV type 1 was classified as severe, moderate, or mild. A transurethral incision (TUI) was mainly performed for anterior wall lesions of the PUV. Voiding cystourethrography and pressure flow studies (PFS) were performed before and 3 to 4 months after TUI. Clinical symptoms were evaluated 6 months after TUI, and outcomes were assessed according to PFS waveform pattern groups (synergic pattern [SP] and dyssynergic pattern [DP]). Results All patients had PUV type 1 and/or 3 (i.e., n = 34 type 1, 7 type 3, and 13 types 1 and 3). There were severe (n = 1), moderate (n = 21), and mild (n = 25) cases of PUV type 1. According to PFS, SP and DP were present in 43 and 11 patients, respectively. TUI was effective in the SP group and symptoms improved in 77.4 and 69.3% of patients with daytime incontinence and nocturnal enuresis, respectively. Almost no effect was observed in the DP group. A significant decrease in the detrusor pressure was observed at maximum flow rate using PFS in the SP group. Conclusions PUV type 1 encompassed lesions with a spectrum of obstructions ranging from severe to mild, with mild types whose main obstructive lesion existed at the anterior wall of urethra occurring most frequently in boys with refractory daytime urinary incontinence and/or nocturnal enuresis.