Development and Validation of the Japanese Version of Quality of Life Assessment of Spina Bifida in Children (QUALAS-C-J).

BACKGROUND: We previously developed the Japanese version of The Quality of Life Assessment of Spina Bifida in Teenagers, a health-related quality-of-life instrument specific to children aged 13-17 years with spina bifida (SB). The Quality of Life Assessment of Spina Bifida in Children is a version of this questionnaire for children aged 8-12 years. The purpose of this study was to develop a Japanese version of the Quality of Life Assessment of Spina Bifida in Children (QUALAS-C-J) and verify its reliability and validity. METHODS: Three urologists specializing in SB, 2 nurses, and 1 statistician developed the QUALAS-C-J and conducted a pilot and main survey. Participants included children with SB and non-disabled (ND) children. Participants completed the QUALAS-C-J and the Japanese version of KIDSCREEN-27 (J-KIDSCREEN) without parental help. RESULTS: Five children with SB participated in the pilot study and provided face and content validity. Sixty-three children with SB and 40 age- and sex-matched ND children participated in the main survey. The intraclass correlation coefficient in the retest was 0.80, and Cronbach's alpha in each domain was 0.73. The validity was verified by factor analysis, convergent / divergent validity, and known-groups validity. Factor analysis converged to the same two-factor structure as the original version. The correlation between QUALAS-C-J and J-KIDSCREEN-27 was weak (r=-0.06-0.30). The scores of both groups for the two domains of the QUALAS-C-J were significantly lower in SB than ND children. CONCLUSIONS: QUALAS-C-J is easy to answer, suitable for Japanese children with SB, reliable, and valid. It can be a communication tool for children with SB, medical staff, families, communities, and school teachers.

Pediatric congenital hydronephrosis (ureteropelvic junction obstruction): Medical management guide.

The prevalence of asymptomatic hydronephrosis, now detected by ultrasonography, has increased. However, definitive management guidelines for the management of congenital hydronephrosis have not been established. The Japanese Society of Pediatric Urology created a "medical management guide" based on new findings for physicians practicing pediatric urology. We developed a medical management guide focused on congenital hydronephrosis caused by ureteropelvic junction obstruction. This medical management guide consists of the definition, pathophysiology, epidemiology, diagnosis, classification, treatment using a clinical management algorithm of hydronephrosis and the long-term course of the disease. The aim of hydronephrosis management is to determine whether surgery should be carried out to avoid renal dysfunction, as there is a possibility for improvement without intervention. Ultrasonography is essential to make treatment decisions. Management is determined by a comprehensive assessment, including the degree of hydronephrosis, anterior-posterior diameter of the renal pelvis and, if necessary, a nuclear medicine evaluation of the status of urine drainage and renal function.

Development and validation of the Japanese version of the Quality of Life Assessment of Spina Bifida in Teenagers (QUALAS-T-J).

BACKGROUND: Spina bifida (SB) is the second-most common birth defect in Japan. In recent years, health-related quality of life measurements have been used to assess the psychosocial status of children with SB. The Quality of Life Assessment of Spina Bifida in Teenagers (QUALAS-T) is a self-reported questionnaire for subjects aged 13-17 years with SB. It focuses particularly on factors related to independence, bladder and bowel. The purpose of this study was to develop and validate a Japanese version of QUALAS-T (QUALAS-T-J). METHODS: Three urologists specialized in SB, two nurses, one statistician, and the author of the original version developed the QUALAS-T-J. Subjects with SB completed the QUALAS-T-J and the Japanese version of KIDSCREEN-27 (J-KIDSCREEN-27) independently from their parents. RESULTS: A pilot study involving seven adolescents with SB and confirmed face and content validity. Sixty-nine adolescents with SB participated in the main survey. The intraclass correlation coefficient in the retest was 0.77, and Cronbach's alpha in each domain was 0.83 and 0.79. Validity was verified on factor analysis and convergent/divergent validity. Five items converged in the one domain, Bladder and Bowel. The remaining five items converged in the concept of Family and Independence. The correlation between each domain of the QUALAS-T-J and J-KIDSCREEN-27 was low-moderate. CONCLUSION: The reliability and validity of the QUALAS-T-J were verified in Japanese adolescents with SB. The QUALAS-T-J would be a useful tool for communication between adolescents with SB and medical staff.

Treatment guidelines for persistent cloaca, cloacal exstrophy, and Mayer-Rokitansky-Küster-Häuser syndrome for the appropriate transitional care of patients.

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer-Rokitansky-Küster-Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH. We were able to publish statements on chronic renal dysfunction, hydrometrocolpos, and pregnancy, based on four CQs about PC, four about CE, and two about MRKH, respectively. However, due to the paucity of proper manuscripts, we were unable to make conclusions about the correct timing and method of vaginoplasty for patients with PC, CE, and MRKH or the usefulness of early bladder closure for patients with CE. These TGs may help clarify the current treatments for PC, CE, and MRKH in childhood, which have been carried out on an institutional basis. To improve clinical outcomes, systematic clinical trials revealing comprehensive clinical data of the urinary and reproductive systems, especially the length of the common channel in PC, are essential.

Male infant with a single-system ectopic ureter that required the removal of the ureteral stump after nephrectomy.

The necessity of removing ureteral stumps after upper tract surgery for ectopic ureters has been debated, because the procedure can be difficult to carry out and might cause injury to bladder continence mechanisms. Fortunately, ureteral stumps rarely cause problems. We herein report the case of a single-system ectopic ureter that required the removal of the ureteral stump after nephrectomy. In our case, reflux into the ectopic ureter adversely affected the dilation of the ureteral stump. Although urine might flow through an ectopic opening during the voiding phase as a result of high pressure, residual urine might be unable to flow during the storage phase because of low pressure in the ureteral stump. The removal of ureteral stumps might be required in patients with reflux into ectopic ureters. When surgical intervention is necessary for patients with megaureters, cystourethroscopy should be carried out to more accurately clarify the anatomy of the lower urinary tract.

Validation of testicular workup for ischemia and suspected torsion score in patients with acute scrotum.

INTRODUCTION: The Testicular Workup for Ischemia and Suspected Torsion (TWIST) score was created to help physicians in the clinical evaluation of acute scrotum in which testicular torsion (TT) is suspected. There have been only a few reports on validation of the TWIST score since the original study was published. OBJECTIVES: To validate the TWIST score as a scoring system. PATIENTS AND METHODS: We reviewed the records of 106 patients with acute scrotum in our prospectively maintained database. The patients were divided into two groups by the final diagnosis: testicular torsion (group TT) or non-testicular torsion (group NTT). We retrospectively calculated the TWIST score of the patients. Patients were divided into low (0-2), intermediate (3-4), or high (5-7) risk groups according to the total score. RESULTS: Fifteen patients (14%) had TT. The median age at presentation was 13.3 years (range 1.6-16.2) in group TT and 10.4 years (range 0.25-18.9) in group NTT, respectively. The sensitivity of clinical and Color Doppler ultrasonography (CDUS) findings to diagnose TT was 100%, whereas the specificity was 93.4%. Positive predictive value (PPV) and negative predictive value (NPV) were 71.4% and 100%, respectively. According to the calculated TWIST score, 65, 23, and 18 patients belonged to low, intermediate, and high risk groups, respectively. TT was found in 1(1.3%), 3(13%), and 11 (61%) patients of low, intermediate, and high risk groups, respectively. The NPV of the TWIST score for the low risk group was 98.5%, whereas the PPV of the TWIST score for the high risk group was 61.1%. Among the four patients with TT in low risk and intermediate risk groups, three patients presented to our institutions within 6 h after the onset. The torsion was 180° in two of the three patients. CONCLUSION: The TWIST score can be useful for clinical diagnosis of testicular torsion, but is inferior to clinical findings along with CDUS. We should consider the possibility that a TWIST score could be underestimated when patients present with acute scrotum immediately after onset or the affected testis is mildly twisted.

[The study of spinal abnormalities associated with cloacal exstrophy].

The treatment of spinal abnormalities with cloacal exstrophy is controversial. Ten cases of this complex treated at Hyogo Prefectural Kobe Children's Hospital between 1991 and 2010 are presented. In our series, all 10 patients had tethered spinal cords. In addition, there were 3 terminal myelocystoceles, 2 meningoceles, 7 lipomas, 5 thickened filums and 3 syrinxes. Eight of 10 patients underwent surgery, and no patient deteriorated. All 3 patients with terminal myelocystocele had lower extremity weakness, but motor functions in two patients improved after surgery. All 4 patients with lipoma and/or thickened filum were asymptomatic. Two of them were conservatively treated, and they remain asymptomatic. Terminal myelocystocele and symptomatic syrinx should be surgically treated.

The learning curve in proximal hypospadias repair.

INTRODUCTION: Although the learning curve in various surgical procedures is increasingly discussed, there have been only a few reports about the learning curve especially in proximal hypospadias repair. OBJECTIVES: To evaluate the learning curve in one-stage hypospadias repair for proximal hypospadias. MATERIALS AND METHODS: We retrospectively reviewed 73 cases of primary hypospadias repair by a single pediatric urologist using one-stage urethroplasty with a preputial skin graft between 2007 and 2018. The overall complication rate included fistula, glans dehiscence, and meatal stenosis. To analyze the impact of the surgeon's experience on the complication rate, we compared the complication rates among different groups of 20 consecutive cases each. In addition, we graphed the actual data of the operative time and cumulative complication rate to evaluate the learning curve. The cumulative complication rate was defined as the surgeon's complication rate (the number of complications accumulated at a time/number of cases at that time). RESULTS: The median age at surgery was 18 months (range: 7-87). The median follow-up was 40 months (range: 3-114). In total, 13 (18%) children had complications: 9 fistulae, 3 glans dehiscence, and 2 metal stenoses. The analysis of each 20 cases revealed that the complication rate declined over time as the surgeon became more experienced. It was mainly attributed to a decline of the fistula rate. The diagram of the operative time showed a reduction with the increasing surgical experience. Additionally, the diagram of the cumulative complication rate demonstrated a plateau after 50 cases. CONCLUSION: Our study showed the positive impact of surgeon experience on the outcome of one-stage repair for proximal hypospadias. In addition, the learning curve in proximal hypospadias repair stabilized after about 50 cases although the supervision of experienced surgeons is required during the early phase of learning curve.

Prepubertal testicular tumors: a 20-year experience with 40 cases.

Testicular tumors are rare in children. In addition, prepubertal testicular tumors (PTT) are fundamentally different from their postpubertal counterparts.We reviewed our 20-year experience with 40 cases to suggest an algorithm for the appropriate surgical approach for patients with PTT. All tumors ultrasonographically suspected of being benign were confirmed to be benign postoperatively. There was a significant difference between median preoperative a-fetoprotein (AFP) levels of infants with yolk sac tumor and teratoma. The majority of our cases were benign, with the most common histopathological subtype being teratoma, as recently reported. Testis-sparing surgery was carried out in five patients with teratoma and three patients with epidermoid cyst. Other than one patient with teratoma, none of the patients developed recurrence or testicular atrophy after testis-sparing surgery. When testis-sparing surgery is considered, our algorithm based on ultrasonographic findings and preoperative AFP levels might be helpful.

[Dextranomer beads in stabilized non-animal sodium hyaluronate gel (NASHA/Dx gel) for vesicoureteral reflux: multi-center study in Japanease patients].

PURPOSE: To investigate the efficacy and safety of endoscopic treatment with the injectable gel of dextranomer beads in stabilized non-animal sodium hyaluronate (NASHA/Dx gel) administered submucosally close to the proximity of ureteral orifice, we performed the multi-center open study of Japanese patients with vesicoureteral reflux (VUR). We herein report the results of the study. SUBJECTS AND METHODS: Patients aged > or = 1 year with grade II-IV VUR underwent endoscopic injection with NASHA/Dx gel. Post-treatment assessment was done by voiding cystourethrography (VCUG) at 3 and 12 months. Patients with VUR grade II-IV at 3 months underwent re-treatment, with VCUG assessment 3 and 12 months after retreatment. Positive response to treatment was defined as reflux grade 0 or 1. RESULTS: The initial treatment was conducted to 116 ureters in 73 patients. The per-protocol efficacy population included 97 ureters in 71 patients. On a per-ureter basis, the positive response rate at 12 months after the last endoscopic treatment was 69.1%, compared with 62.0% on a per-patient basis. Improvement in reflux grade was shown to be statistically significant at both 3 months post initial treatment and 12 months post last treatment. Positive response rate decreased with increasing baseline reflux grade. There were only two mild adverse events (AEs) and one moderate laboratory fluctuation which were potentially relating to NASHA/Dx gel. CONCLUSIONS: This study has shown that endoscopic injection of NASHA/Dx gel is effective and well tolerated in Japanese patients with VUR. First-line use of this treatment for VUR could potentially be considered for Japan also.

A new technique of concealed penis repair.

PURPOSE: Phimosis associated with concealed penis is not amenable to ordinary circumcision. To our knowledge we describe a new technique to repair concealed penis. MATERIALS AND METHODS: From September 2003 to January 2008, 57 consecutive patients with concealed penis were treated using our technique. Median age at surgery was 33 months (range 7 months to 34 years). The technique consists of 3 steps. Step 1 is a ventral incision to slit the narrow ring of the prepuce and expose the glans. Step 2 is a circumferential skin incision made between 2 edges of the ventral diamond-shaped skin defect, followed by midline incision of the dorsal inner prepuce to make 2 skin flaps connected to the glans. Step 3 is skin coverage. Two skin flaps are brought down and sutured together on the ventral side of the penis. The suture line between the penile shaft skin and the flap eventually becomes elliptical. Medical records were reviewed for voiding function, scar formation, and replies from older patients and the parents of younger children about impressions of the surgical results. RESULTS: Median followup was 26 months. No patient had voiding problems. Lymphedema persisted due to suture line constriction in 2 patients who underwent incision of the constriction. All older patients and the parents of younger children were satisfied with the surgical results. CONCLUSIONS: Our new method is easy to design and perform to correct concealed penis. It provides a good cosmetic appearance and seems to be applicable in all cases with deficient penile shaft skin.

Genital Mobilization of Intravesical Phallus Associated With Covered Cloacal Exstrophy: A Case Report.

Cloacal exstrophy is the most severe form of the exstrophy-epispadias complex, occurring in approximately 1 of every 200,000 to 400,000 live births. Variant such as covered cloacal exstrophy presentations are only one-tenth as common. Although exstrophy-epispadias complexes include genital anomalies, intravesical phallus is very rare. We report an extremely rare case of intravesical phallus with covered cloacal exstrophy that was successfully treated by phallic mobilization.

[Individualized treatment of undescended testis].

Undescended testis is one of the most common congenital anomalies requiring surgery. The guideline for the treatment of undescended testis was published by Japanese Society of Pediatric Urology in 2005. However, the management of undescended testis has been still controversial, particularly in case of impalpable testis including abdominal testis. In this article, we review our experience and published reports of orchiopexy for undescended testis and emphasize that the anatomical condition of undescended testis should be applied to individualized surgical treatment.

Bilateral Testicular Loss due to Dog Bite in a Child.

Although animal bites are common, genital trauma caused by animal bites is rare. Here we report a case of bilateral testicular loss in an infant due to a dog bite. The patient was bitten by a friend's dog while his mother was intoxicated and, therefore, did not receive immediate medical attention. Although initial treatment with subsequent genital reconstruction and hormone replacement is important for children with genital trauma, it is too important to make home safe for them.

[The front line on the therapy for VUR in children].

The establishment of a guideline for the therapy of vesicoureteral reflux (VUR) by, the American Urological Association (1997) was one of the most remarkable events in the field of pediatric urology. However, in the daily clinical setting we often have difficulty in deciding whether we should proceed to operate or take a watch and wait policy in patients with high grade VUR. In this debate the indication of surgery, alternative minimal invasive choice, and/or conservative therapy for VUR are discussed from clinical standpoints referring to various cases of VUR.

Vaginal reconstruction in female cloacal exstrophy patients.

OBJECTIVE: To present our experience of vaginal reconstruction in female cloacal exstrophy patients. PATIENTS AND METHODS: The records of 7 postpubertal female cloacal exstrophy patients (median age, 17 years; range, 11-26 years) were retrospectively reviewed. Complete duplication of the uterus and vagina was noted in all patients. All of them underwent various types of vaginoplasty at the time of urinary tract reconstruction. When a pull-through vaginoplasty was difficult, the native vagina was anastomosed to the neovagina using the large or small bowel. Alternatively, the native bladder remaining connected to the vagina was used as a neovagina with simultaneous construction of the gastroileal composite reservoir. RESULTS: The median age at vaginoplasty was 6 years (range, 5-8 years). The techniques were pull-through vaginoplasty in 1 patient, intestinal vaginoplasty in 2 patients, and vaginoplasty using the native bladder in 4 patients. Five patients required revision surgery for hematometrocolpos after the onset of puberty (median age, 14 years; range, 11-16 years). Three patients underwent an end-to-side reanastomosis of the uterus to the neovagina using the bowel or the native bladder, whereas 2 patients received a side-to-side anastomosis of the uterus to the neovagina using the native bladder. No patient developed recurrence of hematometrocolpos after the revision surgery, with a mean follow-up of 71 months. CONCLUSION: Our recent approach to vaginoplasty can be an alternative for female cloacal exstrophy patients. A side-to-side anastomosis of the uterus to the neovagina is a technically easy procedure in surgical management of hematometrocolpos.

VURD Syndrome: Report of Three Cases.

Hoover and Duckett identified the relationship between valves, reflux, and dysplasia, commonly known as VURD (Posterior urethral valve, Unilateral vesicoureteral reflux, Renal dysplasia) syndrome. They noted preserved contralateral renal function in patients with unilateral reflux into a non-refluxing kidney. The proposed mechanism of this protection is that the refluxing collecting system acts as a pressure pop-off. Here we report three cases of VURD syndrome.

Two cases of late-diagnosed ovotesticular disorder of sex development.

Ovotesticular disorder of sex development (ovotesticular DSD) is defined as the presence of testicular and ovarian tissue in the same individual. Both external and internal genitalia of patients with ovotesticular DSD display a spectrum of phenotypes. Most children present with ambiguous genitalia in combination with unilateral or bilateral undescended gonads. We experienced two late-diagnosed children who presented with proximal hypospadias and bilateral scrotal gonads. One should consider the possibility of ovotesticular DSD when managing patients with proximal hypospadias even if both gonads are palpable in the scrotum.

Nephrectomy and upper pole heminephrectomy for poorly functioning kidney: Is total ureterectomy necessary?

OBJECTIVE: We analyzed our experience with nephrectomy and upper pole heminephrectomy combined with subtotal ureterectomy to determine the incidence of the problems associated with the distal ureteral stump. MATERIALS AND METHODS: The records of 48 children who underwent nephrectomy or upper pole heminephrectomy with subtotal ureterectomy for a poorly functioning kidney were reviewed. RESULTS: No patient who underwent nephrectomy for a poorly functioning single-system kidney required secondary surgery. No secondary surgery was required in any patient who underwent upper pole heminephrectomy for ectopic ureter, while secondary surgery was required in five of 17 patients (29%) who underwent upper pole heminephrectomy for ectopic ureterocele. All five patients presented with recurrent febrile urinary tract infection after upper pole heminephrectomy. They also had reflux into the ipsilateral lower moiety, contralateral kidney, or distal ureteral stump. Our results and data from other reports suggest that recurrent urinary tract infections after upper pole heminephrectomy were treatment outcomes based on the initial surgical approach to ectopic ureterocele rather than isolated problems associated with the distal ureteral stump. CONCLUSION: We consider that total ureterectomy is unnecessary at the time of nephrectomy or heminephrectomy for a poorly functioning kidney because the distal ureteral stump rarely causes a problem.