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.

Complete androgen insensitivity syndrome with accelerated onset of puberty due to a Sertoli cell tumor.

Complete androgen insensitivity syndrome (CAIS) is caused by mutations in the androgen receptor gene. Patients with this syndrome have a 46,XY karyotype, male gonads, and normal female external genitalia. While the pre-pubertal risk of developing gonadal tumors is low in these patients, it increases with age. Most gonadal tumors arise from germ cells; stromal cell tumors are uncommon. Herein, we report a CAIS patient with a feminizing Sertoli cell tumor. The patient presented at 8 yr of age with breast enlargement and growth acceleration, concomitant with elevated serum estradiol levels and suppressed serum gonadotropin levels; these findings were inconsistent with CAIS. The patient underwent gonadectomy at 10 yr of age, and histology demonstrated presence of a non-malignant Sertoli cell tumor in the right gonad. We conclude that this is the first reported case of CAIS with accelerated onset of puberty resulting from a Sertoli cell tumor.

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.

Surgical correction of ectopic scrotum and penile torsion in a 5-year-old boy.

Ectopic scrotum is a rare condition, which is described as an anomalous position of one hemiscrotum along the inguinal canal. This anomaly is associated with other genitourinary anomalies. We report a 5-year-old boy with ectopic scrotum and penile torsion, who was successfully treated by simultaneous surgery.

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.

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.

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.

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.

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.

Familial transverse testicular ectopia unrelated to persistent Müllerian duct syndrome.

Transverse testicular ectopia (TTE) is rare. Although several familial cases of TTE have been reported, most of them were associated with persistent Mullerian duct syndrome (PMDS), which may be inherited as an autosomal-recessive or X-linked recessive trait. We experienced two brothers with TTE unrelated to PMDS. A genetic etiology may be involved even in TTE unrelated to PMDS.

[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.

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.