Ureteropelvic Junction Obstruction Caused by Crossing Vessels in Infants and Young Children.

BACKGROUND: To analyze the clinical characteristics of ureteropelvic junction obstruction (UPJO) caused by crossing vessels (CV) in infants and young children. METHODS: A retrospective analysis was performed on children with UPJO who underwent primary surgery. Patients were classified into laparoscopic pyeloplasty (LP) and open pyeloplasty (OP) groups and classified as ≤3 or >3 (years old) groups. Children with CV-caused UPJO were identified. RESULTS: A total of 747 patients were included. Ninety cases of CV were identified. The CV discovery rate was higher in the LP group (78/457, 17.1%) than in the OP group (12/290, 4.1%) (P < 0.001). In the ≤3 group, the CV discovery rate in the LP group (27/144, 18.8%) was higher than that in the OP group (11/274, 4.0%) (P < 0.001). In the LP group, there was no significant difference between ≤3 (27/144, 18.8%) and >3 (51/313, 16.3%) groups in the CV discovery rate. The rate in children with UPJO was not significantly different at any age (P > 0.05). Progressive aggravation of hydronephrosis (21/27, 77.8%) and symptomatic hydronephrosis (44/51, 86.3%) were the main surgical indications in the ≤3 and > 3 groups, respectively. There were no preoperatively confirmed cases of CV in the ≤3 group. In the OP group, five patients underwent reoperation, three of whom were due to failure to detect CV during the initial operation. CONCLUSIONS: The CV distribution is similar in children with UPJO across all ages; CV in infants and young children are not rare. LP should be considered as CV are prone to being missed during OP. LEVELS OF EVIDENCE: III.

Role and significance of SIRT1 in regulating the LPS-activated pyroptosis pathway in children with congenital hydronephrosis.

Objective: To explore the characteristics and mechanism of sirtuin 1 (SIRT1) in lipopolysaccharide (LPS)-activated pyroptosis in the renal tissue of children with congenital hydronephrosis (CHn). Methods: We detected the expression characteristics and clinical significance of SIRT1 and pyroptosis pathway proteins in CHn renal tissues by immunohistochemistry. The degree of renal fibrosis was detected by Masson staining. The human renal tubular epithelial cell line (HK-2) was cultured in vitro and treated with LPS (1 µg/mL), the SIRT1-specific agonist SRT1720 (2.5 µmol/L) and small interfering RNA (siRNA)-SIRT1 for 48 hours. After 48 hours, Cell Counting Kit-8 was used to detect the changes in cell proliferation ability, and ELISA was used to detect the changes in the expression of interleukin (IL)-1ß and IL-18 in the cell supernatant. Real-time PCR (quantitative RT-PCR) and western blot analysis were used to detect the expression of SIRT1, caspase-1, caspase-4, NOD-like receptor thermal protein domain associated protein 3(NLRP3), and cleaved gasdermin D (GSDMD) in each group. Results: Serum inflammatory cytokines were significantly elevated in 13 children with CHn with urinary tract infection, mainly caused by Gram-negative bacteria. Severe renal fibrosis occurred in children with CHn. Compared with the control group, the expression of SIRT1 in CHn kidney tissues was decreased, and the expression of caspase-4 and GSDMD was increased. LPS inhibited the expression of SIRT1 in HK-2 cells, promoted the expression of caspase-1, caspase-4, NLRP3, cleaved GSDMD, promoted the expression of IL-1ß and IL-18 in the supernatant, and promoted pyroptosis in HK-2 cells. SRT1720 can inhibit LPS-activated pyroptosis by promoting SIRT1 expression, while siRNA-SIRT1 can further aggravate LPS-activated pyroptosis after inhibiting SIRT1 expression. Conclusions: LPS can promote the inflammatory response in children with CHn by activating non-canonical pyroptosis and inhibiting SIRT1 expression. Promoting SIRT1 expression can inhibit pyroptosis of renal tubular epithelial cells, reduce the release of IL-18 and IL-1ß, and alleviate the progression of renal fibrosis in children with CHn.

A novel corporoplasty technique with a urethral plate flap in hypospadias repair.

OBJECTIVES: To report a novel corporoplasty technique with a urethral plate flap in hypospadias repair and evaluate its safety and efficacy for ventral lengthening. METHODS: Data were retrospectively collected from consecutive patients with hypospadias who underwent urethral plate flap corporoplasty between July 2021 and March 2022. All patients underwent hypospadias repair using the Duckett technique. The corporoplasty procedure involved the following key steps: the half-spongiosum of the urethral plate was harvested as a flap (with a pedicle attached to the corpus cavernosum); a transverse incision of the tunica albuginea was made adjacent to the pedicle; and the flap was patched onto the corporal defect. RESULTS: The study included 10 patients, with a median age of 20 months. The initial meatal location was penile in two patients, penoscrotal in four patients, and scrotal in four patients. The median ventral curvature was 45° after degloving and urethral plate transection. The median ventral lengthening distance proportional to penis length was 0.21. During the median follow-up of 13.8 months, complications occurred in three cases, including two cases of fistula and one case of urethral stricture with secondary diverticulum. No cases of recurrent ventral curvature, meatal stenosis, or urethral dehiscence were noted. Postoperative ultrasonography showed a good continuation of the tunica albuginea and integrity of the stratum spongiosum at the corporoplasty site. CONCLUSIONS: Urethral plate flap corporoplasty is a simple and effective ventral lengthening procedure. The novel corporoplasty technique allows for anatomical and architectural repair of corporal disproportion.

Unilateral Suprainguinal Ectopic Scrotum: Case Report and Literature Review.

Ectopic scrotum is an infrequent congenital scrotal anomaly. Different surgical methods of correcting ectopic scrotum have been used, but none have produced optimal cosmetic results for all types. We describe a case of left ectopic suprainguinal scrotum in a 14-month-old boy who had an undescended left testicle and a left-sided scrotal skin tag. Single-stage rotational flap scrotoplasty and unilateral orchiopexy were performed; however, we modified the surgical technique of scrotal rotation by excising the intervening longitudinal skin. Eight months after surgery, the repositioned scrotum had a better appearance, and the affected testicle was similar in size to the contralateral one. In comparison with other surgical methods, pedicle flap rotation of the ectopic scrotal skin with excision of the intervening longitudinal skin may produce a better cosmetic outcome.

Laparoscopic versus robot-assisted pyeloplasty in infants and young children.

OBJECTIVE: To compare the characteristics of conventional laparoscopic pyeloplasty (LP) and robotic-assisted laparoscopic pyeloplasty (RALP) in infants and young children with ureteropelvic junction obstruction (UPJO). METHODS: We performed a retrospective study of patients (age: 0-36 months) who underwent dismembered pyeloplasty (Anderson-Hynes) with the fourth-generation RALP or traditional LP between April 2020 and December 2020. RESULTS: A total of 33 patients with UPJO were enrolled: 12 underwent RALP (9 left side; 3 right side) and 21 underwent LP (18 left side; 3 right side). In the RALP group, the median patient age was 17 months (range: 5-36 months). In the LP group, the median patient age was 9 months (range: 2-36 months) (P = 0.182). The mean operation times were 120.25 ± 37.54 min (RALP) and 156.10 ± 51.11 min (LP) (P = 0.042), and the mean lengths of hospital stay were 6.42 ± 1.62 days (RALP) and 8.19 ± 2.25 days (LP) (P = 0.023). Removal of the drainage tube was performed after 3.08 ± 0.69 days (RALP) and after 4.76 ± 1.81 days (LP) (P = 0.001). The postoperative pain showed no significant difference. The mean hospitalization costs were 61464.75 ± 2800.53 yuan (RALP) and 22169.52 ± 3442.15 yuan (LP) (P < 0.001). The mean follow-up time was 10-18 months. Significant improvements in the anteroposterior diameter and parenchymal thickness were observed after surgery. Conversion to laparotomy was not performed. No short-term complications occurred during postoperative hospitalization and follow-up. CONCLUSION: RALP has the advantages of less trauma and faster recovery. It can be safely and effectively performed in infants and young children, and its effectiveness is similar to that of traditional LP.

Histopathological Features of Vanishing Testes in 332 Boys: What Is Its Significance? A Retrospective Study From a Tertiary Hospital.

The purpose of this study is to analyze the histopathological features of resected testicular remnant specimens, ascertain the incidence of the presence of either germ cells (GCs) or seminiferous tubules (SNTs), and assess whether surgical excision of the remnant is necessary. A total of 332 boys with vanishing testis underwent surgical removal of unilateral testicular remnants, with age 7-164 months (median age 25 months). Among the total 332 cases, 212 (63.8%) were younger than 36 months and 143 (66.5%) were found to have hypertrophied contralateral testes larger than 1.6 cm in longitudinal diameter under sonography. SNTs were only present in 21 (6.3%) cases and GCs were present in 7 (2.1%) cases. Compared to the review studies, the very low incidence of SNTs and GCs in which implies extremely low chances of potential malignancy. We propose that surgical removal of vanishing testis remnants in an inguinal or scrotal position may not be necessary.

Surgical management and molecular diagnosis of persistent Müllerian duct syndrome in Chinese patients.

Persistent Müllerian duct syndrome (PMDS) is a rare clinically and genetically overlapping disorder caused by mutations in the anti-Müllerian hormone (AMH) gene or the anti-Müllerian hormone receptor type 2 (AMHR2) gene. Affected individuals present uterus and tubes in normally virilized males and are discovered unexpectedly during other surgeries. Since it is rare and complex, a definitive clinical diagnosis can be missed, and there are no guidelines regarding how to deal with the uterus. In the present study, exome sequencing and Sanger verification were performed for causal variants in 12 PMDS patients. Preoperative diagnoses were made by positive exome sequencing in 8 patients. Of them, 7 patients evoked on the basis of ultrasound indicating bilateral testes on the same side of the body. Twelve different AMH variants (2 frameshift/nonsense, 1 deletion, 8 missense, and 1 in-frame) in 9 patients and 6 different AMHR2 variants (5 missense and 1 splicing) in 3 patients were identified. Seven variants were classified as "pathogenic" or "likely pathogenic", and 4 of them were novel. All but two patients with AMH defects showed low serum AMH concentrations, but all patients with AMHR2 defects showed elevated AMH levels. During surgery, an abnormal vas deferens was observed in half of the patients. Eight patients underwent orchidopexy with uterine preservation. Of them, 2 patients presented complications including irreducible cryptorchidism, and 3 patients developed Müllerian remnant cysts. Three patients underwent subtotal hysterectomy. Of them, one patient had complication of injury to the vas deferens, and one had hemorrhage after operation. This is the first report of PMDS involving a large Chinese population. The present study not only expands the variation spectrum but also provides clinical experience about the management of the uterus.

The Characteristics and Distribution of Nerve Plexuses in the Dartos Fascia From Concealed Penis Children.

The purpose of this study is to analyze the nerve plexus distribution in dartos fascia of concealed penis (CP). A total of 28 CP patients met ASA categories I and II were included, with median age of 3.5 years (8 months-5 years). During the surgery, tissue samples of dartos fascia at points 3, 6, 9, and 12 o'clock of the penile shaft were collected. Standard hematoxylin and eosin (H&E) staining and S-100 immunohistochemical staining were used to analyze the nerve plexus distribution among different positions. The number of nerve plexuses in superficial fascia collected at the 6 o'clock position of the penile shaft was the most abundant among four positions (median 7.25, range 1-24). The abundant nerve plexuses in the dartos fascia of CP patients, especially at the 6 o'clock position, indicate that the surgery on the preputial frenulum should avoid damage to the dartos fascia, as it might be related to maintain the erection and sexual function in adolescence.

Splenogonadal fusion: a case report and review of the literature.

BACKGROUND: Splenogondal fusion (SGF) is a rare congenital anomaly characterized by abnormal association between the splenic tissue and the gonads or mesonephric remnants. SGF that requires separate two-stage laparoscopic staged Fowler-Stephen orchiopexy on both the left and right sides is extremely rare. SGF could be misdiagnosed as testicular malignancy and leads to unnecessary orchiectomy. CASE PRESENTATION: This is a case of an 8-month old male infant presented with bilateral cryptorchidism, B-mode ultrasound visualized the left and right testes in the lower abdominal cavity and the upper margin of the left testicle as a hypoechoic mass extending to the spleen, indicating an undescended right testis and possible SGF on the left side. Single-site laparoscopic examination confirmed the diagnosis of SGF on the left side and an undescended right testis. As both testes were high and the right spermatic vessel was poorly developed and short, a routine single stage orchiopexy would be difficult and risky, therefore, separate two-stage laparoscopic staged Fowler-Stephen orchiopexies for both sides were implemented. Stage 1 of the staged Fowler-Stephen orchiopexy for the right side was performed first without treating the left side, Stage 2 for the right side, separation of the left testis from the spleen as well as Stage 1 for the left side were performed 7 months later, and Stage 2 for the left side was performed 7 months after that. Follow-up ultrasound 1 year after the surgery revealed no obvious abnormalities in the shapes of the testes or their blood supply. This treatment strategy prevented unnecessary orchiectomy. CONCLUSIONS: We reported a rare case of SGF that needed separate two-stage laparoscopic staged Fowler-Stephen orchiopexies for both sides, and a review of the recent literature. SGF is a rare congenital anomaly often diagnosed incidentally during exploration/surgery for scrotal swelling/mass, cryptorchidism or inguinal hernia in young patients. Surgeons, especially pediatric surgeons should be aware of this rare condition to avoid unnecessary, life-altering radical orchiectomy. When routine single stage orchiopexy is not feasible or risky for either side, separate two-stage laparoscopic staged Fowler-Stephen orchiopexies could be performed on both the left and right sides to avoid unnecessary orchiectomy.

Identification of risk factors associated with numerous reoperations following primary hypospadias repair.

INTRODUCTION: Complications remain the top evaluation priority subsequent to hypospadias repair. Complications vary in further management, and usually require one or more reoperations. Patients and/or their parents concern not only with the success rate of reoperation, but also with the risk of numerous reoperations. OBJECTIVE: To identify the risk factors associated with numerous reoperations following primary hypospadias repair. STUDY DESIGN: Data were collected retrospectively from patients who underwent reoperations for complications following primary hypospadias repair at a single institution from August 2008 to October 2017. RESULTS: A total of 507 patients required reoperations following 2754 primary hypospadias repairs. Eventually, 486 patients were eligibly included with a median age of 2.2 years. The median follow-up period was 6.5 years. Preserved urethral plate urethroplasty for primary repair (including Snodgrass, Onlay and Mathieu techniques) was performed in 307 (63.2%) patients, Duckett technique was performed in 121 (24.9%) patients, and staged urethroplasty (including staged Duckett, Byars and Bracka techniques) was performed in 58 (11.9%) patients. The complications included 302 fistulas, 108 dehiscence, 50 urethral strictures, 18 meatal stenosis, 38 diverticula, 24 mild recurrent ventral curvature and 23 severe recurrent ventral curvature. A total of 363 (74.7%) patients needed 1 reoperation, 87 (17.9%) needed 2 reoperations, 19 (3.9%) needed 3 reoperations, and 17 (3.5%) needed >3 reoperations. Ordinal logistic regression demonstrated that severe recurrent ventral curvature, urethral stricture, dehiscence and primary staged hypospadias repair increased the risk of numerous reoperations, with odds ratios of 75.991-fold, 36.967-fold, 11.765-fold and 3.074-fold, respectively. In contrast, diverticulum decreased the risk, with an odds ratio of 0.443-fold. DISCUSSION: Our data demonstrated significant heterogeneity in the risk of numerous reoperations for each complication. Severe recurrent ventral curvature conferred the highest risk of numerous reoperations, followed by urethral stricture, dehiscence. In additional, our data showed an increased risk of numerous reoperations following primary staged repairs. Identification the risk factors confers advantages in the assessment of postoperative outcomes and anticipation of future reoperations. CONCLUSION: Severe recurrent ventral curvature, urethral stricture, dehiscence and primary staged hypospadias repair were associated with numerous reoperations following primary hypospadias repair.

Renal malignant tumor with the loss of INI1 expression and WT1 positive in a 3-year-old boy: a case report.

Wilms' tumor is the most common primary renal malignancy in children (80%) and the less common tumors include renal cell carcinoma, rhabdoid tumor, clear cell sarcoma, cellular congenital mesoblastic nephroma and medullary carcinoma, all of which originate from renal parenchyma. The tumors originating from renal pelvis are rare. The immunohistochemistry (IHC) showed INI1 deletion with the WT1 positive which has not been reported as we know. A 3-year-old boy was admitted to hospital for vomiting. An ultrasonography examination revealed a mass in the right kidney, medium echo, as well as hydronephrosis with collecting system separation of 3.5 cm. The computed tomography and the magnetic resonance (MR) radical showed that the tumor occupied the right renal pelvis and extended into the ureter. A radical nephroureterectomy was accomplished through a transabdominal approach. The pathologic diagnosis was malignant renal tumor with INI1 deficiency which was atypical in morphology and immunophenotype, but according to immunophenotype renal rhabdomyoid tumor could not be excluded. The patient was treated with carboplatin, etoposide and cyclophosphamide chemotherapy for 6 months. Follow-up studies of the patient showed no indication of recurrence or metastasis 22 months after nephrectomy. The novel findings may expand the spectrum of pediatric renal tumors to include the special malignancy.

Prevention and control strategies for emergency, limited-term, and elective operations in pediatric surgery during the epidemic period of COVID-19.

The outbreak of coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has spread to more than 100 countries. Children approved to be susceptible to SARS-CoV-2 infection. Preventing and controlling the epidemic while ensuring orderly flows of pediatric surgery clinical work has proven to be a big challenge for both patients and clinicians during the epidemic. Based on the transmission characteristics of SARS-CoV-2 and the requirements for prevention and control of COVID-19, the authors proposed some concrete measures and practical strategies of managing emergency, limited-term, and elective pediatric surgeries during the epidemic period.

Infantile synchronous primary bilateral testicular germ cell tumor: a case report and review of the literature.

Testicular tumors are uncommon in adults, accounting for <1% of all cancers, with testicular germ cell tumors (TGCTs) representing the majority (>95%) of reported cases. Adult and prepubertal TGCTs are fundamentally distinct and the latter is extremely rare, representing 1% of all pediatric solid tumors and having an annual incidence rate of 0.5-2/100,000 boys. Bilateral TGCTs (BTGCTs) account for 0.5-5% of all testicular tumors; the majority are metachronous, while the synchronous account for approximately 0.5-1%. A 16-month-old boy was admitted to our Urology Department with a 2-week history of a painless scrotal mass. Ultrasonography revealed a homogeneous hyperechoic solid mass with rich blood supply in the right testis, and no discernable testicular tissue. A well-delineated heterogeneous echo mass was found within the left testis. Contrast-enhanced computed tomography (CT) scan showed a significantly enhanced mass in the right testis and a mildly enhanced mass in the left testis. Serum alpha-fetoprotein (AFP) was 12,567 ng/mL, while ß-human chorionic gonadotrophin (HCG) and total testosterone levels were normal. Accordingly, bilateral testicular tumors were the primary consideration. The patient underwent right radical inguinal orchiectomy with high ligation of the spermatic cord and left testicle-sparing surgery. Final pathology confirmed a pure yolk sac tumor (YST) from the right testis and a cystic mature teratoma from the left. The follow-up ultrasonography showed no recurrence, with serum AFP returned to normal by postoperative day 44. Synchronous primary BTGCT with teratoma and YST respectively can occur coincidently and successfully treated by testicle-sparing surgery.

Irf5 deficiency in myeloid cells prevents necrotizing enterocolitis by inhibiting M1 macrophage polarization.

Necrotizing enterocolitis (NEC) is a life-threatening inflammatory disease in newborns, but the mechanisms remain unclear. Interferon regulatory factor 5 (IRF5) is a master regulator of macrophage function and is essential for proinflammatory M1 macrophage polarization. Our previous data indicated that M1 macrophages promote NEC injury. Here, we investigated whether IRF5 is involved in the pathogenesis of NEC. First, we found that IRF5 was upregulated in infiltrated macrophages in human neonates with NEC compared to controls. We further confirmed IRF5 upregulation in macrophages in experimental murine NEC and that the infiltrated macrophages were predominantly polarized into the M1 but not the M2 phenotype. Myeloid-specific deficiency of Irf5, which was associated with reduced M1 macrophage polarization and systematic inflammation, dramatically prevented experimental NEC. Moreover, we found that the ablation of Irf5 in myeloid cells markedly suppressed intestinal epithelial cell apoptosis and further prevented intestinal barrier dysfunction in experimental NEC. Bioinformatic and chromatin immunoprecipitation analysis further showed that IRF5 binds to the promoters of the M1 macrophage-associated genes Ccl4, Ccl5, Tnf, and Il12b. Overall, our study provides evidence that IRF5 participates in the pathogenesis of NEC, while the deletion of Irf5 in myeloid cells prevents NEC via inhibiting M1 macrophage polarization.

[Fetal anteroposterior renal pelvic diameter for predicting antenatal hydronephrosis requiring postnatal surgery].

OBJECTIVE: To assess the value of fetal anteroposterior renal pelvic diameter (APD) in predicting antenatal hydronephrosis requiring surgical treatment after birth. METHODS: A total of 525 cases of antenatal hydronephrosis detected by prenatal ultrasonography (ultrasound index APD ≥ 4 mm in the second trimester and APD ≥ 7 mm in the third trimester) in Zhejiang Prenatal Diagnosis Center from June 2007 to June 2018 were retrospectively analyzed. ROC curve was used to analyze the relationship between these ultrasound indicators and the requirement for surgical treatment after birth. RESULTS: There were 162 cases (30.9%) diagnosed in the second trimester and 363 cases (69.1%) diagnosed in the third trimester; 131 cases were diagnosed pathologically after birth, of which 121 finally underwent surgical treatment. The area under ROC curve (AUC) of APD in middle pregnancy for prediction of requiring surgery 1-12 years after birth was 0.910; the cut-off value of APD was 8.45 mm with a sensitivity of 97.1%, specificity of 70.9%, positive predictive value (PPV) of 47.9%, and negative predictive value (NPV) of 98.9%. The AUC of APD in late pregnancy for prediction of requiring surgery 1-12 years after birth was 0.800; the cut-off value of APD was 12.25 mm with a sensitivity of 66.7%, specificity of 81.2%, PPV of 51.7%, and NPV of 89.1%. CONCLUSIONS: APD in pregnancy can be used to predict whether the fetus with hydronephrosis needs surgical treatment after birth, and the prediction value of APD in the middle pregnancy is better.

Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for treatment of clear cell sarcoma of the kidney in children.

BACKGROUND: Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive malignant renal tumor. We describe our experience with neoadjuvant transcatheter arterial chemoembolization (TACE) and systematic chemotherapy for the treatment of advanced CCSK in children. METHODS: Between January 2010 and December 2016, seven patients (3 boys and 4 girls; median 2.2 years) with advanced CCSK received preoperative TACE of renal artery and systemic chemotherapy. The chemoembolic emulsion for TACE consisted of cisplatin, pirarubicin, vindesine, and iodized oil. Preoperative systemic chemotherapy with vindesine, ifosfamide, and etoposide was administered three weeks after TACE. Nephrectomy was performed three weeks after systemic chemotherapy. After surgery, patients received radiotherapy and postoperative chemotherapy. RESULTS: No cardiotoxicity, renal insufficiency, or hepatic dysfunction was found in any patients. Grade II-III marrow suppression developed in four patients. One patient with tumor progress during neoadjuvant therapy failed to successfully undergo surgery and died. Six patients underwent nephrectomy after neoadjuvant therapy. Median follow-up period was 49.5 months (range, 11-83 months). Five patients have recurrence-free survival. One patient is still in postoperative chemotherapy after nephrectomy, radiotherapy and thoracoscopic resection of lung metastases. CONCLUSIONS: Neoadjuvant TACE and systemic chemotherapy appeared to be feasible in the treatment of advanced CCSK in this pilot study. THE TYPE OF STUDY: A case series with no comparison group. LEVEL OF EVIDENCE: Level IV.

Width proportion of the urethral plate to the glans can serve as an appraisal index of the urethral plate in hypospadias repair.

OBJECTIVES: To find a new appropriate evaluation for urethral plate quality in hypospadias repair, with particular interest in the width proportion of the urethral plate to the glans, serving as an appraisal index. METHODS: Data were prospectively collected from prepubertal boys who underwent primary tubularized incised plate hypospadias repair between January 2014 and April 2016 in one center. Intrinsic parameters of the penis (meatal location, glans width, urethral plate width and curvature degree) were measured during the operation. Urethroplasty complications were recorded during follow up. The correlation between width proportion of the urethral plate to the glans and urethroplasty complications was analyzed. RESULTS: Primary tubularized incised plate repair was carried out in 442 patients (mean age 2.8 years, range 0.5-12 years). At mean follow up of 26 months (range 12-38 months), urethroplasty complications occurred in 59 (13.3%) patients. The width proportion of the urethral plate to the glans was weakly correlated to both the glans width and meatal location. The width proportion of the urethral plate to the glans ranged from 0.18 to 0.73, with a mean of 0.39. The cut-off value of width proportion of the urethral plate to the glans was determined to be 0.36 by the receiver operating characteristic curve. Urethroplasty complications occurred in 17 out of 254 patients (6.7%) with width proportion of the urethral plate to the glans >0.36, and 42 out of 188 patients (22.3%) with width proportion of the urethral plate to the glans ≤0.36. The width proportion of the urethral plate to the glans ≤0.36 showed an increased odds of 4.819-fold (95% confidence interval 2.548-9.112, P < 0.001) risk of urethroplasty complications compared with width proportion of the urethral plate to the glans >0.36. Midshaft and proximal meatal location also increased the risk of urethroplasty complications. CONCLUSIONS: The present study highlights the value of the width proportion of the urethral plate to the glans for objectivity and accuracy in urethral plate evaluation, which in turn serves as an independent factor influencing outcomes in tubularized incised plate repair.

Management and prognosis of adrenocortical tumors in children: can we find out an appropriate points-scoring system to predict prognosis?

BACKGROUND/PURPOSE: Adrenocortical tumors (ACTs) are rare in childhood. There are no recognized criteria to exactly distinguish between benign and malignant forms, or predict prognosis. The incidence of tumor varies across geographic regions or ethnicities, as well as malignant proportion. The aim of this study is to examine a single institution's experience with pediatric ACTs and to validate the prognostic value of the biologic/pathologic criteria of Wienecke. METHODS: Records of 26 pediatric ACTs between 1994 and 2016 in our center were reviewed retrospectively. The data recorded of each patient included clinical characteristics, treatment, pathologic findings, disease stating, and outcome. Tumors were categorized according to the Wienecke criteria. RESULTS: All patients underwent primary surgical excision, including negative margins in 20 cases. Stage distribution at diagnosis was: ST I 12, ST II 8, ST III 5, and ST IV 1. According to Wienecke scoring system, 13 cases were <3 criteria, 6 cases were =3 criteria, and 7 cases were >3 criteria. At median follow-up of 34.5 months, 18 patients survived without evidence of disease and 8 patients had lethal outcome. There was a strong association between high Wienecke score and both high stage and adverse outcome. CONCLUSIONS: Wienecke criteria can be an appropriate points-scoring system to predict prognosis for adrenocortical tumors in children. Complete surgical resection with negative margins is optimal for survival.

Neoadjuvant Intraarterial Chemotherapy for Treatment of Malignant Vaginal Tumors in Children: A Single-Center Experience.

Six patients (aged 3-36 mo) with vaginal tumors (rhabdomyosarcoma and endodermal sinus tumor [EST]; n = 3 each) received intraarterial chemotherapy (IAC) and intravenous chemotherapy. Patients underwent internal iliac artery infusion with cisplatin, pirarubicin, and vindesine. Intravenous chemotherapy with vindesine, ifosfamide, and etoposide was administered after 3 weeks. Vaginal tumors disappeared in all patients after 2 or 3 cycles of alternating therapy. Two patients underwent resection of pelvic metastases. Intravenous consolidation chemotherapy was applied. Four patients were disease-free at a median follow-up of 5.8 years. One patient had pelvic recurrence treated with "salvage" therapy with IAC and surgery and was disease-free for 2.5 years.

[Advances in the diagnosis and hormone replacement treatment of 46, XY disorders of sex development].

Disorders of sex development (DSD) is defined as a congenital condition or atypical development of the chromosomal, gonadal, or anatomic sex. The diagnosis, gender assignment, and treatment of DSD require the guidance from experienced multidisciplinary teams. So far there has been no consensus about it in China. Due to dysgenetic gonads, defects in sex steroid biosynthesis or action, or gonadectomy during the prepubertal years, those with DSD suffer from hypogonadism. The hormone replacement therapy of DSD aims at general physiological health and long-term prognosis as well as the avoidance of unnecessary genital and gonadal surgery. This review focuses on the advances in the studies of the diagnosis and hormone replacement therapy of 46,XY DSD.