A multicenter study on surgical procedure selection and risk factor analysis of postoperative complications after TIP and Duckett hypospadias repair.

BACKGROUND: Hypospadias is a common congenital malformation in pediatric urology with surgery being the only curative treatment. Although there are hundreds of surgical methods for hypospadias, no single method can treat all types, and there are still high rates of postoperative complications. We performed this study to investigate surgical procedure selection and perform risk factor analysis of postoperative complications in hypospadias repair. METHODS: Retrospective analysis was performed of complete clinical and follow-up data of children with hypospadias who were treated and followed up at 15 children's clinical centers in Mainland China from December 2018 to December 2019. Children were divided into groups according to Barcat classification and surgical methods in order to analyze the surgical choice for different types of hypospadias and the influencing factors of different surgical methods for complications. RESULTS: In total, 1011 patients were followed up for 26 months. According to Barcat classification, there were 248 cases of distal type hypospadias, 214 of intermediate, and 549 of proximal type. Transverse preputial island flap urethroplasty (Duckett) and tubularized incised plate urethroplasty (TIP) were performed in 375 (37.1%) and 336 cases (33.2%), respectively. The postoperative complication rate of distal hypospadias was 23.4% (15.8-57.1%), mid shaft 29.0% (22.7-40.0%), and proximal 43.7% (30.2-52.9%). Among the 375 patients in Duckett group, 192 had complications. Multivariate logistic analysis showed that the length of prepuce island flap (OR = 3.506, 95% CI: 2.258-5.442) was an independent risk factor for complications after Duckett operation (P < 0.001). In TIP group, there were 336 cases with 84 complications. Multivariate logistic analysis showed that the width of urethral plate after longitudinal resection (OR = 0.836, 95% CI: 0.742-0.942) and glans width (OR = 0.851, 95% CI: 0.749-0.965) were independent risk factors for postoperative complications after TIP (P = 0.003, P = 0.012). CONCLUSION: Several anatomical features play a role during the selection process among the different surgical approaches, including glans size, urethral plate width, and the meatal position. The width of the urethral plate and glans width were risk factors for postoperative complications after TIP. The length of prepuce island flap was a risk factor for complications after Duckett operation.

Antagonistic Effect of Cuscuta chinensis on a Rat Model with Unilateral Cryptorchidism.

BACKGROUND The aim of this study was to investigate the effect of Cuscuta chinensis Lam. on germ cell apoptosis in a rat model of unilateral cryptorchidism. MATERIAL AND METHODS Thirty male SD rats were randomly and equally divided into a control group, a model group, and a Cuscuta group (5.0 g/kg/d) (n=10). The rat model of unilateral cryptorchidism in the model and Cuscuta groups was established by removal of the right gubernaculum, while rats in the control group received no treatment. After modeling, rats in the Cuscuta chinensis group were intragastrically administered Cuscuta chinensis extract (5.0 g/kg/d), while rats in the control group and model group were administered an equal volume of normal saline. After 90 days, all the rats were sacrificed and the testicles were separated and weighed, followed by TUNEL staining to detect germ cell apoptosis, flow cytometry to measure JC-1, ROS, and MDA, and Western blot analysis to evaluate the expression of Bax, Bcl-2, and cleaved caspase3. RESULTS Ninety days after the operation, Cuscuta chinensis Lam significantly minimized the damage caused by modeling by increasing weight of testis, reducing the germ cell apoptosis, and enhancing the mitochondrial membrane potential of testicles, as shown by levels of JC-1, ROS, and MDA, as well as elevating the level of Bcl-2/Bax and reducing the level of cleaved caspase3 (P<0.05). CONCLUSIONS Treatment with Cuscuta chinensis Lam reduced the germ cell apoptosis in rats with unilateral cryptorchidism, which provides new insight for the development of cryptorchidism therapy in the future.

[Free grafting of internal preputial lamina for penile divided nevus in children].

OBJECTIVE: To evaluate the effect of free grafting of internal preputial lamina in the treatment of penile divided nevus in children. METHODS: This retrospective study included 5 children with penile divided nevus, 4 complicated by redundant prepuce and the other 1 by concealed penis, all treated by free grafting of internal preputial lamina from October 2015 to November 2017. The patients ranged in age from 6 years and 8 months to 10 years and 4 months, averaging 8 years and 6 months. The surgical procedure involved complete excision of the lesions underneath the foreskin with a safety margin of 2 mm, collection of healthy internal preputial lamina for free grafting onto the defected glans, and simultaneously circumcision or concealed penis correction. The removed tissue of the divided nevus was subjected to pathological examination. The patients were followed up for 4－29 (mean 18) months postoperatively. RESULTS: All incisions healed primarily and the free grafts of internal preputial lamina survived well, similar to the glans in color and with a desirable appearance. The patients lost no protopathic sensibility of the glans and experienced no recurrence. Pathological examination confirmed 1 case of intradermal nevus and 4 cases of compound nevus. CONCLUSIONS: Free grafting of internal preputial lamina is an ideal method for the treatment of penile divided nevus in children, with the advantages of easy graft collection, little color difference between the graft and surrounding skin, desirable external appearance, and definite clinical effect.

Evaluation and treatment for ovotesticular disorder of sex development (OT-DSD) - experience based on a Chinese series.

BACKGROUND: The aim of this study is to review and present the clinical features and process of evaluation and treatment for OT-DSD in a single center in recent years in China. METHODS: Sixteen patients with OT-DSD during the past 4 years underwent the evaluation and treatment in a single center. The clinical characteristics and outcomes of surgery were analyzed. RESULTS: The surgical age ranged from 17 months to 66 months with a mean age of 20 months, and the mean follow-up was 30 months (4 months to 56 months). The presentation in 11 patients was ambiguous genitalia, and the rest 5 patients were suspected to have DSD in preoperative examination before hypospadias repair. The karyotypes were 46, XX in 11 patients, 46, XX/46, XY in 3, 46, XX/47, XXY in 1, and 46, XY in 1. Initial reared sex was male in 14 patients, female in 1, and undetermined in 1. After surgery, genders were reassigned in 3 patients, while 15 patients were raised as male with testicular tissue left. Only 1 patient with ovarian tissue left was raised as female. Repair was completed in 11 males and 1 female, and stage I urethroplasty was done in 4 males. No further surgery to remove the gonads was needed for inconsonance of gender assignment. No gonadal tumors were detected. CONCLUSIONS: OT-DSD is a rare and complex deformity with few systematic reports in China. It's important to establish a regular algorithm for evaluation and treatment of OT-DSD.

Diacylglycerol kinase κ (DGKK) variants and hypospadias in Han Chinese: association and meta-analysis.

OBJECTIVE: To investigate whether diacylglycerol kinase κ (DGKK) is a susceptibility gene for hypospadias in the Han Chinese population as has been suggested by previous publications. PATIENTS SUBJECTS AND METHODS: A case-control study involving 466 patients with hypospadias and 402 healthy subjects was conducted to assess the relationship between DGKK single nucleotide polymorphisms (SNPs) and hypospadias risk in the Han Chinese population. The 466 hypospadias patients were further divided into mild, moderate and severe subgroups for analysis. RESULTS: Six SNPs (rs1934179, rs4143304, rs9969978, rs1934188, rs4826632 and rs4599945) were marginally associated with mild and moderate hypospadias [odds ratios (ORs) > 1, P = 0.05 to P < 0.1), whereas no significant relationship was seen with the severe cases (ORs >1, P > 0.1). After correcting for multiple testing, it was determined that neither individual SNPs nor individual haplotypes were associated with hypospadias. To evaluate this relationship in multiple populations, we performed a meta-analysis on six SNPs, using combined data from our present results and those of previous studies of different races (including 1966 patients and 2492 controls). Six SNPs (rs1934179, rs4143304, rs9969978, rs1934188, rs7063116 and rs1934190) were significantly associated with mild/moderate hypospadias (ORs >1, P < 0.05), and rs1934179 was significantly associated with severe hypospadias (OR > 1, P < 0.05). CONCLUSIONS: DGKK gene variants do not appear to play a major role in hypospadias susceptibility in the Chinese Han population. Our meta-analysis supports the hypothesis that DGKK is a common risk gene for hypospadias, particularly in cases of mild or moderate hypospadias in Caucasian populations.

Development and verification of machine learning model based on anogenital distance, penoscrotal distance, and 2D:4D finger ratio before puberty to predict hypospadias classification.

Objectives: To describe the anatomical abnormalities of hypospadias before puberty using current commonly used anthropometric index data and predict postoperative diagnostic classification. Methods: Children with hypospadias before puberty who were initially treated at Sichuan Provincial People's Hospital from April 2021 to September 2022 were selected. We recorded their preoperative penoscrotal distance, anogenital distance, 2D:4D finger ratio, and postoperative hypospadias classification. The receiver operating character curve was used for univariate analysis of the diagnostic predictive value of each index for hypospadias classification in the training set. Binary logistic regression, random forest, and support vector machine models were constructed. In addition, we also prospectively collected data from October 2022 to September 2023 as a test set to verify the constructed machine learning models. Results: This study included 389 cases, with 50 distal, 167 midshaft, and 172 proximal cases. In the validation set, the sensitivity of the binary LR, RF, and SVM was 17%, 17% and 0% for identifying the distal type, 61%, 55% and 64% for identifying the midshaft type, and 56%, 60% and 48% for identifying the proximal type, respectively. The sensitivity of the three-classification RF and SVM models was 17% and 17% for distal type, 64% and 73% for midshaft type, 60% and 60% for proximal type, respectively. In the Testing set, the sensitivity of the binary LR, RF and SVM was 6%, 0% and 0% for identifying the distal type, 64%, 55% and 66% for identifying the midshaft type, and 48%, 62% and 39% for identifying the proximal type, respectively. The sensitivity of the three-classification RF and SVM models was 12% and 0% for distal type, 57% and 77% for midshaft type, and 65% and 53% for proximal type, respectively. Compared with binary classification models, the sensitivity of the three-classification models for distal type was not improved. Conclusion: Anogenital distance and penoscrotal distance have a favorable predictive value for midshaft and proximal hypospadias, among which AGD2, with higher test efficiency and stability, is recommended as the preferred anogenital distance indicator. The 2D:4D finger ratio (RadioL, RadioR) has little predictive value for hypospadias classification.

Identification and functional analysis of a rare variant of gene DHX37 in a patient with 46,XY disorders of sex development.

BACKGROUND: 46,XY sex reversal 11 (SRXY11) [OMIM#273250] is characterized by genital ambiguity that may range from mild male genital defects to gonadal sex reversal in severe cases. DHX37 is an RNA helicase that has recently been reported as a cause of SRXY11. So far, a total of 21 variants in DHX37 have been reported in 58 cases with 46,XY disorders of sex development (DSD). METHODS: Whole exome sequencing (WES) was conducted to screen for variations in patients with 46,XY DSD. The subcellular localization of mutant DHX37 proteins was detected by immunofluorescence. And the levels of mutant DHX37 proteins were detected via Western blotting. RESULTS: A novel pathogenic variant of DHX37 was identified in a patient with 46,XY DSD c.2012G > C (p.Arg671Thr). Bioinformatics analysis showed that the protein function of the variant was impaired. Compared with the structure of the wild-type DHX37 protein, the number of hydrogen bonds and interacting amino acids of the variant protein were changed to varying degrees. In vitro assays revealed that the variant had no significant effect on the intracellular localization of the protein but significantly reduced the expression level of the protein. CONCLUSIONS: Our finding further expands the spectrum of the DHX37 variant and could assist in the molecular diagnosis of 46,XY DSD patients.

Exploring the clinical implications of novel SRD5A2 variants in 46,XY disorders of sex development.

This study was conducted retrospectively on a cohort of 68 patients with steroid 5 α-reductase 2 (SRD5A2) deficiency and 46,XY disorders of sex development (DSD). Whole-exon sequencing revealed 28 variants of SRD5A2, and further analysis identified seven novel mutants. The preponderance of variants was observed in exon 1 and exon 4, specifically within the nicotinamide adenine dinucleotide phosphate (NADPH)-binding region. Among the entire cohort, 53 patients underwent initial surgery at Sichuan Provincial People's Hospital (Chengdu, China). The external genitalia scores (EGS) of these participants varied from 2.0 to 11.0, with a mean of 6.8 (standard deviation [s.d.]: 2.5). Thirty patients consented to hormone testing. Their average testosterone-to-dihydrotestosterone (T/DHT) ratio was 49.3 (s.d.: 23.4). Genetic testing identified four patients with EGS scores between 6 and 9 as having this syndrome; and their T/DHT ratios were below the diagnostic threshold. Furthermore, assessments conducted using the crystal structure of human SRD5A2 have provided insights into the potential pathogenic mechanisms of these novel variants. These mechanisms include interference with NADPH binding (c.356G>C, c.365A>G, c.492C>G, and c.662T>G) and destabilization of the protein structure (c.727C>T). The c.446-1G>T and c.380delG variants were verified to result in large alterations in the transcripts. Seven novel variations were identified, and the variant database for the SRD5A2 gene was expanded. These findings contribute to the progress of diagnostic and therapeutic approaches for individuals with SRD5A2 deficiency.

[Anthropometric assessment on prepubertal boys with hypospadias: a multicenter case series].

Objective: Anthropometric variants in prepubertal boys with hypospadias were assigned and assessed to illustrate anatomical malformation. Methods: A total of 516 prepubertal (Tanner grade Ⅰ) boys with hypospadias who were admitted to three medical centers between March 2021 and December 2021 and met the selection criteria for primary surgery were selected. The age of the boys ranged from 10 to 111 months, with an average of 32.6 months. Hypospadias were classified according to the location of the urethral defect, 47 cases (9.11%) of the distal type (the urethral defect is in the coronal groove or beyond), 208 cases (40.31%) of the middle type (the urethral defect is in the penis body), and 261 cases (50.58%) of the proximal type (the urethral defect is at the junction or proximal side of the penis and scrotum). The following indexes were measured: penis length before and immediately after operation, reconstructed urethral length, and total urethral length. Morphological indicators of the glans area, including preoperative height and width of glans, AB, BC, AE, AD, effective AD, CC, BB, the urethral plate width of the coronal sulcus, and postoperative height and width of glans, AB, BE, and AD. In which point A is the distal endpoint of navicular groove, point B is the protuberance lateral to the navicular groove, point C is the ventrolateral protuberance of the glans corona, point D is the dorsal midline point of the glans corona, and point E is the ventral midline point of the coronal sulcus. The foreskin morphological indicators, including the foreskin width, inner foreskin length, and outer foreskin length. The scrotal morphological indicators, including the left, right, and front penile to scrotum distance. The anogenital distances, including anoscrotal distance 1 (ASD1), ASD2, anogenital distance 1 (AGD1), and AGD2. Results: The penis length of the distal, middle, and proximal types decreased successively before operation, the reconstructed urethral length increased successively and the total urethral length decreased successively, these differences were all significant ( P<0.05). The height and width of the glans of the distal, middle, and proximal types significantly decreased successively ( P<0.05), but the height/width of the glans was generally close; AB value, AD value, and effective AD value significantly decreased successively ( P<0.05); there was no significant difference in BB value, urethral plate width of the coronary sulcus, and (AB+BC)/AD value between the groups ( P>0.05). There was no significant difference in the width of glans between the groups after operation ( P>0.05); AB value and AB/BE value increased successively, and AD value decreased successively, these differences were all significant ( P<0.05). The inner foreskin length in the 3 groups significantly decreased successively ( P<0.05), while the outer foreskin length had no significant difference ( P>0.05). The left penile to scrotum distance of middle, distal, and proximal types significantly increased successively ( P<0.05). ASD1, AGD1, and AGD2 significantly decreased from distal type to proximal type successively ( P<0.05). The other indicators' differences were significant only between some groups ( P<0.05). Conclusion: The anatomic abnormalities of hypospadias can be described by anthropometric indicators, which can be used as the basis for further standardized surgical guidance.

Using of Single-Layer Porcine Small Intestinal Submucosa in Urethroplasty on a Beagle Model.

Purpose: To evaluate the role of porcine small intestinal submucosa (SIS) in reducing fistula during urethroplasty and to observe its degradation process in beagle models. Methods: 22 male beagles were divided into the SIS group and control group. All animals received surgical operation to establish the hypospadias model. Urethroplasty was followed. In the SIS group, the urethra was covered with a single-layer SIS material while no SIS material covered in the control group. At the 2nd, 4th, and 12th weeks after the operation, there were 3, 3, and 5 animals in each group, respectively, sacrificed for surgical site histological examinations. The inflammation reaction and collagen hyperplasia levels were assessed. The fistula was identified by retrograde cystourethrography at the 4th and 12th weeks after the operation. Results: the incidence of urethral fistula was 25% (2/8) in the SIS group and 75% (6/8) in the control group. The inflammation reaction of SIS and control groups had no significant difference (U = 52.50, P = 0.58). The collagen fiber increased in both groups; however, the SIS group had a much more gentle increase compared to the control group (U = -0.00, P < 0.001). In the SIS group, the SIS material was roughly complete on the specimens 2 w after surgery but became loose and discontinuous 4 w after surgery and could not be found 12 w after surgery. Conclusion: The material can decrease the incidence of urethral fistula in the animal models, when used as a coverage layer. The SIS degradation process started 2 w-4 w after the operation and finished before 12 w in the animal model.

Analysis of factors associated with postoperative complications after primary hypospadias repair: a retrospective study.

Background: To determine the risk factors for postoperative complications after primary hypospadias repair. Hypospadias has a high postoperative complication rate, and the risk factors of postoperative complications have attracted extensive attention. Methods: A total of 857 children who received primary surgical repair for hypospadias in our center between 3 January 2017 and 29 January 2021 were retrospectively analyzed. The collected data included age at time of surgery, type of hypospadias, body mass index (BMI), surgeon, operation time, length of reconstructed urethra, method of anesthesia (general anesthesia or general anesthesia combined with caudal anesthesia), and postoperative constipation. The risk factors for postoperative complications were analyzed by multivariate analysis. Results: The follow-up time in this study was 6-54 months, with a mean follow-up time of 29 months. A total of 96 (11.2%) of the 857 pediatric patients had postoperative complications, including 44 (45.8%) cases of urethral fistula, 14 (14.6%) cases of urethral stricture, 5 (5.2%) cases of urethral diverticula, 5 (5.2%) cases of distal dehiscence, 3 (3.1%) cases of poor exposure, 2 (2.1%) cases of residual curvature, 1 (1.0%) case of penoscrotal transposition, 6 (6.3%) cases of urethral stricture and diverticulum, 6 (6.3%) cases of urethral fistula and diverticulum, 3 (3.1%) cases of urethral fistula and postoperative residual curvature, 2 (2.1%) cases of urethral fistula and distal dehiscence, and 1 (1.0%) case each of urethral fistula and transposition, urethral diverticulum and poor exposure, urethral stricture and poor exposure, distal dehiscence and transposition, and residual curvature and transposition. After univariate analysis, type of hypospadias (P=0.038), operation time (P<0.001), length of reconstructed urethra (P=0.007), and postoperative constipation (P=0.019) were included in the multivariate logistic regression analysis. The results showed that postoperative constipation was an independent risk factor for complications [P=0.027, odds ratio (OR) =1.793, confidence interval (CI): 1.067 to 3.012]. Conclusions: Postoperative constipation is an important influencing factor for postoperative complications following primary hypospadias repair. Therefore, defecation management should be strengthened for hypospadias patients during the perioperative period.

Robot Surgery for Midline Cysts of the Prostate With Hypospadias in the Pediatric Population.

OBJECTIVE: To summarize the experience of using robotic surgery to treat midline cysts (MLC) of the prostate in pediatric patients with hypospadias, and to introduce and evaluate the initial outcome of seminal passage reconstruction via robot-assisted laparoscopy during the removal of symptomatic MLCs. PATIENTS AND METHODS: We included a case series of 17 symptomatic MLCs with hypospadias. All patients received both genetic and fertility evaluation. MLCs were removed via robot-assisted laparoscopy and the seminal passage was reconstructed in 7 patients. RESULTS: Five patients were assessed as fertile as adults, while the other 12 were assessed as infertile. All 17 patients received robot-assisted laparoscopic surgery to remove MLCs, and 7 patients received reconstruction of the seminal passage. Patients were followed-up for 5-24 months; there were no serious adverse events except for 1 patient who experienced a single occurrence of epididymitis 7 months after reconstruction of the seminal passage. There were no significant cystic remnants in any of the patients upon ultrasonography and/or urethrography after surgery. Urethroscopy and vasography were performed in 2 patients after reconstruction; in both cases, unobstructed seminal passages were indicated. CONCLUSION: Reconstruction of the seminal passage is practical when carried out by robot-assisted techniques. Initial results indicated that the reconstruction operations were able to restore patency in the seminal duct in some of the patients. Fertility is the goal of this technique, and long-term follow-up is needed.

[Effect of surgical steps in primary hypospadias repair on penile length].

OBJECTIVE: To document the effect of surgical steps, including penile degloving, plate transection, dorsal plication, and fasciocutaneous coverage, in primary hypospadias repair on penile length. METHODS: A consecutive series of 209 prepubertal boys with primary hypospadias repair was included with the age ranged from 10 to 97 months (mean, 31.7 months). Intraoperative stretched penile length (SPL) was measured before operation ( n=209), and after each step, namely penile degloving ( n=152), plate transection ( n=139), dorsal plication ( n=170), and fasciocutaneous coverage ( n=209). SPLs before and after each steps or the entire operation were analyzed. The SPL was compared between plate transection group and plate preservation group, dorsal plication group and non-plication group, and plate preservation with plication group and plate preservation without plication group, respectively. Differences of SPL between before and after each steps were analyzed with factors including neourethra length, rest dorsal penile length, rest ventral penile length, preoperative SPL, and the degree of penile curvature after penile degloving, with multivariate linear regression analysis. RESULTS: All the four steps resulted in SPL difference. The SPL increased after penile degloving and plate transection ( P<0.05), and decreased after dorsal plication and fasciocutaneous coverage ( P<0.05). The SPL increased after all steps were completed ( P<0.05). In patients with plate transection, postoperative SPL increased when compared with that before operation ( P<0.05). No significant difference was noted in patients without plate transection ( P>0.05). And there was significant difference in the increased length of SPL between patients with and without plate transection ( P<0.05). In patients with dorsal plication, a significant increase of postoperative SPL ( P<0.05) was noted. No significant difference was noted in patients without dorsal plication ( P>0.05). And there was no significant difference in increased length between patients with and without dorsal plication ( P>0.05). When patients with plate transection were excluded, dorsal plication resulted in no significant difference on postoperative SPL ( P>0.05). The increased length of SPL after penile degloving, dorsal plication, or cutanofascial coverage was not related to the neourethra length, the rest dorsal penile length, the rest ventral penile length, the preoperative SPL, and the degree of penile curvature ( P>0.05). However, the neourethra length and preoperative SPL were the influencing factors for the increased length of SPL after plate transection ( P<0.05). CONCLUSION: The main steps in primary hypospadias repair can change SPL. The lengthening effect of plate transection would not be counteracted by dorsal plication. Dorsal plication makes no significant difference on postoperative SPL.

Assessment of psychosocial functioning and its risk factors in children with pectus excavatum.

BACKGROUND: Psychosocial functioning is poor in patients with pectus excavatum (PE). However, a comprehensive understanding of this issue does not exist. The aim of this study was to assess the severity of psychosocial problems as associated with PE, as well as to identify its risk factors. METHODS: A comparative study was performed at the Sichuan Academy of Medical Sciences/Sichuan Provincial People's Hospital in Chengdu, China. Patients age 6 to 16 who admitted to the outpatient department for the evaluation or treatment for PE were included in the study. In addition to parental reports of child psychosocial problems on the Achenbach Child Behavior Checklist (CBCL), parents also filled in other structured questionnaires, including socio-demographic variables, patients' medical and psychological characteristics. The severity of malformation was assessed by CT scan. For comparison, an age- and gender- matched control group was recruited from the general population. The socio-demographic and scores on CBCL were compared between patients and control subjects. Univariate and multivariate analysis were performed to examine risk factors for psychosocial problems in patients. RESULTS: No statistically significant differences were found with respect to social-demographic variables between children with PE and control subjects. Compared with control subjects, children with PE displayed higher prevalence of psychosocial problems in the different scales of the CBCL questionnaire such as 'withdraw', 'anxious-depressed', 'social problems' and 'total problems'. Both univariate and multivariate analyses suggested that age, severity of malformation, and being teased about PE were significantly associated with patients' psychosocial problems. CONCLUSIONS: The information derived from this study supports the opinion that children with PE have more psychosocial problems than children from the general population. Multiple medical and psychosocial factors were associated with patients' impairment of psychosocial functioning.

Chylous mesenteric cysts in children.

PURPOSE: To evaluate the clinical features, diagnosis, and treatment of chylous mesenteric cysts (CMCs) in children. METHODS: We analyzed retrospectively the clinical records of 10 children with a CMC, treated in the Department of Pediatric Surgery, West China Hospital of Sichuan University, between 1987 and 2008. RESULTS: This series comprised five girls and five boys. The CMC manifested as abdominal distention (n = 9), acute abdomen (n = 8), or an asymptomatic abdominal mass (n = 1), and included five mesojejunal cysts and five mesoileal cysts. Intestinal volvulus occurred in four patients with mesoileal cysts and acute chylous effusion occurred in two patients with mesojejunal cysts. All ten children were treated surgically. The cystic content was positive for Sudan III staining and the chylous test. CONCLUSIONS: Chylous mesenteric cysts manifest as an abdominal mass, abdominal distention, and/or acute abdomen more often in children than in adults. The clinical presentation suggests some association with the localization of the cysts. A good prognosis can be expected with removal of the cyst and the affected intestinal segment.

Oncocytic adrenocortical tumor with uncertain malignant potential in pediatric population: A case report and review of literature.

BACKGROUND: Oncocytic adrenocortical tumor (OACT) is rare, with few cases reported in the literature. No more than 20 cases in children have been reported. The clinical characteristics, diagnosis, treatment and prognosis of children with OACT are summarized based on a literature review, in order to improve the understanding of OACT in children. CASE SUMMARY: We report a case of a 17-mo-old patient who was admitted to our hospital due to symptoms of odynuria and fever, which are clinical features consistent with a functional adrenocortical tumor. The patient was diagnosed with OACT of uncertain malignant potential. Computed tomography indicated a soft tissue giant tumor in the right adrenal region, approximately 4.3 cm × 5.5 cm in size. Multiple nodular and speckled calcifications were observed in the lesion. The patient received robot-assisted laparoscopic right adrenal tumor resection. Postoperative pathological results were consistent with OACT, and immunohistochemical results showed cytokeratin+/-, chromogranin A+, synaptophysin-, neuron-specific enolase-, S100-, Ki67 about 10%, CD34- and D2-40-. After surgery, urinary tract ultrasonography was reviewed monthly, catecholamine hormone and sex hormone levels were examined every 2 mo and computed tomography was performed every 6 mo. To date, no tumor metastasis or recurrence has been identified in this patient. The levels of sex hormones and catecholamine hormones decreased to normal 1 mo after surgery. CONCLUSION: OACT is rare in the pediatric population, with few cases reported in the literature. Although most pediatric OACTs are benign, malignant cases have been reported. Surgical resection is the preferred option in most patients.

Using Buck's Fascia as an Integral Covering in Urethroplasty to Restore the Anatomical Structure of the Penis in One-Stage Hypospadias Repair: A Multicenter Chinese Study Comprising 1,386 Surgeries.

Objectives: The objective of the study is to investigate the feasibility and efficacy of urethroplasty with a Buck's fascia integral-covering technique (BFIC) to wrap and restore the normal anatomical structure of the penis in one-stage hypospadias surgery. Methods: One-stage surgeries for hypospadias management were performed using BFIC from January 2016 to September 2020 at four high-volume medical centers in China. The technique integrates Buck's fascia with glans wings to mobilize and wrap the urethra and restore penile anatomical relationships. The clinical data, postoperative follow-up data, and complications were recorded, and the results were analyzed. Results: A total of 1,386 patients were included in the study: 1,260 cases of primary hypospadias and 126 cases of re-operations; distal in 382 cases (27.6%), mid-shaft in 639 (46.1%), proximal in 365 (26.3%); tubularized incised plate (TIP) in 748 cases, inlay-graft in 124, onlay-graft in 49, Mathieu in 28, free-tube graft urethroplasty in 406, and 31 of hybrid procedures. One thousand one hundred forty-two patients (82.4%) were found to have penile curvature (>10°) after artificial erection and all corrected by dorsal plication/s or transection of the urethra plate (UP) simultaneously. The median followed-up time was 27 months (6-62). A total of 143 (10.3%) complications were recorded: 114 (9.0%) in the primary operations and 29 (23%) in the re-operations, 15 (3.9%) in distal hypospadias, 61 (9.5%) in mid-shaft, and 67 (18.4%) in proximal. The complication rate in UP preservation and transection was 10.1 and 10.8%, respectively. Of all case complications, there were 73 (5.2%) of fistula, 10 (0.6%) of dehiscence, 22 (1.6%) of meatal stenosis, 21 (1.5%) of stricture, 6 (0.7%) of diverticulum, and resident curvature in 11 cases (1.2%). The overall complication rate in TIP and free-tube procedure was 9.8 and 9.9%, respectively, and fistula occurred in primary TIP of 33 cases (4.9%). Conclusions: Buck's fascia with the glans can be used as an integral covering technique in one-stage distal to proximal hypospadias and primary or re-operative hypospadias repair. It is safe, feasible, and effective for the repair of hypospadias.

Identification and functional analysis of fourteen NR5A1 variants in patients with the 46 XY disorders of sex development.

Human sex determination and differentiation is a complex process, during which NR5A1 plays a central role via the transcriptional regulation of key modulators involved in steroidogenesis. Approximately 8-15% of 46,XY DSD are caused by variants in the NR5A1 gene. Therefore, screening for variants in the NR5A1 gene was performed in a Chinese cohort of sixty-two 46,XY DSD patients with no AR or SRD5A2 variants via next-generation sequencing (NGS). Fourteen variants in the NR5A1 gene were identified in 16 patients from 14 unrelated families, including nine novel variants. These variants included eight heterozygote missense variants, two heterozygote frameshift variants, two heterozygote nonsense variants, one heterozygote nonframeshift deletion-insertion variant, and one homozygous missense variant. Functional assays showed that the transcriptional activity of the 11 variants was significantly reduced. In this study, 11 NR5A1 pathogenic variants were identified. These novel variants further expand the existing spectrum of the NR5A1 variants associated with 46,XY DSD, which will, in turn, assist in the molecular diagnosis of DSD.

[Reoperation methods and effectiveness of urethral stricture after urethroplasty of hypospadias].

Objective: To discuss the reoperation methods of urethral stricture after urethroplasty of hypospadias and their effectiveness. Methods: Between September 2010 and April 2018, 169 patients with urethral stricture after urethroplasty of hypospadias, who underwent ineffective conservative treatments first, were accepted. The age ranged from 1 year and 7 months to 41years with a median age of 5 years and 8 months. The stricture located at the external urethral orifice in 80 cases, internal anastomosis connection in 87 cases, and constructed urethra in 2 cases. The symptoms of urethral stricture occurred at 2 weeks to 52 months after urethroplasty, with a median time of 4.5 months. The patients with external urethral orifice stenosis were treated with urethral meatus augmentation (74 cases) and urethral advancement (6 cases). The patients with internal anastomosis connection stenosis were treated with internal urethrotomy with urethroscopy (10 cases), urethrotomy and one-stage urethroplasty (26 cases), and urethrostomy (51 cases) including 43 cases of two-stage urethroplasty. The patients with constructed urethral stricture were treated with urethrolysis. Results: One hundred and fifty-four patients were followed up 6-86 months with an average of 47 months. The stenosis was relieved in 137 cases, and re-stenosis in 12 cases, urethral fistula in 4 cases, all of which were treated successfully. In addition, 1 case with mild urethral diverticulum did not need to be treated. Conclusion: If it is ineffective for the conservative treatment of urethral stricture after urethroplasty of hypospadias, appropriate surgical treatments could be selected according to the location and length of the stricture, local tissue conditions, complications, and so on.

Combined Laparoscope and Flexible Ureteroscope Pyelolithotomy Dealing with Large-Burden Complex Renal Calculi in Children Less Than 1 Year Old.

Objective: To evaluate the experience and result of combined laparoscope and flexible ureteroscope (f-URS) pyelolithotomy in dealing with large-burden complex renal calculi in children <1 year old. Methods: Eleven patients (7 males and 4 females) <1 year old were retrospectively reviewed between December 2015 and May 2017, who had been diagnosed with renal calculi and received combined laparoscope and f-URS pyelolithotomy. The operations were carried out under general anesthesia. Patient' characteristics, presenting symptoms, operative times, and blood loss, stone-free rate, and postoperative complications were all collected retrospectively. Results: All 11 patients received combined laparoscope and f-URS pyelolithotomy. All operations went smoothly without conversion record and blood transfusion. Average time consumed was 109 minutes (55-187 minutes), and blood loss reported was 16 mL (10-25 mL). Average hospital stay after operation was 7 days (5-11 days). On mean follow-up of 6-12 months, no symptomatic urinary tract infections and urinary calculi were detected. Conclusion: Laparoscope combined f-URS pyelolithotomy is considered safe in the treatment of large-burden multiple renal calculi in infant patients with a high stone clearance rate. It has certain advantages in the treatment of renal calculi in infants <1 year old, and it could be an alternative treatment when other treatments fail or are unavailable.