5α-reductase 2 deficiency prevents testosterone from being converted to dihydrotestosterone, which causes abnormal urogenital sinus development. The aim of this study was to analyze the relationship between genotype-phenotype, surgical selections, and postoperative complications of 5α-reductase 2-deficient patients with hypospadias. We retrospectively evaluated the medical records of patients who were diagnosed with 5α-reductase 2 deficiency after genetic testing in the Department of Endocrinology and underwent initial hypospadias surgery in the Department of Urology in Beijing Children's Hospital, Capital Medical University (Beijing, China), from April 2007 to December 2021. A total of 69 patients were included in this study; the mean age at surgery was 34.1 months, and the average follow-up time was 54.1 months. Sixty children were treated with preoperative hormone stimulation (PHS) to promote penile growth. The average penis length and glans width were increased by 1.46 cm and 0.62 cm, respectively. The most frequent mutations were p.R227Q (39.1%, 54/138), p.Q6* (15.2%, 21/138), p.G203S (12.3%, 17/138), and p.R246Q (11.6%, 16/138). In 64 patients who were followed up, 43 had a one-stage operation and 21 had a staged operation, and there were significant differences in external masculinization score (EMS) ( P = 0.008) and the average number of operation required to cure ( P < 0.001) between one-stage and staged operations. PHS had a positive effect ( P < 0.001) on penile development. The p.R227Q mutation was associated with higher EMS and less severe hypospadias. One-stage surgery can be selected if conditions permit. The growth and development of children are acceptable in the long term, but penis growth remains unsatisfactory. Long-term complications of hypospadias should be considered during puberty.
Male ; Humans ; Child ; Infant ; Hypospadias/surgery* ; Retrospective Studies ; Oxidoreductases ; Postoperative Complications ; Genetic Association Studies

OBJECTIVES: To investigate the clinical phenotypes, genetic characteristics, and pathological features of children with disorders of sex development (DSD). METHODS: A retrospective analysis was conducted on epidemiological, clinical phenotype, chromosomal karyotype, gonadal pathology, and genotype data of 165 hospitalized children with DSD at Children's Hospital of Hebei Province and Tangshan Maternal and Child Health Hospital from August 2008 to December 2022. RESULTS: Among the 165 children with DSD, common presenting symptoms were short stature (62/165, 37.6%), clitoromegaly (33/165, 20.0%), cryptorchidism (28/165, 17.0%), hypospadias (24/165, 14.5%), and skin pigmentation abnormalities/exteriorized pigmented labia majora (19/165, 11.5%). Chromosomal karyotype analysis was performed on 127 cases, revealing 36 cases (28.3%) of 46,XX DSD, 34 cases (26.8%) of 46,XY DSD, and 57 cases (44.9%) of sex chromosome abnormalities. Among the sex chromosome abnormal karyotypes, the 45,X karyotype (11/57, 19%) and 45,X/other karyotype mosaicism (36/57, 63%) were more common. Sixteen children underwent histopathological biopsy of gonadal tissues, resulting in retrieval of 25 gonadal tissues. The gonadal tissue biopsies revealed 3 cases of testes, 3 cases of dysplastic testes, 6 cases of ovaries, 11 cases of ovotestes, and 1 case each of streak gonad and agenesis of gonads. Genetic testing identified pathogenic/likely pathogenic variants in 23 cases (23/36, 64%), including 12 cases of 21-hydroxylase deficiency congenital adrenal hyperplasia caused by CYP21A2 pathogenic variants. CONCLUSIONS Short stature, clitoromegaly, cryptorchidism, hypospadias, and skin pigmentation abnormalities are common phenotypes in children with DSD. 45,X/other karyotype mosaicism and CYP21A2 compound heterozygous variants are major etiological factors in children with DSD. The most commonly observed gonadal histopathology in children with DSD includes ovotestes, ovaries, and testes/dysgenetic testes.
Male ; Humans ; Child ; Disorders of Sex Development/pathology* ; Hypospadias/complications* ; Cryptorchidism/complications* ; Retrospective Studies ; Adrenal Hyperplasia, Congenital ; Steroid 21-Hydroxylase

There is still debate regarding the optimal surgical approach for proximal hypospadias. This retrospective study aims to evaluate the long-term outcomes using transverse preputial island flap urethroplasty. A total of 320 patients were included, with a mean follow-up of 40.2 months (range: 1-156 months). Complications were encountered in 125 patients (39.1%), including fistulas in 53 (16.6%), urethral strictures in 31 (9.7%), and diverticula in 41 (12.8%). The mean timing of presentation with a complication was 15.8 months (median: 1.7, range: 1-145), of which 79.2% were early complications and 20.8% were late complications. In all, 20.8% of the patients with complications presented after ≥1 year, and 12.8% presented after ≥5 years. Univariate analysis revealed that age at the time of surgery, flap length, and location of the urethral meatus were not correlated with complications. A stricture was present in 31.7% (13/41) of those with diverticula (P < 0.001), while late urethral diverticula were accompanied by urethral strictures in 11.1% (1/9) of cases (P = 0.213). These results indicate that transverse preputial island flap urethroplasty still has a high incidence of complications, even when performed by highly experienced physicians. Most complications of hypospadias are diagnosed within 1 year postoperatively, while fistulas and urinary strictures generally occur within 2 months and diverticula tend to be present by 1 year.
Age Factors ; Child ; Child, Preschool ; Diverticulum/therapy* ; Follow-Up Studies ; Humans ; Hypospadias/surgery* ; Incidence ; Infant ; Male ; Postoperative Complications/therapy* ; Retrospective Studies ; Surgical Flaps ; Urethra/surgery* ; Urethral Stricture/therapy* ; Urologic Surgical Procedures, Male/methods*

Objective: To investigate the risk factors for the complications of urethroplasty in patients with primary hypospadias by postoperative follow-up observation. METHODS: We retrospectively analyzed 110 cases of primary hypospadias repair performed from November 2010 to October 2015, including 70 cases of tubularized incised plate (TIP) urethroplasty and 40 cases of inlay internal preputial graft (IIPG) urethroplasty, all with the urethral plate reserved. We followed up the patients for 15.6－36 months, (27.3 ± 0.52) mo for those with and (26.9 ± 0.22) mo for those without complications. The mean age of the two groups of patients was (7.5 ± 0.2) and (7.0 ± 0.5) yr, respectively. RESULTS: The follow-up data were collected from all the patients, 17 (15.5%) with and 93 (84.5%) without complications. The success rate of surgery was 84.5%. There were no statistically significant differences in the follow-up time and age between the two groups of patients (P >0.05). Single-factor analysis of variance showed significant differences between the complication and non-complication groups in the preoperative urethral opening (P <0.01), ventral penile curvature (P <0.01), and length of urethral defect (P = 0.04), while multiple linear regression analysis exhibited that only ventral curvature was associated with the postoperative complications of the patients (OR = 1.12, 95% CI: 1.06－1.19, P<0.01). CONCLUSIONS We chose single-stage urethroplasty with the urethral plate reserved for the treatment of primary hypospadias and achieved satisfactory outcomes. Ventral penile curvature is an independent risk factor for the complications of primary hypospadias, and a higher degree of curvature is associated with a higher incidnece of complications.
Analysis of Variance ; Child ; Foreskin ; transplantation ; Humans ; Hypospadias ; surgery ; Male ; Penis ; anatomy & histology ; Postoperative Complications ; etiology ; Postoperative Period ; Reconstructive Surgical Procedures ; Regression Analysis ; Retrospective Studies ; Risk Factors ; Treatment Outcome ; Urethra ; surgery ; Urologic Surgical Procedures, Male

Objective: To investigate the clinical effect of modified Snodgrass surgical technique in the treatment of hypospadias. METHODS: We retrospectively analyzed the clinical data about 212 cases of hypospadias treated by urethroplasty from January 2008 to October 2016, 94 with the modified Snodgrass technique, namely with a silk line in addition to the urethral suture to make easier postoperative removal of the suture (group A), and the other 118 with the conventional Snodgrass technique (group B). The urethral suture was removed at 10 days after surgery for the patients in group A. We compared the success rate of surgery and incidence of postoperative complications between the two groups. RESULTS: Compared with group B, group A showed a significantly higher success rate of surgery (81.36% vs 91.49%, P <0.05) but lower incidence rates of postoperative incisional infection (12.71% vs 4.26%, P <0.05) and urinary fistula (16.10% vs 6.38%, P <0.05). No statistically significant difference was found in the incidence of urethral stenosis between the two groups (2.54% vs 2.13%, P >0.05). CONCLUSIONS The modified Snodgrass technique can improve the success rate of surgery and reduce the incidence rates of incisional infection and urinary fistula, which deserves wide clinical application.
Child ; Humans ; Hypospadias ; surgery ; Incidence ; Infant ; Male ; Postoperative Complications ; epidemiology ; prevention & control ; Retrospective Studies ; Surgical Wound Infection ; epidemiology ; Suture Techniques ; Urethra ; surgery ; Urethral Stricture ; epidemiology ; Urinary Fistula ; prevention & control

OBJECTIVETo discuss the methods for urethral external meatus and glanuloplasty after correction of hypospadias.

METHODSThe V shape flap on the dorsal side of glan, combined with the bilateral glan flaps were moved to the ventral side. The flap at the ventral side of urethral external meatus was turned over. Then the urethral external meatus was repositioned to the top end of glan with the ventral defects covered by the dorsal flaps. Then the coniform glan was reconstructed.

RESULTSFrom January 2008 to December 2013, 28 cases were treated, including glandular hypospadias, postoperative retraction of external urethral meatus and meatal stenosis. 21 patients were followed up for 1 -12 months (average, 1 month) with marked improvement of glan appearance and retraction of external meatus. No meatal stenosis happened.

CONCLUSIONSPostoperative retraction of urethral external meatus and meatal stenosis can be corrected by V shape flap on the dorsal side of glan combined with the bilateral glan flaps. The flat appearance of glan can be improved. It is an ideal method for glandular hypospadias.

Humans ; Hypospadias ; surgery ; Male ; Penis ; surgery ; Postoperative Complications ; surgery ; Postoperative Period ; Surgical Flaps ; Urethra ; surgery ; Urethral Stricture ; etiology ; surgery

ObjectiveTo compare the effect of inlaid labial mucosal graft repair (LMGR) with that of bladder mucosal graft repair (BMGR) in the treatment of complex urethral skin fistula after hypospadias repair.

METHODSThis study included 55 cases of complex urethral skin fistula following hypospadias repair. We randomly assigned them to receive inlaid LMGR (n=36) or BMGR (n=19). After surgery, we compared the incidence of complications and recurrence rate of urinary fistula between the two groups of patients.

RESULTSThe success rates of operation were 91.7% and 84.2% in the LMGR and BMGR groups, respectively, and the penile appearance was desirable in both groups. Postoperative complications included 2 cases of urinary fistula and 1 case of urethral stricture in each group. There were no statistically significant differences between the two groups in the success rate of operation (P>0.05) or the incidence rate of postoperative complications (P>0.05).

CONCLUSIONSBoth inlaid LMGR and BMGR yield satisfactory results in the treatment of complex urethral skin fistula. However, LMGR involves less injury in mucosa collection and is easier to perform and therefore deserves more clinical attention.

Cutaneous Fistula ; surgery ; Humans ; Hypospadias ; surgery ; Incidence ; Male ; Postoperative Complications ; Reconstructive Surgical Procedures ; Recurrence ; Urethra ; surgery ; Urinary Bladder ; surgery ; Urinary Fistula ; surgery ; Urologic Surgical Procedures, Male

PURPOSE: Hypospadias is the most common congenital penile anomaly. Information about current utilization patterns of inpatient hypospadias repair as well as complication rates remain poorly evaluated. MATERIALS AND METHODS: The Nationwide Inpatient Sample was used to identify all patients undergoing inpatient hypospadias repair between 1998 and 2010. Patient and hospital characteristics were attained and outcomes of interest included intra- and immediate postoperative complications. Utilization was evaluated temporally and also according to patient and hospital characteristics. Predictors of complications and excess length of stay were evaluated by logistic regression models. RESULTS: A weighted 10,201 patients underwent inpatient hypospadias repair between 1998 and 2010. Half were infants (52.2%), and were operated in urban and teaching hospitals. Trend analyses demonstrated a decline in incidence of inpatient hypospadias repair (estimated annual percentage change, -6.80%; range, -0.51% to -12.69%; p=0.037). Postoperative complication rate was 4.9% and most commonly wound-related. Hospital volume was inversely related to complication rates. Specifically, higher hospital volume (>31 cases annually) was the only variable associated with decreased postoperative complications. CONCLUSIONS: Inpatient hypospadias repair have substantially decreased since the late 1990's. Older age groups and presumably more complex procedures constitute most of the inpatient procedures nowadays.
Adolescent ; Adult ; Age Distribution ; Child ; Child, Preschool ; Hospitalization/statistics & numerical data/*trends ; Humans ; Hypospadias/epidemiology/*surgery ; Infant ; Length of Stay/statistics & numerical data/trends ; Male ; Outcome Assessment (Health Care) ; Postoperative Complications/epidemiology ; United States/epidemiology ; Workload/statistics & numerical data ; Young Adult

OBJECTIVETo study 3 different strategies of urine drainage following hypospadias urethroplasty, the clinical nursing in their application, and their effects.

METHODSWe retrospectively analyzed the clinical data of 595 cases of hypospadias treated by urethroplasty. After surgery, 133 of the patients underwent urine drainage by suprapubic cystostomy (group A), 202 by urethral stent- tube indwelling (group B), and 260 by early initiative micturition with the urethral stent-tube (group C). All the patients received routine postoperative nursing care required for hypospadias repair.

RESULTSOperations were successfully completed in all the cases. Group C showed a remarkably shorter hospital stay and lower incidence rates of urinary fistula and urethral stricture than groups A and B (P<0.05), but there were no significant differences in the three indexes between A and B (P<0.05).

CONCLUSIONFor urine drainage following hypospadias repair, early initiative micturition with the urethral stent-tube can significantly reduce postoperative complications, decrease difficulties and workload of nursing care, and shorten the hospital stay of the patient.

Cystostomy ; Drainage ; methods ; Humans ; Hypospadias ; surgery ; Length of Stay ; Male ; Postoperative Complications ; prevention & control ; Reconstructive Surgical Procedures ; Retrospective Studies ; Stents ; Urethra ; surgery ; Urethral Stricture ; prevention & control ; Urinary Fistula ; prevention & control ; Urine ; Urologic Surgical Procedures, Male

OBJECTIVE: To assess the efficacy of different methods of anesthesia on children underwent hypospadias surgery.
 METHODS: A total of 90 children (2-6 years old, 11.5-21.0 kg weight) with I or II grade of hypospadias based on ASA standard, who scheduled for hypospadias angioplasty, were randomly divided into 3 groups: Group I, general anesthesia combined epidural anesthesia; Group II, laryngeal mask airway under general anesthesia; Group III, laryngeal mask airway under general anesthesia combined epidural block. All children were inhaled sevoflurane to keep bispectral index value in a range from 45 and 60. The Group I adopted epidural anesthesia after intravenous induction of anesthesia; the Group II was inserted laryngeal mask after induction; the Group III was inserted laryngeal mask after induction and adopted epidural block. The anesthesia efficacy, hemodynamic changes, adverse reaction and the postoperative complications were observed in the 3 groups.
 RESULTS: Compared with the Group I or the Group II, the blood pressure and heart rate ran more smoothly in the Group III, and the postoperative agitation and incidence of adverse events were also significantly reduced (all P<0.05).
 CONCLUSION The laryngeal mask airway under general anesthesia combined epidural block is a better choice for children scheduled for hypospadias angioplasty.
Anesthesia ; methods ; Anesthesia, Epidural ; Anesthesia, General ; Anesthetics, Inhalation ; Blood Pressure ; Child ; Hemodynamics ; Humans ; Hypospadias ; surgery ; Laryngeal Masks ; Male ; Methyl Ethers ; administration & dosage ; Postoperative Complications ; Sevoflurane