Urethrocutaneous fistula may complicate hypospadias repair. We noticed that double-layered preputial dartos flaps added to tubularized incised plate urethroplasty can reduce the risk of urethrocutaneous fistula. The aim of this study was to compare the outcomes of tubularized incised plate urethroplasty with double-layered preputial dartos flaps to with single-layered local fascial flaps in preventing urethrocutaneous fistula. A retrospective cohort study was conducted between January 2017 and December 2020 at Jordan University Hospital (Amman, Jordan). Boys who were aged between 6 months and 5 years, diagnosed with distal hypospadias, and not circumcised were included. The primary outcome was the occurrence of urethrocutaneous fistula in patients who underwent tubularized incised plate urethroplasty with a double-layered fascial flap. The results showed a total of 163 boys with distal hypospadias; among them, 116 patients underwent tubularized incised plate urethroplasty with a single-layered fascial flap, and 47 underwent tubularized incised plate urethroplasty with a double-layered fascial flap. The development of urethrocutaneous fistula was higher in the group receiving tubularized incised plate urethroplasty with a single-layered fascial flap than in the group receiving tubularized incised plate urethroplasty with a double-layered fascial flap after 1 month, 6 months, and 12 months (6.9% vs 0, 10.3% vs 0, and 5.2% vs 0, respectively), and the difference after 6 months was statistically significant (P = 0.02).
Male ; Humans ; Infant ; Hypospadias/surgery* ; Retrospective Studies ; Urologic Surgical Procedures, Male/methods* ; Urethra/surgery* ; Fistula/surgery* ; Treatment Outcome

OBJECTIVE: To retrospectively analyze sex chromosomal abnormalities and clinical manifestations of children with disorders of sex development (DSD). METHODS: A total of 14 857 children with clinical features of DSD including short stature, cryptorchidism, hypospadia, buried penis and developmental delay were recruited from Zhengzhou Children's Hospital from January 2013 to March 2022. Fluorescence in situ hybridization (FISH) and chromosomal karyotyping were carried out for such children. RESULTS: In total 423 children were found to harbor sex chromosome abnormalities, which has yielded a detection rate of 2.85%. There were 327 cases (77.30%) with Turner syndrome and a 45,X karyotype or its mosaicism. Among these, 325 were females with short stature as the main clinical manifestation, 2 were males with short stature, cryptorchidism and hypospadia as the main manifestations. Sixty-two children (14.66%) had a 47,XXY karyotype or its mosaicism, and showed characteristics of Klinefelter syndrome (KS) including cryptorchidism, buried penis and hypospadia. Nineteen cases (4.49%) had sex chromosome mosaicisms (XO/XY), which included 11 females with short stature, 8 males with hypospadia, and 6 cases with cryptorchidism, buried penis, testicular torsion and hypospadia. The remainder 15 cases (3.55%) included 9 children with a XYY karyotype or mosaicisms, with main clinical manifestations including cryptorchidisms and hypospadia, 4 children with a 47,XXX karyotype and clinical manifestations including short stature and labial adhesion, 1 child with a 46,XX/46,XY karyotype and clinical manifestations including micropenis, hypospadia, syndactyly and polydactyly, and 1 case with XXXX syndrome and clinical manifestations including growth retardation. CONCLUSION Among children with DSD due to sex chromosomal abnormalities, sex chromosome characteristics consistent with Turner syndrome was most common, among which mosaicism (XO/XX) was the commonest. In terms of clinical manifestations, the females mainly featured short stature, while males mainly featured external genital abnormalities. Early diagnosis and treatment are particularly important for improving the quality of life in such children.
Humans ; Male ; Female ; Turner Syndrome/genetics* ; In Situ Hybridization, Fluorescence ; Cryptorchidism ; Hypospadias ; Retrospective Studies ; Quality of Life ; Sex Chromosome Aberrations ; Karyotyping ; Mosaicism ; Disorders of Sex Development/genetics*

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

Cavernous nerve injury is an important cause of erectile dysfunction (ED). Although protective nerve technology has been widely used in nerve-sparing radical prostatectomy (nsRP), the incidence of ED is still very high after surgery. The purpose of our study was to evaluate erectile function (EF) and penile length in the non-erectile state (PLNES) following scheduled phosphodiesterase 5 inhibitor (PDE5i), vacuum erectile device (VED) treatment, and combination therapy after nsRP. One hundred patients with localized prostate cancer and normal EF were randomized to scheduled PDE5i group, VED treatment group, a combined treatment group, and the control group without any intervention. The International Index of Erectile Function-5 (IIEF-5) scores and PLNES were evaluated after 6 months and 12 months of treatment. Sexual Encounter Profile (SEP-Question 2 and SEP-Question 3) were evaluated after 12 months of treatment. Ninety-one of the 100 randomized patients completed the study. We found that the 5 mg tadalafil once a day (OaD) combined with VED can help improve IIEF-5 scores in nsRP patients after both 6 months and 12 months. VED alone or combined with tadalafil OaD can help patients maintain PLNES. VED combined with tadalafil OaD can improve the rate of successful penetration (SEP-Question 2) after 12 months. There were no significant differences in the return to target EF after 12 months among the groups. No significant correlation was noted between the variables and return to target EF (IIEF ≥ 17), and between the variables and effective shortening of the patient's penis (shortening ≥ 1 cm) after 12 months of intervention.
Erectile Dysfunction ; Humans ; Male ; Penile Erection ; Phosphodiesterase 5 Inhibitors ; Prospective Studies ; Prostatectomy ; Prostatic Neoplasms ; Tadalafil ; Treatment Outcome ; Vacuum

Male ; Humans ; Hypospadias/surgery* ; Urologic Surgical Procedures, Male ; Urethra/surgery*

Male ; Humans ; Infant ; Hypospadias/surgery* ; Urologic Surgical Procedures, Male ; Urethra/surgery* ; Penis/surgery* ; Follow-Up Studies

Male ; Humans ; Hyaluronic Acid/therapeutic use* ; Penis ; Penile Diseases ; Syndrome

This study aimed to investigate the curative effect of spiral embedded flap urethroplasty for the treatment of meatal stenosis after penile carcinoma surgery. From January 2015 to January 2021, we used our technique to treat strictures of the external urethral orifice in seven patients, including four cases of meatal stenosis after partial penile resection and three cases of meatal stenosis after perineal stoma. All patients had previously undergone repeat urethral dilatation. The patients underwent spiral embedded flap urethroplasty to enlarge the outer urethral opening. The patients' mean age at the time of surgery was 60 (range: 42-71) years, the mean operative time was 43 min, and the median follow-up period was 18 months. The patients voided well post-operatively, and urinary peak flow rates ranged from 18.3 ml s^-1 to 30.4 ml s^-1. All patients were successful with absence of urethral meatus stricture. The present study showed that using spiral embedded flap urethroplasty to treat meatal stenosis after penile carcinoma surgery is an effective surgical technique with good long-term outcomes.
Male ; Humans ; Urologic Surgical Procedures, Male/methods* ; Penile Neoplasms/surgery* ; Constriction, Pathologic/surgery* ; Treatment Outcome ; Urethral Stricture/surgery* ; Urethra/surgery* ; Amputation, Surgical ; Carcinoma/surgery* ; Retrospective Studies

Male ; Humans ; Penile Neoplasms/surgery* ; Constriction, Pathologic/surgery* ; Urethral Stricture/surgery* ; Surgical Flaps/surgery* ; Amputation, Surgical ; Penis/surgery* ; Plastic Surgery Procedures ; Hypospadias/surgery*