[Study on the effect of simple incision of ventral tunica albuginea on the correction of penile curvature in hypospadias].

Objective: To investigate the therapeutic effect of simple tunica albugineaincision and ventral penile lengthening surgery on the correction of penile curvature due to asymmetry of the cavernous bodies in hypospadias. Methods: A retrospective analysis was performed in 39 children with hypospadias who underwent simple tunica albuginea incision and ventral penile lengthening surgery for correcting asymmetry of the cavernous bodies from January 2016 to December 2018(36 of them were from Department of Pediatric Urology surgery, The Children's Hospital, Zhejiang University School of Medicine, and 3 from Department of Urology surgery, Affiliated Hospital ofJiaxing University), all of whom aged from 0.5 to 5, with a median age of 1.1 years. During the first stage of the operation, firstly penile skin and sarcoma was released by completely degloving the skin and fascia of penis, secondly the factor of short urethral plate was solved by transection of urethral plate, and then the dorsal length of penis (A), the ventral length of the penis before and after straightening by incision of tunica albuginea (B and C) were measured and recorded; onto the second stage of the operation, an artificial erection test was performed to observe the curvature of the penis, the dorsal and ventral length of the penis (D and E) were measured. The dorsal and ventral length of the penis before and after straightening were compared. Results: The dorsal length of penis (A) was 33-39(35.6±3.2) mm, the length of ventral length of penis before straightening (B) was 28-35 (29.8±2.8) mm and the length of ventral length of penis after straightening (C) was 32-38 (34.3±2.1) mm, which were measured during the first stage of operation, and the difference between B and C was statistically significant (P<0.05), while the difference between A and C was not statistically significant (P>0.05). The dorsal length of penis (D) was34-41 (36.4±2.5) mm and the ventral length of penis (E) was 33-40 (35.7±3.6) mm, which were measured during the second stage of operation, and there was no significant difference between D and E (P>0.05). The degree of penile curvature at the time of erection was less than 15° by measuring with the side photos in all patients during 0.5 to 2.5 years of follow-ups with an average of 1.7 years. Conclusions: Penile curvature due to the asymmetry of the cavernous bodies could be effectively corrected by simple incision of ventral tunica albuginea, which showed a good result of early follow-up. The effect of this surgery on ventral penile straightening could be verified by measuring and comparing the ventral and dorsal length of penis during surgery.

[Preliminary investigation of gender assignment in 46,XY disorders of sex development with severe male undermasculinisation].

Objective: To explore the feasibility of gender assignment in 46,XY disorders of sex development (DSD) with severe undermasculinisation mainly based on molecular diagnosis. Methods: A retrospective study of 45 patients of 46, XY DSD with severe undermasculinisation were admitted between November 2015 and October 2018 at Children's Hospital, Zhejiang University School of Medicine. The initial social gender were all female, of whom the external genital manifestations were Prader 0 to 2; the degree of masculinity was scored using external masculinisation score (EMS); the position and development of the gonads were examined by ultrasound, cystoscopy and laparoscopy, also including assessing the development of the Wolffian tube and the Müllerian tube. The level and ratio of testosterone to dihydrotestosterone before and after hCG stimulation were evaluated for the function of Leydig cell and 5α-reductase-2. Gender role scales and sandbox games were used to assess gender role behavior. Genital sensitivity to androgen stimulation was assessed; A panel including 163 genes related to gender development were determined by second-generation sequencing in all 45 patients. Finally, a multidisciplinary team (MDT) makes a gender assignment after a comprehensive analysis mainly based on the molecular etiological diagnosis. Results: Thirty-nine out of 45 patients (87%) had an identifiable genetic etiology, and the remaining 6 (13%) were negative for genetic testing. Forty-five patients had EMS less than or equal to 3 points. Sexual psychological assessment was performed in 39 patients, with male dominance in 24 (62%) and female dominance in 15 (38%). The gender assignment was 23 cases (51%) for male and 19 cases (42%) for female, and 3 cases (7%) were not completely determined. Conclusions: Molecular diagnosis provides a strong basis for appropriate gender assignment of 46, XY DSD children with severe undermasculinisation. Based on molecular diagnosis, each DSD should be analyzed by professional MDT to analyze the clinical symptoms/signs, gonadal development, gonad tumor risk, external genital morphology, sexual psychological assessment, potential fertility opportunities, parental views, Social and cultural factors, etc. make appropriate gender assignment.