[Feminizing genitoplasty for prepubertal children and teenagers female]. / Génitoplastie féminisante dans les anomalies du développement sexuel de l'enfant pré-pubère et de l'adolescente au Cameroun. À propos de 9 cas.

OBJECTIVE: The aim of this study was to report our experience after 10 years of practice of feminizing genitoplasty in prepubertal and adolescent patients with disorders of sex development (DSD) assigned females as females in a developing country. METHODOLOGY: This was a cross-sectional, descriptive and retrospective study over a period of 9 years. All pre-pubertal (8-12 years) and adolescent patients female sex assigned with DSD who had willfully consented to the surgery with their guardians and underwent feminizing genital surgery were enrolled in the study. Data collection included: age at presentation, precise diagnosis, surgical procedures, complications, cosmetic result and duration of follow-up. Each patient had a precise diagnosis and the surgery was planned after discussion with the multidisciplinary team. Cosmetic results were assessed based on: appearance of the clitoris and separation of the vaginal and urethral openings. RESULTS: Nine patients raised as females with a median age of 8 years (IR: 10.75) were recorded. Surgery was performed at a median age of 11 years (IR: 9.5). In this series, 6 had a 46, XY karyotype with varying diagnoses: partial androgen insensitivity syndrome (n=2); 5-alphareductase insufficiency (n=2); 17-ketoreductase insufficiency (n=2); gonadal dysgenesis with a mutation in the NR5A1 gene (n=2), 2 had ovostesticular DSD, (karyotypes 46, XX), and 1 had mixed gonadal dysgenesis (karyotype 45, X/46, XY). Partial or total gonad(s) removal in accordance with assigned gender was the most common associated procedure. It was bilateral in 7 cases and unilateral in 2 cases. Follow-up ranged from 3 months to 4.5 years (median: 26 months, IR:18.25). One patient had acute urinary retention in the early follow-up. No other complication such as incision bleeding was recorded. The cosmetic appearance of the external genitalia was satisfactory in all patients. CONCLUSION: Feminizing genital surgery in Cameroon remains a major challenge and should seldom be realized without a precise diagnosis. Late age at presentation is peculiar to our setting; however, it gives room for the patients' participation and input to decisions that will have a life-long personal impact on their lives in terms of psychosocial development and fertility. LEVEL OF EVIDENCE: 3.

Is it safe to solely use ventral penile tissues in hypospadias repair? Long-term outcomes of 578 Duplay urethroplasties performed in a single institution over a period of 14 years.

OBJECTIVES: Urethral plate tubulization (Thiersch-Duplay procedure) is a widespread procedure mostly used for distal hypospadias. Concerns of long-term outcome have led to this review of the results of a series of 578 cases. PATIENTS AND METHODS: A retrospective review was conducted of 578 patients treated in a single institution following the same procedure and with the same follow-up. Most patients had distal hypospadias (517/578) and were operated on between 12 and 24 months of age (343 patients). The mean follow-up was 25.6 months (6 months-17 years). Evaluation was focused on urethral complications related to inadequate healing of the reconstructed urethra (fistula, urethral dehiscence, urethral stenosis and clinical dysuria). Complications were arbitrarily categorized into early (when occurring less than one year after surgery) and late (after one year). All data were submitted to statistical analysis. RESULTS: Of the 578 patients, 153 (26.5%) had unsatisfactory outcomes, of which 118 (20.4%) had inadequate urethral healing. Of these, 97 appeared early (57%) and 73 appeared late (43%). Fistula and dehiscence were significantly more frequent in the first post-operative year (p<0.0001), whereas stenosis of the reconstructed urethra was more frequent after one year. Follow-up and age at last consultation were significantly higher in patients with complications. Limits and flaws of this study focused on the absence of consensus on evaluation of hypospadias surgery. The paucity of literature on long-term outcomes of urethral plate tubulization was highlighted. A possible explanation of late stenosis of the reconstructed urethra was the poor growth capacity of the dysplastic tissues located beyond the division of the corpus spongiosum. Urethroplasties solely using ventral tissues may represent an additional risk of late failure, as they may not grow with the rest of the genital tubercle. CONCLUSION: Significant short and late complications occur with techniques tubularizing the urethral plate, mostly fistulae in the first post-operative year and urethral stenosis after 1 year following surgery. Urethroplasties using ventral tissues may not grow at the same pace as the rest of the genital tubercle and may explain late urethral dysfunction. This series confirms the necessity of long-term follow-up of hypospadias reconstructions.

Ex vivo absorption of promestriene from oil-in-water emulsion into infant foreskin.

Hypospadias is a birth defect in which the urinary tract opening is not at the tip of the penis. Hypospadias surgery is frequently complicated by healing deficiencies. Topical treatments with oestrogens were reported to improve healing. In the present study, ex vivo percutaneous absorption of promestriene, a synthetic oestrogen resulting of the double esterification of estradiol was conducted as a pre-requisite for further clinical trial in infants. Penetration of promestriene into infant foreskin treated with commercial oil in water emulsion (10 µg mg(-1)) for 24 h was characterized showing controlled release properties enabling epidermal concentration more than six times higher than dermal concentration (4.13±2.46 mg g(-1) versus 0.62±0.84 mg g(-1), respectively). Furthermore, apparent promestriene fluxes into and through the skin (i.e., 1.5 µg cm(-2) h(-1) and<0.89 µg cm(-2) h(-1), respectively) were calculated from (i) drug amount retained into epidermis and dermis, or (ii) the limit of detection into the receptor fluid. In conclusion, less than 2% of initial dose were absorbed within 24h which compared well with others steroids applied topically in colloidal systems.