Hypospadias and urethral development

PURPOSE: Hypospadias is a common congenital anomaly that may be treated with surgical reconstruction. In the majority of cases the etiology remains elusive. Although androgens are clearly critical for penile development, defects in androgen metabolism and/or the androgen receptor explain only a small subset of cases of hypospadias. Strategies are presented for understanding the etiology of hypospadias. MATERIALS AND METHODS: Current scientific reports on the etiology of hypospadias were reviewed, and the embryology and possible mechanisms of urethral and penile formation are presented. RESULTS: A new theory of glandular human urethral development via endodermal cellular differentiation is proposed to replace the classic explanation of ectodermal intrusion. CONCLUSIONS: Careful studies of penile and urethral development have led to a better understanding of genital embryology. Future areas of study, such as endocrine disrupters, mesenchymal-epithelial interactions and mechanisms of penile growth, are proposed to explain the etiology of hypospadias.     

改良TIP尿道成形术:恢复尿道解剖结构

目的 分析尿道板背侧纵切卷管尿道成形术(TIP)基础上覆盖Buck筋膜联合尿道海绵体(BC)和背侧包皮筋膜组织(P)在无阴茎下弯或轻度阴茎下弯的小儿中远段型尿道下裂的治疗效果。 方法 回顾性分析104例中远段型尿道下裂患儿的病例资料。其中行BC-P-TIP手术72例(A组); 行传统术式TIP+背侧包皮筋膜组织(P-TIP手术)32例(B组)。术后1,3,12月进行门诊随访。术后3月参照尿道下裂客观评分系统(HOSE)对患儿进行手术效果评估,对两组病例的一般资料、HOSE评分、并发症情况进行统计学分析。 结果 A,B组术后3月HOSE评分平均分分别为15.42及13.90,差别有统计学意义(P<0.01)。A,B组的尿瘘发生率为8.3%及25%,差别有统计学意义(P<0.05)。A,B组的尿道狭窄发生率为4.2%及0%,差别无统计学意义(P>0.05)。 结论 BC-P-TIP改良术式相对于P-TIP术式能有效减少尿瘘发生,术后外观满意、排尿功能良好,手术操作简单易学,取材方便,解剖损伤小,且接近正常尿道的解剖结构,值得进一步探讨及推广。     

带蒂阴囊中隔皮瓣加盖睾丸鞘膜尿道成形术在失败尿道下裂手术中的应用

目的:评价带蒂阴囊中隔皮瓣加盖睾丸鞘膜尿道成形术在失败尿道下裂手术中的应用,了解其适应证、手术方法、术后并发症及远期效果。方法:回顾性分析2008年1月~2013年12月采用带蒂阴囊中隔皮瓣加盖睾丸鞘膜尿道成形术治疗的43例手术失败的尿道下裂病例及随访资料,描述手术适应证、手术方法,总结术后并发症及处理方法,评价远期效果。结果:平均手术时间110min。术后并发症发生率为14%(6/43),其中尿道皮肤瘘3例,尿道憩室1例,尿道口狭窄2例。术后1年随访,阴茎外观满意率为90.7%(39/43),通过随诊观察排尿评估尿道功能正常率为95.35%(41/43)。5例尿道镜检查无明显毛石形成。结论:带蒂阴囊中隔皮瓣加盖睾丸鞘膜尿道成形术是在失败尿道下裂修复术中有效的手术方式,手术方法相对简单,容易掌握,手术并发症较少,远期效果确切。     

Video presentation of the second stage of a two-stage repair for proximal hypospadias: A safe and reproducible technique

ObjectiveThe aims of proximal hypospadias repair are good cosmetic outcomes with low rates of complication, with a low number of operative exposures, before the age of genital difference realisation. Neo-urethral fistula and stenosis are well recognised complications; with international rates of 3.8–16% and 1.3–15.6% respectively. We present the key steps of the second of a two staged repair in video format.MethodVideo recording of the procedure performed on an 18-month-old with mid-penile hypospadias is presented. The steps are described; the importance of tension free tubularisation, layered urethral closure, suture technique, vascularised prepucal graft technique, optimal glanuloplasty, and penile shaft skin repair are highlighted.ResultThe child was brought back to the day ward for catheter removal at 7 days; follow-up in clinic showed good postoperative appearance. This technique has been performed on 31 boys with a fistula rate and stenosis rate favourable to the literature.ConclusionWe demonstrate a two-stage technique by video format for proximal hypospadias which is reproducible and gives results comparable to the literature.     

Urethral advancement in hypospadias with a distal division of the corpus spongiosum: Outcome in 158 cases

ObjectiveOutcome of urethral mobilization and advancement (Koff procedure) in hypospadias with a distal division of the corpus spongiosum and redo cases with distal urethral failure.Materials and methodsFrom January 1999 to November 2012, 158 children with a distal hypospadias (115 primary cases and 43 redo cases) underwent surgical repair using the Koff technique with a median age at surgery of 21 months (range, 12–217 months).ResultsMean follow-up was 19 months (median, 14 months). Thirty patients (19%) presented with a complication (13.9% in primary cases and 32.5% in redo surgery) mostly at the beginning of our experience. Meatal stenosis was the most common one (3.5% in primary case, 6% overall). Ventral curvature (>10°), which is considered as a possible long-term iatrogenic complication of the Koff procedure, was not found in patients with fully grown penis except in one redo patient who had, retrospectively, an inadequate indication for this type of repair. Of 158 patients, 33 reached the age of puberty (>14 years old) with a mean follow-up of 34 months, only one presented with a significant ventral curvature.ConclusionUrethral mobilization and advancement is a reasonable alternative for anterior hypospadias and distal fistula repair in selected cases. It has two major advantages compared to other techniques: it avoids any urethroplasty with non-urethral tissue and eliminates dysplastic tissues located beyond the division of the corpus spongiosum, which may not grow at the same pace as the rest of the penis. Significant iatrogenic curvature in fully grown penis is not supported by this series.     

Foreskin and penile problems in childhood

Congenital penile abnormalities are common and merit paediatric surgical or urological review. These include hypospadias, congenital mega-prepuce, buried penis and epispadias. The features and management of these conditions are outlined in this article.