Jayanthi V R, Ching C B, DaJusta D G, McLeod D J, Alpert S A
Division of Urology, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA.
Division of Urology, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA.
J Pediatr Urol. 2017 Aug;13(4):353.e1-353.e7. doi: 10.1016/j.jpurol.2017.04.012. Epub 2017 May 18.
INTRODUCTION/OBJECTIVE: Proximal hypospadias is one of the most challenging conditions that pediatric urologists have to deal with. Many procedures have been devised over the years, but nothing has been proven to be the best option. Although there have been some attempts at correcting severe hypospadias in one procedure, most have advocated a staged approach. The classic approach - laying penile skin or a graft within a split glans followed by glanuloplasty at the second stage - by definition requires two operations on the glans. In the Ulaanbaatar procedure the distal glanular urethra is constructed at the first stage, allowing for a single glans procedure and thus potentially better cosmetic outcomes. The present study discusses experience with the Ulaanbaatar procedure for severe hypospadias.
The study retrospectively reviewed every child who underwent both stages of this procedure at the present institution. It reviewed age, associated diagnoses, surgical technique and outcomes.
The first stage was analogous to a classic first-stage procedure with regard to division of the urethral plate and correction of penile curvature. However, an island flap of preputial skin was mobilized and tubularized to create the glanular urethra. No attempt was made to bridge the native meatus and this reconstructed urethra, and the remaining penile skin was placed between the two. The second stage was performed 6 months later by tubularizing the penile skin between the two meatuses.
The series consisted of 34 boys. Mean age at surgery was 18.3 months (range 6-118). Nineteen underwent evaluation for genital ambiguity at birth (56%). Thirty (88%) received pre-operative testosterone or human chorionic gonadotropin (HCG). After urethral plate transection, persistent curvature was addressed during the first stage, with dorsal plication in 12 (35%), urethral plate transection alone in six (18%) or ventral grafting with small intestinal submucosa in 16 (47%). Twenty-three boys (67%) had the neourethra tunneled through the glans, and 11 (33%) had the glans split followed by glanuloplasty. Average time between the two stages was 7 months (range 4.0-13.9). Four patients (12%) developed urethral diverticula that required repair. One developed recurrent epididymitis related to an abnormal ejaculatory duct (no stricture) and underwent vasectomy. No patient developed a fistula. Mean length of follow-up was 15.2 months (range 0.3-55.5).
This modification of the classic staged hypospadias repair may allow for better cosmetic outcome, since the majority of boys required no formal glanuloplasty. There were reduced complications, perhaps because the urethral defect acted like a controlled fistula, allowing for better tissue healing prior to final urethral reconstruction.
引言/目的：近端尿道下裂是小儿泌尿外科医生必须应对的最具挑战性的病症之一。多年来已设计出多种手术方法，但尚无一种被证明是最佳选择。尽管曾有尝试在一次手术中矫正重度尿道下裂，但大多数人主张采用分期手术方法。经典方法——在劈开的龟头内铺设阴茎皮肤或移植物，然后在第二阶段进行龟头成形术——根据定义需要对龟头进行两次手术。在乌兰巴托手术中，第一阶段构建远端龟头尿道，从而实现单次龟头手术，因此可能获得更好的美容效果。本研究讨论了乌兰巴托手术治疗重度尿道下裂的经验。
本研究回顾性分析了在本机构接受该手术两个阶段治疗的每例患儿。回顾了年龄、相关诊断、手术技术和手术结果。
第一阶段在尿道板切开和阴茎弯曲矫正方面类似于经典的第一阶段手术。然而，动员并将包皮皮肤岛状皮瓣管状化以构建龟头尿道。未尝试将原尿道口与重建尿道相连，剩余的阴茎皮肤置于两者之间。第二阶段在6个月后通过将两个尿道口之间的阴茎皮肤管状化来完成。
该系列包括34名男孩。手术时的平均年龄为18.3个月（范围6 - 118个月）。19例（56%）出生时接受了生殖器模糊评估。30例（88%）术前接受了睾酮或人绒毛膜促性腺激素（HCG）治疗。尿道板横断后，在第一阶段处理持续性弯曲，12例（35%）采用背侧折叠，6例（18%）仅行尿道板横断，16例（47%）采用小肠黏膜下层腹侧移植。23名男孩（67%）的新尿道穿过龟头，11名（33%）的龟头劈开后进行龟头成形术。两个阶段之间的平均时间为7个月（范围4.0 - 13.9个月）。4例患者（12%）出现需要修复的尿道憩室。1例因射精管异常（无狭窄）发生复发性附睾炎并接受了输精管切除术。无患者发生瘘管。平均随访时间为15.2个月（范围0.3 - 55.5个月）。
这种对经典分期尿道下裂修复术的改良可能会带来更好的美容效果，因为大多数男孩无需进行正式的龟头成形术。并发症减少，可能是因为尿道缺损起到了可控瘘管的作用，在最终尿道重建之前能实现更好的组织愈合。
