Department of Paediatric Surgery, Christian Medical College, Vellore, Tamilnadu, 632004, India.
Paediatric Surgeon, Coimbatore, Tamilnadu, India.
J Pediatr Urol. 2021 Jun;17(3):414.e1-414.e8. doi: 10.1016/j.jpurol.2021.02.008. Epub 2021 Feb 16.
Y-duplication is a rare subtype of urethral duplication whose surgical correction has traditionally yielded less than satisfactory results. Herein we report 18 patients, 17 of whom have completed successful urethral reconstruction.
A retrospective analysis was done on 18 children who have undergone repair of Y-duplication urethra in two tertiary care institutes from 2013 to 2020. The various subtypes encountered were classified in order to develop a rationale for reconstruction based on the underlying pathology. The various modalities used for reconstruction and the outcomes of the repair were studied. All but one boy voided mainly via the posterior (ventral) channel opening in the ano-rectum or perineum. These boys had the urethral reconstruction incorporating the healthy proximal ventral urethra at its origin. Further reconstruction up to the glans tip depended on the degree of dorsal (orthotropic) urethral patency, which forms the basis of our classification into Types I (completely stenotic), II (penile urethra patent), III (penile and bulbar urethra patent) and IV (fully patent dorsal urethra).
Reconstruction resulting in voiding via a glanular or coronal meatus was completed in 17 boys. One boy is awaiting further surgery while being dependent on Mitrofanoff CIC. While urethral continuity could be achieved using patent segments of native urethra alone into two boys (Types II and III), all other children needed a neo-urethral segment to replace (Type I) or supplement (Type II) the dorsal urethra. Neo urethra was constructed from a tubularized preputial island flap (n = 11), Monti tube constructed from ileum (n = 3) or sigmoid colon (n = 4). A perineal operative exposure alone sufficed in 9, while the rest required an additional trans pubic approach. The only boy with Type IV anomaly underwent excision of the ventral urethral limb. Three boys with initially elevated serum creatinine have normal levels after reconstruction.
Successful reconstruction of boys with Y- duplication of the urethra entailed elucidation of anatomical variations, adequate operative exposure (trans pubic and/or perineal) and innovative utilisation of local and/or distant tissues (preputial island flap, ileal/sigmoid Monti tubes).
Y 型尿道重复畸形是一种罕见的尿道重复畸形亚型,其手术矫正的效果一直不尽如人意。本文报告了 18 例患者,其中 17 例已成功完成尿道重建。
对 2013 年至 2020 年期间在两家三级医疗机构接受 Y 型尿道重复畸形修复的 18 例儿童进行回顾性分析。根据潜在病理情况对各种亚型进行分类,以便为重建制定合理方案。研究了各种重建方法和修复结果。除 1 例男孩外,其余男孩均主要通过肛直肠或会阴部的后(腹)尿道开口排尿。这些男孩的尿道重建包括起源处健康的近端腹侧尿道。在向龟头尖端进一步重建取决于背侧(正常)尿道的通畅程度,这是我们将其分类为 I 型(完全狭窄)、II 型(阴茎尿道通畅)、III 型(阴茎和球部尿道通畅)和 IV 型(完全通畅的背侧尿道)的基础。
17 例男孩完成了通过龟头或冠状沟尿道开口排尿的重建。1 例男孩正在等待进一步手术,同时依赖米托法诺夫 CIC。虽然仅使用通畅的固有尿道段即可将尿道连续性重建至 2 例男孩(II 型和 III 型),但所有其他儿童均需要新的尿道段来替代(I 型)或补充(II 型)背侧尿道。新尿道是由带蒂的包皮岛状皮瓣(n=11)、回肠(n=3)或乙状结肠(n=4)构建的 Monti 管。9 例仅需会阴手术入路,其余均需附加耻骨后入路。唯一的 IV 型异常男孩接受了腹侧尿道支的切除。3 例初始血清肌酐升高的男孩在重建后水平正常。
成功重建男孩的 Y 型尿道重复畸形需要阐明解剖变异,充分的手术暴露(耻骨后和/或会阴),并创新性地利用局部和/或远处组织(包皮岛状皮瓣、回肠/乙状结肠 Monti 管)。