Department of Urology, CACAU-NUPEP, São Paulo, Brazil; Department of Pediatrics, Federal University of São Paulo, São Paulo, Brazil.
Department of Urology, CACAU-NUPEP, São Paulo, Brazil.
J Pediatr Urol. 2017 Dec;13(6):613.e1-613.e4. doi: 10.1016/j.jpurol.2017.04.011. Epub 2017 May 17.
Severe genital abnormalities such as urogenital sinus and urethral duplication with ectopic urethra in the rectum represent a major challenge in reconstructive urology.
We aimed to review our cases presenting with functional ectopic urethra implanted in the rectum that were treated through an ASTRA approach.
We reviewed the medical records of all patients who had undergone an ASTRA approach from 2005-2016. We collected data with interest to primary diagnosis, clinical presentation, additional procedure, immediate clinical outcome, complications, bowel habits after surgery, voiding and bladder emptying pattern and long term follow-up.
Since 2005, we treated 6 cases using this method, consisting of 3 patients with congenital aphallia and 3 with Y-type urethral duplication. Two aphallia patients underwent De Castro's neophalloplasty with simultaneous anastomosis of proximal urethra to a tubed buccal mucosa neourethra and one had a neophalloplasty with transverse skin flaps and primary perineal urethrostomy. Two patients with Y-type urethral duplication had a complete urethroplasty performed (one end-to-end anastomosis and a two-stage repair). Last patient had a definitive perineal urethrostomy. At mean follow-up of 83.5 meses, only one patient voids through the urethra, four have a Mitrofanoff channel and two have a perineal urethrostomy. Immediate follow-up was uneventful and none of our patients had any bowel complications nor fecal incontinence.
Domini et al. were the first proponents of the anterior sagittal transanorectal approach (ASTRA) as an alternative to classic Peña approach. Later, De Castro popularized specifically this technique as a relevant part of his neophalloplasty procedure to treat congenital aphallia. We did not find in this series any complications related to ASTRA technique in regards to bowel habits, fecal incontinence or infection but most of cases we tried to create a new anterior urethra and connect to the proximal stump failed. Moreover, we are skeptic to support simultaneous urethral repair when there is a dysplastic bulbar segment between the proximal stump and the end of the penile urethra normally at penoscrotal junction area by urethral duplication. Treatment at two-stages is also prone to fail in the midterm. At end, most of them will have either a Mitrofanoff or perineal urethral stoma as shown in our data.
The ASTRA approach proved to be useful for cases of rectal implantation of the urethra. No complications related to anterior sphincter incision were documented. We believe it should be regarded as the treatment of choice for cases alike ours.
严重的生殖器畸形，如泌尿生殖窦和尿道重复，伴有异位尿道至直肠，这代表了重建泌尿外科的一个主要挑战。
我们旨在回顾我们的病例，这些病例表现为功能性异位尿道植入直肠，通过 ASTRA 方法进行治疗。
我们回顾了 2005 年至 2016 年间接受 ASTRA 方法治疗的所有患者的病历。我们收集了对原发性诊断、临床表现、附加程序、即刻临床结果、并发症、术后肠道习惯、排尿和膀胱排空模式以及长期随访感兴趣的数据。
自 2005 年以来，我们使用该方法治疗了 6 例患者，包括 3 例先天性无阴茎患者和 3 例 Y 型尿道重复患者。2 例无阴茎患者接受了德卡斯特罗的新阴茎成形术，同时将近端尿道吻合到管状颊粘膜新尿道，1 例接受了横形皮瓣和原发性会阴尿道造口术。2 例 Y 型尿道重复患者进行了完全尿道成形术（1 例端端吻合术和 2 期修复术）。最后 1 例患者行永久性会阴尿道造口术。在平均 83.5 个月的随访中，仅有 1 例患者通过尿道排尿，4 例患者建立了米托法诺夫通道，2 例患者行会阴尿道造口术。即刻随访无并发症，我们的患者均无肠道并发症或大便失禁。
多米尼等人是前矢状经肛门直肠入路（ASTRA）的首批支持者，将其作为经典佩纳入路的替代方法。后来，德卡斯特罗特别推广了这一技术，作为他新阴茎成形术的一部分，以治疗先天性无阴茎。在本系列中，我们没有发现与 ASTRA 技术相关的任何肠道习惯、大便失禁或感染并发症，但我们试图在大多数病例中创建一个新的前尿道，并与近端残端连接，结果都失败了。此外，当近端残端和阴茎尿道末端之间存在发育不良的球部段时，我们对同时进行尿道修复持怀疑态度，通常在阴茎阴囊交界处区域存在尿道重复。中期也容易出现两阶段治疗失败。最后，我们的数据显示，大多数患者最终要么通过米托法诺夫通道，要么通过会阴尿道造口术。
ASTRA 方法已被证明对尿道直肠植入病例有用。没有记录到与前括约肌切开相关的并发症。我们认为，对于我们这样的病例，它应该被视为首选治疗方法。
