Cincinnati Children's Hospital Medical Center, Division of Pediatric Urology, 3333 Burnet Avenue, Cincinnati, OH 45229.
Cincinnati Children's Hospital Medical Center, Department of Pediatric Surgery, 3333 Burnet Avenue, Cincinnati, OH 45229.
J Pediatr Surg. 2020 Sep;55(9):1834-1838. doi: 10.1016/j.jpedsurg.2020.01.050. Epub 2020 Feb 4.
Anorectal malformations (ARMs) represent a complex spectrum of anorectal and genitourinary anomalies and a paucity of evidence is available on long-term urologic outcomes in all ARM subtypes. It was our subjective bias from being a referral center for ARM patients that the subtype of rectovestibular fistula and absent vagina had higher risk of renal and bladder abnormalities than typical rectovestibular fistula patients. Therefore, to confirm or refute our clinical suspicions, the purpose of this study was to review this specific cohort of ARM patients and describe both the clinical urological and urodynamic outcomes.
A retrospective cohort study was performed for 120 patients who were treated for ARM and vaginal replacement at our institution between 1991 and 2017. Fifteen patients with rectovestibular fistula and absent vagina were included in our review. Demographic and clinical data were abstracted from their medical records, including urodynamic findings, need for clean intermittent catheterization (CIC), urinary continence, and renal function.
Vaginal replacement surgery was undertaken concomitantly with ARM repair in 10 of the 15 patients (67%). One patient was lost to follow up, and mean follow up postoperatively was 39 months. In all but one patient, rectum or colon was used as the substrate for vaginal replacement. Of the 15 patients, 13 had continence data available. A total of 10 patients (77%) were able to achieve social continence. Overall six patients used CIC to manage their bladder and 40% of continent patients used CIC. Urinary continence outcomes in patients who had partial vaginal replacement compared to those with total vaginal replacement did not reveal a clinically significant difference. Continence was achieved in 3/4 patients (75%) with a history of tethered cord compared to 7/9 patients (78%) without a history of tethered cord release. Urodynamics were performed postoperatively in 7 of the 157 patients (47%). Uninhibited detrusor contractions (UDCs) were present in 3 out of 7 patients, and a cystometric capacity greater than expected was noted in 4 patients. Additionally, 2 patients had end filling detrusor pressure greater than 40 cm H2O. GFR data were available for 13 of the 15 patients and (85%) were classified as chronic kidney disease (CKD) stage I or not having any significant loss of renal function.
In this cohort of rectovestibular fistula and absent vagina, 77% reported achieving urinary continence. However CIC was employed in 40% of the patients which is higher than prior published noncloaca female ARM patient population. Urodynamic abnormalities were noted when performed and led to change in bladder management. Renal function measured with GFR was normal in 85%. Patients with rectovestibular fistula and absent vagina benefit from urologic screening given higher rates of lower urinary tract dysfunction that can require CIC to protect the upper urinary tract and achieve urinary continence.
Case series.
Level IV.
肛门直肠畸形（ARM）代表了一组复杂的肛门直肠和泌尿生殖异常，所有 ARM 亚型的长期泌尿科结果的证据很少。我们作为 ARM 患者的转诊中心存在主观偏见，认为直肠阴道瘘和阴道缺如的亚型比典型的直肠阴道瘘患者有更高的肾脏和膀胱异常风险。因此，为了证实或反驳我们的临床怀疑，本研究的目的是回顾这一特定的 ARM 患者队列，并描述其临床泌尿科和尿动力学结果。
对 1991 年至 2017 年在我们机构接受 ARM 和阴道置换治疗的 120 名患者进行了回顾性队列研究。我们的研究包括 15 名直肠阴道瘘和阴道缺如的患者。从他们的病历中提取人口统计学和临床数据，包括尿动力学发现、间歇性清洁导尿（CIC）的需求、尿控和肾功能。
在 15 名患者中，有 10 名（67%）同时进行了阴道置换和 ARM 修复手术。1 名患者失访，术后平均随访 39 个月。除 1 名患者外，所有患者均使用直肠或结肠作为阴道置换的基质。在 15 名患者中，有 13 名患者有尿控数据。共有 10 名（77%）患者能够实现社交尿控。总体而言，有 6 名患者使用 CIC 来管理膀胱，40%的有尿控能力的患者使用 CIC。与完全阴道置换相比，部分阴道置换患者的尿控结果没有明显差异。在有或没有脊髓栓系松解史的 4 名患者中，有 3 名（75%）患者达到了尿控。在 15 名患者中的 7 名患者中进行了术后尿动力学检查，其中 3 名患者存在不自主逼尿肌收缩（UDC），4 名患者的膀胱容量大于预期。此外，2 名患者的充盈末期逼尿肌压力大于 40cmH2O。在 15 名患者中有 13 名患者的肾小球滤过率（GFR）数据可用，其中 85%（13/15）患者被归类为慢性肾脏病（CKD）I 期或肾功能无明显丧失。
在本队列的直肠阴道瘘和阴道缺如患者中，77%的患者报告实现了尿控。然而，40%的患者使用了 CIC，这一比例高于之前发表的非 Cloaca 女性 ARM 患者人群。当进行尿动力学检查时，发现了异常，导致了膀胱管理的改变。使用 GFR 测量的肾功能正常，占 85%。直肠阴道瘘和阴道缺如的患者需要进行泌尿科筛查，因为他们更容易出现下尿路功能障碍，需要使用 CIC 来保护上尿路并实现尿控。
病例系列。
四级。
