Robert D. Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institutions, Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Charlotte Bloomberg Children's Hospital, Baltimore, MD, USA.
Robert D. Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institutions, Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Charlotte Bloomberg Children's Hospital, Baltimore, MD, USA.
J Pediatr Urol. 2018 Oct;14(5):427.e1-427.e7. doi: 10.1016/j.jpurol.2018.03.023. Epub 2018 May 10.
Newborns with classic bladder exstrophy (CBE) may present with a bladder template that is inadequate for closure in the neonatal period (figure). In these cases, a delayed primary closure (DPC) is conducted to permit growth of the bladder template. This study reports the surgical and long-term urinary continence outcomes of poor template CBE patients undergoing DPC and compares them to patients who underwent DPC for reasons unrelated to bladder quality (i.e., prematurity, comorbidities, or a late referral).
An institutionally approved, prospectively maintained database of 1330 exstrophy-epispadias complex patients was reviewed for CBE patients who underwent DPC at the authors' institution. A bladder template was considered inadequate for neonatal closure if found to be inelastic, <3 cm in diameter, and/or covered in hamartomatous polyps.
In total, 63 patients (53 male and 10 female) undergoing DPC were identified. Of these, 36 had poor bladder templates (group 1). The remaining 27 patients (group 2) had adequate templates and their bladder closure was delayed for reasons unrelated to bladder quality. At the time of DPC, those in group 1 were relatively than those in group 2 (median of 229 vs. 128 days, p = 0.094). All 36 group 1 patients and 26 (96%) group 2 patients underwent pelvic osteotomy during DPC (p = 0.429). All patients in this study had a successful primary closure. There was little difference in longitudinal bladder capacities between group 1 and group 2 (p = 0.518). Also, there was minimal difference in the median number of continence procedures between groups, with both groups having 1 (IQR 1-1) continence procedure (p = 0.880). Eight patients in group 1, and three patients in group 2 underwent a bladder neck transection with urinary diversion. Of the 13 and 16 patients who have undergone a continence procedure in group 1 and 2, respectively, 11 (84.6%) and 13 (81.3%) are continent of urine. The age of first continence procedure was different between groups 1 and 2 at 8.0 years (5.8-9.9 years) and 4.8 (3.5-6.0 years), respectively p = 0.009. The majority of patients in group 1 established continence at a relatively later age when compared to those in group 2, at 11.4 (8.0-14.8) years and 7.9 (2.6-13.2) years of age respectively p = 0.087.
In the authors' view, neonatal bladder closure is ideal for CBE patients as it minimizes potential damage to exposed bladder mucosa. However, prior studies indicate that the rate of bladder growth for patients undergoing a delayed primary closure does not differ from patients with a neonatal closure. Results from this study show continued evidence that patients with poor templates who undergo delayed closure have excellent primary closure outcomes, which is critical for further management. Furthermore, this study shows that an inadequate bladder does not affect DPC outcomes or the continence outcomes in DPC patients. However, the inadequate template does affect the type of continence procedure available to a DPC patient, the age of first continence procedure, and the age of continence.
DPC of the exstrophic bladder has a high rate of success when pelvic osteotomy is utilized as an adjunct. Patients having a DPC for reasons of an inadequate bladder template have comparable rates of bladder growth when compared to DPC of an adequate bladder template. The inadequate bladder template affects the type of continence procedure, with the majority of patients requiring urinary diversion for continence. Patients with an inadequate bladder template have a later age of first continence procedure and a relatively later age of continence, because of an inherently smaller bladder template at birth. The inadequate bladder template patients require a longer period of surveillance to access bladder growth and capacity in preparation of a continence procedure. Furthermore, as the majority of inadequate bladder template patients require a catheterizable channel for continence, the age of continence is also likely influenced by the patient's preparation as they transition from volitional voiding to catheterization.
患有经典膀胱外翻(CBE)的新生儿可能会出现膀胱模板不足,无法在新生儿期进行闭合(图)。在这些情况下,会进行延迟性初次闭合(DPC),以允许膀胱模板生长。本研究报告了因膀胱质量差而接受 DPC 的不良模板 CBE 患者的手术和长期尿控效果,并将其与因其他原因(即早产、合并症或延迟转诊)接受 DPC 的患者进行了比较。
对作者机构的 1330 例膀胱外翻-尿道上裂复合畸形患者进行了机构批准的前瞻性维护数据库回顾,以确定接受 DPC 的 CBE 患者。如果发现膀胱模板无弹性、直径<3cm 和/或覆盖有错构瘤,则认为膀胱模板不适合新生儿闭合。
共确定 63 例(53 例男性和 10 例女性)接受 DPC。其中 36 例膀胱模板较差(1 组)。其余 27 例(2 组)有足够的模板,其膀胱闭合是由于与膀胱质量无关的原因而延迟。在 DPC 时,1 组的中位数(范围)比 2 组的中位数(范围)晚(229 天[164-295 天] vs. 128 天[85-155 天],p=0.094)。1 组的所有 36 例患者和 2 组的 26 例患者(96%)均在 DPC 期间接受了骨盆截骨术(p=0.429)。本研究中的所有患者均成功进行了初次闭合。1 组和 2 组之间的纵向膀胱容量差异不大(p=0.518)。两组之间的控尿程序中位数也几乎没有差异,两组均有 1 次(IQR 1-1)控尿程序(p=0.880)。1 组 8 例和 2 组 3 例患者接受了膀胱颈横断和尿路分流术。在 1 组和 2 组分别进行了 13 次和 16 次控尿手术的患者中,分别有 11 例(84.6%)和 13 例(81.3%)患者有尿控能力。1 组和 2 组首次控尿手术的年龄分别为 8.0 岁(5.8-9.9 岁)和 4.8 岁(3.5-6.0 岁),p=0.009。与 2 组相比,1 组的大多数患者在相对较晚的年龄时建立了控尿能力,分别为 11.4 岁(8.0-14.8 岁)和 7.9 岁(2.6-13.2 岁),p=0.087。
作者认为,新生儿膀胱闭合术对于 CBE 患者是理想的,因为它最大限度地减少了对暴露的膀胱黏膜的潜在损伤。然而,先前的研究表明,接受延迟性初次闭合的患者的膀胱生长速度与接受新生儿闭合的患者没有差异。本研究结果进一步证明,接受延迟闭合的膀胱模板不良的患者具有出色的初次闭合效果,这对于进一步的管理至关重要。此外,本研究表明,膀胱不足不会影响 DPC 结果或 DPC 患者的控尿结果。然而,膀胱不足确实会影响 DPC 患者可用的控尿程序类型、首次控尿程序的年龄以及控尿的年龄。
当骨盆截骨术作为辅助手段时,DPC 治疗膀胱外翻的成功率很高。因膀胱模板不足而进行 DPC 的患者与因膀胱模板充足而进行 DPC 的患者相比,膀胱生长速度相当。膀胱模板不足会影响控尿程序的类型,大多数患者需要尿路分流术进行控尿。膀胱模板不足的患者首次控尿的年龄和控尿的年龄较晚,因为出生时膀胱模板较小。膀胱模板不足的患者需要更长的监测时间来评估膀胱生长和容量,以便为控尿程序做好准备。此外,由于大多数膀胱模板不足的患者需要可导管化的通道来进行控尿,因此控尿的年龄也可能受到患者从自主排尿过渡到导管化的准备情况的影响。