Frimberger Dominic, Gearhart John P, Mathews Ranjiv
Division of Pediatric Urology, Johns Hopkins Hospital, Baltimore, Maryland 21287-2101, USA.
J Urol. 2003 Dec;170(6 Pt 1):2428-31. doi: 10.1097/01.ju.0000090195.72919.f5.
Bladder exstrophy is a rare malformation affecting only 1 female out of every 5 patients. In the female initial closure is combined with reconstruction of the outer genitalia, and urinary continence can be achieved by some girls without the need for later bladder neck reconstruction. We evaluated the management and outcome of failed initial closures in the female exstrophy population.
We performed a retrospective database review of patients with the exstrophy complex. Females with classic bladder exstrophy with failure of initial closure were identified. Age at initial closure, use of osteotomies, reasons for failure and number of closures, as well as definitive treatment and long-term outcomes were evaluated.
Of 71 females with classic bladder exstrophy 14 had failure of initial closure. Of these patients 1 had undergone initial closure at our institution and 13 were referred for reclosure. Mean followup was 6.5 years (range 3 to 12) and mean age was 10 years (4 to 14). The patients underwent a maximum of 3 closures (mean 2.4). Initial osteotomies were performed in 4 patients, no osteotomy in 8 and status was unknown in 2. Reason for initial failure was dehiscence in 11 patients and prolapse in 3. Five patients underwent a second closure elsewhere. On referral reclosure was successful using osteotomies in all patients. Bladder neck reconstruction was done in 5 patients (3 are daytime continent) and continent diversion in 4 (all are dry). The other patients are awaiting final treatment.
The single most important step to achieve urinary continence is successful initial bladder and posterior urethral closure. Pelvic osteotomies ensure a tension-free closure and enhance bladder outlet resistance. Radical mobilization of the vesicourethral complex allows placement of the bladder deep within the pelvis. Failure of the initial closure in the female exstrophy population has a severe impact on long-term outcome and quality of life.
膀胱外翻是一种罕见的畸形，每5例患者中仅有1例女性患病。在女性患者中，初次闭合手术需与外生殖器重建相结合，部分女孩无需后期膀胱颈重建即可实现尿失禁。我们评估了女性膀胱外翻患者初次闭合失败后的治疗及结果。
我们对膀胱外翻综合征患者进行了回顾性数据库分析。确定为初次闭合失败的典型膀胱外翻女性患者。评估初次闭合时的年龄、截骨术的使用情况、失败原因及闭合次数，以及最终治疗方法和长期结果。
71例典型膀胱外翻女性患者中，14例初次闭合失败。其中1例在我们机构接受了初次闭合手术，13例被转诊进行再次闭合。平均随访时间为6.5年（范围3至12年），平均年龄为10岁（4至14岁）。患者最多接受了3次闭合手术（平均2.4次）。4例患者进行了初次截骨术，8例未进行截骨术，2例情况不明。初次失败的原因是11例患者出现裂开，3例患者出现脱垂。5例患者在其他地方接受了第二次闭合手术。转诊后，所有患者再次闭合手术均成功采用了截骨术。5例患者进行了膀胱颈重建（3例白天能自主控制排尿），4例患者进行了可控性尿流改道（均无尿失禁）。其他患者正在等待最终治疗。
实现尿失禁的最重要一步是成功进行初次膀胱和后尿道闭合。骨盆截骨术可确保无张力闭合并增强膀胱出口阻力。膀胱尿道复合体的彻底游离可使膀胱置于骨盆深处。女性膀胱外翻患者初次闭合失败对长期结果和生活质量有严重影响。
