Zhang Zhibo,Li Xue,Kang Lei,et al.A primary study of female occult epispadias[J].Journal of Clinical Pediatric Surgery,2020,19(11):1002-1006.[doi:10.3969/j.issn.1671-6353.2020.11.008]
女性“隐蔽性”尿道上裂治疗初探
- Title:
- A primary study of female occult epispadias
- Keywords:
- Epispadias/SU; Urinary Incontinence; Female
- 分类号:
- R695;R695.1;R694+.54
- 摘要:
- 目的 探讨女性儿童外阴形态基本正常的完全性尿失禁病例发病情况、命名、病理改变特点、诊断和治疗特点。方法 回顾性分析2006年4月至2018年10月来四川大学华西医院治疗的14例完全性尿失禁女性患儿临床资料，并结合文献进行探讨。结果 14例平均年龄6岁（4~11岁），出生后即出现完全性尿失禁，未见阴唇和阴蒂的分裂，尿道外口存在不同程度的扩张，尿道膀胱镜检查均发现尿道短而宽大，尿道长度0.8~1.5 cm，平均（1.09±0.21）cm，膀胱黏膜血管正常，双侧输尿管口位置形态正常，膀胱容量50~120 mL，平均（79.00±20.25）mL。14例中11例采用经耻骨后的膀胱颈折叠成形术，其中早期的4例仅行膀胱颈折叠术；其后的7例均行膀胱折叠成形加膀胱颈悬吊术。早期4例仅行膀胱折叠成形术的病例中，有2例因疗效不佳术后1年再行膀胱折叠成形加膀胱颈悬吊术；另有3例行Young-Dees-Leadbetter膀胱颈成形术。14例16次手术均顺利完成（包括2例再次手术），术后恢复顺利如期出院，术后13例平均随访18个月（6~24个月），术前均为3级尿失禁，12例行膀胱颈折叠成形术患儿中，4例术后达到尿控0级，5例术后达到尿控1级，以上9例术后控尿满意；另2例仅行膀胱折叠术者仍为3级，1年后再行膀胱折叠加膀胱颈悬吊术后尿失禁缓解，控尿级别为1级，其中1例术后3个月失访；3例行Young-Dees-Leadbetter手术后，2例术后达到尿控1级，1例术后早期尿控2级，半年后经控尿训练达到1级。结论 由于对外阴改变轻微的女性尿道上裂认识不足，可能导致该类疾病的漏诊和误诊，因此女性尿道上裂的实际发病率可能明显高于以往的报道水平，外阴改变的程度并不一定与尿道括约肌受损的程度一致，这类"隐蔽性"尿道上裂诊断的要点是：对于外阴改变轻微的女孩完全性尿失禁应及时进行尿道膀胱镜检查，如果发现尿道短而宽大、膀胱颈扩张、膀胱容量小、膀胱出口漏点压很低时即需考虑该病；膀胱颈成形术是主要的治疗方法，膀胱颈折叠成形加膀胱颈悬吊术是治疗女性"隐蔽性"尿道上裂的有效手术方法。
- Abstract:
- Objective To explore the morbidity,nomenclature,pathologic characteristics,diagnosis and treatment of special female epispadias with slight vulva changes.Methods Retrospective reviews were conducted for 14 girls with primary urinary incontinence between April 2006 and October 2018.The mean age was 6(4-11) years.All patients showed complete incontinence (grade 3) since birth and apparently normal external genitalia.Yet urethral meatus became widened with a short urethra of a mean length of (1.09±0.21)(0.8-1.5) cm.Both mucosal and vascular bladders were normal.The position and appearance of bilateral ureteral orifice were normal.Bladder capacity declined markedly with a mean volume of (79.16±20.25)(50-120) ml.Eleven cases underwent bladder neck placation (BNP) via a retro-symphyseal approach.Four cases underwent BNP alone initially and bladder neck suspension (BNS) was added for two cases 1 year postoperatively due to unsatisfactory results.And BNP plus BNS (n=7) and Young-Dees-Leadbetter procedure (YDL,n=3) were performed.Results A total 16 operations (including 2 re-operations) were performed successfully.And all cases were discharged normally.The mean follow-up period of 13 cases was 18(6-24) months.Among 11 cases undergoing BNP,urinary continence was grade 0 (n=4) and grade 1 (n=5).All 9 cases achieved satisfactory urinary continence.Another two achieved grade 3 after BNP and improved to grade 1 after BNP plus BNS at Year 1.One case was lost to follow-ups at Month 3 after reoperation.Three cases underwent YDL procedure.Urinary continence was grade 1 (n=2) and grade 2 (n=1).The latter one improved to grade 1 after 1-year training.Conclusion Inadequate awareness of female epispadias with slight vulva changes may result in missed diagnosis and underestimated incidence of female epispadias.The actual incidence rate is obviously higher than the figure of previous reports.Vulva changes may not match with damage extent of urinary sphincter.The key examination of diagnosing female occult epispadias is cystourethroscopy revealing short and wide urethra,expended bladder neck,low capacity bladder and low bladder leak point pressure.Bladder neck plasty is a major treatment while a combination of BNP and BNS is an effective supplement.
参考文献/References:
1 Dees JE.Congenital epispadias with incontinence[J].Journal of Urology,1949,62(4):513-522.DOI:10.1111/j.1476-5381.1975.tb07333.x.
2 Allen L,Rodjani A,Kelly J,et al.Female epispadias:are we missing the diagnosis?[J].BJU Int,2004,94(4):613-615.DOI:10.1111/j.1464-4096.2004.04920.x.
3 Burki T,Hamid R,Ransley PG,et al.Injectable polydimethylsiloxane for treating incontinence in children with the exstrophy epispadias complex:long term results[J].BJU Int,2006,98(4):849-853.DOI:10.1111/j.1464-410X.2006.06378.x.
4 Nevéus T,von Gontard A,Hoebeke P,et al.The standardization of terminology of lower urinary tract function in children and adolescents:report from the Standardisation Committee of the International Children’s Continence Society[J].J Urol,2006,176(1):314-324.DOI:10.1016/S0022-5347(06)00305-3.
5 Kelly J.Exstrophy and epispadias:Kelly’s method of repair.In O’Neill JA Jr,Rowe MI,Grosfeld JL,Fonkalsrud EW,Coran AG eds,Pediatric Surgery,5th edn,Vol.2.Chapt 115[M].St.Louis:Mosby 1998:1732-1759.
6 Davis DM.Epispadias in females and the surgical treatment[J].Surg Gynecol Obstet,1928; 47:680-696.
7 Leach GE,Domchowski RR,Appell RA,et al.Female stress Urinary Incontinence Clinical Guidelines Panel summary report on surgical management of female stress urinary incontinence.The American Urological Association[J].J Urol,1997,158(3Pt 1):875-880.DOI:10.1097/00005392-199709000-00054.
8 Bhat AL,Bhat M,Sharma R,et al.Single-stage perineal urethroplasty for continence in female epispadias:a preliminary report[J].Urology,2008,72(2):300-303.DOI:10.1016/j.urology.2007.09.073.
9 Lazarus J,van den Heever A,Kortekaas B,et al.Female epispadias managed by Bladder neck placation via a perineal approach[J].J Pediatr Urol,2012,8(3):244-248.DOI:10.1016/j.jpurol.2011.06.006.
10 Kajbafzadeh AM,Talab SS,Elmi A,et al.Single-stage subsymphysed cystoscopic-guided bladder neck placation and urethrogenitoplasty in female epispadias:presentation of long-term follow-up[J].BJU Int,2011,108(7):1200-1207.DOI:10.1111/j.1464-410X.2010.10022.x.
11 Lazarus J,van den Heever A,Kortekaas B,et al.Female epispadias managed by Bladder neck placation via a perineal approach[J].J Pediatr Urol,2012,8(3):244-248.DOI:10.1016/j.jpurol.2011.06.006.
12 Kajbafzadeh AM,Talab SS,Elmi A,et al.Single-stage subsymphysedcystoscopic-guided bladder neck placation and urethrogenitoplasty in female epispadias:presentation of long-term follow-up[J].BJU Int,2011,108(7):1200-1207.DOI:10.1111/j.1464-410X.2010.10022.x.
13 Yadav SS,Agarwal N,Kumar S,et al.Single-stage female epispadias repair by combined infrasymphyseal bladder neck plication and urethrogenitoplasty:a novel technique[J].Urology,2017,100:240-245.DOI:10.1016/j.urology.2016.09.002.
14 Cheikhelard A,Aigrain Y,Lottmann H,et al.Female epispadias management:perineal urethrocervicoplasty versus classical Young-Dees procedure[J].Journal of Urology,2009,182(suppl):1807-1811.DOI:10.1016/j.juro.2009.04.087.
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备注/Memo
收稿日期:2020-08-18。
基金项目:四川省科技计划重点研发项目（编号：2017SZ0060）
通讯作者:黄鲁刚,Email:lugang992001@163.com