成年期出现完全性膀胱重复:一例难民重建技术的病例报告和对转化泌尿外科的思考

D. Abramowitz, W. Claeys, C. Jamaer, C. Berquin, P. Hoebeke, A. Spinoit
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引用次数: 1

摘要

介绍和研究目的膀胱出血性脑脊髓炎(BEEC)复合体是一系列具有多种变异的先天性畸形。一名从未与BEEC合作的政治难民在抵达欧洲后被转介到我们的管理中心。我们的目的是报告从未对BEEC成人进行过手术的技术和结果,强调成人先天畸形患者过渡期泌尿外科护理的重要性。材料和方法一名27岁的女性患者因出生后完全失禁被难民中心的全科医生转诊到我们中心,怀疑BEEC。在进一步的研究中,突出显示了一个具有盲端输尿管口的脱营养膀胱和脱营养膀胱板尾部的尿道口。第二个非出营养性膀胱有两个原位输尿管,从而诊断出矢状面上的膀胱重复,表现为膀胱颈大开口和7厘米的耻骨分离,导致她最初被转诊的失禁。患者处于仰卧位,进行剖腹探查,切除脐部瘢痕。从尾部解剖出营养不足的膀胱板。由于它具有良好的逼尿肌质量,因此决定将其用作腹侧嵌体,以增强非出营养性膀胱。Mitchell型膀胱颈重建术是用经典的筋膜吊带包裹在膀胱颈上进行的,以在耻骨间隙非常宽的情况下增加失禁机制。考虑到超控尿的风险,还可以进行Continental Mitrofanoff型膀胱造口术。实现了生殖器重建。结果术后1年多,患者完全干燥,在每天5次和夜间1次的导管插入术之间可注入250ml。未观察到术后并发症。结论介绍了一名患有罕见泌尿系统疾病(如重复膀胱膨出)的成年患者的病例,说明了欧洲政治难民在成年患者先天畸形手术方面面临的挑战。多学科方法非常重要,表明了过渡期护理的重要性。
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Complete bladder duplication presenting in adulthood: A case report on reconstructive technique and reflections on translational urology in refugees
Introduction and aim of study The bladder-Exstrophy-Epispadias (BEEC) complex is a spectrum of congenital malformations with many variations. A never operated political refugee with BEEC was referred to our center for management upon arrival in Europe. Our aim is to report the technique and outcomes on a never operated on BEEC adult, highlighting the importance of transitional urologic care for congenital malformations in adult patients. Materials and methods A 27-year old female patient was referred to our center for complete incontinence since birth by the General practitioner from the refugee center who suspected BEEC. Upon further investigation, an exstrophic bladder with blind ending ureteral orifices and a urethral meatus caudal to the exstrophic bladder plate were highlighted. A second non-exstrophic bladder with two orthotopic ureters was demonstrated, thereby a bladder duplication in the sagittal plane was diagnosed, presenting a wide-open bladder neck and a 7 cm pubic diastasis, causing the incontinence she was initially referred for. With the patient in a supine position, laparotomy incision was done with excision of the umbilical scar. The exstrophic bladder plate is dissected caudally. As it presents good detrusor quality, decision is taken to use it as a ventral inlay to augment the non-exstrophic bladder. A Mitchell-type bladder neck reconstruction is performed with a classical fascia sling wrapped around the bladder neck to increase the continence mechanism given the very wide pubic diastasis. Given the risk for hyper-continence, interposition of a continent Mitrofanoff-type vesicostomy is additionally realized. Genital reconstruction is achieved. Results Over 1 year post operatively, the patient is completely dry, can holp up to 250ml between catheterization she performs five times per day and once at night. No post-operative complications were observed. Conclusion The case of one adult patient with a rare urological condition like bladder exstrophy with duplication is presented, illustrating challenges political refugees referred to Europe implicates in terms of surgery regarding congenital malformations in adult patients. A multidisciplinary approach is highly important, demonstrating the importance of transitional care.
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