Abramowitz David, Claeys Wietse, Jamaer Caroline, Berquin Camille, Hoebeke Piet, Spinoit Anne-Françoise
Department of Urology, University at Buffalo Jacobs School of Medicine, Buffalo, NY, United States.
Department of Urology, Ghent University Hospital, eUrogen Accredited Center European Reference Network (ERN), Ghent, Belgium.
Front Urol. 2023 Feb 21;3:1080410. doi: 10.3389/fruro.2023.1080410. eCollection 2023.
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.
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.
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.
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.
膀胱外翻 - 尿道上裂（BEEC）综合征是一系列具有多种变异的先天性畸形。一名从未接受过手术治疗的患有BEEC的政治难民抵达欧洲后被转诊至我们中心接受治疗。我们的目的是报告一名从未接受过手术治疗的BEEC成年患者的治疗技术及结果，强调成人患者先天性畸形过渡性泌尿外科护理的重要性。
一名27岁女性患者因自出生起就完全失禁被难民中心的全科医生转诊至我们中心，该医生怀疑其患有BEEC。进一步检查发现，有一个膀胱外翻且输尿管口呈盲端，尿道外口位于膀胱外翻板的尾侧。还发现了第二个非膀胱外翻的膀胱，有两条原位输尿管，从而诊断出矢状面膀胱重复畸形，伴有宽阔开放的膀胱颈和7厘米的耻骨分离，这导致了她最初因失禁前来就诊。患者仰卧位，行剖腹手术切口，切除脐部瘢痕。将膀胱外翻板向尾侧解剖。由于其逼尿肌质量良好，决定将其用作腹侧镶嵌物来扩大非膀胱外翻的膀胱。鉴于耻骨分离非常宽，采用经典的筋膜吊带环绕膀胱颈进行米切尔式膀胱颈重建，以增强控尿机制。鉴于存在控尿过度的风险，还额外实施了可控性米氏型膀胱造瘘术。完成了生殖器重建。
术后1年多来，患者完全干爽，每天自行导尿5次、夜间1次，每次导尿时可容纳250毫升尿液。未观察到术后并发症。
本文介绍了一名患有罕见泌尿系统疾病（如膀胱外翻伴重复畸形）的成年患者的病例，说明了转诊至欧洲的政治难民在成人患者先天性畸形手术方面所面临的挑战。多学科方法非常重要，体现了过渡性护理的重要性。
