Stein R, Hohenfellner K, Fisch M, Stöckle M, Beetz R, Hohenfellner R
Department of Urology, Johannes Gutenberg University, Mainz, Germany.
Eur J Pediatr. 1996 Aug;155(8):678-83. doi: 10.1007/BF01957152.
After primary bladder closure or urinary diversion, other factors apart from the reconstruction itself gain importance for individuals with the exstrophy-epispadias complex: social integration and, after reaching puberty, sexuality and fertility. Between 1968 and July 1994 115 patients with bladder exstrophy or incontinent epispadias underwent surgery at our institution. A total of 104 patients could be followed, 2 of whom died in the meantime. Of the remaining 102 patients 48 attend school, 4 are in college, 40 have completed or are currently undergoing vocational training, 3 are unemployed, 1 lives in a therapeutic centre and 6 are younger than 6 years of age. A total of 95% of the patients with continent urinary diversion are continent day and night, whereas only three of five patients with a sling plasty (incontinent epispadias) or with primary bladder closure followed by a Young-Dees procedure are continent. None of the patients showed deterioration or renal function. In 25 females the external genitalia were reconstructed. Fixation of the uterus was done in 13 to correct or prevent uterine prolapse. Of the 17 women older than 18 years of age with genital reconstruction, 16 are satisfied with the cosmetic result. All adults engage in sexual intercourse. Five women have delivered seven children by Caesarean section. Of the 35 male adults 32 underwent reconstruction of the external genitalia and 34 males achieve erection. One developed necrosis of the penis early in life following primary bladder closure performed at an outside hospital. Penile deviation was present in 11 of the 32 patients with genital reconstruction, which is distressing in only 2. Thirty patients are satisfied with the cosmetic result. After genital reconstruction 9 males developed epididymitis, necessitating two orchiectomies and three vasectomies. No patients with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas ejaculation was normal in the three men who did not undergo genital reconstruction and in two patients prior to post-pubertal reconstruction. Furthermore, two of these three men have fathered four children.
Education, occupation and social development of patients with urinary diversion are uneventful. The cosmetic results achieved by genital reconstruction are satisfactory. In female patients, antefixation of the uterus should be performed before or together with an introitusplasty to prevent uterine prolapse. In male patients, however, surgery is performed at the expense of fertility. Detailed discussions with the patients and their patients should include not only the question of primary bladder closure versus urinary diversion, but also the pros and cons of correction of the external and-in females-the internal genitalia.
在进行一期膀胱闭合或尿流改道后,除了重建手术本身,对于膀胱外翻 - 尿道上裂综合征患者,其他因素变得至关重要:社会融入,以及进入青春期后的性和生育能力。1968年至1994年7月期间,115例膀胱外翻或失禁性尿道上裂患者在我们机构接受了手术。总共104例患者得到随访,其中2例在此期间死亡。在其余102例患者中,48例上学,4例上大学,40例已完成或正在接受职业培训,3例失业,1例住在治疗中心,6例年龄小于6岁。总共95%的可控性尿流改道患者日夜可控,而接受吊带成形术(失禁性尿道上裂)或一期膀胱闭合加Young - Dees手术的5例患者中只有3例可控。没有患者出现肾功能恶化。25例女性患者进行了外生殖器重建。13例进行了子宫固定术以纠正或预防子宫脱垂。在17例年龄大于18岁且进行了生殖器重建的女性中,16例对美容效果满意。所有成年人都有性行为。5例女性通过剖宫产分娩了7个孩子。在35例成年男性中,32例进行了外生殖器重建,34例男性能够勃起。1例在外部医院进行一期膀胱闭合后早年出现阴茎坏死。在32例进行了生殖器重建的患者中,11例存在阴茎偏斜,其中只有2例为此苦恼。30例患者对美容效果满意。生殖器重建后,9例男性发生附睾炎,需要进行2例睾丸切除术和3例输精管切除术。没有进行外生殖器重建的患者能够正常射精或生育子女,而未进行生殖器重建的3例男性以及2例青春期后重建前的患者射精正常。此外,这3例男性中有2例已生育4个孩子。
尿流改道患者的教育、职业和社会发展情况良好。生殖器重建所取得的美容效果令人满意。对于女性患者,应在进行阴道口成形术之前或同时进行子宫前固定术以预防子宫脱垂。然而,对于男性患者,手术是以牺牲生育能力为代价的。与患者及其家属进行详细讨论时,不仅应包括一期膀胱闭合与尿流改道的问题,还应包括外生殖器以及女性内生殖器矫正的利弊。