Prostatic Utricle

前列腺囊
  • 文章类型: Journal Article
    目的:探讨输尿管镜辅助腹腔镜手术(UALS)治疗儿童症状性前列腺囊(PU)的应用。材料与方法:回顾性分析湖南省儿童医院泌尿外科2014年9月至2022年9月收治的PU手术病例资料。诊断通过膀胱尿道镜检查,然后进行输尿管镜检查,输尿管镜辅助腹腔镜切除PU。结果:本研究共纳入21例PU患者。手术患者的中位年龄为8.1(4.6-11.5)岁。15名儿童可进行核型分析:13名(86.7%)为46XY,1(6.7%)为45X/46XY,1(6.7%)为45X/46XY/47XYY。PU的中值长度为5.0(4.1-7.1)cm。19例患者仅接受输尿管镜辅助腹腔镜切除术,而2例也有会阴切口。所有切除均成功进行。术中出血量中位数为25.0(20.0-37.5)mL。中位住院时间和随访时间分别为18.0(14.5-25.0)天和24.0(13.5-49.0)个月,分别。患者报告无术后临床症状。结论:UALS允许准确的患者定位和解剖结构的彻底暴露,这是一个保险箱,有效,儿童PU的微创治疗。
    Purpose: To investigate the use of ureteroscope-assisted laparoscopic surgery (UALS) in treating symptomatic prostatic utricle (PU) in children. Materials and Methods: Data on surgically treated cases of PU at the Department of Urology in Hunan Children\'s Hospital between September 2014 and September 2022 were retrospectively collected and analyzed. The diagnosis was confirmed by cystourethroscopy followed by ureteroscopy, and PU was excised by ureteroscope-assisted laparoscopy. Results: A total of 21 patients with PU were enrolled in this study. The median age of the patients at surgery was 8.1 (4.6-11.5) years. Karyotyping was available for 15 children: 13 (86.7%) were 46XY, 1 (6.7%) was 45X/46XY, and 1 (6.7%) was 45X/46XY/47XYY. The median length of the PU was 5.0 (4.1-7.1) cm. Nineteen patients underwent only ureteroscope-assisted laparoscopic excision, whereas 2 also had a perineal incision. All excisions were successfully performed. The median intraoperative blood loss was 25.0 (20.0-37.5) mL. The median hospital stay and follow-up durations were 18.0 (14.5-25.0) days and 24.0 (13.5-49.0) months, respectively. The patients reported no postoperative clinical symptoms. Conclusion: UALS allows for accurate patient positioning and thorough exposure of the anatomical structures, and it is a safe, effective, and minimally invasive treatment for PU in children.
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  • 文章类型: Case Reports
    46,XX男性性发育障碍罕见。大约80%的睾丸组织分化病例可能是由于SRY易位到X染色体或常染色体。SRY阴性46,XX男性显示睾丸前基因的过表达,如SOX9和SOX3,或前卵巢基因的失败,如WNT4和RSPO1,诱导睾丸分化,然而,几乎所有的睾丸都表现出发育不全。在胚胎阶段暴露于雄激素不足后,苗勒管的残留物和泌尿生殖窦的不完全闭合导致前列腺增大。这种情况与近端尿道下裂和性发育障碍有关。许多病例是无症状的,但显示术后并发症和手术失败的发生率增加。
    介绍了一个5岁的中国男孩,患有阴囊尿道下裂和双侧隐睾伴前列腺输尿管。性腺组织学显示右侧睾丸组织和左侧睾丸组织;所有睾丸组织均表现出发育不全。此外,染色体核型分析显示46,XX和,通过聚合酶链反应分析排除了SRY的存在.全基因组分析显示,该男孩在涉及SOX3的Xq27.1q27.2区域(arr[hg19]Xq27.1q27.2:139585794-140996652)中有1.4Mb重复。在父母中没有观察到SOX3重复,表型正常的人。
    我们报告了第一例SRY阴性的46XX男性,由SOX3重复引起的前列腺囊。SOX3重复可能会导致性别逆转,所有46,XXSRY阴性男性都应进行SOX3突变筛查。建议进行性腺活检以评估卵巢和睾丸组织的发育。睾丸发育不全和胎儿发育过程中男性荷尔蒙的低暴露会导致前列腺肥大。因此,术前应进行内窥镜检查,以检测SRY阴性46,XX男性的前列腺细胞,以确定手术计划并减少术后并发症。
    46,XX male disorders of sex development are rare. Approximately 80% of cases of testicular tissue differentiation may be due to translocation of SRY to the X chromosome or an autosome. SRY-negative 46,XX males show overexpression of pro-testis genes, such as SOX9 and SOX3, or failure of pro-ovarian genes, such as WNT4 and RSPO1, which induces testis differentiation, however, almost all testicles exhibit dysgenesis. Following inadequate exposure to androgens during the embryo stage, remnants of the Mullerian duct and incomplete closure of the urogenital sinus lead to enlargement of prostatic utricles. This condition is associated with proximal hypospadias and disorders of sex development. Many cases are asymptomatic, but show increased rates of postoperative complications and surgical failure.
    A 5-year-old Chinese boy with scrotal hypospadias and bilateral cryptorchidism with prostatic utricles was presented. Gonadal histology showed ovo-testicular tissue on the right side and testicular tissue on the left side; all testicular tissue exhibited dysgenesis. Furthermore, chromosome karyotype analysis revealed 46,XX and, the presence of SRY was ruled out by polymerase chain reaction analysis. Whole-genome analysis showed the boy has a 1.4-Mb duplication in the Xq27.1q27.2 region (arr[hg19]Xq27.1q27.2:139585794-140996652) involving SOX3. No SOX3 duplication was observed in the parents, who had a normal phenotype.
    We report the first case of an SRY-negative 46 XX male with prostatic utricle caused by SOX3 duplication. SOX3 duplication may cause sex reversal, and all 46,XX SRY-negative males should be screened for SOX3 mutations. Gonadal biopsy is recommended to evaluate ovarian and testicular tissue development. Testicular dysgenesis and low exposure to male hormones during fetal development can lead to enlarged prostatic utricles. Thus endoscopic examination should be performed preoperatively to detect prostatic utricles in SRY-negative 46,XX males to determine the surgical plan and reduce postoperative complications.
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  • 文章类型: Comparative Study
    OBJECTIVE: The aims of this study were to report our clinical experience with laparoscopic excision (LE) and to compare the outcomes of LE versus open transvesical excision (OTE) for the management of prostatic utricle (PU) in children.
    METHODS: This was a retrospective single-center study of 14 children who underwent OTE or LE for managing symptomatic PU between April 2003 and December 2014. Age, utricle size, operative time, estimated blood loss, duration of hospital stay, indwelling time of the urethral catheter, presence of residual postoperative utricular stump, and complications were compared between the two groups.
    RESULTS: There were no significant differences in age or utricle size between the two groups. Compared to the OTE group, the LE group experienced shorter operative times, lower estimated blood losses, and shorter hospital stays. Indwelling time of the urethral catheter was nearly 8days in the OTE group and 6days in the LE group. All patients had a follow-up visit between 6months and 2years after surgery. Two patients in the OTE group had transient UTI. Postoperative VCUG showed minimal residual utricular stump for 3 patients in the OTE group. However, no patient in either group required further operative therapy.
    CONCLUSIONS: LE is a safe and feasible procedure for symptomatic PU in children. Compared to OTE, LE can provide minimally invasive access for achieving good exposure with good short-term outcomes.
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