泄殖腔外翻
| Cloacal exstrophy | |
|---|---|
| 又称 | Omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome |
| 类型 | bladder exstrophy-epispadias-cloacal exstrophy complex[*] |
| 分类和外部资源 | |
| 医学专科 | 医学遗传学 |
| ICD-11 | LB17.3 |
| OMIM | 258040 |
| Orphanet | 93929 |
泄殖腔外翻（ CE ）是一种严重的先天性缺陷，导致大部分腹部器官（如膀胱和肠道）外露。该缺陷通常会引起膀胱、生殖器官和肛门的分裂。它有时也被称为 OEIS 综合征。 [1]
诊断检查可能包括超声波、排尿性膀胱尿道造影（VCUG）、静脉肾盂造影（IVP）、核素肾图检查、计算机断层扫描（CT扫描）和磁共振成像（MRI）。[2]泄殖腔外翻是一种罕见的先天性缺陷，在妊娠中发生率为 1/200,000，在活产中发生率为 1/400,000。该缺陷与腹侧体壁发育不良或闭合缺陷有关，可能由胚胎发育过程中中胚层的迁移受阻引起。[3]此缺陷常与脊柱裂和肾脏异常等其他先天性缺陷并存。许多患有XY泄殖腔外翻的个体，由于外生殖器的外观而在出生时被指定为女性。[4][5]世界卫生组织认为，仅出于美容目的对间性婴儿进行生殖器手术是一种侵犯人权的行为。 [6][7]
参考文献
[编辑]- ^ OMIM Entry - 258040 - OEIS COMPLEX. omim.org. [2018-01-29] （美国英语）.
- ^ G/U Imaging:Home. www.meddean.luc.edu. [2018-01-29].
- ^ Hassan, Moaied A. OEIS complex with a vesico-enteric fistula. Journal of Pediatric Surgery Case Reports. May 2018, 35: 45–47. doi:10.1016/j.epsc.2018.05.016
.
- ^ Myers, C.; Lee, P.A. Communicating with Parents with Full Disclosure: A Case of Cloacal Extrophy with Genital Ambiguity. Journal of Pediatric Endocrinology and Metabolism. March 1, 2004, 17 (3): 273–280. PMID 15112903. S2CID 23729036. doi:10.1515/JPEM.2004.17.3.273 –通过www.degruyter.com.
- ^ Alice D. Dreger; April M. Herndon. Progress and Politics in the intersex rights movement, Feminist theory in action (PDF).
- ^ Carpenter, Morgan. Intersex Variations, Human Rights, and the International Classification of Diseases. Health and Human Rights. December 2018, 20 (2): 205–214. PMC 6293350 . PMID 30568414.
- ^ Greenberg, Julie A. Legal, ethical, and human rights considerations for physicians treating children with atypical or ambiguous genitalia. Seminars in Perinatology. June 2017, 41 (4): 252–255. PMID 28478089. doi:10.1053/j.semperi.2017.03.012.
