A case of right hypodysplastic kidney and ectopic ureter associated with bicornuate uterus in a prepubertal girl
A case of right hypodysplastic kidney and ectopic ureter associated with bicornuate uterus in a prepubertal girl
复制标题
青春期前女孩右肾发育不良及异位输尿管合并双角子宫一例
DOI：
10.1007/s13730-022-00730-1
复制
发表时间：
2022
期刊：
影响因子：
1
通讯作者：
Harita Yutaka
中科院分区：
文献类型：
--
作者：
Nakamura Misako;Kanda Shoichiro;Kajiho Yuko;Hinata Munetoshi;Tomonaga Kotaro;Fujishiro Jun;Harita Yutaka
先天性肾和尿路畸形(CAKUT)常与苗勒氏畸形相关。这可以用这样一个事实来解释：在胚胎发育过程中，米勒管的伸长依赖于预先形成的沃尔菲管。虽然单侧肾发育不全和多囊肾发育不良等CAKUT通常通过常规超声在产前诊断，但苗勒氏畸形的诊断往往被推迟，增加了并发症(如子宫内膜异位症或盆腔炎)的风险。在此，我们报告一例月经前女孩，最初被诊断为右多囊发育不良肾。她在4岁时表现为持续性尿失禁，通过增强CT、膀胱镜、阴道镜、输尿管造影和子宫输卵管造影术的进一步评估，最终诊断为右肾发育不良和异位输尿管合并双角子宫。强烈的子宫畸形家族史促使对子宫进行检查。有人建议进行基因测试，但这家人拒绝了。她计划在青春期被转介到妇科医生那里进行进一步的评估。CAKUT患者并发苗勒氏异常的识别率和筛查率因机构而异。对预诊的CAKUT女孩进行苗勒氏异常筛查可以提供及时的咨询和预防妇科并发症。
Congenital anomalies of the kidney and urinary tract (CAKUT) are frequently associated with Mullerian anomalies. This can be explained by the fact that Mullerian duct elongation depends on the preformed Wolffian duct during embryogenesis. While CAKUT such as unilateral renal agenesis and multicystic dysplastic kidney are commonly identified prenatally by routine ultrasound, the diagnosis of Mullerian anomalies is often delayed, increasing the risk of complications such as endometriosis or pelvic inflammatory disease. Herein, we report a case of a premenarchal girl who had initially been diagnosed with right multicystic dysplastic kidney. She presented with continuous urinary incontinence at 4 years old and further evaluation by contrast-enhanced computed tomography, cystoscopy, colposcopy, ureterography, and hysterosalpingography led to the final diagnosis of right hypodysplastic kidney and ectopic ureter associated with bicornuate uterus. A strong family history of uterine malformations prompted the examination of the uterus. Genetic testing was suggested but the family declined. She is planned to be referred to a gynecologist at puberty for further assessment. The recognition and screening rate of concurrent Mullerian anomalies in CAKUT patients varies between institutions. Screening for Mullerian anomalies in prediagnosed CAKUT girls may enable to provide timely counseling and to prevent gynecological complications.
登录
查看更多内容
影响因子：
19.6
作者：
Faguer, Stanislas;Chassaing, Nicolas;Chauveau, Dominique
通讯作者：
Chauveau, Dominique
影响因子：
2.4
作者：
Tan, Yu Guang;Laksmi, Narasimhan K.;Ong, Caroline C. P.
通讯作者：
Ong, Caroline C. P.
影响因子：
1.8
作者：
O'Brien, Katherine L. O'Flynn;Bhatia, Vinaya;Dietrich, Jennifer E.
通讯作者：
Dietrich, Jennifer E.
影响因子：
8
作者：
Capito, Carmen;Echaieb, Anis;Nihoul-Fekete, Claire
通讯作者：
Nihoul-Fekete, Claire
先天性单侧肾发育不全
影响因子：
9
作者：
D. Collins
通讯作者：
D. Collins