[Concomitant extragenital malformations of female reproductive tract anomalies: analysis of 444 cases in Peking Union Medical College Hospital].

S Su, X M Bao, S Wang, N Chen, Z F Liu, D W Sun, J H Leng, Q B Fan, H H Shi, Z J Sun, J Chen, H Y Liu, X Yu, J J Zhang, Y Dai, J H Lang, L Zhu
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Abstract

Objective: To analyze the incidence and clinical phenotype of the concomitant extragenital malformations in the patients with female reproductive tract anomalies. Methods: A retrospective study was conducted using clinical data of hospitalized patients diagnosed with uterine, cervical, or vaginal malformations from January 2003 to December 2022 in Peking Union Medical College Hospital. The malformations were classified according to American Society for Reproductive Medicine müllerian anomalies classification 2021, and in each type, the incidence and specific manifestations of concomitant extragnital malformations were analyzed. Results: A total of 444 patients were included. The overall incidence of concomitant extragenital malformations was 43.5% (193/444), including urinary system, skeletal system, and other system malformations. Renal malformations on the obstructed side were present in all patients with oblique vaginal septum syndrome (100.0%, 78/78). The total incidence of concomitant extragnital malformations was as high as 8/11 in uterus didelphys, 43.5% (10/23) in unicornuate uterus, 33.6% (79/235) in Mayer-Rokitansky-Küster-Hauser syndrome, 18.8% (6/32) in septate uterus and 18.5% (12/65) in cervical agenesis. Urinary system malformations (30.6%, 136/444) and skeletal system malformations (13.5%, 60/444) were the most common concomitant malformations in all types, in which, unilateral renal agenesis and scoliosis were the most common. Conclusions: Urinary and skeletal system malformations are important features of female reproductive tract anomalies. Urologic ultrasonography and spinal roentgenogram are recommended for all patients with female reproductive tract anomalies.

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[女性生殖道畸形伴发的生殖道外畸形:北京协和医院 444 例病例分析]。
目的分析女性生殖道畸形患者合并生殖道外畸形的发生率和临床表型。方法:采用回顾性研究方法:利用北京协和医院2003年1月至2022年12月期间诊断为子宫、宫颈或阴道畸形的住院患者的临床资料进行回顾性研究。根据美国生殖医学会《2021年穆勒氏畸形分类》对畸形进行分类,并分析了每种畸形中同时伴有诊外畸形的发生率和具体表现。结果共纳入 444 例患者。伴随生殖器外畸形的总发生率为 43.5%(193/444),包括泌尿系统、骨骼系统和其他系统畸形。所有阴道斜隔综合征患者的梗阻侧均存在肾脏畸形(100.0%,78/78)。在双子宫畸形中,8/11的患者同时伴有宫外畸形;在单角子宫中,43.5%(10/23)的患者同时伴有宫外畸形;在Mayer-Rokitansky-Küster-Hauser综合征中,33.6%(79/235)的患者同时伴有宫外畸形;在子宫纵隔综合征中,18.8%(6/32)的患者同时伴有宫外畸形;在宫颈缺如综合征中,18.5%(12/65)的患者同时伴有宫外畸形。泌尿系统畸形(30.6%,136/444)和骨骼系统畸形(13.5%,60/444)是所有类型中最常见的并发畸形,其中单侧肾发育不全和脊柱侧弯最为常见。结论泌尿系统和骨骼系统畸形是女性生殖道畸形的重要特征。建议对所有女性生殖道畸形患者进行泌尿系统超声波检查和脊柱射线照相术。
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