关键词: DSD Disorders of sex development Enlarged prostatic utricle Male vagina Prostatic utricle Proximal hypospadias Severe hypospadias Vagina masculinus

Mesh : Female Humans Male Infant Infant, Newborn Urethra Hypospadias Vagina Cysts Disorders of Sex Development Saccule and Utricle

来  源:   DOI:10.1007/s00345-022-04273-z

Abstract:
OBJECTIVE: To determine the prevalence, radiological characteristics, and clinical symptomatology of enlarged prostatic utricles and vagina masculinus in boys with disorders of sex development.
METHODS: Over 10 years (from February 2012 to March 2022), 102 boys with severe hypospadias underwent voiding cystourethrography. All patients presented with disorders of sex development and Y material in the karyotype (46,XY karyotype, 46,XY/45,X mosaic, etc.). The age of the patients at the first examination ranged from 4 days to 27 months (mean age 2.7 months).
RESULTS: Voiding cystourethrography revealed the presence of a cyst posterior to the urethra in 66 patients (64.7% of cases). There were 15 cases of \"vagina masculinus\" and 51 of enlarged prostatic utricles. These dilations were classified according to the Ikoma classification and cyst size. In more than 2/3 of cases, the cysts were small (less than 20 mm), and in less than 8% of cases, these cysts were large. In addition, retrograde opacification revealed the presence of vesicoureteral reflux in 20% of boys with a male vagina. The most severe hypospadias with a scrotal or perineal meatus are most at risk of developing an enlarged prostatic utricle, and 80% of patients with Ikoma Grade III had a scrotal or perineal meatus.
CONCLUSIONS: This study shows that the prevalence of enlarged prostatic utricles and vagina masculinus is high in patients with severe hypospadias. Therefore, their search should be systematic, and for clinical and therapeutic interest, the enlarged prostatic utricles should be classified according to cyst size.
摘要:
目的:为了确定患病率,放射学特征,以及性发育障碍男孩前列腺肥大和男性阴道的临床症状。
方法:超过10年(从2012年2月到2022年3月),102名患有严重尿道下裂的男孩接受了排尿膀胱尿道造影。所有患者均表现为性发育和Y材料的核型障碍(46,XY核型,46,XY/45,X马赛克,等。).首次检查时患者的年龄为4天至27个月(平均年龄2.7个月)。
结果:排尿膀胱尿道造影显示66例患者(占64.7%)尿道后方有囊肿。“阴道男性”15例,前列腺肥大51例。根据Ikoma分类和囊肿大小对这些扩张进行分类。在超过2/3的案例中,囊肿小(小于20毫米),在不到8%的案例中,这些囊肿很大。此外,逆行混浊显示20%的男性阴道男孩存在膀胱输尿管反流。最严重的尿道下裂伴阴囊或会阴道最容易发生前列腺囊增大,IkomaIII级患者中有80%患有阴囊或会阴道。
结论:本研究表明,重度尿道下裂患者中前列腺增大和男性阴道增大的患病率较高。因此,他们的搜索应该是系统的,为了临床和治疗的兴趣,前列腺增大应根据囊肿大小进行分类。
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