Surgical management of hypospadias in cases with concomitant disorders of sex development
Surgical management of hypospadias in cases with concomitant disorders of sex development
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DOI：
10.1007/s00383-019-04457-6
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发表时间：
2019-05-01
影响因子：
1.8
通讯作者：
Yamataka, Atsuyuki
中科院分区：
文献类型：
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作者：
Ochi, Takanori;Ishiyama, Asuka;Yamataka, Atsuyuki
目的探讨尿道下裂（HP）伴性发育障碍（DSD）的外科治疗方法。方法对例HP伴性发育障碍的患者，通过大体检查、激素水平测定、染色体核型分析等方法，判断其是否为DSD。1999年至2017年期间，共治疗了58例HP病例合并DSD。结果DSD为性染色体异常4例，其中46，XY 5 1例，46，XX 1例，47，XY+2 1例。HP为会阴：（n=26）、阴囊：（n=16）、阴茎阴囊：（n=15）和中段：（n=1）;修复为一期（n=6）或分期（n=52）。最终尿道成形术（UP）时的平均年龄为4.12 ± 0.21岁;所有病例在UP时均有软组织插入。在最终UP后平均随访5.16 +/-0.56年时，观察到UPC后（n=8; 13.8%）尿道狭窄（n=3）、尿道憩室（n=2）、尿道皮肤瘘（n=2）和弯曲（n=1）。UPC后平均发病时间为1.24 ± 0.77年（范围0.1-6.3）。我们病例的后半部分（n=29;治疗时间2015年相似）UPC后（0/29; 0%）显著低于前半部分（8/29; 27.6%）（p=0.0075）。结论虽然HP+DSD的UP具有巨大挑战性，但我们通过手术技术和经验的结合实现了UPC后的显著降低。
IntroductionTo review the surgical treatment of hypospadias (HP) associated with disorders of sex development (DSD).Patients and methodsHP cases were assessed for DSD by gross examination for atypical external genitalia, and assessment of hormone levels and karyotype. There were 58 HP cases with concomitant DSD treated between 1999 and 2017. DSD classification, type of HP, sex assignment, hormonal abnormality, surgical strategy, and post-urethroplasty complications (post-UPC) were reviewed.ResultsDSD were sex chromosome abnormalities (n=4), 46,XY (n=51), 46,XX (n=1), and 47,XY+21 (n=2). HP was perineal: (n=26), scrotal: (n=16), penoscrotal: (n=15), and midshaft: (n=1); repair was primary (n=6) or staged (n=52). Mean age at final urethroplasty (UP) was 4.120.21 years; all cases had soft tissue interposition at UP. At mean follow-up 5.16 +/- 0.56years after final UP, observed post-UPC (n=8; 13.8%) were urethral stenosis (n=3), urethral diverticulum (n=2), urethrocutaneous fistula (n=2), and curvature (n=1). Mean onset of post-UPC was 1.24 +/- 0.77years (range 0.1-6.3). The second half of our cases (n=29; treated 2015 similar to) had significantly less post-UPC (0/29; 0%) than the first half (8/29; 27.6%) (p=0.0075).Conclusions Although UP for HP+DSD was formidably challenging, we achieved a significant decrease in post-UPC through a combination of surgical techniques and experience.