Metts J C, Kotkin L, Kasper S, Shyr Y, Adams M C, Brock J W
Department of Urology, Vanderbilt Children's Hospital, Nashville, Tennessee, USA.
J Urol. 1997 Sep;158(3 Pt 2):1298-300. doi: 10.1097/00005392-199709000-00168.
Urinary tract malformations have been described in up to 50% of patients with imperforate anus but genital anomalies have been less well analyzed in the same patient population. We sought to evaluate the frequency of coexisting genital, urinary and spinal malformations in a population with imperforate anus.
We reviewed 128 cases of imperforate anus, focusing on genital malformations and their relationship to anomalies of the urinary tract and spine. The series included 69 boys and 59 girls with low (59), intermediate (22) and high (47) imperforate anus.
Boys were much more likely to have a genital or upper urinary tract problem than girls (26 or 50 versus 5 or 30%, respectively). The risk for both sets of problems increased with the level of the anorectal lesion. The incidence of genital malformations increased from 14 to 26% in the presence of a renal lesion and from 10 to 23% with an associated spinal problem. Conversely, the incidence of urinary and spinal anomalies increased in patients with genital malformations.
The genitalia of children with imperforate anus should be carefully examined. Patients with anorectal malformations should also be evaluated for urinary tract and spinal problems. The yield of such studies increases in patients with recognized genital malformations.
在高达50%的肛门闭锁患者中已发现存在尿路畸形，但在同一患者群体中，生殖器畸形的分析较少。我们试图评估肛门闭锁患者中并存生殖器、尿路和脊柱畸形的发生率。
我们回顾了128例肛门闭锁病例，重点关注生殖器畸形及其与尿路和脊柱畸形的关系。该系列包括69名男孩和59名女孩，肛门闭锁程度为低位（59例）、中位（22例）和高位（47例）。
男孩出现生殖器或上尿路问题的可能性远高于女孩（分别为26%或50%对5%或30%）。两组问题的风险均随肛门直肠病变程度增加而升高。存在肾脏病变时，生殖器畸形的发生率从14%增至26%，伴有脊柱问题时从10%增至23%。相反，生殖器畸形患者中尿路和脊柱异常的发生率增加。
应对肛门闭锁患儿的生殖器进行仔细检查。肛门直肠畸形患者还应评估是否存在尿路和脊柱问题。对于已确诊生殖器畸形的患者，此类检查的阳性率会增加。
