Currarino G, Weinberg A
Department of Radiology, Children's Medical Center, Dallas, Texas 75235.
Pediatr Pathol. 1991 Mar-Apr;11(2):195-210. doi: 10.3109/15513819109064759.
We report 5 newborns with a contracted lesser pelvis, imperforate anus (severely stenotic and ectopic anus in 1 case), absent or rudimentary urinary tract, and defective or absent external genitalia, vagina, and uterus but normal gonads. The first 2 patients had small pelvic outlet syndrome (SPOS). The findings in the third patient, a possible transition to sirenomelia, were more severe and included malposition of the penis to the posterior sacral area. The fourth and fifth patients had sirenomelia. The remarkable similarity of many abnormalities observed in these cases and related material in the literature suggests that SPOS and sirenomelia may be part of the same malformation complex (SPOS/sirenomelia complex).
我们报告了5例新生儿,他们存在小骨盆收缩、肛门闭锁(1例为严重狭窄和异位肛门)、尿路缺失或发育不全,以及外生殖器、阴道和子宫有缺陷或缺失,但性腺正常。前2例患者患有小骨盆出口综合征(SPOS)。第三例患者的情况可能是向并腿畸形过渡,更为严重,包括阴茎移位至骶骨后部区域。第四和第五例患者患有并腿畸形。在这些病例中观察到的许多异常与文献中的相关资料有显著相似性,这表明SPOS和并腿畸形可能是同一畸形复合体(SPOS/并腿畸形复合体)的一部分。