Kobata R, Tsukahara H, Takeuchi M, Hori C, Hiraoka M, Uchida T, Kotsuji F, Ito Y, Okada K, Sudo M
Department of Pediatrics, Fukui Medical School, Japan.
Acta Paediatr Jpn. 1997 Dec;39(6):705-9. doi: 10.1111/j.1442-200x.1997.tb03673.x.
A report is presented of a male infant with prune belly syndrome (PBS) in whom bladder enlargement was detected by ultrasound (US) as early as 13 weeks of gestation. Subsequent fetal US identified progressive urinary tract dilatation, ascites and oligohydramnios. At 22 weeks, the fetal bladder was drained under US guidance. A gradual resolution of oligohydramnios was detected on US performed after 26 weeks of gestation. Delivery by cesarean section was performed at 34 weeks of gestation. The newborn had typical features of PBS with a vesico-cutaneous fistula but did not show respiratory distress. Imaging studies showed hypoplastic left kidney, slightly dysplastic but functioning right kidney, megaureter, megacystis, vesicoureteral reflux and dilated prostatic urethra. The early detection of genitourinary system abnormalities and serial US suggest that a distal urethral obstruction may be the underlying mechanism of PBS. Spontaneous (or therapeutic) intra-uterine decompression of the bladder might ameliorate obstructive nephropathy and oligohydramnios, allowing adequate lung development.
本文报告了一名患有梅干腹综合征（PBS）的男婴，早在妊娠13周时通过超声（US）检测到膀胱增大。随后的胎儿超声检查发现泌尿系统逐渐扩张、腹水和羊水过少。在22周时，在超声引导下对胎儿膀胱进行引流。妊娠26周后进行的超声检查发现羊水过少逐渐缓解。妊娠34周时进行剖宫产分娩。新生儿具有PBS的典型特征，伴有膀胱皮肤瘘，但未出现呼吸窘迫。影像学检查显示左肾发育不全，右肾轻度发育异常但功能正常，巨输尿管、巨膀胱、膀胱输尿管反流和前列腺尿道扩张。泌尿生殖系统异常的早期检测和系列超声检查表明，远端尿道梗阻可能是PBS的潜在机制。膀胱的自发性（或治疗性）宫内减压可能改善梗阻性肾病和羊水过少，使肺部充分发育。
