PDF(Pubmed)
Abstract：
BACKGROUND: Congenital lower urinary tract obstruction (LUTO) is a rare but significant condition affecting fetal urinary tract development. LUTO has a range of etiologies, with posterior urethral valves (PUV) being the most common cause. The prenatal diagnosis of LUTO plays a crucial role in recognizing the condition and guiding management decisions. Prenatal ultrasound serves as the primary tool for identifying LUTO, with key findings including megacystis, bladder wall thickening, oligohydramnios, hydronephrosis, and the \'keyhole sign\' indicating dilatation of the posterior urethra. We present a case of congenital LUTO with a rare complication of spontaneous fetal bladder rupture and urinary ascites, treated by peritoneo-amniotic shunt placement.
METHODS: A 27-year-old pregnant Caucasian women was referred at 28 weeks of pregnancy due to the presence of megacystis and bilateral hydronephrosis on routine ultrasound and suspicion of LUTO. Repeat ultrasound at 29 weeks showed significant fetal ascites, oligohydramnios and resolution of megacystis and hydronephrosis, after which diagnosis of spontaneous bladder rupture was made. Despite ascites aspiration and amnio-infusion, there was persistent ascites and oligohydramnios. A peritoneo-amniotic shunt was placed with resolution of ascites and normalization of the amniotic fluid volume. At 35 weeks, relapse of the megacystis was observed with bilateral pyelectasis and oligohydramnios, possibly due to healing of the bladder rupture, after which elective cesarean section was planned. Cystography confirmed spontaneous healing of the bladder rupture and the presence of posterior urethral valves, which were resected in the neonatal period with cold knife incision. Total follow-up of 8 years continued to show positive ultrasonographic results and good renal function, but the child suffers from bladder dysfunction, manifesting as overactive bladder disease.
CONCLUSIONS: LUTO might lead to important renal dysfunction and pulmonary hypoplasia in case of increasing disease severity. Spontaneous bladder rupture might improve renal prognosis, acting as a pop-off mechanism by decompression of the urinary tract. However, fetal bladder rupture is rare and only few cases have been reported. Prenatal intervention can be considered for moderate or severe LUTO, but the benefit for long-term outcome remains uncertain and further studies are needed.
METHODS: A 27-year-old pregnant Caucasian women was referred at 28 weeks of pregnancy due to the presence of megacystis and bilateral hydronephrosis on routine ultrasound and suspicion of LUTO. Repeat ultrasound at 29 weeks showed significant fetal ascites, oligohydramnios and resolution of megacystis and hydronephrosis, after which diagnosis of spontaneous bladder rupture was made. Despite ascites aspiration and amnio-infusion, there was persistent ascites and oligohydramnios. A peritoneo-amniotic shunt was placed with resolution of ascites and normalization of the amniotic fluid volume. At 35 weeks, relapse of the megacystis was observed with bilateral pyelectasis and oligohydramnios, possibly due to healing of the bladder rupture, after which elective cesarean section was planned. Cystography confirmed spontaneous healing of the bladder rupture and the presence of posterior urethral valves, which were resected in the neonatal period with cold knife incision. Total follow-up of 8 years continued to show positive ultrasonographic results and good renal function, but the child suffers from bladder dysfunction, manifesting as overactive bladder disease.
CONCLUSIONS: LUTO might lead to important renal dysfunction and pulmonary hypoplasia in case of increasing disease severity. Spontaneous bladder rupture might improve renal prognosis, acting as a pop-off mechanism by decompression of the urinary tract. However, fetal bladder rupture is rare and only few cases have been reported. Prenatal intervention can be considered for moderate or severe LUTO, but the benefit for long-term outcome remains uncertain and further studies are needed.
摘要：
背景：先天性下尿路梗阻（LUTO）是一种罕见但严重影响胎儿尿路发育的疾病。LUTO有一系列的病因，后尿道瓣膜（PUV）是最常见的原因。LUTO的产前诊断在识别病情和指导管理决策中起着至关重要的作用。产前超声作为识别LUTO的主要工具，主要发现包括巨无霸，膀胱壁增厚，羊水过少,肾积水,和指示后尿道扩张的锁孔标志。我们介绍了一例先天性LUTO，罕见的并发症是自发性胎儿膀胱破裂和尿腹水，通过腹膜羊膜分流术治疗。
方法：一名27岁的妊娠白种人妇女在妊娠28周时被转诊，原因是在常规超声和怀疑LUTO的情况下，存在巨大囊肿和双侧肾积水。在29周时重复超声显示显著的胎儿腹水，羊水过少和巨大囊肿和肾积水的解决，之后诊断为自发性膀胱破裂。尽管腹水吸入和羊膜输注，有持续性腹水和羊水过少。进行腹膜羊膜分流术，以消除腹水并使羊水量正常化。35周时,在双侧肾盂扩张和羊水过少的情况下观察到巨细胞的复发，可能是由于膀胱破裂愈合,之后计划选择性剖宫产。膀胱造影证实膀胱破裂的自发愈合和后尿道瓣膜的存在。在新生儿期用冷刀切开切除。总随访8年，超声检查结果阳性，肾功能良好，但是孩子患有膀胱功能障碍，表现为膀胱过度活动症。
结论：LUTO可能在疾病严重程度增加的情况下导致重要的肾功能障碍和肺发育不全。自发性膀胱破裂可能改善肾脏预后，通过尿道减压充当弹脱机制。然而,胎儿膀胱破裂是罕见的，只有少数病例被报道。中度或重度LUTO可考虑进行产前干预，但长期结局的获益仍不确定,需要进一步研究.
方法：一名27岁的妊娠白种人妇女在妊娠28周时被转诊，原因是在常规超声和怀疑LUTO的情况下，存在巨大囊肿和双侧肾积水。在29周时重复超声显示显著的胎儿腹水，羊水过少和巨大囊肿和肾积水的解决，之后诊断为自发性膀胱破裂。尽管腹水吸入和羊膜输注，有持续性腹水和羊水过少。进行腹膜羊膜分流术，以消除腹水并使羊水量正常化。35周时,在双侧肾盂扩张和羊水过少的情况下观察到巨细胞的复发，可能是由于膀胱破裂愈合,之后计划选择性剖宫产。膀胱造影证实膀胱破裂的自发愈合和后尿道瓣膜的存在。在新生儿期用冷刀切开切除。总随访8年，超声检查结果阳性，肾功能良好，但是孩子患有膀胱功能障碍，表现为膀胱过度活动症。
结论：LUTO可能在疾病严重程度增加的情况下导致重要的肾功能障碍和肺发育不全。自发性膀胱破裂可能改善肾脏预后，通过尿道减压充当弹脱机制。然而,胎儿膀胱破裂是罕见的，只有少数病例被报道。中度或重度LUTO可考虑进行产前干预，但长期结局的获益仍不确定,需要进一步研究.
