Single-center outcome analysis of 46 fetuses with megacystis after intrauterine vesico-amniotic shunting with the Somatex®intrauterine shunt,Archives of Gynecology and Obstetrics

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Single-center outcome analysis of 46 fetuses with megacystis after intrauterine vesico-amniotic shunting with the Somatex®intrauterine shunt
Archives of Gynecology and Obstetrics ( IF 2.1 ) Pub Date : 2023-01-05 , DOI: 10.1007/s00404-022-06905-6
I Gottschalk ₁ , C Berg ₁ , T Menzel ₁ , J S Abel ₁ , A Kribs ₂ , M Dübbers ₃ , J Kohaut ₃ , L T Weber ₄ , C Taylan ₄ , S Habbig ₄ , M C Liebau ₄ , T M Boemers ₅ , E C Weber ₁

Affiliation

Objectives

To assess the spectrum of underlying pathologies, the intrauterine course and postnatal outcome of 46 fetuses with megacystis that underwent intrauterine vesico-amniotic shunting (VAS) with the Somatex® shunt in a single center.

Methods

Retrospective analysis of 46 fetuses with megacystis that underwent VAS either up to 14 + 0 weeks (early VAS), between 14 + 1 and 17 + 0 weeks (intermediate VAS) or after 17 + 0 weeks of gestation (late VAS) in a single tertiary referral center. Intrauterine course, underlying pathology and postnatal outcome were assessed and correlated with the underlying pathology and gestational age at first VAS.

Results

46 fetuses underwent VAS, 41 (89%) were male and 5 (11%) were female. 28 (61%) fetuses had isolated and 18 (39%) had complex megacystis with either aneuploidy (n = 1), anorectal malformations (n = 6), cloacal malformations (n = 3), congenital anomalies overlapping with VACTER association (n = 6) or Megacystis–Microcolon Intestinal–Hypoperistalsis Syndrome (MMIHS) (n = 2). The sonographic ‘keyhole sign’ significantly predicted isolated megacystis (p < 0.001). 7 pregnancies were terminated, 4 babies died in the neonatal period, 1 baby died at the age of 2.5 months and 34 (74%) infants survived until last follow-up. After exclusion of the terminated pregnancies, intention-to-treat survival rate was 87%. Mean follow-up period was 24 months (range 1–72). The underlying pathology was highly variable and included posterior urethral valve (46%), hypoplastic or atretic urethra (35%), MMIHS or prune belly syndrome (10%) and primary vesico-ureteral reflux (2%). In 7% no pathology could be detected postnatally. No sonographic marker was identified to predict the underlying pathology prenatally. 14 fetuses underwent early, 24 intermediate and 8 late VAS. In the early VAS subgroup, amnion infusion prior to VAS was significantly less often necessary (7%), shunt complications were significantly less common (29%) and immediate kidney replacement therapy postnatally became less often necessary (0%). In contrast, preterm delivery ≤ 32 + 0 weeks was more common (30%) and survival rate was lower (70%) after early VAS compared to intermediate or late VAS. Overall, 90% of liveborn babies had sufficient kidney function without need for kidney replacement therapy until last follow-up, and 95% had sufficient pulmonary function without need for mechanical respiratory support. 18% of babies with complex megacystis suffered from additional health restrictions due to their major concomitant malformations.

Conclusions

Our data suggest that VAS is feasible from the first trimester onward. Early intervention has the potential to preserve neonatal kidney function in the majority of cases and enables neonatal survival in up to 87% of cases. Despite successful fetal intervention, parents should be aware of the potential of mid- or long-term kidney failure and of additional health impairments due to concomitant extra-renal anomalies that cannot be excluded at time of intervention.

中文翻译：

Somatex®宫内分流术宫内膀胱羊膜分流术后 46 例巨囊胎儿的单中心结果分析

目标

为了评估 46 个巨囊胎儿的潜在病理谱系、宫内病程和出生后结局，这些胎儿在单一中心接受了 Somatex® 分流术的宫内膀胱羊膜分流术 (VAS)。

方法

对 46 个巨囊胎儿进行回顾性分析，这些胎儿在 14 + 0 周（早期 VAS）、14 + 1 至 17 + 0 周（中间 VAS）或妊娠 17 + 0 周后（晚期 VAS）接受了 VAS三级转诊中心。评估宫内病程、基础病理学和产后结局，并将其与首次 VAS 时的基础病理学和胎龄相关联。

结果

46 名胎儿接受了 VAS，其中 41 名（89%）为男性，5 名（11%）为女性。 28 个 (61%) 胎儿被分离，18 个 (39%) 患有复杂巨囊，伴有非整倍体 ( n = 1)、肛门直肠畸形 ( n = 6)、泄殖腔畸形 ( n = 3)、与 VACTER 关联重叠的先天性异常 ( n = 1) = 6) 或巨囊藻-小结肠肠-蠕动功能减退综合征 (MMIHS) ( n = 2)。超声检查“锁孔征”可显着预测孤立性巨囊肿 ( p < 0.001)。 7 例妊娠被终止，4 名婴儿在新生儿期死亡，1 名婴儿在 2.5 个月大时死亡，34 名（74%）婴儿存活至最后一次随访。排除终止妊娠后，意向治疗存活率为87%。平均随访期为 24 个月（范围 1-72）。潜在的病理变化很大，包括后尿道瓣膜（46%）、尿道发育不全或闭锁（35%）、MMIHS或梅干腹综合征（10%）和原发性膀胱输尿管反流（2%）。 7% 的人出生后未检测到任何病理变化。没有发现超声标记来预测产前潜在的病理学。 14 名胎儿接受了早期 VAS，24 名胎儿接受了中期 VAS，8 名胎儿接受了晚期 VAS。在早期 VAS 亚组中，VAS 前羊膜输注的必要性显着降低 (7%)，分流并发症的发生率显着降低 (29%)，产后立即进行肾脏替代治疗的必要性也显着降低 (0%)。相反，与中期或晚期 VAS 相比，早期 VAS 后 ≤ 32 + 0 周的早产更为常见 (30%)，且存活率较低 (70%)。总体而言，直到最后一次随访，90% 的活产婴儿具有足够的肾功能，无需进行肾脏替代治疗，95% 的活产婴儿具有足够的肺功能，无需机械呼吸支持。 18% 的患有复杂巨囊肿的婴儿由于伴有严重的畸形而遭受额外的健康限制。