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先天性泄殖腔畸形并发阴道积水破裂

Prenatal Rupture of Hydrocolpos in a Cloacal Malformation.

机构信息

Division of Pediatric General, Thoracic and Fetal Surgery, University of California Davis Medical Center, Sacramento, California, USA,

Department of Surgery, University of San Francisco- East Bay, Oakland, California, USA.

出版信息

Fetal Diagn Ther. 2023;50(3):158-164. doi: 10.1159/000530438. Epub 2023 Apr 21.

Abstract

INTRODUCTION

Cloacal dysgenesis occurs from failure of embryological division of urogenital sinus and hindgut, leading to a single common perineal opening for genitourinary and gastrointestinal tracts. The prenatal diagnosis of cloacal malformation is imprecise, but the clinical correlation of postnatal findings to prenatal history can help reveal explanations for unusual pathological findings in patients with urogenital abnormalities.

CASE PRESENTATION

A 21-year-old woman was referred after her 20-week ultrasound demonstrated anhydramnios and concern for dilated fetal bowel. Fetal MRI confirmed anhydramnios and a dilated fetal colon, in addition to hydronephrosis and a pelvic cyst. Repeat ultrasound at 27 weeks showed unexpected complete resolution of her anhydramnios but new fetal ascites. The newborn girl was postnatally diagnosed with a cloacal malformation and an unusual near-complete fusion of her labia. She underwent proximal sigmoid colostomy and a tube vaginostomy at birth followed by cloacal reconstruction at 1.5 years old.

CONCLUSION

In female fetus with a pelvic cyst, one should have a high index of suspicion for cloacal anomaly and consider the possibility of urinary obstruction leading to alteration in amniotic fluid.

摘要

引言

泄殖腔发育不全是由于尿生殖窦和后肠的胚胎分裂失败导致的,导致泌尿生殖道和胃肠道共用一个单一的会阴开口。直肠畸形的产前诊断并不准确,但将产后发现与产前病史进行临床相关联有助于揭示泌尿生殖系统异常患者异常病理发现的解释。

病例介绍

一名 21 岁女性因 20 周超声检查显示羊水过少和胎儿肠管扩张而转来我院。胎儿 MRI 证实羊水过少和胎儿结肠扩张,此外还有肾盂积水和骨盆囊肿。27 周时的重复超声检查显示,她的羊水过少意外完全缓解,但出现新的胎儿腹水。新生儿女孩被诊断为直肠畸形和阴唇异常近乎完全融合。她在出生时接受了近端乙状结肠造口术和阴道管造口术,然后在 1.5 岁时进行了直肠阴道重建术。

结论

对于有骨盆囊肿的女性胎儿,应高度怀疑直肠畸形,并考虑可能存在导致羊水改变的尿路梗阻。

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