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产前同时存在的脐膨出和巨大肝囊肿最初被怀疑为脐带囊肿:一例报告

Concurrent Prolapsed Omphalocele and Giant Hepatic Cyst Initially Suspected as an Umbilical Cord Cyst in the Prenatal Period: A Case Report.

作者信息

Takeuchi Yuta, Inoue Seiichiro, Muta Yuki, Sawada Keisuke, Hayashi Taisuke, Kawaguchi Kohei, Odaka Akio

机构信息

Department of Pediatric Surgery, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.

Department of Pathology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.

出版信息

Surg Case Rep. 2025;11(1). doi: 10.70352/scrj.cr.24-0006. Epub 2025 Sep 19.

Abstract

INTRODUCTION

Umbilical cord cysts detected after the 2nd trimester of pregnancy are associated with a variety of complications, including omphalocele, and thus require careful monitoring. Congenital hepatic cysts are rare, as are reports of their coexistence with omphaloceles. Herein, we present an unusual case of an omphalocele complicated by a large hepatic cyst that was initially suspected to be an umbilical cord cyst during the fetal period.

CASE PRESENTATION

A male infant was delivered via caesarean section at 36 weeks of age, with ruptured membranes and cloudy amniotic fluid. Fetal ultrasonography at 16 weeks had previously revealed an omphalocele with intestinal prolapse, and at 20 weeks, an umbilical cord cyst was suspected. At birth, the herniation sac ruptured, and abdominal wall closure and cyst excision were performed. The cyst, initially thought to be umbilical in origin, was instead identified as a hepatic cyst connected to the liver. Pathological examination confirmed the diagnosis. The infant was further diagnosed with Beckwith-Wiedemann syndrome, and was ultimately discharged 32 days postoperatively, with no recurrence to date.

CONCLUSIONS

Although asymptomatic congenital liver cysts can often be managed conservatively with follow-up, we considered surgical intervention necessary in this case due to the presence of an omphalocele associated with suspected Beckwith-Wiedemann syndrome.

摘要

引言

妊娠中期后检测到的脐带囊肿与多种并发症相关,包括脐膨出,因此需要仔细监测。先天性肝囊肿很少见,其与脐膨出并存的报道也很少。在此,我们报告一例罕见病例,一名患有巨大肝囊肿的脐膨出患儿,在胎儿期最初被怀疑为脐带囊肿。

病例报告

一名男婴在36周时通过剖宫产出生,胎膜破裂,羊水浑浊。16周时的胎儿超声检查先前发现有脐膨出伴肠脱垂,20周时怀疑有脐带囊肿。出生时,疝囊破裂,进行了腹壁闭合和囊肿切除。最初认为起源于脐带的囊肿,实际上被确定为与肝脏相连的肝囊肿。病理检查确诊。该婴儿进一步被诊断为贝克威思-维德曼综合征,最终术后32天出院,至今无复发。

结论

虽然无症状的先天性肝囊肿通常可以通过随访保守治疗,但由于该病例存在与疑似贝克威思-维德曼综合征相关的脐膨出,我们认为手术干预是必要的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d1bb/12457713/a00b3abc97a7/scr-11-01-24-0006-g001.jpg

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