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一名9岁患有肠旋转不良女孩的大量乳糜性腹水——病例报告

Massive Chylous Ascites in a 9-Year-Old Girl with Malrotation-A Case Report.

作者信息

Winberg Hans, Gerwins Pär, Hagelsteen Kristine

机构信息

Pediatrics, Department of Clinical Sciences Lund, Lund University, Lund, Sweden.

Department of Pediatric Surgery, Skånes Universitetssjukhus Lund, Lund, Sweden.

出版信息

European J Pediatr Surg Rep. 2024 Jan 10;12(1):e1-e3. doi: 10.1055/a-2221-9682. eCollection 2024 Jan.

Abstract

Malrotation leading to massive chylous ascites is rare. A 9-year-old girl was investigated for slowly increasing abdominal distension under a year. She had no vomiting, weight loss, or pain, but was bothered in social situations. Medical investigations, including ultrasound and computed tomography scans, revealed massive ascites. Laparocentesis yielded milk-colored fluid, confirmed as lymph through laboratory analysis. A complete blood count, liver function and hematologic parameters, chyle cytology, bacterial cultures, and polymerase chain reaction for tuberculosis were all within normal limits. She was referred to a tertiary center for vascular anomalies. A dynamic contrast-enhanced magnetic resonance lymphangiography showed normal lymphatic anatomy without leakage or flow obstruction. A whole-body magnetic resonance imaging revealed a central mesenteric rotation. She was referred to a tertiary center for pediatric surgery, where a laparoscopic Ladd's procedure was performed using a new 5 mm pediatric sealing device, along with an appendectomy using a 5 mm stapler. To derotate the bowel, fenestrations were created in compartments containing a substantial amount of chyle and ascites, resulting in the drainage of 2.4 L of fluid. She was discharged the day after surgery and has been in good health for 1 year. We present a video illustrating the Ladd's procedure steps in this patient.

摘要

导致大量乳糜性腹水的肠旋转不良较为罕见。一名9岁女孩因一年内腹部逐渐膨隆而接受检查。她没有呕吐、体重减轻或疼痛,但在社交场合感到困扰。包括超声和计算机断层扫描在内的医学检查显示有大量腹水。腹腔穿刺抽出乳白色液体,经实验室分析确认为淋巴液。全血细胞计数、肝功能和血液学参数、乳糜细胞学、细菌培养以及结核菌素聚合酶链反应均在正常范围内。她被转诊至一家三级中心进行血管异常评估。动态对比增强磁共振淋巴造影显示淋巴解剖结构正常,无渗漏或血流梗阻。全身磁共振成像显示肠系膜中央旋转。她被转诊至一家三级儿童外科中心,在那里使用新型5毫米小儿密封装置进行了腹腔镜Ladd手术,并使用5毫米吻合器进行了阑尾切除术。为了使肠管复位,在含有大量乳糜和腹水的腔隙中开了几个小孔,排出了2.4升液体。她在术后第二天出院,至今健康状况良好达1年。我们展示了一段视频,演示了该患者的Ladd手术步骤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/53c0/10781518/2d46c776b1ac/10-1055-a-2221-9682-i2023030698cg-1.jpg

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