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巨大腹裂伴肝脏完全疝出：两例患者的病例报告

Giant Gastroschisis with Complete Liver Herniation: A Case Report of Two Patients.

作者信息

Svetanoff Wendy Jo, Zendejas Benjamin, Demehri Farokh R, Cuenca Alex, Nath Bharath, Smithers C Jason

机构信息

Department of General Surgery, Boston Children's Hospital, 300 Longwood Ave, Fegan 3, Boston MA 20115, USA.

Department of Transplant Surgery, Massachusetts General Hospital, 165 Cambridge Street, Suite 301, Boston, MA 02114, USA.

出版信息

Case Rep Surg. 2019 Jan 15;2019:4136214. doi: 10.1155/2019/4136214. eCollection 2019.

DOI:10.1155/2019/4136214
PMID:30775044
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6350565/
Abstract

INTRODUCTION

There are no reported survivors of gastroschisis with complete liver herniation. We describe a case report of two patients, one of whom survived.

CASE #1: The patient was born with gastroschisis and herniation of the entire liver. Along with silo placement, the abdominal fascia was attached to an external traction system for growth. Complete closure was achieved at 5 months. Due to pulmonary hypoplasia, high-frequency ventilation was required. The patient is doing well, on a home ventilator wean, at 20 months.

CASE #2: The patient was born prematurely with gastroschisis, total liver herniation, and a defect extending to the pericardium. A silo was attached to the fascia to provide growth of the abdominal cavity. The patient developed respiratory failure, diffuse anasarca, and renal failure. She died at 38 days of life.

DISCUSSION

We report the first survivor of gastroschisis with complete liver herniation, contrasting it with a death of a similar case. The associated pulmonary hypoplasia may require long-term ventilation, the inflammatory response can lead to anasarca, and renal injury can occur from acute-on-chronic compartment syndrome.

CONCLUSION

External fascial traction systems can help induce growth of the abdominal wall, allowing closure of the challenging abdomen. While critical care management is complex, survival is possible.

摘要

引言

尚无腹裂合并完全性肝脏疝出的幸存者报道。我们描述了两例患者的病例报告，其中一例存活。

病例1：该患者出生时患有腹裂和整个肝脏疝出。除了放置袋子外，还将腹横筋膜连接到外部牵引系统以促进生长。5个月时实现了完全闭合。由于肺发育不全，需要高频通气。该患者在20个月时情况良好，正在进行家庭呼吸机撤机。

病例2：该患者早产，患有腹裂、全肝疝出以及延伸至心包的缺损。在筋膜上连接了一个袋子以促进腹腔生长。该患者出现呼吸衰竭、全身性水肿和肾衰竭。她在出生38天时死亡。

讨论

我们报告了首例腹裂合并完全性肝脏疝出的幸存者，并将其与一例类似病例的死亡情况进行对比。相关的肺发育不全可能需要长期通气，炎症反应可导致全身性水肿，急性慢性间隔综合征可导致肾损伤。

结论

外部筋膜牵引系统有助于诱导腹壁生长，从而实现对具有挑战性的腹部的闭合。虽然重症监护管理很复杂，但存活是有可能的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b014/6350565/cc96d7a57455/CRIS2019-4136214.010.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b014/6350565/90d70eb36772/CRIS2019-4136214.006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b014/6350565/a94d585113ca/CRIS2019-4136214.007.jpg
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2
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J Pediatr Surg. 2013 May;48(5):E13-5. doi: 10.1016/j.jpedsurg.2013.03.005.
3
Fetal MRI-calculated total lung volumes in the prediction of short-term outcome in giant omphalocele: preliminary findings.
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European J Pediatr Surg Rep. 2020 Jan;8(1):e112-e116. doi: 10.1055/s-0040-1721054. Epub 2020 Dec 15.
4
Prenatal Diagnosis of a Ventral Abdominal Wall Defect.腹前壁缺损的产前诊断
Neoreviews. 2020 Apr;21(4):e286-e292. doi: 10.1542/neo.21-4-e286.
胎儿 MRI 计算的全肺容积在预测巨大脐膨出短期结局中的应用：初步发现。
Fetal Diagn Ther. 2012;31(4):248-53. doi: 10.1159/000334284. Epub 2012 Apr 27.
4
Monitoring intravesical pressure during gastroschisis closure. Does it help to decide between delayed primary or staged closure?腹裂修补术中膀胱内压力监测。它有助于决定是进行延迟一期修补还是分期修补吗？
J Matern Fetal Neonatal Med. 2012 Aug;25(8):1438-41. doi: 10.3109/14767058.2011.640366. Epub 2012 Apr 25.
5
Liver herniation in gastroschisis: incidence and prognosis.先天性脐膨出合并肝脏疝出：发生率和预后。
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6
Omphalocele: how big does it have to be a giant one?脐膨出：多大算巨大脐膨出？
J Pediatr Surg. 2009 Jul;44(7):1474-5; author reply 1475. doi: 10.1016/j.jpedsurg.2009.02.060.
7
Impact of omphalocele size on associated conditions.脐膨出大小对相关病症的影响。
J Pediatr Surg. 2008 Dec;43(12):2216-9. doi: 10.1016/j.jpedsurg.2008.08.050.
8
Closure of giant omphaloceles by the abdominal wall component separation technique in infants.采用腹壁成分分离技术闭合婴儿巨大脐膨出。
J Pediatr Surg. 2008 Jan;43(1):246-50. doi: 10.1016/j.jpedsurg.2007.09.051.
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