一例24岁健康女性先天性膈疝伴叶外肺隔离表现为胃扭转

IF 4.6 2区 教育学 Q1 EDUCATION & EDUCATIONAL RESEARCH Educational Technology & Society Pub Date : 2019-12-31 DOI:10.24966/ets-8798/100037
Hannah Medeck
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引用次数: 0

摘要

先天性膈疝(CDH)由于器官发生过程中胸部的压迫作用以及肺组织和血管系统发育受损,具有显著的发病率和死亡率风险。现在,产前筛查通常可以在子宫内诊断CDH,并随后协调新生儿的医疗和外科治疗,以限制早期死亡风险。在这里,我们讨论了一位健康的24岁女性的延迟表现,她患有继发于胃扭转的弥漫性腹痛。在手术中,该患者被发现患有先前未诊断的先天性膈疝和并发的肺叶外肺隔离症,在术前成像中模拟胃穿孔。已经描述了伴有CDH和肺隔离症的先天性器官发生异常。对于对晚期CDH的成人进行手术的外科医生来说,这些并发的病理必须是有区别的。孤立的肺组织有恶变和慢性感染的风险,需要彻底探查疝囊以确定这种并发病理,并在CDH修复过程中仔细切除孤立的肺细胞。
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Congenital Diaphragmatic Hernia With Extra-Lobar Pulmonary Sequestration Presenting As Gastric Volvulus In A Healthy 24-Year-Old Female
Congenital diaphragmatic hernias (CDH) carry significant morbidity and mortality risk due to compressive effects in the thorax during organogenesis and impaired development of lung tissue and vasculature. Prenatal screening now often allows for diagnosis of CDH in utero and for subsequent coordination of neonatal medical and surgical therapies to limit the early mortality risk. Here we discuss delayed presentation in a healthy 24-year-old female with diffuse abdominal pain secondary to gastric volvulus. Intraoperatively, this patient was found to have a previously undiagnosed congenital diaphragmatic hernia and concurrent extralobar pulmonary sequestration which mimicked gastric perforation on preoperative imaging. Concurrent congenital abnormalities of organogenesis with CDH and pulmonary sequestration have been described. These concurrent pathologies must be in the differential for surgeons operating on adults with late presentation of CDH. Sequestered lung tissue has a risk of malignant transformation and chronic infection necessitating thorough exploration of the hernia sac for identification of this concurrent pathology and careful resection of sequestered pulmonary tissue during CDH repair.
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来源期刊
Educational Technology & Society
Educational Technology & Society EDUCATION & EDUCATIONAL RESEARCH-
CiteScore
9.10
自引率
2.50%
发文量
1
审稿时长
20 weeks
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