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成人右先天性膈疝伴肝脏异常

Right congenital diaphragmatic hernia associated with abnormality of the liver in adult.

作者信息

Ayane Gezahen Negusse, Walsh Mikel, Shifa Jemal, Khutsafalo Kadimo

机构信息

University of Botswana, Faculty of Medicine, Botwana.

出版信息

Pan Afr Med J. 2017 Sep 22;28:70. doi: 10.11604/pamj.2017.28.70.11249. eCollection 2017.

Abstract

A Bochdalek hernia (BH) occurs when abdominal contents herniate through the postero-lateral segment of the diaphragm. The right side is affected considerably less commonly than the left. Most BHs present are diagnosed early in life, with some element of cardio-respiratory distress. Rarely, hernias that remain clinically silent until adulthood when they present as life-threatening surgical emergencies. We report a case 35 year old female who emergency exploratory laparotomy for a complete mechanical bowel obstruction. At surgery the redundant transverse colon was twisted and incarcerated within the right hemithorax, creating a closed loop obstruction. The right colon, appendix, terminal ilium, and three accessories right liver lobes were also dragged into the right thoracic cavity. After reducing the hernia, the diaphragmatic defect was primarily repaired with non-absorbable suture. The redundant transvers colon which had been compromised was resected and primary end-to- end anastomosis was carried out. Incidental appendectomy was done. The patient was sent into ICU for post-operative monitoring. She made an uneventful recovery and remains asymptomatic at nine month follow-up. I discuss what i believe to be the first case report of complicated right diaphragmatic hernia in Botswana, associated with another congenital mal-formation (accessories hepatic lobes, partial mal-rotation, and redundant transvers colon) in adult.

摘要

博赫dalek疝(BH)是指腹腔内容物通过膈肌后外侧段发生疝出。右侧受累的情况比左侧少见得多。大多数BH在生命早期被诊断出来,伴有一定程度的心肺窘迫。很少有疝在临床上一直无症状,直到成年时才表现为危及生命的外科急症。我们报告一例35岁女性,因完全性机械性肠梗阻接受急诊剖腹探查术。手术中,冗长的横结肠扭转并嵌顿于右胸腔内,形成闭袢性梗阻。右结肠、阑尾、回肠末端以及右肝的三个附属叶也被拖入右胸腔。将疝内容物回纳后,用不可吸收缝线对膈肌缺损进行了一期修补。对已受损的冗长横结肠进行了切除,并进行了一期端端吻合。同时做了阑尾切除术。患者被送入重症监护病房进行术后监测。她恢复顺利,在9个月的随访中仍无症状。我讨论了我认为是博茨瓦纳首例与成人期另一种先天性畸形(附属肝叶、部分肠旋转不良和冗长横结肠)相关的复杂性右膈疝病例报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54ec/5724727/34b8aa9315f5/PAMJ-28-70-g001.jpg

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