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一名成人被诊断出患有绞窄性先天性Bochdalek型膈疝。

A Strangulated Congenital Diaphragmatic Bochdalek Hernia Diagnosed in an Adult.

作者信息

Warsinggih Warsinggih, Uwuratuw Julianus Aboyaman, Arsyad Arham, Faruk Muhammad

机构信息

Division of Digestive, Department of Surgery, Faculty of Medicine, Hasanuddin University, Makassar, Indonesia.

Division of Digestive, Department of Surgery, Dr. Wahidin Sudirohusodo Hospital, Makassar, Indonesia.

出版信息

Case Rep Med. 2022 Jul 20;2022:3355058. doi: 10.1155/2022/3355058. eCollection 2022.

Abstract

Although Bochdalek hernias are uncommon in adults and difficult to diagnose, such congenital diaphragmatic hernias (CDHs) are some of the most serious malformations among newborns. In particular, CDHs are accompanied by high mortality and marked disability later in life due to concomitant morbidity (i.e., caused by pulmonary lung hypoplasia and persisting pulmonary hypertension of neonates) and require long-lasting neonatal treatment in an intensive care unit. Late-diagnosed CDHs are extremely rare and always show small defects that justify a better prognosis than CDHs with large defects. In most cases, such CDHs go undiagnosed due to their mild, delayed symptoms. In the case described here, an 18-year-old Asian male presented with abdominal pain and a bowel contour in the epigastric region and left upper quadrant. A chest X-ray and thoracoabdominal computerized tomography scan confirmed the diagnosis of bowel obstruction due to a left diaphragmatic hernia. Moreover, a defect was revealed in the posterolateral left diaphragm, and the transverse colon, spleen, and half of the stomach had herniated through it. A segmentectomy was performed on the transverse colon, followed by an end-to-end anastomosis, a diaphragmatic plasty, and the placement of an intrapleural catheter. In conclusion, diagnosing a diaphragmatic hernia before surgery can be difficult due to its rarity and wide range of symptoms. Although prenatal diagnosis using ultrasonography is possible in up to 80% of fetuses, that method may not be available in all regions in Indonesia or in all countries, where doctors thus continue to face a diagnostic challenge when dealing with CDHs.

摘要

尽管波奇达勒克疝在成人中并不常见且难以诊断,但这种先天性膈疝(CDH)却是新生儿中最严重的一些畸形。特别是,CDH伴有高死亡率,且由于伴随的发病率(即由新生儿肺发育不全和持续性肺动脉高压引起),在生命后期会出现明显残疾,需要在重症监护病房进行长期的新生儿治疗。晚期诊断出的CDH极为罕见,且总是表现为小缺损,其预后比大缺损的CDH更好。在大多数情况下,此类CDH由于症状轻微且出现延迟而未被诊断出来。在此所述病例中,一名18岁的亚洲男性因上腹部疼痛以及上腹部和左上象限出现肠轮廓而就诊。胸部X线和胸腹计算机断层扫描证实了因左侧膈疝导致肠梗阻的诊断。此外,还发现左侧膈肌后外侧有一个缺损,横结肠、脾脏和半个胃通过该缺损疝出。对横结肠进行了节段切除术,随后进行了端端吻合术、膈肌修补术,并放置了胸腔内导管。总之,由于膈疝罕见且症状范围广泛,术前诊断可能很困难。尽管使用超声进行产前诊断在高达80%的胎儿中是可行的,但在印度尼西亚的所有地区或所有国家,这种方法可能并不都能使用,因此医生在处理CDH时仍面临诊断挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0798/9328991/7fe2b99f7b89/CRIM2022-3355058.001.jpg

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