Department of Cardiothoracic Surgery, Children's Hospital of Shanxi Province, Taiyuan, Shanxi, China.
Turk J Pediatr. 2023;65(5):881-885. doi: 10.24953/turkjped.2022.512.
Late-presenting congenital diaphragmatic hernia occurs beyond the neonatal period, and is relatively rare, presenting with nonspecific respiratory and gastrointestinal symptoms.
We report a rare case of late-presenting congenital diaphragmatic hernia in a 7-year-old girl, who presented with abdominal pain, shortness of breath and fever on admission. Work-up revealed intrathoracic gastric perforation, acute pancreatitis and septic shock with a diaphragmatic defect. Due to the high content of amylase in pleural effusion, we suspected the presence of a pancreaticopleural fistula, and we were also puzzled whether the gastric perforation was caused by a pleural indwelling catheterization, but this was ruled out. We about performed a laparotomy to reposition the herniated organs, repair the hernia and the gastric perforation, and undergo the gastrostomy. The girl had an uneventful post-operative recovery.
Late-presenting congenital diaphragmatic hernias are often misdiagnosed. Clinicians should combine multiple imaging modalities to make a definite diagnosis and perform surgery as soon as possible to avoid severe complications.
迟发性先天性膈疝发生于新生儿期之后,较为罕见,表现为非特异性的呼吸和胃肠道症状。
我们报告了一例罕见的 7 岁迟发性先天性膈疝病例,患儿以腹痛、呼吸急促和发热入院。检查发现胸腔内胃穿孔、急性胰腺炎和感染性休克,同时存在膈疝。由于胸腔积液中淀粉酶含量高,我们怀疑存在胰性胸腔瘘,也对胃穿孔是否由胸腔留置导管引起感到困惑,但这一点被排除了。我们进行了剖腹手术以重新定位疝出的器官,修复疝和胃穿孔,并进行胃造口术。女孩术后恢复顺利。
迟发性先天性膈疝常被误诊。临床医生应结合多种影像学方法做出明确诊断,并尽快进行手术,以避免严重并发症。