Manson H J, Goh Y M, Goldsmith P, Scott P, Turner P
Lancashire Teaching Hospitals NHS Foundation Trust , UK.
East Lancashire Hospitals NHS Trust , UK.
Ann R Coll Surg Engl. 2017 Feb;99(2):e75-e77. doi: 10.1308/rcsann.2016.0345. Epub 2016 Nov 21.
Congenital diaphragmatic hernia (CDH) usually presents in infancy with respiratory failure requiring urgent surgical correction. Mortality in this group of patients remains poor and persistent pulmonary hypertension is a significant contributor. It is therefore rare for patients to reach adulthood undiagnosed. CDH is often identified incidentally in adults but when symptoms arise, they relate to the organ involved, and include gastrointestinal symptoms of dyspepsia and obstruction, as well as respiratory complaints such as dyspnoea. We present the case of a 30-year-old woman who was admitted with non-specific symptoms of upper abdominal discomfort but whose deteriorating condition culminated in a cardiac arrest, as an unreported presentation of CDH. The patient presented initially with severe left upper quadrant pain. Her chest x-ray on admission suggested a raised left hemidiaphragm. She went on to have computed tomography (CT) of the thorax and abdomen as well as oesophagogastroduodenoscopy, which raised the suspicion of diaphragmatic eventration. Repeat CT was performed after the patient collapsed on the ward five days following admission, revealing tracheal deviation, and a strangulated Bochdalek hernia containing stomach and spleen. After transfer to the anaesthetic room, she suffered a cardiac arrest. Advanced life support was required to return spontaneous cardiac function. She was intubated and ventilated, and a needle thoracostomy was performed to decompress the tension gastrothorax. Emergency laparotomy revealed a gangrenous stomach and spleen. Total gastrectomy with primary Roux-en-Y reconstruction, splenectomy and insertion of a feeding jejunostomy were performed. The patient recovered well postoperatively and was discharged two weeks following surgery.
先天性膈疝(CDH)通常在婴儿期表现为呼吸衰竭,需要紧急手术矫正。这类患者的死亡率仍然很高,持续性肺动脉高压是一个重要因素。因此,未经诊断活到成年的患者很少见。CDH在成人中常为偶然发现,但出现症状时,与受累器官有关,包括消化不良和梗阻等胃肠道症状,以及呼吸困难等呼吸系统症状。我们报告一例30岁女性,因上腹部不适的非特异性症状入院,但其病情恶化最终导致心脏骤停,这是一例未报道过的CDH表现。患者最初表现为左上腹剧痛。入院时胸部X线显示左半膈肌抬高。她接着进行了胸部和腹部的计算机断层扫描(CT)以及食管胃十二指肠镜检查,这引发了对膈膨出的怀疑。入院五天后患者在病房晕倒,之后重复进行CT检查,发现气管偏移,以及一个包含胃和脾脏的绞窄性Bochdalek疝。转至麻醉室后,她发生心脏骤停。需要进行高级生命支持以恢复自主心功能。她被插管并进行机械通气,同时进行了胸腔穿刺减压以缓解张力性胃胸。急诊剖腹探查发现胃和脾脏坏疽。进行了全胃切除术并一期Roux-en-Y重建、脾切除术以及空肠造口置管术。患者术后恢复良好,术后两周出院。