Curtin Medical School, Curtin University Bentley Campus, Perth, Western Australia, Australia.
Apex Radiology, Apex Radiology, Bunbury, Western Australia, Australia.
BMJ Case Rep. 2021 Apr 8;14(4):e239641. doi: 10.1136/bcr-2020-239641.
A 77-year-old female patient with Marfan syndrome presented with a 1-week history of worsening malaise and poor appetite. This was associated with a dramatic unintentional loss of weight as well as shortness of breath worsened by exertion. She has significant medical histories of a hiatal hernia and chronic type B aortic dissection. CT scan of the chest was able to confirm a type IV paraoesophageal hiatal hernia compressing on the lower lobe of her left lung without any progression of her aortic dissection. As surgical intervention was contraindicated in light of her advanced age and comorbidities, she was managed conservatively with a clinically satisfied outcome in the short term. Her long-term prognosis, however, is still poor with a high mortality of 1 and 5 years.
一位 77 岁的女性马凡综合征患者，因不适加重和食欲不振持续 1 周就诊。这与显著的非自愿性体重减轻以及活动后加重的呼吸短促有关。她有明显的食管裂孔疝和慢性 B 型主动脉夹层病史。胸部 CT 扫描证实存在 IV 型食管旁裂孔疝，压迫左下肺叶，主动脉夹层无进展。鉴于她年龄较大和合并症，手术干预被认为是禁忌的，因此她接受了保守治疗，短期临床结果满意。然而，她的长期预后仍然较差，1 年和 5 年的死亡率较高。
