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Rare complication of a type IV paraoesophageal hiatal hernia in a Marfan syndrome patient
  1. Lachlan Hou1,
  2. Sunita Dhanda2 and
  3. Dan Xu1,3,4
  1. 1Curtin Medical School, Curtin University Bentley Campus, Perth, Western Australia, Australia
  2. 2Apex Radiology, Apex Radiology, Bunbury, Western Australia, Australia
  3. 3Curtin School of Population Health, Curtin University Bentley Campus, Perth, Western Australia, Australia
  4. 4Medical Education, Sun Yan-sen University of Medical Sciences, Guangzhou, China
  1. Correspondence to Professor Dan Xu; daniel.xu{at}curtin.edu.au

Abstract

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.

  • stomach and duodenum
  • gastroenterology
  • connective tissue disease
  • vascular surgery

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Footnotes

  • Contributors LH drafted the initial manuscript and revised and critically reviewed the final manuscript. SD provided the radiological images and revised and critically reviewed the final manuscript. DX designed and drafted the initial manuscript, and revised and critically reviewed the final manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.