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Asian J Surg. 2017 Jul;40(4):313-316. doi: 10.1016/j.asjsur.2014.04.003. Epub 2014 Jun 16.

Unusual presentation of adult Marfan syndrome as a complex diaphragmatic hiatus hernia.

Author information

1
Department of Diagnostic Radiology, Indira Gandhi Medical College and Hospital, Shimla, Himachal Pradesh, India. Electronic address: tshruti878@yahoo.in.
2
Department of Diagnostic Radiology, Indira Gandhi Medical College and Hospital, Shimla, Himachal Pradesh, India.
3
Department of Gastroentrology, Indira Gandhi Medical College and Hospital, Shimla, Himachal Pradesh, India.
4
Department of General Surgery, Indira Gandhi Medical College and Hospital, Shimla, Himachal Pradesh, India.

Abstract

Marfan syndrome is multisystem connective tissue disorder that primarily involves the skeletal, cardiovascular, and ocular systems. The gastrointestinal complications in Marfan syndrome are rare, with only a few case reports described in the literature. We present a 25-year-old woman who presented with acute abdominal pain for 1 day. The imaging features revealed complex diaphragmatic hiatus hernia with organoaxial gastric volvulus. This is a unique case report about an adult patient with Marfan syndrome who presented with symptomatic paraesophageal hernia and organoaxial gastric volvulus.

KEYWORDS:

Marfan syndrome; connective tissue; esophageal hiatus; organoaxial; volvulus

PMID:
24947765
DOI:
10.1016/j.asjsur.2014.04.003
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