April Fools' 2015: Ectopia cordis interna - Tin Man syndrome

Case contributed by Dr Matt Skalski

Presentation

Pre-employment screening. Long history of gastro-oesophageal reflux.

Patient Data

Age: 25
Gender: Male

This case is fictitious and the described condition is not a real diagnosis. The images in this case have been digitally altered. The case was originally published as one of Radiopaedia.org's April Fools' cases

Frontal and lateral chest radiographs demonstrating absent cardiomediastinal outline. No aortic outline is evident. Bilateral ectopic inferior pulmonary veins. 

Leonardo Da Vinci's "Organ networks of the thoracoabdominal cavity" (c.1502). There remains academic debate as to whether this was based off a corpse with ectopia cordis interna, or whether the heart's location was a deliberate distortion of reality by the artist. 

Selected coronal images show the intra-abdominal heart closely related to the stomach, distal transverse colon and pancreas. The mesenteric vascular congestion is a common feature. 

Case Discussion

Ectopia cordis interna, also known as Tin Man syndrome, is a rare variant form of ectopia cordis in which the heart is located completely within the abdominal cavity. It is almost always an asymptomatic condition found incidentally on imaging, or less often detected by physicians when attempting to auscultate the chest or at abdominal palpation. 

History

There remains debate as to whether Leonardo Da Vinci's "Organ networks of the thoracoabdominal cavity" illustration (c.1502) was based off a corpse with ectopia cordis interna, or whether his depiction of the heart's location was a deliberate distortion of reality. Most legitimate scholars believe Da Vinci created the work as a flight of anatomical fancy.           

The first ever description of the condition in the medical literature was in a controversial monograph submitted to the Royal Society in 1874 by Dr. Nohear Lubdub. Entitled "An unusual case of ectopia cardia epigasticum in a Haryana boy", the monograph was later retracted when accusations were made that the images accompanying the text had been doctored.

It was not until 1908 that Dr Lubdub's work was vindicated when existence of the condition was confirmed during the early years of chest radiography. Unfortunately, Dr Lubdub had fallen into a deep depression following his expulsion from the Royal Society, only occasionally seen wandering the streets of Chandigarh mumbling "and yet it beats". His death was unrecorded. 

Trivia

In an interview with Rolling Stone magazine, Hilary Duff credited the little known story of Nohear Lubdub which apparently "Like, really really spoke to me" as the inspiration behind her song "Beat of my heart". Duff can be seen beating her hand over her umbilicus during the official video clip in further reference to Lubdub.     

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Case information

rID: 33437
Case created: 13th Jan 2015
Last edited: 25th Jun 2017
System: Chest
Inclusion in quiz mode: Included

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