(CNN) She lived for 99 years with organs in all the wrong places and never knew it
By Sandee LaMotte, CNN Updated 2310 GMT (0710 HKT) April 8, 2019
On an early spring day in 2018, the faint smell of formaldehyde floating in the air, 26-year-old medical student Warren Nielsen and four of his classmates prepped a cadaver in the chilly dissection lab at Oregon Health and Science University in Portland.
Similar groups of five gathered around bodies on the other 15 tables in the anatomy class, all eager to explore the mysteries of the human body they had seen only in textbooks.
The cadaver assigned to Nielsen’s team was a 99-year-old woman who had died of natural causes. Her name was Rose Marie Bentley, but the students didn’t know that then. To honor and respect the privacy of those who offer their bodies to science, no further details are given medical students about the person who had once inhabited the body lying on the silvery slab before them.
But as the students and their professors were soon to find out, Bentley was special, so special she deserved her own unique spot in medical literature and history books.
The reason? A condition called situs inversus with levocardia, in which most vital organs are reversed — almost like a mirror inside the body. That, along with a host of other weird but wonderful abnormalities, made Bentley a sort of medical unicorn.
“I think the odds of finding another person like her may be as remote as one in 50 million,” said assistant professor Cameron Walker, who teaches the Foundations of Clinical Anatomy class at Oregon Health and Science University. “I don’t think any of us will ever forget it, honestly.”
‘This is totally backwards’
On this March day, the assignment was to open the body’s chest cavity to examine the heart. It wasn’t long before Nielsen’s group began to question their fledgling medical knowledge.
“Her heart was missing a large vein that’s normally on the right side,” Nielsen said.
Bewildered, he and his team called the professors over and asked: “Where’s the inferior vena cava? Are we missing it? Are we crazy?”
“And they kind of rolled their eyes,” Nielsen said, “Like, ‘how can these students miss this big vessel?’ And they come over and that’s when the hubbub starts. They’re like ‘Oh, my God, this is totally backwards!’ “
A typical body has a large vein called the vena cava that follows the right side of the vertebral column, curving under the liver and emptying deoxygenated blood into the heart.
Bentley’s vein was on the left, and instead of terminating directly into the heart, which is typical, “her vein continued through her diaphragm, along the thoracic vertebrae, up and around and over the aortic arch and then emptied into the right side of her heart,” Walker said.
“Normally speaking, none of us have a vessel that does that directly,” he added.
That wasn’t the only irregularity Walker and his students found in Bentley’s body.
Numerous veins that typically drain the liver and other parts of the chest cavity were either missing or sprouting from an unusual spot. Her right lung had only two lobes, instead of the standard three, while the right atrium of her heart was twice normal size.
“And instead of having a stomach on the left, which is normal, her stomach was on the right,” Walker said. “Her liver, which normally occurs predominantly on the right, was predominantly on the left. Her spleen was on the right side instead of its normal occurrence on the left. And then the rest of her digestive tract, the ascending colon, was inverted as well.”
The mutations in situs inversus with levocardia occur early, Walker explained, possibly between 30 and 45 days into the pregnancy. No one knows why.
The condition occurs in only 1 out of 22,000 babies and is invariably associated with severe congenital heart disease. Because of the heart defects, only 5% to 13% live past the age of 5; case reports mention one 13-year-old boy and a 73-year-old who at the time was the second-longest survivor.
But Bentley was an anomaly, one of the few born with the condition that didn’t have heart defects, Walker said.
“That is almost certainly the factor that contributed most to her long life,” he said.
And that, along with all her other exceedingly rare anatomical abnormalities, is what makes Bentley 1 in 50 million, Walker estimated.