Category Archives: Cases

Monthly case: Undiagnosed Pancreatic Cancer

Rathke pancreas safe viewWhat is shown?   The top photo shows the pancreas split down the middle (horizontally).  To the left is the tumor.  Next to the tumor is a circle.  This is a tube cut across.  It is the common bile duct (the tube that drains bile from the liver).  The tube is abnormal.  It’s too big (dilated).  It is roughly twice as big as it should be normally. To the right is the spleen (the red organ taking up about a third of the image).  Below, the photo is illustrated.  The split down the middle of the pancreas is shown with a dashed red line.  The tumor is outlined in black.  Two red arrows highlight the common bile duct. The spleen is labelled.

What was the story here?  This case was from an autopsy of a man in his 50’s who had cancer of “undiagnosed primary.”  This means that he had cancer in his body but no one knew where the cancer started.  Specifically, he had cancer that had spread to his lymph nodes.  He had a biopsy of one of these lymph nodes.  Testing of the lymph node tissue showed that one possibility was that the tumor started in the pancreas.  But x-ray studies did not report a mass in the pancreas.  The x-rays showed the common bile duct was dilated, but no mass in the pancreas. The pancreas was never biopsied.

Why did the family request an autopsy?  The family wanted to finally find out where the tumor came from.  They were angry that no diagnosis had been made.

Why was the diagnosis not made here?  In this particular case, the tumor did not increase the size of the pancreas very much and, as mentioned, could not be seen on x-ray.  Some pancreatic tumors are not visible on x-ray.  These tumors are called “isodense.”  “Iso” means “same.”  So these tumors have the same density as normal tissue.  Approximately 10% of pancreatic tumors are isodense.  This means they cannot be distinguished in x-ray appearance from normal pancreatic tissue.

On the other hand, a tell tale sign of a pancreatic cancer is that it can pinch off the tip of the common bile duct and cause the rest of the duct to balloon out.  This happened here. But the finding was not considered in thinking about the possibility of a pancreatic tumor.

Lastly, the lymph node that was positive for cancer was in a unique place in the body.  It was above the left collar bone.  A lymph node here is called a “Virchow’s node.”   It is a tip off that the tumor could come from the pancreas.

So overall, there were three tell-tale signs to consider there might be a tumor in the pancreas:  the studies on the lymph node, the location of the lymph node, and the ballooned (dilated) common bile duct.  But because no mass was seen on x-ray, pancreatic cancer was not considered.  There was no consideration the tumor was hidden on x-ray because it was isodense; and, again, no biopsy.  This was a missed diagnosis.

How did the autopsy help?  The autopsy provided closure for the family because it answered an important question they wanted and needed to know: where did the tumor come from?  And it gave them a context for understanding the factors that went into the missed diagnosis.

Monthly case – Congestive Hepatomegaly

Congestive hepatomegaly Oct 2014 Safe

Diagnosis: Congestive Hepatomegaly

What is shown?  The photo shows a markedly enlarged liver (seen on the left).  The liver is about twice as big as normal.  Also shown is a normal liver (seen on the right and roughly the size of a football). You can see from the rulers that the scale is same in both pictures.  The colors are different because the liver on the right was embalmed before the autopsy while the liver on the left was not embalmed.

How did the liver get so big?  The liver got big because it filled with blood.  The extra blood was entirely responsible for the large size of the liver.  The blood was inside the liver’s blood vessels.  The liver cells themselves did not get bigger nor did they increase in number.

How did so much blood get into the liver?  The blood got into the because this patient had heart trouble.  When the heart stops functioning well (can’t pump blood forward), the blood can back up into nearby organs, for example, the liver, lungs and spleen.  This causes these organs to swell.  That’s what happened in this case.

What does “congestive hepatomegaly” mean?  Congestive (or congestion) is the term for “blood vessels filled and swollen with blood.”  “Hepato” means liver; and “megaly” means “big.”  So this is a “big liver from blood vessels swollen and filled with blood.”

What does this finding mean when seen during an autopsy?  This finding often is part of the process of dying or it can mean the heart had been sick for a while.  It’s important to know a bit more about the story to understand how to make sense of a congested liver.  While very noticeable during an autopsy, the finding does not really give much information about the cause of the heart problem, or even when that problem started.  It just indicates either that the heart had a problem or that the heart failed as part of the process of dying.

What was the story here?  This was an elderly woman who had a complication after a minor surgery on a limb.  She was in the hospital for many weeks after the surgery and then died.

What was the family’s question here?  The family was angry.  They wanted to understand how, if at all, the treatment or surgery may have caused their loved one to become sick and die.

Comment long hospitalizations.  Long hospitalizations are particularly challenging to sort through during an autopsy.  This is because, over the course of the hospitalization, many things happen to the patient.  This makes it hard to figure out – just looking at the body – what problems were there before the patient was hospitalized; what problems developed possibly as a complication of a treatment; what changes indicated healing; and so on.  These cases require close study of the chart, x-ray studies, and the history.  And the answers aren’t always clear.

How did the autopsy help?  In this case, the family was desperate to make sense of what happened.  Understanding which findings (like the liver enlargement) where the result of the process of dying and which caused the death were important to sort through.  Doing that allowed the family to come to terms with the medical issues and their loved one’s experience after her surgery.

Monthly Case: Second lung cancer

Safe Lung cancer

What is shown?  The picture shows a large tumor (oval) of the left lung.  The tumor is about 3 inches wide, from top to bottom. The tumor is right next to the main airway (arrows).

How did this patient die?  The lung cancer grew into the airway and blocked this patient’s breathing.

What type of tumor was this?  Tumors in the lung (and any organ) come in different types. This one was a “small cell carcinoma.”  It’s called this because of the small size of the cells seen under the microscope.  This is a very aggressive (fast growing) lung tumor.  Prognosis is commonly six to twelve months, even with treatment.

What was the clinical story here?  This patient was an elderly woman who had been a smoker and had emphysema.  She had had lung cancer in the other lung – the right lung – about ten years before. That right lung tumor was completely taken out by surgery and was not thought to have spread (metastasized) anywhere back then.

Why did the family request an autopsy?  The family wanted to know why there was a tumor in the left lung if the surgery “got it all out” from the right lung ten year ago.  This was confusing to them.  They began to feel mistrustful of how first treatment went.  Did they really get it all out?  Were they being dealt with honestly?  How could there be a second tumor in the lungs now?

What did the autopsy show?  The autopsy showed the past surgery on the right lung. About a third of that lung had been removed. There were normal healing changes and no “left over” tumor on that side.  The left lung tumor was found (described above).  There were two metastases in the liver and metastases in the bone marrow.

How did the autopsy help?  The autopsy allowed for a discussion of the biology of tumors and gave a perspective on the patient’s care.

Here were the possibilities to consider:

-That the second tumor was part of the first because some was, in fact, left behind after surgery ten years ago and had just kept growing.
This could not be.  Because small cell carcinoma is so fast-growing, if there were any tumor left from the surgery, it would have grown quickly at that time.  It would not grow so slowly to take ten years to come to medical attention.  Also, the tumor was in a completely separate lung.  Any tumor remaining on the right side would have grown in the right lung not the left.

-That there were tiny metastases at the time of the first surgery that were undetected but then grew and showed up ten years later. 
This is also could not be, again because small cell carcinoma grows so quickly it would not take ten years for metastases to come to medical attention.

That the new tumor was a complete different tumor from the first.
This can only be what happened here, because the above possibilities were ruled out.  Also, it is known that once a patient gets one lung cancer, they are at increased risk of a second, different lung cancer. It is something that is known to happen.

How did the discussion impact the family?  The information staved off anger towards the original surgeon.  The family could rest at ease knowing that the original surgery had, indeed, accomplished its medical goals.  The family did not need to feel any concern for any past medical mismanagement, error or dishonesty because there had been none.  Past treatments were completely unrelated to the new tumor.  This was an entirely new and different tumor.  The family had not known this could be a possibility.

The discussion also provided a reminder of the risks of smoking. 

Lastly, learning more about the biology of lung cancer gave the family a language and context to understand and talk about their loved one, each important in the grief process.

Monthly case: Fungal abscess

Fungal abscess protected

What is shown?   The photograph shows the right and left lungs and the unopened sac around the heart (pericardium, dashed line) still inside the chest.  The arrows point to a region of pus in the right lung.  It’s a bit smaller than the size of a playing card and not quite half an inch deep.  This is an abscess.  The white flecks are fungus (mold).  This is a fungal abscess.

What is the clinical history here?  This case is of an elderly man with numerous bouts of “pneumonia” and unexplained fevers over several months. Each time, the patient was treated with antibiotics and improved, but the “pneumonia” always came back.  The “pneumonia” was first diagnosed by CT scan.

How did the autopsy help the family?  The autopsy provided an explanation for a situation that had been a mystery.  The family was distressed and confused as to why treatment did not work.  The autopsy provided the “big picture” here – that, underneath it all, was an abscess, not a pneumonia.  This provided relief to the family, and a sense of peace on one level.  Now they knew why their loved one never got better.

Why didn’t the abscess heal with antibiotics?  Antibiotics reach areas of infection by travelling through the blood stream.  An abscess is a pocket of pus with no blood flow inside it – antibiotics can’t easily get inside.  The treatment of an abscess is to drain it surgically.  A lung abscess that does not get drained will sit in the body and continue to cause symptoms (fever, breathing problems, general weakness, etc.) or, as in this case, death.  In addition, the patient received antibiotics (which treat bacterial infections).  The patient needed antifungals because this was a fungal infection.

How did the family manage with this new information?  While the family was surprised and relieved, they were angry that the abscess had not been detected prior to the patient’s death.  Specifically, they wondered why it was not detected on the initial CT scan.  Also, they were angry because they had asserted themselves with providers, questioning the diagnosis and care, but were dismissed.

How did the family discussion proceed from this point?  In this case, it was very important to make sure that feelings of guilt were not added to the family’s anger; and to help them manage their anger by providing information and perspective.  Specifically, it was not the family’s fault the diagnosis was not made.  It was important to value the family’s efforts during the time they cared for their loved one, validate their commitment to their loved one, and emphasize the complexity of the health care system.  Also, it was important to give the family as many tools as possible to interpret the situation.  For example, regarding their concerns about the first CT scan, the possibilities were that:

-the abscess was not there at that point (e.g., that it developed later)

-the abscess was there but difficult to see for some reason related to its appearance on the CT (e.g., perhaps related to the abscess being relatively flat)

-that the abscess was there but was was missed by the radiologist

While the last possibility was the family’s concern, a discussion of the biology of abscesses and the limitations of radiology studies helped sort through the issues.  The discussion provided tools to the family in thinking about the experience, and helped them formulate questions when seeking information from the providers.  Fungal abscesses are notoriously difficult to diagnose and often missed.

Summary.  By providing concrete information from the autopsy examination and by engaging the family on an educational level, this allowed the family to feel a bit less helpless and a bit more empowered moving forward during their vulnerable and difficult time.

Monthly Case: Blocked stent

July safe

What is shown? The upper left photo shows a metal mesh tubing. This is a stent. It’s about half an inch wide and a few inches long. This stent is inserted into a blood vessel to help keep the vessel open and the blood flowing (illustrated on the top right). The bottom photo shows the vessel in a patient who had an autopsy. In this case, the type of vessel is a vein. It is a large vein that drains the blood from the leg.  The vein, is cut into smaller pieces to see what’s on the inside. You can see metal stent as a circle of dots that that presses up on the inside of the vessel. The inside of the stent is filled with yellow solid tissue. This is scar tissue. The vessel should have been open and and empty.

Did the scar tissue cause a problem?  Yes.  The vessel was completed blocked by the scar tissue, so it could not drain the blood from the leg.  The left leg was swollen.  There was blood return from the leg, but it was through smaller side veins, and not enough to keep the leg from swelling.

Did the blockage cause the patient to die?  No.  The patient did not die from this.  He lived with a swollen leg.  The swelling developed gradually over many years (see below).

Why was the sent placed? The stent was placed in the vein to keep it open. A blood clot had formed in the vein, blocking flow. The stent at first helped with that by opening up the vessel to allow blood flow back from the leg.

Why did the scarring happen? The scarring happened because, even after the stent was put in, more clot formed inside the vessel.  The body’s response to a clot is to heal the clot by forming scar tissue. It is a natural response of the body to any clot that forms inside a vessel.  The process of forming this scar took many years.

Does the scar means there was a problem with the procedure or with the stent? No, stents are not perfect. Any time “foreign” material (like metal) is put into the body, there is a known risk that the body will have a reaction to it. Also, this patient was already at risk for forming clots (see below). There is always a “risk-benefit” decision for any procedure. The thinking is like this: Without the procedure, the patient would have had blocked circulation in the legs. With the procedure there is the risk down the line that the stent could get blocked. The best decision was to do the procedure to help with the “immediate risk” to the patient from his own blockage the first time he had a clot.

What was the story here? This was an 18 year old man with a disease related to sickle cell anemia called with S-C disease. “S” stands for “Sickle”. “C” is a genetic variation of sickle cell anemia. These patients are typically a bit less sick than patients with sickle cell anemia but also have serious problems.

How did this patient die?  This patient died from “Acute Chest Syndrome.”  This is a condition that happens commonly in S-C disease.  Here’s how it works:  In S-C disease (just as in Sickle Cell Anemia), the red blood cells can clump and block the blood flow in many areas of the body.  When that happens specifically in the bone marrow, parts of the bone marrow can die.  The dead bone marrow leaks into the blood stream, travels up to the lungs, and the little clumps of bone marrow block the circulation there.  This causes the patient to die.  Unfortunately, many clinicians are not aware of this complication of S-C disease.  This patient’s clinician did not consider this diagnosis.

How did the autopsy help?  The autopsy helped the family come to terms with the loss.  The family also chose to share the results with the treating physician which served to educate him about this condition.  It was an empowering move by the family in a situation that was otherwise marked by helplessness.  The specific finding of a blocked stent was incidental to the cause of death but facilitated a general discussion of the patient’s medical past.

Monthly case: Heart Rupture

 
Covered Heart Rupture June cropped 2014

What is shown?   The top photo shows the sac around the heart (pericardial sac) cut open and lifted to expose the sac filled with blood (star).  The right lung is seen on the right.  On the bottom left is the heart.  A clamp has been inserted into the site of rupture.  The tip of the clamp (actually inside the heart) is illustrated with dashed lines.  On the bottom right is the clot that had escaped from inside the heart into the pericardial sac.

What was the history here?  This was a functional, active elderly woman who was days earlier released from the hospital after a heart attack.  She collapsed at home.  Paramedics were called and performed CPR, but she could not be resuscitated and died.

Why did the family request an autopsy?  The family was concerned that the mother died shortly after hospital discharge.  They wondered if the hospital had released her “too soon” (i.e., that she should have stayed in the hospital for her safety and monitoring).

What happened here?  The heart broke open (ruptured) causing blood to escape into and fill the pericardial sac.

How did this happen?  This rupture was likely caused by CPR in combination with a weakened heart muscle wall from the patient’s heart attack.  Cardiac rupture from CPR is a rare but known complication of this life-saving procedure.  Rupture of the heart wall is also a known complication after a recent heart attack.  The two can at times go together.

What else can cause the heart muscle to rupture?  Sometimes the heart muscle thins as it scars after a past heart attack from months or years prior.  With large heart attacks, part of the heart muscle can scar to “paper thin” in some patients.  This thin wall can rupture even without CPR.

Why, in this case was CPR considered as a cause of the rupture? In this case there was considerable trauma to the chest wall and other areas of the heart that suggested the CPR was vigorous.  Specifically, there were broken ribs on the left; and blood in the soft tissue underneath the breast bone.  Lastly, there was bruising on the back of the heart.  Since CPR is performed from the front, the force must have been strong enough to mash the back of the heart on back of the chest and cause this bruising.

How did the autopsy help?  The finding of heart rupture from the autopsy was new information for the family.  Families often wish to know if “everything” was done to save the patient.  Since heart rupture is severe and often causes death, it was clear that resuscitation efforts would not have resulted in “bringing back” the patient.  “If only” concerns (e.g., “if only they had worked on her a little longer”) could be laid to rest as continued CPR would not repair a rupture.  The autopsy also served to exclude other unanticipated causes of death (e.g. pulmonary embolism) and validated the family’s concerns regarding the heart itself.  Thus, the autopsy’s definition of the medical issues allowed the family to move forward. Lastly, considering the complex processes here (the patient’s disease, hospital care, hospital discharge planning, cause of the collapse, timing and need for CPR, and heart rupture) allowed for a thorough discussion of the medical issues – always important in the bereavement process.

Note:  CPR is and remains an important and life-saving measure.  CPR-related heart rupture is rare.    

 

 

Monthly Case: Greenfield filter with clot

May Newsletter Covered

What is shown? Shown (right photo) is a piece of metal wiring with blood clot trapped in it. The device is called a Greenfield filter. It’s about two inches long altogether. The left picture shows the device where it was found – in the inferior vena cava (dashed lines). The inferior vena has been opened up along its length. The diagrammatic insert shows the filter without clot attached.

What is a Greenfield filter? A Greenfield filter is a device inserted into the inferior vena cava (the large vein returning blood to the heart). The filter traps clot that might travel up from the legs in some patients. It is inserted through the groin and threaded up the inferior vena cava to rest there.

How does the filter stay put? Little hooks at the ends of the wires keep it in place inside the vein. This keeps it from traveling up the vein closer to the heart

How does the filter help the patient? By trapping clots that travel in the blood stream up from the legs, the filter prevents them from traveling any farther. Specifically, the filter prevents them from reaching the lungs. The Greenfield filter saves lives.

Who gets a Greenfield filter? Patients with clots in their legs (deep venous thromboses – DVT’s) get a Greenfield filter.

What was the story here? This was a 32 year old man with cancer (lymphoma), DVT, and many infections. He spent the six months leading up to his death in the hospital.

Why did the family request and autopsy? The family was looking for closure and peace of mind. They were also concerned about the quality of care and the possibility of missed diagnoses. The family was dedicated and committed and had one member or another stay with him at all times. Their style was centered on control with extensive documentation and detailed note-taking of any medical information or conversations that came their way. Behind all this were deep feelings of grief over the loss of a son and brother.

How did the autopsy help? In the setting of deep grief, it was important to make use of any and all information from the autopsy to provide both a clear picture of the quality of care and the patient’s experience prior to death. The finding of a Greenfield filter with entrapped clot indicated the following:

Filter placement. The presence of the filter itself reflected appropriate clinical judgment and excellent quality care on the part of the providers. It is standard of care to place the filter, which is what this patient had. For a family with concerns about treatment, it was then helpful to objectively show that at least in one area (management of deep venous thrombosis), care had been appropriate. This then, could help the family feel a bit less angry about some of their concerns. It was not clear what, if anything, had already been discussed with the family about the filter’s placement. Its finding at autopsy presented another opportunity to review the clinical course.

Trapped clot. The presence of trapped clot indicated a disaster avoided. The amount of clot was significant. Without the filter the clot would have, without a doubt, traveled up the blood stream to the lungs and possibly caused the patient’s death, if not severe illness.

Conclusion. Therefore, for during a life cut short, when days and weeks had become precious to the patient and to the family, it was clear that the medical care had bought some time in this world for this patient. This was an important thing to share with a family dealing with loss; and a benefit of the autopsy.

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April Case: Liver metastases

Photo Cases Newsletter

Diagnosis: Liver Metastases

What is shown?
The picture shows two slices of the liver, each with a metastasis (arrows). These were the only metastases in the liver.

Where did the metastases come from?
They came from a lung cancer.

How did this patient die?
The patient died from the lung cancer (which blocked her breathing). The metastases did not cause the patient to die.

What is a metastasis?
When a tumor spreads through the blood to other areas of the body (where it takes hold and grows) this is a metastasis.

What do metastases mean for the patient in life?
Metastases mean the tumor is more advanced and often less likely to be curable. This is because surgery to remove the main tumor will still leave behind the metastasis somewhere else in the body. It also often means the amount of tumor is large. This can affect the success of other treatments (e.g., chemotherapy).

Is this always true?
No. Some tumors can still be curable even with metastases. It depends on the number of metastases, their size, where they are in the body, and if the anatomy allows for a surgical approach. Also the type of tumor matters. For example, some Hodgkin’s lymphomas, and a testicular tumor called seminoma can be curable even with metastases. Each case is different.

Is there any other reason to remove or treat a metastasis?

Yes, this can sometimes relieve symptoms (palliation).

In this case, how did the autopsy help the family?
Anxiety over possible suffering. The amount of tumor is often connected in the family’s mind to the amount of suffering. It is important for this reason to give an exact description of the amount of tumor, where it is located, and how it affected that organ. In this case, the majority of the liver was preserved, and the patient would not have had clinical liver problems. The metastases likely caused this patient no problems.

Guilt/anger over treatment issues: Families often need to know if the autopsy findings indicated that some treatment may have saved the patient “if only” it had been tried. They may feel guilty for not having “tried harder,” or angry with the medical system for the same reason. Here, the patient’s lung tumor overwhelmed her breathing and this was the cause of death. No treatment approach dealing with the liver metastases would have helped – they were not the cause of death. This relieved the family from their worry about the liver metastases.

Summary. The finding of metastases allowed for a discussion of the progress of the disease, but did not at all suggest suffering or a missed opportunity for treatment. This allowed the family to proceed with a sense of calm, peace, and understanding.

Airway Mucus Plugging – Sound Cloud

Diagnosis: Airway mucus plugging

Photo Case 1 Feb 2014

What is shown?
The photograph shows the large airway opened from behind. Seen are: the voice box (left, black arrow), trachea (center); two mainstem bronchi (right, white arrows). The airway is completely filled with mucus.

How did this happen?
This patient was very weak (from cancer and from an infection), had advanced Alzheimer’s disease, and could not “clear” the mucus. Normally, people can “clear” mucus by coughing it out of the airway (and then swallowing or spitting it out). The patient was too weak to do this. So the mucus built up and blocked the airway.

How did this patient die?
The mucus blocked the airway and gradually choked the patient, preventing airflow into the lungs.

How did the autopsy help the family?
The autopsy findings allowed the family to address specific, emotionally-charged and distressing medical-related issues:

The patient’s level of comfort. The family noticed gurgling sounds during breathing and was concerned this meant the loved one was uncomfortable or suffered near the time of death. The autopsy confirmed the presence of mucus but cannot specifically comment on suffering. However, an understanding of the specific effect of mucus on air flow helped the family feel more comfortable that the patient may not have suffered.

Here’s how that worked. Because mucus builds up gradually, the patient must have had a gradual decrease in air flow (rather than abrupt, as in sudden choking). This suggests the likelihood of a gradual and long term decrease in body oxygen. This means the brain likely also had a gradual decrease in oxygen supply. Low oxygen levels cause the brain to lose or decrease consciousness. An unconscious person cannot experience suffering. This means there was a high likelihood the patient did not suffer.

The patient’s level of care. The family worried that, with all that mucus, the patient should have been suctioned by nursing staff. The autopsy cannot comment on treatment options. However, by discussing the issues and findings, the family can consider alternate points of view. For this patient, the following questions had to be considered: Given her terminal condition, what were the treatment goals? Was full care the treatment plan? Or was hospice in place? What palliative measures (e.g. suctioning) were agreed upon? Was the treatment plan made clear to the family or, more importantly, made by the family?

A family that agrees to hospice but requests suctioning suggests an active and human struggle in accepting imminent death and letting go of the loved one. A family that requests full care (including suctioning) and does not see it suggests a different set of issues.

Summary.
In this particular case, the autopsy allowed the family to come to terms with the terminal nature of the cancer; understand that their observation (of breathing) reflected the process of dying; focus their energy on bereavement rather than anger (over perceived nursing issues); and thereby achieve a sense of peace.