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.

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.

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.

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.