Introduction to Intestinal Lymphangiectasia (IL):
Intestinal Lymphangiectasia (IL) is an uncommon disorder of the gut. Typically IL leads to loss of protein from the gut, which in turn leads to
lower blood levels of proteins.

In IL the lymph channels in the lining of the gut become congested and burst. This releases the contents of the lymph channels into the
middle of the gut, so that these are lost to the body.

Along with protein being lost in this way, other contents of the lymph channels are also lost: these include lymphocytes (leading to low
white blood cell counts) and antibodies (immunoglobulins).

Sometimes IL can start being a problem right from birth: other times it starts later through childhood. In addition, sometimes IL can be
undiagnosed for a long period of time.

The exact reason for the development of IL is not known. It does not usually run in families. On very rare occasions there have been
descriptions of more than one member of the same family having IL.

Most times IL occurs by itself (primary IL). Some children can have lymphangiectasia secondary to other problems, such as Crohn’s
disease or after surgery for heart disease. Sometimes children with these problems can have improvements when the underlying problem
is fixed. Some types of lymphangiectasia can involve small sections of the bowel (rather than the whole length of bowel): in these cases,
surgery to remove just the involved section can be considered. Although this may lead to relief of symptoms, it is not always permanent.  

Consequences of IL:
Due to the loss of the contents of lymph channels into the gut, people with IL may have problems holding onto fluid in their blood system
and develop swelling (oedema) of their body. Loss of white blood cells can mean that they are less responsive to infections. Similarly, loss
of antibodies also means that children are less able to fight infections (immuno-compromised).

Diagnosis of IL:
The typical findings in IL are low levels of albumin, lymphocytes and immunoglobulins in the blood stream. There are not many other
conditions that lead to this combination of changes.

Extra losses of protein into the bowel, leading to the low blood levels, can be detected by measuring protein in the stool: the usual test is
called the -1-anti-trypsin level. There are some special tests that are not done routinely (such as using labelled albumin) which can help
to show which areas in the gut are leading to extra losses and measure the exact amount of loss in a certain time period. Biopsy of the
surface of the gut (via a scope test) is usually done to show the dilated (swollen) lymph channels. This needs to done when the child has
been having a diet containing long chain fats (LCT) and not when they have been fasting for a long time or when on just intravenous
nutrition.

Most times children should also have some other tests to look for additional changes that can occur due to poor absorption in the bowel.
These include measuring the fat soluble vitamins (vitamins A, D, E and K), calcium and measurement of blood clotting. Most times, your
doctor will also want to rule out uncommon problems, such as carbohydrate deficient glycoprotein syndromes, which can lead to the same
pattern as IL. Also, special X-ray tests (such as barium meal and follow through) may be needed.

Managing IL:
Initially, when albumin levels are very low, most children will need to have extra protein given into the veins (albumin infusions), and may
need extra antibody infusions (IVIG infusions). Some children will need these extra amounts for a long time: others may need them just
when they are unwell.

The ideal way to help manage IL is to use special types of nutrition to help overcome the extra losses from the rupturing lymph channels.
Initially, this can include total parenteral nutrition, which is nutrition given into the veins.  This can help to give the gut a rest and for things to
settle down. Afterwards a special diet is usually recommended. This diet involves using a particular type of fat (medium chain fats known
as MCT) that does not use the lymph channels to get absorbed into the body. This type of fat is absorbed directly into the blood stream,
bypassing the lymph channels, and helping to keep them “unstressed”. Long chain fats  (LCT) are taken up into the lymph channels. A diet
high in MCT and very low in LCT can help to ensure that there is minimal bloating of lymph channels and minimal losses of protein and fat
and other components. This type of diet usually will need to be used indefinitely. However, it will need to be supplemented with several
extra nutrients, such as fat soluble vitamins and essential fatty acids (these are usually linked with LCT).

Even with a very strict diet there will still be some proteins and cells lost from the lymph into the gut: it does not usually go away altogether.
Sometimes when the body is otherwise stressed (such as during a cold), the losses may increase again and cause problems.

Complications of IL:
Because of the losses of white cells and antibodies, people with IL may be more at risk of infections.

Losses of fat and proteins may compromise nutrition leading to poor growth.

Poor absorption of fat-soluble vitamins can lead to low levels of these vitamins: A, D, E, and K. Vitamins usually need to be supplemented
with extra amounts by mouth.

Poor bone growth and poor mineralisation of the teeth have been described in IL. In addition, conditions such as lymphoma have been
described in some adults diagnosed late with IL after many years of uncontrolled losses. It is felt that early diagnosis and good control of
gut losses will mean these complications are very unlikely to occur.

Secondary Lymphangiectasia
Lymphangiectasia can occur in conjunction with a number of other conditions. These can be things affecting the gut such as Crohn’s
disease or bacterial overgrowth, or affecting other organs (such as after some types of heart surgery). Some other conditions that can lead
to protein losses due to lymphangiectasia include Turners syndrome, Hennekam syndrome, or Noonans.

In addition, lymphangiectasia can occur in the lymph channels of other parts of the body. Sometimes, these can be the superficial lymph
channels of the limbs (e.g. the legs) or of the lungs.

Summary
IL is an uncommon condition affecting the lymph channels in the wall of the bowel. It may occur in conjunction with other conditions, or just
by itself. Prompt diagnosis and starting appropriate nutritional management will help to ensure good growth and development. Although
some people with IL can have complications, ongoing close management and follow-up should mean that these are avoided or minimised.
Copyright ©2004 Dr. Andrew S Day, MB, ChB, MD, FRACP All rights reserved
Little Leakers
Intestinal Lymphangiectasia
"What is protein losing enteropathy"?
“Protein-losing Enteropathy” is a fancy way of saying there is something wrong with the intestine such that protein is being lost from the
body through the intestine. This is a serious problem as the body’s proteins are not easily replaced and the only way to replace them
involves the absorption of protein constituents (the amino acids that make up proteins) from the intestine. If the intestine is actually leaking
nutrients out instead of absorbing them in, the result is a nutritional disaster.
The main protein which one cannot afford to lose is called “Albumin.” This protein normally is produced by one’s liver and circulates in the
bloodstream acting as a carrier for biochemicals that require transport but cannot actually dissolve in blood. Albumin can be considered
sort of a mass transit system in the bloodstream, a bus or subway, if you will, carrying important biochemicals from one place to another.
Albumin, by being the most prevalent blood protein, also is responsible for actually keeping water in one’s bloodstream.  When water
cannot be held within the vasculature, it leaks out causing fluid accumulation in tissue (i.e. edema) or in within the chest or abdomen (i.e.
effusion).
Of course, in a protein-losing enteropathy, other proteins are lost, too. Antibodies, proteins of blood clotting, enzymes, etc. all leak out the
intestine and are forever lost in the feces that exits the body.
The body tries hard to maintain its albumin level by extracting protein from other sources (like muscle), and having the liver make albumin
from the components of these other proteins.  This may help maintain a workable amount of albumin in the bloodstream but it comes at
the expense of muscle tissue and other protein.

"What is Lympahngiectasia"?
Lymph is a fluid that circulates through the body similar to the way blood does; though blood is pumped actively through the body by the
heart while lymph is pumped passively via the normal muscle activity of the body.  Lymph consists of cells called “lymphocytes,” which are
cells of the immune system. Lymph also consists of fluid which collects from the tissues and shunts into actual vessels similar to veins.

The word “lymphangiectasia” means “dilated lymph vessels.” In the intestinal tract, lymphangiectasia is usually caused by some kind of
inflammation which puts back pressure on the lymph vessels leading them to dilate. Lymph flow may be blocked by the inflammatory
events in the intestine or local structures.
Lacteals are special lymph vessels in the intestinal tract designed to absorb nutritional fats. When there is high pressure within the lymph
vessels, the tender lacteals burst and instead of absorbing fats, the lymph inside, its cells, fats, and precious proteins are lost.