Intestinal Lymphangiectasia

Introduction:
Intestinal Lymphangeictasia (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 lymphangeciectasia 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 Lymphangectasia
Lymphangectasia 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