From: "Glenn Rees" <grees@...>

does anyone have information on this disease particularly
regarding a 6 mo.
child.

thanks, glenn grees@...



Hi-
www.alltheweb.com has a bunch of references...395 or so.
Anyway, here's one that provides a pretty good overview.
I wish you well...







From: http://www.drgreene.com/990121.html

Can you help me find some information on the subject of
hemihypertrophy (greater-than-normal asymmetry between the right
and left sides of the body)?

Q: Hi, Dr. Greene, I'm hoping that you can help me find some
information on the subject of hemihypertrophy. Like I told you on
ivillage chat today, my darling daughter Jemma was diagnosed at
the age of 4 months. She is now 4 1/2 years old, and although I
have found an on-line support group for it, most of the members
don't know much about it either. We live in Australia and there
is very little information to be found. I seem to be the one
teaching Jemma's pediatrician about what little I do know. At the
moment, Jemma's discrepancy is at 4 cm, but just 2 months ago it
was 2 cm. Since she has been diagnosed the discrepancy has
remained fairly consistent before her most recent growth spurt.

It has been a long and lonely journey for us (the doctors here
don't seem to want to put me in contact with other Hemi
families...if there are any), so any information gives me the
backup I need to deal with this on our own. Once again thank you
for taking the time for me in the chat today and I look forward
to hearing from you.
Take Care
Vonda (a.k.a. Potubby2)
Australia

A: Vonda, I'm so glad you asked! Most people are not aware of
this important medical condition. Hemihypertrophy (also called
hemihyperplasia) is a greater-than-normal asymmetry between the
right and left sides of the body. This difference can be in just
one finger; just one limb; just the face; or an entire half of
the body, including half the brain, half the tongue and the
internal organs, or any variation in between. Someone with
hemihypertrophy might have acne on only one side of the face. The
skin is often thicker, and there may be more hair on the head, on
the larger side. Rarely, children can have crossed
hemihypertrophy (one leg and the opposite arm are larger than
their partners).

Theories abound as to the cause of hemihypertrophy. Perhaps it is
increased blood flow or decreased lymph drainage, or nerve or
hormone abnormalities. To date, not enough research has been
conducted to choose between the theories. We don't know the
cause; we do know that hemihypertrophy is usually not inherited.
People with hemihypertrophy can go on to have healthy, normal
children (Genetic Counseling, 1993; 4:119--126).

Hemihypertrophy, though, is a key warning signal to be on the
lookout for several kinds of cancers. Sadly, hemihypertrophy is
often not looked for and not diagnosed until after the cancer has
been discovered.

None of us is exactly symmetric. I recall seeing a series of
fascinating magazine photos of famous movie stars. The photos
were made by putting together 2 right sides and 2 left sides of
their faces. It was surprising how much this changed their
appearances. I had not noticed the asymmetry until it was
removed.

During World War II, a series of United States Army recruits was
carefully measured, and only 23% were found to have legs of equal
length. The average difference was a little more than 1/4 inch
(American Journal of Roentgenology, 1946; 56:616--623). One of
our ears is usually higher than the other. The two eyes are
slightly different. Only rarely are two nipples at the same
height and the same distance from the midline.

All of us are asymmetric, and where normal variation ends and
hemihypertrophy begins is controversial. But the distinction is
very important because hemihypertrophy carries with it real
risks. A definition first proposed 20 years ago still seems to me
to be the best general guideline: hemihypertrophy is a 5% or
greater difference in size or length between some aspect of the
right and left sides of the body (Clinical Orthopedics, 1979;
144:198--211). This translates into a leg-length difference of
about 1/2 inch for a 1-year-old, of about 1 inch for a
5-year-old, and of about 1-1/2 inches for an adult.

As children with hemihypertrophy grow, the discrepancy between
the two sides increases, but the relative proportions between the
two sides usually remains the same over the long haul. Variations
are found among different children, but in most children, the
discrepancy about doubles between the first and fifth birthdays,
which sounds like what has happened in Jemma.

Hemihypertrophy is thought to occur in about 1 in 14,000 people,
but this number may change as there is more agreement on a
definition and more people looking for it (Fortschritte der
Medizin (Munchen), 1977; 95:831--834). Some children with
hemihypertrophy also have a genetic syndrome, such as
Beckwith-Wiedemann syndrome, neurofibromatosis,
Klippel-Trenaunay-Weber syndrome, or proteus syndrome. Although
these occur in the minority of children, each child with
hemihypertrophy should be evaluated by a geneticist to look for
associated conditions. Inguinal hernias, undescended testicles,
and unusual kidneys (renal cysts or horseshoe-shaped kidneys) are
more common in children with hemihypertrophy whether or not they
have other syndromes.

Because hemihypertrophy is a disorder of the body's normal
controls of growth, it is not surprising that people with this
condition also have a higher rate of cancer. Recently, 168
children with hemihypertrophy were very carefully followed up to
try to determine the true rate of cancer in children with this
condition. Just under 6% developed childhood tumors (American
Journal of Medical Genetics, 1998; 79:274--278). The most common
cancer is Wilms' tumor (of the kidney), followed by adrenal
carcinoma and liver cancer (hepatoblastoma).

Because most of the cancers occur in the abdomen, the
recommendation has been made (by the participants of the First
International Conference on Molecular and Clinical Genetics of
Childhood Renal Tumors--among others) that children with
hemihypertrophy receive a screening abdominal ultrasound every 3
months until age 7 years and at least a careful physical
examination every 6 months until growth is completed (I prefer
ultrasound).

This recommendation has now been called into question, many
arguing that because most children do not get these tumors and,
even for those who do, these tumors are fairly easy to treat even
if caught late, the screening is not cost effective. Be that as
it may, if it were my child, I would insist on the screening.

The next most immediate concerns are the orthopedic problems that
result from any leg-length discrepancy. Over time, scoliosis, or
curvature of the spine, commonly develops. This disappears when
the leg lengths are equalized, either with surgery or with
special shoes or lifts. Close contact with a skilled pediatric
orthopedist is a must.

Plastic surgery for facial discrepancies is sometimes warranted.
The best people to contact are a craniofacial team or the perhaps
people who repair cleft lip and palate in your area if no one has
experience with hemihypertrophy. Computed tomography (CT) scans
and computers can now be used to plan the repair for the best
outcome (Journal of Oral and Maxillofacial Surgery, 1987;
45:217--222).

These, Vonda are the major issues. I'd be happy to talk with you
more about them in chat.

Alan Greene, M.D., F.A.A.P.
January 21, 1999