Hemihypertrophy & Hemihyperplasia

Hi, Dr. Greene, I’m hoping that you can help me find some information on the subject of hemihypertrophy. Like I told you in chat, 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

Hemihypertrophy & Hemihyperplasia

Dr. Greene’s Answer:

Vonda, I’m so glad you asked! Most people are not aware of this 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, but 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 is a key warning 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. Nevertheless, the distinction is very important because hemihypertrophy carries real risks. A definition first proposed over 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, about 1 inch for a 5-year-old, and 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 can occur as an independent condition (isolated hemihypertrophy) or as a part of a genetic syndrome (i.e. Beckwith-Wiedemann syndrome). Isolated hemihypertrophy is thought to occur in about 1 in 86,000 people, but this number may change as there is more agreement on a definition and more people looking for it. 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 can also have a higher rate of cancer. In one study, 168 children with isolated hemihypertrophy were very carefully followed 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 and, at minimum, a careful physical examination every 6 months until growth is completed (I prefer ultrasound). One proposed exception to this recommendation is in hemihypertrophy due to Klippel-Trenaunay Syndrome– the risk of Wilm’s tumor does not appear to be increased in these cases (Pediatrics 2004; 113:326-329).

Some argue that screening for cancer in children with hemihypertrophy is not cost effective 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. 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 perhaps the 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.

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Dr. Alan Greene

Dr. Greene is the founder of DrGreene.com (cited by the AMA as “the pioneer physician Web site”), a practicing pediatrician, father of four, & author of Raising Baby Green & Feeding Baby Green. He appears frequently in the media including such venues as the The New York Times, the TODAY Show, Good Morning America, & the Dr. Oz Show.

  1. Heather and Haley,

    Great questions!

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    Dr. Greene will answer one question a day and he’s going to let our readers decide which question they would most like to see answered. This will be determined by the number of votes each question receives. That means, you have a better chance of getting your question answered if you invite your friends to vote.

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  2. Haley Traub

    My name is Haley Traub I am 27 years old and have hemihypertrophy. Currently I am having difficulty with my hands very bad hand cramping and tingling, there’s a little going on in my toes and feet but not as bad as my hands. I have been extremely tired I tend to think more tired then usual. In December I was hospitalized with a bad migraine. And occasionally I get blurry vision. My feet and my hands swell if I am on them for more then twenty minutes.
    I am interested in knowing if any of this has to do with hemihypertrophy and if so what can I do?

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  3. todd

    Hi my name is todd hemihertrophy. For years they didn’t know what I had but they thought It was hemihypertrophy, or fedal achole syndrom. But the figured I dont have the fedal acohole syndrom. They figured it diagnosed me with hemihypertroph. Wich its not noticable in my face and body but they say if you look really closely you can notice it but in my legs you can really notice it. Then when I was about 13 we went to a diffrent doctor and immdiatly said go to chopp. Its weired how you say you gotta watch out for cancer. Which is weird because as the doctor and my mom and I talked she figured to asked if it would cause anything else. He said no your body will just grow faster. Which is Funny to me bc not once did he say I would get cancer.not during any of my surgeries not during fallow ups nothing. and yesterday I noticed that on here you say we possibly could get cancer. So I brought it up to him and he said that it was a lie that people who think that are Wrong its not true cancer is cancer you get that a diffrent way. But he said you can when you first born to 7 years of age to get it but after that you dont have to worry

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  4. todd

    Hi my name is todd. When I was in my mom stumic the doctor found that I had something wrong with my body which for years they did test and studied my body to figure out what it was, finally were I was born had a name for it and diagnosed me with hemihypertrophy. Wich we went to a doctor around were I live and told me every little thing I had and told us to go to chopp. We talked to the doctors and told me that if you look really closely to my body you can see my right side is bigger the my left side its more noticable in my legs he said. My mom asked “what is my future looking like and does this cause anything else. He said no this does not cause anything else, but it will cause him to lean all the way to one side and he wish he would of seen me sooner. So if it caauses cancer why did he say it doesnt cause anything else and why wouldnt he have told me

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  5. Heather

    I’m doing some googling because as a child I had hemihyperplasia (I
    still do though now that all the surgeries are over, I’m unaffected). I
    needed a number of facial surgeries and you can still see the
    discrepancy when I smile— you can also see it when I hold my thumbs up
    next to each other. I hadn’t thought about it in years, but over the
    past year my 6 year old’s speech has become more and more nasal and one
    side of his mouth is curving upwards more and more. Two different
    speech therapists think it’s structural and one ENT so far has said
    there is no structural problem and he feels it’s a learned speech
    behavior. We’re about to see another ENT for a second opinion. How
    likely is it that my son also has hyperplasia and that there was genetic
    involvement? If this ends up being the issue, how can we make his
    speech more understandable? As a child, my surgeries all waited until
    my teen years— but if my son follows my pattern and needs surgery, I
    would imagine we’d need to do something sooner rather than later as he’s
    very difficult to understand. Thank you! Heather Price
    heatheramyprice@gmail.com

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