David Jeffery Seveland
Attacking all obstacles with a strong mind and heart.
Hemihypertrophy (hem′ē-hī-pur′trə-fē)
Hemihypertrophy, also known as hemihyperplasia, is a greater than normal asymmetry between the left and right side of the body. This asymmetry can include just one finger; just one limb; just the face; or an entire half of the body, including half the tongue, half the brain and the internal organs, or any variation in between.
I have what is known as Complex or Total Hemihypertrophy (hemi). This means that it involves only one side of the body from the head down. The hemi affects the entire left side of my body. My skull is midly affected, including the jaw, the left side of the brain is slightly larger, my left kidney is larger, and the entire left side of my body is larger from the shoulder down. I have both tissue and bone overgrowth.
Here are a few pictures that I have managed to get showing David's LLD. It is about 3/4 of and inch at this time. In the pictures of him laying down it is about 1/2 inch. His feet are a size and a half different in legnth and his left is a 11XXW and his right is a 9 1/2XW. As he is getting older and taller the LLD is becoming more and more noticable. In these pictures it is hard to see how bad it really is. But these were the best shots I could get. Trying to get a toddler to stand still is a major challenge.
He is doing great with his walking and with everything else. He has even started to accomplish running. We are so proud of him. We are also very happy to have Hannah to chase after him for us. It saves our energy.
Description
The cause of hemihypertrophy is not known. However, it has been found that hemihypertrophy is usually not inherited. Hemi is thought to occur in about 1 in 14,000 people. Isolated hemihypertrophy occurs in about one in 86,000 live births. There are approximately 200 cases reported. Females and males are affected equally. Some children with hemihypertrophy also have a genetic syndrome, such as Beckwith-Wiedemann syndrome, neurofibromatosis, Klippel-Trenaunay-Weber syndrome, CMTC(Cutis marmorata talengiectatica congenita), or Proteus syndrome. 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.
**David has been diagnosed with Klippel-Trenaunay-Weber Syndrome and has an unusual left kidney with hydronephrosis.
A general guideline for identifying hemihypertrophy is: a 5% or greater difference in size or length between some aspect of the right and left sides of the body. 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.
**At 9 months David's leg-length discrepancy was 1/2 inch. It has increased since but we are not sure what it is now.
Because hemihypertrophy is a disorder of the body's normal controls of growth, people with this condition also have a higher rate of cancer. 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 that children with hemihypertrophy receive a screening abdominal ultrasound every 3 months until age 8, and, at minimum, a careful physical examination every 6 months until growth is completed. AFP (alpha fetoprotein) levels may also be useful as a marker of hepatic tumors. The AFP testing should be done every 6 weeks until age 4.
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.
**Right now we have a prescription for 1/4 inch shoe lift inserts. If we get a bigger lift then he will need orthopedic shoes because he still has baby feet and higher lifts will take up to much of the inside of his shoe. This is supposed to help him stabilize when he walks. We have not filled the prescription yet because David still has to learn how to walk and keep balanced on his own with the discrepancy first.
**We have also been advised to see a plastic surgeon at about 2 yrs to see what they recommend for the future with the growth of David's skull and facial bones.
Hemihypertrophy does not alter life span, although complications from associated abnormalities such as childhood cancer and mental retardation can cause problems.
**There are several treatments for the leg-length discrepancies that are available as David gets older and we know more about what he is dealing with. I have not researched it at this time because I don't want to overwhelm myself more than I need to. There is also liposuction to help with the bulk of the left leg. This is only to be done if he starts having problems with movement of the left leg.
Limb Length Discrepancy (LLD)
The link below gives information on Limb Length Discrepancy through identification, monitoring, problems caused by LLD and procedures for correcting it. The LLD is caused by the hemihypertrophy and is something that David will have to deal with in his future. At the age of 15 months his LLD is 5/8ths of an inch. This is an estimate we won't know a definite until he recieves an x-ray of his legs and gets the bones measured. This will happen when we return to to Orthopedist in the future. No appt. is made at this time.