Sometimes our children grow very quickly, don't they? But it is not normal for them to suddenly grow much taller than other children, with larger limbs. The reason for this may be a very rare condition. Today we are going to talk about such a condition, that is, 'Gigantism'.
What is 'Gigantism'?
Simply put, gigantism is a rare condition in which a child or young person produces too much growth hormone (GH) . This is why these children grow much taller than others. Normally, this growth hormone (GH) is produced by a small, pea-sized gland located at the base of our brain. It is called the pituitary gland. However, if a small tumor develops in this pituitary gland, it can produce more growth hormone (GH) than is needed. That is when gigantism occurs.
The Pituitary Gland is a small gland located at the base of the brain, below the Hypothalamus. It actually releases about 8 hormones. Growth Hormone (GH) is one of them.
Growth Hormone, also known as Human Growth Hormone (hGH), is essential for the growth of children. It affects many parts of the body and helps the child grow properly. However, there are certain types of growth plates in our bones called Epiphyses/Growth Plates, and once they are completely closed (i.e. after puberty), growth hormone (GH) does not increase height. What it does is maintain the structure and metabolism of bones, cartilage, and other organs. This growth hormone (GH) is closely related to a hormone called Insulin-like Growth Factor 1 (IGF1), which is produced by our liver. Together, these two help with growth and metabolism.
Gigantism is a condition in which the body produces too much growth hormone (GH), causing the muscles, bones, and connective tissue to grow rapidly. This results in abnormal height and several changes in the soft tissues of the body. If left untreated, some people with gigantism can grow to be over 8 feet tall.
Therefore, it is very important to diagnose the disease and begin treatment as soon as possible in the case of gigantism.
What is the difference between 'Gigantism' and 'Acromegaly'?
Gigantism and acromegaly are both conditions caused by excess growth hormone (GH). The difference is in who develops it. Acromegaly develops in adults. Gigantism develops in children and young adults who are still growing, that is, who have not yet reached puberty.
Simply put, gigantism occurs when growth hormone (GH) levels increase before the growth plates in children's bones have closed (i.e., before puberty has finished).
If your growth hormone (GH) levels increase after your growth plates have closed, it can cause acromegaly. You won't grow taller, but the excess GH can affect the shape of your bones, the size of your organs, and other health conditions.
Gigantism is a much rarer condition than acromegaly.
Who gets gigantism? How common is it?
Although this is a very rare condition, gigantism can develop in any child whose growth plates have not yet closed (i.e., they have not completed puberty). It is more common in boys than in girls.
Gigantism is actually a very, very rare condition. For example, only about 100 cases have been reported in the United States so far. So you can imagine how rare it is.
How does normal growth occur in children? What is the difference with gigantism?
Growth hormone (GH) is what controls growth during childhood. That is, it controls metabolism while causing measurable growth in bones, muscles, and tissues. Normally, growth is a fairly stable process until a child reaches puberty.
During puberty, the secretion of sex hormones (estrogen and testosterone) increases significantly. This causes the epiphyses (growth plates) at the ends of a child's long bones to gradually close. When the epiphyses close completely, that is, when puberty is complete, height growth stops.
How quickly a child grows and what their final height will be after reaching puberty is determined by a combination of several genes they inherit from their parents, environmental factors, and their sex.
In the case of gigantism, the child grows very quickly, and much taller than other children of the same age and sex. However, the symptoms of gigantism can initially look like a normal childhood growth spurt, making it difficult for parents to recognize.
If you have any questions or concerns about your child's growth rate, be sure to talk to their doctor.
What are the symptoms of gigantism?
Gigantism is caused by an excess of growth hormone (GH). Also, a tumor in the pituitary gland can cause some symptoms, which can be caused by pressure on the nearby brain and nervous tissue. The main characteristic of gigantism is excessive growth. Children with gigantism grow very quickly in height.
In addition to being significantly taller/larger than one's age, physical characteristics seen in gigantism include:
- A very prominent forehead and a prominent jawline.
- Having gaps between teeth.
- Thickening of facial features.
- Large hands and feet, and thick fingers.
Other symptoms that may be seen include:
- Enlargement of internal organs, especially the child's heart.
- Excessive sweating (hyperhidrosis).
- Double vision or difficulty with peripheral vision.
- Headache.
- Joint pain.
- Delayed puberty.
- Irregular menstruation.
- Sleep problems, for example, sleep apnea.
- Muscle weakness.
If your child has these symptoms, it is very important to talk to a doctor as soon as possible.
What are the causes of gigantism?
Gigantism is often caused by a benign tumor (pituitary adenoma) in the child's pituitary gland. This causes excessive release of growth hormone (GH). When children with gigantism are diagnosed, they almost always have a large pituitary adenoma (a tumor 10 mm or larger), called a macroadenoma. Gigantism can also be caused by an enlargement of the pituitary gland, called pituitary hyperplasia.
Many children with gigantism have a genetic mutation that causes a pituitary tumor. The most common genetic mutation associated with gigantism is a mutation or deletion in the AIP gene. This affects about 29% of people with gigantism.
Gigantism can also occur as part of a number of very rare genetic disorders that increase the risk of developing a pituitary tumor that secretes growth hormone (GH). Some of these disorders include:
- Carney complex: This is a genetic condition that affects skin pigmentation and causes benign tumors of the skin, heart, and endocrine system. About 10% to 13% of people with Carney complex develop growth hormone (GH)-secreting pituitary adenomas, which usually grow slowly.
- McCune-Albright syndrome: This is a genetic condition that affects the bones, skin, and endocrine system. It causes café-au-lait spots on the skin, scar tissue in the bones, and precocious puberty. 20% to 30% of people with McCune-Albright syndrome have excess growth hormone (GH). This is often caused by an enlarged pituitary gland (hyperplasia) or a pituitary adenoma.
- Multiple Endocrine Neoplasias (MEN) types 1 or 4: These are genetic conditions. In this case, one or more of your endocrine glands become overactive, or a tumor develops. This can include a pituitary tumor that secretes growth hormone (GH).
- Neurofibromatosis: This is a genetic condition called neurocutaneous disorders. These affect the skin and nervous system. These conditions are caused by an abnormal increase in cell growth. This can lead to the formation of tumors throughout the body. These can include a tumor that secretes growth hormone (GH).
- Familial Isolated Pituitary Adenomas (FIPA): This is a hereditary condition. It involves the development of pituitary adenomas. These may include growth hormone (GH)-secreting adenomas.
How is gigantism diagnosed?
Gigantism can be a bit difficult to diagnose because it is a very rare condition, and because the growth rate of children can vary greatly due to genetic and environmental factors.
Typically, doctors suspect gigantism when a child's height is three standard deviations above the average height for their sex and age, or two standard deviations above the average height calculated from the child's parents' heights.
If your child's doctor suspects that your child has gigantism, he or she will likely refer you to an endocrinologist (a doctor who specializes in hormone-related diseases). The doctor will make a diagnosis based on your child's medical history, family medical history, a thorough physical examination, and specialized tests such as blood tests and imaging tests.
What tests are used to diagnose gigantism?
If your child's doctor suspects that he or she has gigantism, the following tests may be performed to confirm the condition:
- Growth Hormone and IGF-1 (Insulin-like Growth Factor 1) blood tests: These tests measure the levels of various hormones in a blood sample taken from the child's vein. If the levels are higher than normal, it could be a sign of gigantism.
- Glucose Tolerance Test: This test measures how well a child's growth hormone levels respond to glucose. For this test, a doctor takes blood samples from a vein at various intervals after the child drinks a glucose (sugar) solution.
- Imaging tests: If blood tests confirm that your child has gigantism, your doctor will often recommend an MRI (Magnetic Resonance Imaging) scan or a CT (Computed Tomography) scan. These can clearly see the size and location of the pituitary tumor. They can also help determine the exact treatment that is needed.
If your child is diagnosed with gigantism, the doctor may do some additional tests to see if the condition has affected other parts of the body. These tests may include:
- An echocardiogram to check for heart problems.
- Sleep study tests to see if you have sleep apnea.
- X-rays or a DEXA/DXA scan to check the health of your bones.
How is gigantism treated?
The main goals of treating gigantism are:
- Safely controlling 'growth hormone' (GH) and 'insulin-like growth factor 1' (IGF1) levels.
- Managing the growth of a 'pituitary tumor'.
- Reducing the impact of a pituitary tumor on nearby brain tissue and the optic nerve.
- Treating or reducing the effects of growth hormone (GH) on other body systems.
Doctors usually use a combination of treatments for gigantism , with surgery and radiation therapy being the main treatments.
Although there are effective medications to treat growth hormone (GH) excess in adults (acromegaly), the effects of these medications on children have not yet been well studied.
Surgery for Gigantism
Surgery is the most common treatment for gigantism. The goal is to either remove the pituitary tumor or reduce its size. Because the pituitary tumor that causes gigantism is often large, the child may need to undergo multiple surgeries to effectively control growth hormone (GH) levels.
Your child's surgeon may use a surgical procedure called transsphenoidal surgery to remove a pituitary tumor. This involves performing the surgery through your child's nose and through the sphenoid sinus (a cavity in the skull behind the nasal cavities, below the brain).
Radiation Therapy for Gigantism
If surgery fails to reduce growth hormone levels, radiation therapy may help. This involves using special equipment to aim radiation beams at the tumor. This treatment works slowly. It may require several treatments, with long rest periods in between. It can also take several years to see full results.
What are the complications of treatment?
Due to surgery and/or radiation therapy, about 60% of people with gigantism develop a condition called hypopituitarism after treatment. This is when one, several, or all of the hormones produced by the pituitary gland are underproduced. Hypopituitarism is treated with hormone replacement medications.
Complications that may occur from surgery to remove a pituitary tumor include:
- Bleeding.
- Cerebrospinal Fluid (CSF) leak.
- Meningitis (brain fever).
- Loss of sodium (salt) and water balance in the body.
Side effects of radiation therapy may include:
- Impaired Fertility.
- Vision loss and brain damage (rare).
- Tumor formation several years after treatment (rare).
Can gigantism be prevented? What is the prognosis?
Unfortunately, there is nothing you can do to prevent gigantism. However, early detection is crucial. Early treatment can help prevent or reverse the changes that cause your child to be too tall.
The prognosis for children and young adults diagnosed with gigantism depends on several factors:
- How early or late the disease was diagnosed.
- How effective are treatments for managing growth hormone levels?
- Whether there are complications related to gigantism.
In general, older people at diagnosis tend to have more complications than those diagnosed at a younger age. This is likely due to longer exposure to growth hormone and insulin-like growth factor 1 (IGF1).
Therefore, if you notice unusual, unexpected changes in your child's growth and/or physical characteristics, it is essential to talk to their doctor as soon as possible.
What is the life expectancy of someone with gigantism? What are the long-term complications?
Early detection and treatment of gigantism is essential to prevent excessive height and its associated complications and increase life expectancy.
If left untreated, gigantism is associated with significant complications and the mortality rate can be up to twice that of normal.
Long-term complications that some people with gigantism experience due to their excessive height and the overall effect of having too much growth hormone include:
- Difficulty moving around (Mobility) due to muscle weakness.
- Osteoarthritis (joint inflammation).
- Peripheral Neuropathy (peripheral nerve disorders).
- 'Sleep Apnea'.
- Enlarged heart (cardiomegaly) and heart valve problems.
- Metabolic complications such as Type 2 Diabetes.
Additionally, the quality of life of people with untreated gigantism can be reduced due to problems with daily activities such as shopping for clothes and traveling due to being too tall.
When should you see your child's doctor about gigantism?
If your child has been diagnosed with gigantism, he or she will need to see their doctor and/or endocrinologist regularly to monitor the progress of treatment and to make sure that their hormone levels are in order.
If you notice any changes in your child's growth rate and/or physical characteristics, it's important to talk to your child's doctor as soon as possible. Although gigantism is unlikely to be the cause, it's worth investigating any suspicious changes. Gigantism is best diagnosed early, and people with gigantism usually live healthy lives. If you have any questions about your child's growth, don't be afraid to ask your doctor. They are there to help you.
The most important things to remember (Take-Home Message)
Gigantism is a rare but potentially serious condition. The most important thing is to seek medical advice immediately if you notice anything unusual or suspicious about your child's development, such as growing taller faster than expected or having larger limbs.
Remember, early diagnosis and treatment greatly increase the chances of your child living a healthy, normal life. Don't worry, doctors are there to help you and your child. Talk to them about any concerns you may have.
` Gigantism, Growth Hormone, Pituitary Gland, Growth in Children, Height Increase, Acromegaly
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