Diseases & Conditions

Acromegaly

Acromegaly is a hormonal disorder that develops when your pituitary gland produces too much growth hormone during adulthood. When this happens, your bones increase in size, including those of your hands, feet and face. Acromegaly usually affects middle-aged adults.

In children who are still growing, too much growth hormone can cause a condition called gigantism. These children have exaggerated bone growth and an abnormal increase in height.

Because acromegaly is uncommon and physical changes occur gradually, the condition often isn't recognized immediately. If not treated promptly, acromegaly can lead to serious illness and even become life-threatening. However, available treatments for acromegaly can reduce your risk of complications and significantly improve your symptoms, including the enlargement of your features.

One of the most common signs of acromegaly is enlarged hands and feet. People with this disorder often notice that they can no longer put on rings that used to fit and that their shoe size has progressively increased.

Acromegaly may also cause you to experience gradual changes in the shape of your face, such as a protruding lower jaw and brow, an enlarged nose, thickened lips, and wider spacing between your teeth.

Because acromegaly tends to progress slowly, early signs may not be readily apparent for several years. Sometimes, people notice the condition only by comparing old photographs.

Acromegaly may produce the following signs and symptoms, which can vary from one person to another:

• Enlarged hands and feet
• Coarsened, enlarged facial features
• Coarse, oily, thickened skin
• Excessive sweating and body odor
• Small outgrowths of skin tissue (skin tags)
• Fatigue and muscle weakness
• A deepened, husky voice due to enlarged vocal cords and sinuses
• Severe snoring due to obstruction of the upper airway
• Impaired vision
• Headaches
• Enlarged tongue
• Pain and limited joint mobility
• Menstrual cycle irregularities in women
• Erectile dysfunction in men
• Enlarged liver, heart, kidneys, spleen and other organs
• Increased chest size (barrel chest)

When to see a doctor
If you have signs and symptoms associated with acromegaly, contact your doctor for an evaluation.

Acromegaly usually develops slowly, and even your family members may not initially notice the gradual physical changes that occur with this disorder. Early diagnosis is important, however, so that you can receive proper care. Acromegaly can lead to serious complications if it's not treated.

Progression of acromegaly can result in major health problems. Complications may include:

• High blood pressure (hypertension)
• Cardiovascular disease, particularly enlargement of the heart muscle (cardiomyopathy)
• Osteoarthritis
• Diabetes mellitus
• Precancerous growths (polyps) on the lining of your colon
• Sleep apnea, a condition in which breathing repeatedly stops and starts during sleep
• Carpal tunnel syndrome
• Reduced secretion of other pituitary hormones (hypopituitarism)
• Uterine fibroids, benign tumors in the uterus
• Spinal cord compression
• Vision loss

Early treatment of acromegaly can prevent these complications from developing or becoming worse. If untreated, acromegaly and its complications can lead to premature death.

Treatment focuses on lowering your production of GH, as well as reducing the negative effects of the tumor on the pituitary and surrounding tissues. You may need more than one type of treatment.

Surgery
Doctors can remove most pituitary tumors using a method called transsphenoidal surgery. In this procedure, your surgeon works through your nose to extract the pituitary tumor.

Removing the tumor can normalize GH production and eliminate the pressure on the tissues surrounding your pituitary to relieve associated signs and symptoms. In some cases, your surgeon may not be able to remove the entire tumor. This may result in persistently elevated GH levels after surgery, requiring further medical or radiation treatments.

Because of the complex nature of the procedure, it's important to choose a surgeon experienced in performing this type of surgery. Usually, the more experienced the surgeon, the better the final outcome.

Medications
Drugs used to lower the production or block the action of GH include:

• Somatostatin analogues. The drugs octreotide (Sandostatin, Sandostatin LAR) and lanreotide (Somatuline Depot) are synthetic versions of the brain hormone somatostatin. They can interfere with the excessive secretion of GH by the pituitary, and thus can produce rapid declines in GH levels. When starting octreotide treatment, you initially inject yourself with a short-acting preparation under your skin (subcutaneously) three times a day to determine if you have any side effects from the medication and if it's effective. Then, if it's tolerated and effective, you can take a long-acting form that requires an injection into the muscles of your buttocks (gluteal muscles) by a health care professional, administered once a month. Lanreotide is administered as a subcutaneous injection once a month.
• Dopamine agonists. The medication bromocriptine (Parlodel) is taken as a pill. In some people, this drug can lower levels of GH and IGF-I. The tumor may decrease in size in some people taking a dopamine agonist or somatostatin analogues.
• Growth hormone antagonist. The medication pegvisomant (Somavert), a growth hormone antagonist, acts to block the effect of GH on body tissues. Pegvisomant may be particularly helpful for people who haven't had good success with other forms of treatment. You administer this medication yourself daily by subcutaneous injection. This medication can normalize IGF-I levels and relieve symptoms in most people with acromegaly, but doesn't lower GH levels or reduce the tumor size.

Radiation
Your doctor may recommend radiation treatment when tumor cells remain after surgery. Radiation therapy destroys any lingering tumor cells and slowly reduces GH levels. It may take years for this treatment to noticeably improve acromegaly symptoms.

You receive radiation treatment in one of two ways:

• Conventional radiation therapy. This type of radiation therapy is usually given daily over a period of four to six weeks. You may not realize the full effect of conventional radiation therapy for 10 or more years after treatment.
• Stereotactic radiosurgery. Also known as gamma-knife radiosurgery, stereotactic radiosurgery can deliver a high dose of radiation to the tumor cells in a single dose while limiting the amount of radiation to the normal surrounding tissues. This type of radiation may bring GH levels back to normal within three to five years.

Stereotactic radiosurgery is available at only a few U.S. medical centers and is not recommended for all people undergoing radiation for acromegaly. Your doctor will determine which type of radiation therapy is right for you based on:

• The size and location of your remaining tumor cells
• Your levels of insulin-like growth factor-I (IGF-I)

Even after initial treatment, acromegaly requires periodic monitoring by your doctor to make sure that your pituitary is functioning properly. This follow-up care may last for the rest of your life.