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Acromegaly

Overview

Acromegaly is a disorder that arises with a disturbance in hormones. It enables the pituitary gland to produce too much growth hormone (GH) during adulthood. Due to the increased growth hormone levels, the bones grow in size. Gigantism is the term used for an increase in height in childhood. Most commonly, the increase is seen in the bone size of the hand, face, and feet. It is a rare condition with around 3–4 new cases per million population per year. It is commonly diagnosed in middle-aged people. The symptoms are initially relatively mild and may have been present for several years before diagnosis. 

Causes And Types

An adenoma (tumor) that produces excessive growth hormone is the cause of acromegaly. This adenoma is present in the pituitary gland. According to the function and clinical features, acromegaly is divided into as follows:

Controlled Acromegaly: When growth hormone (GH) and Insulin-like growth factor (IGF-1) are within normal levels after treatment.

Active Acromegaly: It is considered when a person has symptoms. In this type, the tumors are giant and impossible to remove. Also, medications fail to control the levels of GH and IGF-1.

Risk Factors And Epidemiology

A pituitary tumor is the only known risk factor for acromegaly. The prevalence of acromegaly ranges between 2.8 and 13.7 cases per 100 000 inhabitants, and an annual incidence rate lies between 0.2 and 1.1 cases per 100 000 people annually. The average age at diagnosis ranges between 40 and 47 years.

Signs And Symptoms

Common signs include enlarged hands and feet. The earliest sign that could be noticed is that the person cannot put on rings that are the right size, and the shoe size has progressively increased. Acromegaly may also cause slow changes in the shape of the face, such as a protruding lower jaw with prominent brow bone, an enlarged nose, a thick appearance of lips, and visible spaces between the teeth. As acromegaly tends to progress slowly, early signs may not be evident for several years. Sometimes, people may notice the physical changes only by comparing old photos with the recent ones.

Some of the common clinical features include:

·         Increase in size of hands and feet

·         Prominent large facial features, including the facial bones, lips, nose, and tongue

·         Coarse, oily, thickened skin

·         Excessive sweating and body odor

·         Outgrowths of skin tissue (skin tags)

·         Fatigue and joint or muscle weakness

·         Pain and limited joint mobility

·         Enlarged vocal cords and sinuses

·         Obstruction of the upper airway results in too much snoring

·         Vision impairment

·         Headaches (severe persistent)

·         Menstrual cycle irregularities in women

·         Erectile dysfunction

·         Loss of libido

Diagnosis

Acromegaly can be diagnosed in two ways. They include;

Blood tests: Growth hormone and IGF-I blood tests measure multiple types of growth hormone levels in the blood. If the hormone levels are high, the next step is to do a glucose tolerance test to confirm the diagnosis of acromegaly. The body’s reaction to glucose utilization helps figure out the cause of raised hormone levels.

Imaging tests: Once the blood tests confirm acromegaly, the next step is an MRI scan, which indicates an increase in size and location of a tumor and helps determine the appropriate treatment accordingly.

Differential Diagnosis

Some of the similarly appearing disorders include;

·         Sotos syndrome

·         Several genetic overgrowth syndromes, such as Beckwith–Wiedemann (IGF2), Weaver (EZH2), Malan (NFIX), and Tatton–Brown–Rahman (DNMT3) syndromes

·         Diseases that feature tall stature, such as Berardinelli–Seip.

Treatment

Surgery: In most cases, surgery significantly improves acromegaly symptoms or corrects the condition completely. The indications of the surgery will depend on the size and location of the tumor. The goal of surgery is to excise the entire tumor that is causing extra growth hormone production. If doctors have excised enough of the tumor, there is no need for further treatment. If doctors could remove only a part of a tumor, then medication or radiation therapy to manage the symptoms and decrease growth hormone production is required.

Medication: Injection like somatostatin analogs (Sandostatin®) can help manage hormone levels. Medications work in different ways to control the body’s growth hormone levels and improve the symptoms.

In certain cases, a person may take medication until a tumor has shrunk. This can allow doctors to excise the shrunk tumor with surgery safely. Other people may need to take medication for a long duration to control growth hormone levels and associated symptoms.

Radiation therapy: Radiation therapy can help lower growth hormone levels when medications are ineffective. Radiation therapy uses specialized equipment to target the tumor with certain radiation beams and destroy it permanently. This therapy does not work very fast but has tremendous effects of damaging the tumor widely. Several treatment courses may be required with breaks between and may take several years for full effect.

Prognosis

Outcomes of acromegaly depend on the severity of the disease and how effectively therapies treat the symptoms. Many people see a significant improvement in symptoms after medical or surgical treatment.

If it is not treated timely, it can cause lifelong disabling and disfiguring symptoms. The aggravating symptoms can highly affect the person’s self-image and quality of life. Certain community-support groups help people deal with the challenges they face due to acromegaly.

Medical complications such as heart disease or Type 2 diabetes can also decrease the quality of life and even reduce the lifespan. In very severe cases, these complications can cause life-changing effects.

Lifestyle Modifications

Once the diagnosis is made, the following lifestyle should be adopted to cope with the illness;

·         Take your medicines or the proposed treatment properly

·         Since acromegaly can increase your chances of having some diseases like high blood pressure, diabetes mellitus, and increased heart size, routine checkups including blood tests to identify these disorders early, should be undertaken. If they are found, required treatment must be taken.

·         Physical exercise to combat joint and muscular pains.

·         Routine colonoscopy as there are chances of colon tumors.

·         If you are mentally disturbed or upset by changes occurring in your body, try to join support groups or talk to a therapist or a family member to feel better.