Complications of Acromegaly

Introduction:

Acromegaly, also known as gigantism in childhood, is an endocrine disorder occurring due to excessive growth hormone production by the pituitary gland. This excess growth hormone in the body leads to abnormal growth and enlargement of bones, hands, feet, thickening of soft tissues, etc. A benign pituitary tumor known as pituitary adenoma is the most common cause of acromegaly. These adenomas cause overproduction of growth hormone in about 98 % of the cases of acromegaly. These tumors may grow in size, and as they enlarge, they compress nearby brain structures. Some of the common complications of acromegaly are hypertension (high blood pressure), high levels of cholesterol, increased risk of heart problems, type 2 diabetes, etc.

What Is Acromegaly?

• Acromegaly is a disorder characterized by excessive growth hormone production from the pituitary gland, resulting in abnormal enlargement of the body, especially hands and feet.

• Apart from the overgrowth of the bones, acromegaly may also cause the thickening of soft tissues of the lips, heart, and tongue.

• The pituitary gland is the “master” endocrine gland responsible for producing multiple vital hormones that regulate several functions of the body, including growth, development, stimulation of other endocrine glands, reproduction, etc. However, excess production of any hormone is terrible for the body. In about 95 % of acromegaly cases, this extra growth hormone production results from a benign pituitary gland tumor (pituitary adenoma).

• Acromegaly affects males and females equally, and this disorder affects approximately fifty to seventy people per million.

• However, because of the slow progression of the disease and late reflection of the symptoms, this disorder is often underdiagnosed. This is why it is difficult to estimate the exact frequency of the condition in the population.

What Causes Acromegaly?

Acromegaly is a rare disorder caused by excess production of the growth hormone by the pituitary gland.

• In most cases, this overproduction of the growth hormone is due to a benign pituitary gland tumor or a pituitary adenoma.

• The majority of the cases of acromegaly occur sporadically and are not inherited.

• In rare cases, acromegaly occurs due to ineffective control of the growth hormone-secreting cells from the hypothalamus.

• Rarely, non-pituitary tumors in adrenal glands, lungs, or pancreas may cause acromegaly.

What Are the Symptoms of Acromegaly?

Acromegaly generally progresses slowly, and symptoms reflect late and become more noticeable as the affected person ages. Acromegaly shows a wide variety of signs and symptoms that include:

• Enlargement of the bones of hands, feet, and face.

• Protrusion of the lower jaw.

• Soft tissue enlargement results in thick lips and tongue.

• Deep and husky voice due to the vocal cords and sinuses enlargement.

• Overgrowth of bones and enlargement of the cartilage leads to osteoarthritis.

• A prominent brow.

• Exaggerated growth spurts.

• Weight gain.

• Malocclusion of the teeth (misalignment of the teeth).

• Fatigue and headaches.

• Inability to sleep.

• Enlarged sebaceous glands.

• Skin tags.

• Generalized expansion of the skull at fontanelle.

What Are the Complications of Acromegaly?

If left untreated, acromegaly can cause some grave complications. It can even become a life-threatening condition. The complications are of two types; direct effects of the tumor mass and systemic complications.

• Direct Effect of the Tumor: The tumor can affect other hormones secreted by the pituitary gland apart from just the growth hormone. This imbalance in the levels of other hormones can affect the functioning of other endocrine glands leading to endocrine problems in general.

• Systemic Complications: The systemic complications of acromegaly are vast; it affects several organs, tissues, and systems, including:

How Is Acromegaly Diagnosed?

The diagnosis of acromegaly can be difficult because the symptoms develop slowly over several years. Therefore, acromegaly is mainly diagnosed in middle-aged adults. After taking a detailed medical history of the patient and studying the physical symptoms, the following tests are done:

• Blood Tests: Blood samples are collected and checked for elevated growth hormone levels or insulin-like growth factor 1 (IGF1). Measurement of IGF 1 is the most sensitive and accurate test available for diagnosing acromegaly. Growth hormone is often checked in conjunction with glucose tolerance tests. The patient is asked to consume sugar as this will decrease the growth hormone level in the blood in normal people. However, this growth hormone level will continue to be elevated in patients with acromegaly.

• Imaging Tests: Doctors may advise an MRI (magnetic resonance imaging) or a CT scan (computed tomography) to determine the size and location of the pituitary gland and the presence and absence of any pituitary tumor. X-rays are also advised to check excess bone growth.

• Other Pituitary Hormones: It may also be assayed if there is a pituitary tumor present to address the secretory effect of the tumor.

How Is Acromegaly Treated?

The primary goal of the treatment of acromegaly is to:

1. Bring the growth hormone to a normal level.

2. Decrease the size of pituitary tumors, thereby decreasing the pressure on the surrounding structures.

3. Maintaining normal functioning of the pituitary gland.

4. Reverse and improve associated symptoms (this can be different in different individuals).

Acromegaly is usually treated with the help of surgery, medication, and radiation therapy. The treatment is generally based on the patient’s age, overall health, presence or absence of a tumor, and individual symptoms. Standard treatment therapies include:

• Surgery: The standard procedure for treating acromegaly by surgery involves the removal of the pituitary tumor. Complete or partial removal of the pituitary tumor is done, and this procedure is called trans-sphenoidal surgery. This will immediately relieve pressure on surrounding structures and decrease the growth hormone level.

• Medication: Certain medication is given to patients whose surgery is contraindicated or in cases where surgery alone could not bring the growth hormone level down. Somatostatin antagonists (including Octreotide and Lanreotide), growth hormone receptor antagonists (including Pegvisomant), and Dopamine antagonists are used to treat acromegaly.

• Radiation Therapy: Surgery and medication is sometimes supplemented with radiation therapy. This therapy is used in patients in whom the surgery could not successfully reduce the size of the tumor or bring the growth hormone level down.

What Is the Prognosis of Acromegaly?

The prognosis of acromegaly is usually excellent if the condition is discovered in the early stages. Effective treatment will also prevent long-term and systemic complications of the condition. Following up with the doctor and keeping a close check on the hormones is important.

Conclusion:

Acromegaly is a disorder of the growth hormone affecting the growth and development of the individual affected by it. This disorder is characterized by enlargement of the limbs, jaws, and soft tissues. The symptoms of acromegaly usually reflect very late, and it is often diagnosed in middle-aged adults. Several systemic complications may occur if the condition is left untreated. The treatment of the condition usually involves; surgery, medication, and radiation therapy.