Acromegaly

Acromegaly

• Acromegaly is a syndrome that results when the anterior pituitary gland produces excess growth hormone (GH) after epiphyseal plate closure at puberty.
• A number of disorders may increase the pituitary's GH output, although most commonly it involves a GH-producing tumor called pituitary adenoma, derived from a distinct type of cell.
• Acromegaly most commonly affects adults in middle age, and can result in severe disfigurement, serious complicating conditions, and premature death if unchecked.
• Because of its pathogenesis and slow progression, the disease is hard to diagnose in the early stages and is frequently missed for many years, until changes in external features, especially of the face, become noticeable.

Acromegaly is often also associated with gigantism.

Features that result from high level of GH or expanding tumor include:

1. Soft tissue swelling visibly resulting in enlargement of the hands, feet, nose, lips and ears, and a general thickening of the skin.
2. Soft tissue swelling of internal organs, notably the heart with attendant weakening of its muscularity, and the kidneys, also the vocal cords resulting in a characteristic thick, deep voice and slowing of speech.
3. Generalized expansion of the skull at the fontanelle.
4. Pronounced brow protrusion, often with ocular distension.
5. Pronounced lower jaw protrusion with attendant macroglossia (enlargement of the tongue) and teeth gapping.
6. Hypertrichosis, hyperpigmentation, and hyperhidrosis may occur in these patients.
7. Acrochordon (skin tags).
8. Carpal tunnel syndrome.Carpal tunnel syndrome.

• Treatment:

The goals of treatment are to reduce GH production to normal levels, to relieve the pressure that the growing pituitary tumor exerts on the surrounding brain areas, to preserve normal pituitary function, and to reverse or ameliorate the symptoms of acromegaly. Currently, treatment options include surgical removal of the tumor, drug therapy, and radiation therapy of the pituitary.