Cushing's disease is caused by the growth of a benign tumor in the pituitary gland that stimulates the over-production of the hormone cortisol. Total hypophysectomy is a surgical procedure to remove to the pituitary gland and is a treatment course pursued in extreme cases of Cushing's disease. Total hypophysectomy carries numerous risks and is performed rarely, and typically only when other treatment measures prove ineffectual.
Cushing's Disease
The pituitary is a gland located at the base of the skull and is part of the body's complex endocrine, or hormonal system. When a person has Cushing's disease, the development of a pituitary tumor causes the body to produce higher-than-normal levels of adrenocorticotropic hormone, or ACTH. Elevated levels of ACTH transmit signals to the adrenal glands to manufacture more of the steroid hormone cortisol. Cortisol plays an integral role in stress response and is also vital to the function of the immune and nervous systems, and the metabolism of fats, proteins and carbohydrates. The symptoms associated with Cushing's disease result from the sustained over-exposure to cortisol in the body.
Signs and Symptoms
Cushing's disease produces a range of symptoms affecting the skin, neurological function, and the muscular and skeletal systems. Patients with Cushing's disease will typically experience upper body obesity accompanied with thin arms and legs, reddening and swelling of the face, vulnerability to skin infections and the appearance of purple marks across the thighs, abdomen and breasts. Cushing's disease is frequently accompanied by the growth of a fatty protrusion between the shoulder blades, often referred to as a "hump," as well as muscular degeneration and a loss of bone density. While Cushing's disease can be managed by drug therapies, and possibly aided by diet and exercise in general the disorder has a poor prognosis, and if it is not adequately treated it can result in death.
Hypophysectomy
The treatment of Cushing's disease may involve the removal of the pituitary tumor, a procedure known as hypophysectomy. A total hypophysectomy is the removal of the entire pituitary gland, and may be recommended as an option for treatment in cases in which a tumor has grown back after initial surgery or in advanced cases of the disease. Total hypophysectomy is performed by neurosurgeons or surgeons who specialize in endocrinology, who typically access the tumor through the nasal cavity.
Complications
Total hypophysectomy is an extreme surgical intervention and carries the risk of numerous complications. Following the procedure, patients will need to take medications to support endocrine function and may continue to experience adverse symptoms associated with hormonal imbalance as well as potentially reduced adrenal gland function. According to the Mayo Clinic, some patients never regain complete adrenal function and may require lifelong medication. Infection and post-operative nasal bleeding are also potential risk factors, and following surgery most patients are advised to take a course of antibiotics to prevent meningitis or the growth of an abscess.
