Adrenal Gland Removal Effective for Man With Cushing’s, Bone Fractures
Quality of life improved for patient after surgery to remove both adrenal glands
Adrenal gland removal was key to controlling high cortisol levels and improving the quality of life of a 33-year-old male patient with Cushing’s disease and recurrent bone fractures.
Researchers stressed that “when osteoporotic fractures occur at a young age, it is important to exclude secondary causes, such as Cushing’s syndrome.”
The case was detailed in a report, “Multiple Bone Fractures in a Patient With Difficult-to-Treat Cushing’s Disease,” published in the journal Cureus.
Osteoporosis is common symptom of Cushing’s
Cushing’s syndrome occurs when there are excessive levels of cortisol in the body. Cushing’s disease, the most common form of Cushing’s syndrome, is caused by an excess of adrenocorticotropic hormone (ACTH) produced by the brain’s pituitary gland, which in turn triggers the adrenal glands sitting atop the kidneys to produce large amounts of cortisol.
One of the common symptoms of Cushing’s is osteoporosis, a condition that causes bones to become fragile and more prone to fractures.
Early diagnosis of Cushing’s would help to slow disease progression in patients but the disease can be difficult to identify, particularly in mild cases.
In the study, a team of Portuguese researchers reported the case of a 33-year-old man with multiple bone fractures who was suspected of having Cushing’s syndrome.
He was diagnosed with a spine fracture and had gained 8 kg (about 17.6 lbs) in the past two years. He was taking paracetamol for pain relief and said he did not use corticosteroids.
The patient had several Cushing’s-like symptoms, including a “moon face,” muscle weakness, facial redness, and muscle wasting. He also had high blood pressure and fat accumulation in the abdomen, but no purple skin striae, or stretch marks. (Wide purple striae are often caused by excessive levels of cortisol).
Lab tests indicated the patient had high levels of cortisol in his urine and blood, as well as increased ACTH levels.
As Cushing’s disease is often marked by hypothyroidism (thyroid dysfunction), the man’s thyroid gland was physically examined, but no nodules were found.
The corticotropin-releasing hormone (CRH) stimulation test — which evaluates the responsiveness of the pituitary gland to produce ACTH in response to CRH — showed a large boost in ACTH levels in a period of 30 minutes, which finally established the man’s diagnosis of Cushing’s disease.
MRI showed no abnormalities in the patient. However, X-rays and bone scans revealed several bone changes in the chest, back, and ribs, which indicated osteoporosis.
The osteoporosis was found to be related to Cushing’s and the man started treatment with alendronate (commonly used to prevent and treat osteoporosis), calcium tablets, and vitamin D.
The patient underwent pituitary surgery, during which a lesion was identified and immediately removed. Further lab tests revealed there was an increased production of pituitary cells.
After surgery, the man developed hypogonadism — a condition in which the testes start producing little or no hormones — and hypothyroidism. Also, the levels of cortisol and ACTH remained high for three months.
The patient started treatment with ketoconazole, at daily doses of 200 mg/day, which gradually increased to 800 mg. This anti-fungal medication, sold under the brand name Nizoral among others, is sometimes used to treat Cushing’s in patients for whom surgery is not a option or has failed to lower cortisol levels.
The man’s blood pressure also increased, but was treated with proper medication.
Three months later, with cortisol levels still high, he underwent 20 radiotherapy sessions to the pituitary gland. However, his cortisol never returned to normal levels.
Adrenal gland removal deemed necessary as symptoms escalate
Meanwhile, the patient’s condition continued to deteriorate, and he experienced lack of energy, new fractures, and difficulty walking. He also needed help in his daily activities and had lost 25 cm (about 9.8 inches) in height.
All of these symptoms prompted the patient to undergo a bilateral adrenalectomy immediately — a surgery to remove both adrenal glands.
“Given the limited efficacy shown by ketoconazole and radiotherapy in the control of hypercortisolemia [high levels of cortisol], the deterioration of the general condition, and the detection of new osteoporotic fractures, it was decided that bilateral adrenalectomy (three years after the initial diagnosis of Cushing’s disease) would be undertaken for the immediate control of hypercortisolism [the same as hypercortisolemia],” the researchers wrote.
The surgery to remove both adrenal glands is usually performed in Cushing’s patients with other severe conditions, or when all other therapy choices have failed, according to the researchers.
Lab tests performed after the surgery showed a drop in cortisol levels in the patient. Improvements in energy levels, Cushing’s-like appearance, and blood pressure also were observed.
“Bilateral adrenalectomy rapidly improves symptoms in patients and avoids the long-term complications of elevated cortisol,” the researchers wrote. These complications include adrenal crisis — a life-threatening condition that occurs when not enough cortisol is present — and Nelson’s syndrome, “which requires long-term medical follow-up.”
Nelson’s syndrome is a condition characterized by the enlargement of a portion of the pituitary gland, accompanied by abnormal hormone production and release, and the development of large and invasive growths known as adenomas. The disorder is estimated to affect 25% of Cushing’s patients whose adrenal glands have been removed.
After his surgery, the patient in the case report lost 10 kg (about 22 lbs) and regained his independence. He is currently being treated with the corticosteroids hydrocortisone (30 mg/day), fludrocortisone (0.1 mg/day), and alendronate.
Imaging tests revealed no signs of Nelson’s syndrome. The patient’s ACTH blood levels have remained reasonable, and he has shown no acute adrenal insufficiency.