Cushing's disease FAQs
- I have been told I might have Cushing’s syndrome and have been referred to an endocrinologist. Why is this?
- I have been told I need pituitary surgery. What does this involve?
- What are the risks of this surgery?
- Are there other risks associated with this surgery?
- How will I feel after the surgery?
- How will I know if my treatment has been successful?
- Will I feel better?
- What if the treatment is not successful?
1. I have been told I might have Cushing’s syndrome and have been referred to an endocrinologist. Why is this?
An endocrinologist is a physician who specializes in hormone disorders.
Cushing’s syndrome and disease are fairly rare and often quite complex
and the best results can be expected when these are treated by a specialist
endocrinologist, often together with a neurosurgeon.
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2. I have been told I need pituitary surgery. What does this involve?
The most common way to remove pituitary tumors is through the
transsphenoidal approach. This involves entering the pituitary gland by making
an incision in the back of the nose or above the teeth, and approaching the
pituitary gland through the sphenoid sinus. Using a microscope or endoscope, the
surgeon will explore the pituitary gland, hopefully find the tumor, and remove it.
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3. What are the risks of this surgery?
Since these tumors are very small, it can be difficult to find them, and the gland
can be damaged during the procedure. If this happens (about 10 –20% of the
time) other hormone function can be lost. Since the pituitary gland controls the production of thyroid hormone, estrogen in women and testosterone in men,
and growth hormone – in addition to ACTH – replacement therapy for these
other hormones might be required. Also, if the posterior part of the pituitary is
damaged, anti-diuretic hormone can be lost. This hormone is responsible for
water reabsorption by the kidneys, and without it patients urinate frequently and
in large amounts (“diabetes insipidus”), leading to dehydration. This hormone
can be replaced with a daily dose of a nasal spray or pill. These hormone
functions of the pituitary can be replaced with medication.
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4. Are there other risks associated with this surgery?
Since the pituitary gland is bordered by the optic nerves and carotid arteries,
there is a very small risk that these structures could be damaged (less than 1%).
However, if this were to happen, the patient could suffer visual loss or a stroke.
The pituitary is separated from the spinal fluid by a thin membrane. If this
membrane is damaged during the surgery, a spinal fluid leak can result. If spinal
fluid leakage occurs and is undetected, a serious infection, meningitis, can result. Most surgeons take a small piece of fat from the abdominal wall to use as a plug to
prevent this leakage from occurring. The risk of this happening is about 1%. Since
the pituitary gland is involved in water and sodium balance, this can be affected
transiently by the surgery as well, and your endocrinologist will monitor your
sodium levels for a few weeks after the surgery. All of these risks are minimized in
the hands of an experienced surgeon.
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5. How will I feel after the surgery?
Direct effects of the surgery include nasal congestion and possibly headache.
These symptoms will resolve after 1–2 weeks. If the operation is successful,
however, the cortisol levels will drop dramatically. Patients can experience
symptoms of cortisol withdrawal, which can include profound fatigue, and this
can sometimes last for weeks or months after the surgery. If the operation is
successful, the patient will have to take cortisol replacement until the remaining normal pituitary gland recovers.
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6. How will I know if my treatment has been successful?
Your endocrinologist will test your urine and blood cortisol levels a few days after
the surgery. Usually success can be determined within a few weeks of the operation.
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7. Will I feel better?
Almost all of the symptoms of Cushing’s disease are reversible. When the cortisol
levels drop, the obesity improves and appetite normalizes. Muscles and bones
become stronger. Diabetes and hypertension improve.
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8.What if the treatment is not successful?
There are a number of options if the initial transsphenoidal operation is
unsuccessful. Sometimes, a second operation is recommended if no tumor
was found during the initial operation. Alternatively, radiation therapy of the
pituitary gland can be considered. Medical control of cortisol levels is required
while awaiting the beneficial effects of radiation. Finally, the adrenal glands
themselves can be removed. This stops the body from making any cortisol, and
so the symptoms of Cushing’s disease resolve, although the pituitary tumor itself
remains untreated. Choosing between these options requires a careful discussion
between the patient, endocrinologist, and surgeon.
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