Patient's Symptoms Of Cushing's Disease Beginning To Abate After Endoscopic Pituitary Cure

Main Category: Endocrinology
Article Date: 29 Nov 2007 - 2:00 PDT

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Kathie Harrington's diabetes was out of control for three years, and as her insulin requirements, body weight, and blood pressure rose, she experienced a variety of other symptoms, including fatigue, muscle weakness and dry skin. Over time, she also developed a rounder face and a rounded contour on her upper back between her shoulders.

Puzzled and frustrated, Harrington made a January 2007 appointment with an endocrinologist she has seen off and on for 15 years. He recognized the symptoms of Cushing's syndrome and immediately ordered a series of tests and scans. When a tumor was found on Harrington's pituitary gland, the doctor referred the 59-year-old Las Vegas resident to Vivien Bonert, M.D, clinical director of the Pituitary Center at Cedars-Sinai Medical Center, and pituitary neurosurgeon Adam Mamelak, M.D.

In two endoscopic operations - one in April and one in June - Mamelak removed the tumor and the pituitary gland. Harrington quickly dropped 30 pounds, was able to cut her blood pressure medication in half, and reduced her insulin from 105 to 10 units per day. By December, she was down to eight units.

"The first operation was intended to remove the tumor, but because of its location, we were not able to visualize it, either by MRI before surgery or during surgery," said Mamelak. "Because Kathie's pituitary gland had already lost all of its hormone function except the production of cortisol, we opted to remove the entire gland in a second procedure to guarantee a cure. Cushing's disease is lethal if left untreated, so a cure needs to be achieved if at all possible in every case."

Many Cushing's disease symptoms are non-specific, and the rare disease often remains undiagnosed for years, with physicians treating one manifestation or another but failing to put the whole picture together.

"Cushing's syndrome" occurs when too much of the hormone cortisol, which is produced by the adrenal glands near the kidneys, circulates in the bloodstream. It may result from the long-term use of steroid drugs or a tumor in an adrenal gland or elsewhere in the body. "Cushing's disease" is one form of the syndrome, occurring when a tumor of the pituitary gland causes overproduction of the hormone that stimulates cortisol production. The pituitary gland is situated beneath the brain behind the sinuses.

A decade ago, neurosurgeons removing pituitary tumors typically used an operating microscope and a sublabial approach, which required an incision in the lip and destruction of nasal structures. Today, Mamelak employs an endoscope and an endonasal (through the nose) approach. The endoscope enters the skull base through the sphenoid sinus, which is located deep within the skull, below the pituitary gland.

"This procedure reduces pain considerably and leaves no visible scars, and using the endoscope in place of the microscope gives a more panoramic view that reduces some of the surgical risks," said Mamelak, who performs about 75 pituitary operations a year. Many neurosurgeons, even if they have adopted the endonasal approach, still use the operating microscope.

"We're working in a very narrow corridor, which is not ideal for the bulky, fixed microscope. With the microscope, you're working through a very narrow cone from a distance and trying to introduce instruments through the same corridor, pushing tissue out of the way with a speculum," Mamelak said. "The endoscope, on the other hand, fits within the nostril, giving a very wide field of view when you get up to the area of interest. Using the endoscope through the nose, we can do some operations that in the past could only be done by opening the skull. And because there is so little tissue destruction, we don't need to pack the nose after the operation. Patients are much more comfortable and very happy with the surgery."

Harrington, a speech-language pathologist who works with autistic children and their families, said she was told before surgery that she could expect to have very little pain, but she didn't really believe it. "There was no pain. Zero," she said. "It surprised me when I woke up. I thought I should be in pain but there was none."

With her over-stimulated pituitary gland removed, Harrington is relieved to see her symptoms diminish, although she is going through a temporary period of body aches.

"Cortisol is a very strong anti-inflammatory, and the body adjusts to the supranormal levels resulting from Cushing's disease," Mamelak said. "When the high level of cortisol is suddenly withdrawn, there is pain due to joint inflammation that was masked before surgery. Kathie is currently getting normal doses of cortisol through pills, and her pain will gradually subside."

Harrington will share her experience at a Pituitary Patient Awareness Conference on Sunday, Dec. 9. Mamelak and Bonert will be among other featured speakers. The conference is free and open to the public, and will be held from 9 a.m. to 3:30 p.m. at Hotel Casa Del Mar, 1910 Ocean Way, Santa Monica.

The first in Southern California and one of only 10 hospitals in the state whose nurses have been honored with the prestigious Magnet designation, Cedars-Sinai Medical Center is one of the largest nonprofit academic medical centers in the Western United States. For 19 consecutive years, it has been named Los Angeles' most preferred hospital for all health needs in an independent survey of area residents. Cedars-Sinai is internationally renowned for its diagnostic and treatment capabilities as well as breakthroughs in biomedical research and superlative medical education. It ranks among the top 10 non-university hospitals in the nation for its research activities and is fully accredited by the Association for the Accreditation of Human Research Protection Programs, Inc. (AAHRPP).

http://www.cedars-sinai.edu