Outside the Box with Chronic Rhinosinusitis
Gayle Adamson has had sinus disease for virtually her entire life. She started taking allergy shots in her late teens in an effort to mitigate her symptoms, but by the time she was in her early 30s, she was constantly congested and living on nasal spray. Around the age of 35, she underwent an endoscopic sinus surgery that changed her life.
“Before the surgery, I had polyps and about 10 percent breathing capacity left,” Adamson says. “The surgeon basically rebuilt my sinuses, and my ability to breathe improved dramatically. The procedure gave me a good 15 years without significant sinus issues. I remember having relatively few problems between 1991 and 2006.”
Adamson had to stop taking allergy shots in August 2006 at the age of 50, when she was prescribed a beta-blocker for hypertension. In October of that year, she saw an allergist who prescribed fluticasone nasal spray (Flonase®) and saline nasal rinses for symptoms of allergic rhinitis. Her symptoms resurfaced again in late 2006, and she scheduled an appointment with an otolaryngologist who suspected her sinus infection might be related to a fungus. A few months later, after a course of antibiotics, prednisone and a cocktail nebulizer of antibiotic-steroid-antifungal medication, her condition improved, but only transiently.
In late 2007, Adamson was still having nasal symptoms and coughing up yellow phlegm. A CT scan of her sinuses showed some “questionable gray areas.” A nasal culture revealed a staphylococcal infection, and she was again treated with oral and topical antimicrobials, which seemed to help the sinus infection but the chronic productive cough persisted. Her doctor ordered chest CT, which revealed an area in her lung with mucous impaction or bronchiectasis, a localized, irreversible widening of the large airway. More drugs were prescribed.
By February 2008, her sinus symptoms had returned. During the months that followed, she was prescribed various medications for chronic rhinosinusitis (CRS). In July of that year, she had a second sinus CT that showed sphenoid sinusitis, prompting a second endoscopic sinus surgery. A culture done on follow-up showed the presence of methicillin-resistant Staphylococcus aureus (MRSA), a bacterium responsible for several different types of difficult-to-treat infections. She took various medications for the infection, including steroids, antibiotics, antifungal medications and other drugs.
The remainder of 2008 and beginning of 2009 brought more of the same for Adamson. After nearly two years of treatment for recalcitrant CRS, her otolaryngologist noticed something unusual about her case. “Once I’d healed after my second endoscopic sinus surgery, he found an area of exposed bone high in my septum,” Adamson says. “That was a red flag for further exploration before we jumped into another course of antibiotics or other treatments. He ordered a SPECT bone scan that revealed a hot spot in the septum, but because of limited resolution, it wasn’t clear whether the exposed bone was infected or necrotic.”
For a second opinion, he referred her to Samer Fakhri, MD, FACS, FRCS(C), a faculty member at the UTHealth Otorhinolaryngology Texas Sinus Institute and a staff member at Memorial Hermann-Texas Medical Center. “When I reviewed Gayle’s medical record, it was apparent that my otolaryngology colleague had done an excellent job of managing her condition,” says Dr. Fakhri. “His intuition was correct that there might be something unusual in her disease process. When I examined her, it became immediately clear to me that her current problem was not the typical post-sinus surgery issue. I identified an area of exposed bone with associated infected material in the back of her nasal septum and sphenoid sinus.”
Dr. Fakhri ordered special testing for unusual bacteria, and the culture came back positive for Mycobacterium abscessus, a bacterium distantly related to those that cause tuberculosis and leprosy. Part of a group of ubiquitous environmental mycobacteria, M. abscessus is found in water, soil and dust and, according to the Centers for Disease Control and Prevention, has been known to contaminate medications and products, including medical devices. In May 2009, Dr. Fakhri ordered a nuclear medicine scan that confirmed that the bone located in the back of the septum and deep in her sinuses was infected.
He suspected that the impacted secretions seen on CT in her lungs might be related to the same atypical organism and consulted with Adamson’s pulmonologist, who ordered a diagnostic lung lavage that produced evidence of M. abscessus in the lung. “Often, we find a unified disease process in the upper and lower airway, so it’s not unusual, for example, to see the coexistence of chronic rhinosinusitis and asthma in patients,” he says. “This is a functional model with increasing support in the medical literature: what occurs in the sinonasal area will likely affect the lungs. Or, conversely, a disease mechanism that begins in the lungs may ultimately involve the nose and sinuses. Gayle’s background history of allergies, polyps and possible reactive lower airway fits into that model and may have somehow set the stage for the development of a super infection with this very unusual organism. We can’t be certain whether the infection started first in the nose or in the lungs – it’s a kind of classic chicken-and-egg dilemma. We don’t really know where or how the problem originated, but we suspect the nose was inoculated first.”
In early June of 2009, Dr. Fakhri took Adamson to the operating room, where he removed approximately 3 to 4 centimeters of infected tissue and bone. “The surgery was quite involved due to the extent of the infection in the bone,” he says. “I had to remove bone and tissue up to the skull base because the infection had reached that area.”
He coordinated Adamson’s postsurgical care with Charles Ericsson, MD, a professor of medicine in the Division of Infectious Diseases at McGovern Medical School. Under his direction, she underwent a six-week course of intravenous antibiotics, followed by long-term oral antibiotic treatment of 18 months. During this time she showed no evidence of recurrence on nasal endoscopic exam.
“Our final step was determining the endpoint of antibiotic treatment, and we decided that we would withdraw antibiotics when we had no evidence of infection on nuclear medicine bone scan,” Dr. Fakhri says. Adamson’s bone scan in June 2010 was normal. A repeat CT scan of her lungs showed resolution of the bronchiectatic area with impacted secretions. She has been off antibiotics since Jan. 4, 2011, with no recurrence.
“Most patients referred to me have stories similar to Gayle’s. They’ve tried virtually every treatment available and still have recalcitrant disease,” Dr. Fakhri says. “As a result, I have to think outside the box from the beginning. I start my evaluation of new patients with a high index of suspicion that something unusual may be contributing to their condition. Why does a patient have continued disease despite years of treatment?”
Adamson was also searching for answers. “I kept asking myself, how did I get this? Based on the results of all the tests they ran, it appears that somewhere along the way my immune system might have been depressed, possibly due to a lifelong history of allergies, and something occurred that exposed me to the mycobacterium, whether it was due to the tap water I was using in nasal rinses or something I was exposed to as a result of exams or surgery.”
Follow-up was critical in Adamson’s case. “I followed Gayle closely for a year and found no evidence of disease,” Dr. Fakhri says. “Because the presence of Mycobacterium abscessus in the nose and lungs is rare, we don’t have a lot of data on the recurrence of this type of condition. We believe a recurrence is unlikely unless the same predisposing factor surfaces again. But that’s a low possibility.”
Adamson is grateful to have found Dr. Fakhri. “He is brilliant and caring. I was truly blessed to have been referred to him,” she says. “I sincerely believe that his ability to accurately diagnose the type of infection I had, the surgery he performed and his follow-up treatment saved me from a potentially negative outcome.”