Subglottal Stenosis in Wegener's Patients - Robert Lorenz, M.D., F.A.C.S.

Dr. LorenzDr. Robert Lorenz is an ENT physician and surgeon at the Cleveland Clinic Foundation (CCF) in the Department of Otolaryngology, where he is Section Head of Head and Neck surgery. His area of special interest for Wegener’s patients is the larynx. WG is unique in its ability to involve the larynx while the other vasculitides do not. When subglottal stenosis affects the larynx, the cause can be either WG or idiopathic (of unknown origin), or sometimes due to trauma such as breathing tubes.

Almost all WG patients have, to some extent, involvement with their nose and ears. However, few WG patients have problems with their larynx. Only 20% of adult WG patients have subglottal stenosis. This jumps to 50% in patients under 20 years of age, the reason for this unknown. In children, the SS can remain as an issue for years. Dr. Lorenz finds that as long as the WG is not active, the SS stays quiescent . Once the patient has an exacerbation, the SS can flare and then the body’s natural ability to create a stenosis from the scar occurs and the physician and patient struggle to get the airway widely patent while preventing further scaring. SS is a disease that you either have or you do not - there does not seem to be “a little subglottal stenosis”.

Dr. Lorenz has a basic philosophy when it comes to treating subglottal stenosis. If a person is not limited in their activities by their pathology then there should be no intervention. This is an extremely important point. Some physicians may tell a patient that they have a little subglottal stenosis and that something should be done. Unless a patient says they are having problems, Dr. Lorenz will not perform surgery. The only exceptions to this are in patients who are anticipating problems, such as someone needing to undergo general anesthesia and their airway is too tight to get a breathing tube down, or in a pregnant woman who will need a widely open airway to go through the added stress of labor without a restricted airway, but these scenarios are very rare.

This ties into the second part of his philosophy; the more you intervene, the more scarring that will result along with long-term problems. The larynx is an extremely delicate organ and the more you traumatize it, the less chance of recovery in the future. Dr. Lorenz says “Be gentle and do the least amount of trauma to the voice box“.

An important point to understand about subglottal stenosis is that it acts independently of generalized WG disease and systemic therapies. If somebody flares, it doesn’t mean the larynx will get worse. If the larynx is getting worse, it does not mean you are in a WG flare. Lastly, it means that systemic immunosuppression usually does not help reduce the severity of the subglottal stenosis.

Problems do not usually arise unless there is a 50% or greater reduction in the airway. At this point, one returns to the philosophy of “are you having problems?”. You may have a 50% reduction yet not have any significant problems and so you would not have any treatment. Older patients seem to be more subjectively affected with SS - patients over 50 tend to feel more confined by it while younger patients tend to do better.

Dr. Lorenz tries to perform as few surgeries as possible and when he does, the goal is to be as atraumatic as possible. Under general anesthesia, working through the mouth with no tracheotomy or neck incisions in a "minimally invasive", endoscopic manner, the first part of the treatment is the injection of localized steroids which seems to be the most important part of preventing the stenosis from returning. Next, the stenosis is lysed with a small endoscopic knife, and thereafter, the stenosis is lightly dilated. It is very important that the dilation part of the procedure is done very gently so that no further scar tissue is caused. Lastly, a topical application of mitomycin-C is used and the patient is awakened. So far, as long as the patient has not had previous airway surgery by a different surgeon prior to Dr. Lorenz, he has never had to perform a tracheotomy or other more invasive surgeries, and the minimally invasive approach has been able to maintain the airway open. Repeat procedures may be needed at intervals of 8-12 months, but usually are limited to 3 procedures.

Dr. Lorenz does not personally perform the radial incision in the larynx with the laser because there is some evidence that lasers are more traumatic to the larynx in animal studies, but the new generation of lasers may be able to avoid this problem.

Most ENT’s are surgeons, but are generalists who treat all things such as allergy, children, adult, cosmetics, ear, sinus, etc. There are very few with significant interest and experience with Wegener’s and other vasculitides. There are only a handful of ENT’s who have significant experience with subglottal stenosis.

Disease in the sinuses ranges from minor inflammation all the way to tissue destruction. At the least, there is usually some redness, swelling and some crusting. In the worst cases, the septum has completely eroded leaving a large nasal cavity with no real sinuses left.

The difference between disease in the nasal cavity and in the sinuses is as follows … Disease in the nasal cavity centers around crusting, bleeding and a destroyed septum. Disease in the air-filled spaces of the sinus cavity is primarily infection or sinusitis. Sinus surgery is rarely performed when a person is in a WG flare. Once a diagnosis comes back, organ systems are checked, treatment with meds is decided to bring the WG under control and with proper treatment, the nose tends to get better and surgery can be avoided.

It is extremely important to take good care of your nasal and sinus passages. Proper hygiene is the primary method of preventing problems in our WG patient population of approximately 200. It is important for all patients to know the complications that can arise from poor hygiene. They include acute sinusitis, mucoceles (walled off area of mucous which can eventually involve neighboring tissues like the eyes and brain), and ear problems.

Dr. Lorenz performs surgery only with the larynx in WG patients. If care is needed for the sinuses, he refers them to Dr. Citardi or Dr. Batra, both also at the CCF. Ear problems are referred to a specialist as well.

Dr. Lorenz is from New York, north of New York City in the Hudson Valley. His parents were immigrants from Europe after WWII. He currently lives with his wife, who is also a physician, in Shaker Heights, Ohio.

When Dr. Lorenz is not in Cleveland seeing patients and performing surgery, he spends much of his time lecturing. In May he was in Chicago followed by trips to Colombia, Panama, Turkey and then on to Baltimore for the Vasculitis Foundation Symposium in July. When he is not taking care of patients, he likes to relax at home with his wife, Aylin.

As a college student at Yale, Dr. Lorenz became involved with an international medical equipment aid group and visited hospitals in Central America. This is what really inspired him to become a doctor. He is the first member of his family to become a doctor. Dr. Lorenz has also been on medical mission trips to Africa.

Dr. Lorenz credits Drs. Isaac Eliachar and Gary Hoffman as fantastic teachers who taught him an incredible amount about vasculitis patients. Dr. Bob Lebovics and Dr. Carol Langford have also been excellent collaborators.

Dr. Lorenz states that the best part of his job is making a person who has a severe airway narrowing which affects their daily life, breathe normally again. He feels that WG patients are wonderful people - folks just like him, but were very unlucky to develop a disease that could affect anyone. His practice is limited to laryngotracheal reconstruction and Head and Neck tumors.