Empty nose syndrome

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Empty nose syndrome (ENS), is a iatrogenic debilitating and crippling nasal disorder, which occurs when too much of the nasal turbinates (especially the inferior turbinates) are resected in operations known as 'turbinectomies'. Over aggressive turbinectomies cause the nasal airway passages to be too wide and over exposed to the inspired airflow. This causes the remaining nasal lining to be too cold, too dry, over irritated and chronically inflamed. In many cases it has a similar drying and inflaming effect on the mucosal lining of the pharynx and sometimes even on the larynx. The chronically dry, cold and inflamed remaining nasal mucosa loses much of it's sensitivity to airflow and the patient feels constant stuffiness or as if the superior middle and superior region of the nose are unventilated, desensitized and unresponsive. This sensation is known as 'paradoxical obstruction' and is considered the hallmark symptom of ENS. The cold and dry over turbulent currents of airflow cannot be adequately processed by the nose and in addition to nasal dysfunction may cause pharyngeal and laryngeal chronic dryness and inflammation, known as 'dry pharyngitis' and 'dry laryngitis'. This may cause additional breathing difficulties, build up of mucus in throat which has to be constantly expelled, sleep and speech disturbances. The unprocessed cold and dry air can also cause chronic irritation of the trachea and bronchi of the lungs, resulting in chronic bronchitis.

"The symptom that most often indicates ENS is paradoxical obstruction: subjects may have an impressively large nasal airway because they lack turbinate tissue, yet they state they feel they cannot breathe well. There is no clear way to describe the breathing sensation that patients with ENS experience. Some patients may state that their nose feels “stuffy,” for lack of a better word, whereas others state their nose feels too open, yet they cannot seem to properly inflate the lungs; they feel they need some resistance to do so. Patients with ENS do not sense the airflow passing through their nasal cavities, whereas their distal structures (pharynx, lungs) do detect inspiration; the patients’ central nervous systems receive conflicting information. These patients seem to be in a constant state of dyspnea and may describe the sensation of suffocating. The constant abnormal breathing sensations cause these patients to be consistently preoccupied with their breathing and nasal sensations, and this often leads to the inability to concentrate (aprosexia nasalis), chronic fatigue, frustration, irritability, anger, anxiety, and depression."

(Houser SM. Surgical Treatment for Empty Nose Syndrome. Archives of Otolaryngology Head & Neck Surgery\ Vol 133 (No.9) Sep' 2007: 858-863).

Symptoms

As breathing is a bodily function that has perhaps the most pronounced effect on both the physical and mental states of the patient - it is not surprising that ENS sufferers report a wide array of bothering symptoms which significantly impair their physical functions and mental sense of well-being. The main symptoms are:

1. Paradoxical Nasal Obstruction: severely diminished sensation of airflow in the nose, which is known as "paradoxical obstruction", the paradox being that although the nasal passages are very large (following the turbinectomy) their mucosa is dry and insensitive to airflow. In addition – the disruption of normal airflow patterns robs entire sections of healthy remaining nasal mucosa from most of the airflow. Both factors cause the patients to be incapable of sensing the air flowing through their noses, although there is no physical obstruction there. The sensation is not necessarily of a mechanically blocked nose, but rather of the opposite – an absent or nonexistent nose – which results in the same thing – the brain feels that the nose is not ventilated enough. Never the less it's impact can be devastating to the sense of well being and quality of life. 2. Dry Pharyngitis and/or Laryngitis: chronically dry and/or inflamed mucosal lining of those regions that intensify breathing, sleep and speech disorders. Often, patients report a large build up of phlegm in the throat which causes much gagging and discomfort. 3. Sleep disturbances: Caused by chronic dryness and/or inflammation of the nasal and pharyngeal mucosal lining. Quality of sleep is low and patients wake up depressed and unrefreshed. 4. Pulmonary over sensitivity or Chronic Bronchitis: caused by insufficient nasal heating and humidification of the inspired air to the bronchi of the lungs. This overtaxes the lungs and causes over sensitivity of the bronchi walls, resulting in difficulty breathing in deep. Some think that is also due to loss of too much nasal resistance to the lungs, which need this resistance to maintain their elasticity and ability to inflate to their maximum capacity. 5. Speech problems: the nose's reduced abilities to heat and humidify the inspired air causes chronic dryness and inflammation not only to the nasal lining but also to the lining of the regions of the nasal pharynx (back of the nose), the oto-pharynx (back of the mouth), and sometimes the larynx too (which includes the vocal chords region). The pharynx, larynx and nose are all parts of the speech aperture, and chronic dryness and inflammation of their mucosa causes difficulty to project speech and a weak voice. 6. Cognitive problems: nasal caused breathing disorders are known to cause a serious problem concentrating on mental tasks. It has been termed in medical literature as "aprosexia nasalis". 7. Clinical Depression: the sensation of luck of nasal ventilation (paradoxical obstruction), the chronic lack of sleep and the general fatigue that accompany the chronic breathing difficulties are most destructive to the sense of well being of the patients and may cause severe anxieties and depression. It is estimated that 52% of ENS patients develop clinical depression.

Additional nasal related problems affecting some ENS patients are: chronic sinusitis, vasomotor rhinitis, epitaxis, crusting and nasal pain, increased sensitivity to upper and lower respiratory infections of all kinds.

The long-term risks of nasal related breathing problems are well known and include a high risk of cardiovascular and pulmonary complications that stem out of long term breathing difficulties and sleep disordered breathing..

Etiology

The turbinates, especially the inferior and middle nasal turbinates, divide the nasal airway into three main passages of air: the inferior meatus, the middle meatus and the superior meatus. By doing so they effectively increase the inner nasal tissue surface upon which the inspired air is forced to flow, thus achieving as much as possible heating, humidification and filterization of the inspired air.

Sometimes the turbinates become chronically swollen in such a way which causes too much nasal obstruction. ENT and plastic surgeons decrease their volume of tissue in procedures known as 'turbinectomis'. However this is a wide code name that might mean anything from minimal reduction to complete resection of an entire turbinate. While careful and judicial conservative reductions of a turbinate's volume can be beneficial to the patient, an aggressive turbinectomy, in which most or all of the main turbinal body is resected, can be a devastating procedure that causes ENS.

Most of the inhaled and exhaled air through the normal nose has to pass through the middle meatus – which is the narrow groove-like empty passage left between the top of the inferior turbinates and the bottom of the middle turbinate. When either the inferior or the middle turbinate (or both) are over resected (in particular at their front) this passage becomes grossly enlarged, and this can have a dramatic adverse effect on nasal functions resulting in chronically dry and/or inflamed nasal mucosa, diminished nasal sensations or airflow, entire nasal mucosal sections deprived of airflow and sometimes chronic sinusitis, and various degrees of chronic irritation to the lining of the pharynx, larynx, trachea and even the bronchi in the lungs. For example: Over resection of the inferior turbinates, which are the largest nasal turbinates – causes the entire volume of airflow converges into the inferior meatus (along the floor of the nose) therefore – depriving the middle and superior nasal cavities of receiving proper ventilation, and strips the inferior and middle regions of the mucosal lining of it's much needed rates of heat and moisture ruining their ability to safe guard the rest of the respiratory tracts – the pharynx, larynx, trachea and bronchi of the lungs, from cold, dry and unfiltered air, which is the most important role of the nose. Over resection of the middle turbinates causes the same problems, although perhaps to a lesser degree, but also exposes the sinuses to more irritation of cold, turbulent and dry jets of airflow currents. The severity of symptoms in ENS mostly correlates with the degree of resection of the turbinates, although sometimes other more common abnormalities like a deviated septum – can actually cause some compensation and reduce the severity of the symptoms, even in cases of radical turbinectomies.

Diagnosis

Diagnosis of ENS is a challenging issue. Although the mucosal lining of the 'empty nose' is often chronically dry and/or inflamed it is difficult to detect the severity of this condition during a regular visual inspection. A biopsy will probably show various degrees of nasal atrophy or rhinitis sicca of the mucosa in the suspected areas. However the condition of the remaining mucosa is seldom as bad as is seen in the progressive inflammatory condition of atrophic rhinitis. This causes many ENT specialists to over look signs of ENS and under-diagnose it, as their professional eye is trained to search for visible symptoms of atrophic rhinitis and not for what many of them consider as "harmless" degrees of mucosal dryness, which is a condition more consistent with most cases of ENS. Therefore the diagnosis of ENS must rest upon comparing the symptoms reported by the patient to the findings of clearly abnormally wide nasal passages in a CT scan. To finally verify that the patients complaints are caused from his/hers over enlarged nasal passages it is possible to conduct a cotton test: in which the clinician carefully places a rolled piece of cotton wool soaked in saline to try to replace the natural deflection of airflow that would have been caused had the missing turbinate been in place. If done by a well experienced clinician the patient should feel an immediate improvement in all symptoms.

Besides the objective difficulties in diagnosing ENS, there is the problem that ENS a iatrogenic nasal disorder, meaning that it is caused because of a medical error of judgment resulting in over resection of the nasal turbinates. This makes it psychologically very difficult for many doctors to admit that they or their colleagues might have worsened the patients condition instead of helping them, and adds to the high rates of ENS patients that are not properly diagnosed and treated. As a direct result of this misdiagnosis and failure to report cases, until recently ENS has been considered to be a statistical rarity. But, with the increasingly growing understanding of how the nose controls and processes the airflow, and increasingly growing awareness of the adverse effects of radical turbinectomies, ENS is no longer considered a rarity by most ENT specialists.

Terminology

The term "empty nose syndrome" was originally coined in the early 1990s by Dr. E.B. Kern (MD.) who was at the time head of the otolaryngology ward in the Mayo Clinic in Rochester, Minnesota, USA. He and his colleagues began to notice that more and more patients who had undergone aggressive resections of their inferior or middle turbinates seemed to develop symptoms of nasal obstruction and shortness of breath even though their noses appeared to be wide open, following partial or total turbinectomies. Other hallmark symptoms were chronic nasal dryness, difficulty concentrating, and often clinical depression. They found that all these symptoms and more, in all the patients examined, developed only after their inferior or middle turbinate were over aggressively resected.

All the patients had CT scans that showed abnormally wide and empty looking nasal cavities, thus they called it - "Empty Nose Syndrome".

ENS is often referred to also as 'secondary atrophic rhinitis', because it is believed that the over exposed and wide cavities may become atrophic over time ('secondary'= caused by surgery or other medical intervention, or direct trauma to the nose, as opposed to 'primary' which develops because of systemic illnesses). However, developing a grossly atrophic mucosa on top of ENS is not a prerequisite for diagnosing a post-turbinectomy patient with ENS.

Treatment options

Non-surgical treatment options are meant to maintain and slightly improve the health of the remaining nasal mucosa in the ENS nose, by keeping it moist and free as possible from irritation and infection.

Surgical treatment is meant to try to permanently improve the severity of the symptoms.

Non-surgical treatment

Non-surgical treatments will not cure ENS, because it cannot restore the missing turbinates, but it can help control some of the symptoms and make the suffering more tolerable:

• Daily nasal irrigations of regular saline are always recommended.
• Sesame oil can help in cases of extreme dryness and crusts.
• Sleeping with a cool mist humidifier.
• Sleeping with a CPAP machine that has a built-in humidifier.
• Some patients respond well to orally taken vitamin A and D.
• Acupuncture meant to improve nasal blood supply and nerve function.

Surgical treatment

Turbinate tissue is unique and there are no potential donor sites in the body to harvest similar tissue from. However, in the nose, Form = Function. It is therefore possible to restore some function by restoring the natural contours and proportions of the nasal passages: It is possible to create an artificial look alike structure of a turbinate in the nasal cavities, and thus - to regain some of the nose's capabilities to adequately pressurize, streamline, heat, humidify, filter and sense the airflow.

The bulking up of the sub-mucosa and mucosa to create a neo-turbinate structure can be achieved through implanting some supporting material between the bone/cartilage and the submucosal layer. Many materials have been tried over the past 100 years. In most cases this operation was used to restore heat and humidity to atrophic noses.

Generally speaking - the implant materials can be divided into 3 groups:

• autografts: bone, cartilage, fat, etc' from one site to another in the same patient. The problems here are relative shortage of tissue, and long term studies have shown high absorption rates in the nose.
• foreign materials: such as - hydroxyapatite, fibrin glue, Teflon, gortex, and plastipore, which solve the shortage problem of autografts, are easy to shape and don't tend to get absorbed. However they have a high extrusion rate, and sometimes cause infection.
• allografts: In the last decade scientists have been able to harvest and remove away genetic markers of some basic human tissues (like skin dermis) from donors, and thus supplying a human natural implant material which will not stimulate the immune system to reject it. A good example for such material is acellular dermis (brand named - "Alloderm"). It does not get resected and in most areas retains most of it's volume over long periods.

Alloderm implants have already been implanted successfully for a few years now in a small but growing number of ENS patients. At four years follow-up, results seem stable and encouraging. It seems that Alloderm implants cannot fully cure ENS but can help alleviate much of the suffering, with various degrees of success, depending on the individual condition of each patient. Dr. Steven Houser from Cleveland is an American ENT surgeon who has gained probably the most extensive experience to date in surgically treating ENS.

The ideal implant material, other than real original turbinate tissue (which is still an impossibility at this time and age), should be something with low extrusion and rejection rates, minimal infection risk, and very importantly - that will provide a strong and endurable enough structure and at the same time allow good permeability for blood vessel incorporation, which seems to be the key against long term absorption.

Additional Citations

"… The excess removal of turbinate tissue might lead to empty-nose syndrome. Excess resection can lead to crusting, bleeding, breathing difficulty (often the paradoxical sensation of obstruction), recurrent infections, nasal odor, pain, and often clinical depression. In one study, the mean onset of symptoms occurred more than 8 years following the turbinectomies.”

(From: “The turbinates in nasal and sinus surgery: A consensus statement.” By D. H. Rice, E. B. Kern, B. F. Marple, R. L. Mabry, W. H. Friedman. ENT – Ear, Nose & Throat Journal, February 2003, pp. 82-83.)

“Removal of an entire inferior turbinate for benign disease is strongly discouraged because removal of an inferior turbinate can produce nasal atrophy and a miserable person. Such people unfortunately are still seen in the author’s offices; these people are nasal cripples.”

(From: "Otolaryngology – Head and Neck Surgery", Page 496, chapter 23. Chapter written by Dr. Kern. Book by Dr. Meyyerhoff and Dr. Rice, published by the W.B. Saunders Company, 1992).

“Total inferior turbinectomy has been proposed as a treatment for chronic nasal airway obstruction refractory to other, more conservative, methods of treatment. Traditionally, it has been criticized because of its adverse effects on nasophysiology. In this study, patients who had previously undergone total inferior turbinectomy were evaluated with the use of an extensive questionnaire. It confirms that total inferior turbinectomy carries significant morbidity and should be condemned.”

(from – “Extended Follow-Up Of Total Inferior Turbinate Resection For Relief Of Chronic Nasal Obstruction”, G. F. Moore, T. J. Freeman, F. P. Ogren & A. J. Yonkers., Laryngoscope, September 1985, pp. 1095-1099.)

"... The inferior turbinal should never be entirely removed... Excessive removal allows a jet of inspired ventilation, the mucus evaporates and becomes so viscid as to impede ciliary action... In some cases where the inferior turbinal has been too freely removed, the loss of valvular action and undue patency of the nostril produce the discomfort of dry pharyngitis and laryngitis, with difficulty in expelling stagnant secretion from the nose. The loss of the turbinal may lead to a condition simulating atrophic rhinitis or even ozaena."

(Thomson St. C & Negus VE. Inflammatory diseases. Chronic Rhinitis. Diseases of the nose and throat, 6th edition. London: Cassel & Co. Lmt. 1955; 124-145).

"...Resistance to air currents on inspiration and during expiration is necessary to maintain elasticity of the lungs."

(Cottle MH. Nasal Breathing Pressures and Cardio-Pulmonary Illness. The Eye, Ear Nose and throat Monthly. Volume 51, September 1972.)

Additional images

• A schematic sketch of the Right nasal airway passage with all three turbinates intact.
  A schematic sketch of the Right nasal airway passage with all three turbinates intact.
• 
  Right inferior turbinate with its front cut off, showing the unique turbinated structure of the turbinate.
• An internal view of the front inferior part of the nasal airways, after total inferior turbinectomy. Note how empty the inferior passages are.
  An internal view of the front inferior part of the nasal airways, after total inferior turbinectomy. Note how empty the inferior passages are.
• 
  Empty Nose Syndrome after partial inferior turbinectomy (only 25% of inferior turbinates remaining).
• 
  Empty Nose Syndrome after total bilateral inferior turbinectomy + bilateral uncinectomy and destruction of the lower lateral walls.
• 
  All turbinates removed - Right lateral wall view.
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  Anatomy of the nasal cavity
• 
  The skull from the front.
• 
  Sagittal section of nose mouth, pharynx, and larynx.

External links

• Dr. Houser's tutorial pages on ENS
• Dr. Grossan's ear, nose and throat pages on ENS
• Listen to Dr. Kern's lecture on Empty Nose Syndrome
• Interactive Forum for ENS Patients

• "Having nasal surgery - Don't you become an Empty-Nose Victim"