Empty nose syndrome

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File:Samples-of-ENS-CT's.jpg
CT pictures depicting different types of abnormal nasal anatomy following turbinectomies that result in ENS.

In otolaryngology, empty nose syndrome (ENS) describes a nose that has been physiologically crippled by excessive surgical removal of turbinates in the nose (mainly the inferior turbinates) in a surgical procedure known as 'turbinectomy' or 'conchotomy'. It is therefore an iatrogenic condition that can and should be avoided.

Main symptoms

The main physical symptoms are:

  1. Chronic nasal dryness Often leads to chronic mucosal inflammation and pain. Chronic inflammation can cause areas of the mucosa to atrophy and this is why some studies refer to this condition as "secondary atrophic rhinitis".[1][2][3][4][5] At least one large study concluded that it can take 7.1 years, on average, for symptoms of atrophic rhinitis to appear and urges the examining physician to look for early signs and to try to counter them with self-administered daily nasal irrigations and moisturization by the patient.[6]
  2. Paradoxical obstruction - The feeling that the nose is stuffy, often accompanied by a constant or frequently occurring troubling feeling of suffocation generated by poor airflow feedback from the nasal mucosa, either because of atrophy of the trigeminal nerve endings that are supposed to generate this sensation, their lack of responsiveness because of the mucosa becoming too dry or going through metaplasia, or simply because of the loss of normal nasal aerodynamics following the turbinectomy. Without good and constant sensation of the air flowing through the nose, the nervous feedback to the central nervous system is that of suffocation and or undifferentiated breathing difficulties arise.[7][8]
  3. Neuropathic pain - Chronic inflammation and neurophatic pain in nose, pharynx, eustachian tube, throat, larynx, trachea, in more severe cases - in bronchi and lungs. Chronic hoarse voice and cough can also take place. This is caused by insufficiently processed (moisturised, warmed, cleaned) air passing through the respiratory system. ENS can also serve as an prerequisite for asthma.

Other predominant symptoms are lack of a good night's sleep and chronic fatigue.[9] The chronic nature of the physical symptoms has a significant impact on the patient's quality of life and sense of well-being, causing difficulty concentrating, pre-occupation with symptoms, anxiety, and clinical depression.[10] Also, development of asthma and chronic bronchitis is not uncommon, as the nose is the guardian of the lungs.[11][12][13]

Etiology

File:Squamous metaplsia of respiratory epithelium.jpg
Squamous metaplasia of nasal respiratory epithelium.

The turbinates are known as the main humidifying, heat exchanging, air-filtering, airflow controlling and airflow sensing structures of the nose. They control heat, humidify and filter the airflow by streamlining it around them as it progresses through the nose, thus significantly increasing the mucosal surface that comes into contact with the airflow. Their integrity and function is crucial for maintaining nasal and sinus health and physiology. Built aerodynamically from anterior to posterior they are designed[citation needed] to not over obstruct breathing while processing the airflow.[14]

The turbinates play a vital role in protecting the inner nasal mucosa and allowing it to recuperate and regenerate. A normal nose of an adult processes on average 10,000 liters of air in 24 hours, it therefore needs some form of resting period to recuperate and maintain good health and integrity of the delicate respiratory epithelium layer that lines the entire nasal airway and sinuses. The recuperation of the nose is achieved by a phenomenon known as the 'nasal cycle'. Every 3–6 hours one side of the nose congests with blood while the other remains decongested - thus the majority of the workload of breathing is done through the decongested side while the congested side rests and recuperates. When the inferior turbinates are resected the nasal cycle can no longer fully congest the side of the nose where they were resected and therefore much of the natural recuperation capability on that side is lost.[15]

There are typically three pairs of turbinates in the human nose - the inferior concha, the middle concha and the superior concha. Each pair is very different in size and in shape, and each protect a different region of the nasal cavity: The inferior turbinates are the largest and extend all across the lower part of the nasal cavity, from the very front of the nose almost up to the nasopharynx. As the inferior turbinates are the largest and the first to mitigate the inspired air, their loss has the most profound effect on the physiology of the remaining mucosa. The middle turbinates hover above the inferior turbinates and begin from approximately the midsection of the nasal cavity. They are much smaller and unlike the inferior turbinates harbour some olfactory nerve axons and protect the upper regions of the cavity, in particularly the olfactory bundle of nerves at the roof of the nose and the openings of the ethmoid and frontal sinuses. Their loss will play a lesser role in the overall reduction of the retained heat and humidity in the nose, but it might impact the quality of the sense of smell and the health of the ethmoid and frontal sinuses. The superior turbinates are tiny in comparison to the inferior and middle ones and have the sole role of being the last line of protection around the olfactory bulb.

The loss of too much turbinate tissue may cause the remaining mucosa at the vicinity of the site of resection, and directly behind and above that area, to gradually become more and more inflamed, dry, go through metaplasia, endertitis and eventually atrophy. As a result, all four major functions of the nose become impaired: breathing, defense, olfaction, and phonology. This can take many years to fully develop, which may complicate the proper diagnosis. ENS is sometimes seen in patients who have lost relatively little of their turbinate tissue and whose turbinates appear to be almost normal in size; this is especially true in cases of anterior inferior turbinate (IT) resection because of its important role in the internal nasal valve.[9] ENS symptoms are often felt by patients soon after turbinectomy procedures.[9]

Diagnosis

The clinician should suspect ENS when the following findings are present: The patient complains of poor nasal breathing and often a relentless sensation of suffocation or shortness of breath despite having a patent (typically over-patent) nasal airway. The patients typically complain of nasal dryness too. These symptoms appeared only after the patient underwent a turbinate reductive procedure. Sometimes, many years later.

On examination the nasal cavity may look abnormally spacious, lacking (part of) one or both turbinates (the inferior and/or middle turbinates). Mucosal pathology varies greatly. In some patients, the mucosa is dry and pale because of metaplasia; in others, it is red because of chronic infection. Crusting may range from absent to severe. The symptoms and findings are believed to be caused by abnormal aerodynamics, chronic inflammation and dryness leading to loss of airflow sensation that feels like dyspnea.[16]

The diagnosis is often complicated because it is common to find that the remaining tissues are hypertrophied (in response to the dryness and constant aggravation of over-turbulent air currents).

Prognosis

There has been very little research conducted on this condition and hardly any long-term follow-ups. For many years this condition was overlooked or mistaken because of secondary problems that usually occur after radical nasal turbinectomies. For instance, the remaining mucosal structures (the septum and the remaining turbinates) often hypertrophy, causing actual physical obstruction on top of the already existing paradoxical obstruction.

The lack of long-term follow-ups of patients with this condition makes it difficult to estimate what percentage of patients, if any, will enjoy a spontaneous recovery or at least a significant enough improvement in their symptoms. But, given that the main cause of the symptoms is the gross loss of normal inner nasal anatomy, it is not likely that this condition can cure itself.

Dr. Eugene Kern, who coined the term "empty nose syndrome", claims that this condition often gets worse over the years through increasing wear and tear of the remaining mucosa in the nasal cavity, because the lack of turbinates leaves the mucosa overexposed to unduly patent currents of unfiltered, and under-conditioned airflow on every inspiring breath. In fact, he maintains that there is an unknown threshold of loss of turbinate tissue from which the nasal mucosa can not recuperate from the daily onslaught of direct airflow. Kern and Moore conducted a large retrospective study of 242 patients which they carefully examined over several years at the Mayo Clinic in Rochester (Minnesota, US), all of whom had undergone some form of partial or radical turbinectomy, following which they had developed symptoms of atrophic rhinitis. They called this condition "empty nose syndrome" to depict how unnatural these noses looked in CT findings and upon physical examination. They emphasized how negatively this condition had affected their quality of life and sense of well being and the fact that in many of the patients the symptoms seemed to worsen over the years, indicating further damage and wear and tear due to the loss of turbinate protection, as there was no other cause that could explain this.[6] Their findings corroborated early conclusions about turbinectomies that were adopted by ENT communities world-wide after these surgeries first started in the late 19th century and are furthermore supported by several prominent other studies from the late 20th century following patients that had undergone radical inferior turbinectomies, but some supporters of turbinectomies remain unconvinced as there have been several long-term follow-up studies that claim to have found no major long-term ill effect. So, the controversy remains, although the pendulum has nowadays shifted back amongst most nasal surgeons towards the importance of keeping as much as turbinate tissue possible when performing turbinate reductive procedures.[17]

The patients can replace some of the lost moisture to reduce the risks of mucosal atrophy by coating their nasal lining with protective gels and using saline mist sprays and irrigations, but it seems that unless the turbinates are functionally reconstructed there is little hope to fully recover from this condition.

In recent years there have been several reports of attempts to reconstruct the inferior turbinates of the nose through submucosal implantation of various implant materials, in an attempt to restore normal nasal aerodynamics and physiology. The sample of patients reported on was very small and the follow-up was relatively short, but the results showed some promise.[9][18][19] A study published in 2010 concluded that surgical reduction of the open cross-section of an 'empty nose' in order to positively influence aerodynamics and to increase airway-resistance does not, however, alter the conditioning function of the nose, which is irreversibly damaged.[8] Another study reported that when ciliated cells have been lost because of degeneration of the nasal mucosa, even surgical closure of the nostrils does not make the number of cilia increase again.[6]

There is hope among patients that with recent advances made in regenerative medicine, otolaryngologists will begin to explore ways to use stem cells and tissue engineering technology to fully reconstruct the inferior turbinates of the nose and restore it back to normal. A clinical registry has been opened in 2014 to support future clinical trials in regenerative medicine specifically intended to treat patients with ENS.[20]

Currently available treatment options

Non-surgical treatment

Non-surgical treatment options are meant to maintain and improve the health of the remaining nasal mucosa in the ENS nose by keeping it moist and free of infection and irritation and by maintaining a good blood supply:

  • Keeping the nasal passages moist with saline-based mist sprays or gels.*
  • Nasal irrigations of regular saline (Many patients prefer to use Ringer's Lactate solution with added xylitol instead, as they find it soothes the mucosa more than regular saline, and there are some empirical studies that back up that claim[citation needed]).
  • Irrigations of saline with 80 mg of gentimycin if ozena occurs.
  • Systemic medication as indicated for pain and or depression which is common (about 50%) in patients with this syndrome.
  • Sleeping with a cool mist humidifier.
  • Regular daily physical exercise and maintaining good general health to reduce the risk of deterioration of symptoms.

Surgical treatment

File:ENS type pre cotton.jpg
Right partially reduced inferior turbinate before cotton test to verify ENS symptoms
File:ENS type with cotton.jpg
Cotton applied to simulate the resistance that an implant will add to the over-reduced inferior turbinate.

Surgical treatment involves narrowing back the over enlarged nasal cavity—either by bulking up the partially resected turbinates with biological implant material (in cases where at least 50% of the inferior turbinate remain from anterior to posterior) or by creating neo-turbinates through submucosal implantation between the submucosa and bone in key locations in the nasal cavity. Of course, in some cases a combined approach is the best choice. The main difficulty with implant surgery is to achieve a long lasting bulk that will not get absorbed over time. Sometimes a procedure has to be repeated several times to get a sustainable result. The most physiological location for an implant is the lateral wall of the nasal cavity, where the inferior turbinate used to project from. An easier location to implant is the septum, but it is less favorable as it is not the natural location of the turbinates and may over obstruct the airflow.

The underlying rationale of surgery is to restore the natural inner nasal geometrical contours of the nasal passages of air (the inferior, middle, and superior meatuses), as much as possible, to mitigate the airflow just enough to restore normal rates of inner nasal humidity and temperature that will allow the mucosa to recuperate and sense the airflow well enough. It is paramount to do so while trying to restore the normal aerodynamics of the airflow in the nose, otherwise nasal obstruction will occur.

Pre-surgical planning has a tremendous impact on the success of the procedure. The surgeon is advised to perform a cotton test prior to the implantation: the surgeon places saline soaked chunks of cotton wool at the pre-planned site of implantation to simulate the implant. By doing so, he restricts and normalizes the nasal airflow patterns. This restores nasal aerodynamics. By trying different locations in accordance to the patient's feedbacks regarding the quality of his breathing and other ENS symptoms, it is possible to pinpoint the exact placement for the implants and their estimated shape and size.

Turbinate tissue is unique and there are no potential donor sites in the body from which to harvest similar tissue. However, in the nose, to some extent form equals 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 resist, streamline, heat, humidify, filter, and sense the airflow.[9]

Implant materials

File:Before and after Alloderm implant to the lateral wall.jpg
Before and after implantation of the lateral wall with Alloderm to simulate the function of the missing inferior turbinate.

The bulking up of the submucosa and mucosa to create a neo-turbinate structure can be achieved through implanting some supporting material between the bone/cartilage and also into 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. A Chinese study reported long-term success using iliac bone autografts.[21]
  • foreign materials: such as fibrin glue, Teflon, Gore-Tex, and plastipore, which solve the problem of shortage of autografts, are easy to shape and do not tend to get absorbed. However they have a high extrusion rate, and sometimes cause infection. A case study of good retention of hydroxyapatite cement in one patient has been reported in 2000, but the follow-up was only 1 year long.[22]
  • allografts: In the last two decades 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 does not stimulate the immune system to reject it. A good example for such material is acellular dermis (brand named "Alloderm"). It does not get rejected and in most areas retains most of its volume over long periods.[9] Dr Steven Houser had been a proponent of such therapy.

The ideal implant material, other than real original turbinate tissue should be something with low extrusion and rejection rates, minimal infection risk, and—very importantly—that will provide a strong and durable enough structure that at the same time allows good permeability for blood vessel incorporation, which seems to be the key against long term absorption.

Additional images

  • Empty-nose-after-80per-cent-partial-bilateral-turbinectomy.jpeg

    Empty Nose Syndrome after subtotal inferior turbinectomy.

  • All turbinates removed - Right lateral wall view.

  • Anatomy of the nasal cavity

References

  1. ↑ Cottle MH. Nasal Atrophy, Atrophic Rhinitis, Ozena: Medical and Surgical Treatment. Journal Of The International College Of Surgeons. Volume 29(4), pages 472-484, 1958.
  2. ↑ Passàli D, Lauriello M, Anselmi M, et al. Treatment of the inferior turbinate: long-term results of 382 patients randomly assigned to therapy. Ann Otol Rhinol Laryngol. 1999;108:569-75.
  3. ↑ Moore GF, Freeman TJ, Yonkers AJ, Ogren FP. Extended follow-up of total inferior turbinate resection for relief of chronic nasal obstruction. by in Laryngoscope, volume 95, September 1985.
  4. ↑ Berenholz L, et al'. Chronic Sinusitis: A sequela of Inferior Turbinectomy. American Journal of Rhinology, July–August 1998, volume 12, number 4.
  5. ↑ Wang Y, Liu T, Qu Y, Dong Z, Yang Z. Empty nose syndrome. Zhonghua Er Bi Yan Hou Ke Za Zhi. 2001 Jun;36(3):203-5.
  6. ↑ 6.0 6.1 6.2 Moore EJ & Kern EB. Atrophic rhinitis: A review of 242 cases. American Journal of Rhinology, 15(6), 2001.
  7. ↑ Chhabra N. and Houser SM. The diagnosis and management of empty nose syndrome. Otolaryngologic Clinics of north America. 2009 (April);42(2):311-330,ix.
  8. ↑ 8.0 8.1 Scheithauer MO. Surgery of the turbinates and “empty nose” syndrome. GMS Current Topics in Otorhinolaryngology – Head and Neck Surgery 2010. Vol 9:Doc03. doi: 10.3205/cto000067
  9. ↑ 9.0 9.1 9.2 9.3 9.4 9.5 Houser SM. Surgical Treatment for Empty Nose Syndrome. Archives of Otolaryngology Head & Neck Surgery\ Vol 133 (No.9) Sep' 2007: 858-863.
  10. ↑ Rice DH, Kern EB, Marple BF, Mabry RL, Friedman WH. The turbinates in nasal and sinus surgery: a consensus statement. Ear Nose Throat J. 2003;82(2):82-84.
  11. ↑ Bionity Life Science Encyclopedia on empty nose syndrome.
  12. ↑ Hens G, Hellings PW. The nose: gatekeeper and trigger of bronchial disease, Rhinology 2006 Sep; 44(3):179-87.
  13. ↑ Hellings PW, Prokopakis EP. Global airway disease beyond allergy, Curr Allergy Asthma Rep. 2010 Mar;10(2):143-9.
  14. ↑ Wolf M, Naftali S, Schroter RC, Elad D. Air-conditioning characteristics of the human nose. The Journal of Laryngology & Otology February 2004, Vol. 118, pp. 87–92.
  15. ↑ Hasegawa M, Kern EB. The human nasal cycle. Mayo Clin Proc. 1977 Jan;52(1):28-34.
  16. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  17. ↑ Hol MKS. & Huizing EH. Treatment of inferior turbinate pathology: a review and critical evaluation of the different techniques. Rhinology, 38, 157-166, 2000.
  18. ↑ Wang Y, Liu T, Qu Y, Dong Z, Yang Z. Empty Nose Syndrome. Zhonghua Er Bi Yan Hou Ke Za Zhi. 2001 Jun;36(3):203-5. Chinese.
  19. ↑ Rice DH. Rebuilding the inferior turbinate with hydroxyapatite cement. Ear Nose Throat J. 2000 Apr;79(4):276-7.
  20. ↑ http://www.usasinus.org/clinical-registry-for-empty-nose-syndrome
  21. ↑ Wang Y, Liu T, Qu Y, et al. [Empty nose syndrome]. Zhonghua Er Bi Yan Hou Ke Za Zhi 2001;36(3):203–5 [Chinese].
  22. ↑ Rice DH. Rebuilding the inferior turbinate with hydroxyapatite cement. Ear Nose Throat J 2000;79(4):276–7.

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