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Frequently Asked Questions

Last Modified: 31 May 2011

Most of the following information is taken from actual questions and responses from the online discussion forum. The answers provided are based on FSRG clinical faculty and students' clinical experience. Some answers are based on information from the scientific literature. There is a comprehensive bibliography on Freeman-Sheldon syndrome (FSS) on this site, but it may not contain all relevant sources referenced by the individual responders, especially with responses that are not specific to FSS. The period of responses is 2005-2010. Responses are modified as new information is obtained. New material is posted continuously, but no new questions have arisen with sufficient responses to permit posting them here over the past several months. There is still much that remains unknown about FSS and related conditions. Please keep that in mind, as you read these responses. Where a branded product is mentioned, it's mentioning does not imply public endorsement or a relationship of either the responder or questioner to the company. No responders had any conflicts of interest to disclose. Thank you. -- Administrator

1. Does my child, who has FSS, need surgery, braces, or physiotherapy?

The goal of surgery in FSS patients is not to 'correct' a deformity; it is to make the deformity more amenable to physiotherapy. If your child can make any progress without surgery, it is best to avoid surgery. Surgery carries special risks for FSS patients, and it cannot correct the underlying biology of the syndrome that is actually causing the deformities in the first place.

Your child will need braces and physiotherapy regardless of whether your child has surgery or not. The more use your child is able to achieve and muscle your child is able to build (through your child's own efforts), the less your child will have to wear braces.

2. I have a child with FSS and heard about Theratogs, but isn't that the same as vet wrap (cohesive bandage)?

No, not quite. First, vet wrap is cheaper, and second, vet wrap's range of uses are more diverse. It is like a rubber mesh version of cotton conforming gauze bandage rolls. It holds corrections achieved in physiotherapy, just by creating resistance--not pressure. It is easy to learn to apply and use.

Also, according to the Theratogs website, 'the therapist/practitioner [must be able to] effect a change in the client's stability, alignment, sensory integration, mobility, or motor control through unforced handling during a therapy session,' in order for Theratogs to be indicated in therapy. Vet wrap can hold stronger, and multiple rolls and adjunct materials may be used to provide additional support. It is reusable, convenient, and pleasant for patients.

But there is considerable difference amongst brands. We prefer PowerFlex(TM) by Andover Healthcare, Inc. It is priced reasonably but provides a, 'strong (23 lbs tensile strength), flexible cohesive bandage ideal for support.' Other brands provide around 8 lbs tensile strength and are not as durable. It resists constriction and is more breathable than other brands we've tried. We use a single layer of conforming 100% cotton bandage next to the skin, which allows for longer uninterrupted wear without chance for skin irritation. Extra support can be achieved with PowerTape(TM) to reinforce, but we have not yet tried this. Like PowerFlex, PowerTape sticks only to itself or PowerFlex; it is said to be 'cohesive' not 'adhesive'.

3. I have been thinking of getting a puppy to train as a helper dog for my child. What can you tell me about this?

A helper dog is a great idea, but having known people who used them full-time, you really should contact a professional organisation. There are a lot of legal responsibilities and government rules. Because of certain things that the dog and person must be able to do and endure, puppies are not used, and there is a minimum age at which a child can be 'matched' or a certain dog selected for a certain patient. You do not pick a dog; the organisation has set criteria that allows them to match the best dog to the person's needs, lifestyle, and personality. Puppies are 'socialised' for one to two years before they are initially trained. The person has to go away to be trained to benefit from the dog, and usually, they have to be re-trained at least 2-3 times. It's great to have a helpful family pet, but a helper dog is a different matter. I have a black poodle named Sir Issac Newton. He helps me to be aware of my environment and to work on fine motor skills in my hands by playing with him, but I don't have a helper dog.

I hope this is helpful. At whatever level, a dog is always man's best friend and is soothing to the spirit!

4. Because my child and patients with FSS have small airways, I think all patients with FSS should be on supplemental oxygen, since this caused my child not to breathe well. What do you think?

Thank you for your concern. As with all aspects of medicine, individual evaluation of each patient, even when they present with similar findings and diagnoses, is critical. Another problem in FSS is pneumonia, and supplemental oxygen increases fluid build-up in the lungs and can lead to complications, including providing a great environment for germs to settle and multiply. Germs (bacteria, fungi, and viruses) almost universally develop best in warm, wet, and oxygenated environments. With the acknowledged poor swallow in FSS, direct aspiration of normal bacteria in the mouth to the lungs can occur, and ensuing illness may be catalysed by an ideal environment. I do strongly agree that patients with FSS should be evaluated and monitored for poor oxygenation, but instead of providing long-term supplemental oxygen, the cause of the poor oxygenation should be found and treated.

5. I have heard about therapeutic horseback riding, but can this benefit my child with FSS?

Yes, it is thought that any form of equestrian sport or leisure is therapeutic for patients with neuro-muscular-skeletal disorders, including FSS. I am not aware of any studies specifically involving patients with FSS, but there have been studies with patients with other disorders.

6. My child has just been diagnosed with FSS. What kind of doctor does my child need to see first?

A consultation with a craniofacial surgeon should have been the first thing that was done when diagnosis was made. Oral surgery is done by a different type of doctor, a maxillofacial surgeon. It is common that patients with FSS have dental problems and surgery is typically required. Hope the info helps and the very best to everyone.

7. My child has FSS, and now the doctor thinks my child may have craniosynostosis. What are the best ways to diagnose and treat craniosynostosis?

Craniosynostosis may be linked to FSS. An MRI is the better type of imaging. It is not harmful like lots of radiation from a CT can be and gives a better picture. But a good physical examination is really all you need. To treat craniosynostosis, a helmet, not surgery, is a good choice, as a first-line option.

8. How is FSS treated?

Physiotherapy and surgery really are the only ways to deal with the effects of the syndrome.

9. Someone evaluated my child, who has FSS, and made a diagnosis of developmentally delayed. Does this mean my child is mentally retarded?

No, FSS deformities cause you child to not be able to do certain things, hence your child was said to be delayed in accomplishing certain tasks. Developmental delay does not equal mental delay.

10. My child has FSS, and someone said my child was not growing. They said something needed to be done about this. What can you add?

There is no clinical basis for treating a 'small' person--no matter the diagnosis--unless that person is rendered actually unhealthy by sole virtue of his being small. One must consider only whether the child is healthy, not whether he is 'big enough'. Size really isn't a concern, per se. If your child is healthy, there is no concern to do anything. Your child may not be very tall, but unless your child is actually not well, there is no need to be concerned here. The important things are for your child to have a body that works as well as it can, to be happy, and have a good brain.

Measuring fat by simply pinching up skin, as on the arms and legs, is very helpful. I think you have to be careful about 'post hoc urgo propter hoc'--'because of that, this'--and so if your son appears healthy and basically is, then it is likely that he is healthy. All folks with FSS have feeding problems--it is part of the diagnostic criteria--but FSS is not..

11. I have heard they can re-grow teeth; can this help my child, who has FSS?

I've been in contact with this guy who's doing the work over the period of several years. I sent him some radiographs and discussed them with him, but the technology really isn't quite as good as it appears.

12. The doctors want to give my child, who has FSS, human growth hormone (HGH). What do you think?

I think HGH is generally a bad idea; we don't know enough about it or FSS. What I know is that this isn't helpful in the end. I know of people who were treated with HGH and are no different in size than those, who were not. Being short is not a 'disease', being unhealthy is. Failure to thrive is part of the syndrome, and patients with FSS grow out of that 'stage'. If your child's feeding issues concern you greatly, a g-tube would help, but I do not advise that either, per se.

13. What kind of academic situations will my child, who has FSS, require?

You may have trouble with the school officials, but I don't expect your daughter, from what you say and what is known generally about FSS, will have any trouble academically. The school officials usually have trouble understanding what to do, when all they need to do is teach. Your child may have difficulty with other students as well.

If the school system poses much of a barrier to your child's education, you should contact a parent advocacy group; they often are able to help it situations like this, without need for a lawyer. If you end up needing a lawyer, they can oft times get one for free for you. But the bottom line is that you must have medical documentation of your child's situation. The physician doesn't need to write another note; you can request part of the chart that documents this and use it.

14. Do children with FSS cry more often than children who do not have FSS?

No, there is no evidence to support this.

15. My child had surgery for FSS-related deformities, and although it seems to have worked, there have been many post-operative complications. Is there something you can add?

You are consulting specialists that aren't devoted to FSS entirely, and they, therefore, can't appreciate fully it's biological burdens. That is why we have formed a special team. All we do is FSS. I really am happy the surgery seems to have worked. I am also not surprised by the post-op complications; those arise in cases where you don't have a team devoted to FSS more often than when you do have such a team. There is just a lot of difference in being a good specialist and a good FSS specialist. FSS it is such a complicated condition that you could even make the argument that it is as clinically complicated as HIV/AIDS, which, of course, constitutes its own field entirely, now.

16. My child has FSS and is not crawling; my child is scooting. I am concerned. What can you tell me?

Your child does not 'need' to crawl. It may be unnatural, in consideration of your child's particular deformities.

17. Is FSS difficult to diagnose?

FSS presents a lot of trouble to diagnosis. For one, it is really a spectrum, not just 'one' syndrome. I do, however, feel the rate is much higher than one might expect.

18. Isn't FSS similar to several other conditions? Patients with FSS have similar deformities as patients with other syndromes?

Really, FSS has unique histological basis; although the physical features do overlap, the aetiology of them in FSS is quite different. So, something that worked in multiple pterygium syndrome, may not work in FSS and so forth, even though they are for the same deformity.

19. The doctor advised surgery for my child's FSS-related foot deformities. What do you think?

Because of the abnormal muscles in FSS, the bone correction cannot be maintained by the muscles. From review of available medical literature, I've found only one case of successful clubbed foot correction by surgical intervention. Learning to walk with clubbed feet shouldn't be much of an issue. Physiotherapy and braces are the best options in ameliorating the foot deformities. Your child should have no real trouble being active and playing sports with braces. Now, you can do this type of surgery on the hands, kind of. The hands' muscles are a bit different, and the hands are not weight-bearing. My final advice is to cancel the foot surgery.

20. My child has FSS, but now my child has developed a condition that really has nothing to do with bones, muscles, nerves, or metabolism. The doctor still says it's part of the syndrome. What should I think?

Certainly, don't either get in the habit yourself or let the doctors get the habit of laying blame to FSS for everything. If the other condition has nothing to do with the bones, muscles, nerves, or metabolism, it is unlikely to be related to FSS.

21. My child has FSS-related foot deformities. Can the Ponseti method be used for FSS?

Dr. Ponseti has experience with distal arthrogryposis spectrum syndromes and I FSS. FSS patients are likely to require 'walking braces' for the long term, but surgery doesn't work. The problem is not with the bones, which is what the surgery tries to correct, it is, of course, with the muscles and nerves. The muscles cannot hold the correction achieved with the bone. My group advises against surgery for clubbed foot deformity in FSS—always.

22. Does complementary and alternative medicine (CAM) benefit patients with FSS?

Most aspects of CAM are not accepted as routine therapy under any circumstance for any condition; however, I'm not discounting aspects of CAM could or do work. All I mean is that there is no solid body of strong reliable and valid scientific studies on CAM, including chiropractic therapy. For FSS, there have not been studies on 'traditional' medicine and surgery, either. has not been evaluated. That is what the FSRG wants to do--figure out what has been helpful, regardless of what the modality is.

23. Why do patients with FSS get pneumonia (pneumonitis) so often? Is there a difference is how they get it?

About the aetiology of the pneumonitis, it isn't so much the issue of foreign bodies, but aspiration of oral flora that colonise in the warm, moist environment of the lungs. This makes sense in consideration of the pathognomonic dysphagia in FSS, which seperates it from other DAs.

24. How uncommon is FSS really? How many patients suffer from this syndrome?

I think this illustrates a very good point. The criteria for diagnosis is difficult, and we cannot estimate prevalence until we know what we're estimating. When people ask me, which is pretty frequent, I usually quote them the number of papers listed on Medline at the time. Right now there are 109 papers. Given that Medline covers the whole world and that some journals aren't listed, some cases not published, some published cases aren't really FSS, and that some papers included multiple cases, I'd say 220 is pretty close. Some articles report only 40 cases worldwide; some report even less!

Some people in our group, contend that Stevenson et al. (2006) didn't consider some very common features of the syndrome in their criteria. This criteria was then the basis of the Toydemir et al. (2006) gene report. I think on a primary basis, especially in the poorer countries, you're going to have to give a little and not be so strict. Remember, infant mortality must figure in, too, and that is going to push your prevalence rate up. One must also consider aborted matter. So, maybe 200,000 'pregnancies' might be close. You have to also think about what you want to consider pregnancies, live births, survived to x age, or living.

I don't know if I answered the question, but I hope I gave some things to consider, at least.

25. What type of health professional needs to attend delivery of a possable FSS case?

Some definitions are in order. A midwife is a paraprofessional and is appropriate ONLY for uncomplicated pregnancies and routine vaginal deliveries without any chance of complication. If there is any chance of complicated pregnancy or delivery, a midwife is an innappropriate choice. An OB that handles high-risk cases is the best choice. He should be affiliated with a hospital that has a neonatal intensive care unit (NICU) and a wide-range of paediatric specialists. Delivery of a complicated case requires a team-approach.

26. Is prenatal testing is available for FSS?

Yes, there are parameters for ultrasound (US) diagnosis of FSS. If there is any doubt, amniocentesis should be done, not just to 'make a decision' but to plan, if it is positive for FSS or some other syndrome or disorder. I'll try to find and post the information on US diagnosis.

27. How can you determine chance of future conceptions resulting in FSS?

There are two identified modes of transmission of FSS: autosomal dominant and recessive. Autosomal refers to the 22 pairs of non-sex chromosomes, which means that males and females are affected equally. Recessive means that two copies of the genetic defect (allele--not gene; an allele is a version of a gene, like Coca Cola is a type of soft drink) must be inherited before the offspring can inherit the phenotype-- or the malformation (There are some weird exceptions to this rule, but as far as we know, it holds for FSS.); whereas, dominant means that only one copy of the genetic defect must be inherited for the malformations to be present. If it is dominant, then a parent MUST also have the syndrome; if it is recessive, it might have occurred several generations back and the parent need not express it to pass it on. Of course, most cases are not inherited; they are new mutations, or sporadic. The only way to determine route of transmission is by process of elimination using pedigree analysis and genetic testing. Degree of symptoms is not a validated predictor of route of transmission. If it is proven dominant, then the risk is 50%. In recessive cases, it is less and dependent upon the individual case.

28. How is the best way to deliver an FSS neonate?

There are cases of normal vaginal delivery of FSS cases, but it is much safer to consider c-section, in light of the severe contractures of the neonate that could complicate delivery and create a dangerous situation for mother and baby.

29. What role can physiotherapy (PT) have in helping patients with FSS?

Muscle is affected congenitally in FSS. PT, however, does help to maximise range of motion (ROM) and strength and minimise morbidity. In FSS, fibrose connective tissue is interspersed with normal muscle tissue. This fibrose connective tissue restricts the normal tissue, making it stiff and not allowing it to be dynamic--i.e. not allowing it to move, grow, stretch, or resist gravity. This process, however, does not render FSS a progressive syndrome. Obviously, if you are inactive, your functioning level isn't going to be as good as someone who is a swimmer, equestrian, or cyclist. This is one reason why it is so much more important to do some type of PT in FSS; good function can be achieved, not like in a progressive disorder.

To address the pathology of the fibrose connective tissue, there is a drug going through clinical trials in the US for therapy in a progressive myopathy. I am in contact with someone close to the work, and there is hope it could be tried in FSS later.

30. My child has FSS and has difficulty swallowing. I am also wondering if my child's GI problems can be related to FSS, too?

Yes, FSS is a global myopathy, and the GI system is comprised of smooth mucle. A colonscopy and illoscopy are indicated in FSS patients, as well as pressure measurement.

31. If FSS is a disorder in which muscles contract and cannot relax, isn't it a good idea to use Botox to paralyse the muscle contractures?

No, Botox is infiltrated at sites of nerve endings and diffuses very well, too well for some one with little bulk. If you were to inject it in an area where there was little bulk, it could diffues and cause major problems with motor nerves, proprioception nerves (they tell you that your arm is in the air without you looking to see that is), and sensory nerves.

There is also a second reason. We have emerging results to show that FSS may be more than a myopathy. We have found major nerve damage, secondary to myopathologic features (presumably). Until we know more and validate these results with many more patients, it is prudent to avoid radical measures, including Botox.

32. My child has FSS; what do I need to understand about anaesthesia? What causes or triggers an MH event?

In terms of MH triggers, only certain inhalational agents have been implicated, namely ether and halothane. Depolarising muscle relaxants are also triggers. Surgeons like muscle relaxants because it gives a better surgical field. Anaesthesia produces a certain amount of contraction and yet more is part of the FSS package. Muscles with FSS-induced contracture do not respond to muscle relaxants, by and large, but non-depolarising agents may be used (because they are readily reversable) for anaesthesia-induced contracture. Local (everything I've talked about so far has pertained to general anaesthesia, which involves total loss of consciousness, total loss of sensation, and amnesia of different degrees) anaesthesia (which includes: spinal, epidural, caudal, regional nerve block, regional intravenous, and local infaltration) is safe and will not produce MH. An alternative to general anaesthesia is deep sedation with some type of regional anaesthesia. For example, Versed is administered before the spinal needle is placed, but this is done under light sedation (because patient cooperation is necessary for positioning, etc.). The level of sedation may be augmented later to keep the patient comfortable. Sedation only puts you to sleep, while the regional anaesthesia eliminates sensation. Some sedatives may also produce amnesia, which surgeons also like. We don't want for you to remember what we did, just how well we did it, which you can know about when we're done doing it! This is especially true of little children. Another point, a syndrome closely linked to MH is NMS or neuroleptic malignant syndrome. NMS and MH must both be considered in an FSS patient.

33. I have a baby who has FSS and cannot breathe through the nose. Can the nose be corrected in a baby?

Yes, reconstructive rhinoplasty is something that can be achieved in a little child. The first thing is to get good 3-D CT imaging to pinpoint particular issues, of which there may be more than what is just obvious from the outside looking in or there may be different ones than suspected. Anyhow, it is very important to know the anatomy before you go in to try and do something. Second, it may be well to consider mandibular and maxillae correction at this point. Such correction has the 'potential' to help with speech. Third, is to get a consultation with a craniofacial surgeon who is able to deal with the oddities of this unique syndrome. You can contact the American Cleft Palate and Craniofacial Foundation, if you need assistance. You need to treat the underlying problem, not just the symptom.

34. Please explain preventative medicine, especially pre-emptive psychotherapy.

The idea in preventative medicine is to target at risk groups. With respect to who ought to get pre-emptive psychotherapy, the groups are soldiers, trauma medics, police, firemen, families of violent offenders, and of course, persons (and their families) with disabilities, whether chronic or severe acute. We don't do psychotherapy for everyone, and not all the people in the above groups would develop clinically significant psychopathology even if they never got psychotherapy, but on the whole those people in the groups develop psychopathology at a much higher rate than the general population.

35. What are the best types of toys for a baby with FSS?

Textures are very good, especially for children with FSS. Any toy that feels different or has raised bits is good; stuffed animals are a good choice, sometimes. Any shapes, blocks, etc. are good.

36. My child has FSS, and I am concerned about difficulties feeding my child. What needs to be done?

I have read a good deal of conflicting reports on feeding difficulties in FSS. Monitor your child's intake, output, weight, and growth changes--just as one would for any under-weight or small infant. I don't think one may take too much from what other patients recount, but it all needs to be regarded with special consideration of your child's actual clinical picture. Every person is unique. Your child's feeding problems may resolve spontaneously, as happens in some FSS cases, or it may remain static for sometime. Most patients have normal intellect and do very well. Usually the smallest nipple for a premature infant is best to use for FSS patients who have difficulty opening the mouth. Frequent feeds are required. If this concerns you greatly or your child is in clinical need of receiving more nutrition than your child can take orally, then a consideration can be made regarding a gastronomy tube to provide nutrition directly into the gut or stomach via a surgically created opening through which a tube is secured. Nutrition is provided through the tube via a syringe. The tube may be capped when not in use for feeds.

37. My child has FSS and the doctor noticed scoliosis also. Is the scoliosis part of FSS, and what should be done?

The scoliosis you mention is a common feature of this syndrome, and it should be watched. Plain x-ray films should do to quantify the degree of curvature. Follow-up examination and x-ray films, when indicated, are advisable at least yearly. If it is severe and causing symptoms, bracing and surgery need to be considered. Your child should also be evaluated for deformity of the sternum.

38. Generally, when and how should surgery be considered in FSS?

Surgery is only to be considered after conservative measures have not achieved satisfactory results. Further, the hands and feet are much different in terms of dynamics. Surgery is unlikely to help the feet, but it may help the hands, wrists, and arms. Passive manipulation, exercises with toys, and splinting are all very good for the hands.

39. Why do most doctors and health professionals not understand FSS?

I have only met three doctors that had ever seen a case before I met them. To understand something, you must have some experience with it. The point is that the physicians and surgeons must understand that this is a myopathy, not an osteopathy that can be fixed by repositioning bones. The muscles cannot hold the operative correction. Frequently doctors have seen deformities that are also present in FSS, and they try to treat them as they would in a non-FSS patient. That isn't quite the way to go about it. Also, there is very little research that has been done, and there are no books on FSS.

40. I have heard there is a test for FSS mutation. I want it. What do you think about mutation (DNA) testing for FSS?

The DNA testing is not that important. I think patients--not just those with FSS or SHS--get hung up on testing. That is one reason why our healthcare is in such a mess; patients want the test, whether they need it or not. However, if you really want to be tested, be aware that it is expensive. There are only two places in the world doing the testing. The best place is in Germany. If your goal is to confirm a diagnosis of SHS or FSS, then that can be done very well clinically, by taking a good history and doing a good examination of you and looking at your particular features.

41. My child was just diagnosed with FSS. What caused this or what is the hypothesis (educated guess) about what causes this?

Certain mutations in embryonic myosin heavy chain-3 (MYH3) are associated with FSS 98% of the time. This genetic defect associated with FSS occurs in embryonic development in the myosin head, where ATP molecules bind. ATP binding is require to allow the myosin to bind to actin and release from it, which is what allows the muscle to contract and relax. With fewer spots available to allow the ATP to bind to the myosin, the muscle can get 'stuck'. This may be why the hands look as they do and why he 'appears' to have muscle weakness.

42. My child, who has FSS, was diagonsed as having muscle 'weakness' and 'wasting'. What can you tell me?

In fact, it is not 'weakness', and it is improper to speak of it as 'weakness'. By definition, FSS does not involve muscle 'weakness' or 'wasting'. True muscle 'weakness' or 'wasting' excludes FSS as a diagnosis. Either your child has muscle 'weakness' or 'wasting' and NOT FSS or your child has FSS and NOT muscle 'weakness' or 'wasting'. 'Both' is not an option; it is an 'either or' case.

43. My child, who has FSS, had a muscle biopsy, but they didn't find FSS. Can you explain?

It is quite possible that the person reading the pathology slides did not know how to read them. These features are not actually pathological, except in the setting of clinical FSS. For example, the only finding may be 'variable fibre size' but with normal muscle fibres. Although not a pathological feature, it is FSS-associated feature.

44. My child has FSS and had trouble breathing. My child had a tracheostomy and is doing so well! Don't you think it would be good for all children with FSS to have tracheostomies?

Although a valuable procedure in carefully selected patients, tracheotomy is a surgical procedure, which includes the placement of a foreign body in a newly created stomia (opening). As explained above, this increases risks for infection and other complications that are already higher than normal in FSS. The risk of the surgery itself should not be minimised, either. Complications occur in 14-66% of cases. Death results in 5% of cases, overall; pre-term infants have a mortality rate of 11%.

Bleeding in the trachea occurs in 5% of cases. Pneumothorax (air between the lung and chest wall) and pneumomediastinum (air in the space between the lungs, in the middle of the chest call the mediastinum) occurs in 5% of cases overall and 17% in children. In children 2-6 years old, the rate of intra-thoracic (chest) complications are as high as 70%. Other complications include lower respiratory infection, subcutaneous emphysema (air gets trapped under the skin), tube blockage, or decannulation (tube coming out). Infection has an overall rate of 36%, with cellulitis (skin infection) and purulence (discharge) having rates of 3-8%, respectively. One author reported a rate as high as 75% of Pseudomonas colonisation ten days post-tracheotomy placement. Tracheoinnominate artery fistula occurs in 0.4-4.5% of cases.

Long-term complications (defined as greater than 16 weeks) include: tracheal stenosis (narrowing), often caused by direct pressure necrosis (tissue death) from the intra-tracheal tube); tracheoesophageal fistula, which has a rate of 0.01%; failure of skin to close, which occurs in 3.3-29% of cases; erosion (thinning) of the trachea (windpipe) caused by the tube (tracheomalacia); and bumps (granulation tissue) from irritation causing inflammation. Infection and aspiration are constant risks.

The above complications are considerable. By comparison, surgery in general has a complication rate of below 1% and mortality rate below 0.1%.

I hope this has been helpful in giving a better explanation.

Sicard MW. Complications of Tracheotomy. Presented: 1 December 1994, ENT Grand Rounds, Baylor College of Medicine. URL: http://www.bcm.edu/oto/grand/12194.html Last accessed: 17 Jun. 2010.

Tracheostomy Service at Johns Hopkins. Patient Information: Complications and Risks of Tracheostomy. URL: http://www.hopkinsmedicine.org/tracheostomy/about/complications.html Last accessed: 17 Jun. 2010.

National Heart, Lung, and Blood Institute. 'Tracheostomy: What Are the Risks of a Tracheostomy?' in 'Lung Diseases' in Diseases and Conditions Index.
URL: http://www.nhlbi.nih.gov/health/dci/Diseases/trach/trach_risk.html Last accessed: 17 Jun. 2010.