Diaphragmatic endo is an entity little known to most physicians. Many GYNs don't even know it exists, and most pulmonologists (lung specialists) aren't very familiar with endo. It is an incredibly rare manifestation of endo, with only 30 cases reported in the literature, most occuring on the right side. I actually think it's a little more common that this, as I've seen 2 cases since I've been here in Bend (9 months). Patients typically have pain in the right chest and shoulder around the time of their menses, and sometimes can cause pain with taking a deep breath. Rarely, it can cause what's know as a catamenial pneumothorax (a dropped lung around the menstrual flow). This can cause an increase in pain as well as difficulty breathing and air hunger, feeling like you can't breathe deeply enough. Because it's rare to have this dropped lung condition, as usual, endo of the diaphragm won't typically show up on any imaging studies, so once again, we're back to requiring a high index of suspicion to diagnose this entity. Laparoscopy with the scope through the belly button will allow visualization of the anterior part of the diaphragm, which can give you an indication of whether or not there's endo present, but by far most lesions hide out towards the back of the diaphragm behind the liver, which can't be seen with the usual scope position. We use a smaller 5mm scope through a port in the right upper quadrant (just below the ribs) in order to see the entire diaphragmatic surface in patients that complain of these symptoms. When diaphragmatic endo is present, because it's usually full thickness through the entire diaphragm, we have to remove it through an open incision beneath the ribs (like an open gallbladder incision.) This is because the liver can't really be retracted laparoscopically without risking tearing it, which will cause lots of bleeding (typically considered poor form in surgery...). That's also the reason that just lasering the lesions usually doesn't work (because they extend from the abdominal surface to the pleural (lung) surface).
The amazing thing about diaphragmatic endo is that just like bowel endo, the pain magically disappears even before the incisions are healed. It's truly amazing to see.
Well, that's about all there is to say about diaphragmatic endo. Short and sweet, but oh so necessary for those who have it.
Have a great week and Memorial day weekend, and say a prayer for the families of all the troops who have given their lives to defend our freedom. Take care,
Dr. Mos
Thursday, May 24, 2007
Tuesday, May 15, 2007
Eileen left a comment last week asking me to write about adhesions.
This is an interesting subject, and there are some vigorous debates within the academic community about whether or not adhesions cause pain. Throughout the years it has become almost a running joke that the general surgeons don't think adhesions cause pain and gynecologists think they do cause pain. In reality, I think there are some adhesions that are dense, attached to something sensitive like an ovary, or definitely pull on structures like the bowel or abdominal wall that can definitely cause pain, and I've operated on these ladies and their pain resolves after cutting and/or removing the adhesion. There are some adhesions that are more filmy with less density to them that often run from small bowel to other structures, and these adhesions rarely cause pain.
Within the context of endo, I have read many, many operative reports from other GYNs who dictate "findings of adhesions in the culdesac". Most of the time this represents stage IV endo, and the adhesions aren't from prior surgery, they're from endo. The simplistic explanation is that endometriosis lesions are like glue, and they will cause other surfaces to stick together, ie bowel to uterus, bowel to ovary, rectum to cervix, and on and on. What really happens is that the lesion secretes metabolically active substances that cause pain and inflammation. The body's response to the inflammation is that of scarring and formation of fibrosis. Just as a scar on the skin contracts with time as it matures, so do scars and fibrosis in the pelvis, causing tension on adjacent structures. Sometimes the tension adds to the basic endo pain and causes (for example) worse than normal ovulation pain because as the ovary enlarges with the cyst that contains the egg, it can pull on whatever it's attached to (usually bowel or pelvic sidewall) and cause pain.
We don't know what the #1 gynecologic cause of adhesions is, because the only way to reliably find them is to operate, and who do we operate on? Women with pain. In one study, 75% of women with endo had adhesions, and 80% of women without endo had adhesions (essentially no difference). They can be caused from prior surgery (especially when there is post operative bleeding), infections like PID, appendicitis (especially if it ruptures), diverticulitis, cancer, or anything that causes inflammation in the abdomen. Some will say that excising endometriosis causes more adhesions than lasering endo, but I'm not aware of a good quality study comparing that. What I am aware of is the dozen or so women that we have operated on since I've been in Bend who have had surgery by Dr. Redwine in the past. These women on average have a few filmy adhesions mostly in the cul-de-sac (and most of these women have had bowel resections), and not too much else. It is remarkable how often we can see where the peritoneum and endometriosis was removed at the previous surgery and healed in perfectly with no adhesions, no endometriosis, nothin'! The patients who had bowel resections usually have some filmy adhesions around their anastamosis, but compared to patients with active endo that hasn't been excised, the number and quality of adhesions is usually less for both, especially the quality (meaning the adhesions are filmy instead of dense and fibrotic).
While there are still many unanswered questions about adhesions, it is the opinion of both myself and Dr. Redwine that simple filmy adhesions don't usually cause a lot of pain, that endo hides in dense adhesions. If doctors don't know what to look for, or don't have a lot of experience with stage IV endo, they may not realize that the pain in those patients is coming not from the adhesions but from the endo at the cause of the adhesions. For stage IV endo (obliterated cul-de-sac) and bowel disease, the only way to relieve pain and cure the endo is to excise it, remove it and the associated fibrosis. Many studies have looked at Lupron and other medical therapies for this most severe type of endo, and the meds failed miserably. Unfortunately, these are the patients who are most challenging surgically and require a surgeon who is familiar with the patterns of endometriosis, and has a lot of experience with this variety of the disease. These endo specialists are few and far between, but if you are a patient with this particular flavor of endo, I guarantee you that it will be worth your while to seek out one of the handful of us who do this kind of surgery every day.
To those of you who are in pain, I wish you the very best and hope you find relief. If I can do anything for you, please don't hesitate to let me know. Either leave a comment on the blog, or email me at info@endometriosissurgeon.com, and I'll do my best.
Take care, and have a great week enjoying the beautiful spring weather.
Dr. Mos
This is an interesting subject, and there are some vigorous debates within the academic community about whether or not adhesions cause pain. Throughout the years it has become almost a running joke that the general surgeons don't think adhesions cause pain and gynecologists think they do cause pain. In reality, I think there are some adhesions that are dense, attached to something sensitive like an ovary, or definitely pull on structures like the bowel or abdominal wall that can definitely cause pain, and I've operated on these ladies and their pain resolves after cutting and/or removing the adhesion. There are some adhesions that are more filmy with less density to them that often run from small bowel to other structures, and these adhesions rarely cause pain.
Within the context of endo, I have read many, many operative reports from other GYNs who dictate "findings of adhesions in the culdesac". Most of the time this represents stage IV endo, and the adhesions aren't from prior surgery, they're from endo. The simplistic explanation is that endometriosis lesions are like glue, and they will cause other surfaces to stick together, ie bowel to uterus, bowel to ovary, rectum to cervix, and on and on. What really happens is that the lesion secretes metabolically active substances that cause pain and inflammation. The body's response to the inflammation is that of scarring and formation of fibrosis. Just as a scar on the skin contracts with time as it matures, so do scars and fibrosis in the pelvis, causing tension on adjacent structures. Sometimes the tension adds to the basic endo pain and causes (for example) worse than normal ovulation pain because as the ovary enlarges with the cyst that contains the egg, it can pull on whatever it's attached to (usually bowel or pelvic sidewall) and cause pain.
We don't know what the #1 gynecologic cause of adhesions is, because the only way to reliably find them is to operate, and who do we operate on? Women with pain. In one study, 75% of women with endo had adhesions, and 80% of women without endo had adhesions (essentially no difference). They can be caused from prior surgery (especially when there is post operative bleeding), infections like PID, appendicitis (especially if it ruptures), diverticulitis, cancer, or anything that causes inflammation in the abdomen. Some will say that excising endometriosis causes more adhesions than lasering endo, but I'm not aware of a good quality study comparing that. What I am aware of is the dozen or so women that we have operated on since I've been in Bend who have had surgery by Dr. Redwine in the past. These women on average have a few filmy adhesions mostly in the cul-de-sac (and most of these women have had bowel resections), and not too much else. It is remarkable how often we can see where the peritoneum and endometriosis was removed at the previous surgery and healed in perfectly with no adhesions, no endometriosis, nothin'! The patients who had bowel resections usually have some filmy adhesions around their anastamosis, but compared to patients with active endo that hasn't been excised, the number and quality of adhesions is usually less for both, especially the quality (meaning the adhesions are filmy instead of dense and fibrotic).
While there are still many unanswered questions about adhesions, it is the opinion of both myself and Dr. Redwine that simple filmy adhesions don't usually cause a lot of pain, that endo hides in dense adhesions. If doctors don't know what to look for, or don't have a lot of experience with stage IV endo, they may not realize that the pain in those patients is coming not from the adhesions but from the endo at the cause of the adhesions. For stage IV endo (obliterated cul-de-sac) and bowel disease, the only way to relieve pain and cure the endo is to excise it, remove it and the associated fibrosis. Many studies have looked at Lupron and other medical therapies for this most severe type of endo, and the meds failed miserably. Unfortunately, these are the patients who are most challenging surgically and require a surgeon who is familiar with the patterns of endometriosis, and has a lot of experience with this variety of the disease. These endo specialists are few and far between, but if you are a patient with this particular flavor of endo, I guarantee you that it will be worth your while to seek out one of the handful of us who do this kind of surgery every day.
To those of you who are in pain, I wish you the very best and hope you find relief. If I can do anything for you, please don't hesitate to let me know. Either leave a comment on the blog, or email me at info@endometriosissurgeon.com, and I'll do my best.
Take care, and have a great week enjoying the beautiful spring weather.
Dr. Mos
Tuesday, May 08, 2007
Interstitial Cystitis
Aloha!
Sorry I didn't write last week but we were in the OR until 10 pm one night and 8pm the other. Nobody ever said this kind of surgery was easy, but it sure is challenging.
I thought I'd write about interstitial cystitis today. IC as we call it, is the second leading cause of pelvic pain in women, behind (you guessed it) endo. Some studies show as high as 80% of women with chronic pelvic pain (CPP) had evidence of bladder involvement as a cause of pain, sometimes by itself and sometimes accompanying other pain generators such as endo or levator muscle spasm. IC causes pain in the central pelvis that can radiate out towards the groin or back to the sacrum. Some people feel it deeper, like at the top of the vagina. Typically the pain is worse with a full bladder, so these patients tend to void often, and they usually have some degree of urgency and frequency because the bladder wall doesn't like being stretched out. As you can imagine, intercourse can be miserable, although sometimes changing positions so the bladder is out of the way can make it tolerable.
One of the biggest problems with IC is that it can take years for patients to get a diagnosis. IC falls in the gap between gynecology and urology, with both specialties trying to pawn the patients off on the other. This is partially because of a lack of training about IC that hopefully is being rectified, and partially because many docs don't like taking care of chronic pain patients. It is true that patients with chronic pain can be difficult to care for, some requiring a lot of time and hand-holding and some with narcotic abuse issues, but I'll tell you what, there is nothing as satisfying as helping someone who's been in pain for a long time. Most patients don't want drugs, they want pain relief, and when you help them accomplish that, they are so grateful that for me anyway, that's all I need to keep me going. The other thing about pelvic pain is that it usually doesn't show up on ultrasounds, CT scans, or lab tests. Doctors that are good at diagnosing pelvic pain have to rely on listening to the patient and doing a focused, methodical exam, then use their powers of deductive reasoning and gut intuition to come up with an idea of what's causing the pain, and what to do about it. Doctors aren't used to making diagnoses this way these days, without confirmatory imaging studies, so it takes a special person with an interest in pelvic pain and compassion and empathy for the patients to be good at it.
Nobody is quite sure yet just what causes IC, but the leading idea is that of "neurogenic inflammation". What happens is that a painful stimulus that continues over time, (whether from endo or levator spasm or essentially anything in the pelvis) eventually can cause reverse transmission of nerve impulses from the spinal cord on down to the pelvis. When these stimuli reach the end of the nerve (for IC the bladder nerves) certain substances (like histamine, cytokines, and prostaglandins) are released from mast cells that cause swelling and nerve irritation. These inflammatory mediators that the mast cells release then cause more nerve irritation via other mediators that upregulate the pain fibers (nerve growth factor and substance P), eventually causing a vicious circle of pain. Another theory is that there are breaks in the mucousy lining of the bladder that protects the cells from all the irritants that the kidneys are excreting. These two theories are not mutually exclusive and likely the neurogenic inflammation causes the breaks in the lining (the technical term is the "gag layer" because it is composed of glycosamino glycan, or gag, which is a big huge sugar molecule).
It is logical based on the above theory of neurogenic inflammation why there is a significant overlap between those with endo and those with IC, but this doesn't explain why women without endo (or men, for that matter) get IC. Going back to the painful stimuli part of the theory, it is possible that any pain generator in the pelvis can induce the backwards nerve transmission and thereby neurogenic inflammation. It is a well-known fact that we are in an epidemic of abuse in this country. Whether by parents, partners, or strangers, nearly one third of all US women admit to having been abused at some point in their life. Women with IC have a higher percentage of abuse than those without - nearly half of IC patients admit to being the victim of abuse. The reason this is significant is that any abuse, but especially sexual abuse can cause a prolonged spasm of the levators, the muscles of the pelvic floor (the muscles used in Kegel exercises). Prolonged muscle spasm eventually causes increased firing of the pain sensing nerves in the muscles, which starts the vicious cycle. There is a lot of cross-innervation in the pelvis, which means that nerves to the bladder, pelvic floor, upper vagina, rectum, and any other pelvic site come from the same nerve root as it exits the spinal canal. They also interconnect like an LA freeway, joining and separating often on their way to the end-organ. This allows for 2 things to happen - the first is that a painful stimulus can start anywhere in the pelvis and cause the neurogenic inflammation in another pelvic location (this is also the proposed etiology for vulvar vestibulitis, but we won't get into that today). The second is that "neural cross-talk", or this sharing of nerve fibers between various organs in the pelvis, is probably why there are patients with IC, irritable bowel syndrome, and fibromyalgia all at the same time.
IC can be treated in a variety of ways, but none are "curative". Hydrodistention (blowing up the bladder with water under anesthesia) is both diagnostic and therapeutic. When either ulcers (only seen in 10-15% of pts) or glomerulations (small areas of bleeding from surface blood vessels) are seen after hydrodistention, then IC is present. Hydrodistention also is known to give some relief for a few months afterwards. Bladder instillations are my favorite treatment because they work quickly and pretty effectively. For people with significant pain, instilling lidocaine (a local anesthetic) and heparin (rebuilds the mucous lining) can give them immediate pain relief that can last for several days. Initially the instillations are 3 times a week, but with time the interval can be stretched out to a week or 2. Elmiron is a drug designed specifically for IC. It also rebuilds the lining like heparin does, but it takes 3-6 months to see a benefit, which is why I like to start with both oral Elmiron and bladder instillations. Tricyclic antidepressants work well to depress the pain fiber activation, essentially putting a damper on the hypersensitive pain nerves. Since chronic pain does cause depression, having a side effects of a little mood elevation and improved sleep doesn't hurt, either. Some people advocate antihistamines, specifically atarax, because it stabilizes the mast cells so they don't release their nasty inflammatory mediators, but I find that most people can't tolerate the sleepiness it causes.
I think there's a lot we don't yet know about pain, why some patients have minimal disease and a lot of pain, why some patients have a ton of disease and a little pain, and why some people develop new pain after their old pain was fixed. The good news is that a lot of research is being done to identify places in the inflammation cascade as well as the nervous system where interventions can be made. Right now we have some drugs that definitely can help women with IC and those with chronic pelvic pain (non-endo related), but I think that within the next 5-10 years some significant advances will be made. (for those of you that missed the previous articles on excision, that's the cure for endo). Just think, it was only 20 yrs ago or so when they called IC a "disease of hysterical women" and told them to see a shrink!
There is hope on the horizon, and if we're lucky it will come soon.
Take care and have a great week. Don't forget, send me your questions and ideas for next week's blog. My record review are free until further notice, and you get two for the price of one!
Dr. Mos
Sorry I didn't write last week but we were in the OR until 10 pm one night and 8pm the other. Nobody ever said this kind of surgery was easy, but it sure is challenging.
I thought I'd write about interstitial cystitis today. IC as we call it, is the second leading cause of pelvic pain in women, behind (you guessed it) endo. Some studies show as high as 80% of women with chronic pelvic pain (CPP) had evidence of bladder involvement as a cause of pain, sometimes by itself and sometimes accompanying other pain generators such as endo or levator muscle spasm. IC causes pain in the central pelvis that can radiate out towards the groin or back to the sacrum. Some people feel it deeper, like at the top of the vagina. Typically the pain is worse with a full bladder, so these patients tend to void often, and they usually have some degree of urgency and frequency because the bladder wall doesn't like being stretched out. As you can imagine, intercourse can be miserable, although sometimes changing positions so the bladder is out of the way can make it tolerable.
One of the biggest problems with IC is that it can take years for patients to get a diagnosis. IC falls in the gap between gynecology and urology, with both specialties trying to pawn the patients off on the other. This is partially because of a lack of training about IC that hopefully is being rectified, and partially because many docs don't like taking care of chronic pain patients. It is true that patients with chronic pain can be difficult to care for, some requiring a lot of time and hand-holding and some with narcotic abuse issues, but I'll tell you what, there is nothing as satisfying as helping someone who's been in pain for a long time. Most patients don't want drugs, they want pain relief, and when you help them accomplish that, they are so grateful that for me anyway, that's all I need to keep me going. The other thing about pelvic pain is that it usually doesn't show up on ultrasounds, CT scans, or lab tests. Doctors that are good at diagnosing pelvic pain have to rely on listening to the patient and doing a focused, methodical exam, then use their powers of deductive reasoning and gut intuition to come up with an idea of what's causing the pain, and what to do about it. Doctors aren't used to making diagnoses this way these days, without confirmatory imaging studies, so it takes a special person with an interest in pelvic pain and compassion and empathy for the patients to be good at it.
Nobody is quite sure yet just what causes IC, but the leading idea is that of "neurogenic inflammation". What happens is that a painful stimulus that continues over time, (whether from endo or levator spasm or essentially anything in the pelvis) eventually can cause reverse transmission of nerve impulses from the spinal cord on down to the pelvis. When these stimuli reach the end of the nerve (for IC the bladder nerves) certain substances (like histamine, cytokines, and prostaglandins) are released from mast cells that cause swelling and nerve irritation. These inflammatory mediators that the mast cells release then cause more nerve irritation via other mediators that upregulate the pain fibers (nerve growth factor and substance P), eventually causing a vicious circle of pain. Another theory is that there are breaks in the mucousy lining of the bladder that protects the cells from all the irritants that the kidneys are excreting. These two theories are not mutually exclusive and likely the neurogenic inflammation causes the breaks in the lining (the technical term is the "gag layer" because it is composed of glycosamino glycan, or gag, which is a big huge sugar molecule).
It is logical based on the above theory of neurogenic inflammation why there is a significant overlap between those with endo and those with IC, but this doesn't explain why women without endo (or men, for that matter) get IC. Going back to the painful stimuli part of the theory, it is possible that any pain generator in the pelvis can induce the backwards nerve transmission and thereby neurogenic inflammation. It is a well-known fact that we are in an epidemic of abuse in this country. Whether by parents, partners, or strangers, nearly one third of all US women admit to having been abused at some point in their life. Women with IC have a higher percentage of abuse than those without - nearly half of IC patients admit to being the victim of abuse. The reason this is significant is that any abuse, but especially sexual abuse can cause a prolonged spasm of the levators, the muscles of the pelvic floor (the muscles used in Kegel exercises). Prolonged muscle spasm eventually causes increased firing of the pain sensing nerves in the muscles, which starts the vicious cycle. There is a lot of cross-innervation in the pelvis, which means that nerves to the bladder, pelvic floor, upper vagina, rectum, and any other pelvic site come from the same nerve root as it exits the spinal canal. They also interconnect like an LA freeway, joining and separating often on their way to the end-organ. This allows for 2 things to happen - the first is that a painful stimulus can start anywhere in the pelvis and cause the neurogenic inflammation in another pelvic location (this is also the proposed etiology for vulvar vestibulitis, but we won't get into that today). The second is that "neural cross-talk", or this sharing of nerve fibers between various organs in the pelvis, is probably why there are patients with IC, irritable bowel syndrome, and fibromyalgia all at the same time.
IC can be treated in a variety of ways, but none are "curative". Hydrodistention (blowing up the bladder with water under anesthesia) is both diagnostic and therapeutic. When either ulcers (only seen in 10-15% of pts) or glomerulations (small areas of bleeding from surface blood vessels) are seen after hydrodistention, then IC is present. Hydrodistention also is known to give some relief for a few months afterwards. Bladder instillations are my favorite treatment because they work quickly and pretty effectively. For people with significant pain, instilling lidocaine (a local anesthetic) and heparin (rebuilds the mucous lining) can give them immediate pain relief that can last for several days. Initially the instillations are 3 times a week, but with time the interval can be stretched out to a week or 2. Elmiron is a drug designed specifically for IC. It also rebuilds the lining like heparin does, but it takes 3-6 months to see a benefit, which is why I like to start with both oral Elmiron and bladder instillations. Tricyclic antidepressants work well to depress the pain fiber activation, essentially putting a damper on the hypersensitive pain nerves. Since chronic pain does cause depression, having a side effects of a little mood elevation and improved sleep doesn't hurt, either. Some people advocate antihistamines, specifically atarax, because it stabilizes the mast cells so they don't release their nasty inflammatory mediators, but I find that most people can't tolerate the sleepiness it causes.
I think there's a lot we don't yet know about pain, why some patients have minimal disease and a lot of pain, why some patients have a ton of disease and a little pain, and why some people develop new pain after their old pain was fixed. The good news is that a lot of research is being done to identify places in the inflammation cascade as well as the nervous system where interventions can be made. Right now we have some drugs that definitely can help women with IC and those with chronic pelvic pain (non-endo related), but I think that within the next 5-10 years some significant advances will be made. (for those of you that missed the previous articles on excision, that's the cure for endo). Just think, it was only 20 yrs ago or so when they called IC a "disease of hysterical women" and told them to see a shrink!
There is hope on the horizon, and if we're lucky it will come soon.
Take care and have a great week. Don't forget, send me your questions and ideas for next week's blog. My record review are free until further notice, and you get two for the price of one!
Dr. Mos
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