Jin Hee (Jeannie) Kim
Extrapelvic endometriosis
Advancing the Science and Surgery of Endometriosis
Monday and Tuesday, April 18-19, 2016
The Union Club, New York
My name is Jeanie Kim and I am one of the Gyn ___ researchers at Columbia. Thank you so much for the opportunity Dr. Seckin and the Endometriosis Foundation and also thank you Dr. Ja Hyun Shin for providing some of the videos although I am hearing that we may have a little difficulty so we will see what we can do. This is just my disclosure.
In terms of the more typical areas of endometriosis as you might all be aware is usually in the pelvis which is most commonly in the posterior cul-de-sac, ovarian fossa, the ovaries, the anterior cul-de-sac, bowel and appendix. In today’s talk I am going to focus more on endometriosis disease outside of the pelvis even if the endometriosis invades the bladder or the bowel outside of those organs.
In terms of objectives we will discuss some of the types and locations of extra-pelvic endometriosis using some case presentations, namely these five areas we will discuss the diagnosis and treatment and pre-operative considerations. Also we will try to review some of the current literature. I have to point out though that there is probably a dearth of literature in this area. A lot of the literature is mostly case series and case reports.
The first case is a 27 year old para3 with three prior C-sections, the last two years ago. Her third pregnancy was complicated by retained POC and postpartum hemorrhage requiring a D&C and a transfusion. She presented with a 4 cm mass 3 cm above her cesarean scar on the left side. She reports noncyclic pain and swelling. I think in terms of differential it probably may depend on who you are seeing and whether you are a general surgeon or a GYN surgeon but we do have to think about many things including incisional hernia, hematomas, granulomas, abscess and soft tissue tumors either benign or malignant. But as a gyn surgeon we should also think about abdominal wall endometriosis and I am sure many of you have seen this in your practice. In terms of abdominal wall endometriosis and a prior C-section scar the incidence is roughly .03 to in some reports up to 1 percent, up to .5 percent so it is not a common occurrence. It is thought to be iatrogenic seeding of endometrial cells upon delivery at the time of C-section. There usually is a palpable tender nodule, nodural mass that can be either cyclic or non-cyclic in terms of pain and it is typically near the cesarean scar but does not have to be at the cesarean scar. Typically an MRI of the abdomen and pelvis may give you much more information.
This is an example of an MRI. You can see the mass in the circle but it can be many other things. Typically the treatment is surgical excision for definitive diagnosis and treatment. Sometimes one can apply medical treatment especially if it is not so bothersome to the patient or if it is very small but the disadvantage is that you do not have a pathologic or histologic diagnosis. The typical treatment recommendation would be surgical excision for diagnosis as well. At the time of the surgery it is important to get a disease free margin, like adenomyosis or adenomyomas, similar to that these masses typically get very stuck within the subcutaneous tissue and the pre-operative testing with the MRI would also give you the information about the size and the location in terms of how close it is to the actual fascia. In terms of whether or not you want to consult your fellow general surgeon for possible mesh excision or mesh placement if it is a very wide area. You might want to consider concurrent laparoscopy especially if the patient does not have a prior pelvic endometriosis diagnosis and like we discussed medical hormonal therapy is probably less recommended than surgical.
I may just have to show the videos at the end. I am having a little bit of technical difficulty here.
In terms of abdominal wall endometriosis in a prior cesarean scar, again, a retrospective chart review in 2010 by a general surgeon. It was a key series of 40 patients and cyclic pain was noted in 40 percent, non-cyclic 45 percent so about the same and the mean duration of symptoms was about 18 months before surgery was performed. It is interesting that the pre-op diagnosis was correct in only about half the cases. The re-operation rate is also notable, nine percent in terms of residual disease that had to be gotten at a later time.
Another retrospective key series of 12, again, another general surgeon, this was a little bit older in 2003 but again, similar breakdown, cyclic, non-cyclic it does not have to be cyclic pain. The mean duration of symptoms was again about two years prior to surgical treatment. Again, the prep diagnosis was even a bit lower 33 percent correct and 16 percent required mesh. They generally reported that within the follow up period of up to three years there was no recurrence. But again the data is not the most robust.
This is published by an OBGYN looking at 18 cases. Essentially in this cohort of patients there was more non-cyclic pain and in terms of – you can see the varying ultrasounds, CT, MRI diagnostic methods that were used and in terms of their surgery their mass size was about 4 cm and they did report no recurrence in two years but their diagnostic capability was they report no correct in all cases.
There are a few reports of malignant transformation of these endometriosis found in the prior C-section scar. They are basically case reports of these patients. One is of a most recently in 2012 is of a 49 year old with progressive growth of a C-section scar mass with pain for 25 years. Surgical excision confirmed clear cell carcinoma. She received the appropriate chemotherapy and she was free of recurrence in eight months. That is a short follow up. But malignant transformation from an abdominal wall endometriosis is extremely rare. There were 22 cases reported in the literature.
Moving on to a different case, a 30 year old para1 with no prior surgeries. She reports cyclic umbilical pain and bleeding. As you can see here on exam there is a 1 cm nodule on her umbilicus that is tender to touch.
In terms of differentials we would think about potentially other melanoma, keloid, fistula, abscess, hernia and maybe a soft tissue tumor, benign or malignant. We also do have to think about umbilical endometriosis even if it is someone who has never had surgery before. There is a primary umbilical endometriosis, again, not very common. The literature is based on case reports and patients may present with cyclic pain, nodule and spontaneous bleeding that correlates with her menstrual cycle.
This is just a CT showing a mass. In terms of treatment, excision is again touted as the definitive sort of diagnosis in addition to treatment. It is difficult to see because it is 1 cm or sometimes it gets much smaller you do want to time your surgery at the appropriate time so that it is the biggest so you can actually see the mass. Consider laparoscopy if there are any concurrent symptoms and especially if there is no prior diagnosis of endometriosis in the pelvis. You may want to consider a plastics consult, you are essentially removing the actual umbilicus and the stock as well so there may be obliteration of the umbilicus. Surgical therapy is usually preferred over medical.
This is an example of pre and post, and again, there is a video that I do not think is working for whatever reason but I will try to find it and play it back.
Moving onto port site endometriosis, similar in terms of umbilical endometriosis that is primary but this is in patient who have had prior surgery. This is a recent 2015 case report. This is a patient who has had two laparoscopies for endometriosis, the last surgery was three years ago so it was quite a few years ago. In terms of diagnosis, ultrasound, MRI and there was only 16 cases of this reported in the current literature. It has been found to be present after potentially laparoscopy with the endometrial tissues seeding endometriosis tissues seeding, cystectomy, appendectomy, myomectomy and cholecystectomy.
There is another case report of an ovarian remnant syndrome in the port site. This is of a 22 year old with a history of laparoscopic USO for an endometrioma five years prior. After excision they saw ectopic ovary on the pathology specimen. Ovarian remnant syndrome is a rare GYN condition but something to have in the back of your head. Obviously there are many things that you could do to reduce the risk of port site seeding; using a bag, having it intact, perhaps irrigation and making sure that your pathology is not really touching the skin area. And there is consideration for a culdotomy if its patients in terms of not having it seed but potentially I suppose you could seed it in that area as well.
Moving onto our third case. This is a 34 year old para1 with a history of a left mediolateral episiotomy. She has had multiple ER visits for a lump type of pain. On exam she has a palpable tender nodule at 7 o’clock. I am probably not going to spend too much time on this because I think there is a following talk on vaginal excision of endometriosis. In terms of differential I am thinking about in the vaginal area obviously abscess/infection, condyloma and Bartholin’s cyst – cancer, benign mass is also a consideration. But in someone who has had a prior episiotomy potentially we do want to think about vaginal endometriosis. It is based on case reports but most commonly reported with a history of episiotomy, not necessarily all the time. Typically patients present with cyclic pain and nodule. There is a possible delay in treatment with misdiagnosis. A full excision sometimes may not be possible depending on the location but the recommendation is to try to excise it as much as possible.
The fourth case is a 33 year old G0 spontaneous pneumothorax after the onset of menses and the differential in terms of what we can think about are probably what the pulmonary doctors see more so than us but lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and other things and we do want to think about catamenial pneumothorax. I am sure many of you may have seen this. Typically it starts within 72 hours after the onset of menses. There is chest or scapular pain in most patients. Dyspnea, typically it is right sided and frequently the recurrence is due to delay in diagnosis. It is important to elicit prior episodes and timing with the menstrual cycle. In terms of diagnosis typically patients present with a pneumothorax and a CT of the chest would be helpful in terms of looking at this. However thoracoscopy is probably the most accurate way of actually taking a look and possibly biopsying. Treatment – treating the immediate pneumothorax with a chest tube and secondary blebectomy, therapeutic pleurodesis. Sometimes if the hole is too big you have to do a diaphragmatic repair with mesh and hormonal suppression is often times recommended for six to 12 months. Recurrent pneumothorax is as high as 40 percent after the combined VATS procedure and the hormonal suppression so they are at high risk of having this again despite doing all the things that we do to minimize that recurrence rate.
A quick literature review: 2007 in this series 24 percent of the patients who had this type of VATS procedure actually had catamenial pneumothorax and 18 of them had histologic confirmation. The important thing is that you do not always need histologic confirmation if your suspicion is high enough. In these patients six months of Lupron was given with a mean follow up and the recurrence rate was about 30 percent and another smaller case series showing sort of similar information.
So last but not least in terms of other areas where endometriosis can occur just to put it out there in terms of CNS locations including the cerebellum and the cerebrum and the spinal cord as well. A patient with a gait disturbance, headaches, history of congenital hydrocephalus had multiple shunt revisions since her first VP shunt at the age of two months old, and the last was two years prior. This MRI T2 shows fluid cystic levels similar to what we might see in the pelvis like an endometrioma and after surgical excision the pathology confirmed endometriosis. There are three other case reports documenting similar types of endometriosis seen in the CNS system.
A quick summary, it is very important to do a thorough H&P so in terms of painful cyclic palpable mass when does it occur? It does not have to be cyclic. Also, attaining that surgical history and obstetric history and also the review of systems is extremely important. In terms of pre-operative planning with imaging, MRI is usually touted as the most preferred way to aid in diagnosis and also to evaluate the involvement of the surrounding tissue whether or not it is close to fascia in the case of the C-section abdominal scar.
