Yeah. Our next speaker, we're very proud to present to you, we're very proud of him is dr eduardo solution. He is from Venezuela. He is Chief Resident in general surgery and administrative chief Resident in july. He will become a fellow and remain on our staff for another year. Mrs Starkey, our first winter storm, a therapist at MD Anderson will discuss her case with you with dr solution, did you? Mhm. Mhm. Mhm. What's the red light? Green light to wait for the green light? There he goes, Going crazy. His uh pleasure for me to be here. Such a distinguished auditorium. Green light went out okay. Uh huh. Keep them going. Uh We're going to talk today about a uh disease that uh is very unpleasant uh for the patients who get it. And uh he's a little bit frustrating for the people that have to take care of it and we have to Oh yeah, get a lot of courage and patience when we take care of this. Don't hear me over there. Uh a lot of patients and hello human relations when we take care of all these problems. Uh Mhm. Let's start with the first line. But the topic is Gardner syndrome and we would like to start a little bit with the history of the disease. It was first described by Garner, a physician Who in 1951 who found patients with Kalanick public poses that will present many times with other problems such as soft tissue tumors and bone tumors. After he's first research, a lot of people has oh, started this problem and they have found that he's a hereditary problem. That is different from the familiar polly policies and uh it's a yeah, dominant character is an auto samel dominant trade and Which is similar to the diabetes. For example, if two uh the couple both have the disease, 100 of their Children will have it. If only one, Only about 50 will have it. Just following the men the loss, the age and sex. Here we go. The agent sex is variable. We have had several patients over here and they all presented Usually at the age of 30 35. Now he has been described, Children with eight years old presented with the disease. A lot of families have been studied in their multitude of papers with complete uh 23 or four generations of uh families with everybody involved with this problem. Not everybody will have all three uh problems at the same time. The most common uh symptom is usually related to the soft tissue tumors because they usually present in the head and neck area, face, john skull and they're very obvious to the family. So some member of the family will say, what do you have in there. Once this, they go to the physician and they get an X ray. Then they find the other symptoms which is the bone tumors. And the third symptoms is even though it's the most dangerous and the one that causes more problems it's usually not discover unless patients uh reaches a third or fourth decade of life or is a fan because the physician insist or remember something that that he saw when he was in medical school or red. But Gardner's syndrome in order is a environment imma As a matter of fact, we had a patient over here where he was operated uh three or 4 times after being involved in a car accident. And uh he kept on telling everybody that he had rectal bleeding. And uh he was discharged from the hospital telling that he he had a rectal bleeding and nobody did anything about it until about 10 years later when he came over here because the soft tissue tumor and was found to have a Gardner syndrome. So in order to diagnose the disease, you have to think about it. This is a typical X ray. You see multitude of this little ground spots are polyps and this X ray is a little bit is uh rotate around. That's the splenic fletcher over there. These are right colon. And you can see that there are polyps all over the place. I think I have another one over here. And these are all the side again, polyps just throughout the entire colon. Mhm. The natural history of the disease as we started talking a little bit earlier, uh the the soft tissue tumors are classified or described in general as dismount tumors. And they can be space assist. They can be little fibroids in the subcutaneous tissue. They can have sizes up to 10 12 in diameter. And they can be located also in the trunk extremities. The most common areas is to face the trunk in abdominal wall, abdominal wall, mainly after uh surgical procedures. They developed in areas of all scars where somebody's been already operated. The bone tumors, they're usually benign in their classified in Osteo Mazz and osteo con Roma's there too. Types of benign tumors. They are usually also in the skull, the jaw, the cleric als and uh the upper extremities. The column polly poses as you saw is usually a type of nanometers pilot which is benign. Now, what happens if you live alone? You don't operate on them? Well, 100 of them will develop cancer of the colon. So as you can see is uh it presents a problem, especially when you see a child, eight years old with a colon full of polyps. And you uh present with the question, what what do I do now? Uh Some people has waited in these Children Until and follow them on 6 to a year intervals before they take the cold. Sometimes you're obligated to take the colon out because they present to the hops up to the hospital with untreatable bleeding. You have to do something about it. So will describe a little bit about the treatment of this disease. The surgical treatment usually related to the colon. Public posters, He's described as a ah So total collector me or abdominal collected me whatever you want to call it, preserving the rectum. Why do we preserve director? Uh the psychological, from a psychological standpoint, everybody would like to have normal bowel movements, Especially if you have a 15, 20 year old person with this problem. So we try to preserve. The only time that we take the correct amount is when we always do a practice copy dissemination this patient. If the the rectal mucosa is what we call aggressive or presents a tps or the polyps are big enough to uh present suspicious signs to the pathologies that there may be an early uh malignant degeneration in that area. Direct and rectum comes out without any question. So it it presents the total producto collect to me, which is called in the construction of a colostomy. The uh um the treatment of this is uh when do you do the collector me? Well you do it by the time you diagnose the disease. Usually you don't wait. Uh Why that is uh the reason is that one we wouldn't mention uh earlier and uh at what age the age that we should do this operation? Uh as I mentioned before, the Children, we can't afford to wait but not too long. It's been seen that when somebody reaches age of 50 and if they haven't done anything about it, they will have cancer. And if they have cancer, the complications are usually metastases. And when that happens, um this is kind of late. The radio therapy has been used for the uh soft tissue tumors with very bad results. And chemotherapy has been used also in the complicated treatment of complication. We've got on this a little bit later and with extremely poor results. So these diseases, it's bad disease. We can describe it like that. The yeah, the treatment of the complications. And this is a sad part of the disease. It's very easy to take out the colon and construct a really honest to me. But what comes out after he's, he's a, he's a terrible peregrine manage for the patients. They usually developed recurrent small bowel obstructions for two reasons, uh, adhesions and in recurrence of uh, tumors. There have been studies made on the fibroblast in healing process in these patients. And they have a very unusual fiber plants with more chromosomes than usual. And they do form fibrous tissue in unbelievable quantities in areas where they had surgery before. So you can see when somebody has the entire colon removed the entire of the entire abdomens completely. Uhh, yeah, has a rough surface and the entire abdomen just fills up with fibrous tissue, which produces a small vial of structures. Now, if there's not the fibrous tissue, then they form the fibromyalgia. If they're not five romans there, fibra sarcomas, which is the mulling and the generation of the firearm. So, uh, if it's not something it's going to be something else. So that these patients have just no way to go. It's just a matter of time and we just uh mhm. Some are lucky and they will not have any other problems after the collective me, but most of them won't. The treatment of the soft tissue tumors is wide excision and the resection. Whatever they presented and the bone lesions, we don't touch them unless they given the symptoms and uh, they're bothering the patient. We usually leave them, leave them alone. And uh at the end, this is a Elias to me, just to illustrate another complication. The patient had it lost me form and started to develop polyps in the Elias, to me. So that's another problem the holy poses in start to involve the entire balance. And there's been some patients where after all this happened, they started to develop polyps in the duodenum in the stomach, remaining all the bounds and uh the usual cause of death for this patient. Uh He's a multiple small mile obstructions, fistfuls, sepsis complications and they just die malnutrition. In order to illustrate a case that uh can show you the entire history of this disease will have me started to give us uh History of one patient that we have here to wait for the green light. Yeah, that's good. Okay. Where's where's that? Like plants? Okay. Yeah, understand what it's about this cash. Mhm. Yeah. This is a case report of a family with Gardner's syndrome. The original family member was a white male who died in 1952 at the age of 56 was cancer of the colon. He survived only three months after his surgery. It was not until 1972 that the 12 year old grandson of this gentleman was seen at the University of Texas Dental School for some problem teeth. And during the visit, the dentist noticed that he had nodules on us john. He was referred over here at MD Anderson for studies And during these studies with the very minimum bone surveys and so forth. A diagnosis diagnosis of Gardner Syndrome was made and this boy's father was called in and evaluated on a routine basis. The father is the primary patient in this report was 34 years old. Uh He was an only child and with his father's death, his mother lived in the household with a wife and three Children. The patient was a very quiet man, friendly but not outgoing. He described himself as times as a very private person. His activities away from his job. We're mostly concerned with his wife and with his Children. He was a really strict parent and very concerned with the future of his Children. While his marital relationship seemed satisfactory, he referred to his wife as being scatterbrained. However, he and his mother had a very dependent relationship on one another. This patient had a history of occasional but bloody stools, which he attributed to hemorrhoids for about eight months prior to his admission. Other than that he appeared essentially healthy. He ate well and maintained his general wait. He had he had several nodules on his forehead and he thought these were so vicious in nature. He had had one cyst removed from his left chest and thought this was a sebaceous assist. He also had multiple projecting areas of the mandible, particularly on the right side. Uh When they did the practice coptic examination, he had polyps from 100 cm down to the rectal and pure. When the biopsies were done, the pathologist reported that the lesions were benign. Uh There were several small polyps over the remainder of the colon, and these were reported as negative for malignancy. Somewhere. Adenomas, polyps and others were reported as colonic mucosa. On January the 8th 1973, this gentleman underwent a total, collected me and elio practice to me and had an uneventful recovery except for uh an alias. Four months after this surgery, when he was back for a checkup, routine studies were done and he had the same multiple pops which were extremely numerous. However, there was no sign of malignancy, and at this time it was decided to do an abdominal perennial resection on him. So in July of 73 he had this surgery and his recovery was benign before discharge from the hospital. He had learned to change its Elias Tommy appliance. Oh dear. Uh here's a picture of s toma. He had learned to change his appliance and was aware of the importance of caring for his paris tonal skin. He was discharged in a one piece appliance and later on a return visit to the clinic, he was changed to a two piece semi disposable appliance. And after the initial fitting, he assumed the responsibility of caring for his own supplies in obtaining them for himself. He didn't seem to have any problem keeping a paris stone, his paris tonal skin clear On April. In 75 he had an unexplained episode of bleeding and through the Elias to me and this stops spontaneously really before it could be evaluated. Another complaint at that time was low back pain and the IVP showed an obstruction to the curator's. During this time he also complained of gastrointestinal distress and was having symptoms of intermittent bowel obstruction. The general contour of his abdomen had changed and he began for the first time experiencing problems keeping his appliance intact. He was admitted to the hospital in April of 75 was an admitting diagnosis of probable gastrointestinal bowel obstruction with retro peritoneal fibrosis. During this day, following arteriosclerosis fee, he developed a definite bowel obstruction and had surgery hit extensive adhesions in the reception of a large segment of the J Juno ilium plus closures of small several small bowel fistulas. A large Desmarais tumor mass was found in the abdomen and the entire retro peritoneum was five roast. As a result of this reception, the patient had a short bowel syndrome and this is how he looked in the postoperative care unit. Mhm. Here's his J. J. Nice to me and he had to penrose drain sites and his old Elias thomas toma. So uh the care for these openings were initiated in the postoperative care unit. We used a cry a gasket over that J. J. Nice to me and with a loop pasta me bag and then you still um adhesive and official bags over the other penrose drain sites in the in the old Elias to me side. Post operatively, this patient had a multitude of problems. He was federal. Right from the second day poured copious amounts of influence from the J. J. Rasta me pure land drainage from both of the penrose drain sites in the Elias. To me, he was placed on intravenous hyper implementation and was later given feeding through the J. John Ostrom East Oma. He was given chemotherapy in an attempt to reduce the fiber optic mass. The drugs that he had included Ben, Christine, Adrian, Myson Cytoxan and D. I. C. The care of the skin around the J. Genus to me was a constant problem. A number of appliances were dried and finally a two piece of clients with a fully catheter through the gas outlet valve were put in. We had one resident, we had so much trouble keeping the bags on this fella and we'd clear up his excoriated skin and Uh he would just have this huge copious amount of drainage and the bags that come off. And this doctor told us that if we can get them into the moon 50 miles down the road, said we're gonna go to keep a bag on this fellow. So we kept at it. Ah So we inserted that, we took a marlin bag with a gas outlet valve and took part of it out and threaded this fully catheter through there and then put it in the genus to me and blew up the balloon and we drip the feedings into the J. John Ostrom east Oma. And uh, unfortunately, he didn't retain a whole lot of it and it would just, we had to connect it up to straight drainage too, collect the amount of drainage. Some days he had as much as 2500 ccs during an eight hour period. In spite of the intravenous hyper implementation, this patient continued to lose weight and as the JJ Nostra me stolen was high, the rib cage soon became a problem in keeping the face plate intact. He would, in his restlessness roll over on the bags, stopping the drainage and cause leakage. The paris to malaria was maintained with a constant vigilance from all members of the nursing staff. At times it really did seem impossible to keep bags on, but we persevered and sometimes spent hours at a time taking care of this patient. One staff nurse noted in her daily notes that the stomach therapist was working with a patient most of the day. As his condition deteriorated, his mental anxiety increased. He expressed feelings of hopelessness, inability to concentrate and was disinterested in his surroundings. He was extremely restless and had difficulty sleeping. He expressed feelings of being unable to see a future for himself. While many of his concerns involved his Children, he had trouble relating them relating to them into his wife. During the final stages of our patient's life, he developed cardio respiratory failure that resulted in pneumonia and finally in death. The oldest son, now 15 years old, has had several benign polyps removed with a colonoscopy procedure. He's being followed on a routine basis. He has a 50 chance of transmitting this devastating Jean to any offspring. He might have. The younger Children have both had negative physical examinations. They will be followed on an annual basis. And if by the age of 25 they have not developed any unusual cysts or Osteo Mazar tumors, the chances of their transmitting the gene are practically nil. Thank you. Okay.