Your Spouse, Children,Family and Your Lymphedema

Your Spouse, Children, Family and Your Lymphedema

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An often overlooked complication in the lymphedema patients day to day struggle is the response and support received from our families and/or spouses. As foor for thought, I wanted to share some of them here.
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Lymphedema isn't some inconvenient disability that you can ignore and hope it goes away. If effects the patient, their family, spouses.
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Because it is a life long condition that requires life long care, it is a struggle, often a very difficult struggle, moetionally and physically. It is also a progressive condition that continues to slowly but surely rob you of you ability fo do things and be active. In the later stages it is also terribly painful with some of us living with twenty-four hour a day pain.
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So it does effect all our relationships, our spouses, children, family.

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Original Post in thread February 23, 2007

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How does everyone get their spouse to understand lymphedema? I have it in both legs, feet, left than right....and I cannot do much of the house work....and he gets very angry at me...and says I need to try harder....he goes to the store for me...but I think its only because if he didnt, he wouldnt eat.

How does everyone else cope with this problem of getting work done re: house...etc.??? Sorry to whine but I am at my wits end on this matter...I guess he thinks I make this up....but anyone who is cursed with this disease knows....you cant "make up" something like this....can anyone enlighten me...or maybe...just talk to me?? Sorry to whine.....

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Response #1 February 23, 2007

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Speaking for myself, I doubt that too many spouses or family really get it! It is easier to just "ignore" the problem. I have had lymphedema in my legs and feet for over 20 years with chronic cellulitis infections which are hard to deal with. Actually I have a very loving family but they still don't understand the limitations of this condition. I just do the best I can and nobody complains.

If the medical profession doesn't "get it" how can we expect anyone else to understand. Your family can't see the pain you are in. All they see is the swelling. The next time your husband is feeling under the weather don't be too sympathetic Women are the nurturers and for the most part are more compassionate. Try not to get too upset over this problem, you are not alone.

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Response #2 February 25, 2007

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Hey xxx and xxx,

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Yes, it is very difficult for others to understand our illness, just like it is hard for us to understand at the begining and it our daily management of LE. My spouse also did not understand it at first and he was in the service-so their mentality is just do it, no matter how you are feeling. I started educating him on the condition and what it would mean for our future. It took awhile for it all to sink in before he finally got it that some days there is no "just do it" for me. It also helped that I got him to also speak to another husband who's wife had been diagnosised years before I was, so he could talk to him about things he didn't want to share with me. Please remember that this illness not only affects us but also affects our families future lives as well, so it will take them time to adjust as it did for us.

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I have LE all over and i do the housework I can, when I can. This is something I do for me, because it gets me up and moving around the house. If I try to stay in a recliner all day, I noticed I had no energy and dozed on and off all day. I try to do little things throughout the day if I'm able, which could include emptying the diswasher, picking up around the family room, cleaning mirrors, dusting, cleaning bathroom sinks, washing clothes, and anything else I'm up to in the day. Of course I spread out the things I can do during the week, so I'm not trying to clean the house all in one day. I also make dinner, when able and we have lots of things that heat up in no time if I'm not feeling up to making dinner. I am very diligent in my LE management and it helps so that my body can do housecleaning here and there. About a year ago I was very depressed about my LE and just did not feel I was capable of doing little things around the house, but once I received treatment and came out of my depression(still on meds and seeing a therapist about once a month) I have more energy to do some light housekeeping. My husband and son pick up the heaver housecleaning chores. Hope this helps.

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Response #3 February 25, 2007

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I to have had to deal with a husband who at first had a hard time with my as we call them bad days. He now has had many strokes. We take care of each other. Most of my family has no understanding of what is happening to me. They don't want to hear about it. I now claim all of you as my family. I am glad I can vent here. And I can get good advice. I to have limp in both legs and feet and my stomach. I don't have insurance and we live on a limited income so I get no help except what I can do for myself. I also have a infection doc. who keeps me on meds. I love to go places. But now I can only do a few things. I will continue to do what ever I can. I went to the fair last year because they had electric wheelchairs. It was great to be free to go most everywhere. My son said you go mom. They could not keep up with me. and they got tired first. I love my husband and he is really a special man. But like me he had to have time to learn to live with the new me.

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Response #4 February 26, 2007

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Hi Everyone

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I think each of us have walked this path.

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Again I say we have a hard time understanding this condition, one day your feeling ok and suddenly wiping your nose takes every ounce of energy How do you explain fatigue to someone that doesnt have it?

How do you explain legs that you put more effort into moving that most would to run a marathon? or a hand that picking a paper up causes so much pain?

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How do you explain that a stroking of our limbs wrapping and wearing supports needs to be done every day and that it can make such a huge difference.

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How do you explain sitting with your feet up and dinner isnt on when they come home from a days hard graft?

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I think a lot of it is down to our own feelings of guilt for being ill. We have nothing to be guilty about but how many times do you carry on struggling with pain and doing things you know are bad for our condition.

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How many times is it easier to battle thru the pain barrier and tiredens and weeping limbs than go thru the explinations. So many times i read in letters of people working till they are ill because there is no option working with weeping edema and cellulitus when we know the best thing for them is rest and keeping off the limbs.

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Until this condition is recognised around the world as a cronic debilitating condition we will go on dealing with the same problem.

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WHY DON'T PEOPLE UNDERSTAND? I think a lot of it is down to the stubborness off most people with a cronic condition if we give in to it it wins so our loved ones see us day after day gritting out teeth and getting on with it when we finally have to say i cant do it anymore then they look at us and think why not you do it most days lymphedema is a condition that calls for sheer determination and stubborness to live each day maybe that determination and stuborness gives our loved ones the impression that were tough and we cope with anything.

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I have to admit i many times have said, "leave me i can do it."

Stubborn and independant something that seems a nessessity for anyone with a cronic debilitating condition.

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Hugssssssssssss you wonderful people

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Response #5 February 26, 2007

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Thank you.....all of you who responded or just read my story. I appreciate all your help.....it means so much to have a place where you can read other postings...get an idea of how others cope..or just plain talk to others....who live the same debilitating life.......sometimes pain meds help a lot and other times....nothing...helps but getting off your limbs for a long time.....thanks and God bless all of you. It makes me feel like I have a great big family, which is very comforting to me. I lost my dear Mother in May of 2006 and miss her very much. I think stress adds to the overall problem also....JMO. Thanks again...maybe one day....everyone will begin to understand this disease, especially our loved ones.

Association between fetal lymphedema and congenital cardiovascular defects in Turner syndrome.

Association between fetal lymphedema and congenital cardiovascular defects in Turner syndrome.

Pediatrics. 2005 Mar

Loscalzo ML, Van PL, Ho VB, Bakalov VK, Rosing DR, Malone CA,
Dietz HC, Bondy CA.

McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

ABSTRACT

OBJECTIVES: Turner syndrome (TS) is associated with congenital cardiovascular defects (CCVDs), most commonly bicuspid aortic valve (BAV) and aortic coarctation (COARC), congenital renal anomalies, and fetal lymphedema. It has been theorized that compressive or obstructive effects of fetal lymphedema may actually cause cardiovascular and renal dysmorphogenesis in TS. The objective of this study was to determine whether there is a specific association between a history of fetal lymphedema and CCVDs in monosomy X, or TS, independent of karyotype or general severity of the phenotype.

METHODS: This was a prospective study of 134 girls and women who have TS (mean age: 30 years) and were clinically evaluated for evidence of fetal lymphedema, classified as central (signified by the presence of neck webbing) or peripheral (current or perinatal, or dysplastic fingernails). The presence of BAV and/or COARC was detected by magnetic resonance imaging combined with echocardiography, and renal anomalies were determined by ultrasound.

RESULTS: There is a strong association between developmental central lymphedema, signified by neck webbing, and the presence of BAV (chi2 = 10) and COARC (chi2 = 8). The association between webbed neck and CCVDs was independent of karyotype. There was, in contrast, no significant association between renal anomalies and webbed neck or CCVDs.

CONCLUSIONS: The strong, statistically significant association between neck webbing and the presence of BAV and COARC in TS suggests a pathogenetic connection between fetal lymphatic obstruction and defective aortic development. The presence of neck webbing in TS should alert the clinician to the possibility of congenital cardiovascular defects.

Pediatrics

Lymphedema - Just Live With It

Lymphedema - "Just Live with It"

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Barbara Pilvin

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February 2007

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For four decades that was all I was offered. Did the increasingly severe swelling, first in my right foot, ankle and lower leg, then in the left as well, bother me? How about the blisters that accompanied it? They would start small, then grow and sprout new ones, oozing on my skin, my socks, my sheets. What about the fiery streaks, spots and blotches on my lower extremities, the bruises that never went away? And the aches, the sense of pressure in my feet that made me wonder if they would keep puffing up like balloons...and then pop?

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“What are you COMPLAINING about?” people would ask when I told them any of that. Their eyes would travel as they spoke, from my feet, ankles and legs to my face, and they would add, “No one notices your legs, Barbara!”

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None of them had any such complaints, of course, and since neither they nor any doctor I saw knew what was wrong, everyone assumed I was just “whining” about a problem that only existed in my mind. They ignored the fact that lower-extremity swelling plagued both my father’s mother and older brother, and that mine had never completely disappeared since the afternoon it had begun, in December 1961, when I was 10, with an inexplicably painful (but not swollen) right ankle, followed the next morning by blotches and streaks with terrible (but painless) ankle swelling. After five or six years with that swelling, I decided everyone must be right, and vowed to be stoical about it.

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But that didn’t make it go away. In fact, it got worse. So did my fear of it, for if it kept getting worse, what could it do to me? Could it kill me? It could certainly cripple me. By 1969, my first year in college, it affected both legs, and the blisters were unsightly, itchy, painful and chronic. Sometimes my feet were so swollen I couldn’t wear shoes. I remember the evening a friend came to my dorm room, tried to get me to join her and others at a party, and gasped when I tearfully showed her my feet.

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In graduate school in the mid-‘70's, I was told by my doctor that the problem was called lymphedema, and that there was no treatment for it. Again, it was “just live with it.”

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In mid-August 2002 I skinned a toe in a hotel swimming pool. Basic first-aid measures notwithstanding, within four days I had cellulitis, a bacterial infection that can be life- and limb-threatening. While recuperating, I read enough to know that, if I did indeed have lymphedema, I was in a high-risk group for more such infections...and that there actually was a means of treating it. I demanded a diagnosis, and knew within three weeks that I had primary lymphedema.

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Today, thanks to diligent treatment, my lower extremities look very attractive. With my open-toe prescription compression stockings, custom-made toecaps (they look like gloves with open fingertips), special foam under the toecaps with divets cut to follow the line of the metatarsals, and non-prescription compression stockings (my idea), no one can tell there’s anything seriously wrong with my legs. The left looks a bit larger than the right, but not enough to draw attention. I can wear normal shoes, and only occasionally have skin infections. This is because the lymphatic fluid, which contains bacteria and other toxins, is moving better through my genetically-inadequate lymphatic system, thanks to those compression garments and the other medical devices that are an essential component of the only standard treatment for lymphedema. It’s called CDT, for complex (or complete or comprehensive) decongestive therapy, and the garments are the daytime key, while compression sleeves or short-stretch bandages and accessories are the nighttime key, as well as the 24/7 key for initial treatment.

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Sounds good, right? Well, it’s better than nothing, and I’m a lot better off than many others with lymphedema. Any cancer survivor whose treatment includes lymph-node removal or irradiation is at risk for it, and about 30% of this country’s three million breast cancer survivors have it, according to the Lymphatic Research Foundation. So do survivors of other cancers, injury, and surgery, while only about one in 6,000-10,000 people, like me, inherit it, developing it either in childhood or adolescence (some are born with it), or as adults. Worldwide, millions of people develop it as a result of a mosquito-born parasite. This form is called filariasis, and in the photos I’ve seen, it causes elephantiasis: stage 3 lymphedema.

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Mine had hit late stage 2 by the time it was diagnosed. Why not stage 3? I was smart and lucky: I had taken good care of my skin and kept my weight down by walking, swimming, skating, biking, watching what I ate and being blessed with some slimness genes.

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I was lucky in another sense, too: I had access to expert professional care and insurance that covered my prescription stockings and toecaps.

I’m no longer so lucky. My insurance plan, like so many others, has followed the lead of Medicare, which now terms “secondary surgical dressings” the expensive compression garments that people with lymphedema need to remain healthy. In other words, the garments aren’t covered. Without them, the effects of the disease on our skin and tissues will become increasingly severe, making us unable to resist infections like cellulitis. Since any injury, even surgical, to the affected area can have this effect, amputation of a lymphedematous limb won’t improve our condition.

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With help from the staff of Senator Arlen Specter, I was able to get my prescription garments covered until last December. Now, like many others, I’m up a creek–and scared. Without insurance coverage, we won’t be able to “live with” lymphedema. We’ll deteriorate with it. We’ll become unable to work, take care of ourselves and our families, or stay out of hospital beds and emergency rooms. We’ll have infection after infection, and we’ll need increasingly powerful antibiotics for them because we’ll become resistant to the bacteria that cause them.

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And then, from a disease that’s treatable and controllable, we’ll die.

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Barbara Pilvin

2nd International NLN Patient Summit

The 2nd International NLN Patient Summit

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Atlanta, Georgia

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Lymphedema: Sharing Our World Of Knowledge

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2007 NLN Patient Summit Schedule

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Friday, October 5

5:00 - 7:00 PM

Registration

Check-In

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Early Bird Exhibits

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Saturday, October 6

7:00 - 8:15 AM

Registration Check-In

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Continental Breakfast

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Exhibits 8:15 - 8:30 AM Welcome

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8:30 AM - Noon PLENARY SESSIONS

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Overview of the Lymphatic System

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Primary and Secondary Lymphedema

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Diagnostic Procedures

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Infection and Lymphedema

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Risk Reduction

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Genetics and Lymphedema

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Quality of Life and Sexuality

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Wounds and Lymphedema

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Noon - 2:00 PM Lunch

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Exhibits

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2:00 - 3:30 PM INSTRUCTIONAL SESSIONS

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[P1] Complete Decongestive Therapy for the Advanced Patient

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[P2] Complete Decongestive Therapy for the Newly Diagnosed Lymphedema Patient

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[P3] Legislation: Insurance/Medicare Issues [P4] Meet the Professor

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[P5] Parent to Parent Networking (PLAN)

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3:30 - 4:00 PM Break

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Exhibits

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4:00 - 5:30 PM INSTRUCTIONAL SESSIONS

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[P6] Lymphedema Advocacy and Awareness

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[P7] Self Care for Upper Extremity Lymphedema

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[P8] Pain Management

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[P9] Exercise

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[P10] Special Session: Teen Space (networking session, 10 & up)

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6:00 - 8:00 PM

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Networking Reception (Exhibit Hall)

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Sunday, October 7

7:30 - 9:00 AM

Continental Breakfast

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Exhibits

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ROUNDTABLE SESSIONS

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9:00 - 10:30 AM INSTRUCTIONAL SESSIONS

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[P11] Basic Lymphedema Exercises - Pool Exercise

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[P12] Bandaging for Upper and Lower Extremity Lymphedema (hands-on)

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[P13] Meet the Professor

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[P14] Alternative Modalities

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[P15] Lymph Science Advocacy Program (LSAP)

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10:30 - 11:00 AM Break

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Exhibits

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11:00 AM - 12:30 PM INSTRUCTIONAL SESSIONS

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[P16] Obesity and Lymphedema

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[P17] Problem Solving Compression Garments

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[P18] Self care for Lower Extremity Lymphedema

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[P19] Treatment for Newborns and Pediatrics

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12:30 - 2:00 PM Lunch

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Exhibits (closes at 1:30 pm)

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2:00 - 3:30 PM PLENARY SESSIONS

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Overview of the Lymphatic System

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Primary and Secondary Lymphedema

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Risk Reduction

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Treatment of Lymphedema

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Genetics and Lymphedema

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Infection Management

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Lipedema

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Wounds and Lymphedema

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3:30 - 4:00 PM Break

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4:00 - 5:15 PM PATIENT CLINIC

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Complicated case histories presented by patients/parents and reviewed by a panel of experts 5:15 - 5:30 PM Closing Remarks

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National Lymphedema Network

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On behalf of: Lymphedema People

We can not urge lymphedema patients strongly enough to go to this conference.

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See you there - Pat O'Connor

Lymphedema Tarda After Liver Transplantation: A Case Report and Review of the Literature

Lymphedema Tarda After Liver Transplantation: A Case Report and Review of the Literature
Sammy Saab,1 Stephen Nguyen,2 James Collins,3 Gregg Kunder,4 Ronald W Busuttil5

We present a patient with lymphedema that developed after orthotopic liver transplantation. The cause of the posttransplant lymphedema was likely related to a developmental abnormality of the lymphatic system that was exaggerated by refractory chylous ascites. A peritoneal fluid with a milky appearance, chylous ascites is rich in triglyceride and is caused by the obstruction or disruption of abdominal lymphatic channels. It is a rare complication that may develop after trauma or abdominal surgery or as a result of a malignant disease [1], and it is even more uncommon after liver transplantation [2]. Therapy for chylous ascites involves treating its underlying cause. In the patient we describe, lymphedema tarda, which was diagnosed 6 months after liver transplantation, was likely caused by chylous ascites and a developmental abnormality of the lymphatic system.

Keywords: Chylous ascites, Lymphedema tarda, Evaluation, Liver transplantation

Case Report: In June 2004, a 49-year-old man underwent orthotopic liver transplantation (OLT) without venovenous bypass for decompensated liver disease caused by hepatitis C and alcohol abuse. The manifestations of his liver disease included hepatic encephalopathy, refractory ascites, extreme fatigue, lower-extremity edema, and hepatocellular carcinoma. The ascitic fluid was clear and the color of straw. The serum ascites gradient was greater than 1.1 g/dL. After surgery, this patient was discharged with instructions to continue treatment with mycophenolate mofetil, prednisone, tacrolimus, trimethoprim/sulfamethoxazole, and ganciclovir. At discharge, his aspartate amino transferase (AST) and amino alanine transferase (ALT) values were 39 U/L and 78 U/L, respectively. One month after the transplantation, the patient underwent a liver biopsy to determine the cause of newly elevated values of AST (284 U/L) and ALT (596 U/L). The results of a percutaneous liver biopsy were consistent with recurrent mildly active chronic hepatitis C and portal fibrosis. Two months after his surgery, straw-colored ascites developed, and the following results of liver testing revealed worsening hepatic function: serum total bilirubin, 9.0 mg/dL; serum creatinine, 1.8 mg/dL; alkaline phosphatase, 286 U/L; ALT, 397 U/L; and AST, 508 U/L. The hepatitis C viral load at that time was 2,840,000 IU/mL. A 10-cm 10-Fr biliary stent was placed during endoscopic retrograde cholangiopancreatography in the common bile duct to ensure a smooth anastomotic stricture. A repeat liver biopsy revealed chronic hepatitis C with mild lymphocytic infiltrates and periportal fibrosis. Treatment with pegylated interferon and ribavirin for recurrent hepatitis C was initiated.

In March 2005, 9 months after the transplantation, bilateral nonpitting lower-extremity edema and diuretic-refractory ascites developed and were treated with large-volume paracentesis every 2 weeks (± 0.66 week). During each paracentesis treatment, a mean (± SD) volume of 9 L (± 2.5 L) was removed. The ascitic fluid was milky, and the patient’s triglyceride value was 173 mg/dL (± 46.2 mg/dL). The ascitic fluid albumin value was 1g/dl and the mean serum albumin value was 2 g/dL (± 0.22 g/dL). The mean serum creatinine value was 1.7 mg/dL (± 0.2 mg/dL), and the serum triglyceride value ranged from 248 to 255 mg/dL. The results of bacterial and fungal cultures and cytologic studies were consistently negative.

Magnetic resonance imaging (MRI), including MRI angiography and venography of the abdomen and pelvis, revealed marked ascites and patent blood vessels. The results of the hepatic venogram and pressure measurements showed no peritoneal abnormality, portal hypertension, or hepatic vein thrombosis. The results of a transjugular liver biopsy indicated mild lymphocytic infiltration and periportal fibrosis. Because of the patient’s persistent chylous ascites, a lymphangiogram was performed to determine whether the thoracic duct was intact. The diagnosis of lymphedema tarda (Meige disease) was based on the results of the bipedal lymphangiogram, which demonstrated insufficient lymphatic drainage from the left lower extremity and lymph nodes within the femoral triangle as well as mild fibrosis over the dorsal fascial planes (Figures 1, A and B; and 2, A and B) [3]. Fluoroscopically guided cannulation of a single lymphatic vessel over the dorsum of the right foot revealed impedance to the flow of ethiodized oil (Ethiodol) contrast medium. Fluoroscopically guided cannulation of the lymphatic vessel over the left foot showed the flow of Ethiodol into a single dilated lymphatic vessel (as opposed to the usual 6 to 10 such vessels) over the medial left thigh and into a single superficial inguinal lymph node (Figure 1, A and B). Ethiodol contrast medium was noted in the superficial inguinal, hypogastric, and iliac divisions.

Eventually, the contrast medium advanced into the left lumbar lymphatic vessels and crossed over to the right of midline, partially outlining the periaortic lymphatic vessels up to the level of the second to the third lumbar vertebra of the cisterna chyli. The contrast medium was contained within the thoracic duct at the middle of the twelfth thoracic vertebra. A fluoroscopically guided study showed the extravasation of Ethiodol from the thoracic duct at the level of the twelfth thoracic vertebra. A metal wire was placed over the site to mark the level of extravasation of the contrast medium (Figure 3, A and B). Initial and delayed 24-hour follow-up radiographs documented the swelling of both feet as well as the circumvention of residual Ethiodol contrast medium into the superficial and dermal lymphatic vessels of the left foot and leg (Figure 2, A and B). The images featured in this report best display the causes of the patient’s lymphedema tarda and the extravasation of Ethiodol as previously described. In July 2005, treatment with pegylated interferon and ribavirin was discontinued. The following month, therapy with a low-fat medium-chain triglyceride and orlistat was begun. Although the frequency of paracentesis was not altered, the ascitic fluid triglyceride level decreased from a mean of 173 mg/dL to 75 mg/dL.

The patient underwent the placement of a peritoneovenous shunt in October 2005. From October to February 2006, he required 3 paracentesis treatments. The mean (± SD) ascitic fluid triglyceride value on the day of each paracentesis treatment was 75 mg/dL (± 10.6 mg/dL), and the mean (± SD) serum albumin value was 3.7 g/dL (± 0.33 g/dL). Furthermore, the appearance of the ascitic fluid changed from milky to clear and straw-colored. Since February 2006, this patient has not required further paracentesis, and his lymphedema has resolved. Abdominal ultrasonography in March 2006 revealed no ascites, and his hepatitis C viral load was undetectable.

Discussion: Chylous ascites is a rare finding that was shown in a study by Press and colleagues to occur with an incidence of approximately 1 in 20,000 admissions to a large university-based hospital over a 20-year period [4]. However, it is widely believed that the incidence of chylous ascites has increased because of more aggressive cardiothoracic and abdominal surgery as well as the longer survival of patients with cancer [5]. Chylous ascites can be caused by any of 3 mechanisms: obstruction of the major lymphatic channels at the base of the mesentery or the cisterna chyli, which causes leakage of chyle from dilated mesenteric lymphatic vessels; direct leakage of chyle through a lymphoperitoneal fistula created by abnormal or injured retroperitoneal lymphatic vessels; or the exudation of chyle through the retroperitoneal megalymphatic vessels without a visible fistula or obstruction of the thoracic duct [6]. Chylous ascites is caused by an abdominal malignacy or cirrhosis in more than two-thirds of all patients [1]. As many as half of those malignancies are lymphomas [1, 5]. Injury during abdominal aortic surgery, which is the most common cause of postoperative chylous ascites, results in 81% of all chylous complications [7]. Nevertheless, chylous ascites accounts for fewer than 1% of all complications after vascular surgery involving the abdominal aorta [8]. Warren shunts are an uncommon cause of chylous ascites [9], and chylous ascites rarely develops after liver transplantation [2, 10, 11]. We presumed that the development of chylous ascites after OLT in our patient (who did not exhibit ascites before his liver transplant) was caused by the disruption of lymphatic vessels near the porta hepatis or the retrohepatic area during the dissection and removal of the native liver. Primary lymphedema, which results from aplasia or hypoplasia of the lymphatics, is thought to be a genetically determined disease, the expression of which varies by the patient’s age at onset [12].

There are several primary causes for lymphedema: Milroy disease, a rare disorder in which the patient demonstrates a lymphatic malformation at birth; lymphedema praecox, which has an onset at or near puberty; and lymphedema tarda (Meige disease). Lymphedema tarda is the rarest developmental abnormality of the lymphatic system and is not clinically evident until the patient is 35 years of age or older. The manifestation of lymphedema tarda usually follows a precipitating event such as trauma or an inflammatory reaction [12]. The time of onset of lymphedema may be associated with the relative number of functioning lymphatic vessels. It is generally thought that the fewer the lymphatic vessels, the earlier the onset of lymphedema. Patients with lymphedema often exhibit absent or incompetent lymphatic valves. The results of a lymphangiogram revealed lymphedema tarda in our patient. In our opinion, our patient’s lymphedema was clinically manifested only after a secondary insult (in his case, the development of chylous ascites that was likely caused by a leaking lymphatic vessel, located in the area of surgical dissection, that impeded lymph return). Chylous ascites frequently causes painless abdominal distension [13]. Abdominal paracentesis is the most important diagnostic tool in evaluating and managing patients with ascites. In contrast to the straw-colored and transparent appearance of ascites caused by cirrhosis and portal hypertension, chylous ascites usually has a cloudy and turbid appearance. The triglyceride value in ascitic fluid (which is typically higher than 200 mg/dL, although some clinicians use a cutoff value of 110 mg/dL [14, 15]) is very important in identifying chylous ascites. Radiographic studies useful in the diagnosis and management of chylous ascites include computed tomography (which reveals pathologic intra-abdominal lymph nodes and masses) and lymphangiography and lymphoscintigraphy (which show abnormal retroperitoneal nodes, leakage from dilated lymphatic vessels, fistulization, and the patency of the thoracic duct) [16, 17]. Lymphangiography, which is the gold standard for identifying obstructions, may result in complications such as reactions to the contrast medium, transient lymphedema, tissue necrosis, or fat embolism [5, 16]. The management of chylous ascites remains controversial, but current treatments include repeated therapeutic paracentesis; a high-protein, low-fat, medium-chain–triglyceride diet; total parenteral nutrition (TPN); diuretics; the placement of a transjugular intrahepatic portosystemic shunt; peritoneovenous shunting; percutaneous coiling of the thoracic duct; or surgical ligation of the disrupted lymphatic channel [1, 18, 19]. The use of TPN in combination with octreotide has been reported to result in the rapid resolution of chylous ascites after liver transplantation [7]. It has been speculated that somatostatin improves chylous ascites by inhibiting lymph fluid excretion through specific receptors found in the normal intestinal wall of lymphatic vessels [7].

In a retrospective review of 156 patients with chylous ascites from various causes, 51 patients were successfully treated surgically (41% with the ligation of a leak, 39% with a peritoneovenous shunt, 10% with resection, and 10% with another surgical technique such as a peritoneal fluid filtration circuit) [5]. Of the 105 remaining patients who were treated conservatively, 67% were treated successfully. Forty-three percent of those individuals achieved success by dietary modification alone; a combination of diet, TPN, and paracentesis was successful in 26%; TPN alone produced resolution in 14%; and paracentesis alone was successful in 7% [5]. Our patient was initially treated with orlistat (which was recently reported to have benefitted a patient with chylous ascites [20]) and a high-protein, low-fat, medium-chain–triglyceride diet. When those measures were unsuccessful, he underwent the placement of a peritoneovenous shunt. Eventually, he experienced the resolution of both his chylous ascites and the edema caused by abnormal lymphatic drainage.

Our case report should increase the awareness that congenital anomalies of the lymphatic system, when exacerbated by surgical procedures such as OLT, are often indicated by edema of the lower extremities.

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