Case Study—Klippel Trenauny Syndrome- Optimizing well being Jan Rice, Coordinator: Wound Education La Trobe University, World of Wounds

1. Case Study—Klippel Trenauny Syndrome- Optimizing well being
Jan Rice, Coordinator: Wound Education
La Trobe University, World of Wounds
www.worldofwounds.com

2. AIMS
This case study will highlight just how the clinician can impact on the well being of someone
born with a congenital syndrome that has impacted on her young life resulting in hard to
heal wounds. Klippel Trenaunay Syndrome is a rare syndrome usually involving port wine
stains, excess growth of bones and soft tissue, and varicose veins. My patient was thirteen
years old when I first met her—she is now twenty one years of age and has had multiple
venous ulcers, surgery and scleropathy sessions with her vascular surgeon. My goal in her
management plan was to provide support and strategies to self care and fulfil her life
ambitions.
METHODS
As a health professional it is easy to pay lip service to the terms –holistic care. The reality is
that this is sometimes very difficult to achieve in our cost constrained environments.
Accurate assessment, appropriate referral, ongoing education and continual support
through modern media has allowed my patient to explore her dreams and lead a fulfilling
life to date
RESULTS/DISCUSSION
My patient never spoke when I first met her—her mother answered all my questions. Today
my patient is self caring-and with encouragement has managed to travel to India, Nepal
and Israel in the past 2 years. Emails photographs allow us to chat about wound care and
provide her with the support she has required to gain self confidence in self care.
Abstract

3. Klippel Trenaunay Syndrome
This syndrome is rare and involves port wine stains, excess
growth of bones and soft tissue and varicose veins. Present
at birth, there is no known cause although a few cases are
thought to be inherited, possibly an autosomal dominant
trait. (1)
This condition in general does not affect life expectancy
however due to the port wine stains or dark spots on the
skin, varicose veins which often become troublesome in
adolescence and the excessive growth of the long bones,
more often in the legs than arms these patients may suffer
from psychological or self confidence issues.
This is not the case in
discussion. Images of her legs
were not taken to avoid
embarrassment. The image is
similar to her lower legs

4. • Diagnosed at birth with a discoloured legs and prominent veins-DoB
August 1991
• 2005 first ulcer
• 2006 vein surgery by Vascular surgeon
• 2006 diagnosed with Graves disease
• 2008 toe ulcer after someone stood on her foot accidentally-
osteomyelitis
• 2009 HBO for osteomyelitis –developed peripheral neuropathy
• 2010-foot wound healed
• Since 2013 has had approx 13 ulcers and multiple sclerotherapy
injections for bulging veins
• 2013-another trauma foot wound when someone stood on her foot in
Paris
Medical & surgical history

5. •Very loving caring family, mum, dad, and sisters
•Mother accompanied Pt A to most of her
appointments
•Pt A spend many days at home off school and many
days at various medical appointments
•Throughout the care of the toe wounds in 2010 Pt A
expressed a desire to travel to India, Cambodia,
Burma and Pakistan with her church group. She was
very anxious about infection risks and felt she could
not travel until the ulcers healed.
Patient A—social situation

6. We got the toe ulcers healed and another ulcer appeared in her medial gaiter of
the (L) leg- the humanitarian trip she so desperately wanted to go on was being
put off yet again.
I encouraged her to begin communicating to me by email and she was able to
see that with my advice she was able to be even more independent and did not
need to come to clinic as often. This was the beginning of using the electronic
technology available to still have her primary wound care clinician from afar.
―Hey Jan,
I've had this spot on my foot for a while, even before I left for Nepal. However I
never showed you because thought it wasn't a big deal. However the skin around
the spot is starting to get a bit red and sensitive. So I have attached some photos
for you to look at. If you have any advice on what I should put on it. I have been
putting moisturizer on it so far‖
Thanks Jan!!

7. With more and more encouragement Pt A did go on her
Indian missionary trip—multiple emails, photos and
descriptions of what she should do helped her to complete
her trip and not suffer any further complications. Of note was
the impact of the heat and her wound dressings—more
regular dressings with greater ability to wick sweat away was
necessary, together with changing her socks twice per day
and never wearing open toes sandals.
Since the trip to India Pt A has now also been to Israel and
France and has further plans to travel. Some of our email
communications can be seen below.

8. How r u going? How’s the new year going for u?? I hope u are doing well! Palestine is amazing! I'm
based in Bethlehem at the moment, and it is so beautiful! I’m going to a Palestinian refugee camp
today, which although I’ve heard is very sad I’m really excited to experience it and learn about the
problems they face. I'm sorry that I only come to you when my ulcer isn't doing so well. (I've lost my
phone so I couldn't send you any photos, however the ulcer on my right foot is looking bigger. When I
opened up my dressing a part of the area around the ulcer looks like its deteriorating. On the area
around the ulcer that looks like its deteriorating it was wet (before I cleaned it up) with white wet stuff
on the area, and a little bit of that yellowy matter that I had to pick of.
Also its very dry around the rest of the ulcer. Should I put moisturizer on that? and should Ipick the dry
skin of? I've been putting .......the ulcer and .....on top to cover. I don't have anything else with me. The
ulcer hurt last night when i was doing my dressing but I think that's when it started to dry out. Can I
ask you how do you know if the ulcer is infected?
Thank you so much for letting me contact you about my foot. I am a little worried about it but I
assumed it would get bigger while I was away.
Take Care and have a beautiful day!!
Email--25/1/13

9. Hey Jan, these are photos of my ulcer. It looks better now that I
no longer use ......... So I might just keep doing what you
suggested and do my ulcer more often than what I
was previously doing it. Before I left it 5 days sometimes.
Do you think this is a good idea?
Thank you so much Jan!
Email 28/1/2013

10. Conclusion
The rapid electronic means of communication can help
a patient suffering from a chronic disease manage their
condition while still maintaining optimal contact with
their health care provider. This may not always suit the
health care provider to have emails from patients but
occasionally there is someone who touches your heart
and the tiny amount of time it takes to answer a query
provides far greater support than you or I can imagine.