Case Study: Caring for an Infant with Klippel Trenaunay Syndrome (KTS) Complicated by Microcystic Malformation of Left Lower Quadrant
By Phyllis Tubbs-Gingerich, RN, BSN, LE, CLT-LANA


The parents brought the patient to our clinic when he was 3 months old. In the interim since his birth, his parents experienced a wide range of feelings so characteristic of loving parents who have an infant with a congenital disease.

Before beginning therapy, we immediately began intense emotional support by giving “lots of hugs”, touching both the patient and his parents, teaching and empowering them.  We stressed the fact that the patient was a beautiful, intelligent, good-looking little boy who just happened to have KTS and lymphedema.

Our goals were to teach the parents to interact with him as a normal boy; teach them how to care for his needs and teach the patient, as he grows, how to care for himself.
 

At 18-20 weeks gestation, a malformation of left lower quadrant was detected upon routine ultra sound.  Soon after his delivery on January 9, 2001, a thorough medical examination of the patient indicated he had an extremely large soft tissue overgrowth of the left foot, especially the dorsum, with massive alteration of the entire leg extending posteriorly into the buttock with irregularity of the gluteal cleft.  In addition, phlebectatic blood vessels were detected within the small vascular stain overlying the left buttock

Upon measurement, the affected left leg was found to be 2cm longer than the right leg.  Further tests concluded the presence of large microcystic lesions containing massive amounts of stagnant lymphatic fluid especially in the foot and lower leg.  An MRI performed on February 13, 2001, indicated the extent to which the patient’s left lower quadrant was affected. 

Basically, the lymphatic system in the patient’s left quadrant was immature and inadequate, body wastes & fluids were backing up distally and became hardened (fibrosed) and stagnant.

Our immediate concerns were the possibility of leakage of lymphatic fluid and the susceptibility of infection creating life-threatening circumstances.

Other critical issues were:

  1.  The potential function of the affected left leg – with 2 cm difference in length, would the affected left leg continue to grow more rapidly that the unaffected right leg?
  2.  KT Syndrome and port wine stain – to what degree is soft tissue and bone affected.
  3. What is the status of lymphatic connections/anastamosis between the foot and lower leg?     
  4.  How efficiently will fluids move through these affected areas?
  5.  How will intensive bandaging affect the integrity of the skin of an infant so small – bandaging must begin conservatively because of sensitivity of the patient’s affected foot and very small toes.
  6.  Design and availability of gradient compression garments for an infant with the patient’s condition.

 

Combined decongestive therapy for the patient began in early April 2001, within days of his first visit.  Protocol for manual lymph drainage was designed to move fluids medial to lateral torso and entire leg, anterior and posterior, into left ipse-lateral axilla. The directional flow garment, full left leg with hip attachment, was designed to follow MLD protocol, moving fluids into superficial ipse-lateral pathways bypassing the left groin and deep abdominal lymphatic pathways.

Skin care consisted of cleansing the entire affected area with Eucerin Gentle Skin Cleanser, rinsing the area with water very well, applying Eucering Light Lotion followed by an emollient A&D Ointment, applied over the entire leg and liberally over the foot and between the toes.  After applying stockinette over the entire foot and leg, we then wrapped each individual toe with very thin strips of Aqua-phor impregnated gauze, and

Dermagran N Impregnated Guaze over the toes or webs that had open or pressure areas.

Thin Elastamul strips were then wrapped around each toe to hold protective impregnated gauzes in place and to complete gradient compression for the toes.  This was very tedious but it served to prevent the patient’s toes from breaking down during extended bandaging periods.  Soft foam “chocolates” or “swell spots” were placed on fibrotic areas such as the calf and dorsum foot to break up fibrosis (hardened tissue) and soften tissues. Artiflex “rolls” were placed within Tricofix tubular gauze strips and applied in folds of the ankle and popliteal space.  Three (3) or four (4) Elastamul rolls wrapped spirally from toes to groin held everything in place and protected the patient’s little toes.  Short stretch bandage rolls were wrapped over elastamul from tip of toes to inguinal, completing compression of the leg for daytime.

Skin care and bandaging of the foot and toes remained the same for night time care.  A directional flow garment (DFG), full left leg with hip attachment was applied over stockinette.  Short stretch bandages were then applied over the DFG from toes to waist to secure its placement and for additional gradient compression.

As reduction progressed, we closely monitored the patient’s skin response to bandaging and skin care products in order to ascertain how his skin would possibly react to a future tight fitting daytime gradient compression garment.

It became very clear that skin integrity meant everything to satisfactory garment compliance especially for a fast growing, active little boy.  We also felt we needed to be “ready for the right gradient compression garment” because as the patient was increasing mobility and activity, any muscular movement on his part while wearing gradient compression, could increase the flow of lymph out of the calf, foot and buttocks.  As the patient began standing alone, he was placed in a compression class – 2, with compression beginning on the foot, full left leg with hip attachment and biker short right leg.  The crotch was left open for frequent diaper changes.  This was perfect for the patient’s transition into crawling and walking and brought about very good reduction of the entire left quadrant.  Wearing these garments, the foot softens in texture but always remains a challenge compelling us to seek new and different designs for gradient compression and directional flow garments.

As the patient increased in growth and mobility, the challenge of the gradient compression and directional flow garments having been met, shoes and body alignment became our next focus of attention.  Children need soft, lightweight, well-made leather shoes for good walking gait and body alignment.  After visiting several “shoe makers” it became apparent that their shoes would be too heavy for the patient to pick up his feet during his transition into walking. 

The patient’s condition and needs called for a “specialty shoe designer” who could “revise Stride Rites” and adapt them to his feet. Arney “the angel” was able to do just that.  Arney revised the left walking shoe to accommodate the patient’s affected left foot and then did a build-up of the right shoe to equalize leg lengths.  The patient loved these shoes and had no problem lifting his little feet to walk. Arney also revised sandals and sneakers in the same manner to accommodate the patient’s growing walking needs.

In September 2001, after several days of 100+ degree weather, the patient was hospitalized for 5 days with an infection that had begun in the affected left lower quadrant.  Three (3) days of IV antibiotic therapy finally perfused the tissues sufficiently to begin “killing” bacteria to reduce the patient’s body temperature.  During his hospitalization, we began reassessing his entire therapy program:

  1. Looking at increasing frequency of therapy
  2. revising gradient compression and directional flow garments for better coverage of affected areas
  3. decongestion and reduction of lymphedema following infection process by using more creative bandaging techniques
  4. more skin protection by using more prophylactic skin care products
  5.  more garments for frequent changes into clean garments
  6. more intense daily garment sanitation