Sunday, August 27, 2006
Treatment of Pediatric Lymphedema
The Treatment of Pediatric Lymphedema
Any discussion of pediatric lymphedema must include information about our most current scientific understanding of the underlying causes. Pediatric lymphedema may be uncomplicated in its presentation, akin to a benign adult primary lymphedema, or may indeed be very medically complex. One must assume in any pediatric form of lymphedema that there is either a defect in the normal development of lymphatic tissue, or that the visible swelling may be one of the first signs and symptoms of malignant disease. For this reason no condition involving edema should be casually viewed or dismissed by the medical professional and immediate clarification as to the presence or absence of cancer must be investigated.
The two main classifications of lymphedema are primary and secondary and are labeled as such according to the cause of the lymphatic impairment. Primary lymphedema can be described as an inherent defect within the lymphatic tissue itself. This malformation may involve either the absence of tissue or a malformation of tissue, (usually lymph vessels) which results in the presence of abnormally high levels of proteins and tissue fluids within the tissue spaces (interstitium). Secondary lymphedema is always associated with a traumatic event or series of events. Causes include multiple infections of the skin or soft tissues, surgical removal of lymph nodes, as in cancer treatment, radiation therapy, acute thrombo-phlebitis (DVT) or other traumas. Again the consequence is similar (lymphedema) and results in a disturbance of normal lymphatic drainage.
Following astute investigation and differential diagnosis, benign primary lymphedema (not associated with cancer) occurring in an infant at birth or within the first year of life is classified as Type 1 Nonne-Milroy Syndrome ( Milroy's Disease). This ''congenital hereditary'' (present at birth, sometimes inherited) form of lymphedema is described as usually involving the lower extremities but sometimes the arms, hands and face and may also be associated with malformations of the intestinal lymphatics (hyperplasia or other dysplasia). The additional intestinal involvement may result in poor absorption of proteins into the blood stream. This is due to congestion created by large fatty molecules that also rely upon lymphatic vessels for absorption. Symptoms may include bloating of the abdomen (ascites), low blood albumin levels (hypo-proteinemia), high protein levels in the stool (protein losing enteropathy) and in advanced cases, a malnourished appearance. Some Milroy's patients may require special dietary guidelines involving less complex fatty acids, which are often times successful in alleviating the congested intestinal lymphatic system.
Additional classifications of primary lymphedema occurring in children include Type 2 Meige Syndrome. This non-congenital familial (not present at birth, sometimes inherited) form is associated with the changes of puberty and mainly occurs in females. As in Milroy's disease this form is associated with lower extremity lymphedema, is chronic and progressive if left untreated, but does not involve intestinal lymphatic malformations. Both Type 1 and Type 2 primary lymphedema can further be classified as lymphedema praecox, which is an umbrella label used to describe any primary lymphedema occurring before the age of 35. Of the two types, type 1 (Milroy's Disease) accounts for 6-7% of all lymphedema praecox cases. (1)
Other diseases associated with pediatric lymphedema, which are beyond the scope of this article include Klipple Trenaunay Weber Syndrome (KTWS, otherwise termed Angio-Osteohypertrophy Syndrome), Noonan's Syndrome, Turner's Syndrome, Amniotic Band Syndrome (3 chromosomal disorders) and others. Of the aforementioned syndromes, KTWS is not uncommonly encountered in a busy lymphedema clinic and is a serious medical disorder involving overgrowth of veins, arteries, bones and lymphatic tissues. Practicing lymphedema therapists and diagnosing physicians must be able to identify this condition and make treatment adjustments to accommodate the complications of this disease. It is recommended that those pediatric patients with KTWS and associated lymphedema seek expert physician supervision, preferably from a vascular surgeon with additional training in lymphology.
As previously mentioned, pediatric lymphedema may be ''pure'' (uncomplicated) or complicated by other associated disorders of the vascular system or may involve physical obstruction, as in the case of malignancy. For this reason it is imperative to seek expert physician assessment in all cases. The correct diagnosis is nearly always gained clinically however, and relies in large part upon skilled observation, palpation and historical analysis. One safe and minimally invasive imaging procedure currently used to diagnose primary lymphedema is called Lymphangioscintigraphy (LAS). This procedure involves subcutaneous (within the dermis) injections of radioisotope tracers, which are photographed at set intervals to show the amount and speed of uptake and transport within the individuals' lymphatic system. However, when primary lymphedema is accurately diagnosed clinically, LAS may be omitted, as it will not change the course of treatment. Clearly, LAS is of great academic value and more importantly, may contribute to supporting the accuracy of the clinical diagnosis. Many times, as in the case of pediatric lymphedema, a clear image of the insufficient lymphatic drainage may put to rest parental concerns that lymphedema is not the only disease process at work.
Other testing procedures such venography and lymphography, which involve injections of dye are both painful and potentially damaging to vessel structures and have also been associated with deadly allergic reactions (anaphylaxis). In more complicated cases involving combination forms of lymphedema, Magnetic Resonance Imaging (MRI), Computer Tomography (CT scan) and Venous Doppler studies may be required to assist in designing a successful treatment plan. In uncomplicated cases, they are not necessary and only incur additional expense.
At present there is no cure for lymphedema. It is unfortunate that surgical interventions are still sometimes undertaken as a form as treatment for all types of lymphedema. Although many brilliant and well-intentioned surgeons have attempted a myriad of ingenious techniques, it remains a fact that no reliable benefits have come from these procedures. More often surgery results in significant additional cosmetic disfigurement or lymphatic impairment. The most common type of surgery can be categorized as a ''debulking procedure.'' It is not logical to believe that the removal of substantial amounts of subcutaneous tissue, containing functional lymphatic vessels, will result in an improvement of the overall condition. Surgery is therefore not indicated for extremity lymphedema.
Another common treatment for lymphedema is the pneumatic compression pump. Although pumps provide some relief and may temporarily halt the advancement of swelling, the benefits are nearly always temporary and may result in abnormally large accumulations of lymph at the root of the limbs and within the trunk. Significant numbers of patients with lower extremity lymphedema who have been treated by pumps report genital lymphedema following daily use (2). This consequence is nearly always avoided by comprehensive treatment with Complete Decongestive Therapy (CDT).
Complete Decongestive Therapy (CDT)
When the condition is present during infancy parents can benefit their child greatly by implementing a course of conservative treatment as early as possible. By initiating a sensible treatment strategy early on, it is hoped that the chronic and progressive consequences of the disease can be significantly lessened. The treatment method universally accepted as the gold standard of care for lymphedema is called Complete Decongestive Therapy (3). This program of treatment relies upon four interdependent components and is successful in halting the progression of the swelling, the chronic inflammatory changes within the skin (lymphostatic fibrosis) and promotes healthier immune response in the affected areas (4).
Additionally, CDT liberates the patient from encumbrances such as pneumatic compression pumps or limb elevation, and requires healthful activity levels and proactive patient involvement. The four components of CDT are: Manual Lymph Drainage (MLD), compression bandaging, remedial exercise and infection prevention (meticulous skin and nail care). Self-care training involving any of these components with the goal of optimizing the clinical treatment results in homecare should be seen as an important additional component.
CDT Guidelines for Pediatric Patients It has been the authors' experience that typical approaches to treatment must be significantly modified for the pediatric patient, especially in the case of infantile lymphedema. The following suggestions are the result of clinical experiences gathered through treatments involving dozens of children.
All parents of children with lymphedema must be educated about the condition so that a realistic picture of this chronic condition can be gained. No surgical cure or pharmaceutical medication is available. It is unfortunate that lymphedema is presently an incurable condition, however, with early competent guidance the secondary tissue changes associated with lymphedema can be greatly reduced. Overall limb size can be maintained at nearly normal girth and immune function can be enhanced with ongoing Manual Lymph Drainage treatments. These benefits cannot be underestimated, because the long-term impact is quite positive and rewarding. Normal limb function and socialization can be expected in most cases. Children with lymphedema can live very normal lives.
Be patient with the process
Overly aggressive care is never productive for the adult or child with lymphedema. Especially in pediatric cases where all tissues are delicate, great care must be taken to avoid creating injury or discomfort. One must remember that a comfortable and happy patient will be a compliant patient. This special child must not be mishandled. Parents can be ''consumed with worry'' and approach compression bandaging overzealously, with the mistaken impression that ''more pressure is better''. It is more likely however to produce discomfort, pain or skin infections which can actually cause a worsening in the condition. Pediatric patients cannot tell us what is bothering them early on, and without this feedback the caregiver must adopt a relaxed and careful approach to compression therapy.
Similarly, the Manual Lymph Drainage (MLD) treatments delivered by the parent may be counterproductive if they are heavy handed or rough. Lymphatic vessels are delicate, threadlike structures, which may spasm or become otherwise injured by inappropriate treatment. In the clinic, parents must invest time into learning the appropriate skills. This investment cannot be underestimated in its importance and also allows for question and answer sessions between parent and therapist. The better educated the parent the more likely they will be to provide high quality care for their child.
Depending upon the age of the child, most will initially need to adjust to this ''strange new routine'' as well as to the intervening therapist. Time must be budgeted for this necessary process to take place and may require ''play sessions'' at first to create a feeling of comfort and safety. Suggestions include setting up a television and video player in the treatment room as a distraction; bring favorite toys, music and foods. Place a soft clean quilt on the floor and attempt treatment and bandaging from this vantage point. Allow the parent to hold the child on his/ her lap while the therapist works. All the while verbal education can take place between therapist and patient, which is usually of great value. At first this format may seem unproductive and can be a source of further anxiety for the parent, however, should this step be omitted, the young child will not comply with any of the components of the program and will ultimately create an even less productive outcome. This approach may also allow for a more ''semi-intensive'' approach, in that the total treatment duration is reduced to 2 weeks versus 4+ weeks as in the case of an adult. Treatments can therefore be budgeted for future use involving frequent follow up visits where parent, patient and therapist can review home care techniques and remain current concerning the overall condition of the child.
Start with MLD and self-care suggestions
For the very young child of less than one year of age, therapists may be quite productive in administering MLD. This ''massage like'' treatment involving gentle, soothing skin manipulation is usually well received by the infant. Parents can observe and repeat the agreed upon treatment sequences at home and may find that the best time to administer treatment is during nap times or at night while the child is sound asleep. Older children of age 18 months to 3 years who are considerably more active and less agreeable to this routine may also benefit from similar timing. The quality of touch required for proper MLD treatment is so gentle that children will become positively conditioned, and in time will view it as another form of loving touch from the parent and therapist. They will also be able to develop skills for their self- care and can be engaged in practice as they mature.
The goals of MLD are to create more efficient drainage within the affected area and systemically offset the chronic skin changes associated with lymphedema. In children the early intervention of MLD is extremely valuable in slowing or reversing these effects especially on the dorsum of the hand and fingers and similarly on the dorsum of the foot and toes. In some cases the genital area is also chronically involved, MLD application is essential and may again, offset chronic skin changes.
Compression Therapy Considerations
As previously mentioned, pediatric tissues are far more delicate than those found in an adult. Compression therapy can be counterproductive if it is not administered with great care and skill. This becomes an additional burden to parents and must only be undertaken by responsible and cautious caregivers.
It has been the authors experience that some patients are simply too young to intervene with compression therapy. However, the skilled therapist may provide a modified bandage in the clinic to gain a clearer picture of the potential for improvement. This bandage may not be practical for parental application until a later date. A very young infant (1-4 months old) will benefit from the stimulation of MLD alone and, as the child begins to stand, gravity becomes an exacerbating factor, necessitating external support in the form of compression bandaging and/ or medically correct compression garments. The only situation where intensive compression bandaging would be provided before this time is in the case of massive swelling (elephantiasis), a situation rarely encountered in infants.
Once the child begins to stand, lymphedema of the legs and feet may begin to worsen. This is the point in time where some compression strategies should be explored and employed. Therapist and parent must consider the benefits and tradeoffs carefully however, as bulky, multi-layered bandaging may impact the child's ability to safely ''toddle''. Bending the knees to crawl is made more difficult, and in the case of arm lymphedema, tactile skills and simple grasping tasks may be largely compromised. For these reasons and including the child's tolerance level, an intensive phase of 2 weeks (10+ treatments) is usually adequate to deliver quality care, and homecare education to astute parents. Within this time frame the therapists goals should include: Achieving a fair amount of reduction (without force), learning the unique compression gradient for optimal long-term improvement, effectively educating one or more caregivers in basic tailored MLD techniques, applying compression bandages safely, and measuring for any necessary compression garments.
Special Compression Guidelines
Most bandaging materials are sized for the adult frame and are too large for most young children and especially the infant patient. Select 4cm compressions bandaging materials such as DemaBand® in these cases and graduate to 6cm Comprilan/Rosidal®, or at the maximum, 8cm materials for most children. Another special product that is kind to pediatric skin is Velfoam®. This fleece-lined foam padding material is quite kind to delicate tissues and should be incorporated or substituted for other forms of padding. Artiflex®, and Cellona® synthetic cotton padding products can also be used in combination with foam but should not be exclusively applied otherwise shifting will occur. Toes are usually too tiny to affectively wrap. A tourniquet effect is more likely on small circumferences and great caution should be used if an attempt is made. It is the authors' opinion that gentles MLD and manual manipulation can affectively treat toes. Fingers may be wrapped with traditional materials such as Transelast® or Elastomull® of an appropriate width. Take care to double the bandage into two-ply as it will concentrate the bandage on a smaller skin area
.Compression garments are usually fitted to age appropriate patients (12+ months). The benefit of these elastic sleeves is that they provide medically correct gradient pressure. By wearing a sleeve and glove or leg stocking daily, pediatric patients will experience liberation from cumbersome bandages. Normal developmental milestones can be reached without delay such as coordinated walking. Furthermore, since bandaging is practiced at night, parents can rest assured that less than expert-bandaging technique can be corrected by daytime gradient compression.
Activity and Lifestyle
Due to the chronic and lifelong nature of lymphedema, parents are encouraged to permit their child to engage in normal activities such as sports, arts and crafts, and outdoor play. With lymphedema comes an increased risk of acute infection (cellulites) especially when the skin is injured. However, to disallow normal play may have significant and more troubling side effects that may be mental or emotional in nature. Furthermore, many lymphedema patients have no history of infection following numerous traumas and are therefore capable of normal activity. If infections are an occurrence, some activities should be modified accordingly but with an awareness of these larger concerns. Lymphedema in the majority of cases is not a disabling condition, however children who have been raised to feel as though they have a disability are truly at a disadvantage and may not experience the joy and freedom of childhood that is their birthright.
Children with Lymphedema - Yahoo Support Group
Children's and Pediatric Lymphedema
National Lymphedema Network - Parents' Lymphedema Action Network