Q: My 15-year-old son has lymphedema that actually began in 1999. There is a strong suspicion that it was a result of surgery he had in December 1998. The surgery was an "incision and drainage" in the groin area for a ruptured infection. Unfortunately, the physicians were never able to advise us of the infection's cause. During this time period from 1999 to present, he has had intermittent infections in both of his legs. An Infectious Disease Specialist treats him for this condition. Usually he is prescribed Levaquin for a period of 2-4 weeks and then the infection goes away. However, the infected leg appears to remain enlarged, which is an obvious symptom. Our primary concern is that, last February, one of his infections disappeared, yet his scrotum and penis area became enlarged and has remained enlarged through the present time. In February, his Infectious Disease Specialist requested a pediatric urologist see him. When the pediatric urologist saw him, she became alarmed because she had never seen such swelling in the scrotum and penis area in any other cases. She recommended he take 2 baths per day for 2 months and she would follow up with him in late August. The baths were recommended to reduce swelling. She also prescribed an ultrasound prior to his visit in August. Simultaneously, his pediatric infectious disease specialist, after consulting with his pediatric urologist, believed it would be a good idea if he attended a therapy program (to be everyday for 2 weeks, then 2-3 times per week after up to 4 weeks) which he began immediately. At that time, his status would be evaluated. He was also told to wear a Jobst stocking everyday. The therapy was to be done on his leg and groin area, but not on the scrotum and penis area. The therapist is a lymphedema specialist, but was not specifically a scrotum and penis lymphedema therapist. He was not even sure if there is a form of therapy that could be done on these sensitive parts. Both my wife and I are extremely frustrated; of all the physicians he is seeing, no one has seen a lymphedema case like his. Furthermore, no one appears to know what can be done. They are attempting to think of and we were told to solicit feedback from a therapist who specializes in lymphedema of the penis and scrotum. To further complicate matters, my son also has histiocytosis. Is there any assistance you could provide us regarding where we could seek further education and support for his condition?
A: The swelling that your son suffers A. is most likely lymphedema. Indeed, it may be the result of the surgical intervention in 1998, injury and subsequent scarring. However, the swelling may also be caused by underlying dysplasia (malformation) of the lymphatic system in which case the surgery added "insult to injury." It is not uncommon that the genital area is part of the lymphedema and when this happens, it adds a host of problems for the patient and therapist. The ultrasound examination that your pediatric urologist prescribed is indicated in order to rule out other conditions that could be combined with the lymphedema of the scrotum, e.g., hydrocele or cancer. Meticulous hygiene in the genital and lower extremity areas must be part of your son's daily routine, however, bathing twice per day may be "overboard." Lymphedema of the penis and scrotum usually can be reduced through Manual Lymphatic Drainage (MLD) and compression therapy (bandaging). After a few treatment sessions with a knowledgeable therapist, your son should be able to perform these techniques daily and independently. Lymphedema is a chronic condition; therefore, it is important to teach the self-management of this condition to your son.
At this time, I am unable to be more specific about your son's self-management program (MLD, bandaging and garment use), because it differs from person to person. I would urge you however, to find treatment for the genital lymphedema and not simply the leg edema as treatment of the latter may force some fluid into the genital area, exacerbating the condition. It may take some time to find a lymphedema therapist who is also competent in the treatment of genital lymphedema in your area-a list of expert therapists is available through the NLN website. Also, there are a small number of lymphedema expert physicians available for consultation in the U.S. and the NLN can provide assistance in identifying them. I understand your frustration in this matter, but also believe that you are now one step closer to finding help and support for your son's condition. I also think that once your son is independent with the edema management, the entire family will feel better. The fact that your son also suffers from histocystosis should not have any bearing on the above treatment suggestion for the scrotal and penile lymphedema.
Q: My 4-year-old daughter had a sebaceous cyst removed from her upper left thigh (approximately 3 cm from groin) about a year and a half ago. The surgeon decided to remove an adjacent swollen lymph node for biopsy. The node was infected with mycobacteria and she was treated for that condition. She recovered well and, other than a scar, she showed no unusual signs. Then, about three months ago, she received a number of traumas in the same leg. She was bitten by a chipmunk, contracted Lyme disease from a tick bite to the leg, was exposed to poison ivy, and had a toenail crushed in a door. Soon after, her foot and lower leg began to swell. The toe with the crushed nail is the most noticeably swollen. She received two 3-week treatments of antibiotics for the Lyme disease, which we expected would clear up the swelling. It didn't and, after being evaluated by a number of doctors, the consensus seems to be that she has lymphedema. So far, the swelling is fairly mild, though noticeable. My question is: Since she had so many traumas to the left leg in a short period of time, could the remaining lymph nodes be having difficulty transporting the fluid out of the leg? If so, could the condition improve without significant long-term intervention as long as she has no other major traumas to the leg? I imagine that the immune system must have worked harder than usual to fight these infections and, therefore, could have produced more lymph than usual.or is such hope just denial?
A: Your daughter's case is unusual A. because of the number and kinds of traumas she suffered in a short period of time. We realize that young children are active and can injure themselves during play, etc., but every effort must be made to supervise children closely and minimize the chance of injury which can cause a potentially life-threatening infection in the involved limb. The question of whether the current swelling is caused entirely because of the traumas or perhaps part of an underlying dysplasia of the lymphatic system (primary lymphedema) is difficult to answer, even for an expert physician. In any event, the remaining lymph nodes and lymphatic vessels are trying to compensate for the part that is injured (removed). The current swelling indeed may improve over time provided that there are no additional traumas to your daughter's legs; however, the long term prognosis is uncertain. At this time, it is imperative that trauma and infections are avoided. A list with risk reduction practices is available on the NLN website. Lyme disease needs to be treated aggressively (as was the case with your daughter) as this could pose a risk for complications with swelling in the future. At this time, I would suggest that your daughter receives Manual Lymphatic Drainage (MLD), mild compression therapy (depending on the severity of the edema-bandaging or garments) and instruction in self-management from a certified lymphedema therapist. This approach will help to reduce the current swelling and teach your daughter to manage/control it in the future. The prognosis of your daughter's leg swelling is somewhat uncertain, however, it is possible that it will turn into a chronic condition.
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