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Lymphedema praecox of the lower extremity
Lymphedema praecox of the lower extremity Thomas Bauer, MD, Gottfried Wechselberger, MD, Thomas Schoeller, MD, and Hildegunde Piza-Katzer, MD, Innsbruck, Austria
From the Department of Plastic and Reconstructive Surgery, University of Innsbruck, and the Ludwig Boltzmann Institute for Quality Control in Plastic Surgery, Innsbruck, Austria
A 30-YEAR-OLD PATIENT was referred to our department with a giant lymphedema of the right lower leg (Fig 1). The anamnesis revealed an onset of the swelling of the right lower leg during puberty. Recurrent erysipelas (cellulitis) and ulcerations occurred, and the swelling increased until the patient could no longer bend his knee. At this time, a Thompson’s operation had been performed elsewhere with limited success which, after 1 year, resulted in giant swelling and a lower leg that was not movable. We performed a lymphangiogram preoperatively, which showed malformation of the lymphatic vessels in the inguinal region. Therefore, we decided to perform a Charles’ operation with the modification of keeping the vena saphena magna and vena saphena parva intact. A total of 20 kg of skin, subcutaneous tissue, and muscle fascia were removed, and the split-thickness skin grafts salvaged from the specimen were stored for 3 days because of fear of skin loss. Meanwhile, we used Epigard (BioVisioN GmbH Ilmenau, Germany) for coverage. Another operation was necessary because of bleeding, but after the third day, coverage of the lower leg with the skin grafts was performed and further healing was uneventful. The patient is now permanently wearing a compression stocking and has gained full knee-joint mobility again. No further erysipelas (cellulitis) or ulcerations occurred after 1 year postoperatively, and the aesthetic result is acceptable (Fig 2).
Accepted for publication September 28, 2001. Reprint requests: Thomas Bauer, MD, Univ Klinik für Plastische und Wiederherstellungschirurgie, Innsbruck, Anichstr. 35, A6020 Innsbruck, Austria. Surgery 2002;132:899-900. © 2002, Mosby, Inc. All rights reserved. 0039-6060/2002/$35.00 + 0 11/60/120669 doi:10.1067/msy.2002.120669
Fig 1. Preoperative view of the giant lymphedema praecox of the right lower leg of the 30-year-old patient.
DISCUSSION Lymphedema is defined as an abnormal collection of interstitial protein-rich fluid. The cause for this disorder can be either an embryologic maldevelopment (primary lymphedema) or an acquired obstruction (secondary lymphedema). The most common form of the primary lymphedema is the so-called lymphedema praecox (Meige’s disease), with its typical onset during puberty. It is found in nearly 1 in 100,000 girls and 1 in 400,000 boys. There is mostly difficulty in the lower extremity, and in 70%, it is unilateral.1 The primary management of lymphedema is conservative and includes SURGERY 899
900 Bauer et al
Surgery November 2002
the Charles’ procedure. It removes all the tissue superficial to the muscle compartments and resurfaces the pared extremity with skin grafts salvaged from the specimen.2 In their article, Dellon and Hoopes3 state that the Charles’ procedure has excellent long-term results (mean follow up: over 10 years) without recurrence. He also rules out the importance of taking very thick split-thickness or, more importantly, full-thickness skin grafts, which we can confirm. We believe it is safer to perform a 2-stage technique, with resection and storage of skin grafts in 1 operation and resurfacing after a few days when bleedings no longer occur, which lowers the risk of losing the salvaged extremity skin. Miller et al4 also report excellent long-term results (mean follow up: 14 years) after excisional techniques but stresses the foot and ankle region as a problem area. We left the vena saphena magna and vena saphena parva intact to reduce the postoperative problem of swelling of the foot; no swelling of the foot occurred in our patient. We think that with this modification of the Charles’ procedure, excellent functional and aesthetic results can be achieved. We recommend it especially for younger patients who are psychologically and physically impaired by their huge extremity. Fig 2. View of the same patient 1 year after operation.
elevation, compression, and prevention or immediate treatment of infections, which is successful for most patients. When the medical management fails, an operation is the last option. The surgical procedures can be categorized into 2 groups: (1) methods that improve lymph flow and (2) those which remove lymphedematous tissue by excision. The most extensive of the excisional procedures is
REFERENCES 1. Kenkel JM. Vascular anomalies and lymphedema. Selected Readings Plast Surg 2000;9:31-9. 2. Charles RH. Elephantiasis scroti. In: Latham A, editor. A System of Treatment. Vol III. London: Churchill; 1912. p. 504-13. 3. Dellon AL, Hoopes JE. The Charles procedure for primary lymphedema. Plast Reconstr Surg 1977;60:589-95. 4. Miller TA, Wyatt LE, Rudkin GH. Staged skin and subcutaneous excision for lymphedema: a favorable report of longterm results. Plast Reconstr Surg 1998;102:1486-98.
From the Department of Plastic and Reconstructive Surgery, University of Innsbruck, and the Ludwig Boltzmann Institute for Quality Control in Plastic Surgery, Innsbruck, Austria
A 30-YEAR-OLD PATIENT was referred to our department with a giant lymphedema of the right lower leg (Fig 1). The anamnesis revealed an onset of the swelling of the right lower leg during puberty. Recurrent erysipelas (cellulitis) and ulcerations occurred, and the swelling increased until the patient could no longer bend his knee. At this time, a Thompson’s operation had been performed elsewhere with limited success which, after 1 year, resulted in giant swelling and a lower leg that was not movable. We performed a lymphangiogram preoperatively, which showed malformation of the lymphatic vessels in the inguinal region. Therefore, we decided to perform a Charles’ operation with the modification of keeping the vena saphena magna and vena saphena parva intact. A total of 20 kg of skin, subcutaneous tissue, and muscle fascia were removed, and the split-thickness skin grafts salvaged from the specimen were stored for 3 days because of fear of skin loss. Meanwhile, we used Epigard (BioVisioN GmbH Ilmenau, Germany) for coverage. Another operation was necessary because of bleeding, but after the third day, coverage of the lower leg with the skin grafts was performed and further healing was uneventful. The patient is now permanently wearing a compression stocking and has gained full knee-joint mobility again. No further erysipelas (cellulitis) or ulcerations occurred after 1 year postoperatively, and the aesthetic result is acceptable (Fig 2).
Accepted for publication September 28, 2001. Reprint requests: Thomas Bauer, MD, Univ Klinik für Plastische und Wiederherstellungschirurgie, Innsbruck, Anichstr. 35, A6020 Innsbruck, Austria. Surgery 2002;132:899-900. © 2002, Mosby, Inc. All rights reserved. 0039-6060/2002/$35.00 + 0 11/60/120669 doi:10.1067/msy.2002.120669
Fig 1. Preoperative view of the giant lymphedema praecox of the right lower leg of the 30-year-old patient.
DISCUSSION Lymphedema is defined as an abnormal collection of interstitial protein-rich fluid. The cause for this disorder can be either an embryologic maldevelopment (primary lymphedema) or an acquired obstruction (secondary lymphedema). The most common form of the primary lymphedema is the so-called lymphedema praecox (Meige’s disease), with its typical onset during puberty. It is found in nearly 1 in 100,000 girls and 1 in 400,000 boys. There is mostly difficulty in the lower extremity, and in 70%, it is unilateral.1 The primary management of lymphedema is conservative and includes SURGERY 899
900 Bauer et al
Surgery November 2002
the Charles’ procedure. It removes all the tissue superficial to the muscle compartments and resurfaces the pared extremity with skin grafts salvaged from the specimen.2 In their article, Dellon and Hoopes3 state that the Charles’ procedure has excellent long-term results (mean follow up: over 10 years) without recurrence. He also rules out the importance of taking very thick split-thickness or, more importantly, full-thickness skin grafts, which we can confirm. We believe it is safer to perform a 2-stage technique, with resection and storage of skin grafts in 1 operation and resurfacing after a few days when bleedings no longer occur, which lowers the risk of losing the salvaged extremity skin. Miller et al4 also report excellent long-term results (mean follow up: 14 years) after excisional techniques but stresses the foot and ankle region as a problem area. We left the vena saphena magna and vena saphena parva intact to reduce the postoperative problem of swelling of the foot; no swelling of the foot occurred in our patient. We think that with this modification of the Charles’ procedure, excellent functional and aesthetic results can be achieved. We recommend it especially for younger patients who are psychologically and physically impaired by their huge extremity. Fig 2. View of the same patient 1 year after operation.
elevation, compression, and prevention or immediate treatment of infections, which is successful for most patients. When the medical management fails, an operation is the last option. The surgical procedures can be categorized into 2 groups: (1) methods that improve lymph flow and (2) those which remove lymphedematous tissue by excision. The most extensive of the excisional procedures is
REFERENCES 1. Kenkel JM. Vascular anomalies and lymphedema. Selected Readings Plast Surg 2000;9:31-9. 2. Charles RH. Elephantiasis scroti. In: Latham A, editor. A System of Treatment. Vol III. London: Churchill; 1912. p. 504-13. 3. Dellon AL, Hoopes JE. The Charles procedure for primary lymphedema. Plast Reconstr Surg 1977;60:589-95. 4. Miller TA, Wyatt LE, Rudkin GH. Staged skin and subcutaneous excision for lymphedema: a favorable report of longterm results. Plast Reconstr Surg 1998;102:1486-98.