An unusual case of lymphedema tarda

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An unusual case of lymphedema tarda

Postby patoco » Sat Feb 03, 2007 3:58 pm

An unusual case of lymphedema tarda

Mubin Hosnuter1A, Mustafa Buyukates2F, Berfu Babuccu1E

1 Department of Plastic Reconstructive and Aesthetic Surgery, Zonguldak Karaelmas University Medical School,
Kozlu/Zonguldak, Turkey
2 Department of Cardiovascular Surgery, Zonguldak Karaelmas University Medical School, Kozlu/Zonguldak, Turkey

Source of support: Departmental sources

Summary

Lymphedema is the result of the equilibrium between the load to be cleared and the transport capacity of the clearing system. Lymphedema may be classified as primary or secondary, based on
the underlying etiology. Primary lymphedema is an unusual disorder characterized by inadequate lymphatic drainage. Lymphedema tarda is a rare form of primary lymphedema.

The case of lymphedema tarda documented here was chronic, progressive, and resistant to medical therapy and recurred several times after previous operations.

We performed two-staged operations and we recommend that the staged excisional procedures offers reliable long-term improvement and minimizes postoperative complications in chronic advanced lymphedema.

Keywords: lymphedema • classification • lymphedema tarda • treatment • surgery

Mustafa Buyukates, MD, ZKÜ Medical School, 67600 Kozlu/ZONGULDAK, Turkey,
e-mail: mustafabuyukates@yahoo.com

BACKGROUND

Lymph is normally cleared from tissue spaces through a network of thin-walled lymphatics [1]. The primary function of this system is to clear the interstitial spaces of excess water, large molecules, and particles and to transport them to the intravascular circulation [2]. Lymphedema is the accumulation of lymph in soft tissues, intersititial spaces, and subcutaneous fat due to a fault in the lymphatic system [1–3].

In its clinical presentation, lymphedema is the swelling of
a body part, most commonly the lower limb, but it can also
occur in the arms, face, trunk, and external genitalia [3].
Lymphedema may be classified as primary or secondary,
based on the underlying etiology.

Lympedema caused by a primary abnormality or disease of
the lymph vessels or nodes is called primary lymphedema [1–3]. In the 1930’s, Allen suggest that primary lymphedema was caused by congential underdevelopment of lymph vessels [4]. Primary lymphedema has been further subdivided into three forms: congenital lymphedema, lymphedema praecox, and lymphedema tarda, depending on patient age at presentation. These conditions are most often sporadic, with no family history, and involve the lower extremity almost exclusively. These non-syndromic hereditary lymphedemas are categorized by patient age at onset, being either congenital (Milroy’s disease) or having onset in childhood or around puberty (Meige’s disease). Hereditary lymphedemas that are not associated with other malformations usually affect the lower limbs and are inherited in an autosomal dominant fashion [5,6]. Congenital lymphedema represents all forms that are clinically evident at birth and accounts for 10–25% of all primary lymphedema cases.

Secondary lympedema is more common and its causes are
surgery, irradiation, trauma, chronic wound, tumor, venous
disease, neurological disorders, filariasis, and infectious disorders. Lymphedema is found in both sexes, but females are affected twice as often as males. The lower extremity is involved three times more frequently than the upper extremity. Two thirds of patients have bilateral lymphedema, and this form may improve spontaneously with increasing age [2,3,6,7].

CASE REPORT

A 47-year-old woman was referred to the Cardiovascular
Surgery Department for evaluation of swelling of the left
leg. The patient complained of swelling which had started
16 years before, when she was pregnant. The patient was
treated on repeated occasions with medical and surgical
therapies. There was an incision scar on the left groin and
calf, but swelling of the leg was not stopped. On physical
examination, the left leg was markedly enlarged from groin
to foot. On the calf there were multiple ulcerative skin lesions. The circumference of the left calf was 106 cm and of the right calf 52 cm (Figure 1). The right leg were not involved. Laboratory results were unremarkable. Doppler findings of the leg were normal.

We performed a two-staged operation to prevent excessive
tissue and blood loss. In the first operation we excised diseased tissues on the posteromedial of the leg by the Charles procedure. The weight of excised tissue was 20 kg. The excised tissue also included deep muscular fascia, and the bared tissues were covered by a full-thickness skin graft using trimmed resected tissues. After the operation, the leg was immobilized with a long splint and elevated, and a lymphatic drainage system was applied. One year later, the patient came for the second operation and was operated by the Sistrunk procedure (subcutaneous excision) and we made a correction of the anterolateral portion of the leg.
During the operation, as much subcutaneous tissue and skin as possible was removed while attempting to maintain a viable skin flap and achieve successful closure. The weight of removed tissue was 11 kg. After the second operation, the left calf measurement was decreased to 57 cm (Figure 2).

There were no major complications after the operation
and the patient was discharged from hospital three weeks
postoperatively.

Physical therapy was started on the sixth postoperative day
and included compressive garments, manual lymphatic drainage, and meticulous skin care. We recommended physical therapy, bandaging, and compressive stocking during the three postoperative months. Recurrence did not appear at the postoperative follow-up period of one year.

DISCUSSION

Lymphedema is the result of the equilibrium between the
load to be cleared and the transport capacity of the clearing
system, the build-up of macromolecules and proteins leading
to an increase in oncotic pressure to produce more edema,
and in this way it also leads to fibrosis [1,2]. Drinker et al.
suggested that the fibrosis and increased interstitial connective tissue in a lymphedematous limb was the reaction to the excess protein and fibrinogen. Pillar suggested that lympedema is a progressive pathophysiological process in which the excess accumulation of protein and fluid stimulates infl ammation and macrophage activity [2]. The lymphatic walls undergo fibrosis, and fibrinoid thrombi accumulate within the lumen, obliterating much of the remaining lymph channels. As a results of that process, the affected limb or body part is markedly enlarged and its function improved.

The result of this inflammatory reaction is a change from
the initial pitting edema to the brawny non-pitting edema
characteristic of lymphedema. Swelling is usually the initial
symptom and seen as a pitting edema, while fibrosis is seen
with classical non-pitting signs. With time, skin changes are
seen over the affected area, the skin becomes hyperkeratotic, and turgor is increased [3]. Secondary lymphedema has an identifiable cause that destroys or renders inadequate the otherwise normal lymphatics [2,3].

Clinical staging has proven useful for the classifi cation of
lymphedema:


Stage 1: spontaneous, reversible, tissue swelling leaving indentations, negative or borderline Stemmer sign, no palpable fi brous tissue;
Stage 2: spontaneous, irreversible tissue swelling with moderate or pronounced fibrosis, indentations are difficult to produce, Stemmer sign is positive, lymphostatic dermatosis;
Stage 3: lymphostatic elephantiasis, usually with pronounced
skin alterations.

Severity is based on differences in limb volume and are assessed as minimal (<20% increase), moderate (20–30% increase), or severe (>40% increase) [8].

The diagnostic procedures of lymphedema include lymphangiography, lymphoscintigraphy, computerized tomography, magnetic resonance imaging, and Doppler ultrasonography.

Lymphoscintigraphy can be used to define anatomy and patency, evaluate the dynamics of flow and flow reversal, and determine the severity of obstruction [9,10]. The common differential diagnosis in patients with lower limb swelling is secondary lymphedema, venous diseases, and lipedema. Lymphedema can be confirmed by a lymphoscintigram, computed tomography, magnetic resonance imaging, or ultrasound [3].

Lymphedema treatment protocols includes non-surgical and
surgical management. Non-surgical management includes limb elevation, compression, manual physical decongestion, prompt treatment of infectious complications, pharmacotherapy, and diet and is usually only effective during the early stages of lymphedema [6,9]. Excisional surgery is indicated for patients with cases of advanced, chronic, and lymphedema unresponsive to physical therapy. There are two main surgical approaches: excisional procedures, where part or all of
the lymphedematous epifascial tissue is removed [11–13], and
microsurgical interventions, for the creation of lymphatico lymphatic, lymphatico-veno-lymphatic, lymphatico-venous, or lymph node-venous anastomoses [14,15]. Other surgical techniques include treatment with transferred omental pedicle and myocutaneous flap intertransposition. Suction techniques can also be helpful for the removal of excessive subcutaneous tissue and can be utilized in concert with skin excision [16,17]. With excisional surgery of the lower extremity, typically referred to as the Charles procedure, all the skin and subcutaneous tissues are excised. The defect is closed with a split-thickness or a full-thickness skin graft from the resected specimen or with an STSG from an uninvolved donor site [11]. In the buried dermal flap operation, the Thompson procedure, a portion of the subcutaneous lymphedematous tissue is resected beneath the flaps, the flap edge is de-epithelized, and the resulting dermal flap is buried into the underlying muscle compartment. The buried flap provides a physical barrier against deep fascia regeneration [12]. The staged excision of subcutaneous tissue was popularized by Homans and is thought to be safer and able to produce the desired functional results [18]. Servelle described a technique of total superfi cial lymphangiectomy which entailed a two-stage removal of lymph-edematous subcutaneous tissue and fascia [12].

Patients with chronic lymphedema have a 0.07–0.45% risk
of developing lymphangiosarcoma within 10 years [19–21].
Stewart Treves syndrome (lymphangiosarcoma) is a very rare
complication of lymphedema, especially after breast cancer
treatment, with a rate <0.5% [22]. This tumor is highly aggressive, requires radical amputation of the involved extremity, and has a very poor prognosis. Other complications of lymphedema include recurrent bouts of cellulitis and/or lymphangitis, deep venous thrombosis, severe functional impairment, cosmetic embarrassment, and necessary amputation. Complications following surgery are common and
include partial wound separation, seroma, hematoma, skin
necrosis, and exacerbation of foot or hand edema [8].

CONCLUSIONS

In conclusion, we recommend staged excisional surgery for
advanced and chronic lymphedema cases. Microsurgical procedures are usually performed either in patients with postsurgical lymphedema or in those with primary lymphedema who have proximal occlusion. Postinfl ammatory lymphedema, primary lymphedema with distal occlusion, and previously operated patients are not suitable for microsurgery.
Accordingly, we prefer staged excisional surgery. Staged
excisional procedures offer reliable long-term improvement and minimize postoperative complications. Their results are comparable to the other excisional techniques, but with a lower complications rate. Additionally, postoperative physiotherapy should be performed to support lymphatic drainage. Complex physical therapy (decongestive therapy, combined physiotherapy) is a routine physiotherapeutic approach in the postoperative period in our clinic. Additionally, intermittent pneumonic compression does effectively remove excess fluid from the extremity.

REFERENCES:

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3. Tiwari A, Cheng KS, Button M et al: Differential Diagnosis, Investigation,
and Current Treatment of Lower Limb Lymphedema. Arch Surg, 2003;
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4. Allen EV: Lymphoedema of extremities. Classification, etiology and differential diagnosis: a study of three hundred case. Arch Intern Med,
1934; 54: 606–24
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