4.  Reconstructive surgery to restore lymph transport

5.  Excisional surgery to remove overgrown fibrosclerotic tissue (Answer – 5)

Treatment failure, despite a full year of maximum conventional care, was accepted as the indication of additional treatment by the multidisciplinary team.

Question 10

What is NOT an essential part for the treatment in such an advanced stage?

1.  Encouraging patient compliance to maintain physical therapy 2.  Multidisciplinary team approach to improve quality of life

3.  Repeated assessments of lymphatic function with lymphography 4.  Active mobilization for better social and psychological rehabilitation 5.  Vigorous control and prevention of infection (Answer – 3)

53.1  Commentary

The clinical history of this young lady illustrates how primary lymphedema progresses when timely appropriate care is neglected.

Her left lower limb swelling since the birth should have been recognized as a primary lymphedema until proven otherwise. A basic assessment of her clinical appearance should not have been delayed especially after the contralateral limb went down the same path of the swelling as a ‘precox’ type.1–8

It would have ideal for basic investigations to confirm primary lymphedema when both limbs got involved. Focused management combined with active prevention of infection could then have been started at a much earlier point.

With early aggressive management, her lymphedema condition would have not reached such an advanced stage so quickly with suchlife-threatening sepsis.

If lymphedema advances to a late stage, a simple edematous condition of the soft tissue becomes a fibrosclerotic one. This will then resist conventional treatments such as manual lymphaticdrainage(MLD)-basedcomplexdecongestivetherapy(CDT).9–14 hardenedlocal tissue becomes a harbinger for infection which often advances to systemic sepsis.

Sepsis is the most serious complication as it is a potentially life-threatening. This often leads a vicious cycle with more tissue damage and subsequent vulnerability condition to recurrent sepsis/infection.15–18

This young lady is a typical example of advanced lymphedema (Fig. 53.1).

Neglect carewithout regularCDT acceleratedthedeterioration of her tissues provoking local sepsis and septic shock prior to her emergency admission

Prompt management of septic shock is warranted with appropriate resuscitation to stabilizehervitalsigns.Antibioticsshouldbegivenassoonaspossibleafterthebloodculture specimenisobtainedandcontinuedparenterallyuntilsuitableselectionofculture-sensitive

antibiotics. Once the patient condition is stabilized, the diagnosis of her primary condition should be initiated. For an appropriate differential diagnosis on such limb swellings, not only the local causes but also the regional and systemic causes should be included, especially if there is any doubt on currently established diagnosis.

In addition, a thorough investigation on the cause of sepsis should be repeated (e.g., fungal infection: tinea pedis). [Q1: 4]

Limbswellingsincebirthalongtheleftlowerlegandtoesandsubsequentlyontheright lower limb, gives ample evidence for primary lymphedema as her diagnosis. Nevertheless the causes of secondary lymphedema should also be ruled out regardless of age of onset.

Primary lymphedema represents a “truncular” type of lymphatic malformation (LM)19– 22 since the majority have associated congenital structural abnormalities of the lymphatic system (e.g., aplasia, hypoplasia, hyperplasia).

LMs are a common form of congenital vascular malformations (CVMs)23–26 either as an independent (predominant) lesion or as a combined condition with other CVMs: venous malformations (VMs),27–30 arterio-venous malformations (AVMs),31–34 and/or capillary malformations (CMs).

The diagnosis and management of primary lymphedema should therefore consider such coexistence with other CVMs, including the extratruncular LM (e.g., Klippel-Trenaunay Syndrome)35–38 (Fig. 53.2).

A basic understanding on the LM as a CVM, including its embryological background is required in order to lead appropriate investigations on primary lymphedema.

Like other CVMs, these LMs are also classified into two different groups39–41 depending upon the embryological stage of developmental arrest: extratruncular and truncular LM’s.

Extratruncular LMs are also known as “lymphangiomata” – and they are embryonic tissue remnants as a result of developmental arrest/defect occurring in the “earlier” stage of lymphangiogenesis. Mesenchymal cell characteristics are maintained with the evolutionary potential to grow when stimulated (Fig. 53.3).

In contrast truncular lesions are a result of developmental arrest/defect at the “later” stage of lymphangiogenesis and lack these critical embryonic characteristics.42,43

Following stabilization from septic shock, the treatment of sepsis should start with parenteral antibiotic administration while searching for the source of the infection. Compression therapy with MLD-based CDT or a pneumatic compression device is contraindicated until the sepsis is under full control. Early ambulation is recommended to control decubitus development. [Q3: 1]

Vigorous application of high pressure compression therapy should be deferred until the cardiovascular system is fully stabilized. CDT is under close observation when the overall cardiovascular condition can afford the additional loading of mobilized edema fluid. [Q4: 4] Rapidfluidmobilizationfromamassivelyswollenlimbisdangeroussoonaftersepticshock. If the forceful evacuation of fluid from the interstitialspace is too effective, massive influx into

the intravascular space will accompany a high risk of acute pulmonary edema. [Q5:3]

Inordertoprovideanappropriatetreatmentstrategyforlongtermcare,afullassessment canbeachievedbasedonacombinationofbasicnon-toless-invasivetests44–46:Radionuclide lymphoscintigraphy (LSG),47–49 Duplex ultrasonographic assessment,50–52 Limb volume measurement,53–55 and Magnetic Resonance Imaging (MRI) study.56–58 Although the MRI is NOT essential for the investigation of primary lymphedema caused by the truncular LM

Fig. 53.2  (continued)

c

d

itself, the MRI may give not only additional information on the lymphedema status as well as coexisting CVMs like extratruncular VM’s and/or LM’s. [Q6: 1]

Oil contrast lymphography59,60 is invasive and not needed for the diagnosis. Due to the risk of further damaging the lymph vessels it’s use is now strictly limited as a special indication for the selection of surgical reconstruction candidates.

Suchinvestigationstoprovidethecriticalinformationneededtosetupaadequatehome maintenance care regimen following discharge from acute care.

Accurate information on the clinical and laboratory stage of the lymphedema46,61 is important for the selection of the right therapy. Abnormalities of the deep venous system would affect the response to therapy. Coexisting CVMs may also give a profound impact to the overall management.

Finally, an accurate evaluation on self-motivation to maintain care is absolutely essential since the life-time commitment to CDT based home maintenance therapy is totally depending on patient compliance.18,44 [Q7: 3]

Fig. 53.3  Clinical photo (a) shows diffuse swelling along the upper thigh extended to the groin, which is due to the extratruncular LM, different from the truncular LM causing the primary lymphedema. This extratruncular LM, often called as “lymphangioma,” is the outcome of developmental arrest/defect from the “early” stage of lymphangiogenesis. Therefore, it possesses a mesenchymal cell characteristic to grow when the condition should meet (e.g., hormone, menarche, pregnancy, trauma, surgery). As shown in MRI (b), this extratruncular lesion in upper thigh is extended to the pelvic cavity affecting entire pelvic soft tissue and the left lower limb lesion/swelling is a tip of the iceberg. The lesion is now complicated with the lymphatic leakage and sepsis

CDT9–14 remains a main stay of contemporary treatment of chronic lymphedema although it does not cure the condition. It is the most effective means to prevent the progress of the condition. Among various components of CDT, MLD is the most essential part together with compression bandage.62,63 MLD is a physiological means to stimulate paralyzed lymph vessels to restore peristalsis and relieve lymph stasis. Although MLD lacks theoretical evidence, it is now accepted as an effective therapy in the early stages of lymphedema (Fig. 53.4).

Incontrastthesequentialpneumaticcompression-therapy(SIPC)64–66 remainscontroversial due to risk of selective transfer of the fluid component out of the interstitial tissue leaving the protein component of the lymph behind to precipitate progressive tissue damage.

Among the many different components of CDT, compression bandage therapy remains the most effective and proven component. These recommendations are based on the grading according to scientific evidence,67 where compression therapy belongs to 1C or 2A. 1C

d

In advanced lymphedema however, there are no more salvageable lymphatic vessels remaining and therefore, it is too late for reconstructive surgery.

Since overgrown fibrosclerotic tissue is a known harbinger for t recurrent sepsis (e.g., cellulitis/erysipelas) (Fig. 53.5). Excisional surgery18,44,75 and 76 would improve not only the efficacy of CDT but also the overall risk of sepsis thereby improving quality of life. Although surgery remains as a valid option to assist CDT, current evidence-based recommendations still classify this as 2C (Table 53.2).

Liposuction77,78 obliterates the epifascial compartment by a selective removal of excessive adipose tissue. This procedure is not indicated for advanced lymphedema. In end stage lympedema entire tissues become fibrosclerotic with very limited fat tissue available for liposuction.

Therefore, excisional surgery remains the only viable option among the various surgical treatment modalities in end stage disease. All candidates for the excisional surgery, require mandatory postoperative CDT with a life-time. The guarantee of satisfactory outcome of the surgery should not be expected without appropriate postoperative CDT.18,44

[Q9: 5]

This patient met the indication for excisional surgery in order to improve the efficacy of CDT, this followed the documented failure of CDT alone despite maximum care for 2

560 B.-B. Lee and J. Laredo

Table 53.1  Guidelines 6.3.0 of the American venous forum on lymphedema: medical and physical therapy

infection we recommend antibiotics with superior coverage of Grampositive cocci, particularly streptococci, at full strength for 1 week/ month, Examples include cephalexin, penicillin, clindamycin, cefadroxil

years. A modified Homan-Auchincloss procedure79,80 was implemented to remove, the entire skin and soft tissues including the muscle fascia, to facilitate lymph absorption through the deep system. An excellent surgical outcome was maintained with a postoperative CDT regimen during the follow up period of 4 years (Fig. 53.6).

A multidisciplinary team approach should always be organized to support surgical therapy. This should improve quality of life provided patient compliance is good enough for maintenance CDT.

Active mobilization for better social and psychological rehabilitation should accompany CDT in order to provide an incentive to the patient to remain on therapy.

Vigorouscontrolandpreventionofinfectionshouldalwaysremaintheultimategoalfor end stage lymphedema. [Q10: 3]

a

b

Fig. 53.6  (a) photo illustrates the magnitude of the excisional surgery to remove entire soft tissue together with the skin, which all became sclerotic; Homan-Auchincloss technique was modified and excision was further extended to remove the muscle fascia as well to promote the absorption of the lymph by the deep lymphatic system. (b) photo demonstrates the surgical specimen showing the staging excision for the optimum amount of the tissue removal. Clinical photo (c) shows clinical outcome of the surgery 6 months later, achieving the goal of effective mobilization from the bed and increased efficacy of the postoperative CDT. This approach delivered improved quality of life with better social/physical/ psychological adaptation