Byung-Boong Lee and James Laredo

A 19 year old female was brought in to the Emergency Room (ER) in a “septic shock” condition with massively swollen bilateral lower limbs (Fig. 53.1).

This patient was well known to the ER staff for many years with recurrent episodes of systemic sepsis often triggered by local cellulitis and/or erysipelas involving one of her swollen limbs. The intervals between her sepsis got shorter lately and the control of her sepsis became more difficult, to manage.

Question 1

Which of the following would you deter in your first step towards managing the situation?

1.  Initiation of the differential diagnosis 2.  Resuscitation

3.  Blood cultures before the antibiotic administration

4.  Anticoagulation

5.  Thorough investigation on the cause of sepsis (Answer – 4)

The past history reveals that she was born with a swelling of the left lower leg, including her toes but did not receive any treatment. Before she reached her menarche she developed a similar swelling on her right side starting from the mid-thigh region downwards. Initially her limb swellings were relieved by nocturnal elevation but soon improvements diminished following recurrent local sepsis.

B.-B. Lee ( )

Department of Vascular Surgery, Georgetown University School of Medicine, Washington, DC,

USA

Fig. 53.1  The clinical photo shows 19 years old female in a bed-ridden condition at the ICU after successful resuscitation from the septic shock. Massively swollen bilateral lower extremities is due to the primary lymphedema in the end stage complicated with recurrent sepsis

Question 2

What is the most probable cause of her bilateral lower limb swellings?

1.  Chronic deep vein thrombosis

2.  Congenital vascular malformation, probably of lymphatic origin

3.  Early stage of cardiac failure

4.  Early stage of anasarca from congenital hypothyroidism

5.  Secondary chronic lymphedema, probably from filariasis infection (Answer – 2)

The patient neglected medical care until puberty, and her limb condition deteriorated steadily following repeated episodes of sepsis through the year. The pitting nature of her swelling was now replaced with a rubbery firm leg, which became massively swollen during the last few months.

Examination in the ER revealed extremely swollen bilateral limbs. There was a resolving patch of redness along the left buttock but no clear evidence of infection. A shallow decubitus ulcer (3.0 × 3.0 × 0.5 cm) was identified in right presacral region without evidence of invasive infection.

This ulcer recently developed lately when the patient became bed-ridden secondary to the debilitating swelling of her lower limbs (Fig. 53.1).

The patient was promptly resuscitated to stabilize her condition. Due to her labile vital signs however, the patient required admission to the intensive care unit for further management.

Question 3

What is the next stage of her management?

1.  Parenteral antibiotic administration

2.  Immediate application of compression bandages

3.  Immediate application of sequential pneumatic compression therapy

4.  Radical debridement of the decubitus ulcer

5.  Absolute bed rest under full anticoagulation (Answer – 1)

Following the control of systemic sepsis, the management of her limb swelling remained a challenge especially since she gained over 40 kg from 55 to 99 kg following abandoned therapy.

Question 4

What is the most appropriate way to reduce her massive swelling and improve her mobility?

1.  Vigorous application of high pressure compression therapy

2.  Weight control regimen with strict dietary control

3.  Immediate plan for the surgical excision of swollen tissue 4.  Timed initiation of complex decongestive therapy

5.  Angioplasty and/or stent insertion to iliac vein stenosis (Answer – 4)

Question 5

What is the most frequent and potentially serious risk involved in the management of massive edema?

1.  Acute tissue gangrene

2.  Acute pulmonary thromboembolism

3.  Acute pulmonary edema

4.  Acute limb paralysis

5.  Acute deep vein thrombosis (Answer – 3)

Following successful management of her crisis, further investigations and assessments of her swollen limbs were carried out to establish a long term care plan. A home maintenance care regimen was also prescribed.

Question 6

Which of the following tests would not be needed in general for diagnosis and assessment?

1.  Oil contrast lymphography/lymphangiography

2.  Duplex assessment of the deep venous system

3.  Volume measurements

4.  Radionuclide lymphoscintigraphy

5.  Magnetic Resonance Imaging study (Answer – 1)

Question 7

The aim of the investigations on the current condition include all of the following EXCEPT?

1.  Clinical and laboratory staging of the edematous limb 2.  Assessment of deep vein status of the lower extremity 3.  Selective investigation on the iliac vein stenosis/occlusion

4.  Assessment for the possibility of coexisting vascular malformation 5.  Patient compliance to maintain her care (Answer – 3)

Question 8

What is the most essential and reliable part of the therapy in general?

1.  Diet

2.  Compression bandage

3.  Exercise

4.  Bed rest with leg elevation

5.  Anticoagulation (Answer – 2)

Despite the rigorous home care regimen, her current limb condition continued to deteriorate. Physical therapy became increasingly difficult with very limited response. Her leg became much firmer and more frequent episodes of aborted and/or full blown cellulitis occurred through the year.

Question 9

What kind of the treatment can be instituted as a supplement to her current physical therapy?

1.  Mercury bath-combined microwave therapy

2.  Cross-femoral bypass surgery to relieve venous hypertension 3.  Thromboembolectomy of the iliac-femoral vein