Lymphedema

Question 1

A 50-year-old man is referred for consultation regarding surgical treatment of panniculus morbidus (abdominal elephantiasis). History includes a recent infection of the panniculus morbidus, resulting in protracted treatment in the intensive care unit. Current examination shows abdominal lipodystrophy with lymphedema and ulcerations. The patient has extreme difficulty with ambulation and is unable to care for his personal hygiene. Whichof the following is the most appropriate surgical management?
A) Abdominoplasty
B) Liposuction of the panniculus
C) Lymphaticovenous anastomosis
D) Panniculectomy and leaving the wound open for future skin grafting
E) Panniculectomy with primary closure

Answer 1

E) Panniculectomy with primary closure

Panniculus morbidus is a severe form of abdominal lipodystrophy with profound consequences. The condition prevents weight loss, as the patient cannot exercise. It prevents hygiene, leading to a profound odor, and ultimately results in intertrigo, cellulitis, and/or abdominal ulceration. The correct procedure to perform in the scenario described is a conservative panniculectomy with primary closure, although some authors have recently advocated using a negative pressurewound dressing in addition to partial primary closure.

A liposuction device, whether ultrasonic or traditional, would probably be dangerous in this situation, given the caliber of some of the blood vessels that can be present, as well as the occasional hernia that can be encountered. Performing more extensive procedures, such as rectus muscle plication, undermining of the flaps, and umbilical transposition, would be unnecessary and lead to more complications.

Question 2

Panniculus morbidus

Answer 2

Panniculus morbidus is a severe form of abdominal lipodystrophy with profound consequences. The condition prevents weight loss, as the patient cannot exercise. It prevents hygiene, leading to a profound odor, and ultimately results in intertrigo, cellulitis, and/or abdominal ulceration.

Question 3

Procedure for panniculus morbidus

Answer 3

Conservative panniculectomy with primary closure, although some authors have recently advocated using a negative pressurewound dressing in addition to partial primary closure.

Question 4

Why would liposuction for panniculus modbidus be contraindicated?

Answer 4

A liposuction device, whether ultrasonic or traditional, would probably be dangerous in this situation, given the caliber of some of the blood vessels that can be present, as well as the occasional hernia that can be encountered.

Question 5

A 45-year-old woman is evaluated because of a 6-year history of lymphedema of the lower extremities that causes chronic fatigue and swelling of both legs that impairs daily activities. Conservative treatment to control the swelling, including weight loss, compression garments, and daily intermittent pneumatic pump compression, was not successful. Physical examination of the left thigh shows thick, fibrotic, nonpitting tissues. The circumference of the left thigh is 10 cm larger than the right thigh. Which of the following is the most appropriate surgical treatment?
A) Buried dermal flaps
B) Microvascular lympholymphatic anastomoses
C) Omental transposition
D) Staged skin and subcutaneous excision
E) Total excision of all skin and subcutaneous tissue

Answer 5

D) Staged skin and subcutaneous excision

Staged skin and subcutaneous excision has become theoption of choice for many authors. This procedure is safe, reliable, and has shown the most consistent improvement with the lowest incidence of complications. Physiologic procedures, including omental transposition, buried dermal flaps, enteromesenteric bridging, lymphangioplasty, and microvascular lympholymphatic or lymphovenous anastomoses, have not shown favorable long-term results.

Question 6

Treatment for edema of the lower extremities

Answer 6

Staged skin and subcutaneous excision has become theoption of choice for many authors. This procedure is safe, reliable, and has shown the most consistent improvement with the lowest incidence of complications. Physiologic procedures, including omental transposition, buried dermal flaps, enteromesenteric bridging, lymphangioplasty, and microvascular lympholymphatic or lymphovenous anastomoses, have not shown favorable long-term results.

Question 7

A 46-year-old man comes to the office because of a 10-year history of a painless mass in the mons (overlying skin is thickened and displays the typical peau d’orange(orange skin) appearance of congested dermal lymphatics). He reports that it has enlarged gradually and prevents him from conducting daily activities. He has had episodes of cellulitis in the skin overlying the mass several times a year for the past 5 years. BMI is 51 kg/m2. Which of the following is the most appropriate management?
A ) Elevation and compression
B ) Incision and drainage
C ) Resection and negative pressure wound therapy
D ) Suction lipectomy and Charles procedure
E ) Weight reduction and physical therapy

Answer 7

C ) Resection and negative pressure wound therapy

Massive localized lymphedema (MLL) is an emerging complication of morbid obesity. Also known as lymphedema of obesity, MLL is usually a benign overgrowth of soft tissue

Incision and drainage is performed for wound infection. Weight reduction, physical therapy, elevation, and compression are allconservative management options in lymphedema and would not be appropriate in a patient who is bed-bound as a result of MLL and has recurrent cellulitic episodes. Suction lipectomy would not be effective for the patient described and does not address skinexcess.

Question 8

Massive localized lymphedema

Answer 8

Massive localized lymphedema (MLL) is an emerging complication of morbid obesity. Also known as lymphedema of obesity, MLL is usually a benign overgrowth of soft tissue

Question 9

Etiology of massive localized lymphedema

Answer 9

MLL is a form of secondary lymphedema; it represents an acquired dysfunction of otherwise normal lymphatics.
Secondary lymphedema has an identifiable cause - in this case, obesity - that destroys or renders inadequate the otherwise normal lymphatics.

Question 10

Most common cause of secondary lyphedema

Answer 10

Worldwide, the most common cause is filariasis, the direct infestation of lymph nodes by the parasite Wuchereria bancrofti.

Question 11

Causes of secondary lymphedema

Answer 11

Worldwide, the most common cause is filariasis, the direct infestation of lymph nodes by the parasite Wuchereria bancrofti. Other causes include vein stripping, peripheral vascular surgery, oncologic surgery, radiation, infection, and tumor invasion or compression.

Question 12

Surgical treatment of massive localized lymphedema

Answer 12

Surgical treatment is palliative, not curative, and it does not obviate the need for continued conservative therapy. Resection is indicated for restoration of mobility, prevention of recurrent infections, or if there is a question of malignancy. Rarely, chronic lymphedema may predispose to cutaneous angiosarcoma.

Question 13

Rare predisposition of chronic lymphedema

Answer 13

Rarely, chronic lymphedema may predispose to cutaneous angiosarcoma.

Question 14

Charles procedure

Answer 14

The Charles procedure is a radical surgery performed for lower extremity lymphedema, where fascial excision of skin and soft tissue is performed and then skin from the resected specimen is used for immediate autografting.

Question 15

A 28-year-old woman is evaluated because of a 2-month history of progressive swelling in the left upper extremity. Physical examination shows edema extending from the hand to the arm. Diameter of the left upper limb is larger than that of the right upper limb. Ultrasonography shows no venous obstruction, and CT scan is negative for a tumor or mass. Which of the following is the most appropriate next step in management? 
A ) Administration of a diuretic
B ) Elevation and compression
C ) Liposuction
D ) Microlymphatic anastomosis
E ) Placement of buried dermal flap

Answer 15

B ) Elevation and compression

The most appropriate next step in management is elevation and compression.Lymphedema is caused by inadequate clearance of fluid from the interstitial space, resulting in buildup of fluid and protein. The classic finding involves edema, beginning in the distal extremity.

In general, simpler methods are recommended for patients with newly diagnosed lymphedema, such as elevation and a compression garment, with or without manual lymphatic drainage or massage therapy. Pneumatic compression pumps have also been used as an adjunct to compression and elevation. Surgery is generally recommended for patients who have failed conservative therapy.

Question 16

Diuretics and lymphedema

Answer 16

Ineffective

Question 17

Buried dermal flap for lymphedema

Answer 17

The placement of a buried dermal flap has been performed in an attempt to reestablish lymphatic communications. It is generally believed that the primary benefit is excision of the tissue, rather than the flap component

Question 18

Excision of subcutaneous tissue and skin grafting for lymphedematous tissues

Answer 18

Excision of subcutaneous tissue and skin grafting has been described, with the goal of removing lymphedematous tissues. However, skin graft problems and poor cosmetic outcomes may result, as well as edema that occur distal to the area of excision

Question 19

A 40-year-old woman comes to the office because of a 2-year history of lymphedema of the lower extremity. She has been unsuccessful in her attempts to lose weight through diet. BMI is 32 kg/m2. Physical examination shows asymmetry of the left lower extremity with enlarged circumference of the thigh and calf. Which of the following is the most appropriate treatment of this patient’s lymphedema?
A ) Administration of a benzopyrene
B ) Administration of a diuretic
C ) Application of Unna boots
D ) Caloric restriction
E ) Intermittent pneumatic pump compression therapy

Answer 19

E ) Intermittent pneumatic pump compression therapy

Intermittent pneumatic pump compression therapy should be instituted onan outpatient basis and/or in the home. The mainstay of treatment is medical; this includes meticulous hygiene and regular inspection, as well as encouraging patients to lose weight, avoid even minor trauma, and avoid constrictive clothing and elevation of the affected extremity.

Benzopyrenes, including flavonoids and coumarin, have become a useful adjuvant in other countries but are currently not available for clinical use in the United States. These drugs bind to accumulated interstitial proteins, inducing macrophage phagocytosis and proteolysis.

Unna boots are used for venous stasis ulcers of the lower extremity

Question 20

Unna boots are used for:

Answer 20

Unna boots are used for venous stasis ulcers of the lower extremity

Question 21

A 50-year-old woman has lymphedema of the right leg. Height is 5 ft 6 in (168 cm); weight is 250 lb (113 kg). Which of the following is the LEAST appropriate medical treatment of the lymphedema?
A ) Administration of diuretics
B ) Compression garment therapy
C ) Decompressive physical massage
D ) Intermittent pneumatic pump compression therapy
E ) Recommendation of weight loss

Answer 21

A ) Administration of diuretics

Diuretics play no role in the treatment of lymphedema. The mainstay of treatment is medical.

Question 22

Benzopyrenes vs lymphedema

Answer 22

Benzopyrenes, including flavonoid and coumarin, have become a useful adjuvant in other countries but are currently not available for clinical use in the United States. These drugs bind to accumulated interstitial proteins, inducing macrophage phagocytosis and proteolysis

Question 23

A 45-year-old woman with a six-year history of chronic lymphedema of the entire lower extremity comes to the office for consultation about surgical treatment. She says her legs always feel tired. Conservative management, including compression garment and pump compression therapy, has been unsuccessful. The size of the extremity impairs her activities of daily living. Which of the following is the most appropriate surgical management?
A ) Microvascular lympholymphatic anastomoses
B ) Microvascular lymphovenous anastomoses
C ) Omental transposition
D ) Staged excision of all excess skin and subcutaneous tissue
E ) Suction-assisted lipectomy

Answer 23

D ) Staged excision of all excess skin and subcutaneous tissue

Staged excision of all excess skin and subcutaneous tissue has become the option of choice for many authors. This procedure has shown the most consistent improvement with the lowest incidence of complications.

Physiologic procedures, including omental transposition, buried dermal flaps, enteromesenteric bridging, lymphangioplasty, and microvascular lympholymphatic or lymphovenous anastomoses, have not shown favorable long-term results. Suction-assisted lipectomy does notreduce the skin envelope, and the lymphedema often rapidly recurs

Question 24

A 14-year-old boy is brought to the emergency department by his parents one hour after he had sudden onset of swelling, tenderness, and tension of a lymphatic malformation on the right leg. The boy’s parents say that the lesion has been present since 3 years of age and has been enlarging since that time. Which of the following is the most appropriate initial management?
(A)Administration of an antibiotic
(B)Compression of the entire leg
(C)Lymphovenous shunting
(D)Pulsed-dye laser therapy
(E)Surgical decompression

Answer 24

(A)Administration of an antibiotic

In a patient with a large lymphatic malformation who presents with sudden expansion of a lesion along with tenderness and tenseness, the most likely diagnosis is infection. Although prophylactic antibiotics are generally not indicated for patients with lymphatic malformations, it is wise to prescribe a broad-spectrum antibiotic with instructions to administer it and see a physician at the first signs of infection.

Question 25

A 1-year-old female infant is evaluated for a 12 × 12-cm macrocystic lymphatic malformation of the posterior trunk that has been enlarging and causing infections for the past 6 months. The lesion involves the skin, subcutaneous tissue, and muscle. Which of the following is the most appropriate next step in management?
A) Embolization
B) Prednisolone therapy
C) Propranolol therapy
D) Resection
E) Sclerotherapy

Answer 25

E) Sclerotherapy

First-line intervention for a large, symptomatic, macrocystic, lymphatic malformation is sclerotherapy. Sclerotherapy is the injection of an anti-inflammatory substance (e.g., doxycycline) into a lesion, which causes endothelial damage, fibrosis, and shrinkage of the malformation. Sclerotherapy is more effective and less morbid than resection. Propranolol and prednisolone are treatment options for a problematic infantile hemangioma, but they have no efficacy for vascular malformations. Embolization is first-line intervention for an arteriovenous malformation, and is not a treatment option for lymphatic malformation. Resection is second-line therapy for a large macrocystic, lymphatic malformation. Extirpation can cause significant morbidity (i.e., bleeding, nerve injury, infection, wound breakdown). In addition, excision leaves a cutaneous scar, and recurrence is common because a lymphatic malformation can rarely be completely removed.

Question 26

A 16-year-old girl with no history of international travel is brought to the office because of gradually increasing size of the feet, swelling of the legs, and loss of definition of the calves. Her parents say they first noticed that the soft tissue of her ankles was compressed by her shoes 2 years ago. Family history includes foot, leg, and ankle swelling. Physical examination shows relative effacement of the knee-calf and calf-ankle junctions. Pitting edema is noted. Which of the following is the most likely diagnosis?
A) Filariasis
B) Lymphedema praecox
C) Lymphedema tarda
D) Milroy disease
E) Secondary lymphedema

Answer 26

B) Lymphedema praecox

Lymphedema praecox is the most likely form of hereditary lymphedema. It typically manifests at puberty and has a chronic course. The scenario describes a classic case of lymphedema praecox—bilaterality, occurring sometime around puberty.

Filariasis is the most common cause of lymphedema worldwide, but it is not endemic to nontropical countries. Wuchereria bancrofti, Brugia malayi, Brugia timori, Onchocerca volvulus, and Loa loa are parasites that cause filariasis.

Lymphedema tarda arises in middle age by definition. It is another form of primary lymphedema.

Milroy disease is not the appropriate response. This is a congenital form of lymphedema, usually present at birth or manifesting shortly after birth. It often has asymmetries to the symptoms. The scenario described would not be typical of Milroy disease.

Secondary lymphedema could theoretically occur in a 16-year-old girl, bilaterally and symmetrically with no additional symptoms or examination findings, but it is very unlikely. Malignancies that cause secondary lymphedema would also cause other findings, particularly if cancer had been present for 2 years or more. The child has no history of other trauma, insult, or infection at the groin. Also, the patient described has a family history of leg swelling, which is strongly suggestive of some form of primary lymphedema.

Question 27

A 35-year-old woman is evaluated because of a 15-year history of bilateral lower extremity lymphedema. She reports no trauma that led to the lymphedema. Physical examination shows extensive bilateral non-pitting edema from the pelvis to the knees. Ultrasonography and MRI show adipose tissue hypertrophy and diffuse soft-tissue edema. Which of the following is the most appropriate treatment for this patient?
A) Furosemide and compression therapy
B) Furosemide and subcutaneous drain placement
C) High-dose furosemide therapy
D) Subcutaneous drain placement
E) Suction-assisted lipectomy and compression garments

Answer 27

E) Suction-assisted lipectomy and compression garments

The patient described has severe primary lower extremity lymphedema. Primary lymphedema is the result of an absence or abnormality of the lymphatic system, and it is characterized according to the age of onset (e.g., birth, puberty, early adulthood). Mild or moderate lymphedema can be treated with compression garments, massage therapy, or manual lymphatic drainage. However, severe lymphedema can benefit from surgical intervention. Surgical intervention (i.e., suction-assisted lipectomy) is generally reserved for the most severe cases because of the risks of the procedure, which include multiple stages, wound dehiscence, scarring, and thromboemolism. Liposuction is typically followed by the use of compression garments. Therefore, suction-assisted lipectomy followed by the use of compression garments is the correct answer since this option involves surgical intervention.

Question 28

A 40-year-old woman is evaluated because of a 1-year history of lymphedema of the right lower extremity. BMI is 28 kg/m2. Physical examination shows asymmetry of the right lower extremity and increased circumference of the thigh and calf compared with the left lower extremity. Which of the following is the most appropriate initial treatment?
A) Administration of a diuretic
B) Excision of the affected skin and subcutaneous tissue
C) Formation of a diet and exercise regimen
D) Intermittent pneumatic pump compression
E) Microvascular lympholymphatic anastomosis

Answer 28

D) Intermittent pneumatic pump compression

Intermittent pneumatic pump compression therapy should be instituted. The mainstay of treatment is medical, including meticulous hygiene and regular inspection. Patients should be encouraged to lose weight, avoid even minor trauma, avoid constrictive clothing, and elevate the affected extremity. All patients should also use compression garments continuously during the day.

Lymphedema is the accumulation of protein-rich fluid in the interstitial space caused by lymphatic dysfunction. Lymphatic flow must be decreased by 80% before interstitial fluid begins to accumulate as compensatory mechanisms such as increased macrophage activity and spontaneous lymphovenous anastomoses are exhausted. Increased concentrations of interstitial protein cause inflammation and fibrosis, leading to a cycle of further damage to lymphatics, worsening inflammation, and an enlarged extremity. Chronic lymphedema leads to deposition of fat and fibrous tissue.

The classic finding involves edema, beginning in the distal extremity. Measurements of limb circumference at multiple levels may show enlargement compared with the contralateral side. Other conditions may cause peripheral edema, but in general, bilateral lower extremity edema is caused by systemic disease. Unilateral edema is more likely a result of venous insufficiency or lymphedema.

Lymphedema is a chronic disease that is difficult to manage and treat, and there is no medical or surgical cure. In general, simpler methods are recommended for patients with newly diagnosed lymphedema, such as in this patient. Surgery is generally recommended for patients who have failed conservative therapy.

Diuretics play no role in the treatment of lymphedema.

Physiologic procedures, such as microvascular lympholymphatic or lymphovenous anastomoses, have not shown favorable long-term results.

Question 29

A 45-year-old woman treated with axillary dissection and mastectomy for Stage II breast cancer has onset of lymphedema of the right upper extremity. Conservative management with compression garments and lymphatic massage has not led to adequate resolution. Which of the following procedures is most appropriate to address the lymphedema in this patient?
A) Brachioplasty
B) Charles procedure
C) Homan procedure
D) Liposuction
E) Lymphatico-venous bypass

Answer 29

D) Liposuction

Liposuction has been reliably shown to improve lymphedema post-breast cancer therapy.

Lymphatico-venous bypass is a procedure that is gaining popularity, but the literature is still controversial with regard to its efficacy. The Homan and Charles procedures have become less popular due to drastically increased morbidity compared with liposuction.

Brachioplasty is a technique to address excess skin of the upper arm and will not improve lymphedema.

Resection approach, or debulking, involves surgical excision of subcutaneous tissue, which may or may not include excision of the overlying skin. Charles first described this resection method in 1912, and variations of this technique of radical excision of the subcutaneous tissue and primary or delayed skin grafting are still used today. Debulking procedures are not designed to directly address lymphatic vessel dysfunction but instead provide improved comfort by removing redundant skin and subcutaneous tissues.

Homan described and popularized subcutaneous excision beneath flaps. This may be performed in two stages on a single extremity. A medial resection may be performed first, as more tissue can be removed from the medial than from the lateral aspect of both the arm and the leg. If necessary, a lateral procedure may be performed 3 months after the initial operation. If bilateral disease is present, the operation may be performed on both involved limbs during the initial procedure, although in cases of massive edema, the prolonged operative time and excessive blood loss mitigate against this approach.

Question 30

A 345-lb (156-kg), 5-ft 1-in (155-cm), 59-year-old woman comes to the office because of the condition of the lower extremities in the photograph shown. BMI is 65 kg/m2. She says she has tried “everything” and “nothing seems to work.” She states that the left leg is worse than right. The condition first appeared over 20 years ago after she underwent surgery of the left leg. She was hospitalized 10 times for cellulitis/infection in the past 1 year. She refuses compression therapy because it has become too painful. Which of the following are the most likely diagnosis and most appropriate management?
A) Primary lymphedema; medical management
B) Primary lymphedema; surgical management
C) Secondary lymphedema; medical management
D) Secondary lymphedema; surgical management

Answer 30

D) Secondary lymphedema; surgical management

The morbidly obese patient described suffers from severe secondary lymphedema. Assuming she doesn’t have any contraindications to an operative procedure, she has failed medical management and should be offered surgery.

Lower extremity lymphedema can be considered either primary or secondary. Primary lymphedema can be congenital, praecox, or tarda based on the age at presentation. Secondary lymphedema can be due to either lymphatic obstruction (due to cancer, infection, or radiation) or lymphatic interruption (due to groin surgery or lymph node excision).

By far, the most common approach to lymphedema (either primary or secondary) is medical management. However, the most common indication for surgery is failure of medical management.

There are a variety of surgical options. These include procedures to improve lymphatic flow and procedures to debulk the affected tissue.

Question 31

A 52-year-old man comes for evaluation of a 2-year history of gradual swelling of the right lower extremity. He says he first noticed symptoms after returning from a year working in Africa. Physical examination shows pitting edema to the level of the knee. The skin over the toes cannot be tented. Results of thyroid function studies are within the reference ranges. Which of the following is the most likely diagnosis?
A ) Deep venous thrombosis
B ) Lymphedema
C ) Myxedema
D ) Venous insufficiency

Answer 31

B ) Lymphedema

The most appropriate diagnosis is lymphedema.

Lymphedema is caused by inadequate clearance of fluid from the interstitial space, resulting in buildup of fluid and protein. The classic finding involves pitting edema beginning in the distal extremity. Measurements of extremity circumference at multiple levels may show enlargement compared with the contralateral side. Other conditions may cause peripheral edema, but, in general, bilateral lower extremity edema is due to systemic disease, while unilateral edema is more likely a result of venous insufficiency or lymphedema. Worldwide prevalence of lymphedema is estimated at 90 million cases, with approximately 90% of these attributed to filariasis. This is relatively rare in the United States, however, and surgery or radiation therapy is a much more likely etiology for secondary lymphedema in this country. A history of residence in an endemic area, such as Africa, may increase the suspicion of lymphedema resulting from filariasis.

Diagnosis of lymphedema is typically made through history and physical examination. Classic signs include peau d’orange changes in skin, inability to tent the skin over the toes (Stemmer sign), and blunted appearance of the digits.

Deep venous thrombosis can lead to lower extremity swelling but would have a faster onset and more rapid time course. Generally, this would be unilateral in nature.

Myxedema is typically associated with thyroid disease and can be distinguished from lymphedema by finding dry and roughened skin, dry thinning hair, reduced sweat production, and yellowish skin.

Venous insufficiency can also result in extremity swelling, but it is usually associated with skin changes, pruritus, and hemosiderin deposits. Patients may also have symptoms of varicosities and ulceration. Additionally, the Stemmer sign is negative in venous insufficiency.