Lymphedema / venous Inssuficiency

Question 1

A 19-year-old female with has a history of gradually increasing size of her feet, swelling and loss of definition of the calf of the right leg. She started noticing these changes 2 years ago. She has not traveled outside the United States and has not had surgery. Physical examination shows relative effacement of the knee-calf and calf-ankle junctions and positive Stemmer sign. Which of the following statement is true regarding her most likely diagnosis?
A) Meige’s disease is a hereditary form of this type of lymphedema
B) Milroy’s disease is a hereditary form of this type of lymphedema
C) Males are more commonly affected than females
D) Wuchereria bancrofti has been implicated in the pathogenesis
E) It is caused by a mutation in the VEGFR-3 gene

Answer 1

Correct answer is option A.

Lymphedema is classified as primary or secondary. Primary lymphedema results from developmental abnormalities of the lymphatic system and it is considered congenital when the disease develops before one year of age.

This patient likely has Lymphedema Preacox, which is the most common type of lymphedema. It presents in 60% to 80% of cases, affects females more frequently than males (4:1 ratio), and can be bilateral but typically involves only one extremity in 70% of cases.

Meige’s disease is a hereditary form of lymphedema praecox that manifests at puberty.

Milroy’s disease is a hereditary form of lymphedema congenita and it is caused by a mutation in the VEGFR-3 gene.

Lymphedema tarda only comprises 10% of cases, and it presents after the age of 35.

Secondary lymphedema results from obstruction or injury of the normal lymphatic system from a defined disease process. Filariasis due to Wuchereria bancrofti is the most common cause worldwide.

Question 2

A 22-month-old female infant presents with a large mixed micro- and macrocystic lymphatic malformation of the right lower extremity. A photograph is shown. Medical history includes two rounds of sclerotherapy for the macrocystic component at 6 and 12 months of age and repeated infections, requiring a long hospital stay for intravenous antibiotic therapy 4 months ago. Which of the following is the most appropriate therapy for this patient?

A) Amputation
B) Embolization
C) Power-assisted liposuction
D) Repeated sclerotherapy
E) Surgical debulking

Answer 2

The correct response is Option E.

This 22-month-old female has undergone treatment of the macro cystic component of the lymphatic malformation and continues to present with complication secondary to the disease. In addition to difficulty of mobilization, fitting of regular clothes, nutrition problems due to repeated infections, there is also significant life threatening risk of severe infection. Even though compression garment and repeated sclerotherapy are potential options, there will be no control of the disease. The best next step is surgical debulking. Embolization is not appropriate for a lymphatic malformation, and amputation is not indicated at this time. Power-assisted liposuction would be indicated for lymphedema, not for a lymphatic malformation.

Question 3

A 45-year-old woman is referred to the lymphedema center for evaluation of bilateral lymphedema of the lower extremities. The edema has been present for at least 5 years. She is morbidly obese with a BMI of 58 kg/m2, has well-controlled diabetes, and no history of operations or trauma to the lower extremities. She has seen a lymphedema specialist for ongoing conservative management and continues to wrap and elevate her legs with reasonable compliance. Despite this, she feels that the increase in size, heaviness, and fatiguability of her legs inhibits her activity level. The patient has enlarged legs and a positive Stemmer sign. Bilateral lower extremity lymphoscintigraphy demonstrates delayed radiotracer transit to the groins with dermal backflow. Which of the following is the most appropriate initial surgical option for this patient?

A) Bariatric surgery
B) Combined lymph node transfer and lymphovenous bypasses
C) Liposuction
D) Lymphovenous bypasses
E) Vascularized lymph node transfer

Answer 3

The correct response is Option A.

In the evolving area of surgical management of lymphedema, one difficult set of patients is the morbidly obese. In these patients, there is often a component of lymphedema in the bilateral lower extremities, abdominal pannus, or development of focal areas of massive localized lymphedema in the thighs or genitals. A positive Stemmer sign indicates an inability to pinch the base of the dorsum of the second toe. On the basis of her physical examination and lymphoscintigraphy results, this patient has lymphedema of her bilateral lower extremities. Development of lymphedema in the super morbidly obese can occur above a BMI of 50 kg/m2, and is likely above a BMI of 60 kg/m2. It has been found that the lymphedema may be reversible in up to 50% of these patients with weight loss to a point below a BMI of 50 kg/m2. If the patient has not been able to lose weight through nonsurgical interventions, the most appropriate next step is referral to a bariatric center for possible weight loss surgery. While the other options are all considerations for patients with lymphedema at various stages, the most appropriate initial treatment for this patient is weight loss surgery.

Question 4

Which of the following is the most common infectious cause of secondary lymphedema worldwide?

(A) Actinomyces israelii
(B) Candida albicans
(C) Coccidioides immitis
(D) Mycobacterium tuberculosis
(E) Wuchereria bancrofti

Answer 4

The correct response is Option E.

In the United States, secondary lymphedema is most likely to occur as a complication of surgery, radiation therapy, or malignant tumors. Infectious causes are more common worldwide; the most common is infection with Wuchereria bancrofti organisms, which directly invade the lymph nodes. Filariasis has been shown to be the most common noninfectious cause of secondary lymphedema worldwide.

Actinomyces israelii typically produces infection in immunocompromised patients. Although this organism is normally found within the oral cavity, it can cause infection throughout the body.

Candida albicans is a normal inhabitant of the gastrointestinal tract and the female genitourinary tract that also causes infection in patients with compromised immune systems. Overgrowth of Candida organisms can also be a complication of antimicrobial therapy.

Coccidioides immitis is a fungus that inhabits hot, dry, and dusty climates, including portions of the western and southwestern United States. Pulmonary symptoms are associated.

Mycobacterium tuberculosis infection occurred in epidemic proportions several centuries ago, resulting in severe pulmonary manifestations, but has since been controlled through mass screening procedures, including skin testing and chest radiographs. This organism does not typically result in secondary lymphedema.

Question 5

A 65-year-old woman comes to the office with swelling of her left upper extremity 18 months after undergoing radiation therapy to her left axilla for breast cancer. Which of the following is the most appropriate test to confirm the diagnosis of lymphedema?

A) CT scan
B) Lymphoscintigraphy
C) MRI
D) Skin biopsy
E) Ultrasonography

Answer 5

The correct response is Option B.

The most appropriate test to confirm the diagnosis of lymphedema is lymphoscintigraphy. Radiation to the axilla causes fibrosis of the axillary lymph nodes, which impairs the proximal drainage of lymph fluid from the extremity, resulting in lymphedema. Lymphoscintigraphy is the gold-standard test to diagnose lymphedema. A radiolabeled protein injected into the hands is taken up by the lymphatic vasculature. Images are obtained as the protein travels proximally to the axillary lymph nodes. Delayed transit of the tracer and/or dermal backflow of tracer into the superficial lymphatics confirms lymphatic dysfunction and lymphedema. The test is 96% sensitive and 100% specific for lymphedema. Ultrasonography, CT scan, and MRI may show subcutaneous fluid, but are not sensitive or specific for lymphedema. Lymphedema cannot be diagnosed using a biopsy; histopathology of lymphedematous tissue only may show non-specific inflammation.

Question 6

A 43-year-old woman is evaluated because of lymphedema of the lower extremities. She demonstrates pitting edema, which does not improve with limb elevation. Her skin feels otherwise normal, with no evidence of fibrotic change. Which of the following International Society of Lymphology stages best describes this patient’s lymphedema?

A) 0
B) 1
C) 2
D) 3

Answer 6

The correct response is Option C.

Lymphedema results from congenital or acquired dysfunction of the lymphatic system. It results from changes to the lymphatic vessels, including ectasia and valve dysfunction. This results in reflux of lymphatic fluid into the interstitial space. Lymphatic fluid accumulation leads to chronic inflammation, extracellular matrix remodeling and fibrosis, adipose tissue differentiation, progressive fibrosis/sclerosis, and eventual obliteration of the lymphatic vessel lumen. Over time, accumulation of interstitial lymphatic fluid causes subcutaneous fibroadipose production.

Lymphedema is a chronic condition that slowly worsens over time. It progresses through four stages. Stage 0 indicates a clinically normal extremity but with abnormal lymph transport (identified via lymphoscintigraphy). Stage 1 demonstrates a relative accumulation of fluid high in protein content, which improves with limb elevation. Pitting may occur. Stage 2 represents pitting edema that does not resolve with elevation, but no evidence of fibrotic skin changes. Late in Stage 2, the limb may not pit as excess fat and fibrosis begins. Stage 3 describes fibroadipose deposition and fibrotic skin changes.

The presence of dermal backflow on contrast-enhanced imaging of the lymphatic system is diagnostic for lymphedema, and the severity and distribution of this backflow correlate closely with the pathologic condition of the lymphatic vessels. Indocyanine green fluorescent lymphography enables detailed dynamic functional evaluation of the superficial lymphatic system and can also be used for intraoperative lymph node mapping for vascularized lymph node transplantation.

Question 7

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 7

The correct response is Option A.

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.

Although elevation of the extremity may be reasonable, there is no role for compression if infection is ongoing. Laser therapy may be used to treat lymphatic vesicles on a mucosal surface but will not treat the deeper malformations. In the case of acute infection, there is no role for surgical decompression, and lymphatic malformations do not respond to shunting of the lymphatic fluid.

Question 8

An otherwise healthy 25-year-old woman has a diffuse, microcystic, lymphatic malformation in her pelvis and perineum. It involves the skin and subcutaneous tissues of the region, along with diffuse involvement of the pelvic muscles. Which of the following would be the most effective treatment for her condition?

A) Intralesional steroid injection
B) Propranolol therapy
C) Sclerotherapy
D) Sirolimus therapy
E) Surgical excision

Answer 8

The correct response is Option D.

The most effective and reasonable treatment option would be systemic sirolimus therapy. Recent studies have shown that sirolimus is extremely effective in treating and clinically reducing the size of venous (90%) and lymphatic malformations (LM) (95%). It is especially useful in treating vascular tumors associated with Kasabach-Merritt phenomenon (96%, along with 93% normalization of coagulopathy).

Propranolol therapy and intralesional steroid injection are treatments for hemangiomas, not malformations. Sclerotherapy would be useful in macrocystic LM but not microcystic LM. Surgical excision would be too destructive an option in this case given the location, diffuse nature of the disease, and the structures involved.

Question 10

A 70-year-old male with a past medical history of diabetes, obesity and smoking presents to your clinic with an ulcer located in the lower extremity that has been present for 4 weeks. Which of the following characteristics on you physical examination would suggest that this ulcer is secondary to chronic venous insufficiency?
A) Insensate ulceration in the plantar aspect of the foot.
B) Ulceration located over bony prominences, round with sharply demarcated borders, necrosis at the base and absent pedal pulses.
C) Painful ulceration located over the lateral malleolus with irregular borders, granulation tissue and fibrin present at the base and lower extremity edema.
D) Ulceration located at the heel with a necrotic base with normal pedal pulses.
E) Deep non-healing ulceration associated with protruding edges and scarring, bleeding, bone destruction and periosteal reaction.

Answer 10

The correct answer is option C.

Numerous lower extremity wounds look similar to a venous ulcer; therefore, it is imperative to know the differential diagnosis. Venous insufficiency ulcers are secondary to venous hypertension. They present as shallow, painful ulcers located in the gaiter region of the lower extremity. The distribution relative to the malleolus is medial in 70% of patients, lateral in 20%, and bilateral in 10%. The edge of the ulcer is irregular and smooth. Granulation tissue and fibrin are present at the base. Associated findings include edema, venous dermatitis, varicosities and lipodermatosclerosis. Ulcers secondary to arterial insufficiency are commonly described as deep, round or punched out with sharply demarcated borders and yellow base or necrosis often located over bony prominences with exposure of tendons. Associated findings include abnormal pedal pulses, cool limbs, femoral bruit and prolonged venous filling time. Ulcers secondary to diabetic neuropathy usually occur on the plantar aspect of the feet and are insensate. Pressure ulcers are associated with prolonged immobilization and prolonged pressure over bony prominences such as in the heel region. Lastly, chronic ulcers can undergo malignant transformation. Chronic ulcers may require biopsy at regular intervals as malignant change in these ulcers is directly related to their duration. Bleeding, bone destruction, soft tissue mass and periosteal reaction suggest malignancy, and a tissue biopsy must be performed.

Question 11

A 6-year-old girl has had swelling of the right cheek for the past five years. Her mother says that the area of swelling has grown commensurately with the child and enlarges when the child has a sore throat. Physical examination shows a 4-cm mass that is soft and doughy on palpation. Which of the following is the most likely diagnosis?

(A) Arteriovenous malformation
(B) Branchial cleft cyst
(C) Capillary malformation
(D) Lymphatic malformation
(E) Venous malformation

Answer 11

The correct response is Option D.

The most likely diagnosis is lymphatic malformation, a localized malformation in the development of the lymphatic system. Approximately 50% of lymphangiomas are diagnosed at birth, and 90% are diagnosed before age 2 years. Lymphangiomas are soft and doughy to palpation and typically occur in the head and neck. They may vary in size from a few millimeters to larger than 30 cm. Although the overlying skin is usually normal, it may be thin, atrophic, or have bluish discoloration.

Patient symptoms correlate with the size, location, and extent of the lymphangioma. Respiratory compromise is the most common significant complication associated with lymphangiomas affecting the head and neck. Viral infections can cause enlargement of the lesion, as in this patient.

Arteriovenous malformations are high-pressure, high-flow lesions that grow in rapid spurts. These lesions are often pulsatile and warmer than the surrounding skin. A port-wine-stainBlike appearance of the skin is commonly associated. Branchial cleft cysts present as smooth, nontender masses overlying the anterior border of the sternocleidomastoid. They can fluctuate in size. Capillary malformations, or port-wine stains, are red macular lesions that are present at birth and do not regress. They can be localized or extensive. In patients with capillary malformations of the face, further darkening of skin and hyperplastic skin changes occur over time.

Hemangiomas are vascular anomalies that appear shortly after birth, proliferate rapidly during infancy, and then spontaneously regress during childhood. They do not occur in adults.

Question 12

A 71-year-old woman presents to your office with a 6 week history of an enlarging superficial ulcer with irregular borders located just proximal to the medial malleolus. There is induration and brown pigmentation affecting the skin surrounding the ulcer. She has a remote history of an ankle fracture on the affected leg. The initial diagnostic procedure of choice is:
A) MRI
B) Plain films
C) Transcutaneous oxygen measurement
D) Duplex ultrasound of the saphenous vein with proximal compression
E) Tissue biopsy

Answer 12

The correct answer is option D.

This physical exam describes classic findings for venous leg ulcer with a location in the gaiter area, hemosiderin deposits and lipodermatosclerosis. The patient has an advanced age and a history of previous leg trauma and both of these are associated with the development of venous leg ulcers. The venous drainage of the medial lower leg is through the saphenous vein and compression will cause pooling of the blood to visualize venous reflux. Duplex ultrasound is inexpensive, non-invasive and readily available in most institutions and is the procedure of choice to diagnose valvular incompetence that causes venous stasis and leg ulcerations

Question 13

A 46-year-old woman comes to the office for initial evaluation and management of the right upper extremity because of swelling and discomfort. Medical history includes right modified radical mastectomy for T3 N2 breast cancer 2 years ago. The patient reports that the swelling started 9 months after her procedure, and that she has had two episodes of cellulitis of the right upper extremity that required treatment with oral antibiotics. Physical examination shows marked non-pitting edema of the right upper extremity and a well-healed surgical incision along the right chest wall. There is currently no evidence of recurrent breast cancer. BMI is 27 kg/m2. Which of the following is the most appropriate initial management for this patient?

A) Complete decongestive therapy
B) Magnetic resonance lymphangiography
C) Pentoxifylline therapy
D) Vascularized lymph node transfer
E) Weight loss

Answer 13

The correct response is Option A.

This patient has post-mastectomy lymphedema. Incidence of lymphedema after axillary node dissection has been reported to be up to 14%. It very often presents after a period of latency after surgical intervention. The mainstay of initial treatment for this condition is complete decongestive therapy (CDT). CDT is a therapist-driven modality that incorporates a multimodality approach including manual lymphatic drainage, daily bandaging, exercise, and skin care. After a period of initial intensive therapy, patients are transitioned to self-directed care under the supervision of a therapist with compression garments and adjunctive modalities. The relative value and benefit of each of the components of CDT are the source of active investigation. Initial management of lymphedema with some form of compression and therapy is the currently accepted approach.

Significant advances have been made in understanding the pathophysiology of lymphedema. There is a complex interplay between the lymphatic, venous, and interstitial systems with venous insufficiency and obesity contributing to the incidence and severity of lymphedema. These conditions should be investigated and treated in conjunction with CDT. The patient in this question is not obese and weight loss would not be an initial appropriate step in management of her lymphedema.

Various pharmacotherapies have been investigated in the treatment of lymphedema. These include diuretics, coumarin, vitamin E, pentoxifylline, and nonsteroidal anti-inflammatory drugs (NSAIDs). None have been demonstrated to provide efficacy in the treatment of lymphedema.

In patients who have persistent infections or symptomatic lymphedema after conservative management, there may be a role for treatment with physiologic surgical therapies such as vascularized lymph node transfer or lymphaticovenular anastomosis. Imaging modalities such as magnetic resonance lymphangiography may provide high-resolution visualization of the lymphatic system and assist in procedure selection, but should not be employed as a portion of the initial treatment of a patient with lymphedema.

Question 14

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 14

The correct response is Option D.

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 15

A 46-year-old man comes to the office because of a 10-year history of a painless mass in the mons (shown). 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 15

The correct response is Option C.

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.

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. 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.

As shown in the photograph, the overlying skin is thickened and displays the typical peau d €™orange (orange skin) appearance of congested dermal lymphatics. The epidermis forms thick, scaly deposits of keratinized debris and may display a warty verrucosis. Cracks, pits, fissures, and open wounds may develop and accommodate debris and bacteria, which lead to lymphorrhea, the leakage of lymph onto the surface of the skin.

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.

Incision and drainage is performed for wound infection. Weight reduction, physical therapy, elevation, and compression are all conservative 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 skin excess. 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 16

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 16

The correct response is Option B.

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 17

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 17

The correct response is Option E.

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 18

Mutation in which of the following genes has been implicated in lymphangiogenesis?
A) FLT4 and FOXC2
B) VEGF-C
C) PDGF
D) TGF-β
E) IL-6

Answer 18

Correct answer is option B.

VEGF-C promotes lymphangiogenesis and is hypothesized to stimulate reconnections in the distal obstructed lymphatic system with the proximal lymphatic system. Platelet derived growth factor is released initially by platelets in the inflammatory phase of wound healing during the formation of the initial thrombus. It is an important chemoattractant and activator of macrophages. Transforming growth factor beta is released by macrophages and platelets. It is a potent chemoattractant and activator of fibroblasts, stimulating them to form collagen. IL-1,6, and 8 play a role in wound healing but not in lymphangiogenesis. Some genes, including FLT4 and FOXC2 have been linked to primary lymphedema correct answer is option B.

Question 19

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 19

The correct response is Option D.

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 20

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 20

The correct response is Option E.

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 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

The correct response is Option A.

Diuretics play no role in the treatment of lymphedema. 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. Intermittent pneumatic pump compression therapy should be instituted on an outpatient basis and/or in the home. 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. Decompressive physical massage is therapeutic.

Question 22

An 80-year-old male presents to your office with a 8 x 5cm chronic wound on the lateral aspect of the right lower leg with scar tissue, irregular borders, granulation tissue at the base and an 1.5 cm2 central area of deep ulceration. He has a history of chronic venous insufficiency and stage III secondary lymphedema. The wound has been present for the past 30 years with intermittent periods of healing and relapse. Which of the following is the most appropriate diagnostic test for the evaluation and treatment of this patient?
A) Tissue biopsy
B) Indirect lymphography
C) Computed tomorgarphy
D) Magnetic resonance imaging
E) Lymphography using indocyanine green

Answer 22

The correct answer is option a.
The most important diagnostic test for the evaluation and treatment of this patient is a tissue biopsy. Marjolin’s ulcer is a rare and aggressive cutaneous malignancy that arises on previously traumatized and chronically inflamed skin. It is a long-term malignant complication of the scars resulting from chronic wound secondary to burns, pressure sores and venous stasis. There are two variants: acute and chronic. In the former, the carcinoma occurs within 1 year of the injury. The chronic form is more frequent, and malignancy tends to develop slowly, with an average time to malignant transformation of 35 years. The major histological type is squamous cell carcinoma. Marjolin’s ulcers are generally considered to be aggressive tumors with a high rate of regional metastases. Radical excision is the treatment of choice, but there is no consensus on lymph node dissection.

Question 23

Which of the following is the most appropriate initial management of lower extremity lymphedema?

A) Charles operation
B) Compression
C) Liposuction
D) Lymph node transfer
E) Lymphatic venous bypass

Answer 23

The correct response is Option B.

Lymphedema is a chronic condition that creates significant dysfunction and disability. Causes can be developmental or the result of lymphatic injury, such as in oncologic procedures. Although there is no cure for the condition, surgical techniques have demonstrated promise. However, these should be reserved for patients who have failed conservative management with compression therapy, lifestyle modification, and therapeutic exercise. Complete decongestive therapy consistently demonstrated effectiveness in limb volume reduction but requires ongoing treatment and compression to maintain its benefits.

Question 24

A 12-year-old boy is brought to the office because of penile and scrotal lymphedema. He has had several infections and is dissatisfied with the appearance of his genitalia. Which of the following is the most appropriate next step in management?

A) Charles procedure
B) Liposuction
C) Lymph node transfer
D) Lymphatic venous anastomosis
E) Tissue excision and skin grafting

Answer 24

The correct response is Option E.

First-line surgical intervention for penile and/or scrotal lymphedema is resection of the overgrown skin and subcutaneous tissue. Liposuction is generally considered first-line operative treatment for extremity lymphedema but does not have efficacy for penile/scrotal disease. Lymphatic venous anastomosis and vascularized lymph node transfer are microsurgical procedures reserved for early extremity lymphedema and do not have efficacy for penile/scrotal lymphedema. The Charles procedure involves the removal of the entire skin, subcutaneous tissue, and muscle fascia with grafting of the underlying muscle. The Charles procedure is rarely performed and is used only for extremity lymphedema and not penile/scrotal disease.

Question 25

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 25

The correct response is Option E.

Intermittent pneumatic pump compression therapy should be instituted on an 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. Diuretics play no role in the treatment of lymphedema. All patients should also use compression garments continuously during the day; Unna boots are used for venous stasis ulcers of the lower extremity. The patient described has failed weight loss attempts, so caloric restriction may also have little effect.

Question 26

An obese, 43-year-old woman has onset of lymphedema of her right arm 4 months after undergoing modified radical mastectomy with adjuvant radiation therapy. In addition to decongestive therapy, she is fitted for a daytime compression garment. Which of the following factors is most critical to the effectiveness of this modality?

A) Compression should be avoided when cellulitis is present
B) Compression should be constant throughout the limb
C) Compression should be a minimum of 20 mmHg
D) Garments must be worn continuously
E) Garments should be custom fit to the patient

Answer 26

The correct response is Option C.

Compression garments are a necessary adjunct to lymphedema therapy. They come in a variety of pressures. In the US, these are designated by class: 1) 20-30 mm Hg; class II) 30-40 mmHg, class III) 40-50 mmHg; and class IV) >50 mmHg. Generally, more severe cases of lymphedema require higher-class sleeves. Compression garments may be removed while sleeping as long as the extremity is elevated. In addition, compression should be graduated from distal to proximal. Garments can be custom fit or purchased over the counter in standard sizes; there is no evidence that custom garments are better so long as a good fit is achieved. An open wound or infection is not a contraindication to wearing a compression garment.

Question 27

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 27

The correct response is Option D.

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 28

The photograph shown (none available) above is of an otherwise healthy 7-year-old boy who has had progressive enlargement of the right upper extremity with pitting edema since birth. Radiographs of the right upper extremity show bone growth that is normal and equal to the left upper extremity. An enhanced MRI shows normal soft tissues.

This patient’s findings are most consistent with

(A) Klippel-TrŽnaunay syndrome
(B) lymphatic malformation
(C) lymphedema praecox
(D) Milroy’s disease
(E) proteus syndrome

Answer 28

The correct response is Option D.

The findings in this 7-year-old boy are most consistent with Milroy’s disease, a rare, X-linked, autosomal dominant form of primary lymphedema that is diagnosed at birth. Unilateral pitting edema is seen in this patient and is in fact associated with all forms of lymphedema. Ocular manifestations have occurred in some patients. Radiographs will show normal bone growth and no hemihypertrophy of the affected extremity. MRI of the soft tissues may also be unremarkable.

Klippel-TrŽnaunay syndrome is characterized by limb hemihypertrophy, most frequently affecting the leg, and lymphovenous malformations, often with a cutaneous component. A T1-weighted MRI will show a slow-flow lesion. Lymphedema is seen in as many as 30% of these patients. In infrequent cases, limb hypoplasia has been present in place of hypertrophy. A gadolinium-enhanced MRI is needed to distinguish lymphovenous malformations from mere lymphatic or venous lesions.

Lymphatic malformations are composed of lymph-filled dysplastic vessels that can be classified as macrocystic, microcystic, or combined. Ultrasonography can be used to diagnose lymphatic malformations in utero or after birth. MRI will also aid in delineating a lymphatic malformation from lymphedema.

Lymphedema praecox is a noncongenital form of lymphedema; signs and symptoms usually first occur before puberty. Another form, lymphedema tarda, occurs in midlife.

Proteus syndrome is a sporadic condition characterized by vascular, skeletal, and soft-tissue abnormalities. Common findings include growth asymmetry and subcutaneous tumor-like structures, such as lipomas, Schwann cell structures, and lipomatosis. Associated vascular anomalies may include capillary malformations, lymphatic malformations, capillary venous malformations, and combined lymphovenous malformations, all of which will appear abnormal on MRI.

Question 29

A 55-year-old woman presents for management of upper extremity lymphedema that developed after modified radical mastectomy and external beam radiation therapy for breast cancer. Which of the following findings would make the patient a poor candidate for a vascularized lymph node transfer?

A) Dermal back flow on lymphoscintigraphy
B) Extensive lipomatous changes in the extremity on MRI
C) Frequent bouts of cellulitis
D) Severe pitting edema
E) Short duration of symptoms

Answer 29

The correct response is Option B.

Upper extremity lymphedema has an incidence of 29 to 49% after an axillary lymph node dissection and 5 to 7% after sentinel lymph node biopsy. Surgical treatment options are divided into excisional procedures (excision, liposuction) and physiologic procedures (lymphovenous anastomosis, vascularized lymph node transfer). Although their exact mechanism is unknown, vascularized lymph node transfers are increasing in popularity for surgical management of lymphedema. Commonly used donors for vascularized lymph node transfer are the groin flap, supraclavicular flap, lateral thoracic flap, sub-mental flap and omental flap.

Lymphedema after cancer treatment occurs when the lymphatic drainage system becomes overwhelmed due to impairment in collection pathways from surgical removal of lymph nodes. Lymphatic fluid stasis leads to a proinflammatory state, which over time results in subcutaneous fat deposition. Vascularized lymph node transfers are thus most effective early in the disease process when changes of “lipedema” have not yet occurred. Once there is extensive fatty tissue in the limb, excisional procedures like liposuction or direct excision may be more effective. The finding of pitting edema on physical examination indicates presence of lymphatic fluid in the tissues, thus indicating that physiological procedures may be effective.

Lymphoscintigraphy is the most commonly used imaging modality for evaluating the status of the lymphatic system and is considered by many as the gold standard imaging modality. A radiotracer (technicium-99m) is injected into the subdermal tissue and its movement through lymphatic tissue is imaged sequentially. Impaired lymphatic function is indicated by dermal backflow and blockage of flow of the tracer. Frequency of upper extremity cellulitis has been shown to decrease after vascularized lymph node transfers, and is not a contraindication for surgery. It can, in fact, be considered an indication for lymph node transfer.

Question 30

A 1-year-old boy presents with a 3-cm mass on the cheek. Which of the following characteristics is most likely to support a diagnosis of lymphatic malformation in this patient?

A) Firm and rubbery to palpation
B) History of recurrent infections
C) Presence of a bruit
D) Presence of calcified phleboliths
E) Rapid growth followed by involution

Answer 30

The correct response is Option B.

Lymphatic malformations (LM) are benign masses of abnormal lymphatic vessels. There are many historic classifications, such as microcystic and macrocystic, as well as historic terms for specific areas affected, such as cystic hygroma, found in the head and neck. Cystic hygromas may lead to airway compromise in the newborn. LM often fluctuate in size and are frequently associated with recurrent bouts of infection. They tend to be soft and compressible on palpation, not firm and rubbery. LM are present at birth, although they might not become evident until a little later in life, especially in the lower extremities.

However, they do not follow the growth pattern of hemangiomas, specifically rapid growth (proliferation) followed by a period of involution. Treatment of LM may include observation for asymptomatic lesions, surgical resection, sclerotherapy for larger cystic structures, or laser treatment for small, superficial lesions. More recently, a mutation in the PIK3CA gene, which affects a tyrosine kinase cellular signaling pathway, has been linked to lymphatic malformations.

Further elucidation of this genetic link may lead to improved understanding and directed treatments in the future.

The presence of phleboliths is a common finding in venous malformations.

The presence of a bruit suggests the turbulent flow of an arteriovenous malformation.

Question 31

Select the incorrect statement regarding the blood supply to the following lymph nodes:
A) Vascularized superficial inguinal nodes can be based on branches from superficial circumflex iliac or superficial inferior epigastric vessels
B) The main blood supply to the supraclavicular nodes originates from the external carotid artery
C) The thoracic lymph node flap harvests lymph nodes at the lower axilla based on branches from the thoracodorsal or lateral thoracic vessels
D) The main artery supplying the submental lymph node flap is a branch of the facial artery

Answer 31

The correct answer is option B.

The main blood supply to the supraclavicular nodes originates comes from the transverse cervical artery, which is a branch of the thyrocervical trunk. The thyrocervical trunk arises from the subclavian artery and not from the external carotid artery. The main artery supplying the submental lymph node flap is a branch of the facial artery, which comes off the external carotid. The thoracic lymph node flap harvests lymph nodes at the lower axilla based on branches from the thoracodorsal or lateral thoracic vessels. One of the most frequent utilized donor sites is the groin, where the superficial inguinal lymph nodes are harvested on the superficial circumflex iliac artery or the superficial inferior epigastric artery. The correct answer is option B.

Question 32

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 32

The correct response is Option B.

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. 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. 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 breast cancer therapy is a much more likely etiology for secondary lymphedema in this country.

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 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.

Diuretics are ineffective in treating lymphedema.

A variety of surgical options have been attempted for management of lymphedema. Liposuction has been used for milder cases, but there is a high rate of edema recurrence. Combination with lymphatic drainage and compression helps to improve outcomes.

Microlymphatic procedures have been proposed to restore lymphatic drainage channels. However, long-term patency of these channels is uncertain.

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.

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 (ie, hand).

Question 33

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 33

The correct response is Option B.

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.

Question 34

Which of the following findings is not considered a poor prognostic indicator for the healing of venous insufficiency ulcerations?
A) Ulceration size of 25cm2.
B) The presence of venous reflux in the greater saphenous vein and deep venous thrombosis of the popliteal vein.
C) Venous insufficiency secondary to incompetence of the popliteal vein, greater saphenous vein and perforating system in a 62 year old
D) A Body Mass Index of 31 in a 55 year old
E) Venous insufficiency secondary to incompetence of the greater and lesser saphenous vein systems

Answer 34

Correct answer is option E.

More than 85% percent of patients with chronic venous insufficiency have disease in their superficial veins. Among the superficial veins, the great saphenous and its tributaries are involved in 70 to 80% of chronic venous insufficiency cases, the small saphenous system in 15 to 20%, and the non-saphenous veins in about 10%. Among the deep veins, involvement of the popliteal vein has demonstrated the strongest correlation with the severity of chronic venous insufficiency. This is important because it has been shown that when venous ulceration is due to superficial and perforator vein incompetence alone, surgical treatment of venous abnormality may heal up to 90% of the ulcers. A combination of reflux and obstruction due to a deep vein thrombosis had the worst prognosis for the development of skin damage. Other factors associated with ulcer non-healing include advanced age, increased body mass index, history of deep venous thrombosis, non compliance with compression therapy and large ulcer area.

Question 35

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. Which of 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 35

The correct response is Option E.

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 pressure wound dressing in addition to partial primary closure. Skin grafting and radical abdominal wall resections are unnecessary. 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 36

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 36

The correct response is Option D.

Staged skin and subcutaneous excision has become the option 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 37

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 37

The correct response is Option D.

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 not reduce the skin envelope, and the lymphedema often rapidly recurs.

Question 38

A 19-year-old woman presents with bilateral lower extremity edema, which developed 2 years ago. Physical examination shows pitting edema involving the entirety of the lower extremities with involvement of the feet. Positive Stemmer’s sign is noted. BMI is 27 kg/m2. She is a recent immigrant from India. Which of the following is the most likely cause of this patient’s swelling?

A) Filariasis
B) Lipedema
C) Lymphedema praecox
D) Lymphedema tarda
E) Podoconiosis

Answer 38

The correct response is Option A.

The most common cause of lymphedema worldwide is filariasis. It is an infection caused by the round worm Wuchereria bancrofti. The infection is transmitted by mosquito bite, which deposits larvae on the skin. The larvae migrate into the lymphatics and cause lymphatic blockage. The disease is endemic in India and sub-Saharan Africa. The lower extremities are most commonly affected.

Primary lymphedema is a developmental hypoplasia of the lymphatic system. It is a rare condition affecting 1 in 100,000 people. The most common site of involvement is the lower extremities. It is classified according to the time of onset: congenital lymphedema presents at or around birth, lymphedema praecox presents at or shortly after puberty, and lymphedema tarda presents after age 35 years. Mutation of vascular endothelial growth factor C has been identified in approximately 30% of primary lymphedema patients.

Podoconiosis is the second most common cause of tropical lymphedema. The cause is chronic exposure in barefoot walkers to soil containing high concentrations of silica. The silica particles are absorbed by the skin and collect in lymphatic vessels. This results in subendothelial lymphatic edema which gradually progresses to fibrosis and obstruction. The lower extremity lymphedema is ascending and bilateral, although it is usually asymmetric. The disease is seen in Africa, Central America, and India. Individuals afflicted with the disease have a genetic susceptibility to silica-induced lymphatic inflammation.

Morbid obesity can cause lymphedema by obstruction of lymphatics by the high amounts of adipose tissue. Individuals with BMI greater than 60 kg/m2 are at high risk.

Lipedema is abnormal subcutaneous accumulation of fat, and it is frequently mistaken for lymphedema. It occurs in women, with onset in the second and third decades, and is thought to have a genetic component. It involves the buttocks, thighs, and legs, and it spares the ankles and feet, resulting in a characteristic “cuff sign.” Patients also have tenderness and easy bruising of the affected areas. Lipedema can progress to lower extremity venous hypertension and lymphatic dysfunction.

The most common cause of lymphedema in the United States is secondary to oncologic therapy. Lymphadenectomy results in impairment of normal lymphatic flow. Radiation results in destruction of lymphatics and may also impair their regeneration. Breast cancer treatment is the most common cause, with upper extremity lymphedema developing in 4 to 49% of patients undergoing axillary lymphadenectomy and 5 to 7% of patients undergoing axillary sentinel lymph node biopsy. The incidence of lymphedema in patients undergoing treatment for upper extremity melanoma is 5% and lower extremity melanoma is 28%.

Question 39

Which finding is a contraindication for compression therapy of venous leg ulcers:
A) active infection in the ulcer
B) ankle-brachial index <0.7
C) ankle-brachial index >1.1
D) immunosuppressive therapy for autoimmune disease
E) excessive drainage from the ulcer

Answer 39

Correct answer is option B.

Approximately 30% of patients with venous ulcers have concomitant arterial insufficiency. The use of compressive leg dressings in this patient population will cause tissue ischemia and make the ulcer worse. The Wound Healing Society consensus guidelines on the management of venous leg ulcers cites level 1 evidence to support their recommendation that an ABI < 0.7 is a contraindication to compression therapy(1).

Question 40

A 25-year-old female with a history of lymphedema praecox presents to your clinic for evaluation. She has stage II lymphedema according to the International Society of Lymphology classification. What is the best imaging diagnostic modality to evaluate her lymphatic function?
A) Magnetic resonance lymphangiography
B) Tc-99m Lymphoscintigraphy
C) Ultrasound
D) Lymphangiogram
E) Indocyanine green lymphography

Answer 40

The correct answer is option B.
Lymphoscintigraphy is now the gold standard for assessing the lymphatics. This technique was introduced in 1953. The radiolabeled protein used is usually technetium Tc-99 colloid. It allows measurement of lymphatic function, lymph movement, drainage and response to treatment. Before lymphoscintigraphy became the gold standard, lymphangiogram was the main technique used to visualize the lymphatics. It involves direct cannulation of the lymphatics. Studies have shown that Imaging findings of MR lymphangiography and lymphoscintigraphy show a clear concordance. With lymphoscintigraphy, better visualization of inguinal lymph nodes is achieved, whereas with MR lymphangiography, better depiction of lymph vessels and morphologic features of lymph vessel abnormalities are achieved. Indocyanine green lymphography is helpful to Identify only superficial lymphatic vessels (1 to 2 cm deep); however, lymphoscintigraphy is more useful to identify deeper lymphatic vessels.

Question 41

A full-term baby is born via cesarean delivery because of a prenatally diagnosed mass in the posterior neck. An MRI is shown. After birth, there is no airway compromise. Which of the following is the most appropriate next step in management?

A) Administration of propranolol
B) Incisional biopsy
C) Sclerotherapy
D) Surgical resection
E) Observation only

Answer 41

The correct response is Option C.

This is a macrocystic lymphatic malformation (LM) that was prenatally diagnosed. Former terms for this malformation include cystic hygroma. The most important management issue is control of the airway, and sometimes these children have to be delivered by EXIT procedures. After delivery, the airway should be evaluated and secured before making management decisions.

While some macrocystic LMs can be resected, this is a risky procedure in a neonate, and this LM extends toward midline structures. Sclerotherapy can be performed in neonates and is now the first choice of treatment.

Infantile fibrosarcoma appears different from LM on MRI. Infantile fibrosarcomas appear as heterogeneous vascular masses and not as a cystic structure.

Propranolol is efficacious in the treatment of problematic infantile hemangiomas. Infantile hemangiomas do not start proliferating until after birth, so this is not an infantile hemangioma.

A congenital hemangioma would also appear as a vascular heterogeneous mass rather than a cystic structure, and there is no evidence that congenital hemangiomas respond to propranolol therapy.

Question 42

A 40-year-old male presents to your clinic for evaluation and management of lower extremity lymphedema. Which of the following is not considered a basic strategy for non-surgical therapy of lymphedema?
A) Weight loss
B) Multilayered inelastic banding
C) Manual lymphatic drainage
D) Diuretic therapy
E) Compression garments

Answer 42

Correct answer is option D.

The basic strategies for non surgical therapy consist of a combination of elevation of the extremities, exercises designed to optimize lymphatic flow, manual lymphatic drainage, and compression therapy. If a reconstructive procedure is being contemplated, conservative treatment should be performed first for a minimum of about 6 months because during this time period spontaneous regression of edema is possible. Diuretics do not have a place in the regular treatment protocol since removal of fluid may lead to enhanced fibrosis.