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Flashcards in Lymphedema Deck (19):
1

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

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.

 

2

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

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.

 

3

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

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.

 

4

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

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.

5

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

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.

 

6

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

Q image thumb

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.

7

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

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.

 

8

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

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.

 

9

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

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.

 

10

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 

 

Q image thumb

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. 

 

11

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 

 

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). 

 

12

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

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. 

 

13

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
 

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.

14

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

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.

 

15

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

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.

 

16

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
 

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.
 

17

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

 

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.

18

The photograph shown 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

 

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.

 

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