Lymphedema Flashcards
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
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.
2018
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
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.
2018
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
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.
2017
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.
2015
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.
2015
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.
2015
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.
2015
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.
2014
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
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.
2019
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
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.
2019
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
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.
2019
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
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.
2020
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
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.
2020
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
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.
2021
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
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.
2021
