RECONSTRUCTIVE
Differential Diagnosis of Lower Extremity
Enlargement in Pediatric Patients Referred with
a Diagnosis of Lymphedema
Carolyn C. Schook, B.A.
John B. Mulliken, M.D.
Steven J. Fishman, M.D.
Ahmad I. Alomari, M.D.
Frederick D. Grant, M.D.
Arin K. Greene, M.D.,
M.M.Sc.
Boston, Mass.
Background: There are many causes for a large lower limb in the pediatric age
group. These children are often mislabeled as having lymphedema, and incorrect diagnosis can lead to improper treatment. The purpose of this study was to
determine the differential diagnosis in pediatric patients referred for lower
extremity “lymphedema” and to clarify management.
Methods: The authors’ Vascular Anomalies Center database was reviewed between 1999 and 2010 for patients referred with a diagnosis of lymphedema of
the lower extremity. Records were studied to determine the correct cause for the
enlarged extremity. Alternative diagnoses, sex, age of onset, and imaging studies
were also analyzed.
Results: A referral diagnosis of lower extremity lymphedema was given to 170
children; however, the condition was confirmed in only 72.9 percent of patients.
Forty-six children (27.1 percent) had another disorder: microcystic/macrocystic
lymphatic malformation (19.6 percent), noneponymous combined vascular
malformation (13.0 percent), capillary malformation (10.9 percent), KlippelTrenaunay syndrome (10.9 percent), hemihypertrophy (8.7 percent), posttraumatic swelling (8.7 percent), Parkes Weber syndrome (6.5 percent), lipedema
(6.5 percent), venous malformation (4.3 percent), rheumatologic disorder (4.3
percent), infantile hemangioma (2.2 percent), kaposiform hemangioendothelioma (2.2 percent), or lipofibromatosis (2.2 percent). Age of onset in children
with lymphedema was older than in patients with another diagnosis (p ⫽ 0.027).
Conclusions: “Lymphedema” is not a generic term. Approximately one-fourth
of pediatric patients with a large lower extremity are misdiagnosed as having
lymphedema; the most commonly confused causes are other types of vascular
anomalies. History, physical examination, and often radiographic studies are
required to differentiate lymphedema from other conditions to ensure the child
is managed appropriately. (Plast. Reconstr. Surg. 127: 1571, 2011.)
L
ymphedema is the chronic, progressive swelling of tissue caused by inadequate lymphatic
function. Lymphedema may result from
anomalous development (primary) or injury (secondary) to lymph nodes or lymphatic vessels. Although lymphedema is a specific condition, children with a large lower limb are often labeled as
having lymphedema, regardless of the underlying
cause. Because lymphedema is much more prevalent in the general population than other potenFrom the Departments of Plastic and Oral Surgery, Surgery,
and Radiology, Vascular Anomalies Center, Children’s Hospital Boston, Harvard Medical School.
Received for publication July 22, 2010; accepted October 11,
2010.
Copyright ©2011 by the American Society of Plastic Surgeons
DOI: 10.1097/PRS.0b013e31820a64f3
tial causes of congenital lower extremity overgrowth, referring physicians likely are most
familiar
with
the
term
“lymphedema.”
Lymphedema, however, is typically an adult disease caused by lymphadenectomy and/or irradiation to the axilla or groin. Primary pediatric
lymphedema is rare; a child erroneously diagnosed with lymphedema may undergo unnecessary tests and be given incorrect treatment. The
purposes of this study were to determine the accuracy of the term “lymphedema” for pediatric
lower limb enlargement and to document other
causes with which it is frequently confused.
Disclosure: The authors have no financial interest
to declare in relation to the content of this article.
www.PRSJournal.com
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Plastic and Reconstructive Surgery • April 2011
PATIENTS AND METHODS
After approval from the Committee on Clinical Investigation at Children’s Hospital Boston,
our Vascular Anomalies Center database was reviewed for patients sent between 1999 and 2010
labeled with lower extremity “lymphedema.” Individuals with disease onset after 21 years of age
were excluded to ensure that only a pediatric cohort was studied. The correct diagnosis was determined by history, physical examination, photographs, imaging studies, and/or histopathology.
Alternative conditions, sex, and age-of-onset were
recorded. The Mann-Whitney U test was used to
differentiate age of onset between patients with
lymphedema and children with other disorders.
Age of onset was defined in years and, if present
before 12 months of age, was recorded as 0. Results
are presented as median with interquartile range
(25th to 75th percentile). A two-tailed value of p
⬍ 0.05 was considered significant. Statistical analysis was performed using the SPSS software package (version 16.0; SPSS, Inc., Chicago, Ill.).
RESULTS
A referral diagnosis of lower extremity
lymphedema was identified in 170 children of
9477 patients in the database (1.8 percent).
Lymphedema was confirmed in 124 patients (72.9
percent). The remaining 27.1 percent (46 of 170)
of patients referred with lymphedema had a different diagnosis (Table 1). Seventy percent (32 of
46) had another type of vascular anomaly: microcystic/macrocystic lymphatic malformation (19.6
percent), noneponymous combined vascular malformation (13.0 percent), diffuse capillary malformation with overgrowth (10.9 percent), Klippel-Trenaunay syndrome (10.9 percent), Parkes
Table 1. Causes of an Enlarged Lower Extremity
Referred with an Incorrect Diagnosis of Lymphedema
No. (%)
Microcystic/macrocystic lymphatic
malformation
Noneponymous combined vascular
malformation
Klippel-Trenaunay syndrome
Capillary malformation
Hemihypertrophy
Posttraumatic swelling
Parkes Weber syndrome
Lipedema
Venous malformation
Rheumatologic disease
Infantile hemangioma
Kaposiform hemangioendothelioma
Lipofibromatosis
Total
1572
9 (19.6)
6 (13.0)
5 (10.9)
5 (10.9)
4 (8.7)
4 (8.7)
3 (6.5)
3 (6.5)
2 (4.3)
2 (4.3)
1 (2.2)
1 (2.2)
1 (2.2)
46 (100)
Weber syndrome (6.5 percent), venous malformation (4.3 percent), infantile hemangioma (2.2 percent), or kaposiform hemangioendothelioma (2.2
percent). Other conditions confused with
lymphedema were hemihypertrophy (8.7 percent),
posttraumatic swelling (8.7 percent), lipedema (6.5
percent), rheumatologic disease (4.3 percent), and
lipofibromatosis (2.2 percent).
Female patients constituted 62.1 percent of
patients (77 of 124) with lymphedema and 58.7
percent (27 of 46) of children without the disorder (p ⫽ 0.68). Median age of onset in children
with lymphedema was older [4 years (range, 0 to
12 years)] than in patients with another diagnosis
[0 years (range, 0 to 4 years)] (p ⫽ 0.027). At least
one imaging study was obtained in 84.7 percent
(144 of 170) of children: magnetic resonance imaging in 93 (54.1 percent), ultrasonography in 78
(45.9 percent), plain film radiography in 53 (31.2
percent), lymphoscintigraphy in 35 (20.6 percent), computed tomography in 27 (15.9 percent), echocardiography in 13 (7.6 percent),
and/or angiography in 12 patients (7.1 percent).
DISCUSSION
Lymphedema is an often loosely used label for
an enlarged lower extremity in children, regardless of the cause. Lymphedema is a precise term
that defines chronic, progressive swelling of subcutaneous tissue caused by abnormal development or injury to the lymphatic vasculature. Primary lymphedema most commonly affects the
lower extremity (Fig. 1). Patients have a significant
risk of infection, and limb enlargement occurs
over time from adipose deposition and fibrosis.
Lymphedema can be differentiated from other
causes of extremity overgrowth by history and
physical examination in approximately 90 percent
of patients. The foot is always involved and swelling proceeds proximally. Edema becomes less pitting over time as adipose and fibrous tissue are
deposited.1 Definitive diagnosis of lymphedema
requires lymphoscintigraphy, which is 92 percent
sensitive and 100 percent specific for the
disorder.2 Compromised lymphatic function is illustrated by delayed transit and/or cutaneous accumulation (dermal backflow) of radiolabeled
colloid injected into the feet.
Management of lymphedema requires daily
washing and moisturizing of the extremity to minimize desiccation, skin breakdown, and subsequent cellulitis. Patients should wear protective
clothing to prevent incidental trauma, which also
can lead to infection. Compression therapy using
a custom-fitted garment and a pneumatic pump
Volume 127, Number 4 • Pediatric Lower Limb Enlargement
Fig. 1. Images demonstrating primary lymphedema in a 2-year-old boy with left lower
extremity swelling that had been present since birth (left). (Above, right) Lymphoscintigraphy demonstrates dermal backflow and absent inguinal node drainage 21⁄2 hours after
injection. (Below, right) Axial T2-weighted magnetic resonance imaging with fat saturation
shows a hyperintense reticular network of dilated subcutaneous channels between the
dermis and fascial plane.
helps to reduce the volume of the extremity and
slow progression. Patients with significant morbidity who have failed conservative treatment
may be considered for contour resection. We
prefer suction-assisted lipectomy for moderately
enlarged extremities and staged skin/subcutaneous resection for patients with major overgrowth and skin excess.3– 6
Approximately one-fourth of patients labeled with lymphedema referred to our center
had another condition. The most commonly
confused diagnoses were other vascular anomalies: single-vessel forms (capillary malformation,
lymphatic malformation, venous malformation),
combined malformations (capillary-venous, lymphatic-venous), and eponymous syndromes (Klippel-Trenaunay, Parkes Weber). It is important to
separate these conditions from lymphedema because their natural history, prognosis, and treatment are very different. Like lymphedema, vascular anomalies are diagnosed by history and
physical examination in at least 90 percent of
patients. Although primary lymphedema can present at birth, it also may develop later in childhood
or in adolescence. In contrast, most vascular
anomalies are obvious during infancy. Girls are
not more likely to have lymphedema than boys;
therefore, sex should not be considered when differentiating lymphedema from other conditions.
If the diagnosis remains equivocal following history and physical examination, lymphoscintigraphy will rule out lymphedema, and magnetic resonance imaging can confirm the diagnosis of
another vascular anomaly.
Diffuse capillary malformation with overgrowth can cause soft-tissue and skeletal enlargement of a limb (Fig. 2). Patients are not at increased risk for infection, and the progression of
overgrowth is slow. The cutaneous stain is treated
by pulsed-dye laser. The circumferential overgrowth may be improved with suction-assisted lipectomy; occasionally, axial enlargement can
cause a leg-length discrepancy.
Venous and noneponymous combined malformations (e.g., capillary-venous, lymphatic-venous) are localized in more than 90 percent of
patients; 50 percent affect deep structures (e.g.,
muscle, bone, joints, viscera) (Figs. 2 and 3).7,8
One-half of sporadic venous malformations have
a somatic mutation in the TIE2 receptor.8 Venous
malformations of the lower limb typically involve
the skin and/or muscles. If all tissues, including
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Plastic and Reconstructive Surgery • April 2011
Fig. 2. Examples of patients with single-vessel type vascular malformations with a referring diagnosis of lymphedema. (Above, left)
A 9-year-old boy with a diffuse capillary malformation and overgrowth of the left lower extremity; capillary malformation is not a
component of lymphedema. (Below, left) Coronal T1-weighted magnetic resonance imaging shows thickening of the subcutaneous
fat with normal blood channels. (Above, center) An 8-year-old girl with a venous malformation of the right lower extremity
(lymphedema does not have cutaneous varicosities). (Below, center) Coronal T2-weighted magnetic resonance imaging scan showing deep intramuscular and cutaneous venous anomalies. (Above, right) A 12-month-old boy with a lymphatic malformation of the
right lower limb. Although history and physical examination were consistent with lymphedema, macrocystic lymphatic malformation was diagnosed based on magnetic resonance imaging findings. (Below, right) Sagittal T2-weighted magnetic resonance imaging scan with fat saturation demonstrates fluid-filled lymphatic macrocysts within the intermuscular space.
bone, are affected, the disorder is called phlebectasia of Bockenheimer.9 Extremity venous malformation can cause leg-length discrepancy, hypoplasia resulting from disuse atrophy, fibrosis,
pain, pathologic fracture, hemarthrosis, and degenerative arthritis.10,11 A large venous malformation involving the deep venous system is at risk for
thrombosis and pulmonary embolism. Stagnation
within a lesion can cause localized intravascular
coagulopathy and painful phlebothromboses.
1574
Treatment of lower extremity venous malformation includes compression garments, sclerotherapy, and/or resection.
Primary lymphedema differs from microcystic, macrocystic, and combined lymphatic malformations because it is not cystic and involves
only the subcutaneous layer. Microcystic/macrocystic lymphatic malformation can affect all
structures of the extremity, including muscle and
bone (Fig. 2). Several germ-line mutations cause
Volume 127, Number 4 • Pediatric Lower Limb Enlargement
Fig. 3. Example of a noneponymous combined vascular malformation with a referring diagnosis of lymphedema. (Left) A 15-year-old girl with a capillary-venous malformation of the right lower limb; a lymphedematous lower extremity typically has
normal overlying skin. (Right) Axial T2-weighted magnetic resonance imaging scan
shows dilated veins within the subcutaneous fat underlying the capillary stain.
primary lymphedema (VEGFR3, FOXC2, SOX18,
and CCBE1).12–15 In contrast, microcystic/macrocystic lymphatic malformations are thought to result from paradominant inheritance; an individual
with a predisposed inherited mutation develops a
somatic second hit in the same gene that causes
expression of the lesion.16 Symptomatic microcystic/macrocystic lymphatic malformations of the
lower extremity are managed by compression,
sclerotherapy, and/or resection.
The eponymous vascular malformations, KlippelTrenaunay and Parkes Weber syndrome, usually
are more problematic than lymphedema (Fig. 4).
Klippel-Trenaunay syndrome is a capillary-lymphatic-venous malformation of the lower extremity with
overgrowth. Patients are at risk for infections,
deep venous thrombosis, pulmonary embolism,
leg-length discrepancy, and difficulty with ambulation. Large embryonal veins are removed to prevent venous thrombosis. A shoe lift or epiphysiodesis may be necessary to correct leg-length
discrepancy. Occasionally, selective amputation is
required to permit footwear and to facilitate ambulation. Soft-tissue overgrowth can be improved
with staged skin/subcutaneous resection. Parkes
Weber syndrome is a capillary-arteriovenous or
capillary-lymphatic-arteriovenous malformation
of the lower extremity with overgrowth. Parkes
Weber syndrome can be caused by a mutation in
RASA1, and patients with multiple capillary malformations are likely on the spectrum of capillary malformation-arteriovenous malformation.17 Patients
are at risk for congestive heart failure because of
extensive arteriovenous shunting. Genetic counseling is required for children with capillary malformation-arteriovenous malformation because it is an
autosomal dominant condition. Symptomatic patients are managed by embolization; epiphysiodesis
or amputation may be necessary.
Lipedema is commonly misdiagnosed as
lymphedema (Fig. 5). It is characterized by bilateral, fatty hypertrophy of the lower extremities in
disproportion to the rest of the body.18 Pubertal
female patients are primarily affected, and a positive family history may be reported. Unlike
lymphedema, the foot is not involved, pain is common, and patients are not at an increased risk for
infection. Lipedema can be differentiated from
lymphedema by history and physical examination.
Magnetic resonance imaging shows increased subcutaneous adipose tissue without stranding or
thickened skin; lymphoscintigraphy is normal.
The size of the limb can be reduced by suctionassisted lipectomy.
1575
Plastic and Reconstructive Surgery • April 2011
Fig. 4. Examples of children with eponymous combined vascular anomalies with a referring diagnosis of lymphedema. (Above, left) An 18-year-old female patient with KlippelTrenaunay syndrome of the left lower extremity. The capillary malformation, venous varicosities, and cutaneous lymphatic vesicles were not consistent with lymphedema.
(Below, left) Axial T2-weighted magnetic resonance imaging scan with fat saturation demonstrates a marginal venous system and subcutaneous lymphatic malformation with
subfascial involvement. (Above, right) A 6-month-old with Parkes Weber syndrome. A
cutaneous stain and warm extremity indicated a fast-flow malformation. Diagnosis was
confirmed by magnetic resonance angiography (below, right), demonstrating enlargement of the arteries and early opacification of the dilated veins.
Hemihypertrophy, the idiopathic enlargement of an area compared with the contralateral
side, may be confused with lymphedema (Fig. 6).19
Hemihypertrophy is usually a diagnosis of exclusion. Lymphoscintigraphy is normal and magnetic
resonance imaging demonstrates enlargement of
all components of the extremity (i.e., adipose,
muscle, bone) without pathologic findings. Unlike lymphedema, there is overgrowth of the tissues below the muscle fascia. Occasionally, symmetry may be improved with suction-assisted
lipectomy of the subcutaneous compartment. In
contrast to other causes of extremity overgrowth,
children with hemihypertrophy are at risk for
Wilms tumor and require screening.20
A lower extremity tumor can be mistaken for
lymphedema (Fig. 7). Infantile hemangioma, the
1576
most common tumor of infancy, is usually localized and easily distinguished from lymphedema. A
diffuse, reticular form may involve the lower extremity and present with hypertrophy.21 Reticular
hemangioma is differentiated from lymphedema
because it appears shortly after birth, proliferates,
and then involutes. The tumor is infiltrative and
can involve fascia or muscle. In addition, reticular
hemangioma often ulcerates and is associated with
ventral-caudal malformative anomalies.21 Management may require corticosteroid treatment for recalcitrant ulceration or congestive heart failure;
imaging is necessary to rule out underlying structural disorders.
Kaposiform hemangioendothelioma is a rare
vascular neoplasm that is locally aggressive but
does not metastasize.22,23 Although one-half of le-
Volume 127, Number 4 • Pediatric Lower Limb Enlargement
Fig. 5. Example of a patient with lipedema referred with a mistaken diagnosis
of lymphedema. (Left) The patient was a 34-year-old woman who had had
enlarged lower extremities since adolescence. Because the feet were not involved, lipedema was diagnosed by physical examination and confirmed with
imaging. The lymphoscintigram was normal (prompt tracer uptake into abdominal nodes bilaterally) (above, right), and coronal T1-weighted magnetic
resonance imaging showed circumferential overgrowth of the subcutaneous
fat (below, right).
sions are present at birth, onset can occur in infancy or childhood.24 The tumor is often diffuse,
and an extremity is affected in approximately onethird of patients.24,25 The skin is reddish purple
and pain is common. In addition, 50 percent of
patients have Kasabach-Merritt phenomenon
[thrombocytopenia (⬍25,000/mm3), petechiae,
bleeding].22,26,27 Kaposiform hemangioendothelioma enlarges in early childhood and then partially
regresses after 2 years of age into mid childhood.
Usually, there is residual tumor and fibrosis that
causes chronic pain and stiffness.28 Kaposiform
hemangioendothelioma also has a lymphatic
component, which stains positive for the lymphatic marker D2-40.25 First-line treatment is
vincristine to control Kasabach-Merritt phenomenon and pain; resection is rarely possible
because the tumor involves multiple tissue
planes and important structures.29
Lipofibromatosis is a rare, slow-growing neoplasm that, when diffuse, can cause enlargement
of the lower limb.30,31 It is not aggressive and does
not metastasize. The leg usually is enlarged at birth
or by early childhood; subcutaneous fatty over-
growth is the predominant finding. Lipofibromatosis can involve the subfacial compartments and
cause bony overgrowth; the foot may be spared.
Diagnosis is confirmed by histopathologic examination showing abundant adipose tissue, spindled fibroblasts, focal fascicular growth, and limited mitotic activity.30,31 Suction-assisted lipectomy
can improve limb contour.31
Posttraumatic swelling is erroneously labeled
lymphedema in some children. Prolonged edema
of the lower extremity can occur following injury,
particularly after a severe sprain or fracture. Unlike lymphedema, the lymphatic function of the
extremity is normal. If the diagnosis is equivocal,
lymphoscintigraphy will demonstrate intact lymphatic flow. Some patients will have a ligamentous
tear or small fracture that can be identified by
plain film and/or magnetic resonance imaging.
Rarely, rheumatologic disorders can cause
chronic lower extremity swelling in children.
Lymphedema and other conditions are ruled out
by lymphoscintigraphy and magnetic resonance
imaging. If there is no history of trauma and no
other potential cause of the swelling can be
1577
Plastic and Reconstructive Surgery • April 2011
Fig. 6. Example of a child with hemihypertrophy with a referring diagnosis of lymphedema. (Left)
An 11-year-old boy with right lower extremity enlargement present since birth. History and physical examination potentially were consistent with lymphedema. (Right) Axial pilot magnetic resonance imaging scan demonstrated overgrowth of the lower extremity, including subcutaneous fat,
muscle, and bone. Because lymphedema affects only the skin and subcutaneous tissue, and a
vascular abnormality was not present, hemihypertrophy was diagnosed.
Fig. 7. Examples of patients with tumors erroneously labeled as lymphedema on referral. (Left) A 3-month-old with a reticular
infantile hemangioma of the left lower extremity that appeared shortly after birth. The red appearance of the skin and rapid
postnatal progression were inconsistent with lymphedema but typical for infantile hemangioma. (Center) A 3-month-old with
kaposiform hemangioendothelioma diagnosed by history, physical examination, and laboratory findings. The patient
suffered from diffuse discoloration of the extremity, bruising, and thrombocytopenia, which do not occur with
lymphedema. (Right) A 5-year-old with lipofibromatosis. History and physical examination possibly were consistent with
lymphedema. Because imaging studies were inconclusive, biopsy specimens were obtained and the diagnosis was made
histopathologically.
1578
⫾
Unilateral/bilateral
⫹
⫾
⫺
⫺
⫹
1 Subcutaneous
adipose/edema
Diagnostic histopathology
⫺
Management
Compression,
operative
Familial transmission
Laterality
Foot/hand involvement
Cutaneous changes
Abnormal blood tests
Risk for Wilms tumor
Lymphoscintigraphy
MRI findings
M, male; F, female; MRI, magnetic resonance imaging; ⫹, with; ⫺, without; ⫾, with or without; 1, increased.
*Capillary malformation, lymphatic malformation, venous malformation, combined vascular malformation (e.g., lymphatic-venous malformation, Klippel-Trenaunay syndrome, Parkes Weber
syndrome, CLOVES syndrome).
†Infantile hemangioma, kaposiform hemangioendothelioma, lipofibromatosis.
‡Posttraumatic, rheumatologic, and systemic fluid overload (e.g., cardiac, hepatic, renal disease).
M⫽F
Infancy/childhood/
adolescence
⫺
⫾
⫺
⫺
⫺
Unilateral
Bilateral
Unilateral
Unilateral
Unilateral/bilateral
⫾
⫺
⫾
⫾
⫾
⫹
⫺
⫹
⫺
⫺
⫾
⫺
⫾
⫺
⫾
⫺
⫺
⫺
⫹
⫺
⫺
⫺
⫺
⫺
⫺
Abnormal vasculature 1 Subcutaneous Tumor-specific
1 Adipose/muscle/bone 1 Subcutaneous/
adipose
(enhancing lesion)
(normal vasculature)
subfascial edema
⫹
⫺
⫹
⫺
⫺
Sclerotherapy,
Operative
Pharmacotherapy,
Wilms tumor screening,
Treat underlying
embolization,
operative
operative
condition
operative
M⫽F
Infancy
M⫽F
Infancy
F
Adolescence
Hemihypertrophy
Tumor†
Lipedema
Vascular
Malformation*
M⫽F
M⫽F
Infancy/adolescence Infancy
Male-to-female incidence
Onset
Fig. 8. An infant with CLOVES syndrome, an additional cause of
lower extremity asymmetry in children, diagnosed by physical
examination. Foot anomalies and lipomatosis of the trunk do not
occur with lymphedema.
Lymphedema
CONCLUSIONS
Approximately one-fourth of pediatric patients with a large lower extremity are misdiagnosed as having lymphedema. The most commonly confused causes are other types of vascular
anomalies, lipedema, and hemihypertrophy. Although history and physical examination usually
are sufficient to differentiate lymphedema from
other conditions, lymphoscintigraphy and/or
magnetic resonance imaging may be necessary if
the cause is unclear (Table 2 and Fig. 9). If
lymphedema is suspected, a lymphoscintigram is
Table 2. Typical Characteristics of Conditions Causing Lower Extremity Enlargement in the Pediatric Age Group
identified, children are referred for rheumatologic consultation.
Additional conditions may cause lower extremity asymmetry in children but were excluded
from this study because patients were not erroneously diagnosed with lymphedema (Fig. 8). For
example, CLOVES syndrome (congenital lipomatosis overgrowth, vascular malformations, epidermal nevi, and scoliosis) represents a newly described overgrowth disorder.32,33 Rare vascular
tumors, such as nonkaposiform hemangioendotheliomas can involve the lower limb. Finally, conditions that reduce the size of one lower extremity,
such as hemiatrophy, may cause the contralateral
limb to appear enlarged.
Nonspecific
Edema‡
Volume 127, Number 4 • Pediatric Lower Limb Enlargement
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Plastic and Reconstructive Surgery • April 2011
Fig. 9. Diagnostic algorithm for pediatric lymphedema.
the first imaging test ordered. A patient thought
to have a condition other than lymphedema on
history and physical examination usually undergoes magnetic resonance imaging evaluation to
confirm the suspected diagnosis and/or to define
the extent of disease. Magnetic resonance imaging
also is commonly used as a secondary imaging
study if lymphoscintigraphy is negative. Correct
diagnosis is important because the natural history
and management of lymphedema are very different compared with other lower extremity diseases
in children.
Arin K. Greene M.D., M.M.Sc.
Department of Plastic Surgery
Vascular Anomalies Center
Children’s Hospital Boston
300 Longwood Avenue
Boston, Mass. 02115
arin.greene@childrens.harvard.edu
ACKNOWLEDGMENT
The authors thank David Zurakowski, Ph.D., for
statistical expertise.
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Contacting the Editorial Office
To reach the Editorial Office, please use the following contact information:
Plastic and Reconstructive Surgery威
Rod J. Rohrich, M.D., Editor-in-Chief
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Tel: 214-645-7790
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