Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema

Carolyn C. Schook, John B. Mulliken, Steven J. Fishman, Ahmad I. Alomari, Frederick D. Grant, Arin K. Greene

Research output: Contribution to journalArticle

35 Citations (Scopus)

Abstract

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), Klippel-Trenaunay 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.

Original languageEnglish (US)
Pages (from-to)1571-1581
Number of pages11
JournalPlastic and Reconstructive Surgery
Volume127
Issue number4
DOIs
StatePublished - Apr 2011

Fingerprint

Lymphedema
Lower Extremity
Differential Diagnosis
Pediatrics
Age of Onset
Blood Vessels
Klippel-Trenaunay-Weber Syndrome
Sturge-Weber Syndrome
Vascular Malformations
Hemangioma
Diagnostic Errors
Physical Examination
Referral and Consultation
Extremities
Age Groups
History
Databases

ASJC Scopus subject areas

  • Surgery

Cite this

Schook, Carolyn C. ; Mulliken, John B. ; Fishman, Steven J. ; Alomari, Ahmad I. ; Grant, Frederick D. ; Greene, Arin K. / Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema. In: Plastic and Reconstructive Surgery. 2011 ; Vol. 127, No. 4. pp. 1571-1581.
@article{02db407745b04860b443c6fa1a13659e,
title = "Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema",
abstract = "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), Klippel-Trenaunay 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.",
author = "Schook, {Carolyn C.} and Mulliken, {John B.} and Fishman, {Steven J.} and Alomari, {Ahmad I.} and Grant, {Frederick D.} and Greene, {Arin K.}",
year = "2011",
month = "4",
doi = "10.1097/PRS.0b013e31820a64f3",
language = "English (US)",
volume = "127",
pages = "1571--1581",
journal = "Plastic and Reconstructive Surgery",
issn = "0032-1052",
publisher = "Lippincott Williams and Wilkins",
number = "4",

}

Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema. / Schook, Carolyn C.; Mulliken, John B.; Fishman, Steven J.; Alomari, Ahmad I.; Grant, Frederick D.; Greene, Arin K.

In: Plastic and Reconstructive Surgery, Vol. 127, No. 4, 04.2011, p. 1571-1581.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema

AU - Schook, Carolyn C.

AU - Mulliken, John B.

AU - Fishman, Steven J.

AU - Alomari, Ahmad I.

AU - Grant, Frederick D.

AU - Greene, Arin K.

PY - 2011/4

Y1 - 2011/4

N2 - 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), Klippel-Trenaunay 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.

AB - 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), Klippel-Trenaunay 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.

UR - http://www.scopus.com/inward/record.url?scp=79955005060&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=79955005060&partnerID=8YFLogxK

U2 - 10.1097/PRS.0b013e31820a64f3

DO - 10.1097/PRS.0b013e31820a64f3

M3 - Article

C2 - 21187804

AN - SCOPUS:79955005060

VL - 127

SP - 1571

EP - 1581

JO - Plastic and Reconstructive Surgery

JF - Plastic and Reconstructive Surgery

SN - 0032-1052

IS - 4

ER -