Abstract
Background/purpose
Abdominal lymphatic malformations (LM) are a rare subset of vascular anomaly caused
by abnormal development of the lymphatic system. They are classified as macrocystic,
microcystic or combination macrocystic and microcystic. Surgical resection, percutaneous
sclerotherapy, and medical therapy are all employed to treat these complex and often
symptomatic lesions. No standardized treatment algorithm exists currently. The purpose
of this study was to establish a multidisciplinary treatment approach to abdominal
LMs.
Methods
A retrospective observational study was conducted from 2013 to 2019 on patients with
abdominal LMs at a single tertiary children's hospital vascular anomalies center.
Demographics, imaging, and treatment modality were recorded. Clinical and/or radiographic
response to the primary treatment modality as well as complications was the outcomes
measured.
Results
Nineteen patients (12 macrocystic, 5 microcystic and 2 combined) were identified,
with a median age at diagnosis of 2.2 years (range 0.1–20.8 years). Sclerotherapy
was the most common primary treatment, followed by surgical resection and sirolimus.
No difference in clinical response (p = 0.58) or complications (p = 0.31) was observed
based on primary treatment or subtypes.
Conclusions
Based on our institutional experience, we propose an LM subtype-based treatment algorithm
for abdominal LMs. It employs a multidisciplinary approach, and results in satisfactory
patient outcomes with minimal complications.
Level of evidence
Level III, retrospective comparative study.
Abbreviations:
LM (lymphatic malformation), ISSVA (International Society for the Study of Vascular Anomalies)Key words
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Article info
Publication history
Published online: October 18, 2020
Accepted:
October 10,
2020
Received in revised form:
September 18,
2020
Received:
June 24,
2020
Footnotes
☆Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Identification
Copyright
© 2020 Published by Elsevier Inc.
