Management and Outcomes of Pediatric Lymphatic Malformations: A Systematic Review From the APSA Outcomes and Evidence-Based Practice Committee

Topic overview

Carlos Theodore Huerta, Alana L. Beres, Brian R. Englum, Katherine Gonzalez, Tamar Levene, Derek Wakeman, Yasmine Yousef, Brian C. Gulack, Henry L. Chang, Emily R. Christison-Lagay, Phillip Benson Ham, III, Sara A. Mansfield, Afif N. Kulaylat, Donald J. Lucas, Rebecca M. Rentea, Christopher P. Pennell, Jason P. Sulkowski, Katie W. Russell, Robert L. Ricca, Lorraine I. Kelley-Quon, Jun Tashiro, Kristy L. Rialon on behalf of the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee

Methods: Three questions regarding LM management were generated according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Publicly available databases were queried to identify articles published from January 1, 1990, to December 31, 2021. A consensus statement of recommendations was generated in response to each question.

Results: The initial search identified 9326 abstracts, each reviewed by two authors. A total of 600 abstracts met selection criteria for full manuscript review with 202 subsequently utilized for extraction of data. Medical therapy, such as sirolimus, can be used as an adjunct with percutaneous treatments or surgery, or for extensive LM. Sclerotherapy can achieve partial or complete response in over 90% of patients and is most effective for macrocystic lesions. Depending on the size, extent, and location of the malformation, surgery can be considered.

Conclusion: Evidence supporting best practices for the safety and effectiveness of management for LMs is currently of moderate quality. Many patients benefit from multi-modal treatment determined by the extent and type of LM. A multidisciplinary approach is recommended to determine the optimal individualized treatment for each patient.

Intended audience: Healthcare professionals and clinicians.