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

Part of Pediatric Surgical Outcomes 11 items

Infographic · Nov 2024 · 1 min read

In brief

Systematic review of 202 studies examining pediatric lymphatic malformation treatment found sclerotherapy achieves >90% response rates for macrocystic lesions, while sirolimus serves as effective adjunct therapy. Optimal outcomes require individualized, multidisciplinary approach combining medical therapy, sclerotherapy, and surgery based on lesion characteristics.

Sclerotherapy achieves >90% partial or complete response and is most effective for macrocystic lymphatic malformations
Sirolimus can be used as adjunct therapy with procedures or as primary treatment for extensive lymphatic malformations
Multi-modal treatment tailored to lesion size, extent, and type yields best outcomes for pediatric lymphatic malformations
Multidisciplinary team approach is recommended to individualize treatment strategy for each patient
Current evidence for lymphatic malformation management is moderate quality; treatment choice depends on lesion characteristics

Written by the GCMD Library team from the infographic.

A three-column comparison layout with medical illustrations of esophageal procedures. Each column contains anatomical diagrams in blue and pink tones showing the esophagus and surgical technique, accompanied by statistical data boxes below. The left column shows balloon dilation, the middle depicts Heller myotomy, and the right illustrates POEM procedure with endoscopic visualization.

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

Background: Significant variation in management strategies for lymphatic malformations (LMs) in children persists. The goal of this systematic review is to summarize outcomes for medical therapy, sclerotherapy, and surgery, and to provide evidence-based recommendations regarding the treatment.

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.

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