A multidisciplinary approach to management of abdominal lymphatic malformations
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In brief
In brief
Retrospective study of 19 pediatric patients with abdominal lymphatic malformations treated at a tertiary vascular anomalies center. Multiple treatment modalities (sclerotherapy, surgical resection, sirolimus) showed comparable clinical response across macrocystic and microcystic subtypes, supporting a multidisciplinary treatment algorithm.
Written by the GCMD Library team from the article.
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.