Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies-expert opinion consensus

Pediatr Blood Cancer. 2020 Jan;67(1):e28036. doi: 10.1002/pbc.28036. Epub 2019 Oct 16.


Objective: Complicated lymphatic anomalies (CLAs) are chronic, progressive, and debilitating conditions that share clinical features, yet key elements for optimal evaluation and management have not been established. We aimed to formulate expert opinion consensus-based guidelines for comprehensive evaluation of CLAs.

Study design: Patient support groups dedicated to CLAs organized an international conference for vascular anomaly experts from 16 specialties to address the objective. Participants received a set of questions before the meeting and reviewed the literature. Data extracted from international lymphatic anomaly registries were presented and the group separated for panel discussions during the conference. The recommendations achieving consensus within the panel were presented to the entire audience. Open debate occurred until majority approval was achieved.

Results: The expert group was composed of 52 physicians who defined the clinical elements required to evaluate and diagnose a CLA. The radiology panel established the preferred anatomical and functional imaging methods for diagnosis and the elements required to be described during interpretation. Two medical panels compiled the metabolic and hematologic tests at diagnosis and also recommended functional studies. The surgical group recommended precautions for biopsy and the pathology panel provided biopsy specimen processing guidelines.

Conclusions: Patients with CLAs require a comprehensive and targeted diagnostic plan for appropriate management, prevention of complications, and conservation of resources. As this population is managed by diverse medical and surgical specialties, we offer an expert multidisciplinary consensus-based opinion on the current literature and on data extracted from international lymphatic anomaly registries.

Keywords: Gorham-Stout; central conducting lymphatic anomaly; complicated lymphatic anomaly; generalized lymphatic anomaly; guidelines; kaposiform lymphangiomatosis.

Publication types

  • Evaluation Study

MeSH terms

  • Adult
  • Child
  • Consensus
  • Diagnostic Imaging
  • Expert Testimony*
  • Humans
  • Lymphatic Abnormalities / diagnosis*
  • Lymphatic Abnormalities / diagnostic imaging
  • Practice Guidelines as Topic / standards*
  • Prognosis
  • Vascular Malformations / diagnosis*
  • Vascular Malformations / diagnostic imaging
  • Young Adult