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Lymphatic malformations: a 20-year single institution experience

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Abstract

Purpose

Lymphatic malformations (LMs) are congenital abnormalities which result from disturbances in the embryologic development of the lymphatic system. We sought to determine the characteristics and treatment patterns for LMs in a rural setting, and the effect of a specialized vascular malformations clinic on triage and follow-up.

Methods

This is a retrospective cohort study at a single tertiary care institution. Sixty-two patients were identified; chart review was completed to obtain demographic, surgery/sclerotherapy session and follow-up information.

Results

The head/neck region was the most predominant LM location (N = 26, 41.9%), followed by trunk (N = 16, 25.8%), extremity (N = 11, 17.7%), and intraabdominal/retroperitoneal (N = 7, 11.3%). Twenty-eight patients were managed non-surgically, while 21, 7 and 6 patients required surgery, sclerotherapy, or both. Head/neck LMs were the most likely to recur (73%, p = 0.028). Patients seen in specialty clinic had similar duration of follow-up and time to intervention, but were more often below 1 year of age (p = 0.030). Average LM volume among patients with available imaging was much larger in those referred to specialty clinic (73.2 cm3 versus 14.8 cm3, p = 0.022).

Conclusion

Our experience reiterates not only the wide variety of clinical presentations of lymphatic malformations, but also demonstrates the necessity of multiple subspecialties and their collaboration to achieve prompt and efficacious treatment.

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Acknowledgements

This study was conducted under the supervision and sponsorship of Christa N. Grant, MD. Dr. Grant was involved in the study design, the analysis and interpretation of data, the writing of the report, and the decision to submit the manuscript for publication.

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Correspondence to Anthony P. Kronfli.

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Supplementary Information

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383_2021_4859_MOESM1_ESM.tif

Supplementary file1. Supplementary Figure 1: Statewide distribution of patients seen for lymphatic malformations (LM), represented in light grey, based on zip code. (TIF 24656 KB)

Supplementary file2 (DOCX 17 KB)

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Kronfli, A.P., McLaughlin, C.J., Moroco, A.E. et al. Lymphatic malformations: a 20-year single institution experience. Pediatr Surg Int 37, 783–790 (2021). https://doi.org/10.1007/s00383-021-04859-5

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  • DOI: https://doi.org/10.1007/s00383-021-04859-5

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