Related Experiment Video
Updated: Nov 5, 2025
Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting
Published on: May 1, 2015
Management of cervicofacial lymphatic malformations requires a multidisciplinary approach
Michael J Zobel1, Donna Nowicki1, Gabriel Gomez2
1Division of Pediatric Surgery, Department of Surgery, Children's Hospital Los Angeles, 4650 Sunset Blvd., MS#100, Los Angeles, CA, USA 90027.
Insights
Sclerotherapy effectively treats macrocystic cervicofacial lymphatic malformations (CFLM), while microcystic CFLMs often need surgery. Sirolimus offers adjunctive benefits for microcystic types.
Area of Science:
- Vascular Anomalies
- Pediatric Surgery
- Interventional Radiology
Background:
- Cervicofacial lymphatic malformations (CFLM) are rare, potentially life-threatening vascular anomalies.
- Multidisciplinary treatment strategies for CFLM are not well-documented.
Purpose of the Study:
- To evaluate treatment outcomes for CFLMs using sclerotherapy, surgical resection, and medical management.
- To assess the efficacy of different interventions based on CFLM subtype.
Main Methods:
- Retrospective review of 63 children with CFLM treated between 2004 and 2019.
- Analysis of outcomes following sclerotherapy (doxycycline/bleomycin), surgical resection, and sirolimus therapy.
- Primary outcome: >50% volume reduction by imaging; mean follow-up: 27.5 months.
Main Results:
- Sclerotherapy showed high efficacy for macrocystic (97%) and mixed (82%) CFLMs, but lower for microcystic (67%).
- Surgical resection significantly improved outcomes in 75% of cases.
- Sirolimus provided benefits for microcystic lesions in 33% of patients.
Conclusions:
- Sclerotherapy is highly effective for macrocystic CFLM components.
- Microcystic CFLMs often necessitate surgical intervention.
- Sirolimus serves as a valuable adjunct, particularly for microcystic disease, warranting further investigation.
Background/Purpose:
Cervicofacial lymphatic malformations (CFLM) are rare, potentially life-threatening vascular anomalies, yet reports on multidisciplinary treatment strategies are lacking. We evaluated outcomes for CFLMs following sclerotherapy, surgical resection, and/or medical management.
Methods:
We identified children with a CFLM at a vascular anomalies center from 2004 to 2019.
Exclusion Criteria:
retro-orbital malformations, untreated malformations, patients without follow-up. Primary clinical outcome was contour improvement, with significance defined as LM volume reduction of >50% by cross-sectional imaging.
Results:
Sixty-three children met inclusion criteria: 35 with macrocystic CFLMs, six with microcystic CFLMs, and 22 with mixed-type malformations. Mean post-intervention follow-up was 27.5 months. Fifty-eight patients underwent sclerotherapy (median: two treatments). Doxycycline and/or bleomycin were used in 95% of patients. After sclerotherapy, 97% of macrocystic CFLMs improved significantly compared to 82% of mixed and 67% of microcystic lesions. Sixteen children underwent surgical resection with 75% significantly improving; two additional patients were successfully treated with sclerotherapy after debulking surgery. Six children received sirolimus for microcystic disease, of which 33% significantly improved.
Conclusion:
Sclerotherapy is very effective for macrocystic components of CFLMs, albeit less so for microcystic disease. Microcystic CFLMs frequently require surgical resection. Sirolimus is a helpful therapeutic adjunct, particularly for microcystic lesions, but more study is needed.
Level Of Evidence:
Level II, prognosis study.
