Management of cervicofacial lymphatic malformations requires a multidisciplinary approach

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 addi-tional 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 dis-ease. Microcystic CFLMs frequently require surgical resection. Sirolimus is a helpful therapeutic adjunct, particu-larly for microcystic lesions, but more study is needed. Level of Evidence: Level II, prognosis study (c) 2020 Elsevier Inc. All rights reserved.

Automated summary

This study evaluated the treatment outcomes of children with CFLM, finding that sclerotherapy is very effective for macrocystic CFLMs but less so for microcystic disease, which often requires surgical resection. Sirolimus may be a helpful therapeutic adjunct for microcystic lesions.