Management of cervicofacial lymphatic malformations requires a multidisciplinary approach

Michael J. Zobel, Donna Nowicki, Gabriel Gomez, Jessica Lee, Lori Howell, Joseph Miller, Chadi Zeinati, Dean M. Anselmo

Research output: Contribution to journalArticlepeer-review

Abstract

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

Original languageEnglish (US)
Pages (from-to)1062-1067
Number of pages6
JournalJournal of pediatric surgery
Volume56
Issue number5
DOIs
StatePublished - May 2021
Externally publishedYes

Keywords

  • Cervicofacial
  • Lymphatic malformation
  • Resection
  • Sclerotherapy
  • Vascular anomalies

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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