Multidisciplinary Approach to the Management of Lymphatic Malformations of the Head and Neck

Milton Waner; Teresa M O

doi:10.1016/j.otc.2017.09.012

Multidisciplinary Approach to the Management of Lymphatic Malformations of the Head and Neck

Otolaryngol Clin North Am. 2018 Feb;51(1):159-172. doi: 10.1016/j.otc.2017.09.012.

Authors

Milton Waner¹, Teresa M O²

Affiliations

¹ Lenox Hill Hospital, 210 East 64th Street, 7th Floor, New York, NY 10065, USA. Electronic address: mwmd01@gmail.com.
² Department of Otolaryngology-Head and Neck Surgery, Vascular Birthmark Institute of New York, Facial Nerve Center, Manhattan Eye, Ear and Throat Hospital, 210 East 64th Street, 7 Floor, New York, NY 10065, USA.

PMID: 29217060
DOI: 10.1016/j.otc.2017.09.012

Abstract

Lymphatic malformations (LMs) occur in 2.8 to 5 per 100,000 live births. Most involve the head and neck and they are equally common in men and women. They are developmental anomalies of unknown cause, although recent evidence suggests that an upregulation of the mammalian target of rapamycin (mTOR) pathway may be a causal factor leading to the overproduction of abnormal lymph vessels. These vessels are likely dilated lymphatic sacs sequestered from the lymphatic and venous systems. This overproduction results in the accumulation of lymph in dilated cystic spaces, which in turn results in the clinical features of an LM.

Keywords: Head; Lymphatic malformations; Multimodal treatment; Neck; Sclerotherapy; Surgery.

Publication types

Review

MeSH terms

Combined Modality Therapy
Head and Neck Neoplasms / diagnostic imaging*
Head and Neck Neoplasms / therapy*
Humans
Lymphangioma / diagnostic imaging*
Lymphangioma / therapy*
Magnetic Resonance Imaging
Sclerotherapy
Sildenafil Citrate / therapeutic use
Sirolimus / therapeutic use
Surgical Procedures, Operative
Treatment Outcome

Substances

Sildenafil Citrate
Sirolimus