The malformation arises from when the primitive lymph sac is unable to connect with the rest of the lymphatic system during embryonic development.
[1] This disruption leads to the production of a thick coat of muscle fibers that cause rhythmic contractions which increase the intramural pressure.
Findings from a study performed in 1976 by Whimster, suggested that the vesicles noted in lymphangioma are actually outpouchings of the dilated projected vessels.
[1] This idea was furthermore supported by radiographic studies that displayed large multiobulated cisterns that lay deep into the dermis and beyond clinical lesions.
Furthermore, emphasizing the idea that the cause of this disease is a failure of primitive lymph sacs to connect with the lymphatic system.
[3] The general outlook for people who have been diagnosed with this disease is considered to be good due to the fact that the masses are known to be benign and do not develop into cancer.
[8] It is recommended that these types of cases be managed by an interprofessional health team that includes a pediatrician, surgeon, dermatologist, and primary care provider.
[9] The Lymphatic Education and Research Network in New York City, is an organization that has been established in order to provide support and improve the quality of life of those with the disease.