Treatments

Unfortunately, there is no standardized treatment for lymphangiomatosis and no cure.

is no standard approach to the treatment of lymphangiomatosis and treatment often is palliative, i.e. aimed at reducing symptoms. 1, 18 Surgical intervention may be indicated when complications arise and a number of reports of response to surgical interventions, medications, and dietary approaches can be found in the medical literature. 1, 18, 20

Unfortunately, there is no standardized treatment for lymphangiomatosis and no cure.

The LGDA has worked with special centers for care and treatment of our patients with lymphangiomatosis and Gorham’s disease. These centers are called Vascular Anomalies Centers (VACS). VAC’s are staffed with teams of specialists who are experienced in our diseases. Inform your physician about the existence of these centers. Consultation and collaboration with the experts around the world can be important to any case; and certainly increase the understanding of our rare diseases in general. Questions? Send an email to patient@lgdalliance-europe.org.

Treatment modalities have been reported in the medical literature. Be aware that the vast majority of them represent only very small number of cases; even anecdotally. Treatment modalities, by system, include:

Cardiothoraric

  • thoracocentesis
  • pericardiocentesis
  • pleurodesis
  • ligation of thoracic duct
  • pleuroperitoneal shunt
  • radiation therapy
  • pleurectomy,
  • pericardial window
  • pericardiectomy
  • thalidomide
  • interferon alpha 2b
  • Total Parenteral Nutrition (TPN)
  • medium chain triglyceride (MCT) and high protein diet
  • chemotherapy
  • sclerotherapy
  • transplant

Gastrointestinal / liver / kidney / spleen

  • interferon alpha 2b
  • sclerotherapy
  • resection
  • percutaneous drainage,
  • Denver shunt
  • Total Parenteral Nutrition (TPN)
  • medium chain triglyceride (MCT) and high protein diet
  • transplant
  • splenectomy

Skeleton

  • interferon alpha 2b
  • bisphosphonates (i.e. pamidronate)
  • surgical resection
  • radiation therapy
  • sclerotherapy
  • percutaneous bone cement
  • bone grafts
  • prosthesis
  • surgical stabilization

References

 

Source: www.lgdalliance.org (April 5th, 2016)