Introduction

Our son Caleb is diagnosed with lymphangiomatosis.  He is 2 1/2 years old.  My main reasons in creating this blog: to raise awareness of the disease and to share our experiences with others.  

I should start from the beginning.  To view a rather detailed synopsis of Caleb's first nine months, one can click here.  The first blog I created was all about Caleb, mostly to update my friends and family.  However, it would be remiss not to mention the blog was created prior to the diagnosis of lymphangiomatosis.

To better understand the disease, I turned to wikipedia.org as a resource.

DEFINITION 

Lymphangiomatosis (LYMF) is a condition where a lymphangioma is not present in a single localised mass, but in a widespread or multifocal manner. It is a rare neoplasm which results from an abnormal development of the lymphatic system. This may involve one or several organs.

SIGNS & SYMPTOMS

Lymphangiomatosis can present with pericardial effusions, pleural effusion, ascites (fluid in the abdominal cavity), and internal bleeding. CT scans on some patients have been similar to lymphoma as tumor-type masses have been found in the mediastinum. The fluid involved in these effusions is chylous (milky type fluid) in nature.

LYMF can involve tissues like the lung, spleen, digestive system, and reproductive system.  Symptoms include shortness of breath, difficulty breathing in (inhaling), severe pain in the abdominal cavity and pelvic cavity, and lymphedema (swelling).

DIAGNOSIS

There is no current standard for diagnosing this condition. The symptoms, clinical presentation, and typical radiographical findings can assist in the diagnosis.  A biopsy is the ultimate method for diagnosis, but is not always possible. The disease can even occur with no painful symptoms at all.

LYMF is frequently diagnosed in children nearing puberty. Although this disease is not limited to being identified in infants, young children, and adults of both genders.



TREATMENT

Treatment with vascular growth inhibitors may slow the progress of the disease. More recently, it has been effectively proven that Interferon Injections (Chemotherapy treatment)help slow the growth of these lymphatic masses.

PROGNOSIS

LYMF has a common fatality rate, although patients may live for ten years or more.