What is Waldmann Disease?
Waldmann Disease which is also known by the name of Primary intestinal Lymphangiectasia is an extremely rare pathological condition of the digestive system which is characterized enlarged lymph vessels which line the small intestine.
The characteristic features of this condition are severe abdominal discomfort along with swelling of the extremities. Waldmann Disease usually is diagnosed very early in life but in some cases the diagnosis may not be made until much later in life of Waldmann Disease.
What Are The Causes of Waldmann Disease?
The root cause of Waldmann Disease is not yet known but there have been some rare cases of multiple family members having this condition indicating a familial link to this condition. Other than this, researchers have still not been able to pinpoint an exact cause of Waldmann Disease.
What Are The Symptoms of Waldmann Disease?
The most common symptom of Waldmann Disease is swelling or edema of the lower extremities. This swelling then progresses to the face, abdominal area, and the external genitalia. This is all because of fluid retention as a result of low levels of albumin in the blood.
There may also be presence of lymphedema. Symptoms like abdominal discomfort or pain, diarrhea, vomiting, fatigue, unintentional weight loss, not enough weight gain in childhood despite adequate nutrition is also something that is seen in Waldmann Disease.
Additionally, an individual with this condition will have low albumin and blood cholesterol levels as a result of Waldmann Disease. Additionally, pericarditis, pleural effusion, and abdominal effusion are also some of the presenting features of Waldmann Disease.
How is Waldmann Disease Diagnosed?
If Waldmann Disease is suspected in an individual based on the symptoms that he or she presents with then an endoscopy will have to be performed to look at the status of the abdomen and the surrounding areas of the intestines to look for any abnormalities that are classic for Waldmann Disease. A biopsy may also be conducted to look for any signs of abdominal dilation. The results of all of these tests will confirm the diagnosis of Waldmann Disease.
How is Waldmann Disease Treated?
The mainstay of treatment for Waldmann Disease is strict dietary modifications with a low fat diet for a prolonged period of time. Additionally, triglycerides will be supplemented for nutritional purposes and to supply essential fatty acids required by the body for normal functioning. Vitamin D supplements will also be given for treatment of Waldmann Disease.
The need for continuation of a low fat diet is more or less permanent because it has been seen that the symptoms recur if there is an interruption in the diet control of a patient with Waldmann Disease. Additionally, diuretics will also be prescribed to treat edema associated with Waldmann Disease.
In cases where there is significant deficiency of albumin in individuals with Waldmann Disease, then albumin infusion is also recommended. Under very rare cases, surgical removal of the intestines is recommended as a mode of treatment for Waldmann Disease.