Differentiating PIL from other Diseases. Epidemiology and Demographics. Natural History, Complications and Prognosis. Editor-In-Chief: C. Michael Gibson, M. Primary intestinal lymphangiectasia Waldmann's disease was first discovered by Waldmann T. PIL develops as a result of enlargement or dilatation of intestinal lymphatic vessels lacteals with leakage of their contents into the bowel lumen leading to hypoalbuminemia , hypogammaglobulinemia , and lymphopenia.
The cause of PIL has not yet been identified. The prevalence of PIL is unknown. It can be asymptomatic. It primarily affects children generally diagnosed before the age of 3 and young adults but may be diagnosed later in adults.
Symptoms include pitting edema , diarrhea , fatigue , abdominal discomfort , and malabsorption syndrome. PIL is diagnosed by biopsy revealing dilated intestinal lymphatics. Video-capsule endoscopy may be helpful when endoscopic findings are not conclusive. Common complications of PIL include infections , malignant transformation lymphoma , and skin warts. There is no definitive treatment for PIL; the mainstay of therapy is a low- fat , high protein diet with medium-chain triglyceride oral supplementation and supplemental calcium and vitamins.
PIL is associated with significant morbidity and requires adherence to dietary modification and oral supplements ; otherwise. It can be fatal in some cases if anasarca or malignancy develops. Historical Perspective In , primary intestinal lymphangiectasia Waldmann's disease was first discovered by Waldmann T. Assessment using radio-labeled I- albumin resulted in low levels in those patients. In addition, small intestinal biopsies were examined under a microscope revealing different degrees of lymphatic vessel dilatation.
This syndrome is associated with the following symptoms : [ 3 ] Dystrophic yellow nails Nail ridging Loss of lunula Lymphedema Pleural effusions Pathophysiology Pathology PIL develops as a result of enlargement or dilatation of intestinal lymphatic vessels lacteals with leakage of their contents into the bowel lumen leading to hypoalbuminemia , hypogammaglobulinemia , and lymphopenia.
