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Lymphangiectasia, also known as "lymphangiectasis", [1] is a pathologic dilation of lymph vessels. [2] When it occurs in the intestines it is known as intestinal lymphangiectasia, colloquially recognized as Waldmann's disease in cases where there is no secondary cause. [ 3 ]
Hennekam syndrome, also known as intestinal lymphagiectasia–lymphedema–mental retardation syndrome, [1] is an autosomal recessive disorder consisting of intestinal lymphangiectasia, facial anomalies, peripheral lymphedema, and mild to moderate levels of growth and intellectual disability. [1] [2]
Waldmann's disease, Primary intestinal lymphangiectasia The lower legs and feet of a 23-year-old woman with Waldmann’s since infancy Waldmann disease, also known as Primary Intestinal Lymphangiectasia (PIL), is a rare disease [ 1 ] characterized by enlargement of the lymph vessels supplying the lamina propria of the small intestine . [ 2 ]
Treatment is often pursued for troubling symptoms (itching, pain) or for cosmetic reasons. Surgical removal (excision) of the affected skin layers is the most common and effective treatment. Ablative carbon dioxide laser therapy is a less invasive method that can improve the appearance.
Macrocystic have cysts greater than 2 cubic centimetres (0.12 cu in), and microcystic lymphatic malformation have cysts that are smaller than 2 cubic centimetres (0.12 cu in). [2] These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and involve the head and neck.
Aplasia cutis congenita-intestinal lymphangiectasia syndrome is a very rare genetic disorder which is characterized by aplasia cutis congenita, intestinal lymphangiectasia-induced generalized edema, hypoproteinemia, and lymphopenia. [1] [2] It has been described in two Ashkenazi Jewish brothers. [3] [4]
This page was last edited on 1 November 2024, at 13:47 (UTC).; Text is available under the Creative Commons Attribution-ShareAlike 4.0 License; additional terms may apply.
Multidisciplinary treatment, especially occupational therapy, is designed to address this issue and aims to increase children's engagement in activities of daily living. Occupational therapy empowers children to heal through creative activities and roles that help distract them from their pain in a purposeful, functional way.