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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.
The age of onset is almost always before 3 months of age. Many infants are born preterm (1/3 cases) and dysmature. The babies are frequently small for dates. The placenta may be abnormal with non-specific inflammation on histology. Umbilical cord anomalies have occasionally been reported.
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 for cystic hygroma involves the removal of the abnormal tissue; however, complete removal may be impossible without removing other normal areas. Surgical removal of the tumor is the typical treatment provided, with the understanding that additional removal procedures will most likely be required as the lymphangioma grows.
The WHO Model List of Essential Medicines for Children (aka Essential Medicines List for Children [1] or EMLc [1]), published by the World Health Organization (WHO), contains the medications considered to be most effective and safe in children up to twelve years of age to meet the most important needs in a health system.
Njølstad syndrome is a syndrome characterized by non-immune hydrops fetalis (NIHF), congenital pulmonary lymphangiectasia (CPL). [1] Clinical features found in Njølstad syndrome include: facial and limbs lymphedema, [1] [2] facial abnormalities (thin upper lip, protruding ears), pectus excavatum and vulvar and labial edema.
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]
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