Medical Definition of Congenital dysplastic angiectasia

1. A congenital malformation syndrome characterised by the triad of asymmetric limb hypertrophy, haemangiomata, and nevi. Asymmetric limb hypertrophy is enlargement of one limb and not the corresponding limb on the other side, the enlarged limb being 3 times more likely to be a leg than an arm in ktw; and the limb enlargement is of bone as well as soft tissue. The haemangiomas, abnormal nests of blood vessels that proliferate inappropriately and excessively, cover a remarkable range from small innocuous capillary haemangiomas ( strawberry marks ) to huge cavernous haemangiomas. The nevi are pigmented moles on the skin; in ktw there are often also dark linear streaks on the skin, streaks due to too much pigment. There can be other abnormalities but the triad is the consistent clinical centrepiece of the disease. most persons with ktw have an enlarged leg and do relatively well without treatment or, for example, with only compression from an elastic stocking. Skin ulcers and other skin problems can occur over the swollen leg. Usually, the treatment is conservative. Surgery is almost never needed. The only possible exceptions are the very rare situations in which the leg reaches gigantic proportions or secondary clotting difficulties arise (due to trapping and destruction of blood platelets in a huge haemangioma). Then, amputation may become necessary. The cause of ktw syndrome is unknown. (12 Dec 1998)

Lexicographical Neighbors of Congenital Dysplastic Angiectasia

congenital cardiomyopathy
congenital cataract
congenital cerebellar atrophy
congenital cerebral aneurysm
congenital choreoathetosis
congenital clasped thumb with mental retardation
congenital conus
congenital defect
congenital defect
congenital diaphragmatic hernia
congenital disease
congenital disorder
congenital dyserythropoietic anaemia
congenital dysphagocytosis
congenital dysplasia of the hip
congenital dysplastic angiectasia (current term)
congenital dysplastic angiomatosis
congenital ectodermal defect
congenital ectodermal dysplasia
congenital elephantiasis
congenital epulis of newborn
congenital erythropoietic porphyria
congenital facial diplegia
congenital fibrosis of the extraocular muscles
congenital generalised fibromatosis
congenital giant pigmented nevus
congenital glaucoma
congenital haemolytic anaemia
congenital haemolytic icterus
congenital haemolytic jaundice

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