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December 1985

Aplasia Cutis Congenita and Lymphangiectasia

Author Affiliations

Department of Pediatrics University of Texas Medical School at Houston PO Box 20708 Houston, TX 77225

Am J Dis Child. 1985;139(12):1178. doi:10.1001/archpedi.1985.02140140012004

Sir.—I noted with interest the report by Bronspiegel et al of the association between aplasia cutis congenita and intestinal lymphangiectasia.1 Table 2 in that article described clinical phenomena associated with intestinal lymphangiectasia and included a claimed association between Turner's syndrome and intestinal lymphangiectasia. The article cited to support that association was given as "Benson PF: Turner syndrome with intestinal lymphangiectasia. Arch Dis Child 1965;40:27."

An article by Benson and his associates entitled "Lymphangiography and Chromosome Studies in Females with Lymphoedema and Possible Ovarian Dysgenesis" appeared on page 27 of volume 40 of the Archives of Diseases in Childhood in 1965.2 This report contained details of lymphangiography in seven cases of Turner's syndrome, but nowhere did it mention intestinal lymphangiectasia.

Bronspiegel et al correctly pointed out the association between Noonan syndrome and intestinal lymphangiectasia. Considering the similarity between Noonan's and Turner's syndromes, the finding of intestinal lymphangiectasia in a patient with