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December 1985

Aplasia Cutis Congenita and Lymphangiectasia

Author Affiliations

Department of Pediatrics Walter Reed Army Medical Center Washington, DC 20307-5001

Am J Dis Child. 1985;139(12):1178-1179. doi:10.1001/archpedi.1985.02140140012006

Sir.—Having recently cared for three nonrelated infants with different presentations of aplasia cutis congenita (ACC), we read with much interest the article by Bronspiegel et al1 concerning the association of ACC and intestinal lymphangiectasia. The tragic outcome in their second patient and our review of the literature and successful treatment of a similar infant prompted this letter.

The second patient of Bronspiegel et al was an infant with a large midline scalp and bony defect that exposed the superior sagittal sinus. According to the abstract, the infant was 2 months old when he died "from sudden massive bleeding from the superior sagittal sinus." Not included in the case report was vital information on the size of the defect, whether any treatment was used, and, most impor