SYNDROME OF HYPERPLASTIC FETAL VISCEROMEGALY AND NEONATAL HYPOGLYCEMIA (BECKWITH'S SYNDROME)

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Abstract
The syndrome of hyperplastic visceromegaly (Beckwith's syndrome) was studied clinically in three patients and at autopsy in four patients. The findings in these cases were compiled with those in 31 previously reported cases. Clinical features in decreasing order of frequency were omphalocele, macroglossia, increased birth weight, nephromegaly, facial flame nevus, and hepatomegaly. Neonatal hypoglycemia has been chemically documented in only 18% of the cases but is likely to be one of the most common features of the syndrome. Pathologic features include adrenal cortical cytomegaly, renal medullary dysplasia, pancreatic islet hyperplasia, adrenal cortical cysts, pancreatic acinar hyperplasia, diaphragmatic hernias, and persistent glomerulogenesis in decreasing order of frequency. Two of the three surviving patients in this series were mentally retarded at 13 and 8½ years of age respectively. Four patients studied at autopsy disclosed, in addition to features common to the syndrome, many incidental developmental defects of the cardiovascular system, the genitourinary system, the gastrointestinal tract, and other organ systems. The significance in recognition of this syndrome lies in the sometimes disastrous effects of neonatal hypoglycemia. In those patients who survive beyond the neonatal period, the possible development of renal or adrenal malignancy should be borne in mind.

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