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March 1968

Picture of the Month

Author Affiliations

From the Boston Floating Hospital, Boston.

Am J Dis Child. 1968;115(3):349-350. doi:10.1001/archpedi.1968.02100010351009

Denouement and Discussion 

Syndrome of Neonatal Hypoglycemia, Macroglossia, Visceromegaly, and Omphalocele 

Manifestations  Hypoglycemia is present in the newborn period and may recur later in life. The infants are usually large at birth but lose weight during the neonatal period and then gain rapidly thereafter and approach gigantism. The tongue is very large and protrudes from a normal-size mouth. The tongue may remain large. An omphalocele or umbilical hernia is a frequent feature of this syndrome. Hepatomegaly and pancreatic hyperplasia may also be present. Some of the patients thus far reported have had mild retardation although others have been normal. Also reported are microcephaly, hemihypertrophy, renal hyperplasia and polycythemia.

Genetics  No specific mode of inheritance has yet been detected.

Treatment  It is important to be on the alert for hypoglycemia during the neonatal period so it may be treated as soon as it is discovered. The omphalocele should be repaired as

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