Denouement and Discussion
Syndrome of Neonatal Hypoglycemia, Macroglossia, Visceromegaly, and Omphalocele
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.
No specific mode of inheritance has yet been detected.
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