True omphalocele may best be described as a herniation of abdominal viscera through the umbilicus, in which the sac is composed only of a clear, thin membrane. O'Leary and Clymer1 summarized several reports of collected cases and concluded that the incidence is approximately 1 in 3,500 births. Their review of the literature indicated that omphalocele is twice as common among males.
Although most authors believe that there is little tendency toward a familial incidence of this anomaly, several reports of true omphalocele among siblings have been uncovered. Paucot and Gellé2 in 1936 recorded three successive stillborn fetuses of a syphilitic mother, who had large omphaloceles. McKeown, MacMahon, and Record3 found one instance of omphalocele among siblings in a study of 62 families in which this condition existed. A thorough search of the literature would indicate that the patients reported herein are the only ones in which large
ROTHENBERG RE, BARNETT T. Omphalocele in SiblingsReport of Two Cases with Surgical Recovery. AMA Arch Surg. 1957;75(1):131-134. doi:10.1001/archsurg.1957.01280130137024