Exomphalos (syn. omphalocele). A review of 45 cases.

In rare anomalies personal experience of a few patients can lead to biased or incorrect impressions unless analysis of a larger series is applied as a corrective. The result should be a better appreciation of the difficulties to be met and overcome in the future. A congenital hernia into the base of the umbilical cord is known as an exomphalos in British usage and an omphalocele in American usage. Smaller examples are readily repaired but the largest constitute one of the greatest challenges in paediatric surgery. Other serious malformations are often present, frequently cause complications and may vitiate the results of surgery. The earliest description of an exomphalos is found in the writings of Ambroise Pare (1510-1590) who recognized its serious nature and poor prognosis (1634). Mery (1716) wrote ' . . a yet more extensive degree ... in which the very nature of the case seems to preclude all hope of assistance from the art of surgery. The dissection of such cases has shown the liver, stomach, spleen, omentum, large and small intestines lying in the umbilical hernia'. The first reports of successful surgical treatment are those of Hey (1803) and Hamilton (1806). Scarpa (1812) drew attention to the association of exomphalos with other malformations and concluded that the condition was almost always fatal because of them or because the hernia was too large to be reduced. Ahlfeld (1899) used alcohol dressings on the sac in a patient successfully treated without operation.