An international survey of gastroschisis and omphalocele (490 cases)

Abstract

An international survey of gastroschisis and omphalocele by questionnaire yeilded 490 cases from 16 pediatric surgery centers on four continents. Factors influencing the management outcome of 203 cases of gastroschisis and 287 cases of omphalocele in the period 1954–1977 are reviewed. In 81% of the gastroschisis cases, the defect was 3 cm or larger in diameter; in only 6% was it as dangerously small as 1 cm. In 58% of cases, only intestines were eviscerated; stomach was included in the eviscerated mass in another 36% and genitourinary organs seldom (6%) were included. In this comparatively early experience with gastroschisis, the operative mortality was 39% but varied widely by and within areas (from 10%–75%). The lowest mortality was associated with primary and skin flap closures. The use of a variety of protheses to cover the eviscerated mass was associated with a high incidence of sepsis and intestinal fistulation. There were 246 cases of non-syndrome omphalocele and 41 cases of syndrome omphalocele. In the non-syndrome omphaloceles, the defect was 2.5 cm or larger in 80% and over 5 cm in 46%. Liver was present in 91% of the large omphalocele sacs, 43% of the intermediate-size sacs and none of the small sacs. Management mortality in non-syndrome omphalocele was 37%. It was lowest in Australia, where only sac painting and primary closure were employed. In syndrome omphalocele mortality was highest (68%) in lower midline syndrome, intermediate (33%) in upper midline syndrome and lowest (0%) in Beckwith-Wiedemann syndrome. The presence of multiple and serious additional malformations was the major factor in omphalocele management mortality. The incidence of additional malformations in small (under 2.5 cm) and large (over 5 cm) omphaloceles was identical (45% vs 46%).