Objective: The aim of this study was to follow up the 19 infants born in Tyrol province with abdominal wall defects between 1985 and 1996 whose malformation had been diagnosed prenatally, who were operated on immediately postpartum and who are alive today.
Method: There were seven children in the omphalocele group and 12 in the gastroschisis group; 18 parents of affected infants took part in the study.
Results: Four out of seven children with omphalocele had major associated malformations (two Beckwith-Wiedemann syndrome, one porencephalic cyst, one with skeletal defects). These children presented handicaps related to the associated malformations but not to the abdominal wall defect. The three other children with omphalocele are developing normally. Five out of 11 children with gastroschisis had associated intestinal but no extraintestinal malformations. After discharge, ten of 11 children with gastroschisis were developing normally; one child shows signs of mental retardation. Of 14 mothers who had originally planned another pregnancy prior to the birth of the malformed child, nine decided against becoming pregnant again; the others delayed a further pregnancy for several years.
Conclusions: In our group, associated malformations were the main factor affecting the long-term quality of life of children with omphalocele and gastroschisis. Although most of the children were developing normally, fear of a repetition of the malformation in a subsequent pregnancy dominated reproductive choices in all couples.