Children with Anatomical Congenital Anomalies; a Portrait Follow-up over five years

Annually some 5.000 newborns (2 - 3% of all births) in the Netherlands present with major anatomical congenital anomalies.140,395 The causes of most of these are still unknown. So far, genetic or chromosomal causes have been implicated in 13 - 14% of all anomalies,17,120,214,249,337 and environmental factors, including maternal disease, can explain another 6 - 7%.149,345,472 Some 20% are known to be multifactorial. Many of these anomalies require immediate surgical correction in order for these children to survive. The pediatric surgeon Ravitch classified six of these anomalies as the so called index diagnoses.351 These are esophageal atresia/tracheo-esophageal fistula, congenital diaphragmatic hernia, intestinal atresias, Hirschsprung's disease, anorectal malformations, and abdominal wall defects (gastroschisis and omphalocele). They occur either in isolated form or as part of more complex syndromes.59 Multiple congenital anomalies refers to any combination of two or more major anomalies. As a governmental policy patients presenting with an index diagnosis are to be treated in one of six pediatric surgical centers in the Netherlands.170 Improved antenatal detection, surgical techniques, and peri-operative care have increased these children's chances of survival.210 Survivors may show considerable morbidity, however, and many will have to rely on the healthcare system for life.

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