Vater association and anorectal malformations.
In a twenty-year series of 208 patients with anorectal malformations there were 39 infants with three or more Vater association anomalies. The neonatal mortality of Vater association patients was 28%; beyond the neonatal period only two patients died. Most early deaths were associated with the combination of cardiac anomalies and oesophageal atresia. The incidence of prematurity (30%) and lethal anomalies (15%) was high. High and complex anorectal anomalies were frequent (85%) and some kind of urogenital malformation was present in 95% of the patients. Despite the grave early prognosis and multiple hospitalisations and surgical procedures required in the management of these patients, the long-term outlook and quality of life does not differ significantly from other patients with corresponding anorectal anomalies.
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000601:surgery; D000293:Adolescent; D001003:Anal Canal / Q000002:abnormalities* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D004933:Esophageal Atresia / Q000175:diagnosis; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007235:Infant, Premature, Diseases / Q000175:diagnosis*; D007668:Kidney / Q000002:abnormalities; D008297:Male; D011183:Postoperative Complications / Q000401:mortality; D012007:Rectum / Q000002:abnormalities* / Q000601:surgery