Rintala R, Lindahl H, Louhimo I
Z Kinderchir. 1986 Feb;41(1):22-6. doi: 10.1055/s-2008-1043301.
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.
在一项对208例肛门直肠畸形患者长达20年的研究中，有39例婴儿存在三种或更多与Vater综合征相关的异常情况。Vater综合征患者的新生儿死亡率为28%；新生儿期过后仅有2例患者死亡。大多数早期死亡与心脏异常和食管闭锁的合并情况有关。早产发生率（30%）和致命性异常发生率（15%）较高。高位和复杂的肛门直肠畸形很常见（85%），95%的患者存在某种泌尿生殖系统畸形。尽管这些患者早期预后严重，治疗过程中需要多次住院和手术，但长期前景和生活质量与其他患有相应肛门直肠畸形的患者相比并无显著差异。
