无脾综合征是先天性脾脏缺如伴多个内脏畸形和心血管畸形的综合征,其发病率约占出生的1/40000[1]。该病临床罕见,预后极差。现将我院近年收治的一例报道如下。1病例患儿,女,13min,因“早产低体重生后13min”入院。患儿为第2胎第1产,孕36+1w,顺产娩出,出生体重2.6kg,Apgar评分:1min、5min、10min均评10分,羊水、胎盘、脐带均未见明显异常。患儿生后不久即出现口周发绀,给 Spleen-free syndrome is a congenital spleen absent syndrome with multiple visceral malformations and cardiovascular malformations, the incidence of approximately 1/40000 birth [1]. The disease is clinically rare, the prognosis is poor. Now in our hospital admitted in recent cases are as follows. 1 case of children, female, 13min, due to “premature low birth weight 13min ” admission. Children with the first 2 births, 36 + 1w pregnant, delivered by natural delivery, birth weight 2.6kg, Apgar score: 1min, 5min, 10min were rated 10 points, amniotic fluid, placenta, umbilical cord were no significant abnormalities. Perioral cyanosis occurs shortly after birth