Developmental pathogenesis of short palpebral fissure length in children with fetal alcohol syndrome.
Developmental pathogenesis of short palpebral fissure length in children with fetal alcohol syndrome.
复制标题
胎儿酒精综合征儿童睑裂长度短的发育发病机制。
DOI：
10.1002/bdra.20585
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发表时间：
2009
期刊：
影响因子：
--
通讯作者：
Chambers,ChristinaD
中科院分区：
文献类型：
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作者：
Jones,KennethLyons;Hoyme,HEugene;Robinson,LutherK;delCampo,Miguel;Manning,MelanieA;Bakhireva,LudmilaN;Prewitt,LelaM;Chambers,ChristinaD
背景：从正常胚胎发育的角度来看，睑裂通常被认为是由并依赖于发育中的胎儿大脑额叶区域的视神经囊的形成。有人认为，短睑裂是前脑发育特定区域潜在缺陷的反映。另外，短睑裂见于与小枕额围（OFC）相关的多种畸形综合征，如胎儿酒精综合征（FAS），可能是小头畸形的反映。本研究的目的是检查短睑裂是否独立于OFC或由OFC决定。方法对273例FAS患儿、272例具有FAS部分特征的患儿和385例无FAS结构特征的患儿进行特异性睑裂长度（PFL）和OFC百分位的相关性分析。结果在所有三个研究组中，OFC和PFL的百分位数具有统计学意义，但相关性较弱。在FAS患儿中，OFC仅占PFL总变异的10.2% （p= 0.0001）。在具有FAS某些特征的儿童（r2= 0.142;p= 0.0001）和没有FAS结构特征的儿童（r2= 0.110;p= 0.0001）中也观察到类似的模式。结论伴有和不伴有FAS特征的患儿的颞裂长度与枕额围围无关。短睑裂可能很好地反映了前脑发育的缺陷，而不是作为小头畸形的反映而成比例地缩小。出生缺陷研究（甲）2009。©2009 Wiley‐Liss, Inc。
BACKGROUNDFrom the standpoint of normal embryologic development, the palpebral fissures are generally considered to be determined by and dependent on the underlying optic vesicles, outpouchings of the frontal area of the developing fetal brain. It has been suggested that short palpebral fissures are a reflection of an underlying defect in specific areas of forebrain development. Alternatively, short palpebral fissures, seen in a number of multiple malformation syndromes associated with small occipitofrontal circumference (OFC), such as the fetal alcohol syndrome (FAS), might be proportionally small as a reflection of the microcephaly. The purpose of this study was to examine whether short palpebral fissures are independent of or determined by the OFC.METHODSAge‐specific palpebral fissure length (PFL) and OFC centiles were correlated in 273 children with FAS, 272 children with some features of FAS, and 385 children with no structural features characteristic of FAS.RESULTSThe OFC and PFL centiles demonstrated a statistically significant but weak correlation in all three study groups. Among children with FAS, only 10.2% of the total variation in PFL could be accounted for by OFC (p= 0.0001). A similar pattern was observed for children with some features of FAS (r2= 0.142;p= 0.0001) and children with no structural features of FAS (r2= 0.110;p= 0.0001).CONCLUSIONSPalpebral fissure length is predominately independent of occipitofrontal circumference in children with and without features of FAS. Short palpebral fissures may well reflect a defect in forebrain development rather than being proportionally reduced in size as a reflection of microcephaly. Birth Defects Research (Part A) 2009. © 2009 Wiley‐Liss, Inc.