O'Marcaigh A S, Folz L B, Michels V V
Department of Pediatrics, Mayo Clinic, Rochester, MN 55905.
Pediatrics. 1992 Jul;90(1 Pt 1):47-9.
Malformations of the umbilicus are a feature of many dysmorphic syndromes including Rieger syndrome, Robinow syndrome, and Aarskog syndrome. The characteristic umbilical malformation in Rieger syndrome consists of redundant periumbilical skin which extends along the cord for an excessive distance. Although the measurement of umbilical skin length plays an important role in the neonatal diagnosis of Rieger syndrome, normal values for this measurement in healthy neonates have not been established. Umbilical skin length was measured in 104 healthy neonates. The length to which the umbilical skin extended along the cranial aspect of cord (mean 11.53 mm, SD 3.58) was significantly longer than the umbilical skin length along the caudal aspect (mean 8.71 mm, SD 2.89) (P less than .05). Multiple regression analysis revealed a significant association between age and umbilical skin length. Birth weight, length, and gestational age were not significantly associated with umbilical skin length when adjusted for the other three variables. No significant differences in umbilical skin length were observed between male and female groups. The above normal values should aid in the neonatal diagnosis of Rieger syndrome, and furthermore it is recommended that cranial umbilical skin length measurement be included in the examination of the dysmorphic child.
脐部畸形是许多畸形综合征的特征，包括里格尔综合征、罗宾诺综合征和阿斯克格综合征。里格尔综合征的典型脐部畸形表现为脐周皮肤冗余，沿脐带延伸过长。虽然脐部皮肤长度的测量在里格尔综合征的新生儿诊断中起着重要作用，但健康新生儿该测量的正常值尚未确定。对104例健康新生儿进行了脐部皮肤长度测量。脐部皮肤沿脐带头侧延伸的长度（平均11.53毫米，标准差3.58）明显长于沿尾侧的脐部皮肤长度（平均8.71毫米，标准差2.89）（P小于0.05）。多元回归分析显示年龄与脐部皮肤长度之间存在显著关联。在对其他三个变量进行校正后，出生体重、身长和孕周与脐部皮肤长度无显著关联。男女组之间脐部皮肤长度未观察到显著差异。上述正常值应有助于里格尔综合征的新生儿诊断，此外，建议在畸形儿童检查中包括测量头侧脐部皮肤长度。
