Department of Pathology, Diakonissenkrankenhaus Flensburg, Flensburg, Germany.
Department of Pathology, Hannover Medical School, Hannover, Germany.
Pediatr Dev Pathol. 2020 Mar-Apr;23(2):107-114. doi: 10.1177/1093526619865426. Epub 2019 Jul 25.
This study focused to investigate a possible association of extensive umbilical hypercoiling (displaying an umbilical coiling index [UCI] of at least 1.0 coils/cm), clinical outcome, and associated pathoanatomical placental lesions. Of the 771 singleton placentas from the second and third trimesters submitted for pathoanatomical evaluation, 15 cases (2%) displayed extensive hypercoiling. There was an association of excessive hypercoiling with hypotrophy of fetuses and children (11 cases) and fetal demise (12 cases). Thin cord syndrome and umbilical stricture were observed in 9 cases and 4 cases, respectively. Seven of the 15 cases with excessive umbilical hypercoiling showed increased placental fibrin deposition (47% of the cases with hypercoiling), in 4 cases sufficient for rendering the diagnosis of massive perivillous fibrin deposition. Signs of maternal vascular malperfusion (n = 6) and chorangiosis (n = 2) were also detected in cases with hypercoiling. Recurrence of excessive umbilical hypercoiling was observed in 2 families, suggesting a genetic predisposition for the development of this lesion. Extensive hypercoiling could be a hitherto underrecognized pathogenetic factor for the development of massive perivillous fibrin deposition. A high UCI measured in the second trimester by ultrasound may be predictive of fetal hypotrophy, and intensified fetal monitoring is warranted, particularly if there is a history of hypercoiling and adverse fetal outcome.
本研究旨在探讨广泛的脐绕颈(脐绕颈指数[UCI]至少为 1.0 圈/cm)与临床结局及相关的胎盘病理损伤之间的可能关联。在提交进行病理评估的 771 例来自第二和第三个孕期的单胎胎盘中有 15 例(2%)表现为广泛的脐绕颈过度。过度绕颈与胎儿和儿童的生长迟缓(11 例)和胎儿死亡(12 例)有关。在 9 例中观察到脐带过细综合征,在 4 例中观察到脐狭窄。15 例脐带过度绕颈中有 7 例显示胎盘纤维蛋白沉积增加(绕颈病例的 47%),足以诊断为大量绒毛膜外纤维蛋白沉积。在绕颈病例中还发现了 6 例母体血管功能不全(n=6)和 2 例绒毛膜血管瘤(n=2)的迹象。在 2 个家族中观察到了过度的脐绕颈复发,提示该病变的发展存在遗传易感性。广泛的脐绕颈过度可能是导致大量绒毛膜外纤维蛋白沉积的一个迄今被低估的发病因素。超声在孕中期测量的高 UCI 可能预示着胎儿生长迟缓,需要加强胎儿监测,特别是如果有绕颈病史和不良的胎儿结局。