Pathoanatomical Lesions in Placentas With Excessively Hypercoiled Umbilical Cords: Frequent Detection of Massive Perivillous Fibrin Deposition

Abstract

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.

Cesarean section per se is not a risk factor for non‐anatomical placenta creta

“…24 It should be stressed that the focal and diffuse post-uterine hypoxic placental patterns do not correlate. In the current study, we did not observe an increased incidence of such placental features of umbilical cord compromise as massive perivillous fibrin deposition or placental maturation defect, the lesions reported to be associated with umbilical cord hypercoiling, 22 despite using E-cadherin/CD34 immunostaining to highlight the latter. 19 Features of shallow placental implantation were also reported in both subsets of pre-eclampsia (early onset and late onset), reflecting the underlying common pathological mechanisms thereof, while hypoxic lesions and patterns were more common in early-onset pre-eclampsia, which correlates with a more severe clinical outcome of this subtype.…”

Fetal akinesia deformation sequence and massive perivillous fibrin deposition resulting in fetal death in six fetuses from one consanguineous couple, including literature review

“…Of these, 12 could not be included because no full text was available. After reading the full text of all of the remaining articles, 63 were included (Abdulghani et al, 2017 ; Achuthan et al, 2017 ; Adams‐Chapman et al, 2002 ; Al‐Adnani et al, 2008 ; Al‐Sahan et al, 2014 ; Ananthan et al, 2019 ; Andres et al, 1990 ; Bane & Gillan, 2003 ; Batcup et al, 1985 ; Bendon & Hommel, 1996 ; Benirschke et al, 2000 ; Brown et al, 2002 ; Chaiworapongsa et al, 2016 ; Chang et al, 2006 ; Chisholm et al, 2016 ; Clewell & Manchester, 1983 ; da Cunha Castro & Popek, 2007 ; Devisme et al, 2017 ; Eom et al, 2008 ; Faye‐Petersen et al, 2018 ; Feist et al, 2015 , 2019 , 2020 ; Gao et al, 2021 ; Gestrich et al, 2020 ; Gibbins et al, 2020 ; Griffin et al, 2012 ; Gupta et al, 2004 ; Hannaford et al, 2019 ; He et al, 2018 ; Heller et al, 2016 ; Hung et al, 2006 ; Jaiman et al, 2020 ; Katz et al, 1987 , 2002 ; Kim et al, 2019 ; Leavey et al, 2019 ; Leong et al, 2013 ; Linn et al, 2013 ; Makino et al, 2004 ; Maloney & Baergen, 2010 ; Man et al, 2016 ; Mandsager et al, 1994 ; Matern et al, 2001 ; Minamoto et al, 2019 ; Mongula et al,…”

“…Other congenital anomalies reported were renal anomalies in seven cases with contractures in four out of seven (Chang et al, 2006;Leong et al, 2013;Linn et al, 2013;Taweevisit & Thorner, 2010), three cases of mutation in LCHAD (Griffin et al, 2012;Matern et al, 2001) pulmonary valve dysplasia (Montenegro et al, 1997). The combination of MPFD with hypercoiling of the umbilical cord was reported in six cases (Feist et al, 2020;Gestrich et al, 2020;Taweevisit & Thorner, 2016). It is remarkable that borderline MPFD resulted in live born infants in the majority (84/89) and except for 1 trisomy 21, no other congenital or chromosomal anomalies were reported.…”

“…Other congenital anomalies reported were renal anomalies in seven cases with contractures in four out of seven (Chang et al, 2006 ; Leong et al, 2013 ; Linn et al, 2013 ; Taweevisit & Thorner, 2010 ), three cases of mutation in LCHAD (Griffin et al, 2012 ; Matern et al, 2001 ), one osteochondral junction lesions (da Cunha Castro & Popek, 2007 ), one aplasia of the left diaphragm, and one pulmonary valve dysplasia (Montenegro et al, 1997 ). The combination of MPFD with hypercoiling of the umbilical cord was reported in six cases (Feist et al, 2020 ; Gestrich et al, 2020 ; Taweevisit & Thorner, 2016 ).…”

Hiperenrollamiento con torsión del cordón umbilical como causa de muerte fetal: reporte de caso

“…En el cordón umbilical se observa torsión con estrechez principalmente en la unión feto-umbilical 4 , y con menos frecuencia se puede observar en el extremo placentario o en asa libre, y en algunos casos la gelatina de Wharton está ausente. En el estudio histológico se observa estenosis con obliteración en la luz de los vasos del cordón umbilical en el segmento afectado, junto con formación de trombosis, la cual se presenta como signo de mala perfusión fetal 2 .…”