Umbilical Cord Diseases Affecting Obstetric and Perinatal Outcomes

Abstract

(1) The aim of this article is to describe the physiopathology underlying umbilical cord diseases and their relationship with obstetric and perinatal outcomes. (2) Methods: Multicenter case series of umbilical cord diseases with illustrations from contributing institutions are presented. (3) Results: Clinical presentations of prenatal ultrasound findings, clinical prenatal features and postnatal outcomes are described. (4) Conclusions: Analysis of our series presents and discusses how umbilical cord diseases are associated with a wide variety of obstetric complications leading to a higher risk of poor perinatal outcomes in pregnancies. Knowing the physiopathology, prenatal clinical presentations and outcomes related to umbilical diseases allow for better prenatal counseling and management to potentially avoid severe obstetric and perinatal complications.

Keywords: adverse perinatal outcomes; fetal mortality; maternal–fetal complications; neonatal mortality; perinatal mortality; umbilical cord anomalies.

Figure 1

(a) Two-dimensional ultrasound with HD-Flow color Doppler showing a hypocoiled umbilical cord and (b) a hypercoiled umbilical cord.

Figure 2

Umbilical cord displayed in HDlive Flow. Measurement of sonographic umbilical cord index (UCI): 1/one coil. One coil corresponds to a complete coil of 360° of the umbilical vessels around each other measured in centimeters. Basically, the distance between the inner edge of the umbilical artery wall to the outer edge of the arterial wall itself should be measured up to the next spiral (left–right arrow). Interestingly, in twin–twin transfusion syndrome, the recipient usually displays a hypercoiled cord, probably having been developed as a protective mechanism against volume overload [24] (Figure 2 and Figure 3).

Figure 3

Umbilical cords displayed in HDlive Flow. The cord is hypocoiled if UCI is lower than the 10th centile for gestational age (on the left); hypercoiled if UCI is above the 90th centile for gestational age (on the right).

Figure 4

Umbilical cords displayed in HDlive Flow. Uncoiled cord: two straight umbilical arteries with UCI equal to 0.

Figure 5

Three-dimensional ultrasound with HDlive Flow can detect hypercoiled and uncoiled cord segments in the same fetus.

Figure 6

True umbilical cord knot diagnosed at 21 weeks of gestation using spatiotemporal image correlation (STIC) using HD-Flow (a) and “glass-body” mode in iFlow (b).

Figure 7

Umbilical cord knot associated with notching (white arrows) in the umbilical artery (UA) Doppler waveform. The plausible cause for notching in the UA is constriction: UA diameter section: 2.5 mm (red arrows), UV diameter section: 5.3 mm (yellow calipers). Waveform notching is determined by flow separation induced by constriction. The notching is not present in cases of less than 75% constriction and disappears as the vortex wave is attenuated at distances downstream of the constriction. Notching in the UA Doppler waveform should be considered important even in the absence of increased peak systolic velocity of the fetal mean cerebral artery (MCA) because it might indicate impending intrauterine fetal death (IUFD).

Figure 8

A reduced or absent coiling may be localized, causing a reduction in the diameter of the cord, with a constricting and/or twisting effect (indicated by the curved arrow). This phenomenon is more frequent near the umbilical ring. The reduced or absent coiling may be associated with a reduction in Wharton’s jelly.

Figure 9

Two-dimensional ultrasound and color Doppler. Umbilical cord diameter anomaly: a thick cord depends on the amount of Wharton’s jelly that has formed and deposited in the cord (diameter section (yellow calipers): 25.4 mm). It may be associated with maternal diabetes, fetal hydrops, polyhydramnios, and coiling anomalies (uncoiled/hypocoiled).

Figure 10

Color Doppler and 3D ultrasound with “glass-body” mode. Absent left umbilical artery. Single umbilical artery (SUA) associated with intra-abdominal umbilical vein varix (UVV) and absence of the ductus venosus. The umbilical vein connects to the inferior vena cava in the suprahepatic area. In the presence of a large varix and bidirectional turbulence flow, the risk of developing a thrombus is higher. (Abbreviation. B: bladder).

Figure 11

Two-dimensional ultrasound and color Doppler reveals the presence of four vessels in the umbilical cord. Axial plane of the fetal abdomen highlights the left (LUV) and right (RUV) umbilical veins.

Figure 12

Two-dimensional ultrasound (a) and 3D ultrasound using Crystal Vue showing a fetus with exomphalos associated with a large umbilical cord cyst detected at 16 weeks’ gestation (b).

Figure 13

Umbilical cord angiomyxoma detected at 16 weeks’ gestation. Angiomyxoma is located at the level of the cord insertion into the placenta. Multiplanar display color Doppler and 3D ultrasound with “glass-body” rendering mode shows the angiomyxoma and its effects on the cord vessels. It appears as a rounded echogenic area (indicated by yellow curved arrows) which envelops, squeezes, and sometimes stretches the umbilical vessels.