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True knots of the umbilical cord (UC) are a rare occurrence and are reported in 0.4–1.2% of deliveries. The compression of true knot of the UC can cause obstruction of the fetal circulation, leading to intra-uterine growth retardation or fetal death. Predisposing factors for the genesis of the true UC knot are numerous and include all the conditions, which lead to a relatively large uterine volume. This situation may predispose to free and excessive fetal movements. Although not all true knots lead to perinatal complications, they have been associated with adverse pregnancy outcomes, including fetal distress, fetal hypoxia, intra-uterine growth restriction (IUGR), long-term neurological damage, caesarean delivery and stillbirth. We present a rare case of operative delivery with vacuum in a multiparous woman at term of pregnancy with a double true knot of the UC. As in most cases, the diagnosis was made after delivery, as there were no fetal symptoms during pregnancy. Some authors assume that 3D power sonography may be useful in the diagnosis of true UC knots. However, 3D power Doppler cannot be considered as a definitive method. There are no specific prenatal indications to induce the physician to look for ultrasound signs suggestive of umbilical true knot. Some studies argue that cases of fetal death and fetal risk are directly related to the number of knots. We also support this thesis, even if further observational and retrospective studies are needed to demonstrate it.

Objectives To quantify the risk of true cord of the umbilical cord for perinatal death and identify additional risk factors. Methods This retrospective study included singleton deliveries between 24 and 42 weeks of gestation that took place between 2003 and 2017 in two medical centers. For patients with true knot, data regarding the number of cord knots as well as the location and number of loops of cord were obtained. The rest of the birth data set comprised the control group. The primary outcome was fetal demise. Secondary outcomes included mode of delivery and perinatal outcomes. Results The final data set included 139,458 deliveries, of which 364 cases (0.26%) comprised the study group (true knot group) and 139,094 cases (99.74%) the control group. Higher rated of unfavorable outcomes were found among cases than controls, including perinatal death, delivery mode, lower Apgar and cord blood umbilical artery pH as well as higher rates of NICU admission and perinatal death. Multivariate analysis showed that true knot of cord (aOR 15.46, 95% CI 9.30–25.70) was a strong predictor of perinatal death. Analysis of predictors of perinatal death within the study group showed that only four or more nuchal loops of cord was an independent predictor (four loops OR 13.40 95% CI 1.12–160.34). Conclusions True knot of the umbilical cord is a strong predictor of perinatal death. Fetuses with true knot of cord and four or more nuchal cord loops are at significantly increased risk of perinatal death. If diagnosed before onset of labor, delivery before 37 weeks may prevent perinatal death.

Background This study aimed to estimate population-based frequencies of various fetal heart rate (FHR) evolution patterns in cases with umbilical cord abnormalities and to identify particular FHR evolution patterns associated with different types of umbilical cord abnormalities. Methods We conducted a retrospective cohort study. FHR evolution patterns, evaluating the trend of all FHR tracings from admission to delivery, were retrospectively analyzed and classified into five categories: persistent non-reassuring (p-NR), persistent bradycardia, Hon’s pattern, reactive-prolonged deceleration (PD), and persistent reassuring. The study included pregnant women who delivered after 37 weeks of gestation. Frequencies of the five FHR evolution pattern categories were stratified by the type of umbilical cord abnormalities. Results Among 1,195 participants, 1,074 had no cord abnormalities, and 122 had abnormalities. Overall, the prevalence of FHR patterns in cases with the cord abnormality was: 2% p-NR, 3% Hon’s pattern, 21% reactive-PD, and 74% persistent reassuring. The frequencies of various FHR evolution patterns did not differ significantly between cases with and without umbilical cord abnormalities. However, when analyzing specific cord abnormalities, velamentous cord insertion showed a higher prevalence of p-NR (7% vs. 1%, p  = 0.14) and reactive-PD (40% vs. 17%, p  < 0.05) compared with cases without cord abnormality, along with a lower prevalence of persistence reassuring patterns (53% vs. 79%, p  < 0.05). Conclusion Analysis of FHR evolution patterns in a population-based sample demonstrated a higher occurrence of reactive-PD patterns in cases with umbilical cord abnormalities, which result in sudden deterioration of fetal condition during delivery, compared with cases without umbilical cord abnormalities.

BackgroundTrue knot of the umbilical cord (TKUC) is found in 0.3–2.1% of pregnancies and is associated with an increased risk of adverse perinatal outcomes.MethodsA retrospective cohort study including all singleton pregnancies delivered from 2011 to 2019 was performed. Diagnosis of TKUC was made postnatally, immediately after delivery of the baby. Comparison was made between pregnancies with and without TKUC regarding maternal, fetal and neonatal adverse outcome.ResultsOverall, 867/85,541 (1%) pregnancies were diagnosed with TKUC. Maternal age, BMI, gravidity and parity were significantly higher in pregnancies with TKUC as well as higher rate of induction of labor, meconium-stained amniotic fluid, and delivery prior to 37 weeks. The rate of cesarean deliveries due to non-reassuring-fetal monitor was significantly higher in pregnancies with TKUC. Overall, there were 2.5% IUFD in pregnancies with TKUC vs. 1% in pregnancies without TKUC (p < 0.001). Importantly, the rate of IUFD prior to 37 weeks of gestation was not significantly higher in the group with TKUC, however, the rate of IUFD after 37 weeks of gestation was 10 folds higher in fetuses with TKUC, 0.9% vs. 0.08% (p < 0.001). Significantly, more neonates with TKUC needed phototherapy or suffered from hypoglycemia. There were no differences in the 5 min Apgar scores, admission to the NICU and number of days of hospitalization.ConclusionPregnancies complicated with TKUC are associated with a tenfold higher risk of IUFD beyond 37 weeks of gestation. To the results of this study suggest that it would be prudent to induce labor around 37 weeks of gestation in pregnancies with prenatal diagnosis of TKUC. It may be warranted to use continuous fetal monitoring during labor and delivery in those cases were antenatal diagnosis of TKUC is made.

Purpose To compare perinatal outcomes between active and routine management in true knot of the umbilical cord (TKUC). Methods A retrospective study of singletons born beyond 22 6/7  weeks with TKUC. Active management included weekly fetal heart rate monitoring(FHRM) ≥ 30 weeks and labor induction at 36–37 weeks. Outcomes in active and routine management were compared, including composite asphyxia-related adverse outcome, fetal death, labor induction, Cesarean section (CS) or Instrumental delivery due to non-reassuring fetal heart rate (NRFHR), Apgar 5 score < 7, cord Ph < 7, neonatal intensive care unit (NICU) admission and more. Results The Active (n = 59) and Routine (n = 1091) Management groups demonstrated similar rates of composite asphyxia-related adverse outcome (16.9% vs 16.8%, p = 0.97). Active Management resulted in higher rates of labor induction < 37 weeks (22% vs 1.7%, p < 0.001), CS (37.3% vs 19.2%, p = 0.003) and NICU admissions (13.6% vs 3%, p < 0.001). Fetal death occurred exclusively in the Routine Management group (1.8% vs 0%, p = 0.6). Conclusion Compared with routine management, weekly FHRM and labor induction between 36 and 37 weeks in TKUC do not appear to reduce neonatal asphyxia. In its current form, active management is associated with higher rates of CS, induced prematurity and NICU admissions. Labor induction before 37 weeks should be avoided.

Background Intestinal knot formation, in which two segments of the intestine become knotted together, can result in intestinal obstruction. An ileo-ileal knot refers to knot formation between two ileal segments and is a very rare benign disease. We report a case of strangulated bowel obstruction caused by true ileo-ileal knot formation. Case presentation An 89-year-old woman was referred to our hospital with the diagnosis of intestinal obstruction. Contrast-enhanced computed tomography revealed the small bowel forming a closed loop, with poor contrast effect. Based on the findings, the patient was diagnosed as having strangulated bowel obstruction, and emergency surgery was performed. At laparotomy, two segments of the ileum were found to be tied together forming a knot, and both segments were necrotic. Although it was necessary to release the strangulated small bowel, we did not immediately release the knot, but first proceeded with ligation of the mesenteric vessels to the strangulated small bowel to prevent dissemination of toxic substances from the necrotic bowel into the systemic circulation. The surgery was completed with resection of the necrotic ileum and anastomosis of the small intestine. The postoperative course was uneventful, and the patient was discharged home. Conclusion We encountered a case of strangulated bowel obstruction caused by true ileo-ileal knot formation. Resection of the necrotic small intestine without releasing the knot could be performed safely, and might be considered as an option of surgical procedure.

Diagnosis of potential umbilical cord compromise, namely, true knots of the umbilical cord and nuchal cords has been enabled with increasing accuracy with current enhanced prenatal sonography. Often an incidental finding at delivery, the incidence of true knots of the umbilical cord has been estimated at between 0.04% and 3% of deliveries. This condition has been reported to account for a 4 to 10-fold increase of stillbirth and perinatal morbidity of 11% of cases. Nuchal cords, commonly observed at the delivery of uncompromised, non-hypoxic non-acidotic newborns occur more frequently with single nuchal cords noted in between 20% and 35% of all deliveries at term. Multiple nuchal cords are considerably less frequent, with decreasing frequencies inverse to the number of nuchal cord loops. While clearly single (and likely double) nuchal cords are almost uniformly associated with favorable neonatal outcomes, emerging data suggest that cases of ≥3 loops of nuchal cords are more likely to be associated with an increased risk of adverse perinatal outcome (either stillbirth or compromised neonatal condition at delivery). We define cases of a true knot of the umbilical cord, cases of ≥3 loops of nuchal cords, any combination of a true knot and nuchal cord, or any umbilical cord entanglement (nuchal or true knot) in the presence of a single umbilical artery, in singleton gestations as complex umbilical cord entanglement. Two concurrent developments, the increase in accuracy of prenatal sonographic diagnosis of complex umbilical cord entanglement and recent data confirming fatal compromise of the umbilical circulation in approximately 20% of cases of stillbirth, suggest that establishing governing body guidelines for reporting of potential umbilical cord compromise, and recommendation of consideration for early-term delivery of select cases, may be warranted. This commentary will address current perspectives of prenatal diagnosis and clinical management challenges of complex umbilical cord entanglement.

Umbilical cord accidents preceding labor are rare. Single and multiple nuchal cords, and true knot(s) of the umbilical cord, are often incidental findings noted at delivery of non-hypoxic non-acidotic newborns without any evidence of subsequent adverse neonatal outcome. In contrast to single nuchal cords, true knots of the umbilical cord, which occur in between 0.04% and 3% of all deliveries, have been associated with a reported 4 to 10 fold increased risk of stillbirth. First reported with real-time ultrasound, current widespread application of color Doppler, power Doppler and three-dimension sonography, has enabled increasingly more accurate prenatal sonographic diagnoses of true knot(s) of the umbilical cord. Reflecting the inability to visualize the entire umbilical cord at prenatal ultrasound assessment, despite detailed second and third-trimester scanning, many occurrences of incidental true knot of the umbilical cord remain undetected and are noted only at delivery. Although prenatal sonographic diagnostic accuracy is increasing, false positive sonographic diagnosis of true knot of the umbilical cord cannot be ruled out with certainty, and must continue to be considered clinically. Notwithstanding the inability to diagnose all true knots, currently there is a clear absence of clinical management guidelines by governing bodies regarding patients in whom prenatal sonographic diagnosis of true knot(s) of the umbilical cord is / are suspected. As a result, in many prenatal ultrasound units, suspected sonographic findings suggestive of or consistent with true knot of the umbilical cord are often disregarded, not documented, and patients are not uniformly informed of this potentially life-threatening condition, which carries an associated considerable risk of stillbirth. This commentary will address current perspectives of prenatal sonographic diagnostic and management challenges associated with true knot(s) of the umbilical cord in singleton pregnancies.

We aimed to study both the short- and long-term neurological implications in offspring born with confirmed knotting of the umbilical cord—“true knot of cord”. In this population based cohort study, a comparison of perinatal outcome and long-term neurological hospitalizations was performed on the basis of presence or absence of true knot of cord. A Kaplan–Meier survival curve was constructed to compare the cumulative incidence of neurological hospitalizations between the study groups. Multivariable regression models were used to assess the independent association between true knot of cord, perinatal mortality and long term neurological related hospitalizations, while controlling for potential confounders. The study included 243,639 newborns, of them 1.1% (n = 2606) were diagnosed with true knot of the umbilical cord. Higher rates of intrauterine fetal demise (IUFD) were noted in the exposed group, a finding which remained significant in the multivariable generalized estimation equation, while controlling for confounders. The cumulative incidences of neurological hospitalizations over time were comparable between the groups. The Cox regression confirmed a lack of association between true knot of cord and total long term neurological related hospitalizations. While presence of true knot of the umbilical cord is associated with higher IUFD rates, in our population, however, its presence does not appear to impact the long term neurological health of exposed offspring.

Umbilical cord knots are very rare cases. As opposed to single loop knots, double-and more loop knots are extremely rare. When they occur, adverse outcomes become inevitable in most cases. We present this rare case diagnosed coincidentally on cesarean section at term with good fetal outcome.