The benefit of active management in true knot of the umbilical cord: a retrospective study.

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), Apgar5 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.

Hernia of Umbilical Cord: An Enigma.

Introduction: Hernia of the umbilical cord (HUC) is an uncommon problem which is often misdiagnosed, leading to inadequate treatment and various complications which cause increased morbidity and mortality in neonates. To address this issue, we took up this study. Materials and Methods: Patients diagnosed with HUC from January 1, 2017, to December 31, 2021, were retrospectively analyzed. The following data of all these patients were collected and retrospectively analyzed: demography, radiological investigations, echocardiography, contents of hernia, type of surgery performed, and outcome. Results: Eighteen out of 19 patients included in the study were taken up for surgery after baseline investigations and echocardiography. Out of 19 patients, 15 were discharged successfully and are on regular follow-up. Of the remaining four patients, an anastomotic leak occurred in two and they went into sepsis and succumbed. One of the patients died before any intervention and one succumbed on 3rd postoperative day due to sepsis. Conclusion: Timely referral and intervention can save precious lives. We need to educate doctors and health-care providers so that proper diagnosis and timely management can be done for this anomaly which is associated with less morbidity and a lower rate of associated anomalies.

Hemangioma of the umbilical cord: A case report on a rare entity.

Several hundred cases of placental hemangiomas have been reported in the literature. However, the umbilical cord is extremely uncommon as a site of occurrence. We present a case of postnatal discovery of giant hemangioma of the umbilical cord (HUM) in a Coronavirus Disease 2019 (COVID 19) positive mother. To our knowledge, this is the first reported case of HUM synchronous to a maternal infection with COVID 19. We aim, through this case and a review of the literature, to study the clinicopathological characteristics of this singular entity. Our patient, a 37-year-old woman, presented to the Department Of Obstetrics And Gynecology for respiratory distress and loss of fetal movements. Ultrasound examination concluded to intrauterine fetal desmise. After stabilization of the patient, a cesarean section was performed. A macerated fetus was extracted. Placenta showed a giant mass attached to the cord. It was submitted for pathological examination. Gross examination showed that the umbilical cord was inserted eccentrically with a fusiform dilation. Near its placental end, three cohesive solid angiomatous nodules were noted. Microscopic examination revealed lobules of dilated blood-filled capillaries set in a myxoid stroma. The diagnosis of HUM have been established. HUM arise from endothelial cells of the umbilical vessels. Their etiology, physio-pathology and pathways of tumorigenesis are not yet well defined. Further studies are needed to explore the pathways of tumorigenesis and to determin the implication of COVID-19 in HUM.

Current Perspectives of Prenatal Sonographic Diagnosis and Clinical Management Challenges of Complex Umbilical Cord Entanglement.

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.

Current Perspectives of Prenatal Sonographic Diagnosis and Clinical Management Challenges of True Knot of the Umbilical Cord.

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.

Multiple Family Members With Delayed Cord Separtion and Combined Immunodeficiency With Novel Mutation in IKBKB.

Background: Inhibitor of kappa kinase 2 (IKK2) deficiency is a recently described combined immunodeficiency. It undermines the nuclear factor-kappa B (NF-κB) activation pathway. Methods: The clinical and immunological data of four patients diagnosed with combined immunodeficiency (CID) from two related Saudi families were collected. Autozygosity mapping of all available members and whole exome sequencing of the index case were performed to define the genetic etiology. Results: The patients had early onset (2-4 months of age) severe infections caused by viruses, bacteria, mycobacteria, and fungi. They all had hypogammaglobulinemia and low absolute lymphocyte count. Their lymphocytes failed to respond to PHA mitogen stimulation. A novel homozygous non-sense mutation in the IKBKB gene, c.850C>T (p. Arg284*) was identified in the index patient and segregated with the disease in the rest of the family. He underwent hematopoietic stem cell transplantation (HSCT) from a fully matched sibling with no conditioning. The other three patients succumbed to their disease. Interestingly, all patients had delayed umbilical cord separation. Conclusion: IKK2 deficiency causes CID with high mortality. Immune reconstitution with HSCT should be considered as early as possible. Delayed umbilical cord separation in CID patients may be a clue to IKK2 deficiency.

A disadvantage of cesarean section en caul: Umbilical velamentous insertion, a risk factor and proposed mechanism of neonatal anemia.

Cesarean section en caul could cause neonatal anemia, but the mechanism remains unknown. We demonstrate an association between neonatal anemia and velamentous insertion of the umbilical cord in cesarean section en caul, and suggest a way to make this procedure safer. We performed cesarean section en caul, but the placenta and the membrane sac were delivered separately. The neonate was severely anemic. The umbilical cord was attached to the membrane and the blood vessel connecting the umbilical cord and placenta was torn. The amniotic membrane covering the placental surface had peeled away. Velamentous insertion of the umbilical cord could be a cause of neonatal anemia associated with cesarean section en caul.

Genes and transcription factors related to the adverse effects of maternal type I diabetes mellitus on fetal development.

PURPOSE: Maternal type I diabetes mellitus (T1DM) increases the risk of adverse pregnancy outcomes, but the corresponding mechanism is unclear. This study aims to investigate the mechanism underlying the adverse pregnancy outcomes of maternal T1DM. METHODS: Gene expression microarray (GSE51546) was down-loaded from the Gene Expression Omnibus. This dataset included 12 umbilical cord samples from the newborns of T1DM mothers (T1DM group, N = six) and non-diabetic mothers (control group, N = six). RESULTS: Consequently, 1051 differentially expressed genes (DEGs) were found between the two groups. The up-regulated DEGs enriched in 30 KEGG pathways. HLA-DPA1, HLA-DMA, HLA-DMB, HLA-DQA1, HLA-DQA2 and HLA-DRA enriched in "Type I diabetes mellitus". This pathway was strongly related to 14 pathways, most of which were associated with diseases. Then, a protein-protein interaction network was constructed, and 45 potential key DEGs were identified. The 45 DEGs enriched in pathways such as "Rheumatoid arthritis", "Chemokine signaling pathway" and "Cytokine-cytokine receptor interaction" (e.g. CXCL12 and CCL5). Transcription factors (TFs) of key DEGs were predicted, and a TF-DEG regulatory network was constructed. CONCLUSIONS: Some genes (e.g. CXCL12 and CCL5) and their TFs were significantly and abnormally regulated in the umbilical cord tissue from the pregnancies of T1DM mothers compared to that from non-T1DM mothers.

Resultados périnatales en embarazos múltiples monocoriales relacionados con las características placentarias / Perinatal outcomes in monochorionic multiple pregnancies regarding the placental characteristics

Resumen OBJETIVO: evaluar las características placentarias de pacientes con embarazos múltiples monocoriales relacionados con el resultado obstétrico en nuestro medio. MATERIAL Y MÉTODO: estudio observacional, descriptivo, prospectivo efectuado en dos instituciones de tercer nivel de atención de Bogotá, Colombia, entre el 31 julio de 2009 y el 31 de agosto de 2011. Evaluación de pacientes con embarazos monocoriales, complicaciones, tratamientos y resultados perinatales; análisis placentarios relacionados con el número y tipo de anastomosis, forma y distancia entre las inserciones de los cordones umbilicales y distribución placentaria. RESULTADOS: se incluyeron 72 embarazos monocoriales: 93.1% correspondieron a embarazos dobles y 86.1% a biamnióticos. De las placentas analizadas, 91.6% tuvieron al menos una anastomosis, específicamente arterioarterial (70.8% de los casos). Se encontraron complicaciones propias de los embarazos monocoriales (41.6%), principalmente: restricción de crecimiento intrauterino selectivo (16 casos) y trasfusión feto-fetal (11 pares de gemelos). Se registró un caso de trasfusión arterial reversa y una muerte fetal (10.7%) provocada por síndrome de transfusión feto-fetal. Entre las causas de morbilidad neonatal se encontraron: taquipnea transitoria del recién nacido y enfermedad de membrana hialina. La mortalidad neonatal fue de 5.4%. No hubo casos de mortalidad materna. CONCLUSIÓN: las características placentarias determinan las complicaciones propias del embarazo monocorial, principalmente: síndrome de trasfusión feto-fetal, restricción de crecimiento intrauterino selectivo. La detección ecográfica prenatal de estas alteraciones es importante para ofrecer consejería y vigilancia gestacional programada.

Abstract OBJECTIVE: To evaluate placental characteristics in monochorionic multiple pregnancies in relation to obstetric outcome in our environment. MATERIAL AND METHOD: Prospective and observational study was made; carried to 31 July 2009 and 31 August 2011 in two high complexity institutions in Bogota, Colombia. We included monochorionic pregnancies for diagnosis of complications, management and perinatal outcome, placental analyzes were evaluated with respect to the number and type of anastomosis, type and distance between umbilical cord insertions and placental sharing. RESULTS: We registrered 72 monochorionic pregnancies, twin pregnancies were 93.1% and 86.1% diamniotic. Placentas analyzed 91.6% had at least one placental anastomosis, arterio-arterial mainly in 70.8% of patients. Complications of monochorionic pregnancies in 41.6% of cases mainly selective growth restriction in 16 cases, followed by twin to twin transfusion in 11 pairs of twins were found. A case of blood transfusion reverse was presented; and a fetal mortality of 10.7% remains the leading cause twin to twin transfusion syndrome. Among the causes of neonatal morbidity transient tachypnea of the newborn, followed by hyaline membrane disease were found. The neonatal mortality rate was 5.4%. There were no maternal deaths. CONCLUSION: Placental characteristics determine the complications of monochorionic pregnancy, as the presence of twin to twin transfusion syndrome selective growth restriction and perinatal outcome. Prenatal sonographic identification of these features is important for prenatal counseling and the frequency of gestational surveillance.

[Vasa previa: Of the diagnosis to neonatal prognosis]. / Vasa prævia: du diagnostic au pronostic néonatal.

Vasa previa is a rare complication of pregnancy (1/2000 to 1/6000) with a high fetal mortality rate (75 to 100%). We will discuss two case reports of vasa previa: the first was diagnosed before labor, while the second was during delivery. In the first case, the diagnosis of vasa previa was confirmed by a transvaginal ultrasound and color doppler, while the second case involved late diagnosis during delivery and after gross examination of the placenta. Risk factors for vasa previa are: low-lying placenta, bilobed or succenturiate lobed placenta, velamentous and in vitro fertilization (IVF). Antenatal diagnosis of vasa previa is crucial because it allows for prophylactic caesarean section and prevents severe Benckiser's hemorrhage responsible for a very high neonatal mortality.