True knot of the umbilical cord usually follows fetal passage through a loop of a cord. It is a rare occurrence [1], with 4–10 fold increased risk of stillbirth [2].

Single true knot of the umbilicus is very rare and the incidence is documented to be between 0.3% and 2% of pregnancies. The findings of two (2) true knots in the umbilical cord of our index case with good fetal outcome are more astonishing because any form of disorder in the umbilical cord can be life-threatening to the fetus.

It is generally considered that true knots of the umbilical cord occur in early gestation (between 9 and 12 weeks) when the overall amniotic fluid volume is considerably larger than the fetus [3].

Predisposing factors in the formation of true knots of the umbilical cord include: long umbilical cords, polyhydramnios, excessive fetal movements, gestational diabetes, multiparity, male fetus, small sized fetus, chronic hypertension [4], and interestingly, patients who have undergone genetic amniocentesis [5]. Generally, the average length of the umbilical cord is about 55 cm, and any length more than 80 cm is regarded to be unduly long and capable of causing cord entanglement [2].

Wharton’s jelly ordinarily surrounds the umbilical cord and reduces the probability of collapse of vessels. In event of loss of this protection as noted with true knots, the umbilical cord may tighten leading to decrease or abrupt cessation of blood flow resulting to intrauterine fetal demise [6],[7]. In Nnamdi Azikiwe University Teaching Hospital Nnewi, South-East Nigeria, a case of true single umbilical knot leading to fetal demise was reported [8].

Here, we present a case of a male fetus that had a good outcome following caesarean delivery on account of frank breech presentation with an incidental finding of two true knots in the umbilical cord that measured 110 cm.

Our patient is Mrs. M.A, a booked 22-year-old G2p1A1. Her last normal menstrual period was on 28th November, 2020 and based on her regular 28th day cycle and non-use of hormonal oral contraceptive, her expected date of delivery (EDD) was on 5th September, 2021.

Pregnancy was desired, spontaneously conceived and confirmed with an abdominal ultrasound. She booked for antenatal care in ESUT Teaching Hospital at a gestational age of 12 weeks, prior to booking and at booking she had no complaint. Her booking weight was 70 kg, height was 1.6 m, and blood pressure was 110/70 mmgH. Her Hepatitis B surface antigen (HBsAg), venereal disease research laboratory (VDRL), and human immunodeficiency virus (HIV) 1 and 2 tests were not reactive. Her blood group was O Rhesus D positive, hemoglobin genotype was AA, and packed cell volume was 34%. She slept under insecticide treated net, was compliant with her routine antenatal drugs, and received two doses each of intermittent preventive therapy for malaria and tetanus toxoid at 22 and 26 weeks of gestation respectively. She was not a known hypertensive or diabetic patient and pregnancy progressed uneventfully until at 36 weeks when it was noted that her fetus was in breech presentation and routine 36 weeks ultrasound confirmed frank breech presentation of the fetus. She was subsequently counseled on options of management of her condition. She chose an elective caesarean delivery which she had at 38 completed weeks of gestation. The outcome was a live male neonate who cried immediately with Apgar scores of 8 and 10 in the 1st and 5th minutes of live respectively, birth weight was 3.4 kg.

Unsuspected findings of two (2) true knots of the umbilicus at 6 cm apart were noted 9 cm distal to cord attachment to the fetus (Figure 1). The umbilical cord length was measured and found to be 110 cm; no other fetal anomaly was noted. Baby remained stable postpartum without any reason for neonatal intensive care unit admission or institution of any medical treatment (Figure 2).

True knots of the umbilicus are more likely to be detected when they are checked for especially when risk factors exist [9],[10]. Specifically, 4-dimensional color Doppler and power Doppler examination are the most important modalities for assessment and diagnosis of true knot [11]. Cloverleaf pattern [7] or hanging noose [2] may be seen incidentally.

Hence, scholars suggest that effort at prenatal diagnosis should be made especially when the suspicion or risk factor for the diagnosis exist [12].

Our patient had prenatal ultrasound investigation during antenatal care visits and at 36 weeks gestation, however the knots were missed.

Díaz de la Noval et al. observed that prenatal diagnosis of true knot umbilical cord is an exceptional and unexpected event that shouldn’t change planned obstetric approach [7].

However, other researchers consider caesarean delivery more appropriate to ensure good outcome [10]. They argued that in the course of labor and passage of fetus through the birth canal, the knots can tighten and fetal circulation may be compromised [9].

Our patient was offered caesarean delivery on account of frank breech presentation with an incidental finding of two true knots. This intervention (caesarean delivery) may be responsible for the good fetal outcome observed.

Given the association of true knot of the umbilical cord and 4–10 fold increased risk of stillbirth, high index of suspicion, routine Doppler study of the umbilical cord especially in patient with high risk of true knot formation and adequate intrapartum monitoring are recommended to avert disservice to our patients and their fetuses.