Management of Delivery of Neonate with Large Umbilical Pseudocyst and Fetal Heterotaxy: A Case Report

Purpose: Present a unique case of a large umbilical cord pseudocyst with a two-vessel cord associated with fetal heterotaxy and extensive congenital vascular malformations. Introduction: Umbilical pseudocysts found during the first trimester are a rare anomaly, occurring in 0.4%-3.4% of pregnancies and are most commonly transient. An umbilical cord cyst is one that communicates with the urachus, while a pseudocyst is composed of Wharton’s jelly. The prevalence of a pseudocyst in the third trimester, however, is unknown due to insufficient data. In literature reviews, the prevalence of fetal anomalies with a third trimester umbilical cyst is reported to be 38-100%. It is worth noting that this data is likely biased by the tendency to report abnormal and unique cases in literature. Case Description: The patient was a 23-year-old G3P2 who desired a trial of labor after cesarean (TOLAC) under the care of a midwife. She had an anatomy ultrasound at 30 weeks’ gestation which demonstrated fetal heart defects and an umbilical cyst. The fetal echocardiogram showed a double outlet right ventricle (DORV) with mild pulmonary stenosis, right aortic arch, large ventricular septal defect, and visceral situs inversus with levocardia. A two-vessel umbilical cord with umbilical cyst measuring 4.1 x 3.1 x 2.8cm with absent flow within was also noted. The parents decided at this time to do cell-free DNA which was low risk. On repeat ultrasound at 37 weeks’ gestation, the azygous vein was noted coursing into the inferior vena cava. The abdominal anatomy was also clarified – the stomach and spleen were on the right side and liver and gallbladder on the left side. Upon discovery of the DORV on fetal echo, consultation with pediatric cardiology was initiated with close monitoring of the degree of pulmonic stenosis (PS) of the fetus. It was discussed whether the fetus could be delivered at our center, a level III NICU – or need immediate cardiac surgery upon delivery. It was determined the PS was mild, and the fetus would be well-supported by our NICU. Additionally, no additional complications were anticipated for the patient’s planned TOLAC. The patient presented in latent labor at 39+0 weeks’ gestation with a VBAC success calculated score of 73.4%. During labor, the patient was monitored using external tocodynamometry and fetal doppler. She opted for no epidural and delivered a viable baby girl. After 60 seconds, delayed cord clamping was performed, and the neonate was passed to a NICU team for standard resuscitation. The neonate had APGAR’s of 7 and 8 at 1 and 5 minutes, respectively. She initially required NCPAP but was quickly transitioned to room air. The cord was tied off distal to the umbilical pseudocyst and secured to the neonate for transfer to the NICU. In the NICU, the umbilical cord pseudocyst was noted to have minimal bleeding but was clamped, cut, and sent for pathology. The neonate spent 11 days in the NICU and was discharged to home with close cardiology follow up and referral to pediatric cardiothoracic surgery. Pathology noted a 5.3 cm diameter 2-vessel umbilical cord consistent with a pseudocyst – segmental myxoid degeneration and edematous Wharton’s jelly. Also sent to pathology was the placenta, which showed both maternal and vascular malperfusion. Comments included findings consistent with increased resistance to blood flow in the umbilical vein, probably secondary to congenital vascular alterations due to heterotaxy and congenital heart anomalies. Conclusions: While data is very limited on the prevalence and comorbidities of umbilical cord cysts and pseudocysts, the presence of an umbilical cyst on prenatal ultrasound should warrant advanced imaging and referral to a tertiary care center.