Baylor University Medical Center Proceedings April 2017 - 209

Giant cystic umbilical cord associated with patent urachus
and intrauterine fetal demise
Kira Brooks, MD, Joseph Guileyardo, MD, Don Le, DO, and Jesy Joykutty, DO

True cystic structures within the umbilical cord are rare, and when they
persist into the second and third trimester, they are often associated with
an abnormal karyotype or other developmental abnormalities. Clinically
significant pseudocysts resulting from massive edema of the umbilical
cord have been associated with a congenitally patent urachus. We present
a case of intrauterine fetal demise at 28 weeks' gestation in which cystic
dilatation of the umbilical cord was diagnosed prenatally by ultrasound
imaging. At autopsy, a congenitally patent urachus was contiguous with
the massively edematous umbilical cord.

mbilical cord cysts detected beyond the ﬁrst trimester
have a strong association with congenital anomalies
and aneuploidy (1). In a case series by Smith et al, 18
of 23 neonates diagnosed with cystic umbilical cords
were found to have an abnormal karyotype or other congenital
anomalies (1). Pseudocysts are not true epithelial-lined cysts but
result from edema of the umbilical cord and may be associated
with a patent urachus (2, 3). Herein we describe a stillbirth of
a fetus previously diagnosed, by ultrasound, with a large, cystic
umbilical cord in the second trimester.
CASE DESCRIPTION
A 21-year-old gravida 2 para 0 woman presented to her
obstetrician at 8 weeks and 1 day gestation for conﬁrmation of
pregnancy. Her medical history was unremarkable aside from a
previous ectopic pregnancy treated with salpingectomy. Routine
ultrasonography, performed at 19 weeks' gestation, disclosed a large
cystic umbilical cord measuring 42 × 34 × 30 mm. A sonogram
performed at 24 weeks' gestation conﬁrmed the large cystic cord
(Figure 1) and polyhydramnios. Noninvasive perinatal testing,
antiphospholipid antibody screening, and TORCH titers drawn
during the pregnancy were normal. Amniocentesis for karyotyping
was declined by the patient, who was evaluated again at 26 weeks
and had a normal umbilical Doppler exam. A short cervix was
noted during this encounter, and the patient was given steroids to
promote fetal lung maturity. At 27 weeks there were no changes,
but at 28 weeks and 4 days the patient reported excessive fetal
movement followed by absence of fetal activity. Fetal demise was
documented, induction and vaginal delivery followed, and a massively enlarged and cystic umbilical cord was seen at delivery.
Proc (Bayl Univ Med Cent) 2017;30(2):209-210

Figure 1. Sonographic image of umbilical cord cyst.

Figure 2. Patent urachus extending up to the clamped portion of the fetal
umbilical cord.

Autopsy ﬁndings of the male fetus included a patent urachus
extending from the dome of the urinary bladder into the threevessel umbilical cord. The urachus could be easily probed to the
clamped portion of the cord nearest the baby (Figure 2). This
patent urachus was contiguous with the massively edematous
and cystic portion of the umbilical cord (Figure 3). Additional
From the Departments of Obstetrics and Gynecology (Brooks, Joykutty) and
Pathology (Guileyardo, Le), Baylor University Medical Center at Dallas, Texas.
Corresponding author: Kira Brooks, MD, Department of Obstetrics and
Gynecology, Baylor University Medical Center at Dallas, 3500 Gaston Avenue,
Dallas, TX 75246 (e-mail: kira.brooks@bswhealth.org).
209

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