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Figure 3. Placenta with attached giant edematous umbilical cord. The umbilical
vessels can be clearly seen within the edematous portion of the cord.

autopsy ﬁndings included focal cord stricture away from the
edematous portion and focal umbilical vessel thrombosis. Only
two vessels were seen in microscopic sections of the cord stricture. G-band chromosomal analysis conﬁrmed a normal male
46 XY karyotype; however, microarray DNA analysis was unsuccessful.
DISCUSSION
The mechanism of pseudocyst formation of the umbilical
cord associated with a patent urachus is not known, but it has
been theorized that reﬂux of hypoosmotic fetal urine into the
cord may result in swelling of Wharton's jelly (3). A patent
urachus is rare, with an incidence of only 1 to 2 per 100,000
deliveries (4). The diagnosis may be made antenatally, but in
utero diagnosis is diﬃcult, since a communication between
the bladder and the adjacent cystic mass may not always be
visible by sonogram (4). Fortunately, a patent urachus usually

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carries a favorable prognosis, but infectious and even neoplastic
complications are known to occur. Clinical appreciation of this
anomaly can lead to further evaluation and follow-up in order
to determine the need for additional therapy such as surgical
ligation or excision. Therefore, the ﬁnding of a cystic umbilical
cord should prompt careful search for a patent urachus, which
may be the cause of this condition.
Normal live birth without other developmental abnormalities or aneuploidy may be seen with cystic umbilical cord dilatation, but cystic cords have also been associated with vascular
thrombosis and stillbirth (5). No standard exists for evaluation,
monitoring, and treatment of cystic umbilical cords, but due to
the high occurrence of congenital anomalies and aneuploidy, detailed sonographic imaging and karyotyping have been recommended (3). However, umbilical cord Doppler echograms were
performed weekly on this patient with no abnormal ﬁndings,
and it is unlikely that closer surveillance would have changed
the outcome in this case. It has also been proposed that color
Doppler may distinguish vascular and nonvascular cord masses
and may aid in detecting impaired blood ﬂow (4).
1.

Smith GN, Walker M, Johnston S, Ash K. The sonographic ﬁnding of
persistent umbilical cord cystic masses is associated with lethal aneuploidy
and/or congenital anomalies. Prenat Diagn 1996;16(12):1141-1147.
Kilicdag EB, Kilicdag H, Bagis T, Tarim E, Yanik F. Large pseudocyst of
the umbilical cord associated with patent urachus. J Obstet Gynaecol Res
2004;30(6):444-447.
Nobuhara KK, Lukish JR, Hartman GE, Gilbert JC. The giant umbilical cord: an unusual presentation of a patent urachus. J Pediatr Surg
2004;39(1):128-129.
Schiesser M, Lapaire O, Holzgreve W, Tercanli S. Umbilical cord edema associated with patent urachus. Ultrasound Obstet Gynecol 2003;22(6):646-
647.
Sepulveda W, Wong AE, Gonzalez R, Vasquez P, Gutierrez J. Fetal death
due to umbilical cord hematoma: a rare complication of umbilical cord
cyst. J Matern Fetal Neonatal Med 2005;18(6):387-390.

Baylor University Medical Center Proceedings

Volume 30, Number 2

Table of Contents for the Digital Edition of Baylor University Medical Center Proceedings April 2017