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\begin{document}

\title{UMBILICAL CORD HEMANGIOMA AND PSEUDOCYST WITH FAVORABLE FETAL OUTCOME}



\author[1]{Sikolia Wanyonyi}%
\author[1]{Felix Nyagaka}%
\author[1]{Patricia Okiro}%
\author[1]{Lilian Ogutu}%
\author[1]{Alice Nyaichowa}%
\author[1]{Felix Oindi}%
\author[1]{Evan Sequeira}%
\affil[1]{The Aga Khan University Hospital Nairobi}%


\vspace{-1em}



  
  \date{June 11, 2024}


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\textbf{TITLE: UMBILICAL CORD HEMANGIOMA AND PSEUDOCYST WITH FAVORABLE
FETAL OUTCOME}

\textbf{ABSTRACT}

Umbilical cord hemangiomas are rare neoplasms of vascular origin,
commonly found in the free section of the umbilical cord proximal to
placental insertion. They are associated with an increased risk of fetal
mortality. We present a rare co-occurrence of an umbilical cord
hemangioma and a pseudocyst managed conservatively, with favorable fetal
outcome despite the interval increase in size, decreased caliber of the
umbilical arteries and fetal chest compression.

\textbf{CASE PRESENTATION}

A 33-year-old gravida 2 woman, with well-controlled early onset
gestation hypertension, presented at 20 weeks for an anomaly scan. Her
antenatal profile and aneuploidy screening were unremarkable. Her first
pregnancy was complicated with pre-eclampsia at term, but had a
spontaneous vaginal delivery, following induction of labor with good
outcome.

On the anomaly scan, a large complex multi-cystic mass with solid
components was noted proximal to the cord insertion (\emph{figure 1a,
b)} . The lesion measured 10.91cm by 8.22cm. Both umbilical arteries had
thickened tunica with positive end diastolic flow (EDF). However, there
was discordance in velocity of blood flow of the umbilical arteries
(14cm/s and 49cm/s) \emph{(Figure 1c, d).} The umbilical cord was
markedly oedematous with increased Wharton's jelly. The rest of the
fetal anatomy was normal.

Follow-up ultrasound done every two weeks showed an interval increase in
size of the solid components. Despite the variance in calibre and
velocity of the umbilical arteries, the positive EDF in the vessels
persisted till delivery. From 32 weeks onwards, the mass appeared to
compress the chest, but there were no signs of obstruction, and the lung
volumes remained normal for gestational age. The abdominal circumference
and estimated fetal weight were above the 90\textsuperscript{th} centile
and the amniotic fluid volume was normal \emph{(Figure 2).} An elective
caesarean delivery was planned at 38 weeks due to uncertainly on the
ability of the fetus to tolerate labour.

A live male infant was delivered with a birthweight of 3480 grams and an
Apgar score of 10 at 5 minutes. The umbilical cord cyst ruptured during
delivery. The placenta spontaneously separated. The umbilical cord mass
was noted (\emph{figure 3a,b} ). The placenta was taken for
histo-pathological examination and immunohistochemistry.

The gross formalin-fixed specimen showed a translucent and hyper-coiled
cord with prominent vascular pattern and a multinodular mass at the
insertion site measuring 7x7x4cm and a collapsed cyst measuring
15x10cm\emph{(Figure 4a).} Upon dissection, a variegated lobular lesion
with peripheral hemorrhage measuring 3.8x2.2cm was seen \emph{(Figure
4b} ). The diameter of the cord at the lesion site was 5.7 cm. The
lesion was limited to the umbilical cord.

Histology confirmed an umbilical hemangioma with a pseudocyst, mild
acute funisitis, inter-villous hematomas, delayed villous maturation and
avascular fibrotic villi \emph{(Figure 4c).} The findings were
collaborated on CD34 immunohistochemistry staining \emph{(Figure 4d).}

\textbf{DISCUSSION}

Umbilical cord hemangiomas are rare benign endothelial-derived neoplasms
composed of blood vessels. Typically, hemangiomas are found in skin and
soft tissues, but their presence in other organs has been
demonstrated\textsuperscript{1}. Umbilical hemangiomas tend to have a
predilection for the placental-end of the cord and are likely to arise
from one or more of the major umbilical vessels\textsuperscript{2-6}.
Umbilical cysts may present as true cysts, resulting from damage to the
amniotic surface of the cord by presence of the hemangioma. The
epithelial-lined cysts are embryological remnants of either the
allantois or omphalomesenteric duct. On the other hand, pseudo-cysts
arise from degeneration of Wharton's jelly surrounding the cord
vessels\textsuperscript{2}. Differential diagnoses include cord
teratomas, aneurysms, omphalomesenteric duct cysts, allantoic cysts,
hematomas and varicose veins, abdominal wall defects, and metastatic
neuroblastoma\textsuperscript{2}. Although no clear causal-relationship
is established, an association between cord hemangiomas and increased
risk of fetal mortality has been exhibited. So far, no successful
in-utero intervention has been described\textsuperscript{3}.

Prenatal ultrasound can reliably differentiate hemangioma and pseudocyst
from other umbilical cord tumors\textsuperscript{7}. The presence of
positive EDF of the umbilical artery Doppler with normal pulsatility
index and progressive fetal growth provides buoyancy to prolong the
pregnancy\textsuperscript{4,8}.

Even though rare, umbilical cord neoplasms are predominantly hemangiomas
and less commonly teratomas\textsuperscript{1,2}. Hemangiomas arise from
endothelial cells especially of the umbilical arteries and have an
undefined pathophysiology\textsuperscript{9}. In a unique occurrence,
Thayer and colleagues recently documented an umbilical cord hemangioma
occurring synchronously with maternal Covid-19 infection with adverse
fetal outcome\textsuperscript{1}.

Hemangiomas may present as fusiform swellings of the cord comprising of
an angiomatous nodule, surrounded by edema of the Wharton's jelly with
myxoid cystic degeneration. They are clearly demarcated from surrounding
stroma. These nodules vary in dimensions, ranging from 0.2 to 7 cm in
largest dimension, and if associated with edema may measure up to 18
cm\textsuperscript{1-3,9}. A hyper-coiled umbilical cord was noted with
a multi-nodular mass at the insertion site. A collapsed cyst 15cm by
10cm, a residual nodule 7cm x7cm x 4cm with an edematous cord were also
seen and this is in keeping with dimensions of other hemangiomas
reported. Extensive myxoid degeneration of Wharton's jelly was also
confirmed in our case.

A strong association exists between cord hemangioma and intra-uterine
fetal demise (IUFD). Possible reasons include vascular compression,
intravascular thrombosis, intravascular tumor proliferation and
hemorrhage from ruptured vessels. The risk of IUFD is highest if
compression of the umbilical veins is
present\textsuperscript{3,4,10,11}. Umbilical cord pseudocysts have also
been linked to aneuplodies\textsuperscript{12}. In our case the fetus
was euploid and had a good outcome despite reduced velocity in one of
the umbilical arteries and interval increase in size of the lesion. The
flow in the umbilical veins remained unaffected.

Management of fetuses with umbilical cord hemangiomas depends on
gestational age and early delivery may reduce the risk of IUFD, but the
decision is weighed against possible complications of
pre-maturity\textsuperscript{3,4,11}\textbf{.} In our case, the patient
had serial ultrasound scans done every 2 weeks to monitor the velocity
of the umbilical arteries. The decision to delivery at 38 weeks was
based on the compressive effect observed, maternal hypertension and
uncertainty on the outcome.

\textbf{CONCLUSION}

The outcome of cord hemangiomas is not well understood, and neither is
their pathophysiology elaborated. However, despite its high association
with fetal mortality, a favorable outcome is possible with close
monitoring of the umbilical artery Doppler flows and timely delivery,
the size of the lesion notwithstanding.

\textbf{REFERENCES}

1.Thayer BM, Helal I, Khanchel F, Mbarki C, Bettaieb H, Ben Brahim E, et
al. Hemangioma of the umbilical cord: A case report on a rare
entity.\emph{Clinical case reports} 2022;\textbf{10} (10):e6441.

2.Angelico G, Spadola S, Ieni A, Gurrera A, Arena MG, Arciuolo D, et al.
Hemangioma of the umbilical cord with associated amnionic inclusion
cyst: two uncommon entities occurring simultaneously.
\emph{Pathologica}2019;\textbf{111} (1):13-7.

3.Lisovaja I, Franckevica I, Vedmedovska N. Large Angiomyxoma of the
Umbilical Cord-Uncomplicated Rupture of Tumor Membranes at 32 Weeks of
Gestation. \emph{Diagnostics (Basel, Switzerland)} 2022;\textbf{12} (6).

4.Papadopoulos VG, Kourea HP, Adonakis GL, Decavalas GO. A case of
umbilical cord hemangioma: Doppler studies and review of the
literature.\emph{Eur J Obstet Gynecol Reprod Biol} 2009;\textbf{144(}
1):8-14.

5.Leung KY, Poon CF, Teotico AR, Hata T, Won HS, Chen M, et al.
Recommendations on routine mid-trimester anomaly scan. \emph{J of Obstet
Gynaecol Res} 2015;\textbf{41} (5):653-61.

6.Caldarella A, Buccoliero AM, Taddei A, Savino L, Taddei GL. Hemangioma
of the umbilical cord: report of a case. \emph{Pathol, Research and
Practice} 2003;\textbf{199(} 1):51-5.

7. Lok WY, Law KM, Ho CL, Leung TY. Prenatal diagnosis of umbilical cord
hemangioma. \emph{Ultrasound Obstet Gynaecol} 2022;\textbf{59(}
3):392-393

8. Krzy\selectlanguage{polish}ż\selectlanguage{english}anowski A, Kwiatek M, G\selectlanguage{polish}ę\selectlanguage{english}ca T, Stupak A, Kwa\selectlanguage{polish}ś\selectlanguage{english}niewska A. Modern
Ultrasonography of the Umbilical Cord: Prenatal Diagnosis of Umbilical
Cord. Abnormalities and Assessment of Fetal Wellbeing. \emph{Med Sci
Monit} . 2019, 30;25:3170-3180

9. Smulian JC, Sarno AP, Rochon ML, Loven VA. The natural history of an
umbilical cord hemangioma. \emph{J Clin Ultrasound} . 2016;\textbf{44(}
7):455-8.

10. Kamitomo M, Sueyoshi K, Matsukita S, Matsuda Y, Hatae M, Ikenoue T.
Hemangioma of the umbilical cord: stenotic change of the umbilical
vessels. \emph{Fetal Diagn Ther} . 1999; \textbf{14} (6):328-31

11. Matsuda S, Sato Y, Marutsuka K, Sameshima H, Michikata K, Ikenoue T,
Soma H, Asada Y. Hemangioma of the umbilical cord with
pseudocyst\emph{.} \emph{Fetal Pediatr Pathol.}2011;\textbf{30}
(1):16-21

12. Sepulveda W, Pryde PG, Greb AE, Romero R, Evans MI. Prenatal
diagnosis of umbilical cord pseudocyst. \emph{Ultrasound Obstet Gyna}
ecol \textbf{4} (1994); 147-150

\textbf{CONSENT STATEMENT}

Written informed consent was obtained from the patient to publish this
report in accordance with the journal's consent policy

\textbf{CONFLICT OF INTEREST}

The authors have no conflict of interest to declare

\textbf{Hosted file}

\verb`Figures case report .docx`  available at \url{https://authorea.com/users/604710/articles/634464-umbilical-cord-hemangioma-and-pseudocyst-with-favorable-fetal-outcome}

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