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Umbilical Cord Stricture Causing Intrauterine Death

Authors:
Issue: Ir Med J; Vol 113; No. 4; P62
Umbilical Cord Stricture Causing Intrauterine Death
R. Langhe1, E. Akpan1, S. Selvamani1, N. McEntagart2
1. Department of Obstetrics and gynaecology, Our Lady of Lourdes hospital, Drogheda, Ireland.
2. Department of Perinatal pathology, Rotunda hospital, Dublin, Ireland.
Dear Sir,
A 27-year old woman in her first pregnancy was diagnosed with intra uterine fetal death (IUFD) at 37 weeks gestation.
She was booked at 11 weeks gestations. Her booking bloods were normal and serology was negative. The woman was
non-smoker and of a normal BMI. A booking ultrasound scanning (USS) was done at 11 weeks and 6 days gestation,
which revealed a viable intrauterine pregnancy with low lying placenta. Her pregnancy was categorised as normal risk.
The woman had a combined regular antenatal visits at 30, 34 and 37 weeks gestation which was unremarkable.
USS for placenta site at 30 weeks gestation was normal and the estimated fetal weight (EFW) was between 50th and
90th centiles.
At 37 weeks and 4 days, she presented to the clinic with a history of reduced fetal movements of 1 day duration. On
abdominal examination, the uterine size was equivalent to 37 weeks gestation. The fetus was in longitudinal lie with
a cephalic presentation. No foetal heart was heard on abdominal examination and an USS confirmed IUFD. The couple
were debriefed and a plan was made to induce labour. At 38 weeks gestation a female infant of birth weight 2.97 kg
was delivered vaginally.
Postnatal maternal and fetal investigations did not detect any abnormalities. Neither growth restriction nor anatomical
abnormalities were noted. The 50.5cm long umbilical cord had 2 strictures at 20cm and 39cm from the foetus. The
foetal death was thought to be due to a possible stricture in the umbilical cord. The woman’s age, health and previous
history showed no link with this condition.
Umbilical cord constriction is an uncommon event that can cause intrauterine fetal demise 1. The condition is
characterized by localised absence of Wharton's jelly, causing a narrowing of the cord, thickening of the vascular walls
and narrowing of the vascular lumens 2,3,4. The exact aetiology is unclear, it is thought to result from twisting of the
cord during foetal movements 1. Usually a decrease in foetal movements is the only symptom and foetal death occurs
shortly after 3.
Families who have IUFD due to cord constriction should be appropriately counselled about the possibility of small risk
of recurrence and the need for strict foetal monitoring for future pregnancies 2. A sudden change in foetal activity or
physiologic signs warrants consideration of this uncommon condition in all pregnancies by clinicians and pathologist.
Currently, stricture of umbilical cord is not a preventable condition, however, further research perhaps could help in
identifying risk factors and precautions to decrease future occurrence.
Corresponding Author:
Ream Langhe
Department of Obstetrics and gynaecology,
Our Lady of Lourdes hospital,
Drogheda,
Ireland.
Email: reamlanghe@rcsi.ie
References:
1. Ling SY, Hwang JL, Huang LW. Umbilical cord stricture causing intrauterine fetal death in a 22-week fetus.
Taiwanese Journal of Obstetrics and Gynecology. 2006 Mar 1;45(1):73-5.
2. Hallak M, Pryde PG, Qureshi F, Johnson MP, Jacques SM, Evans MI. Constriction of the umbilical cord leading to
fetal death. A report of three cases. The Journal of reproductive medicine. 1994 Jul;39(7):561-5.
3. Sun Y, Arbuckle S, Hocking G, Billson V. Umbilical cord stricture and intrauterine fetal death. Pediatric Pathology
& Laboratory Medicine. 1995 Jan 1;15(5):723-32.
4. Weber J. Constriction of the umbilical cord as a cause of foetal death. Acta obstetricia et gynecologica
Scandinavica. 1963 Jan 1;42(3):259-68.
... [206][207][208][209] or iatrogenic, usually following amniocentesis. [209][210][211][212] It has been considered that the underlying cause of umbilical cord stricture is a primary deficiency of Wharton's jelly, a concept, which indirectly reflects/infers the previously detailed importance of this inherent protective mechanism of the cord. 1 Absence of Wharton's jelly, stenosis, or obliteration of umbilical vessels, usually close to the fetal insertion of the umbilical cord and intravascular thrombosis at the narrowed segment of the cord are often noted. 1,208 The location close to the fetal insertion may reflect the gradually decreasing amount of Wharton's jelly near the abdomen. 1 Strictures of the umbilical cord are often noted in association with stillbirth, although rare cases of survival following emergency delivery have been noted, usually following notation of decreased fetal movements. ...
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Full-text available
The umbilical cord constitutes a continuation of the fetal cardiovascular system anatomically bridging between the placenta and the fetus. This structure, critical in human development, enables mobility of the developing fetus within the gestational sac in contrast to the placenta, which is anchored to the uterine wall. The umbilical cord is protected by unique, robust anatomical features, which include: length of the umbilical cord, Wharton's jelly, two umbilical arteries, coiling, and suspension in amniotic fluid. These features all contribute to protect and buffer this essential structure from potential detrimental twisting, shearing, torsion, and compression forces throughout gestation, and specifically during labor and delivery. The arterial components of the umbilical cord are further protected by the presence of Hyrtl's anastomosis between the two respective umbilical arteries. Abnormalities of the umbilical cord are uncommon yet include excessively long or short cords, hyper or hypocoiling, cysts, single umbilical artery, supernumerary vessels, rarely an absent umbilical cord, stricture, furcate and velamentous insertions (including vasa previa), umbilical vein and arterial thrombosis, umbilical artery aneurysm, hematomas, and tumors (including hemangioma angiomyxoma and teratoma). This commentary will address current perspectives of prenatal sonography of the umbilical cord, including structural anomalies and the potential impact of future imaging technologies.
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