Severe Consumptive Coagulopathy in an Extremely-Low-Birth-Weight Infant with Intra-Abdominal Umbilical Vein Varix: A Case Report

Haga, Mitsuhiro; Itoh, Kanako; Horikoshi, Toru; Namba, Fumihiko; Kabe, Kazuhiko

doi:10.1055/s-0041-1727288

Cited by 3 publications

(4 citation statements)

References 12 publications

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“…Despite increased vulnerability to thrombosis due to UVV during pregnancy, coagulopathy and DIC seem rare. Only four cases have been reported, and all four are preterm neonates born before 34 weeks of gestation ( 9 ). Plasma activities of coagulation factors in preterm neonates are reduced and the fibrinolysis system shows reduced plasminogen activity and increased plasminogen activator inhibitor activity.…”

Section: Discussionmentioning

confidence: 99%

“…Plasma activities of coagulation factors in preterm neonates are reduced and the fibrinolysis system shows reduced plasminogen activity and increased plasminogen activator inhibitor activity. Furthermore, the presence of UVV could be a predisposition for thrombosis, resulting in the consumption of coagulation factors and the induction of fibrinolysis ( 9 , 10 ). Premature neonates could be more susceptible to thrombosis and coagulopathy.…”

Section: Discussionmentioning

confidence: 99%

“…Neonatal outcomes associated with isolated UVV in premature infants have been shown to have favorable outcomes without complications. However, only one previous case of ELBWI with isolated UVV has been reported, wherein the infant also experienced neonatal coagulopathy but ultimately survived ( 9 ). Notably, ELBWI may be more susceptible to complications due to their prematurity or other factors, such as the location of the UVV and the length of time between detection of UVV and birth.…”

Section: Discussionmentioning

confidence: 99%

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Case report: A case of fetal umbilical vein varix presenting disseminated intravascular coagulation, polycythemia, and neonatal hepatitis in an extremely low birth weight infant

Sekiguchi

Mukai²,

Shitara³

et al. 2023

Front. Pediatr.

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Reports on the clinical course of fetal umbilical vein varix in premature infants are limited. We report a case of an extremely low body weight infant with intra-abdominal umbilical vein varix who developed disseminated intravascular coagulation, polycythemia, and hyperbilirubinemia after birth; late-onset neonatal hepatitis; and fetal thrombotic vasculopathy confirmed by placental histopathology. Ultrasonography after birth showed a dilated portion of the umbilical vein at the hepatic hilum with thrombi inside. We speculate that the umbilical vein varix caused the fetal thrombotic vasculopathy, and the presence of umbilical vein varix and fetal thrombotic vasculopathy in combination with prematurity caused coagulopathy, polycythemia, hyperbilirubinemia, and hepatitis. Despite the favorable outcomes reported in the literature, premature infants with umbilical vein varix may require careful observation and management for coagulopathy and late-onset hepatitis. Furthermore, placental histopathology could aid in the understanding of various clinical outcomes in infants with umbilical vein varices.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Case report: A case of fetal umbilical vein varix presenting disseminated intravascular coagulation, polycythemia, and neonatal hepatitis in an extremely low birth weight infant

Sekiguchi

Mukai²,

Shitara³

et al. 2023

Front. Pediatr.

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“…The FIUVV was diagnosed at the gestational age of 19w1d with a rapid increase in the size of the varix. In the neonatal period complications includes consumptive coagulopathy and cerebral hemorrhage have been reported recently [13].…”

Section: Discussionmentioning

confidence: 99%

FIUVV: Associations and Outcome

Alam¹,

Goswami²,

Joshi³

et al. 2022

Journal of Fetal Medicine

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To investigate the ultrasound characteristics, associations and obstetric outcomes in pregnancies with intra-abdominal umbilical vein varix of the fetus. This was a retrospective cohort study conducted over 2 years from March 2019 to February 2021. The data collected were demographic characteristics, varix characteristics, obstetric complications (fetal growth restriction/IUFD) and neonatal outcomes. Descriptive statistics (means, standard deviations, and percentages [%]) were calculated using SPSS, version 21.0 (SPSS Inc, Chicago, USA). Over the period of 2 years, six cases of FIUVV were diagnosed putting the incidence of FIUVV at 5/10000 pregnancies. The incidence of isolated FIUVV was 4.4/10000 cases. Mean gestational age at diagnosis was 25 weeks 3 days with earliest diagnosis at 19w1D. Mean varix size was 11.85 mm ± 2.28 mm (range 8.9–14.8 mm). No incidence of turbulence or thrombus formation within the varix was seen in the current study. One case had absent DV with anomalous drainage of the umbilical vein in to the IVC along with pleural effusion, polyhydramnios and increased nuchal fold thickness. Perinatal death after planned LSCS at term was noted in this pregnancy. One case had rapid dilatation of the varix from 9.5 at 19 weeks to 15.4 mm at 27 weeks. The patient reported sudden IUFD at 33 weeks. The association of FIUVV with the adverse obstetrical outcomes was seen in some cases during the current study. Increased surveillance to look for rapid dilatation/ turbulence/ thrombosis may prevent adverse perinatal outcomes.

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Intraabdominal Aneurysm of the Umbilical Vein of the Fetus. (A Rare Clinical Case)

P.H.,

L.V.,

K.P.

2024

Mediko-farmacevtičeskij žurnal "Pulʹs"

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The umbilical cord is an extension of the fetal cardiovascular system. According to all available scientific data, umbilical cord abnormalities play an important and often decisive role in the development of complications during pregnancy and childbirth, including perinatal mortality. Thus, umbilical cord abnormalities include skinny umbilical cord, umbilical cord presentation, absolutely short umbilical cord, sheath attachment of the umbilical cord, presentation of umbilical cord vessels, hypoisvite and hyperisvite umbilical cord, single umbilical artery, persistence of the right umbilical vein, vein aneurysm and umbilical cord hematoma. There are several variants of localization of this vascular anomaly: in the area of attachment to the placenta, in the free part, in the area of the umbilical ring and in the intraabdominal (intrahepatic) part. There are no clinical recommendations for the management of pregnancy with an umbilical vein aneurysm. When detecting umbilical cord abnormalities, it is necessary to monitor the dynamics of changes in the size of the aneurysm with an assessment of the fetal condition. The article describes a clinical case of intraabdominal fetal umbilical vein aneurysm, which is extremely rare and accounts for less than 1% of all umbilical cord abnormalities. The patient's reproductive and genetic history is not burdened. The somatic history was aggravated by neurodegenerative diseases of the spine. The pregnancy proceeded against the background of repeated threats of termination of pregnancy and anemia.

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Severe Consumptive Coagulopathy in an Extremely-Low-Birth-Weight Infant with Intra-Abdominal Umbilical Vein Varix: A Case Report

Cited by 3 publications

References 12 publications

Case report: A case of fetal umbilical vein varix presenting disseminated intravascular coagulation, polycythemia, and neonatal hepatitis in an extremely low birth weight infant

Case report: A case of fetal umbilical vein varix presenting disseminated intravascular coagulation, polycythemia, and neonatal hepatitis in an extremely low birth weight infant

FIUVV: Associations and Outcome

Intraabdominal Aneurysm of the Umbilical Vein of the Fetus. (A Rare Clinical Case)

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