scholarly journals Prenatal and Postnatal Sonographic Confirmation of Congenital Absence of the Ductus Venosus in a Child with Noonan Syndrome

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Christopher L. Newman ◽  
Matthew R. Wanner ◽  
Brandon P. Brown

The ductus venosus serves as an important vascular pathway for intrauterine circulation. This case presents a description of an absent ductus venosus in a female patient with Noonan syndrome, including both prenatal and postnatal imaging of the anomaly. In the setting of the anomalous vascular connection, the umbilical vein courses inferiorly to the iliac vein in parallel configuration with the umbilical artery. This finding was suspected based on prenatal imaging and the case was brought to attention when placement of an umbilical catheter was thought to be malpositioned given its appearance on radiography. Ultrasound imaging confirmed the anomalous course. This is in keeping with prior descriptions in the literature of an association between Noonan syndrome and aberrant umbilical venous drainage. This case illustrates the need for awareness of this condition by the radiologist, allowing for identification on radiographs and the recommendation for further confirmatory imaging. Further, the case illustrates the value of paying particular attention to the fetal course of the umbilical vessels in patients with suspected Noonan syndrome, as this population is particularly at risk for anomalous vasculature.

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Kenji Horie ◽  
Hironori Takahashi ◽  
Daisuke Matsubara ◽  
Koichi Kataoka ◽  
Rieko Furukawa ◽  
...  

Absent ductus venosus (ADV) is a rare vascular anomaly. We describe a fetus/neonate with ADV with a partial liver defect. A 41-year-old woman was referred to our institute because of fetal cardiomegaly detected by routine prenatal ultrasound, which revealed absence of ductus venosus with an umbilical vein directly draining into the right atrium, consistent with extrahepatic drainage type of ADV. She vaginally gave birth to a 3,096-gram male infant at 38 weeks of gestation. Detailed ultrasound examination revealed a defect of the hepatic rectangular leaf at half a month postnatally. He showed normal development at 1.5 years of age with the liver abnormality and a Morgagni hernia. Liver morphological abnormality should also be considered as a complication of ADV.


2007 ◽  
Vol 23 (2) ◽  
pp. 136-139 ◽  
Author(s):  
J. Hajdú ◽  
T. Marton ◽  
M. Kozsurek ◽  
B. Pete ◽  
Z. Csapó ◽  
...  

2017 ◽  
Vol 11 (4) ◽  
pp. NP80-NP82 ◽  
Author(s):  
Peter C. Chen ◽  
Julia C. Swanson ◽  
Prakash Masand ◽  
Jose R. Rodriguez ◽  
Carlos M. Mery

Umbilical vein anomalies are a rare congenital defect, which have been associated with absent ductus venosus, with few cases also involving a congenital diaphragmatic hernia. We describe a case of postnatal development of an anterior diaphragmatic hernia of Morgagni in a four-year-old patient diagnosed prenatally with mesocardia, absent ductus venosus with a large umbilical vein, a large secundum atrial septal defect, and patent ductus arteriosus.


2003 ◽  
Vol 18 (4) ◽  
pp. 247-251 ◽  
Author(s):  
Zeev Perles ◽  
Amiram Nir ◽  
Michael Nadjari ◽  
Zivanit Ergaz ◽  
Annick Raas-Rothschild ◽  
...  

2009 ◽  
Vol 12 (3) ◽  
pp. 239-243 ◽  
Author(s):  
Makoto Takeuchi ◽  
Masahiro Nakayama ◽  
Arihiro Tamura ◽  
Hiroyuki Kitajima

We describe the clinical course and autopsy findings of a male infant with hydrops fetalis due to agenesis of the ductus venosus. Fetal echocardiography at 27 weeks in gestation demonstrated hydrops fetalis due to unknown causes. The baby was born at 28 weeks in gestation by emergency caesarean section because of preeclampsia and progressive hydrop fetalis but died immediately at birth. The umbilical vein catheter ran an unusual course: left renal vein and inferior vena cava were opacified after postmortem injection of radiopaque dye into the umbilical vein. The autopsy demonstrated agenesis of the ductus venosus without extrahepatic umbilical venous drainage. The type without extrahepatic venous drainage is rare but shows a favorable outcome in general. However, our findings illustrate that in addition to sinusoidal dilatation, some cases may induce significant medial hypertrophy of portal veins, leading to hydrops fetalis and neonatal demise.


2021 ◽  
Vol 14 (9) ◽  
pp. e244023
Author(s):  
Monika Kantilal Kotecha ◽  
Jonathan, Tze Liang Choo ◽  
Sreekanthan Sundararaghavan

We present a rare case of premature low birthweight neonate with right diaphragmatic hernia and transposition of great vessels requiring balloon atrial septostomy. Congenital diaphragmatic hernia poses a unique challenge to umbilical venous catheterisation. Based on the radiographic position of umbilical vein catheter, umbilical venous cannulation was attempted; however, the catheter could not be navigated to the right atrium. Saline contrast echocardiography was used to delineate the abnormal umbilical and ductus venosus drainage. Eventually, the procedure was successfully completed via the femoral venous approach. We emphasise the importance of defining ductus venosus anatomy and umbilical venous drainage using a simple tool like saline contrast echocardiography before performing catheterisation using the umbilical venous access in such cases.


2000 ◽  
Vol 279 (3) ◽  
pp. H1166-H1171 ◽  
Author(s):  
Torvid Kiserud ◽  
Takashi Ozaki ◽  
Hidenori Nishina ◽  
Charles Rodeck ◽  
Mark A. Hanson

To study the regulation of the ductus venosus (DV) inlet in vivo, we measured the effect of vasoactive substances and hypoxemia on its diameter in nine fetal sheep in utero at 0.9 gestation under ketamine-diazepam anesthesia. Catheters were inserted into an umbilical vein and a fetal common carotid artery, and a flowmeter was placed around the umbilical veins. Ultrasound measurements of the diameter of the fetal DV during normoxic baseline conditions [fetal arterial Po2(Pao2) 24 mmHg] were compared with measurements during infusion of sodium nitroprusside (SNP; 1.3, 2.6, and 6.5 μg · kg−1· min−1) or the α1-adrenergic agonist phenylephrine (6.5 μg · kg−1· min−1) into the umbilical vein or during hypoxemia (fetal PaO2reduced to 10 mmHg). SNP increased the DV inlet diameter by 23%, but phenylephrine had no effect. Hypoxemia caused a 61% increase of the inlet diameter and a distension of the entire vessel. We conclude that the DV inlet is tonically constricted, because nitric oxide dilates it but an α1-adrenergic agonist does not potentiate constriction. Hypoxemia causes a marked distension of the entire DV.


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