Megacystis Associated With an Umbilical Cord Cyst

Objective. The purpose of this series was to report the first‐trimester sonographic findings, antenatal course, and outcome in fetuses with a patent urachus. Methods. We conducted a review of ultrasound reports and medical charts from 3 pregnancies complicated by a congenital patent urachus detected in the first trimester. Results. All 3 fetuses had megacystis and an umbilical cord cyst close to the fetal abdominal insertion that was detected in the first trimester. In 2 cases, the megacystis resolved spontaneously while the umbilical cord cyst worsened in appearance; among them, massive edema of the umbilical cord was documented in 1, and evidence of partial bladder exstrophy was detected in the third trimester in the other. Both cases required neonatal surgery with no complications. In the remaining case, the fetus died in the early second trimester. Postmortem examination confirmed the diagnosis of a patent urachus, an allantoic cyst, and thrombosis of the umbilical vessels. Conclusions. Megacystis is a warning sign of a patent urachus in the first trimester. The prognosis of this condition is generally good; however, fetal death can occur secondary to compression of umbilical vessels due to the expanding allantoic cyst.     

Prenatal diagnosis of umbilical cord aneurysm in a fetus with trisomy 18.

Aneurysm of the umbilical cord is an extremely rare vascular anomaly. We report a case of umbilical cord aneurysm with arteriovenous fistula in a fetus with trisomy 18. At 34 weeks' gestation a fetus with suspected intrauterine growth restriction and oligohydramnios was referred to our institution. Ultrasound examination was confirmatory and additionally revealed multiple markers for trisomy 18. In the umbilical cord an aneurysm was diagnosed characterized by a cystic lesion with hyperechogenic rim measuring 18 x 18 x 19 mm in diameter. Color flow and spectral Doppler examinations showed a jet originating from one of the umbilical arteries entering the cystic lesion which appeared to be the dilated umbilical vein. Fetal blood sampling and subsequent karyotyping revealed trisomy 18 (47, XY, +18). The patient elected to terminate the pregnancy. Pathologic examination of placenta and umbilical cord confirmed the prenatal diagnosis of umbilical cord aneurysm and arteriovenous fistula. Histology demonstrated a strongly dilated umbilical vein, one moderately dilated artery and a second, apparently normal artery.     

Large pseudocyst of the umbilical cord detected in the second trimester

We present a case of a large umbilical cord cyst detected at 21 weeks of gestation. Serial ultrasonographic examination revealed a single umbilical artery and progression of the cystic mass. A 2,842-g male infant was delivered at 37 weeks of gestation, and we confirmed that the umbilical cord cyst was a pseudocyst in our pathological examination. This case demonstrated an uneventful course of pregnancy despite the large umbilical cord pseudocyst.     

Umbilical cord cyst detected in the first trimester by two- and three-dimensional sonography

A case of umbilical cord cyst was identified via 2-dimensional and 3-dimensional sonographic examination at 8 weeks' menstrual age. The cyst was solitary, measuring 18.0 mm, and it was located close to the placental insertion on the umbilical cord. The gestational sac and yolk sac diameters and the fetal heart rate were within normal ranges for menstrual age. Follow-up 3-Dimensional sonographic examination in the second trimester showed complete resolution of the cyst. Amniocentesis revealed a normal karyotype, and a normal infant was delivered at term.     

产前3D彩色多普勒超声在脐带真结节评估中的应用价值

目的探讨产前3D彩色多普勒高分辨仿真血流模式在脐带真结节评估中的应用价值。 方法选取2019年1月至2019年6月于首都医科大学附属北京妇产医院超声科产检并于产前拟诊为脐带打结的胎儿6例，6例均于产后得到证实。所有病例均行二维超声及彩色多普勒超声检查，可疑脐带打结时，应用3D高分辨仿真血流进行观察。对6例脐带真结节病例的超声影像学表现进行分析。 结果6例病例中，1例为单绒毛膜单羊膜囊双胎，余均为单胎；5例剖宫产结束妊娠，1例自然分娩。6例脐带真结节病例中，1例单绒毛膜单羊膜囊双胎的两胎儿脐带互相缠绕形成双"8"字、类似中国结样复杂的结节特征，5例单胎胎儿表现为单个"8"字结样特征，其中2例伴脐带局部增粗、水肿及脐带囊肿，1例同时合并脐带静脉血栓。 结论对于可疑脐带打结及存在高危因素的病例，需重点探查脐带以减少漏诊脐带真结节；3D彩色多普勒高分辨仿真血流技术可立体、直观显示脐带形态结构及血流，有助于脐带真结节的确定诊断。     

Prenatal diagnosis of placental chorioangioma: contribution of color Doppler ultrasound.

A case of placental chorioangioma was diagnosed at 35 weeks' gestation using color Doppler ultrasound. Color flow imaging showed an intraplacental hypoechoic mass fed directly by the anomalous chorionic tumor vessels arising from the insertion of the umbilical cord. Hypervascularization was present in some areas of the tumor. The diagnosis of typical 'angiomatous' type was confirmed by histopathological examination following delivery.     

Prenatal diagnosis of abnormal umbilical cord insertion: a rare case of furcate insertion

Furcate insertion (FI) is an extremely rare abnormality of umbilical cord insertion. One of the complications associated with FI is hemorrhage from the umbilical vein at the site of FI of the umbilical cord, which can cause sudden intrauterine fetal death. Because of its rarity, no prenatal diagnosis of FI has been reported. A 31-year-old woman at 34 weeks’ gestation was referred to us for suspected abnormal cord insertion. Ultrasonography showed normal fetal growth and amniotic fluid volume, with no fetal anomalies. Although the umbilical cord contained three vessels inserted at the center of the placenta, the umbilical vessels separated from the cord substance before their insertion to the placenta. Based on these findings, the patient was diagnosed with FI. During labor at 37 weeks’ gestation, fetal heart rate was normal and a healthy neonate was delivered. At macroscopic examination, the umbilical cord was inserted in the middle of the placenta, and the placental parenchymal tissue just under the cord insertion was deficient and had been changed to white, elastic hard tissue. Pathological examination of the white tissue revealed fibrin deposition and focal infarction. Although FI is a very rare condition, prenatal diagnosis can be achieved through detailed color Doppler ultrasound studies. Therefore, taking precautions and keeping in mind the poor fetal outcome associated with FI are preferred.     

Value of a random single Doppler study of the umbilical artery for predicting perinatal outcome

A prospective blinded study was performed on 191 high-risk patients with pregnancies ranging from 25 to 42 weeks gestation to investigate the value of a single Doppler analysis of the umbilical artery blood flow waveform (systolic-to-diastolic ratio, S/D) for predicting poor perinatal outcome. This was defined as the presence of heavy meconium, delivery of a growth-retarded infant, an umbilical cord arterial pH less than 7.2, or a 5-minute Apgar score less than 7. The interval between Doppler examination and delivery ranged from 12 hours to 15 weeks. No clinical data were available to the examiner performing the Doppler study. Moreover, the Doppler measurements were unknown to the attending physicians. The sensitivity, specificity, and positive and negative predictive values of the Doppler study in predicting outcome were 30.4%, 92.9%, 36.8%, and 92.6%, respectively, with an adverse outcome prevalence of 12%. These results indicate that a single random S/D ratio from the umbilical artery is not an adequate screening test for the risk of perinatal complications.     

Antenatal umbilical coiling index and Doppler flow characteristics.

OBJECTIVE: To evaluate whether a relationship exists between the antenatal umbilical coiling index (UCI) and umbilical cord Doppler flow characteristics. METHODS: During the fetal anatomical survey in 200 consecutive pregnant patients at 18-23 weeks' gestation, we recorded umbilical coiling patterns and blood flow characteristics. The antenatal UCI, calculated as a reciprocal value of the distance between a pair of umbilical cord coils, was compared with Doppler parameters including umbilical vein blood flow volume (in mL/min/kg), and mean resistance index (RI) and peak systolic velocity (PSV in cm/s) averaged from both umbilical arteries. RESULTS: A total of 154 patients met the inclusion criteria of singleton pregnancy and having adequate sonographic umbilical cord images, Doppler flow indices, and all demographic, antenatal and labor data. The mean antenatal UCI was 0.40, with 10th and 90th centiles of 0.20 and 0.60, respectively. The mean +/- SD umbilical artery RI and PSV and umbilical vein blood flow volume were 0.74 +/- 0.07, 25.1 +/- 6.4 cm/s, and 264 +/- 106 mL/min/kg, respectively. All Doppler variables correlated significantly with antenatal UCI, with lower RI and higher PSV and umbilical vein blood flow volume values being associated with higher antenatal UCI (P = 0.016, P < 0.001, and P = 0.032, respectively). However, when stratified by antenatal UCI into hyper- (above 90th centile), normo- (10th-90th centile), and hypocoiled (below 10th centile) umbilical cord groups, a significant difference was observed for PSV only (P = 0.016). CONCLUSION: It appears that umbilical cord coiling modulates noticeably blood flow through the umbilical cord. We speculate that more prominent umbilical coiling (higher antenatal UCI values) has a protective effect on blood flow in terms of decreased arterial resistance and higher blood flow velocities, as well as increased venous blood flow. However, due to lack of significant differences between Doppler characteristics when stratified by antenatal UCI into hypo-, normo-, and hypercoiled groups, the clinical implications of this observation are uncertain.     

Umbilical vein blood flow in fetuses with normal and lean umbilical cord.

OBJECTIVE: To evaluate whether umbilical vascular coiling is correlated with the umbilical vein blood flow profile and to investigate if this is different between fetuses with a lean and those with a normal umbilical cord. METHODS: Consecutive women with a singleton gestation who delivered at term and who underwent an ultrasound examination within 24 h from delivery were studied. Umbilical cord and vessel areas were calculated. Umbilical vein blood flow parameters were obtained by digital color Doppler velocity profile integration. After delivery, the umbilical coiling index was calculated. RESULTS: One hundred and sixteen women were studied. Twelve (10.3%) had a lean umbilical cord (area < 10th centile). A significant correlation was found between the umbilical coiling index and the umbilical vein blood flow (r = 0.67, P < 0.001). A significant difference between fetuses with and without a lean cord was found in terms of: umbilical coiling index (0.18 +/- 0.08 vs. 0.29 +/- 0.09, P < 0.005), cord area (87.6 +/- 5.1 mm2 vs. 200.6 +/- 34.6 mm2, P < 0.001), Wharton's jelly amount (25.7 +/- 10.3 mm2 vs. 122.1 +/- 33.4 mm2, P < 0.001), umbilical vein blood flow (93.7 +/- 17.8 ml/kg per min vs. 126.0 +/- 23.4 ml/kg per min, P < 0.001), and umbilical vein blood flow mean velocity (6.6 +/- 2.7 cm/s vs. 9.0 +/- 3.6 cm/s, P < 0.05). The proportion of fetuses with an umbilical vein blood flow < 80 ml/kg per min was higher when the cord was lean than when it was normal (25% vs. 1.9%, P < 0.01). CONCLUSIONS: Lean umbilical cords differ from normal cords not only from a structural point of view but also in the umbilical vein blood flow characteristics. This could explain the increased incidence of intrapartum complications and fetal growth restriction among fetuses with a lean and/or hypocoiled cord.     

Fetal umbilical cord cyst may evolve to omphalocele during pregnancy

Omphalocele is rarely complicated by umbilical cord cysts. In our case, an umbilical cord cyst and fetal ascites were detected at 26 weeks' gestation in a fetus with trisomy 13. This changed to omphalocele with subsequently absorbed fetal ascites at 35 weeks' gestation. We propose two hypotheses. The abdominal wall may have been physically pierced or an omphalocele might have preexisted, and the intestinal tract in the hernia sac was pushed by fetal ascites.     

Sonographic diagnosis of placental teratoma

Placental teratomas are very rare, and the features that distinguish them on sonography and allow their differentiation from other placental tumors have not been fully described. Prenatal recognition of this tumor is prognostically useful because, unlike other neoplasms, placental teratoma is benign and almost never associated with congenital deformities in the fetus. We describe the case of a 27-year-old pregnant woman in whom prenatal color Doppler sonographic examination performed during early-stage labor revealed a heterogeneous mass at the placental margin. This lesion, which measured 10 x 8 x 5 cm, had an echogenic focus consistent with calcification and hyperechoic foci consistent with fat. Placental teratoma and fetus acardius amorphus were considered in the differential diagnosis, but the segmental organization and umbilical cord-like structures that characterize the latter diagnosis were absent. The sonographic diagnosis, placental teratoma, was confirmed postnatally by histopathologic examination. The neonate, a healthy boy, was delivered vaginally at term. Although the presence of tissues of varied echogenicity, such as calcification, fat, and fluid, and the absence of both polarity and an umbilical cord-like structure support the sonographic diagnosis of placental teratoma, fetus acardius amorphus should be considered in the differential diagnosis. Histopathologic examination may be needed to establish a definitive diagnosis in such cases.     

Abnormal ductus venosus blood flow: a clue to umbilical cord complication.

We report a case of umbilical cord complication causing, fetal hypoxemia and acidemia. At 30 weeks of gestation, the patient was referred because of slightly increased amniotic fluid volume and a non-reactive cardiotocogram. Biometry was appropriate for gestational age. Umbilical artery and fetal aortic Doppler findings were normal, whereas diastolic blood flow velocities in the middle cerebral artery were increased and the ductus venosus showed severely abnormal flow velocity waveforms with reversal of flow during atrial contraction. Since other reasons for fetal hypoxemia could be excluded, careful examination of the umbilical cord was performed. Traction of the hypercoiled umbilical cord due to its course around the fetal neck and shoulders was suspected. Cesarean section confirmed the sonographic findings and fetal blood gases revealed fetal acidemia. This case indicates that investigation of fetal venous blood flow may also help to identify fetal jeopardy due to reasons other than increased placental vascular resistance.     

Sonographic findings of the umbilical cord: implications for the risk of fetal chromosomal anomalies.

In this review we summarize current knowledge on sonographic findings of the umbilical cord and the risk they impose for chromosomal abnormalities of the fetus. A Medline search of the literature was performed and the pertinent English-language literature was reviewed. Anatomical and Doppler abnormalities of the umbilical cord may be associated with an increased risk of chromosomal aberrations in the fetus. Therefore, level II prenatal sonography should also include a careful examination of the umbilical cord.     

Ductus venosus blood flow velocity characteristics of fetuses with single umbilical artery.

OBJECTIVES: Sonographic Doppler evaluation of the fetal ductus venosus has been proved to be useful in the evaluation of fetal cardiac function. The aim of this study was to investigate the ductus venosus blood flow profile in fetuses with single umbilical artery and to correlate it with the umbilical cord morphology. METHODS: Fetuses at >20 weeks' gestation with single umbilical artery who were otherwise healthy were consecutively enrolled into the study. The sonographic examination included evaluation of the following Doppler parameters: umbilical artery resistance index, maximum blood flow velocity of the ductus venosus during ventricular systole (S-peak) and atrial contraction (A-wave), ductus venosus time-averaged maximum velocity (TAMXV), and pulsatility index for veins (PIV). The cross-sectional area of the umbilical cord and its vessels were measured in all cases. The Doppler and morphometric values obtained were plotted on reference ranges. RESULTS: A total of 88 fetuses with single umbilical artery were scanned during the study period. Of these 52 met the inclusion criteria. The S-peak velocity, A-wave velocity, and TAMXV were below the 5th centile for gestational age in 57.7%, 59.6%, and 57.7% of cases, respectively. The PIV was within the normal range in 80.1% of cases. The umbilical vein cross-sectional area of fetuses with single umbilical artery was above the 95th centile for gestational age in 34.6% cases. CONCLUSIONS: The ductus venosus blood flow pattern is different in fetuses with single umbilical artery from that in those with a three-vessel cord. This difference may be caused in part by the particular morphology of umbilical cords with a single artery.     

Early prenatal diagnosis of cord entanglement in monoamniotic multiple pregnancies.

OBJECTIVES: Cord entanglement is a severe complication in monoamniotic multiple pregnancies. Three cases were reviewed to determine how early ultrasound diagnosis might improve counselling and management. METHODS: In two monoamniotic twin and one dichorionic diamniotic triplet pregnancies, cord entanglement was detected between 10 and 18 gestational weeks by color Doppler and pulsed Doppler velocimetry. Pregnancies were followed up on a weekly basis with special observation of fetal behavior and use of color Doppler velocimetry. RESULTS: In Case 1, a monoamniotic twin pregnancy with cord entanglement close to the umbilical insertions was diagnosed at 10 weeks. Longitudinal follow-up showed intrauterine death of both twins at 15 weeks. In Case 2, entanglement of the umbilical cords of two monoamniotic triplets within a dichorionic diamniotic triplet pregnancy was diagnosed at 10 weeks. The pregnancy continued uneventfully until 35 weeks when cord entanglement was confirmed at Cesarean section. All triplets have since developed normally. In Case 3, monoamniotic twins were diagnosed at 18 weeks. Color Doppler detected side-by-side insertion of the umbilical cords and Doppler velocimetry suggested an entanglement at the chorionic plate. The pregnancy was complicated by polyhydramnios. Cesarean section at 36 weeks confirmed cord entanglement at the chorionic plate. Postnatal computer angiography and morphological examination of the placenta showed the presence of superficial artery-to-artery and vein-to-vein anastomoses and of deep arteriovenous shunts. The development of the twins was uneventful. CONCLUSIONS: Diagnosis of cord entanglement is feasible early in gestation. Future protocols are proposed to document the gestational age at detection, the location, and the Doppler flow patterns and to facilitate the assessment of short- and long-term development.     

Testicular epidermoid cysts: sonographic features with histopathologic correlation

Testicular epidermoid cysts are rare, accounting for 1% of all testicular tumors. We present the sonographic appearances of epidermoid cysts in 3 cases, together with the histopathologic correlation. In case 1, sonography showed an intratesticular hypoechoic mass with a well-defined echogenic rim; the mass measured 1.8 x 1.5 x 1.5 cm, and there was no evidence of calcification. In case 2, sonography showed a well-circumscribed mass measuring 1.3 x 1.3 x 1.0 cm, with alternating hypoechoic and hyperechoic rings (onion-ring appearance) and no calcifications. In case 3, sonography showed a 2.4- x 2.3- x 2.3-cm, well-circumscribed, oval mass with a heterogeneous echotexture and an outer hypoechoic halo. The mass contained plaque-like regions of increased echogenicity, with peripheral acoustic shadowing from refraction artifact. Hypoechoic clefts were visualized posterior to the plaque-like areas. The triad of findings-sonographic appearance of an onion ring, avascularity on Doppler sonography, and negative results of tumor marker studies-is highly suggestive of an epidermoid cyst.     

Umbilical cord morphologic characteristics and umbilical artery Doppler parameters in intrauterine growth-restricted fetuses.

OBJECTIVE: To compare prenatal morphometric changes of umbilical cord components in intrauterine growth-restricted fetuses with and without abnormal umbilical artery Doppler parameters. METHODS: Consecutive singleton intrauterine growth-restricted fetuses at a gestational age of older than 20 weeks were compared with matched appropriate-for-gestational-age fetuses. Intrauterine growth restriction was defined in the presence of a sonographic abdominal circumference below the 5th percentile for gestational age at the time of sonography and a birth weight below the 10th percentile. The sonographic examination included pulsed Doppler measurements of the umbilical artery resistance index and measurements of the umbilical cord cross-sectional area and the umbilical cord vessel area. RESULTS: A total of 84 intrauterine growth-restricted fetuses and 168 appropriate-for-gestational-age fetuses were included in the study. All umbilical cord components (umbilical cord cross-sectional area, vein area, artery area, and Wharton jelly area) were smaller in the intrauterine growth-restricted fetuses. The prevalence of lean umbilical cords (cross-sectional area < 10th percentile for gestational age) was significantly higher in intrauterine growth-restricted fetuses compared with appropriate-for-gestational-age fetuses (73.8% versus 11.3%; P < .0001). A significant and progressive reduction of the umbilical vein area corresponding to the degree of umbilical artery Doppler parameter abnormality was found. The umbilical artery area was not related to the hemodynamic changes of the blood flow in the umbilical arteries. CONCLUSIONS: The proportion of lean umbilical cords was higher in intrauterine growth-restricted fetuses than in appropriate-for-gestational-age fetuses. Umbilical vein caliber decreases significantly with worsening of umbilical artery Doppler parameters.     

Prenatal color Doppler sonographic evaluation of nuchal encirclement by the umbilical cord

PURPOSE: The aim of this study was to assess whether Doppler flow velocimetry of the fetal middle cerebral and umbilical arteries is affected by nuchal encirclement by the umbilical cord (nuchal cord) in the prenatal period. PATIENTS AND METHODS: The position of the fetal umbilical cord was assessed on color Doppler sonography in pregnant women who were referred to our radiology department between September 14, 1998, and January 14, 2000. Pulsatility and resistance indices and the ratio of peak systolic blood flow velocity to diastolic velocity of the umbilical arteries and middle cerebral arteries of all fetuses were prospectively obtained with Doppler flow velocimetry. The fetuses were categorized into 2 groups: group 1 consisted of fetuses without sonographic evidence of nuchal cord and group 2 of fetuses with sonographic evidence of nuchal cord. The results were statistically analyzed with independent-groups t test. A p value of less than 0.05 was considered significant. RESULTS: During the study period, 230 pregnant women underwent sonographic examination of the fetus, and 68 fetuses were delivered. The sonographic examinations were performed at 24-41 weeks' menstrual age. Of the 46 fetuses in group 1, 1 fetus had nuchal cord at delivery; of the 22 fetuses in group 2, 4 did not have nuchal cord at delivery. The sensitivity of color Doppler sonography in detecting nuchal cord was 95% (18 of 19 fetuses), the specificity was 92% (45 of 49), the negative predictive value was 98% (45 of 46), and the positive predictive value was 82% (18 of 22). No statistically significant differences in middle cerebral artery or umbilical artery Doppler flow velocimetry values were detected between the 2 groups. CONCLUSIONS: Color Doppler sonography is a sensitive and specific method of diagnosing nuchal cord, but fetal middle cerebral artery and umbilical artery Doppler flow velocimetry values are not affected by the presence of nuchal cord in the prenatal period.     

Sirenomelia apus after trimethoprim exposure: First‐trimester ultrasound diagnosis—a case report

We report the early prenatal ultrasound diagnosis of sirenomelia apus at 12+4 weeks in a patient with trimethoprim exposure in the vulnerable period. First‐trimester scan revealed a malformed fetus with one femur, one small tibia, no feet, intraabdominal unilocular cystic structure, and two‐vessel umbilical cord with allantoic cyst. Ultrasound visualization with two/three/four‐dimensions was helpful in the process of parental counseling. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound, 2012;