Fetal middle cerebral artery peak systolic velocity in the investigation of non-immune hydrops.

OBJECTIVE: In some cases of non-immune hydrops there is congenital or acquired fetal anemia. The aim of this study was to investigate the potential value of fetal middle cerebral artery peak systolic velocity (MCA-PSV) in the assessment and management of non-immune hydrops due to anemia. METHODS: Fetal MCA-PSV and fetal hemoglobin concentration, in blood obtained by cordocentesis, were measured in 16 singleton pregnancies referred to our unit for further investigations because of a diagnosis of non-immune hydrops fetalis. In all cases a detailed ultrasound examination demonstrated moderate or severe ascites, with or without skin edema, and pericardial or pleural effusions. Furthermore, there were no obvious malformations to account for the hydrops. In each fetus the measured MCA-PSV and hemoglobin concentration were expressed as delta values (the difference in SD from the normal mean for gestation). Regression analysis was used to determine the significance of the association between delta MCA-PSV and delta fetal hemoglobin concentration. In addition, we searched our database to identify the sonographic features and hemoglobin concentration of fetuses with congenital infection. RESULTS: In the 16 cases of non-immune hydrops there were seven with parvovirus B19 infection, one each of alpha-thalassemia and primary cardiomyopathy and seven with no obvious explanation for the hydrops. There was a significant association between delta MCA-PSV and delta hemoglobin concentration (delta hemoglobin = (delta MCA-PSV + 0.1437)/-0.4154; R(2) = 0.7202; P < 0.0001). In 10 of the cases the fetal hemoglobin concentration was more than 4 SD below the normal mean for gestation and in all these cases the MCA-PSV was more than 2 SD above the normal mean for gestation. Our computer search identified an additional nine fetuses with parvovirus B19 infection and in all cases the predominant sonographic finding was ascites and the hemoglobin concentration was more than 4 SD below the normal mean. In contrast, only 3/14 fetuses with cytomegalovirus, toxoplasmosis, coxsackie B or Treponema infection had ascites and only 2/14 had a hemoglobin deficit of 4-6 SD. CONCLUSION: In the management of non-immune hydrops, measurement of fetal MCA-PSV can help identify the subgroup with fetal anemia.     

Pathophysiology and treatment of fetal anemia due to placental chorioangioma.

Placental chorioangiomas occur in 1% of pregnancies. Large chorioangiomas may cause serious complications such as fetal anemia, hydrops and fetal death. In this case report, a pregnancy complicated by a large placental chorioangioma is described. Severe fetal anemia without the occurrence of hydrops fetalis was suspected using ultrasound and Doppler examinations. Successful intrauterine blood transfusion was performed, with an unusually large amount of blood needed to obtain an adequate rise in fetal hematocrit. Two weeks later, at 32 weeks, the infant was born in good condition. In pregnancies with large chorioangiomas, we advise regular ultrasound and Doppler examinations, with the aim of detecting fetal anemia before hydrops develops. When anemia is suspected, fetal blood sampling is indicated and intrauterine transfusion therapy may be beneficial to preserve fetal health until maturity is reached.     

Intrauterine management of fetal parvovirus B19 infection.

OBJECTIVES: The aim of our study was to determine the outcome of pregnancies after intrauterine management of fetal parvovirus B19 infection. DESIGN: Retrospective study. SUBJECTS: A total of 37 cases of maternofetal parvovirus B19 infection, 35 of which were associated with hydrops fetalis, were referred to our tertiary level center between 1989 and 1996. With regard to fetal hydrops, no apparent cause other than parvovirus B19 infection was found in any patient. METHODS: In all patients, cordocentesis was performed to assess the degree of fetal anemia. When anemia was present, cordocentesis was followed by intrauterine transfusion with packed red cells into the umbilical vein. Further management depended on the degree of fetal anemia and gestational age and included follow-up fetal blood sampling/transfusion as well as ultrasound examinations as deemed appropriate. RESULTS: Packed red cell transfusion was performed in 30 patients with significant fetal anemia (Z-score 1.6-7.8 below the mean for gestational age). The fetal hemoglobin values ranged from 2.1 to 9.6 g/dl. Serum levels of platelets in the transfusion group were 9-228 x 10(9)/l with Z-scores in the range of < 1 to 3.8 below the mean. During treatment and follow-up, there were five intrauterine deaths (13.5%), one neonatal death (2.7%) and 31 live births (83.8%). CONCLUSIONS: Fetal parvovirus infection can lead to marked anemia and hydrops formation. Cordocentesis allows precise assessment of fetal anemia which can then be corrected by intravenous transfusion. Under this regimen, the outcome proved favorable in the majority of fetuses, even those that were severely anemic.     

Jugular vein and carotid artery blood flow in fetuses with increased nuchal translucency at 10-14 weeks' gestation.

OBJECTIVE: The aim of our study was to obtain measurements of the jugular vein and carotid artery pulsatility index (PI) at 10-14 weeks' gestation in chromosomally normal and abnormal fetuses with or without increased nuchal translucency (NT), in order to explore whether a relationship exists between increased NT and overperfusion of the head. METHODS: This was a prospective study involving 179 pregnant women at high risk for chromosomal anomalies or structural malformations who were referred for chorionic villus sampling or first-trimester ultrasound examination at 10-14 weeks' gestation, respectively. Color and pulsed Doppler ultrasound were used to obtain jugular vein and carotid artery blood flow velocity waveforms at the level of the mid-neck. All Doppler measurements were obtained by a single investigator. The PIs of the jugular vein and carotid artery were correlated with NT measurement and fetal karyotype. RESULTS: Doppler measurements of the jugular vein and carotid artery were successfully obtained in 90.5% of the fetuses. The fetal karyotype was abnormal in 13 cases, including three trisomies 21 and two trisomies 18, and normal in 149 cases. In the group with normal karyotype the NT was above the 95th percentile in 22 cases (15%). No correlation between the jugular vein or the carotid artery PI and the thickness of the NT was found. There were no significant differences when comparing the values of the jugular vein and carotid artery PI between the group with normal NT and the group with increased NT, or between the group with a normal karyotype and an abnormal karyotype. CONCLUSION: Our results suggest that NT is not related to blood flow impedance in either the carotid artery or the jugular vein. Overperfusion and venous congestion of the head do not appear to be a causative pathophysiological mechanism involved in increased NT.     

Serial sonographic findings of four fetuses with homozygous alpha-thalassemia-1 from 21 weeks onwards.

OBJECTIVES: To evaluate the potential usefulness of noninvasive ultrasound assessment of fetal anemia in the diagnosis and management of fetuses with homozygous alpha-thalassemia-1. METHODS: We describe four pregnancies complicated by fetal homozygous alpha-thalassemia-1. They presented with ultrasound abnormalities before the development of hydrops. As part of evaluating the fetal condition, we performed ultrasound and Doppler studies aimed at identifying fetal anemia. These studies included evaluation of intrahepatic umbilical venous maximum flow velocity, middle cerebral artery peak flow velocity, fetal liver length and spleen perimeter. RESULTS: In all four fetuses, ultrasound and Doppler studies suggested the presence of fetal anemia. Homozygous alpha-thalassemia-1 was diagnosed in all cases, with fetal blood sampling confirming anemia in three fetuses. The majority of the intrahepatic umbilical venous maximum flow velocity and middle cerebral artery peak flow velocity measurements were above the 95th centile. Two fetuses underwent intrauterine transfusion and fetal blood flow velocities returned to normal after correction of the fetal anemia. The fetal liver length and spleen perimeter measurements showed a similar trend, although they were less consistent before 28 weeks. CONCLUSION: Non-invasive ultrasound parameters, in particular quantification of intrahepatic umbilical venous maximum flow velocity and middle cerebral artery peak flow velocity, were found to be useful in the diagnosis and management of fetal anemia in pregnancies with fetal homozygous alpha-thalassemia-1.     

Doppler sonography for predicting fetal anemia caused by massive fetomaternal hemorrhage

Fetomaternal hemorrhage (FMH) can cause severe anemia in the fetus. Untreated, this may cause hydrops or even fetal death. However, correct diagnosis of FMH followed by blood transfusion can prevent these life-threatening consequences. We describe two cases in which fetal anemia was suspected because of maternal reporting of decreased or absent fetal movements, the detection of a sinusoidal heart rate pattern and increased blood flow velocities of the middle cerebral artery and umbilical vein. Together with the Betke-Kleihauer test showing fetal cells in the maternal circulation, this led to the correct diagnosis of severe fetal anemia caused by FMH. A Cesarean section was performed within a few hours. Both neonates were severely anemic and received immediate blood transfusions. They are currently alive and well.     

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.     

Twin reversed arterial perfusion sequence in twin-to-twin transfusion syndrome after the death of the donor co-twin in the second trimester.

A twin-to-twin transfusion syndrome was diagnosed in a monochorionic-diamniotic pregnancy at 18 weeks' gestation without any malformation, especially heart defect. In spite of the aggressive treatment (serial amnioreduction, digoxin treatment) the donor twin died at 25 weeks and twin reversed arterial perfusion (TRAP) sequence developed and was documented by Doppler ultrasound. In the TRAP-twin, the route of the reversed blood flow from the umbilical arteries was as follows: descending aorta, aortic arch, ascending aorta, aortic valve, left ventricle, mitral valve, left atrium, foramen ovale, right atrium, inferior vena cava, ductus venosus; and back to the placenta through the umbilical vein. After a 12-h observation period the twin reversed arterial perfusion sequence disappeared. During this period ultrasound and fetal blood sampling revealed no sign of fetal anemia or disseminated intravascular coagulation in the surviving twin. Based on our observations, we propose, that the death of one of the twins in monochorionic pregnancy can result in twin reversed arterial perfusion sequence, which is an ultimately rare phenomenon in the second trimester. To our knowledge, this is the first reported case of twin reversed arterial perfusion sequence subsequent to the intrauterine demise of one twin in twin-to-twin transfusion syndrome in which the TRAP-twin had no cardiac malformation.     

Spontaneous rupture of sacrococcygeal teratoma associated with acute fetal anemia.

Sacrococcygeal teratoma (SCT) is the most common congenital tumor, with affected fetuses having a high risk of perinatal complications and death. We report a case of a fetus with an SCT that developed acute anemia due to spontaneous rupture of the tumor in utero. The fetus was referred at 25 weeks' gestation for evaluation of a large solid and cystic mass in the sacral region. There were no signs of hydrops or placentomegaly. At 33 weeks' gestation, loss of variability in the fetal heart rate pattern was recorded. Doppler ultrasonography showed increased middle cerebral artery peak systolic velocity, suggesting fetal anemia. Markedly bloody amniotic fluid, with 82% hemoglobin F, was observed on amniocentesis, suggesting rupture of the SCT with active fetal bleeding. An emergency Cesarean section was performed. At delivery, the tumor was bleeding actively and the neonatal hemoglobin concentration was 3.1 g/dL. There were no findings of hemorrhage or necrosis within the tumor. The neonate received a blood transfusion, and surgical resection of the tumor was carried out on the first day after delivery. Postoperatively, the baby did well. We suggest that fetal SCTs run the risk of inducing acute fetal anemia due to intrauterine hemorrhage of the tumor, a finding which could lead to an earlier and more appropriate management of this condition.     

Prediction of fetal anemia in rhesus disease by measurement of fetal middle cerebral artery peak systolic velocity.

OBJECTIVE: In red blood cell (RBC) isoimmunized pregnancies fetal anemia is associated with a hyperdynamic circulation. The aim of the present study was to examine further the possible value of fetal middle cerebral artery peak systolic velocity (MCA-PSV) in the management of affected pregnancies. METHODS: A reference range of fetal MCA-PSV with gestation was constructed from the study of 813 normal singleton pregnancies at 20-40 weeks' gestation. Fetal MCA-PSV was also measured in 58 fetuses from RBC isoimmunized pregnancies, with maternal hemolytic antibody concentration of >15 IU/mL at 19-38 weeks' gestation and within 10 days of measurement of fetal hemoglobin concentration in blood obtained either by cordocentesis (n = 43) or at delivery (n = 15). In the RBC isoimmunized pregnancies each of the measured MCA-PSV and hemoglobin concentrations was expressed as a delta value (difference in SDs from the normal mean for gestation). Regression analysis was used to determine the significance of the association between delta MCA-PSV and delta fetal hemoglobin concentration. RESULTS: In the normal pregnancies there was a significant increase in fetal MCA-PSV with gestation (mean MCA-PSV = 10(0.0223 x GA + 0.963)). In RBC isoimmunized pregnancies the fetal MCA-PSV was increased and there was a significant association between delta MCA-PSV and delta hemoglobin concentration (delta hemoglobin = (delta MCA-PSV + 0.093)/-0.356; R(2) = 0.638, P < 0.0001). An MCA-PSV of mean + 1.5 SDs detected 96% of severely anemic fetuses, with a hemoglobin deficit of at least 6 SDs, for a false-positive rate of 14%. CONCLUSION: Measurement of fetal MCA-PSV is a useful method of assessing fetal anemia. In the clinical management of isoimmunized pregnancies a cut-off in MCA-PSV of mean + 1.5 SDs can identify nearly all severely anemic fetuses with a low false-positive rate.     

Assessment of umbilical arterial and venous flow using color Doppler.

OBJECTIVE: To estimate the umbilical artery and vein blood volume flow using B-mode and Doppler ultrasound in the second and third trimesters of pregnancy. DESIGN: This was a cross-sectional study of 129 singleton, healthy pregnancies at 23-33 weeks' gestation. The umbilical artery and vein cross-sectional area, time-averaged velocity and pulsatility index were measured in a free loop of cord, and the fetal weight was estimated. Ranges for each parameter were obtained; from these the blood flow for the vein and artery was calculated, and the average flow corrected for fetal weight was derived. RESULTS: The median time for examination was 6 min. The mean cross-sectional area and time-averaged velocity for both the vein and artery increased linearly with gestation. The umbilical artery flow correlated closely with the average vein flow (r = 0.9, p < 0.001). There was a significant, though poor, inverse correlation between the umbilical artery pulsatility index and the average umbilical flow (r = -0.25, p < 0.05). The average umbilical flow (calculated from the mean of arterial and venous flow), corrected for estimated fetal weight, decreased from 189.2 ml/kg per min at 23 weeks to 176.2 ml/kg per min at 33 weeks' gestation. CONCLUSION: The estimates of fetal umbilical flow obtained by this Doppler method are consistent with previously published data. Averaging the arterial and venous flow is theoretically advantageous in reducing the inherent errors in estimating either the arterial or the venous flow. This method of measuring umbilical flow may have clinical potential in assessing fetal health and disease processes.     

Fetal hemolytic anemia and intrauterine death caused by anti-M immunization

BACKGROUND: Antibodies with anti-M specificity are detected in 10 percent of pregnant women with a positive antibody screen, but anti-M is only rarely associated with hemolytic anemia in the fetus. STUDY DESIGN AND METHODS: This study reports on three pregnancies in one family that all resulted in severe fetal anemia. The first fetus died in utero with hydrops fetalis during the 20th gestational week and the second child was delivered after 28 weeks of gestation with hydrops fetalis and a hemoglobin level of 16 g per L whereas the third affected child was treated with intrauterine red cell (RBC) transfusions before delivery at 28 weeks of gestation. RESULTS: The direct antiglobulin test was negative but anti-M in a low titer was detected through the three pregnancies, and its clinical relevance, which initially was uncertain, was confirmed by pronounced in vivo hemolysis in maternal blood of chromate ((51)Cr)-labeled M+ RBCs and normal survival of (51)Cr labeled M- RBCs. CONCLUSION: It is concluded that anti-M immunization in a few cases may cause severe fetal hemolytic anemia and intrauterine death. It remains to be elucidated why a normally clinically insignificant antibody is this aggressive in a small proportion of cases. Because the condition is treatable, anti-M must be considered as a possible cause of fetal anemia and intrauterine death.     

Non-invasive tests to predict fetal anemia in Kell-alloimmunized pregnancies.

OBJECTIVES: To compare test characteristics of ultrasound and Doppler parameters in the prediction of fetal anemia in Kell-alloimmunized pregnancies. METHODS: In this prospective study, 27 fetuses at risk for anemia due to Kell alloimmunization were evaluated with ultrasound and Doppler imaging. Spleen perimeter, liver length, middle cerebral artery (MCA) peak systolic velocity and intrahepatic umbilical venous (IHUV) maximum velocity were measured. Results were compared with fetal hemoglobin values at first intrauterine blood sampling or delivery. Severe fetal anemia was defined as a hemoglobin deficit of at least 5 SD below the mean for gestational age. RESULTS: Eighteen fetuses were anemic and required intrauterine transfusions. In the other nine pregnancies no transfusions were performed; one of these fetuses was severely anemic at birth. MCA and IHUV flow velocities were the best predictors of fetal anemia in Kell alloimmunized fetuses (sensitivity 89% for each test). Sensitivities for spleen perimeter (15%) and liver length (14%) were disappointing. CONCLUSIONS: Doppler evaluation of MCA peak systolic velocity and IHUV maximum velocity can be used to reliably predict severe fetal anemia in Kell alloimmunization.     

Interstitial pregnancy undetected during earlier first-trimester screening for fetal aneuploidy at 13 weeks' gestation

First-trimester screening for fetal aneuploidy using nuchal translucency (NT), pregnancy-associated plasma protein A, free or total beta-hCG, and maternal age constitutes a very effective screening test for fetal Down syndrome. We describe a case in which a patient presented at 14 weeks' gestation with an acute abdomen 1 week after first-trimester screening (including NT measurement) performed elsewhere, which was negative for trisomies 21 and 18. Sonographic examination revealed an interstitial pregnancy with a singleton fetus with present cardiac activity, which had not been noted 1 week earlier at the time of earlier transabdominal NT measurement. This case indicates that successful acquisition of a NT measurement during first-trimester screening for fetal aneuploidy does not negate the rare possibility of an unusual ectopic pregnancy.     

Marked splenomegaly in fetal cytomegalovirus infection: detection supported by three-dimensional power Doppler ultrasound.

An enlarged fetal spleen can be associated with fetal infection, anemia and different syndromes but its prenatal diagnosis is rare. We report on a diagnosis of splenomegaly at 32 weeks' gestation in a fetus which was found to be affected by cytomegalovirus infection. An enlarged spleen was suspected when the stomach was found to be displaced anteriorly and medially and the diagnosis was supported on visualization of the splenic vessels by color and three-dimensional power Doppler ultrasound. The patient had been referred because of fetal growth restriction and intracerebral anomalies and the additional finding of splenomegaly was highly suspicious for cytomegalovirus infection. This was confirmed by positive maternal serology and by neonatal virus excretion in urine. Retrospectively, examination of stored blood samples from 9 and 23 weeks' gestation revealed an early cytomegalovirus infection. Antenatal and neonatal magnetic resonance imaging examinations showed microcephaly, lissencephaly and the presence of microcalcifications. At the age of 9 months, the child suffers from severe neurological impairment and blindness due to severe optical atrophy. This case emphasizes that color Doppler and three-dimensional power Doppler ultrasound can facilitate the antenatal diagnosis of splenomegaly and can help to delineate the spleen from the similar-looking neighboring liver.     

Intrauterine transfusion via umbilical vein in severe Rh isoimmunization

Although the widespread use of anti-D immune globulin has dramatically reduced the incidence of Rh isoimmunization, an occasional pregnant patient becomes a candidate for intrauterine transfusion because of sensitization to Rh antigens or irregular red blood cell antigens. Current methods of ultrasonography provide needle guidance to the umbilical vein, permitting fetal intravascular transfusion. We have reported a case involving five separate intrauterine transfusions via the umbilical vein.     

The pathophysiology of acute syphilitic funisitis.

There is an increase in the number of cases of syphilis in pregnancy in the United States. Fetal death may occur in syphilis from acute or chronic infections. A case is presented in which an acute fetal infection occurred. The patient presented at 31 weeks' gestation, with a decrease in fetal movements and non-reactive cardiotocography. Ultrasound and Doppler analysis of the fetal heart, cerebral and umbilical arteries, aorta and umbilical vein led to the suspicion of acute cardiac failure. An amniocentesis yielded a white cell count of 1122 white blood cells, with 91% polymorphs, but the Gram stain was negative. The fetus developed a persistent bradycardia and was delivered. The diagnosis of acute severe syphilitic funisitis was suspected from histological sections of the cord. Diagnosis was established from maternal and fetal blood. Modern ultrasound techniques, including imaging, Doppler and cardiotocography, can lead to the analysis of the pathophysiology of disease states. An acute syphilitic infection should be suspected when this constellation of findings is found.     

First-trimester diagnosis of micrognathia as a presentation of Pierre Robin syndrome.

We describe a case of first-trimester diagnosis of micrognathia. A transvaginal ultrasound scan performed at 13 weeks' gestation revealed an abnormal fetal facial profile consisting of a small mandible and a receding chin. A subsequent amniocentesis at 15 weeks' gestation revealed a normal karyotype. A morphology scan and subsequent postmortem examination at 19 weeks confirmed the first-trimester findings and revealed, in addition to the facial pathology, a complex cardiac abnormality and unilateral talipes equinovarus. These findings are consistent with the diagnosis of Pierre Robin syndrome.     

胎儿呼吸运动对于脐静脉血流的影响及意义

目的探讨妊娠晚期胎儿呼吸运动对脐静脉血流的影响及意义.方法随机选择75例孕龄36～40周,无产兆的正常孕妇,用脉冲多普勒观察胎儿呼吸运动时脐静脉血流的频谱形态、速度,并与无呼吸运动时进行自身对照.结果妊娠晚期胎儿呼吸运动时,脐静脉血流频谱形态、速度与非呼吸运动时明显不同.胎儿呼吸运动状态下,脐静脉血流频谱表现为周期性、波浪状静脉血流频谱,并且血流瞬时平均速度明显高于后者(P＜0.05).结论呼吸运动时脐静脉血流速度暂时、波浪式增加,间断性地提高了心脏的前负荷,促进着胎儿心脏收缩功能的发育.     

孕早期颈项透明层超声联合孕中期彩色多普勒超声可提高产前胎儿畸形检出率

目的探究孕早期颈项透明层（NT）超声联合孕中期彩色多普勒超声在产前胎儿畸形筛查中的应用价值。方法选择2017年8月~2019年12月在我院行胎儿畸形筛查的2417例孕妇作为研究对象,所有孕妇于孕11~14周行NT超声检查,并于孕22~28周行二维、四维彩超检查。以引产或分娩结果为“金标准”,比较畸形胎儿、正常胎儿的NT值及NT异常率,对比NT超声检查、二维联合四维彩超检查结果,并就NT超声、二维及四维彩超及二者联合在诊断胎儿畸形中的诊断率进行比较。结果2417例孕妇最终确诊异常胎儿88例,发病率为3.64%。畸形胎儿的NT值明显高于正常胎儿,NT异常率（93.18%）明显高于正常胎儿（1.33%）,差异比较有统计学意义（P < 0.05）。NT超声检查共检出异常胎儿82例,诊断准确率为93.18%;二维联合四维彩超共检出异常胎儿85例,诊断准确率为96.59%。二维联合四维彩超的诊断准确率略高于NT超声检查,差异无统计学意义（P>0.05）。NT超声联合二维、四维彩超在诊断胎儿畸形中的敏感度、特异度和准确度分别为100.00%、99.57%、99.59%,高于NT超声、二维和四维彩超（分别为93.18%、98.67%、98.47%和96.59%、99.06%、98.97%）,组间比较差异有统计学意义（P < 0.05）。结论孕早期NT超声及孕中期彩色多普勒超声在产前筛查胎儿畸形上各具有优势,均具有较好的诊断价值。二者联合应用能够进一步提升胎儿畸形的检出率,对尽早终止胎儿畸形孕妇继续妊娠,减少畸形胎儿出生具有重要意义。