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Larsen syndrome consists of skeletal dysplasia with multiple joint dislocations and a characteristic facies. The basis of this abnormality is a generalized mesenchymal disorder involving connective tissues. We describe our findings in a woman who was referred at 28 weeks' gestation due to multiple fetal anomalies suspected initially at an 18-week ultrasound examination. On three-dimensional (3D) ultrasound we found the fetus had bilateral genu recurvatum. Further 3D examination at 36 weeks confirmed the lower limb anomaly and revealed facial anomalies that led to the diagnosis of Larsen syndrome. An elective Cesarean section was performed at 38 weeks' gestation to minimize neurological sequelae. Magnetic resonance imaging was performed postnatally and showed pachygyria, colpocephaly and agenesis of the corpus callosum. In this case, 3D ultrasound facilitated the prenatal diagnosis of Larsen syndrome. A careful prenatal investigation for other associated anomalies such as those of the cardiovascular or neurological systems is warranted with this diagnosis. These associated lesions are likely to have a greater impact on prognosis than the classic symptoms of Larsen syndrome and a collaborative approach is necessary to optimize delivery and postnatal management of an affected fetus., (Copyright 2004 ISUOG. Published by John Wiley & Sons, Ltd.)

A fetus with a huge hepatic tumor was detected by sonography at 36 weeks of gestation. The mass appeared as a single, solid and polylobular tumor located in the right lobe of the liver. Foci of hemorrhage, necrosis and some tiny calcifications were seen. The adjacent right kidney appeared normal but was displaced. The right adrenal gland was not visualized. Three-dimensional power Doppler sonography further depicted the corresponding vascular anatomy of the tumor, including its vascularization pattern and blood supply. The tumor was situated to the right of the umbilical vein and portal sinus, possibly deriving its blood supply from the portal circulation. The fundamental findings suggested the diagnosis of hepatoblastoma by exclusion of other possibilities. The baby was delivered by Cesarean section at 36 weeks' gestation, due to signs of fetal distress. Unfortunately, hypotension, tachycardia, and tachypnea developed shortly after birth. Surgical intervention was performed, but intractable bleeding occurred intra-operatively. The infant died at 6 days of age. Autopsy confirmed the diagnosis of hepatoblastoma. We believe this is the first reported case of the antenatal diagnosis of congenital hepatoblastoma.

A fetus with a large supratentorial cyst and cardiomegaly was encountered at 33 weeks of gestation. The cyst appeared as an aneurysmal, fluid-filled structure extending posteriorly with a finger-like appendage. Using color flow mapping, we disclosed rapid in-and-out blood flow with marked turbulence within the cyst. For evaluation of its blood supply and venous drainage of the vascular malformation, a three-dimensional reconstruction of the power Doppler image was conducted. The results revealed that the vascular malformation was supplied by a small contralateral aneurysm from the branches of Willis' circle, draining posteriorly into an abnormal falcine sinus and then into the superior sagittal sinus. No other fetal abnormality such as hydrocephalus or hydrops was discovered. The prenatal diagnosis of an aneurysm of the vein of Galen was made on the basis of the gray-scale, color Doppler findings, and also the angioarchitecture obtained by three-dimensional power Doppler imaging. The woman was admitted at 37 weeks of gestation due to labor onset and delivered the baby via the vaginal route without complication. Postnatal angiography and magnetic resonance imaging confirmed the diagnosis of an aneurysm of the vein of Galen, and the angioarchitecture depicted it before birth. We suggest that three-dimensional power Doppler ultrasonography may assist in the diagnosis of an aneurysm of the vein of Galen, and precisely delineate the complicated corresponding vasculature. This may guide postnatal management and predict the prognosis more accurately.

Objective: To evaluate the feasibility of examining the fetal ear with three-dimensional ultrasound., Methods: In 125 pregnancies between 19 and 38 weeks of gestation, fetal ears were evaluated by three-dimensional ultrasound. The volume images with surface rendering were analyzed to depict the morphology, lying axis, orientation, and cranial location of the fetal ears., Results: Three-dimensional images of one or both ears were successfully reconstructed in 105 fetuses. Among them, 18 fetuses had anomalous ears. The anomalous ears, including microtia, low-set ear with slope axis, abnormal ear orientation, and edematous ear, were confirmed after delivery. Three-dimensional ultrasound consistently displayed fetal ear abnormalities with greater accuracy and clarity., Conclusion: Because anomalous ears may be a part of complex fetal malformations, it is important to recognize ear abnormalities. Due to the complexity of the fetal ear, three-dimensional ultrasound offers more important information than two-dimensional ultrasound, which simply gives auricular geometry. We suggest that three-dimensional ultrasound can be used better to examine the fetal ear and may prove to be useful for prenatal diagnosis and genetic counseling.

Klippel Trenaunay-Weber syndrome is a complex developmental disorder characterized by a triad of cutaneous haemangioma, varicosities of the body, and unilateral limb hypertrophy. We describe the prenatal diagnosis of Klippel-Trenaunay-Weber syndrome at 15 weeks' gestation using the surface rendering technique of three-dimensional ultrasound. The vivid three-dimensional images of the affected fetus are invaluable in prenatal diagnosis and parental counselling.

Objective: To determine the effect of cardiac valve replacement on pregnancy outcome., Methods: We reviewed retrospectively 151 pregnancies in 88 women: 56 pregnancies in 31 women with mechanical valves and anticoagulation therapy, and 95 pregnancies in 57 women with porcine tissue valves. Student t, chi 2, and Fisher exact tests were used for analysis., Results: There was a significantly greater rate of fetal loss in patients with mechanical valve replacements than in those with porcine tissue valves (27.7 versus 12.3%, respectively; P < .05). No significant differences were found in prematurity (5.9 versus 7.7%) or small for dates infants (8.8 versus 10.8%). Two congenital anomalies were noted in the mechanical valve group. Maternal complications in patients with mechanical valves included valve dysfunction (three), thromboembolism (three), abruptio placentae (two), postpartum hemorrhage (two), severe oligohydramnios (two), and puerperal fever caused by brain abscess (one). Four cases of valve dysfunction and one of infectious endocarditis complicated the condition of patients with porcine tissue valves. The 10-year graft survival rate in the porcine valve group was lower following two subsequent pregnancies (16.7%) than following one (54.8%)., Conclusion: Fewer fetal and maternal complications occurred in subsequent pregnancies after porcine valve replacement. However, the need for reoperation is more likely, and pregnancy might accelerate the degenerative process.

The article presents a case study of a 30-year-old woman who is at her 26th week of gestation with fetal growth restriction (FGR). A repeated ultrasound evaluation was performed and showed that the estimated fetal weight is 460 grams. It notes that the woman received 25 milligrams (mg) sildenafil citrate for three times daily for the management of FGR. It concludes that the sildenafil titrate could show its efficacy in treating early-onset GFR by decreasing uterine artery impedance.