Idiopathic rapidly progressive (crescentic) glomerulonephritis in pregnancy.

Idiopathic rapidly progressive glomerulonephritis is an uncommon kidney disorder characterized by the rapid deterioration of renal function in association with glomerular crescent formation on renal biopsy. End-stage kidney failure is often the final outcome. There is only one complete case report of this condition during pregnancy. We describe a patient who initially presented with classic signs and symptoms of severe preeclampsia. She delivered a premature neonate that ultimately did well, but the mother subsequently demonstrated incremental deterioration in renal function. A kidney biopsy documented crescentic glomerulonephritis, and her clinical course was consistent with rapidly progressive glomerulonephritis.

Sturge-Weber syndrome in pregnancy.

Sturge-Weber syndrome is a rare disease involving a port-wine facial nevus and an associated intracranial venous malformation. There are no reports of this disorder in association with pregnancy in the English literature, and the effects of pregnancy on this syndrome remain unknown. We present a case of Sturge-Weber syndrome diagnosed in the early postpartum period and document the possible deleterious effects of gestation on the neurologic symptoms and ultimate prognosis.

Intrathoracic congenital fibrosarcoma. A case report.

Congenital fibrosarcoma is a rare tumor that usually presents as a mass lesion involving the arm or leg of a neonate. No ultrasonographic description of such a neoplasm in the prenatal period has been reported. We present the sonographic findings of a tumor, discovered in an unusual site in utero, that, on postmortem examination, was demonstrated to be a fibrosarcoma.

Antenatal diagnosis of pulmonary sequestration: a review.

The prenatal diagnosis of pulmonary sequestration has been demonstrated in 17 published reports, including a new case report presented here. In 11 cases (65 per cent), the definitive diagnosis was not appreciated until after birth, although a fetal mass was recognized on ultrasonographic study in the antenatal period. Seventy-five per cent of cases were thoracic sequestrations, and 25 per cent were abdominal lesions. Fetal hydrops, found in 35 per cent of cases, was universally associated with stillbirth or neonatal death. Polyhydramnios, pleural effusions, mediastinal shifts, pulmonary hypoplasia, and preterm labor were not uncommon findings. The high incidence of related malformations, well described in the postnatal literature, was remarkably absent in this antepartum series. Although the perinatal outcome for abdominal lung sequestrations is reasonably good, the prognosis in cases of thoracic sequestration remains guarded. Aggressive respiratory support after birth will not salvage those infants with underlying severe pulmonary hypoplasia. Early in utero catheter drainage may provide the best treatment for the pleural effusions associated with thoracic sequestrations, although this therapy remains under investigation.

Indomethacin therapy and chronic hemodialysis during pregnancy. A case report.

Numerous complications can prevent the successful outcome of a pregnancy conceived while a woman is undergoing chronic hemodialysis. Among the difficulties encountered, premature labor and polyhydramnios are common. In such a patient indomethacin was used to treat those problems, with the subsequent delivery of a healthy infant.

Discordant umbilical arteries: ultrasonographic and Doppler analysis.

Discordancy in size between the umbilical arteries was detected during real-time ultrasonographic study of six patients. Cross-sectional and longitudinal views from multiple locations of the three-vessel umbilical cords documented the size discrepancies in a total of 23 serial examinations. The size difference was defined by cursor measurement of the artery diameters. Pulsed- and continuous-wave Doppler evaluation of the dissimilar arteries in each cord demonstrated discordant flow velocity waveforms. The mean difference between small and large artery systolic-diastolic ratios (S/Ds) was significant (P less than .0001). Two of the six patients studied had poor perinatal outcomes. Pathologic confirmation of the artery discordance was provided by gross and microscopic examination of an undrained cord segment from a seventh patient who had dissimilar artery sizes and S/Ds on ultrasonographic inspection. The finding of umbilical artery discordance has not been previously described in the obstetric ultrasound literature, and further investigation is warranted.

Transvaginal fetal echocardiography in early pregnancy: normative data.

Fifty-two transvaginal ultrasonographic examinations were performed between 10.0 and 14.9 weeks' gestation for the purpose of documenting normal fetal cardiac anatomy. All standard cardiac projections could be obtained by gestational week 12, and many were imaged by week 11. The four-chamber view was visualized in 90% of fetuses at 12 weeks' gestation and in 100% of fetuses examined at 13 weeks. The aortic root in short axis projection and the left ventricle in long axis view could be imaged in 70% and 40% of fetuses, respectively, by 12 weeks' gestation. Aortic and pulmonary valves were first visualized at 12 weeks, as were five-chamber, ductus arteriosus, and aortic arch views. The mitral and tricuspid valves were resolved in 60% of fetuses by 11 weeks' gestation. This study of normal cardiac anatomy suggests that there may be significant potential for the diagnosis of many fetal cardiac anomalies during the late first and early second trimesters of pregnancy.

Effect of alpha 1 receptor blockade upon maternal and fetal cardiovascular responses to cocaine.

Previous studies in pregnant ewes have demonstrated that intravenous (IV) cocaine produces increased maternal blood pressure and vasoconstriction of the uterine arteries, resulting in decreased uterine blood flow and decreased fetal oxygen levels. To determine whether these responses to cocaine were mediated by alpha 1-adrenergic receptor stimulation, cocaine was administered to four pregnant ewes before and after phenoxybenzamine hydrochloride, an alpha 1 receptor antagonist. Before phenoxybenzamine infusion, cocaine 2.0 mg/kg produced a 53% increase in maternal mean arterial pressure (MAP), a 50% reduction in total uterine blood flow, and a 191% increase in uterine vascular resistance. Cocaine also increased fetal MAP by 24%, increased the fetal heart rate (FHR) by 51%, and reduced fetal PO2 by 29%. Alpha 1 receptor blockade after phenoxybenzamine 5.0 mg/kg was confirmed by a lack of change in uterine blood flow to IV norepinephrine 30 micrograms before cocaine administration. After phenoxybenzamine, cocaine produced no increase in maternal or fetal MAP. However, total uterine blood flow decreased 44%, uterine vascular resistance increased 59%, FHR increased 36%, and fetal PO2 fell 18%. Because the fetal responses mimicked the maternal responses to cocaine both before and after phenoxybenzamine, phenoxybenzamine apparently crossed the placenta to block fetal alpha 1 receptors as well. Alpha 1-adrenergic receptor stimulation is the major mechanism for the maternal and fetal hypertensive responses to cocaine. Although cocaine produces uterine artery vasoconstriction primarily by alpha 1 adrenergic receptor stimulation, its vasoconstrictive effects may involve other vasoactive neurotransmitters, such as dopamine or serotonin.

Intracranial teratoma: prolonged neonatal survival after prenatal diagnosis.

The prenatal diagnosis of an intracranial teratoma has been generally associated with either fetal death or very brief postnatal infant survival. A case is reported in which such a neonate survived for a month and was discharged home, despite the in utero diagnosis of a massive tumor.