Epoprostenol treatment for idiopathic pulmonary arterial hypertension in pregnancy.

Idiopathic pulmonary arterial hypertension is a rare condition associated with significant maternal mortality. We report the management of a 37-year-old multigravida with severe disease using epoprostenol, a multidisciplinary approach, and a planned delivery. Although the patient survived the pregnancy, her pulmonary function significantly worsened. Epoprostenol, a pulmonary vasodilator, should be considered when indicated during pregnancy. Neither fetal nor neonatal harm was identified.

Leukemia in pregnancy and fetal response to multiagent chemotherapy.

BACKGROUND: Leukemia is rare in pregnancy and treatment with intensive, multiagent chemotherapy produces complete remission in most adults, but might have deleterious effects on fetuses. CASE: A 24-year-old gravida 3 para 2 presented at 24 weeks with pruritus, rash, pancytopenia, and hepatitis. A bone marrow biopsy found acute lymphocytic leukemia. She completed three cycles of intensive multiagent chemotherapy with transient oligohydramnios in each cycle. Although there was decreased fetal growth rate, umbilical artery Doppler scans were normal. She delivered a normal 2150-g male infant at 36 weeks. CONCLUSION: Pregnant women with newly diagnosed leukemia should not delay treatment, but multiagent chemotherapy might have transient effects on fetuses, most notably oligohydramnios. However, if fetal testing is normal, delivery might not be indicated.

Trauma and pregnancy.

Trauma and/or accidental injury complicates 6-7% of all pregnancies. The management protocols for trauma in pregnancy are based largely on case reports and small series. The purposes of this study were to: describe the demographics of pregnant trauma patients at a tertiary care center and a large community hospital; identify variables predictive of fetal outcome including an examination of Kleihauer-Betke and nonstress testing; and recommend an evaluation and management protocol after trauma based on empirical data rather than anecdotal reports. Data from pregnancies complicated by trauma from July 1987 through October 1993 were retrospectively reviewed. Statistical analysis included Chi-square and Kruskall-Wallis testing. There were 476 medical records available for review. Of the trauma cases, 54.6% were motor vehicle accidents, 22.3% were domestic abuse and assaults, 21.8% were associated with falls, and 1.3% were secondary to burns, puncture wounds, or animal bites. Mean maternal age was 24 years, 49.9% were Caucasian, and 43% were primigravid. Mean gestational age at occurrence of trauma was 25.9 weeks and mean gestational age of delivery was 37.9 weeks. Domestic abuse occurred most frequently before 18 weeks, falls between 20-30 weeks' gestation, and motor vehicle accidents occurred with equal frequency throughout gestation. Uterine contractions occurred in 39.8% of patients and as often as every 1 to 5 min in 18% of patients. Preterm labor occurred in 11.4%, preterm delivery in 25%, and abruptions in 1.58% of the trauma population. Fetal heart rate monitoring was abnormal in 3% of cases. Twenty-seven perinatal deaths were noted and in 14 pregnancies the deaths were related to trauma. Eight of these perinatal deaths were associated with motor vehicle accidents, four with domestic violence, and two with falls. The only preventable perinatal deaths were a twin pregnancy transferred with nonreassuring fetal heart tones. Early warning symptoms of vaginal bleeding, uterine contractions, and/or abdominal and/or uterine tenderness were not predictive of either preterm delivery or adverse pregnancy outcome, sensitivity 52%, specificity 48%. Abnormal monitoring and positive Kleihauer-Betke tests were also not predictive of adverse pregnancy outcome. However, there were no adverse outcomes directly related to trauma when monitoring was normal and early warning symptoms were absent (negative predictive value 100%). Two hundred eighty-nine Kleihauer-Betke tests were performed and only one affected management. Repetitive monitoring over several days did not uncover any patients whose heart rate tracings evolved from normal to abnormal monitoring. Given our findings that prolonged monitoring was not helpful in management of pregnant trauma patients, we support the recommendation that initial external fetal monitoring be performed for 4 hr, and, if reassuring, the patient may be sent home with precautions. We also recommend an Rh-immunoglobulin work-up for all Rh-negative pregnant trauma patients, but do not recommend Kleihauer-Betke testing for Rh-positive women. Given the frequency with which trauma affects pregnancy and the difficulty encountered with identifying variables predictive of pregnancy outcome, there may be great benefits of incorporating trauma prevention into routine prenatal care.

Maternal uniparental disomy of chromosome 2 and confined placental mosaicism for trisomy 2 in a fetus with intrauterine growth restriction, hypospadias, and oligohydramnios.

We present a case of maternal uniparental heterodisomy for chromosome 2 (UPD 2) detected after trisomy 2 mosaicism was found on placental biopsy. This case presented prenatally with severe intrauterine growth restriction (IUGR) and oligohydramnios. The diploid newborn had hypospadias and features consistent with oligohydramnios sequence. He died shortly after birth of severe pulmonary hypoplasia. The term placenta had high levels of trisomy 2 in both the trophoblast and the stroma. A comparison of this case with others reported in the literature suggests that the IUGR and oligohydramnios are likely related to placental insufficiency due to the high levels of trisomy 2 present in the trophoblast of the term placenta and the presence of UPD 2 in the diploid placental line.

Mandibular arteriovenous malformation in pregnancy.

Benign vascular lesions, such as hemangiomas of childhood, arteriovenous malformations (AVMs) and venous malformations, are uncommon and have been found in a variety of organs throughout the body. Vascular lesions of bone are found most often in the vertebral column. Vascular tumors of the mandible are rare. We report a case of a mandibular AVM found in a pregnant woman at 35 weeks' gestation. A pregnant woman presented at 35 weeks' gestation with right-sided jaw pain and was found to have a pulsatile mass on the internal surface of the right mandible in the area of the premolars. Profuse bleeding of the lesion required emergent selective arterial embolization of a vascular tumor of the mandible, which resulted in successful occlusion of the vessels. Uncomplicated vaginal delivery followed induction of labor. Excision and reconstructive surgery occurred 1 week postembolization. Pathology confirmed an AVM. Mandibular AVMs are rare vascular tumors that, when correctly diagnosed, can be successfully managed during pregnancy. Whether or not vascular or hormonal changes associated with pregnancy can exacerbate a preexisting AVM is debatable.

Fatal lupus pleuritis presenting in pregnancy. A case report.

BACKGROUND: Lupus, one of the most common autoimmune diseases in pregnancy, may involve multiple organ systems with varying severity. The diagnosis and treatment of the disease may be complicated by the physiologic changes of pregnancy. CASE: A 22-year-old woman presented at 29 weeks' gestation with a 4-week history of dyspnea and mild hypertension. She was found to have large bilateral pleural effusions. Her antinuclear antibody level was 1:640, with a speckled pattern, and her complement levels were low. Her urine had 2+ hemoglobin and 2+ protein with hyaline casts. Over the next three days, respiratory compromise increased despite high-dose steroids. A cesarean delivery was performed for fetal compromise. The infant did well after moderate respiratory distress. The mother developed worsening respiratory distress with adult respiratory distress syndrome, and she could not be weaned from the ventilator. Bilateral chest tubes were placed to control her effusions. Urine output remained poor despite pressors and diuretics. Staphylococcal sepsis occurred on postoperative day 5 and precluded our use of other antiinflammatory agents. Over the next 14 days the mother developed seizures, hypotension and eventual respiratory collapse. Autopsy was notable for lupus nephritis and serositis. CONCLUSION: The diagnosis and management of systemic lupus in pregnancy may be extremely difficult. Serositis and nephritis may lead to maternal compromise despite early diagnosis and treatment.

Fetal ovarian torsion appearing as a solid abdominal mass.

Fetal and neonatal ovarian cysts can be small asymptomatic lucencies or large symptomatic echocomplex masses that can cause intestinal obstruction and peritonitis. Solid masses are the most rare form of ovarian cysts. A fetus, anatomically unremarkable at 16 weeks' development, was found at 35 weeks to have a 5 x 5 cm solid mass. After delivery a laparotomy was performed, and a torsed ovary measuring 8 x 7 cm was removed. The infant did well after the procedure. Over 400 cases of perinatal ovarian cysts have been reported in the literature. Only 1% of these are purely echodense. When a fetal abdominal mass is present, separate from bowels and kidneys, a torsed ovary should be considered in the differential diagnosis. Management of the pregnancy and infant may be guided by the fetus or neonate's symptoms.

Transcranial Doppler findings of cerebral vasospasm in preeclampsia.

The objective of this study was to evaluate the effect of preeclampsia and its severity on maternal mean middle cerebral artery blood flow velocity (mean MCA-CBFV) using transcranial doppler sonography (TCD), as well as the effect of magnesium on mean MCA-CBFV in preeclampsia. This study used a prospective, comparative design. TCD was used to examine maternal mean MCA-CBFV in both healthy subjects (controls) and preeclamptic subjects (cases). The two groups were similar in age, gestational age, and parity. Healthy subjects were categorized into three groups: Group I, 6-14 weeks, n = 10; Group II, 24-40 weeks, n = 27; Group III, postpartum n = 15, 12-36 h. Serial TCD examinations of the middle cerebral artery were completed in 21 preeclamptic subjects at four different points in time: Time I is an initial measurement before delivery; Time 2 is also before delivery but after magnesium had been administered. Time 3 is postpartum while on magnesium (12-24 h), Time 4 is postpartum off magnesium, (24-48 h). Preeclamptic subjects had significantly increased mean MCA-CBFV when compared to healthy subjects: antepartum (mean 78.2 vs. 55.1 cm/sec, P < 0.0005); postpartum (mean 101.3 vs. 69.8 cm/sec, P < 0.0001). Severe preeclamptics had significantly higher mean MCA-CBFV than mild preeclamptics at each point in time: Time 1: P < 0.016; Time 2: P < 0.040; Time 3: P < 0.002; and Time 4: P < 0.028. These data support the theory that cerebral vasospasm of the smaller diameter vessels is a major component of preeclampsia.

An association between fetal parvovirus B19 infection and fetal anomalies: a report of two cases.

The association between fetal parvovirus B19 infection and hydrops was first reported in 1984. The virus has a predilection for the erythroid cell line, which in the fetus may produce anemia. Recent cases of parvovirus infection in other fetal cell lines have raised concern that the infection may induce fetal anomalies in rare cases. We report two pregnancies complicated by parvovirus B19 infection. In each instance the patient had normal second trimester ultrasounds but subsequently developed fetal abnormalities--disruptions of normal structure. One infant has myocardial infarction, splenic calcifications, and mild hydrocephalus. The other had moderate hydrocephalus with central nervous system scarring. There are two possible mechanisms in which parvovirus may induce fetal anomalies. Both direct infection of fetal organs and vascular inflammation have been documented in association with B19 parvovirus. Although fetal abnormalities associated with parvovirus are rare, continued study of this organism may indicate a greater pathologic potential than is now thought.

Pregnancy complicated by Guillain-Barré syndrome.

We report a case of a pregnant patient with Guillain-Barré syndrome. Her symptoms, including weakness, vague numbness, urinary incontinence, and unsteady gait, were initially mild and mimicked many of the changes seen in pregnancy. Her progressive weakness was not preceded by any known illness. Evaluation of symptoms revealed an elevated cytomegalovirus IgM titer. When symptoms became severe, electromyography was done, showing a severe demyelinating motor-sensory neuropathy. After plasma exchange and vaginal delivery, symptoms resolved. Neither the patient nor her infant have had any long-term sequelae. Guillain-Barré syndrome may be difficult to diagnose in pregnancy but may be effectively treated with plasmapheresis.

Cesarean delivery: a reconsideration of terminology.

The terminology we employ influences our perceptions. Thus, we believe in reviewing terminology regularly. The term "cesarean section" should be abandoned. A medical procedure is rarely named after a historic figure, and almost never after an ancient Roman law. Cesarean section has recently been amended to cesarean birth; however, the term is still inadequate. The word "hysterotomy" more appropriately describes the procedure we regularly perform. The adjectives used to describe cesarean birth should also be reevaluated. If we are to keep the term "classical" for the original fundal vertical uterine incision, then the other designations of hysterotomy should retain the same architectural context. Contemporary hysterotomy should be used in reference to a low transverse cesarean. Neoclassical hysterotomy might be used to describe the low vertical procedure. Transitional hysterotomy may be an appropriate term for the "hockey stick" or "J" incision. Cesarean delivery best describes a postmortem cesarean. Vaginal birth after cesarean (VBAC) is inappropriate because a vaginal birth after a cesarean section would not occur if cesarean section refers to a perimortem procedure. Vaginal birth after hysterotomy should be substituted for VBAC. Such revision of our terminology will keep obstetrics at the forefront of medical science and bring us most efficiently into the 21st century.

Ultrasonographic measurement of fetal nuchal skin to screen for chromosomal abnormalities.

OBJECTIVE: The purpose of this prospective investigation was to determine the utility of ultrasonographic measurement of the fetal nuchal skin in screening for chromosomal abnormalities. STUDY DESIGN: In 1510 patients undergoing genetic amniocentesis at 14 to 21 weeks' gestation, the fetal nuchal skin fold was measured. A measurement of > or = 6 mm was considered abnormal. RESULTS: In fetuses with normal karyotype the nuchal skin increased with advancing gestational age (r = 0.45, p < 0.001). Only 27 fetuses with normal karyotype (2.0%) had an abnormal nuchal skin thickness. Fifty-seven fetuses had abnormal karyotype. Eight of 14 fetuses with Down syndrome had abnormal ultrasonographic findings; four had increased nuchal skin as the only ultrasonographic abnormality. Increased nuchal skin alone, in the absence of other ultrasonographic dysmorphologic features, did not help to identify fetuses with other abnormal karyotypes. CONCLUSION: Nuchal skin thickness is a useful noninvasive measurement to screen for Down syndrome but does not help to identify fetuses with other chromosomal abnormalities.

Massive ovarian tumor complicating pregnancy. A case report.

Adnexal masses complicate approximately 1/2,000 pregnancies. Teratomas are the most common ovarian neoplasms found in pregnancy, representing up to 36% of adnexal masses. However, almost all these dermoids are less than 10 cm in diameter. We present a case of a massive benign cystic teratoma complicating pregnancy. The patient initially presented with what appeared to be ascites. Imaging evaluation included ultrasound, magnetic resonance and angiography. These studies demonstrated a large teratoma. The tumor was resected without complications. Important aspects of perioperative care are discussed.

The cisterna magna in second-trimester fetuses with abnormal karyotypes.

Six hundred thirty-eight gravidas at 14-21 weeks' gestation, who were at increased risk for an abnormal fetal karyotype, had sonographic measurement of the fetal cisterna magna at the level of the posterior fossa before genetic amniocentesis. The size of the fetal cisterna magna increased significantly with advancing gestational age in the normal fetus (P less than .001). Twenty-eight fetuses had abnormal karyotypes: 12 with trisomy, eight with translocation or structural rearrangements, seven with sex chromosome abnormalities, and one with triploidy. In each of these fetuses with abnormal karyotypes, the cisterna magna measurement was normal. We conclude that measurement of the fetal cisterna magna at 14-21 weeks' gestation is not useful as a screening test for abnormal fetal karyotype.