Persistent cloaca: a 10-year review of prenatal diagnosis.

OBJECTIVE: The purpose of this study was to review antenatal sonographic findings in children born with persistent cloaca. METHODS: Infants (n =145) with persistent cloaca followed at a center for colorectal congenital anomalies were identified by a retrospective chart review. Fifty female infants with a persistent cloaca met inclusion criteria and had prenatal records and imaging studies available for review. Sonographic data were retrospectively abstracted from charts. RESULTS: Anomalies were detected in 27 of 50 cases (54%). A correct antenatal diagnosis of persistent cloaca occurred in 3 of 50 (6%). Common findings misinterpreted on antenatal sonography include urinary tract anomalies, dilated bowel, and a cystic pelvic mass (representing hydrocolpos). CONCLUSIONS: Antenatal diagnosis of persistent cloaca is difficult. Persistent cloaca should be considered in the differential diagnosis if urinary tract malformations, dilated bowel loops, or cystic pelvic masses are visualized by prenatal diagnosis.

Prenatal MRI findings of fetuses with congenital high airway obstruction sequence.

OBJECTIVE: To define the MRI findings of congenital high airway obstruction sequence (CHAOS) in a series of fetuses. MATERIALS AND METHODS: Prenatal fetal MR images were reviewed in seven fetuses with CHAOS at 21 to 27 weeks of gestation. The MRI findings were reviewed. The MRI parameters evaluated included the appearance of the lungs and diaphragm, presence or absence of hydrops, amount of amniotic fluid, airway appearance, predicted level of airway obstruction, and any additional findings or suspected genetic syndromes. RESULTS: All the fetuses viewed (7 of 7) demonstrated the following MRI findings: dilated airway below the level of obstruction, increased lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, massive ascites, centrally positioned and compressed heart, as well as placentomegaly. Other frequent findings were anasarca (6 of 7) and polyhydramnios (3 of 7). MRI identified the level of obstruction as laryngeal in five cases and tracheal in two cases. In four of the patients, surgery or autopsy confirmed the MRI predicted level of obstruction. Associated abnormalities were found in 4 of 7 (genetic syndromes in 2). Postnatal radiography (n = 3) showed markedly hyperinflated lungs with inverted or flattened hemidiaphragms, strandy perihilar opacities, pneumothoraces and tracheotomy. Two fetuses were terminated and one fetus demised in utero. Four fetuses were delivered via ex utero intrapartum treatment procedure. CONCLUSION: MRI shows a consistent pattern of abnormalities in fetuses with CHAOS, accurately identifies the level of airway obstruction, and helps differentiate from other lung abnormalities such as bilateral congenital pulmonary airway malformation by demonstrating an abnormally dilated airway distal to the obstruction.

High-risk fetal congenital pulmonary airway malformations have a variable response to steroids.

BACKGROUND/PURPOSE: Anecdotal reports suggest that maternal steroids may arrest the growth of congenital pulmonary airway malformations (CPAMs), preventing or reversing hydrops. We reviewed our experience with CPAMs to determine the fetal response to steroid therapy. METHODS: This study is a retrospective review of all fetal CPAMs from 2004 to 2008. Fetuses with high-risk CPAMs that received at least one course of steroids were identified. Fetal magnetic resonance imaging and ultrasound data were used to classify the CPAMs, identify hydrops fetalis and follow the fetuses poststeroid dosing. RESULTS: Forty-four fetuses with CPAM were identified. Fifteen patients were found to have received at least one course of steroids. Thirteen were hydropic and 2 were nonhydropic. Seven of the 13 hydropic fetuses (54%) showed an initial response to steroid administration, whereas the 2 nonhydropic high-risk fetuses progressed to birth without developing hydrops. Seven of the 15 patients, however, resulted in fetal demise or early postnatal death, giving a survival rate of 53%. CONCLUSIONS: High-risk CPAMs have a variable response to steroids. This variable response demonstrates the need for a placebo-controlled randomized study to more accurately determine the effect of steroids on hydrops and CPAM growth rates. Repeated steroid courses may not be helpful, and progression in CPAM volume to head circumference ratio (CVR) or hydrops should prompt open fetal surgery to prevent irreversible fetal insult.

Intrafetal radiofrequency ablation for twin reversed arterial perfusion (TRAP): a single-center experience.

OBJECTIVE: The objective of the study was to review perinatal outcomes in pregnancies treated with intrafetal radiofrequency ablation (RFA) for twin reversed arterial perfusion (TRAP) sequence. STUDY DESIGN: Perinatal outcome data from a quaternary care referral center were abstracted from a chart review of pregnancies with TRAP sequence treated in the midtrimester with umbilical cord RFA of the perfused twin. RESULTS: Twenty-one pregnancies with TRAP sequence were evaluated. Two women had a pump twin demise prior to therapy, 1 with trisomy 21 declined treatment. Four of 20 were treated successfully with RFA but remain undelivered, and 1 was treated with fetoscopic cord coagulation. Twelve of 13 pump twins treated with RFA (94%) survived to 30 days of life. Mean preoperative cardiac combined cardiac output was 588 mL/kg and pump/twin ratio was 0.7 (range 0.4 to 1.1). The effect of RFA on postoperative cardiac output was variable (6-85%). The average gestational age at birth was 37 weeks (range 26-39 weeks). CONCLUSION: Primary therapy with RFA is a successful modality for pregnancies complicated by TRAP sequence.

Mirror syndrome: a novel approach to therapy with fetal peritoneal-amniotic shunt.

BACKGROUND: Mirror syndrome is a rare entity characterized by maternal disease mimicking fetal hydrops. In mirror syndrome, there is maternal hypertension, edema, and often proteinuria in association with fetal hydrops. The causal link between mirror syndrome and hydrops fetalis remains elusive. CASE: This is a case report of a pregnant woman who developed mirror syndrome associated with fetal hydrops. A fetal pelvic mass resulted in bladder outlet obstruction, subsequent bladder rupture, and massive urinary ascites. The resultant massive ascites caused thoracic and cardiac compression and subsequent hydrops fetalis. Placement of a peritoneal-amniotic shunt resolved the fetal hydrops and maternal mirror syndrome. CONCLUSION: In utero treatment of hydropic fetus can result in the cure of maternal mirror syndrome.

Second-trimester asynchronous multifetal delivery results in poor perinatal outcome.

OBJECTIVE: The purpose of this study is to estimate the maternal and fetal morbidities associated with asynchronous delivery. METHODS: A review of maternal and fetal medical records was performed at 2 tertiary care centers over 12 years. Charts were identified by the International Classification of Diseases, 9th Revision, Clinical Modification codes for twin and triplet gestations. Asynchronous delivery was defined as an active attempt (tocolysis and/or emergent cerclage placement) to increase latency between delivery of the first fetus and subsequent fetuses. RESULTS: Fourteen cases of asynchronous delivery were identified out of 96922 deliveries including 1352 pregnancies complicated by multifetal gestation. The occurrence rate of asynchronous delivery was 0.14 per 1000 births. The etiology of preterm birth of the first fetus in 12 (86%) of 14 cases was second-trimester rupture of membranes. The mean gestational age for delivery of the first fetus was 21.+/- 2.0 weeks. All women received tocolysis and intravenous antibiotics. Two of 3 attempts at cerclage placement were successful. Median latency obtained was 2 days (range less than 1-70 days). There was 1 survival of a first born. There were 19 retained fetuses, 2 died in utero, 10 died between birth and day 57 of life, and 7 survived (37%; 95% confidence interval 16%, 62%) until hospital discharge. Six of 7 survivors had major sequelae from prematurity. One of 19 fetuses was discharged without major sequelae (5%; 95% confidence interval 0%, 25%). Maternal morbidity included 2 placental abruptions and 8 cases of infectious morbidity including 1 case of septic shock. CONCLUSION: Attempts at asynchronous deliveries are uncommon and are associated with a high rate of perinatal death. Most fetal survivors have significant damage from preterm birth.

Subsequent pregnancy outcome in women with a history of HELLP syndrome at < or = 28 weeks of gestation.

OBJECTIVE: The purpose of this study was to describe subsequent pregnancy outcome in women with a history of hemolysis, elevated liver enzymes, and low platelet count syndrome for which delivery occurred at < or = 28 weeks of gestation during the index pregnancy. STUDY DESIGN: A descriptive report of women with previous hemolysis, elevated liver enzymes, and low platelet count syndrome who were delivered between August 1984 and July 1998 at the E.H. Crump Women's Hospital (Memphis, Tenn) and between March 1994 and July 1998 at the Central Baptist Hospital (Lexington, Ky). To have adequate time to study subsequent pregnancy outcome, only patients who were delivered >2 years before the analysis were included. Medical records of the index pregnancy and subsequent outcomes were available for review. RESULTS: Adequate follow-up data were available in 69 patients; the median duration of follow-up was 5 years (range: 2-14 years). There were 76 subsequent pregnancies among 48 women, of which 62 pregnancies (82%) progressed beyond 20 weeks of gestation. Preeclampsia developed in 34 of 62 subsequent pregnancies (55%). Recurrent hemolysis, elevated liver enzymes, and low platelet count syndrome developed in 4 of these pregnancies (6%), and abruptio placentae developed in 3 of these pregnancies (5%). There were no cases of eclampsia in our population. Delivery before 37 weeks of gestation occurred in 33 of the cases (53%), and 17 of the newborn infants (27%) were small for gestational age (<10th percentile). The perinatal mortality rate was 11%. CONCLUSION: Patients with a history of hemolysis, elevated liver enzymes, and low platelet count syndrome at < or = 28 weeks of gestation during the index pregnancy are at increased risk for obstetric complications in subsequent pregnancies. Overall, however, the rate of recurrent hemolysis, elevated liver enzymes, and low platelet count syndrome is only 6%.

Magnesium sulfate in women with mild preeclampsia: a randomized controlled trial.

OBJECTIVE: To determine whether magnesium sulfate prevents disease progression in women with mild preeclampsia. METHODS: A total of 222 women with mild preeclampsia were randomized to receive intravenous magnesium sulfate (n = 109) or matched placebo (n = 113). Mild preeclampsia was defined as blood pressure of at least 140/90 mm Hg taken on two occasions in the presence of new-onset proteinuria. Patients with chronic hypertension or severe preeclampsia were excluded. Patients were considered to have disease progression if they developed signs or symptoms of severe preeclampsia, eclampsia, or laboratory abnormalities of full or partial HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome. RESULTS: The groups were similar with respect to maternal age, ethnicity, gestational age, parity, and maternal weight at enrollment. Fourteen women (12.8%) in the magnesium group and 19 (16.8%) in the placebo group developed severe preeclampsia after randomization (relative risk = 0.8, 95% confidence interval 0.4, 1.5, P =.41). None in either group developed eclampsia or thrombocytopenia. Women assigned magnesium had similar rates of cesarean delivery (30% versus 25%), chorioamnionitis (3% versus 2.7%), endometritis (5.3% versus 4.3%), and postpartum hemorrhage (1% versus 0.9%), compared to those assigned placebo. Neonates born to women assigned magnesium had similar mean Apgar scores at 1 and 5 minutes as those born to women assigned placebo (7.7 +/- 1.5 versus 7.8 +/- 1.6 and 8.7 +/- 0.7 versus 8.8 +/- 0.6, respectively). CONCLUSION: Magnesium sulfate does not have a major impact on disease progression in women with mild preeclampsia. Magnesium use does not seem to increase rates of cesarean delivery, infectious morbidity, obstetric hemorrhage, or neonatal depression.

Gentamicin and clindamycin therapy in postpartum endometritis: the efficacy of daily dosing versus dosing every 8 hours.

OBJECTIVE: The objective of the study was to evaluate the efficacy of gentamicin and clindamycin given once daily versus the more common 8-hour dosing regimen for the treatment of postpartum endometritis. STUDY DESIGN: In a prospective, placebo-controlled, double-blinded study, patients who had postpartum endometritis diagnosed were randomly selected to receive 1.5 mg/kg gentamicin and 900 mg clindamycin phosphate administered every 8 hours versus gentamicin 5 mg/kg and clindamycin phosphate 2700 mg administered as a single-daily dose. The single-dose group received an infusion of gentamicin and clindamycin, followed by an administration of intravenous placebo 8 and 16 hours later to maintain blinding. Treatment success was defined as absence of fever 72 hours after initiation of antibiotic therapy. RESULTS: One hundred ten patients were enrolled. The daily-dose group (n = 55) and the thrice-daily dose group (n = 55) were similar with respect to age, gravidity, parity, gestational age, and maternal weight. Clinical characteristics (including maximum temperature, presence of predelivery chorioamnionitis, white blood cell count, and mode of delivery) were also similar. There was no difference in the mean time from initiation of therapy until becoming afebrile in the daily-dose group (27.4 +/- 24.9 hours) compared with the thrice-daily dose group (32.9 +/- 26.3 hours). Forty-five of 56 (82%) patients in the daily-dose group and 38 of 55 (69%) patients in the thrice-daily dose group had treatment success (P =.12). CONCLUSION: Once-daily dosing with gentamicin and clindamycin in women with postpartum endometritis has a similar success rate as the standard every 8-hour dosing schedule.

Concentrations of estrogens in patients with preeclampsia.

The role of estrogens in the pathophysiology of preeclampsia remains to be determined. The aim of our study was to compare serum concentrations of 17 beta-estradiol and estriol in women with preeclampsia to normotensive pregnant controls. Serum concentrations of estrogens were measured in women with mild (n = 24) and severe (n = 24) preeclampsia as well as is normotensive pregnant controls (n = 24). Patients were matched for gestational age. Pregnancies complicated by early onset severe preeclampsia are associated with increased rates of maternal and fetal morbidity. Subsequently, we created further subgroups before and after 34 weeks of gestation (34 + 0). Serum estrogen concentrations were determined by standard ELISA technique. Compared to normotensive controls, the differences between the overall median serum concentrations of 17 beta-estradiol in women with mild (3811 v. 3730 pg/ml, P = 0.9) and severe (3811 v. 3630 pg/ml, P = 0.1) preeclampsia were statistically not significant. The differences between the overall median serum concentrations of estroil in controls and in patients with mild (121 v. 76 ng/ml, P = 0.6) and severe (121 v. 79 ng/ml, P = 0.4) preeclampsia were similar. The differences between the median concentrations of 17 beta-estradiol in patient with early onset severe preeclampsia compared to patients with mild preeclampsia (3061 v. 3715 pg/ml, P = 0.004) and controls (3061 v. 3807 pg/ml, P = 0.006) were statistically significant. In addition, the differences between the median concentrations of estriol in women with early onset severe preeclampsia compared to controls were statistically significant (20 v. 92 ng/ml, P = 0.02). The differences between the median concentrations of estrogens in those with late onset severe preeclampsia compared to women with mild preeclampsia were not significant. We found significantly lower concentrations of estrogens in women with early onset severe preeclampsia.

Heat shock protein 70 is not increased in women with severe preeclampsia.

OBJECTIVE: The purpose of this study is to determine if heat shock protein 70 (Hsp 70), a marker of cellular stress, is elevated in pregnancies complicated by severe preeclampsia. METHODS: Maternal blood was collected from women with severe preeclampsia (n=47) matched for delivery gestational age to normotensive pregnant controls (n=51). Hsp 70 concentrations were measured by standard ELISA techniques. Data were analyzed with the Student's t-test and chi-square test. MAIN OUTCOME MEASURES: The primary outcome measured was Hsp 70 concentrations. Our hypothesis prior to data collection was that HSP 70 would be increased in women with severe preeclampsia. RESULTS: Compared with normotensive women, those with severe preeclampsia had similar maternal age, parity, delivery gestational age, maternal weight, and ethnicity. There was no difference in mean concentrations of Hsp 70 between women with severe preeclampsia and controls (35.4 +/- 96.7 vs. 30.1 +/- 11.5, p=0.80). Similar numbers of women with severe preeclampsia (n=28) and controls (n=30) had Hsp 70 concentrations below the 0.02 ng/dL level of detection (chi-square value=0.024, p=0.88). CONCLUSION: Hsp 70 concentrations are not elevated in women with severe preeclampsia.

Late postpartum eclampsia: a preventable disease?

OBJECTIVE: The purpose of this study was to determine whether there is a shift in the timing of eclampsia in relation to delivery and whether traditional symptoms precede impending postpartum eclampsia. STUDY DESIGN: A multicenter analysis of data from patients with eclampsia from March 1996 through February 2001 at the University of Cincinnati, the University of Tennessee, Memphis, and Central Baptist Hospital, Lexington. Data were collected regarding the relationship of the patient's first seizure to delivery, prodromal symptoms, neuroimaging studies, use of magnesium sulfate, antihypertensive therapy, and follow-up medical care. The analysis focused on women who had late postpartum eclampsia. RESULTS: During the study period, 89 patients were diagnosed with eclampsia. Twenty-nine women (33%) had postpartum eclampsia, of whom 23 women (79%) had late onset (>48 hours). Interestingly, only 5 of these 23 women (22%) had been previously diagnosed with preeclampsia. Twenty-one patients (91%) with late postpartum eclampsia had at least 1 prodromal symptom, and 12 patients (52%) had >1 symptom that heralded the seizure: 20 women (87%) had headache; 10 women (44%) had visual changes; 5 women (22%) had nausea or vomiting; and 2 women (9%) experienced epigastric pain. Only 7 of these 21 women (33%) sought care for their symptoms, of whom 6 women (86%) had clinical evidence of preeclampsia that was not considered by the treating physician. Among all patients with eclampsia, there were 7 cases of aspiration pneumonia, 3 cases of pulmonary edema, 3 cases of pleural effusion, 2 cases of disseminated intravascular coagulation, and no cases of maternal death. CONCLUSION: Current obstetric treatment in the United States has resulted in a shift of eclampsia toward the postpartum period, with most cases being seen as late post partum. To reduce the rate of late postpartum eclampsia, efforts should be directed to the education of the health care providers and patients regarding the importance of prompt reporting and evaluation of symptoms of preeclampsia during the postpartum period.