Use of an Amnioport to Maintain Amniotic Fluid Volume in Fetuses with Oligohydramnios Secondary to Lower Urinary Tract Obstruction or Fetal Renal Anomalies.

OBJECTIVE: We describe a technique to maintain amniotic fluid in fetuses with severe oligo-/anhydramnios secondary to lower urinary tract obstruction or fetal renal disease when urine production is inadequate to maintain a normal amniotic fluid volume (AFV). METHODS: An amnioport was inserted into the amniotic space. The catheter was secured to prevent dislodgment and tunneled to a subcutaneous reservoir. The reservoir was accessed as necessary, infusing normal saline to maintain AFV. Pregnancy continued until term or indicated delivery. RESULTS: Since 2010, 15 patients in this category were considered for an amnioport. Six chose comfort care and one elected percutaneous amnioinfusions. Nine amnioport procedures were performed in eight patients. There were no fetal deaths. All eight had successful restoration and maintenance of amniotic fluid. Delivery ranged from 9 to 96 days after placement (mean 63.7 days). One died due to unrecognized laryngeal web and another one died of pulmonary hypoplasia after preterm premature rupture of membranes. None of the remaining six had pulmonary hypoplasia. Three remain alive. DISCUSSION: Severe oligo-/anhydramnios in the second trimester secondary to fetal anomalies is almost uniformly lethal due to pulmonary hypoplasia without restoration of amniotic fluid. The amnioport procedure may allow pulmonary survival but commits families to postnatal care decisions regarding pulmonary and renal complications.

Intrauterine fetal demise after prenatal diagnosis of congenital heart disease: assessment of risk.

OBJECTIVES: Elective deliveries in fetal congenital heart disease (CHD) attempt to balance fetal and neonatal risk with the goal of optimizing overall outcome. However, the magnitude of the risk for intrauterine fetal demise (IUFD) is unclear. This study aimed to (1) determine the rate of IUFD and (2) identify fetal risk factors associated with IUFD. METHODS: Retrospective review of pregnancies complicated by CHD between 1998 and 2010. Data were collected regarding pregnancy outcome, extracardiac anomalies (ECA), genetic and cardiac diagnoses, severity of valve regurgitation, gestational age at birth and birth weight. Fisher's exact test and odds ratios were used to compare outcomes between groups. RESULTS: A total of 501 pregnancies analyzed resulted in 445 live births, 22 IUFD, 16 terminations and 18 unknown outcomes. Amongst IUFD, 27% had a genetic diagnosis, 50% had an ECA and 27% had severe valve regurgitation. IUFD odds increased threefold with ECA and sevenfold with severe valve regurgitation. IUFD occurred in 1.2% without risk factors. CONCLUSIONS: IUFD in fetuses with CHD is associated with ECA, genetic syndromes and severe valve regurgitation. In absence of these fetal characteristics, the occurrence of IUFD is low, although it remains higher than in fetuses without CHD.

Fetal MRI of Torsed Bronchopulmonary Sequestration with Tension Hydrothorax and Hydrops in a Twin Gestation.

While bronchopulmonary sequestration typically has a benign course, this congenital lung malformation has a high mortality rate when associated with untreated in utero tension hydrothorax and hydrops. Hydrops related to bronchopulmonary sequestration is believed to result from torsion of the mass with compromise of the associated blood supply. The impaired venous return of the mass then leads to tension hydrothorax with compression of the heart and mediastinal vessels, impairing global venous return. To our knowledge, this scenario has only been described prenatally by ultrasound. We present the imaging findings of a dichorionic, diamniotic twin gestation with one fetus developing tension hydrothorax and hydrops from presumed intermittent torsion of a bronchopulmonary sequestration. This diagnosis was only able to be confirmed by MRI prior to the use of ultrasound-guided interstitial laser photocoagulation for the treatment of this anomaly.

Giant chorioangiomas: perinatal outcomes and techniques in fetoscopic devascularization.

OBJECTIVE: To evaluate the intrauterine management and perinatal outcome of pregnancies complicated by giant placental chorioangioma (>4 cm) and elaborate on various devascularization techniques. MATERIALS AND METHODS: Retrospective review of 10 cases of giant placental chorioangioma evaluated between January 2005 and August 2012. Maternal demographics, prenatal imaging, response to fetoscopic treatment, obstetrical complications, and perinatal outcomes were evaluated. RESULTS: Overall survival was 80%. Seven (70%) cases were associated with obstetrical complications, including polyhydramnios (n = 7), non-immune hydrops (n = 3), and high cardiac output state (n = 5). Five patients underwent fetoscopic devascularization at a mean gestational age of 24.03 weeks with 80% survival. The tumors were devascularized by bipolar coagulation (n = 1), combination of bipolar and diode laser (n = 2), bipolar and radiofrequency ablation (n = 1), and surgical clip application (n = 1). Postoperatively, all survivors had resolution of hydrops and high cardiac output states with survival beyond 60 days of life. DISCUSSION: Fetoscopic devascularization is indicated for high cardiac output states or non-immune hydrops and may require multiple techniques including bipolar coagulation, clip application, and/or laser to interrupt arterial inflow and devascularize the mass.

Fetal cervical teratoma: What is the role of fetal MRI in predicting pulmonary hypoplasia?

OBJECTIVE: To determine whether total fetal lung volumes estimated by MRI could predict lethal pulmonary hypoplasia in a cohort of fetuses with cervical teratomas. METHODS: We performed a retrospective cohort study of fetal cervical teratomas from January 1, 2005, through April 1, 2012. The primary outcome was the ability of total lung volumes measured by MRI to predict neonatal mortality specifically due to pulmonary hypoplasia. Measured lung volumes were compared to previously reported normal values. The percent of observed-to-expected lung volume and the percent predicted lung volume were calculated. The positive and negative predictive values were calculated for each variable. RESULTS: Fetal MRI-derived total lung volumes 1 standard deviation below the median for gestational age had a positive predictive value of 100% in predicting lethal pulmonary hypoplasia. Conversely, total lung volumes above this level were uniformly associated with pulmonary survival (100% negative predictive value). Additionally, percent predicted lung volume ≤75.7 and observed-to-expected lung volume ≤68.3 were associated with lethal pulmonary hypoplasia. CONCLUSION: In this small cohort, MRI-estimated lung volumes were helpful in predicting the presence of pulmonary hypoplasia complicating fetal cervical teratoma.

Late gestation fetal magnetic resonance imaging-derived total lung volume predicts postnatal survival and need for extracorporeal membrane oxygenation support in isolated congenital diaphragmatic hernia.

PURPOSE: Magnetic resonance imaging (MRI) has been used as an imaging modality to assess pulmonary hypoplasia in congenital diaphragmatic hernias (CDHs). The objective of this study was to determine if there is a correlation between late gestational fetal MRI-derived total lung volumes (TLVs) and CDH outcomes. METHODS: From 2006 to 2009, 44 patients met criteria of an isolated CDH with a late gestational MRI evaluation. The prenatal TLV (in milliliters) was obtained between 32 and 34 weeks gestation. The measured study outcomes included survival, need for extracorporeal membrane oxygenation (ECMO), and length of stay. RESULTS: There were 39 left and 5 right CDH patients. The average TLV was significantly lower for nonsurvivors (P = .01), and there was a significant association between lower TLV and the need for ECMO (P = .0001). When stratified by TLV, patients with a TLV of greater than 40 mL had a 90% survival vs 35% survival for a TLV of less than 20 mL. Furthermore, patients with a TLV greater than 40 mL had a lower rate of ECMO use (10%) than patients with a TLV of less than 20 mL (86%). Shorter length of stay was found to correlate with increasing TLV (P = .022). CONCLUSION: Late gestation fetal MRI-derived TLV significantly correlates with postnatal survival and need for ECMO. Fetal MRI may be useful for the evaluation of patients who present late in gestation with a CDH.

Improved recipient survival with maternal nifedipine in twin-twin transfusion syndrome complicated by TTTS cardiomyopathy undergoing selective fetoscopic laser photocoagulation.

OBJECTIVE: The purpose of this study was to evaluate the effect of maternal nifedipine on fetal survival when started 24-48 hours before selective fetoscopic laser photocoagulation (SFLP). STUDY DESIGN: We conducted a case control study of consecutive cases of twin-twin transfusion syndrome (TTTS) in which TTTS cardiomyopathy was treated with maternal nifedipine 24-48 hours before SFLP, compared with gestational age and stage-matched control cases. The primary outcome was recipient and donor survival. RESULTS: One hundred forty-one cases of TTTS were treated with nifedipine, and 152 gestational age- and stage-matched control cases were analyzed. There was a significant increase in overall fetal survival in nifedipine-treated cases compared with control cases (237/284 [83%] vs 232/308 [75%]; P = .015). There is an increase in survival of recipients who were treated with nifedipine in stage IIIA (100% vs 81%; P = .021) and IIIB (93% vs 71%; P = .014); however, there was no difference in donor survival. CONCLUSION: Maternal nifedipine is associated with improved recipient survival in TTTS that undergoes SFLP. This is the first study to suggest a benefit of adjunctive maternal medical therapy in patients with TTTS who undergo SFLP.

Severe micrognathia: indications for EXIT-to-Airway.

The ex utero intrapartum treatment (EXIT) procedure has become an important management option in cases of fetal airway obstruction. Select cases of severe micrognathia may be candidates for EXIT-to-Airway due to high-risk of airway obstruction at birth. Here we present three successful EXIT-to-Airway procedures for the management of congenital micrognathia in its most severe manifestations. CASE 1: A 23-year-old G3P1011 with a pregnancy complicated by severe micorgnathia, jaw index <5th percentile, as well as polyhydramnios. At 36 weeks EXIT-to-Airway was performed utilizing a bronchoscopically positioned laryngeal mask airway (LMA) during 23 min of uteroplacental support followed by tracheostomy. CASE 2: A 26-year-old G4P0120 with a pregnancy complicated by severe micrognathia, jaw index <5th percentile, and an obstructed oropharynx associated with polyhydramnios. At 37 weeks EXIT-to-Airway was performed with placement of tracheostomy. CASE 3: A 36-year-old G6P3023 with fetal magnetic resonance imaging (MRI) revealing esophageal atresia, polyhydramnios, and severe micrognathia with a jaw index <5th percentile. At 35 weeks the patient underwent EXIT-to-Airway with formal tracheostomy during 35 min of uteroplacental bypass. In the most severe cases of fetal micrognathia, EXIT-to-Airway provides time to evaluate and secure the fetal airway prior to delivery. We propose indications for EXIT-to-Airway in micrognathia to include a jaw index <5%, with indirect evidence of aerodigestive tract obstruction such as polyhydramnios, glossoptosis or an absent stomach bubble.

Expectant management of preterm premature rupture of membranes and nonvertex presentation: what are the risks?

OBJECTIVE: Preterm premature rupture of membranes (PPROM) continues to be a major cause of preterm births. The objective of this study was to compare the morbidity of patients with PPROM nonvertex presentations with patients with PPROM with vertex presentations. STUDY DESIGN: A retrospective analysis of data from 74 patients with PPROM with nonvertex presentations (study group) and 74 patients with PPROM with vertex presentations (control group) comprised the 2 study groups. All patients that met the study admission criteria were treated in a similar manner at 1 of 3 level-III hospitals. The gestational ages at delivery of all patients were between 23-34 weeks, and the gestational ages between case and control patients were matched for gestational age. RESULTS: A statistically significant (P = .03) higher incidence of a prolapsed umbilical cord was found in the study group (n = 8; 10.8%) relative to the control group (n = 1; 1.4%). More infants in the study group had low 5-minute Apgar scores (<5) and/or low cord pH (<7.20; n = 25 [33.8%]) than in the control group (n = 12 [16.2%]; P = .02). Five infants with breech presentations underwent a precipitous unplanned vaginal delivery. Significant morbidity was not detected in these 5 infants. CONCLUSION: After transfer to an antenatal ward, patients with PPROM with nonvertex presentations appear to have a significantly higher risk for prolapsed umbilical cords, lower Apgar scores, and/or lower umbilical cord blood pH values, when compared with their vertex counterparts. Additionally, there appears to be substantial risk of an unintended, vaginal breech delivery.

Precise prenatal diagnosis of tuberous sclerosis by sequencing the TSC2 gene.

BACKGROUND: The presumptive prenatal diagnosis of tuberous sclerosis (TSC) previously depended upon fetal imaging. Cloning of the two TSC genes (TSC1 and TSC2) now enables precise molecular diagnosis by gene sequencing. We used this approach for the prenatal diagnosis of a fetus showing multiple intracardiac tumors. METHODS: DNA extracted from cultivated amniotic fluid cells underwent sequencing of all coding regions and exon-intron boundaries of the TSC1 and TSC2 genes. RESULTS: A mutation (R611Q) was found in exon 16 of the TSC2 gene. Thus far, neither clinically unaffected parents has provided blood samples for mutation analysis. CONCLUSION: For the first time, mutation analysis of a TSC gene enabled a precise prenatal diagnosis.

Antibiotic therapy in preterm premature rupture of membranes: Are seven days necessary? A preliminary, randomized clinical trial.

OBJECTIVE: The purpose of this study was to determine whether 3 days of broad-spectrum antibiotic therapy, which is intended to prolong latency in patients with preterm premature rupture of membranes, is comparable to 7 days of therapy. STUDY DESIGN: Patients with preterm premature rupture of membranes at three separate study sites were asked to participate in this intent-to-treat, prospective, randomized trial. They were assigned randomly to either 3 or 7 days of ampicillin-sulbactam (3 g intravenously every 6 hours). The primary outcome of interest was the latency period from membrane rupture to delivery. RESULTS: Forty-two individuals were enrolled in each group. No difference was noted in the latency interval between the two groups (3 days, 214 +/- 225 hours, vs 7 days, 229 +/- 218 hours). A significantly higher number of patients in the 3-day group completed therapy (80.1% vs 47.6%, P =.003). No other parameters were significantly different between the two groups. No adverse events or trends were noted in either group. CONCLUSION: There appears to be no difference in the latency period between 3 and 7 days of ampicillin-sulbactam antibiotic therapy. More patients are needed to exclude a type II error.

Urolithiasis in pregnancy. Diagnosis, management and pregnancy outcome.

OBJECTIVE: To review our experiences with ureterolithiasis and nephrolithiasis in pregnancy and compare their outcomes with those in the rest of the obstetric population. STUDY DESIGN: A database of obstetric deliveries was used to identify patients with (cases) and without (controls) urolithiasis and to compare demographics and pregnancy complications between the groups. Furthermore, retrospective chart review of the cases group was utilized to obtain additional pertinent information. RESULTS: Over a 3-year period, there were 21,010 deliveries, 86 of which had symptomatic urolithiasis, for an incidence of 1 in 244 pregnancies. Renal calculi occurred more commonly in Caucasians than African Americans. Patients were more likely to become symptomatic in the second or third trimester, and most stones passed spontaneously. Pregnancy complications were similar between the groups; however, there was a higher percentage of preterm premature rupture of membranes in the nephrolithiasis cases (7.0% vs. 2.9%, P < .05). CONCLUSION: Nephrolithiasis and ureterolithiasis occurred more commonly in Caucasians during pregnancy. The majority of patients became symptomatic in the last two-thirds of pregnancy and usually passed the calculus spontaneously. A higher incidence of preterm premature rupture of membranes was noted in pregnancies complicated by urolithiasis.

Perinatal outcomes in preeclampsia that is complicated by massive proteinuria.

OBJECTIVE: Current treatment of preeclampsia no longer mandates delivery for proteinuria of >5 g per 24 hours. We sought to determine whether delayed delivery of preeclampsia with massive proteinuria (>10 g/24 h) increased maternal or neonatal morbidity. STUDY DESIGN: Records of all women with preeclampsia who were delivered at <37 weeks of gestation between January 1, 1997, and June 30, 2001, were reviewed. Patients with underlying renal disease or multiple gestation were excluded. Patients were characterized as having mild (<5 g/24 h), severe (5-9.9 g/24 h), or massive (>10 g/24 h) proteinuria. Outcomes were compared using the chi(2) test, one-way analysis of variance, or Fisher exact test. RESULTS: Two hundred nine patients met the inclusion criteria: 125 patients had mild proteinuria, 43 patients had severe proteinuria, and 41 patients had massive proteinuria. No significant differences in maternal morbidity were seen. Massive proteinuria was associated with earlier onset of preeclampsia, earlier gestational age at delivery, and higher rates of prematurity complications. After correction for prematurity, massive proteinuria has no significant effect on neonatal outcomes. CONCLUSION: Women with preeclampsia and massive proteinuria did not have increased maternal morbidity compared with women with severe or mild proteinuria. Massive proteinuria appears to be a marker for early-onset disease and progression to severe preeclampsia. Neonatal morbidity appears to be a function of prematurity rather than of massive proteinuria itself.

Respiratory morbidity in well-dated twins approaching term. What are the risks of elective delivery?

OBJECTIVE: To evaluate morbidity, particularly respiratory, in well-dated, near-term twins delivered electively and to compare them to those delivered either spontaneously or for obstetric indications. STUDY DESIGN: Charts from twin deliveries were reviewed for inclusion in this study. Inclusion criteria were well-dated twins (American College of Obstetricians and Gynecologists criteria for dating) and absence of (1) corticosteroids, (2) intravenous tocolysis, (3) lung maturity studies, (4) fetal malformations, (5) diabetes, and (6) medical indications for delivery in the elective delivery group. Those meeting the criteria were divided into elective and nonelective (both spontaneous and indicated) delivery groups. Outcome variables included incidence of transient tachypnea and respiratory distress syndrome in each group. RESULTS: During the study period, 168 sets of twins met the admission criteria. Controlling for gestational age, no difference was noted in the incidence of respiratory distress syndrome, transient tachypnea and admission to the special care nursery. Infants born during the 36th week of gestation were more likely to be admitted to the special care nursery (37.8% vs. 10.6%, P < .05) and had a greater incidence of respiratory complications (23.2% vs. 6.7%, P < .01) than those born after 37 weeks. No difference was identified between the 37th and 38th weeks. CONCLUSION: In this large series of well-dated twin pregnancies, there was no evidence of increased respiratory morbidity or special care nursery admissions with elective delivery at or beyond 37 weeks. Twins delivered during the 36th week of gestation had increased respiratory morbidity and special care nursery admissions.