Contemporary Outcomes of Patients with Isolated Bilateral Renal Agenesis with and without Fetal Intervention.

Introduction: Bilateral renal agenesis (BRA) is a lethal diagnosis, specifically meaning that natural survival beyond birth is not expected secondary to pulmonary hypoplasia. Limited contemporary data are available about intervention and the impact of restoring amniotic fluid volume in relation to the risk for lethal pulmonary hypoplasia and other factors that might influence survival in cases of fetal BRA.

Objective: We report the largest series of patients undergoing fetal intervention and postnatal care for BRA at a single comprehensive fetal center.

Congenital high airway obstruction syndrome (CHAOS): Natural history, prenatal management strategies, and outcomes at a single comprehensive fetal center.

Purpose: Congenital high airway obstruction syndrome (CHAOS) is a devastating fetal condition of complete airway discontinuity resulting in significant hydrops and extreme lung hyperplasia. It is universally fatal with survival reported only in the rare spontaneous fistulization or EXIT intervention (Ex Utero Intrapartum Treatment). Even in these cases, mortality remains high, and current investigations are targeting prenatal interventions.

National Practice Patterns for Prenatal Monitoring in Gastroschisis: Gastroschisis Outcomes of Delivery (GOOD) Provider Survey.

Background: Gastroschisis is an abdominal wall defect with increasing incidence. Given the lack of surveillance guidelines among maternal-fetal medicine (MFM) specialists, this study describes current practices in gastroschisis management.

Materials And Methods: An online survey was administered to MFM specialists from institutions affiliated with the North American Fetal Therapy Network (NAFTNet).

Ethical Considerations Concerning Amnioinfusions for Treating Fetal Bilateral Renal Agenesis.

Congenital bilateral renal agenesis has been considered a uniformly fatal condition. However, the report of using serial amnioinfusions followed by the live birth in 2012 and ongoing survival of a child with bilateral renal agenesis has generated hope, but also considerable controversy over an array of complex clinical and ethical concerns. To assess the ethical concerns associated with using serial amnioinfusions for bilateral renal agenesis, we assembled a multidisciplinary group to map the ethical issues relevant to this novel intervention.

Natural History of Fetal Lower Urinary Tract Obstruction with Normal Amniotic Fluid Volume at Initial Diagnosis.

Objective: The aim of this study was to define the natural history of lower urinary tract obstruction (LUTO) with normal midgestational amniotic fluid volumes.

Materials And Methods: We performed a retrospective review of 32 consecutive patients with LUTO with normal midgestational amniotic fluid volume followed at 11 North American Fetal Therapy Network (NAFTNet) centers from August 2007 to May 2012. Normal amniotic fluid volume was defined as an amniotic fluid index (AFI) of ≥9 cm.

Circulating Cell-Free DNA to Determine the Fetal RHD Status in All Three Trimesters of Pregnancy.

Objective: To estimate the accuracy of a new assay to determine the fetal RHD status using circulating cell-free DNA.

Methods: This was a prospective, observational study. Maternal blood samples were collected in each trimester of pregnancy in 520 nonalloimmunized RhD-negative patients.

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.

Hypoplastic left heart syndrome is associated with structural and vascular placental abnormalities and leptin dysregulation.

Introduction: Hypoplastic left heart syndrome (HLHS) is a severe cardiovascular malformation (CVM) associated with fetal growth abnormalities. Genetic and environmental factors have been identified that contribute to pathogenesis, but the role of the placenta is unknown. The purpose of this study was to systematically examine the placenta in HLHS with and without growth abnormalities.

Innovation in maternal-fetal therapy: a position statement from the North American Fetal Therapy Network.

For conscientious practitioners, innovative medical therapy often occupies an uncomfortable space between formal research and established clinical practice. Newer and more rapid methods of information dissemination increase the risk of advertising unproven therapies. These and other concerns should not stifle medical progress and innovation but require guidelines and boundaries.

Diastolic dysfunction and cerebrovascular redistribution precede overt recipient twin cardiomyopathy in early-stage twin-twin transfusion syndrome.

Background: Indications for intervention in early-stage (Quintero I and II) twin-twin transfusion syndrome (TTTS) are not standardized. Fetal echocardiography can be used to guide the management of early-stage patients. The aim of this study was to identify early cardiovascular findings that may precede progression to overt recipient twin (RT) cardiomyopathy in early-stage TTTS.

Altered amniotic fluid leptin levels in twin-twin transfusion syndrome with concurrent placental insufficiency.

Objective: To measure amniotic fluid leptin levels in fetuses with twin-twin transfusion syndrome (TTTS) with and without placental insufficiency (PI) and determine its usefulness as a biomarker of PI.

Study Design: A retrospective case control study of TTTS stage III patients from 2009 to 2011 was conducted. Cases were pregnancies with PI (TTTS-PI, n = 18) matched by stage, gestational age, and degree of cardiomyopathy to controls without PI (TTTS, n = 26).

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.

Tracheal agenesis with tracheoesophageal fistulae: fetal MRI diagnosis with confirmation by ultrasound during an ex utero intrapartum therapy (EXIT) delivery and postdelivery MRI.

Tracheal agenesis is a rare and essentially lethal anomaly with divergent prenatal imaging findings depending on the presence or absence of a tracheoesophageal fistula. All prenatally diagnosed cases of tracheal agenesis reported to date have not had a tracheoesophageal fistula and presented with thoracoabdominal findings similar to congenital high airway obstruction syndrome. We present the case of a 32-week gestation fetus with rapid onset of polyhydramnios and no persistent findings of congenital high airway obstruction syndrome that was ultimately diagnosed with tracheal agenesis plus tracheoesophageal fistula by fetal MRI.

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.

The relationship between amniotic fluid levels of brain-type natriuretic peptide and recipient cardiomyopathy in twin-twin transfusion syndrome.

Objective: We sought to evaluate amniotic fluid brain natriuretic peptide (BNP) levels as a biomarker of recipient twin (RT) cardiomyopathy (RTCM) in twin-twin transfusion syndrome.

Study Design: Amniotic fluid samples were obtained from 157 twin-twin transfusion syndrome RTs and from 6 singletons (controls) from 2007 through 2009. N-terminal prohormone BNP (NT-proBNP) levels were quantified by enzyme-linked immunosorbent assay.

Congenital high airway obstruction syndrome due to complete tracheal agenesis: an accident of nature with clues for tracheal development and lessons in management.

Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition characterized by complete blockage of the fetal airways associated with hydrops. We present a case of CHAOS due to the rare cause of complete tracheal agenesis. The ex utero intrapartum therapy (EXIT) strategy was employed to allow for neck and mediastinal exploration.

Incidence of complications in twin-twin transfusion syndrome after selective fetoscopic laser photocoagulation: a single-center experience.

Objective: The purpose of this study was to evaluate the incidence of complications after selective fetoscopic laser photocoagulation for twin-twin transfusion syndrome (TTTS).

Study Design: One hundred fifty-two cases of TTTS were treated with selective fetoscopic laser photocoagulation from 2005-2008. Complications were TTTS recurrence, amniotic band syndrome, iatrogenic monoamnionicity, and twin anemia-polycythemia sequence.

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.

Pregnancy complicated by triploidy: a comparison of the three karyotypes.

We evaluated triploid pregnancy to determine whether there are clinically important differences between the three karyotypes: 69,XXX, 69,XXY, and 69,XYY. Prospectively maintained cytogenetic databases at five tertiary care centers were retrospectively reviewed over a 10-year period to identify all triploid pregnancies. Targeted ultrasounds were reviewed to identify fetal and placental findings.

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.

Sonographic findings in trisomy 9.

Objective: The purpose of this study was to identify the most common prenatal sonographic findings in fetuses with complete trisomy 9.

Methods: A retrospective review of all cases of trisomy 9 at 5 participating institutions over a 15-year interval was conducted. Indications for referral and sonographic findings in each case were reviewed to identify characteristic fetal structural anomalies.

Hypoplastic left heart syndrome with intact atrial septum: Utilization of a hybrid catheterization facility for cesarean section delivery and prompt neonatal intervention.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (IAS) is a severe congenital cardiac malformation with little possibility of postnatal survival, unless an intra-atrial connection is created promptly after delivery. The timing of that intervention is often dictated by logistic restrictions, such as the proximity of the delivery room to the cardiac catheterization laboratory. This report details the use of a hybrid cardiac catheterization suite to expedite the delivery, transfer, and initial treatment of a fetus with HLHS/IAS.