Julie S. Moldenhauer

Radiofrequency ablation vs bipolar umbilical cord coagulation in the management of complicated monochorionic pregnancies

The application of radiofrequency ablation (RFA) termination procedures to complicated cases involving monochorionic twins offers the potential of a less invasive option when compared to endoscopic techniques. The purpose of this study was to compare outcomes between these two techniques.

Fetal Myelomeningocele Repair: The Post-MOMS Experience at the Children's Hospital of Philadelphia

Background: Fetal myelomeningocele (fMMC) repair has become accepted as a standard of care option in selected circumstances. We reviewed our outcomes for fMMC repair from referral and evaluation through surgery, delivery and neonatal discharge. Material and Methods: All patients referred for potential fMMC repair were reviewed from January 1, 2011 through March 7, 2014. Maternal and neonatal data were collected on the 100 patients who underwent surgery. Results: 29% of those evaluated met the criteria and underwent fMMC repair (100 cases). The average gestational age was 21.9 weeks at evaluation and 23.4 weeks at fMMC repair. Complications included membrane separation (22.9%), preterm premature rupture of membranes (32.3%) and preterm labor (37.5%). Average gestational age at delivery was 34.3 weeks and 54.2% delivered at ≥35 weeks. The perinatal loss rate was 6.1% (2 intrauterine fetal demises and 4 neonatal demises); 90.8% of women delivered at the Childrens Hospital of Philadelphia and 3.4% received transfusions. With regard to the neonates, 2 received ventriculoperitoneal shunts prior to discharge; 71.1% of neonates had no evidence of hindbrain herniation on MRI. Of the 80 neonates evaluated, 55% were assigned a functional level of one or more better than the prenatal anatomic level. Conclusion: In an experienced program, maternal and neonatal outcomes for patients undergoing fMMC repair are comparable to results of the MOMS trial.

Comparison of ultrasound and magnetic resonance imaging parameters in predicting survival in isolated left‐sided congenital diaphragmatic hernia

To compare test characteristics of ultrasound‐ and magnetic resonance imaging (MRI)‐derived parameters in predicting newborn survival in cases of isolated left‐sided congenital diaphragmatic hernia (CDH).

Position statement on fetal myelomeningocele repair

Following the promising multicenter randomized trial results of in utero fetal myelomeningocele repair; we anticipate that an increasing number of tertiary care centers may want to offer this therapy. It is essential to establish minimum criteria for centers providing open fetal myelomeningocele repair to ensure optimal maternal and fetal/pediatric outcomes, as well as patient safety both short- and long-term; and to advance our knowledge of the role and benefit of fetal surgery in the management of fetal myelomeningocele. The fetal myelomeningocele Maternal-Fetal Management Task Force was initially convened by the Eunice Kennedy Shriver National Institute of Child Health and Human Development to discuss the implementation of maternal fetal surgery for myelomeningocele. The decision was made to develop the optimal practice criteria presented in this document for the purpose of medical and surgical leadership. These criteria are not intended to be used for legal or regulatory purposes.

Ex Utero Intrapartum Therapy

The Ex Utero Intrapartum Therapy (EXIT) procedure was initially developed to secure the airway in fetuses at delivery after they had undergone in utero tracheal occlusion for congenital diaphragmatic hernia. Indications for the EXIT procedure have been expanded to include any delivery in which prenatal diagnosis is concerned for neonatal airway compromise, such as large neck masses and Congenital High Airway Obstruction Syndrome, or when a difficult resuscitation is anticipated such as with large lung lesions. Uteroplacental blood flow and gas exchange are maintained through the use of inhalational anesthetics to allow optimal uterine relaxation with partial delivery of the fetus and amnioinfusion to sustain uterine distension. Using the EXIT procedure, sufficient time is provided on placental bypass to perform life-saving procedures such as bronchoscopy, laryngoscopy, endotracheal intubation, tracheostomy, cannulation for extracorporeal membrane oxygenation, and resection of lung masses or resection of neck masses in a controlled setting, thus avoiding a potential catastrophe.

Thoracoamniotic shunts for the management of fetal lung lesions and pleural effusions: a single-institution review and predictors of survival in 75 cases

PURPOSE Hydrops and pulmonary hypoplasia are associated with significant morbidity and mortality in the setting of a congenital lung lesion or pleural effusion (PE). We reviewed our experience using in utero thoracoamniotic shunts (TA) to manage fetuses with these diagnoses. METHODS A retrospective review of fetuses diagnosed with a congenital lung lesion or pleural effusion who underwent TA shunt placement from 1998-2013 was performed. RESULTS Ninety-seven shunts were placed in 75 fetuses. Average gestational age (±SD) at shunt placement and birth was 25±3 and 34±5 weeks. Shunt placement resulted in a 55±21% decrease in macrocystic lung lesion volume and complete or partial drainage of the PE in 29% and 71% of fetuses. 69% of fetuses presented with hydrops, which resolved following shunt placement in 83%. Survival was 68%, which correlated with GA at birth, % reduction in lesion size, unilateral pleural effusions, and hydrops resolution. Surviving infants had prolonged NICU courses and often required either surgical resection or tube thoracostomy in the perinatal period. CONCLUSION TA shunts provide a therapeutic option for select fetuses with large macrocystic lung lesions or PEs at risk for hydrops and/or pulmonary hypoplasia. Survival following shunting depends on GA at birth, reduction in mass size, and hydrops resolution.

Effect of single and multiple courses of maternal betamethasone on prenatal congenital lung lesion growth and fetal survival

PURPOSE Administration of maternal betamethasone (BMZ) is a therapeutic option for fetuses with large microcystic congenital lung lesions at risk for, or causing, hydrops. Not all fetuses respond to a single course of BMZ. We review our experience with the use of single and multiple courses of maternal BMZ for the management of these patients. METHODS A retrospective review of fetuses with congenital lung lesions managed with maternal BMZ from 2003 to 2014 was performed. RESULTS Forty-three patients were managed with prenatal steroids (28 single course, 15 multiple courses). Single course recipients demonstrated a reduction in lesion size and resolution of hydrops in 82% and 88% of patients respectively compared to 47% and 56% in recipients of multiple steroid courses. Survival of multiple course patients (86%) was comparable to that of single course patients (93%) and improved compared to non-treated historical controls. Multiple course recipients demonstrated an increased need for open fetal surgery and postnatal surgery at a younger age. CONCLUSION Fetuses who fail to respond to a single course of BMZ may benefit, as indicated by hydrops resolution and improved survival, from additional courses. However, failure to respond is indicative of a lesion which may require fetal or immediate neonatal resection.

Ex utero intrapartum treatment (EXIT) in the management of cervical lymphatic malformation

PURPOSE The purpose of this study was to review the outcomes and technical details of EXIT procedures performed in fetuses with large cervical lymphatic malformations. METHODS A retrospective chart review of fetuses with a prenatal diagnosis of cervical lymphatic malformation evaluated at our center between 1995 and 2013 was performed. RESULTS We evaluated a total of 112 fetuses with a prenatal diagnosis of cervical lymphatic malformation. Thirteen of the 112 fetuses (11%) were delivered by an EXIT procedure. Criteria to deliver by EXIT were: 1) deviation/compression/obstruction of the airway, and 2) involvement of the floor of the mouth. Two fetuses developed hydrops. Five fetuses developed polyhydramnios. Eleven EXITs were performed electively at term (n=7; 37-38 weeks) or late pre-term (n=4; 34-36/6 weeks), whereas two patients underwent emergency EXIT at 33 and 38 weeks, respectively. The airway was accessed successfully in 12 of 13 cases. Laryngoscopy only was sufficient in 7, rigid bronchoscopy was required in 4, and 1 required a tracheostomy. In one case with a massive lymphatic malformation of the face, neck, and airway, a tracheostomy was not attempted, and the fetus expired. Four patients had invasion of the larynx by the lymphatic malformation. Five patients required a tracheostomy later. Median time from fetal exposure to intubation was 8 (2-29) min. Median total EXIT time was 105.5 (67-142) min. Median maternal blood loss was 800 (300-1000) ml. Median maternal hospital stay was 4 (3-6) days. CONCLUSION The EXIT procedure allows controlled airway access in fetuses with cervical lymphatic malformations and evidence of airway impairment on prenatal images.

The Role of Echocardiography in the Intraoperative Management of the Fetus Undergoing Myelomeningocele Repair

Introduction: Fetal surgery for myelomeningocele (MMC) results in better outcomes compared to postnatal treatment. However, risks are present. We describe our experience with intraoperative fetal echocardiography during repair of MMC and report on the management of serious cardiovascular events. Material and Methods: The subjects included fetuses with intent to repair MMC from January 2011 to February 2014. The protocol involved continuous echocardiography in a looping, sequential manner of systolic function, heart rate and tricuspid and mitral valve regurgitation. Results: A total of 101 cases intended fetal MMC repair; 100 completed surgery. Intraoperative ventricular dysfunction was present in 60% (20 mild, 25 moderate, 15 severe). Heart rate <100 bpm was noted in 11 cases. Tricuspid valve regurgitation was present in 35% (26 mild, 7 moderate, 2 severe); mitral valve regurgitation was present in 19% (15 mild, 4 moderate). Serious cardiovascular events were experienced in 7 cases, which affected the conduct of surgery and/or outcome. In 4 of these, medications were given via the umbilical vein and external cardiac compressions were performed. Fetal echocardiography was used to gauge the efficacy of compressions and to guide resuscitation. Discussion: Cardiovascular compromise is common during fetal surgery for MMC. Intraoperative fetal echocardiography is recommended as a growing number of centers contemplate offering this form of novel, but potentially risky, therapy.

In utero repair of spina bifida.

Open spina bifida or myelomeningocele (MMC) is the most common congenital malformation of the central nervous system compatible with long-term survival and is associated with significant lifelong disabilities. Postnatal care of MMC involves covering the exposed spinal cord, infection prevention, and ventricular shunting for hydrocephalus. The aim of postnatal MMC surgery is not to reverse or prevent the neurologic injury seen in MMC, but to palliate. The neurologic defects result from primary incomplete neurulation and secondary chronic in utero damage to the exposed neural elements through mechanical and chemical trauma-the two-hit hypothesis. With the ability to accurately diagnose spina bifida prenatally and the concept of the two-hit hypothesis, in utero repair to decrease exposure and alter the antenatal course of neurologic destruction was conceived. Through animal models and human pilot studies, the feasibility of fetal spina bifida repair was demonstrated. Subsequently, the prospective randomized multicenter Management of Myelomeningocele Study (MOMS trial) revealed a decreased need for shunting, reversal of hindbrain herniation, and preservation of neurologic function, making in utero repair an accepted care alternative for select women carrying a fetus with spina bifida. This article will highlight the background and rationale for in utero repair, and the progression to becoming an alternative standard of care. The future directions of fetal spina bifida repair will also be addressed.