Maternal and child life years gained by transfusing low titer group O whole blood in trauma: A computer simulation.

BACKGROUND: Using low titer group O whole blood (LTOWB) is increasingly popular for resuscitating trauma patients. LTOWB is often RhD-positive, which might cause D-alloimmunization and hemolytic disease of the fetus and newborn (HDFN) if transfused to RhD-negative females of childbearing potential (FCP). This simulation determined the number of life years gained by the FCP and her future children if she was resuscitated with LTOWB compared with conventional component therapy (CCT). METHODS: The model simulated 500,000 injured FCPs of each age between 0 and 49 years with LTOWB mortality relative reductions (MRRs) compared with components between 0.1% and 25%. For each surviving FCP, number of life years gained was calculated using her age at injury and average life expectancy for American women. The number of expected future pregnancies for FCPs that did not survive was also based on her age at injury; each future child was assigned the maximum lifespan unless they suffered perinatal mortality or serious neurological events from HDFN. RESULTS: The LTOWB group with an MRR 25% compared with CCT had the largest total life years gained. The point of equivalence for RhD-positive LTOWB compared to CCT, where life years lost due to severe HDFN was equivalent to life years gained due to FCP survival/future childbearing, occurred at an MRR of approximately 0.1%. CONCLUSION: In this model, RhD-positive LTOWB resulted in substantial gains in maternal and child life years compared with CCT. A >0.1% relative mortality reduction from LTOWB offset the life years lost to HDFN mortality and severe neurological events.

Development and Validation of a Scale to Measure Person-Centered Care in Fetal Care Centers.

INTRODUCTION: Fetal care centers (FCCs) in the USA lack a standardized instrument to measure person-centered care. This study aimed to develop and validate the Person-Centered Care in Fetal Care Centers (PCC-FCC) Scale. METHODS: Initial items were developed based on literature and input from clinicians and former patients. A Delphi study involving 16 experts was conducted to validate the content and construct. Through three rounds of online questionnaires using open-ended questions and Likert scales, consensus on item clarity and relevancy was established. The resulting items were then piloted with former fetal care center patients via a web-based survey. The instrument's reliability and validity were validated using Cronbach's α and exploratory factor analysis, respectively. Concurrent validity was assessed by comparing scores with the Revised Patient Perception of Patient-Centeredness (PPPC-R) Questionnaire. RESULTS: 258 participants completed the 48-item pilot PCC-FCC survey, categorized into six domains. Factor analysis yielded a 2-factor, 28-item scale. Internal consistency of the final scale had good reliability (α = 0.969). Data supported content, construct, and concurrent validity. CONCLUSION: The PCC-FCC Scale is a reliable and valid measure of person-centered care in U.S. FCCs. It can be used to enhance services and begin connecting person-centered care to maternal-child health outcomes.

Prenatal Diagnosis of Fetal Aqueductal Stenosis: A Multicenter Prospective Observational Study through the North American Fetal Therapy Network.

INTRODUCTION: A critical component of an evidence-based reassessment of in-utero intervention for fetal aqueductal stenosis (fetal AS) is determining if the prenatal diagnosis can be accurately made at a gestational age amenable to in-utero intervention. METHODS: A multicenter, prospective, observational study was conducted through the North American Fetal Therapy Network (NAFTNet). Pregnancies complicated by severe central nervous system (CNS) ventriculomegaly (lateral ventricle diameter >15 mm) not secondary to a primary diagnosis (myelomeningocele, encephalocele, etc.) were recruited at diagnosis. Imaging and laboratory findings were recorded in an online REDCap database. After evaluation, investigators were asked to render their degree of confidence in the diagnosis of fetal AS. The prenatal diagnosis was compared to the postnatal diagnosis obtained through neonatal neuroimaging. Performance characteristics of ultrasound and magnetic resonance imaging (MRI) were calculated, as was the mean gestational age at diagnosis. RESULTS: Between April 2015 and October 2022, eleven NAFTNet centers contributed 64 subjects with severe fetal CNS ventriculomegaly. Of these, 56 had both prenatal and postnatal diagnoses recorded. Ultrasound revealed 32 fetal AS true positives, 4 false positives, 7 false negatives, and 13 true negatives, rendering a sensitivity of 0.82, a specificity of 0.76, a positive predictive value of 0.89, and a negative predictive value of 0.65. The mean gestational age at diagnosis by ultrasound was 25.5 weeks (std +/- 4.7 weeks). The proportion of agreement (true positive + true negative/n) was highest at 24 weeks gestation. For fetal MRI (n = 35), the sensitivity for fetal AS was 0.95, specificity was 0.69, positive predictive value was 0.84, and negative predictive value was 0.90. MRI was performed at 25 weeks on average. CONCLUSION: The prenatal diagnosis of fetal AS can be made with accuracy at a gestational age potentially amenable to in-utero intervention. Only 7% of subjects were incorrectly diagnosed prenatally with fetal AS by ultrasound and 11% by MRI. Diagnostic accuracy of fetal AS will likely improve with increased experience.

North American Fetal Therapy Network: Maternal Outcomes in Fetal Aqueductal Stenosis.

INTRODUCTION: Fetal aqueductal stenosis (AS) affects approximately 1:1,000 pregnancies. Obstruction of cerebral spinal fluid (CSF) circulation occurs at the aqueduct of Sylvius, leading to progressive hydrocephalus, often necessitating cesarean section (CS) with a classical uterine incision. The purpose of this study is to describe maternal outcomes associated with fetal aqueductal stenosis. METHODS: This study is conducted through the North American Fetal Therapy Network (NAFTNet). Subjects were recruited at the diagnosis of severe CNS ventriculomegaly and were followed longitudinally. Maternal events around the delivery of fetuses with AS were recorded and analyzed. RESULTS: Thirty-seven subjects with fetal AS confirmed by neonatal neuroimaging were analyzed. The average gestation at delivery was 36.7 weeks. Overall, 86% were delivered by cesarean section (CS), and 62% of these were elective. Sixty-two percent were delivered electively by cesarean section (CS). Eighty-six percent were delivered by cesarean section. Ninety one percent of CSs were performed through a Pfannenstiel abdominal incision. A classical uterine incision was required in 13% of cesarean deliveries. The peripartum complication rate was 27%. CONCLUSION: Women carrying a fetus with AS were at risk for preterm birth, cesarean delivery, a classical uterine incision, and peripartum complications. These data highlight the maternal morbidity associated with fetal AS, and the potential benefit of in-utero therapy not only for neonatal but also maternal outcomes.

Fetoscopic laser photocoagulation versus expectant management for stage I twin-to-twin transfusion syndrome: A systematic review and meta-analysis.

To investigate the outcomes of asymptomatic stage I twin-to-twin transfusion syndrome (stage I TTTS) among patients treated with fetoscopic laser photocoagulation (FLP) versus expectant management. Databases such as PubMed, Web of Science and Scopus were systematically searched from inception up to March 1st, 2022. The primary outcome was at least one fetal survival at birth and secondary outcomes included gestational age at delivery, preterm premature rupture of membranes < 32 weeks, preterm birth < 32 weeks, and single and dual fetal survival. Five studies were included in the meta-analysis. There was no significant difference in terms of at least one survival (odds ratio (OR) = 1.40, 95%CI= (0.26, 7.43), P = 0.70), single survival (OR = 0.87, 95%CI= (0.51, 1.48), P = 0.60) and dual survival (OR = 1.63, 95%CI= (0.74, 3.62), P = 0.23) among FLP and expectant groups. Gestational age at delivery (mean difference = 1.19, 95%CI= (-0.25, 2.63), P = 0.10), the risk of PTB<32 weeks (OR = 0.88, 95%CI= (0.50, 1.54), P = 0.65), and pPROM<32 weeks (OR = 1.80, 95% CI= (0.41, 7.98), P = 0.44) were also comparable between the groups. Routine FLP of the placental anastomoses before 26 weeks of gestation is unlikely to be beneficial among asymptomatic stable stage I TTTS patients without cervical shortening as the procedure does not offer a survival advantage compared with expectant management.

Placental Location in Maternal-Fetal Surgery for Myelomeningocele.

INTRODUCTION: Uterine incision based on the placental location in open maternal-fetal surgery (OMFS) has never been evaluated in regard to maternal or fetal outcomes. OBJECTIVE: The aim of this study was to investigate whether an anterior placenta was associated with increased rates of intraoperative, perioperative, antepartum, obstetric, or neonatal complications in mothers and babies who underwent OMFS for fetal myelomeningocele (fMMC) closure. METHODS: Data from the international multicenter prospective registry of patients who underwent OMFS for fMMC closure (fMMC Consortium Registry, December 15, 2010-June 31, 2019) was used to compare fetal and maternal outcomes between anterior and posterior placental locations. RESULTS: The placental location for 623 patients was evenly distributed between anterior (51%) and posterior (49%) locations. Intraoperative fetal bradycardia (8.3% vs. 3.0%, p = 0.005) and performance of fetal resuscitation (3.6% vs. 1.0%, p = 0.034) occurred more frequently in cases with an anterior placenta when compared to those with a posterior placenta. Obstetric outcomes including membrane separation, placental abruption, and spontaneous rupture of membranes were not different among the 2 groups. However, thinning of the hysterotomy site (27.7% vs. 17.7%, p = 0.008) occurred more frequently in cases of an anterior placenta. Gestational age (GA) at delivery (p = 0.583) and length of stay in the neonatal intensive care unit (p = 0.655) were similar between the 2 groups. Fetal incision dehiscence and wound revision were not significantly different between groups. Critical clinical outcomes including fetal demise, perinatal death, and neonatal death were all infrequent occurrences and not associated with the placental location. CONCLUSIONS: An anterior placental location is associated with increased risk of intraoperative fetal resuscitation and increased thinning at the hysterotomy closure site. Individual institutional experiences may have varied, but the aggregate data from the fMMC Consortium did not show a significant impact on the GA at delivery or maternal or fetal clinical outcomes.

The risk to future pregnancies of transfusing Rh(D)-negative females of childbearing potential with Rh(D)-positive red blood cells during trauma resuscitation is dependent on their age at transfusion.

BACKGROUND: A risk assessment model for predicting the risk of haemolytic disease of the fetus and newborn (HDFN) in future pregnancies following the transfusion of Rh(D)-positive red blood cell (RBC)-containing products to females of childbearing potential (FCP) was developed, accounting for the age that the FCP is transfused in various countries. METHODS: The HDFN risk prediction model included the following inputs: risk of FCP death in trauma, Rh(D) alloimmunization rate following Rh(D)-positive RBC transfusion, expected number of live births following resuscitation, probability of carrying an Rh(D)-positive fetus, the probability of HDFN in an Rh(D)-positive fetus carried by an alloimmunized mother. The model was implemented in Microsoft R Open, and one million FCPs of each age between 18 and 49 years old were simulated. Published data from eight countries, including the United States, were utilized to generate country-specific HDFN risk estimates. RESULTS: The risk predictions showed similar characteristics for each country in that the overall risk of having a pregnancy affected by HDFN was higher if the FCP was younger when she received her Rh(D)-positive transfusion than if she was older. In the United States, the overall risk of HDFN if the FCP was transfused at age 18 was 3·4% (mild: 1·20%, moderate: 0·45%; severe: 1·15%; IUFD: 0·57%); the risk was approximately 0% if the FCP was 43 years or older at the time of transfusion. CONCLUSION: This model can be used to predict HDFN outcomes when establishing transfusion policies as it relates to the administration of Rh(D)-positive products for massively bleeding FCPs.

Fetal aqueductal stenosis: Prenatal diagnosis and intervention.

Fetal severe central nervous system ventriculomegaly is associated with poor neurologic outcomes, usually driven by a primary malformation, deformation, or disruption of brain parenchyma. In utero shunting of excess cerebrospinal fluid (CSF) in hopes of improving neurologic outcomes was attempted in the 1980s but was abandoned due to perceived lack of effect, likely due to technological limitations of the time that precluded proper patient selection. Little progress on the antenatal management of severe ventriculomegaly has been made in the intervening decades. A multidisciplinary, evidence-based reassessment of ventriculoamniotic shunting for isolated fetal aqueductal stenosis (FAS), a unique form of severe ventriculomegaly (supratentorial intracranial hypertension), is currently underway. An accurate diagnosis of FAS must precede in utero intervention. Magnetic resonance imaging (MRI) will be an excellent adjunct to high-resolution prenatal ultrasound and next-generation genetic testing to correctly diagnose FAS in a timely fashion while excluding other intracranial and extracranial anomalies. This manuscript will briefly discuss the history, current management, and future directions of the prenatal diagnosis and potential intervention for FAS.

Impact of intrauterine transfusion on fetal coagulation physiology by thromboelastography.

OBJECTIVE: Thromboelastography (TEG) is a point-of-care device used to evaluate whole blood coagulation function. The TEG is unique as a test of coagulation function in that it measures the interaction of all components of clot formation, which is different than traditional laboratory-based tests that measure isolated components of coagulation. Little is known about fetal coagulation physiology. We sought to evaluate the impact of severe fetal anemia and intrauterine transfusion (IUT) on fetal coagulation physiology by use of the TEG and to compare fetal TEG values to those of healthy neonates and adults. METHOD: One milliliter of fetal blood was collected immediately before (pre) and after (post) IUT of packed red blood cells (PRBCs). Sampling and transfusion were performed for fetal anemia due to hemolytic disease of the fetus and newborn. Samples were run in duplicate. For descriptive summary, duplicate pre-IUT and post-IUT values were averaged. Values for R (initiation of clot in minutes), K (clot firmness in minutes), angle (kinetics of clot development in degrees), and MA (maximum strength in mm) were obtained for each sample and presented using mean ± SE. Pre-IUT values for R, K, angle, and MA were compared with post-IUT values using linear mixed-effect model to account for clustering due to repeated observation from the same fetus. Pre-IUT values are compared with normal healthy term neonates and healthy adults using Wald test. The study was approved by the University of Pittsburgh Institutional Review Board (PRO14050051). RESULTS: Four fetuses underwent nine IUTs rendering 17 pre-IUT and 17 post-IUT specimens. The mean gestational age at IUT was 31 weeks 2 days (25 weeks 4 days to 35 weeks 2 days). The mean IUT volume transfused was 69 mL (30-170 mL). The mean estimated percent intravascular volume transfused was 33.4% (19%-52%). Of the four variables analyzed, only R showed a significant difference, with the initiation of clot formation being modestly delayed after transfusion by an estimated 2.87 minutes (95% CI, 0.82-4.92, P = .0480). Pre-IUT values were compared with 100 normal term neonates and 118 healthy adults. Compared with pre-IUT, only R was affected (shorter) compared with term neonates (mean ± SE = 5.46 ± 0.16 minute, P < .001) and healthy adults (mean ± SE = 6.8 ± 0.13 minute, P < .001). CONCLUSION: OTHER THAN A MODEST PROLONGATION OF CLOT FORMATION TIME, IUT OF PRBCS OF UP TO 52% OF THE ESTIMATED INTRAVASCULAR VOLUME DID NOT AFFECT FETAL COAGULATION FUNCTION BY TEG. OTHER THAN A SHORTER CLOT FORMATION TIME (R), THERE IS NO DIFFERENCE IN TEG VALUES BETWEEN ANEMIC PRETERM FETUSES AND HEALTHY TERM NEONATES AND HEALTHY ADULTS. BEYOND GENERALIZABLE KNOWLEDGE, THIS INFORMATION COULD BE EXPLOITED FOR FUTURE FETAL INTERVENTION TECHNIQUES.

Histologic Appearance of Iatrogenic Obstructive Hydrocephalus in the Fetal Lamb Model.

INTRODUCTION: Documentation of histologic findings associated with congenital hydrocephalus in the fetal lamb model is a critical step in evaluating the efficacy of ventriculoamniotic shunting in the human fetus. METHODS: Four fetal sheep had hydrocephalus induced at approximately 95 days' gestation. Two co-twins remained as controls. The ewes were euthanized at term. The lamb brains were fixed in formalin, paraffin-embedded, stained, and analyzed for markers of neuropathology. Astrocytosis, microgliosis, and axonal loss were assessed with immunocytochemistry for glial fibrillary acidic protein, ionized calcium-binding adapter, and neurofilament/amyloid precursor protein, respectively. Cortical gray matter extracellular matrix was assessed with staining for the lectin Wisteria Floribunda agglutinin. RESULTS: Hydrocephalic lamb brains demonstrated deep white matter damage with loss of projecting axonal tracts in regions physically distorted by hydrocephalus, similar to that seen in hydrocephalic humans. There was no evidence of abnormal neocortical neuronal migration; however, there was evidence for delayed maturation of the neocortical gray matter, possibly from increased intracerebral pressure and subsequent ischemia. Control lamb brains demonstrated none of the above findings. CONCLUSION: This histological approach can be used to further define the mechanism of brain damage associated with hydrocephalus and interpret the efficacy of ventriculoamniotic shunting on fetal lamb brain neuroanatomy.

Subsequent pregnancy outcomes after open maternal-fetal surgery for myelomeningocele.

BACKGROUND: Open maternal-fetal surgery for fetal myelomeningocele results in reduction in neonatal morbidity related to spina bifida but may be associated with fetal, neonatal, and maternal complications in subsequent pregnancies. OBJECTIVE: The objective of this study was to ascertain obstetric risk in subsequent pregnancies after open maternal-fetal surgery for fetal myelomeningocele closure. STUDY DESIGN: An international multicenter prospective observational registry created to track and report maternal, obstetric, fetal/neonatal, and subsequent pregnancy outcomes following open maternal-fetal surgery for fetal myelomeningocele was evaluated for subsequent pregnancy outcome variables. Institutional Review Board approval was obtained for the registry. RESULTS: From 693 cases of open maternal-fetal surgery for fetal myelomeningocele closure entered into the registry, 77 subsequent pregnancies in 60 women were identified. The overall live birth rate was 96.2%, with 52 pregnancies delivering beyond 20 weeks gestational age and median gestational age at delivery of 37 (36.3-37.1) weeks. The uterine rupture rate was 9.6% (n = 5), resulting in 2 fetal deaths. Maternal transfusion was required in 4 patients (7.7%). CONCLUSION: The risk of uterine rupture or dehiscence in subsequent pregnancies with associated fetal morbidity after open maternal-fetal surgery is significant, but is similar to that reported for subsequent pregnancies after classical cesarean deliveries. Future pregnancy considerations should be included in initial counseling for women contemplating open maternal-fetal surgery.

Blood Group Antigen Matching Influence on Gestational Outcomes (AMIGO) study.

BACKGROUND: Red blood cell (RBC) antigen matching policies to prevent alloimmunization in females of childbearing potential (FCP) vary between centers. To inform transfusion centers responsible for making decisions about matching policies for FCPs, the causal stimulus of the antibodies implicated in severe hemolytic disease of the fetus and newborn (HDFN) must be determined. STUDY DESIGN AND METHODS: We conducted a multinational retrospective study of women with offspring affected by severe HDFN requiring neonatal exchange transfusion and/or intrauterine transfusion. Mothers treated at centers that provide extended antigen-negative RBCs (MATCH, five centers) and those that do not (NoMATCH, nine centers) were compared. RESULTS: A total of 293 mothers had at least one affected pregnancy: 179 at MATCH centers and 114 at NoMATCH centers. Most alloimmunization (83%) was attributed to previous pregnancy: 3% to transfusion (two cases at MATCH, six at NoMATCH centers) and 14% undetermined (both antecedent transfusion and pregnancy). Only 50 mothers had received transfusions; 13 had HDFN due to anti-K at MATCH and four at NoMATCH centers. Most (12/13, 92%) of the anti-K HDFN cases at MATCH centers had K+ paternal antigen status. Mothers at the MATCH centers do not appear to be protected from HDFN due to K, C, c, and E antibodies, although the low number of FCPs who received transfusions precluded drawing firm conclusions. CONCLUSION: The causal stimulus of antibodies that cause HDFN is predominantly from previous pregnancy. Although extended RBC matching for FCPs may impart some protection from allosensitization, we were unable to show a positive effect, possibly because matching policies are not uniform and there was a small number of mothers who previously received transfusions.

Fetal Treatment 2017: The Evolution of Fetal Therapy Centers - A Joint Opinion from the International Fetal Medicine and Surgical Society (IFMSS) and the North American Fetal Therapy Network (NAFTNet).

More than 3 decades ago, a small group of physicians and other practitioners active in what they called "fetal treatment" authored an opinion piece outlining the current status and future challenges anticipated in the field. Many advances in maternal, neonatal, and perinatal care and diagnostic and therapeutic modalities have been made in the intervening years, yet a thoughtful reassessment of the basic tenets put forth in 1982 has not been published. The present effort will aim to provide a framework for contemporary redefinition of the field of fetal treatment, with a brief discussion of the necessary minimum expertise and systems base for the provision of different types of interventions for both the mother and fetus. Our goal will be to present an opinion that encourages the advancement of thoughtful practice, ensuring that current and future patients have realistic access to centers with a range of fetal therapies with appropriate expertise, experience, and subspecialty and institutional support while remaining focused on excellence in care, collaborative scientific discovery, and maternal autonomy and safety.

The impact of mode of delivery on infant neurologic outcomes in myelomeningocele.

BACKGROUND: Controversy exists regarding the optimal route of delivery for fetuses who are diagnosed prenatally with myelomeningocele. Current recommendations are based partly on antiquated studies with questionable methods. All studies that have been published to date suffer from nonstandardized outcome measures, selection bias, and small sample size. The larger studies are >15 years old. OBJECTIVE: The purpose of this study was to provide information for evidence-based decision-making regarding the impact of route of delivery on motor outcomes for pediatric patients with prenatally were diagnosed myelomeningocele in a well-defined retrospective cohort. STUDY DESIGN: Medical records were reviewed retrospectively for all neonates who had been diagnosed with a myelomeningocele at birth from 1995-2015 within the University of Pittsburgh Medical Center system, as identified through the Children's Hospital of Pittsburgh Neurosurgery Department operative database. Records were matched with maternal records with the use of the Center for Assistance in Research that used eRecord. Data from 72 maternal-neonatal pairs were analyzed for multiple variables. The primary outcome measure was the difference between the functional and anatomic motor levels in the child at the age of 2 years, stratified by mode of delivery and presence or absence of labor. The sample size necessary to detect a difference between the groups with power of 0.8 and significance of .05 was calculated to be 52 subjects total (26 per group). RESULTS: Functional levels were slightly better than predicted by anatomic levels for all pediatric patient groups, regardless of mode of delivery or presence of labor. Anatomic levels were slightly lower (better), and defects were smaller for those infants who underwent vaginal delivery or a trial of labor, likely attributable to selection bias. Attempts to correct for this selection bias did not change the results. No other outcomes that were analyzed were associated significantly with mode of delivery or presence of labor. CONCLUSION: No benefit to motor function from delivery by cesarean section or avoidance of labor was demonstrated statistically in this mother-infant cohort.

North American Fetal Therapy Network: intervention vs expectant management for stage I twin-twin transfusion syndrome.

BACKGROUND: Stage I twin-twin transfusion syndrome presents a management dilemma. Intervention may lead to procedure-related complications while expectant management risks deterioration. Insufficient data exist to inform decision-making. OBJECTIVE: The aim of this retrospective observational study was to describe the natural history of stage I twin-twin transfusion syndrome, to assess for predictors of disease behavior, and to compare pregnancy outcomes after intervention at stage I vs expectant management. STUDY DESIGN: Ten North American Fetal Therapy Network centers submitted well-documented cases of stage I twin-twin transfusion syndrome for analysis. Cases were retrospectively divided into 3 management strategies: those managed expectantly, those who underwent amnioreduction at stage I, and those who underwent laser therapy at stage I. Outcomes were categorized as no survivors, 1 survivor, 2 survivors, or at least 1 survivor to live birth, and good (twin live birth ≥30.0 weeks), mixed (single fetal demise or delivery between 26.0-29.9 weeks), and poor (double fetal demise or delivery <26.0 weeks) pregnancy outcomes. Outcomes were analyzed by initial management strategy. RESULTS: A total of 124 cases of stage I twin-twin transfusion syndrome were studied. In all, 49 (40%) cases were managed expectantly while 30 (24%) underwent amnioreduction and 45 (36%) underwent laser therapy at stage I. The overall fetal mortality rate was 20.2% (50 of 248 fetuses). Of those managed expectantly, 11 patients regressed (22%), 4 remained stage I (8%), 29 advanced in stage (60%), and 5 experienced spontaneous previable preterm birth (10%) during observation. The mean number of days from diagnosis of stage I to a change in status (progression, regression, loss, or delivery) was 11.1 (SD 14.3) days. Intervention by amniocentesis or laser therapy was associated with a lower risk of fetal loss (P = .01) than expectant management. The unadjusted odds of poor outcome were 0.33 (95% confidence interval, 0.09-01.20), for amnioreduction and 0.26 (95% confidence interval, 0.09-0.77) for laser therapy vs expectant management. Adjusting for nulliparity, recipient maximum vertical pocket, gestational age at diagnosis, and placenta location had negligible effect. Both amnioreduction and laser therapy at stage I decreased the likelihood of no survivors (odds ratio, 0.11; 95% confidence interval, 0.02-0.68 and odds ratio, 0.07; 95% confidence interval, 0.01-0.37, respectively). Only laser therapy, however, was protective against poor outcome in our data (odds ratio, 0.29; 95% confidence interval, 0.07-1.30 for amnioreduction vs odds ratio, 0.12, 95% confidence interval, 0.03-0.44 for laser), although the estimate for amnioreduction suggests a protective effect. CONCLUSION: Stage I twin-twin transfusion syndrome was associated with substantial fetal mortality. Spontaneous resolution was observed, although the majority of expectantly managed cases progressed. Progression was associated with a worse prognosis. Both amnioreduction and laser therapy decreased the chance of no survivors, and laser was particularly protective against poor outcome independent of multiple factors. Further studies are justified to corroborate these findings and to further define risk stratification and surveillance strategies for stage I disease.

Ultrasound assessment of congenital fetal lung masses and neonatal respiratory outcomes.

OBJECTIVE: Fetal congenital lung masses generate concern for compromised postnatal respiratory function. Congenital pulmonary adenomatoid malformation volume ratio (CVR) has been used to predict the risk of hydrops fetalis and need for antenatal intervention. This study investigates whether CVR could be used to predict neonatal respiratory outcomes. METHODS: The ultrasounds of fetuses diagnosed with a lung mass between 2005 and 2013 were reviewed. CVR was calculated at each ultrasound using the formula for a prolate ellipse divided by head circumference. The pregnancy outcome and information about NICU admission for respiratory insufficiency were collected. RESULTS: Forty-two fetuses were diagnosed with a lung mass during the study period. CVR prior to 24 weeks and between 24 and 32 weeks were associated with NICU admission (p < 0.0001 and <0.008, respectively). CVR increased up to 32 weeks and decreased thereafter for most subjects. The decrease in CVR beyond 32 weeks was larger for cases that required NICU admission (p = 0.002). For a CVR cut-off of <0.5, the sensitivity was 100%, the specificity 85.7%, and negative predictive value was 100% for regular nursery care. CONCLUSION: In pregnancies diagnosed with fetal lung masses, CVR predicted normal respiratory outcomes and need for NICU admission. This information may be helpful for delivery planning. © 2015 John Wiley & Sons, Ltd.

Accuracy of prenatal diagnosis of isolated aqueductal stenosis.

OBJECTIVE: The objective of this article is to determine the success rate of prenatally diagnosed isolated aqueductal stenosis (AS) as a first step in an evidence-based reassessment of ventriculoamniotic shunting for isolated AS. METHODS: Cases of ventriculomegaly at Magee-Womens Hospital between 2006 and 2013 were ascertained. AS was suspected when prenatal ultrasound and magnetic resonance imaging (MRI) demonstrated signs of pressure hydrocephalus. The prenatal diagnosis generated by ultrasound and MRI was compared with the postnatal diagnosis based upon neonatal neuroimaging. RESULTS: The initial query of the Magee-Womens Hospital database resulted in 370 cases of ventriculomegaly. After exclusion for associated central nervous system malformations, 110 cases of severe ventriculomegaly remained. The imaging studies on each fetus were reviewed, and cases of prenatally diagnosed AS were identified. The median gestational age of the ultrasound diagnosis and prenatal MRI was 23 weeks 3 days and 33 weeks 3 days, respectively. All cases of prenatally suspected isolated AS (six of six) and cases of AS with other associated central nervous system anomalies (six of six) were correctly identified. CONCLUSION: An accurate prenatal diagnosis of isolated fetal AS is possible. This is an important first step in an evidence-based reassessment of ventriculoamniotic shunting for isolated AS. © 2014 John Wiley & Sons, Ltd.

Fetal Therapy for Isolated Aqueductal Stenosis.

Aqueductal stenosis (AS) is a form of noncommunicating hydrocephalus, which causes increased intracranial pressure secondary to obstruction of the aqueduct of Sylvius. Relief of intracranial pressure in the fetus by ventriculoamniotic shunting may diminish or even prevent permanent neurologic injury. Shunting was attempted in the 1980s but was abandoned due to technical difficulties. Given the advances in prenatal diagnosis and fetal intervention over the last 3 decades, we believe that an evidence-based reevaluation of the option is timely and appropriate. The aim of this review article is to discuss the clinical significance of the diagnosis of AS, current management strategies, current diagnostic capabilities, new shunt technology, and barriers to progress. Finally, we will advance a research agenda that will provide evidence-based management recommendations.