Monochorionic Twins: TTTS, TAPS, and Selective Fetal Growth Restriction.

With an increasing incidence of twin gestations, understanding the inherent risks associated with these pregnancies is essential in modern obstetrics. The unique differences in placentation in monochorionic twins leads to unique complications, including twin-to-twin transfusion syndrome, the twin anemia-polycythemia sequence, and selective fetal growth restriction. Not only does the understanding of the monochorionic placenta lead to an understanding of the pathophysiology of the complications of monochorionic twins, but it also has led to the development of highly effective directed fetal therapy via fetoscopic laser coagulation used in twin-to-twin transfusion syndrome.

The Special Delivery Unit: A Novel Concept for Mother-Baby Continuity for Major Fetal Abnormalities.

OBJECTIVE: This article describes the experience in the planning and development of a special delivery unit (SDU) at our free-standing children's hospital in Austin, Texas. STUDY DESIGN: Description of various aspects of the development of the SDU. In addition, telephone surveys were obtained from five other institutions regarding the planning and current status of their SDUs. RESULTS: Since the advent of the SDU at Children's Hospital of Philadelphia in 2008, several free-standing children's hospitals have opened similar units at their institutions. Developing an obstetrical unit in a children's hospital can be a daunting task on many fronts. The costs of providing 24-hour obstetrical, nursing, and anesthesiology coverage must be considered. Although most SDUs are associated with a fetal center and fetal surgery/interventions, some units function exclusively for the delivery of pregnancies complicated by major fetal conditions where the neonate will require immediate surgical care or other interventions. CONCLUSION: Research on the cost-effectiveness and the effect of SDUs on clinical outcome, teaching, and patient satisfaction is warranted. KEY POINTS: · Specialized delivery units are becoming more common at free-standing children's hospitals.. · The primary aim of the SDU is to maintain mother-baby continuity in cases of congenital anomalies.. · Developing an obstetrical unit at a pediatric hospital is a daunting task..

Open and endoscopic fetal myelomeningocele surgeries display similar in-hospital safety profiles in a large, multi-institutional database.

BACKGROUND: Open intrauterine fetal myelomeningocele repair has demonstrated decreased ventriculoperitoneal shunting and improved motor outcomes despite maternal and fetal risks. Few data directly compare the safety of open vs endoscopic approaches. OBJECTIVE: This study aimed to analyze in-hospital maternal and fetal outcomes of pregnant patients undergoing open vs endoscopic fetal myelomeningocele repair using a large, multi-center database. STUDY DESIGN: This was a review of the Pediatric Health Information System database from October 1, 2015, to December 31, 2021. All patients who underwent open or endoscopic fetal myelomeningocele repair according to the International Classification of Diseases, Tenth Revision, were identified. Demographics, gestational age, and outcomes were analyzed. Descriptive and univariate statistics were used. RESULTS: A total of 378 pregnant patients underwent fetal myelomeningocele repair. The approach was endoscopic in 143 cases (37.8%) and open in 235 cases (62.2%). Overall postprocedural outcomes included no maternal in-hospital mortalities or intensive care unit admissions, a median length of stay of 4 days (interquartile range, 4-5), 14 cases (3.7%) of surgical and postoperative complications, 6 cases (1.6%) of intrauterine infections, 12 cases (3.2%) of obstetrical complications (including preterm premature rupture of membranes), 3 cases (0.8%) of intrauterine fetal demise, and 16 cases (4.2%) of preterm delivery. Compared with an open approach, the endoscopic approach occurred at a later gestational age (25 weeks [interquartile range, 24-25] vs 24 weeks [interquartile range, 24-25]; P<.001) and had an increased rate of intrauterine infection (6 [4.2%] cases vs 0 [0%] case; P=.002). There was no difference between approaches in the rates of surgical complications, obstetrical complications, intrauterine fetal demise, or preterm deliveries. CONCLUSION: Compared with an open approach, endoscopic fetal myelomeningocele repair displays a comparable rate of fetal complications, including intrauterine fetal demise and preterm delivery, and a similar in-hospital maternal safety profile despite an association with increased intrauterine infection.

Placental Anatomy and Function in Twin Gestations.

With an increasing incidence of twin gestations, understanding the inherent risks associated with these pregnancies is essential in modern obstetrics. The unique differences in placentation in twins contribute to the increased risks. Monochorionic twins are susceptible to complications because of their unique placental architecture, including twin-to-twin transfusion syndrome, the twin anemia-polycythemia sequence, selective intrauterine growth restriction, and the twin reversed arterial perfusion sequence. Knowing the clinical correlations of placental anatomy in these gestations helps perinatal pathologists perform a more informed placental evaluation, allowing for better care for the mother and her children.

Recipient umbilical artery elongation (redundancy) in twin-twin transfusion syndrome.

BACKGROUND: Chronic hypertension in adults causes arterial lengthening in major arteries, but the effects of early fetal hypertension on the twin-twin transfusion syndrome recipient's vascular architecture remains unknown. OBJECTIVE: We hypothesize that arterial cord redundancy is related to recipient hypertension and subsequent heart failure. Our objectives were to: (1) establish a 3-dimensional color Doppler ultrasound method of measuring umbilical arterial length relative to its corresponding venous segment in the umbilical cord using artery vein angle; (2) compare recipient artery vein angle to gestational age-matched controls; and (3) test the association of artery vein angle with recipient heart failure. STUDY DESIGN: We compared 3 groups prospectively: twin-twin transfusion syndrome pregnancies undergoing fetoscopic laser surgery (preoperatively) and 2 groups of gestational age-matched controls: uncomplicated monochorionic-diamniotic twin pregnancies and healthy singletons. Using a 3-dimensional color-Doppler volume image of 5 cm of cord near the placental insertion, we traced the umbilical artery and vein producing umbilical artery:vein length, (artery vein index) and measured the artery vein angle between umbilical artery and vein. Correlation of artery vein angle to twin-twin transfusion syndrome stage, maximum vertical pocket, umbilical arterial indices, ductus venosus Doppler, and brain natriuretic peptide were performed. We used pulsed-wave and tissue Doppler to measure tissue Doppler velocities and indexed cardiac output and correlated these with artery vein angle. Comparative statistics, including multivariable linear regression, examined the relationship between umbilical arterial Doppler indices and artery vein angle. RESULTS: Artery vein angle and artery vein index correlated significantly (R2, 0.86; P < .0001), hence, artery vein angle was used for analysis. Mean artery vein angle was 33.1 ± 31.5 degrees in recipients (n = 44), 9.5 ± 6 degrees in monochorionic-diamniotic (n = 11; 22 fetuses), and 8.9 ± 8.3 degrees in singleton controls (n = 16) (P < .001). An artery vein angle ≥26 degrees (>95th percentile for controls) was measured in 52% recipients. Artery vein angle was higher in twin-twin transfusion syndrome stage 3R vs 1 (P = .001). Artery vein angle increased with increasing umbilical arterial pulsatility index (P < .001), and decreased with increasing resistance index (P = .02) after adjusting for gestational age. Interrater agreements to categorize abnormal artery vein angle values was 95% (P < .001). Abnormal ductus venosus Doppler and elevated recipient amniotic fluid N-terminal pro-brain natriuretic peptide/protein levels correlated significantly with artery vein angle. Abnormal artery vein angles were associated with decreased indexed cardiac output, lower tissue Doppler velocities, higher right-sided Tei indices, and severe tricuspid regurgitation. CONCLUSION: Umbilical arterial lengthening occurs in 52% of recipients and is associated with abnormal Doppler flows, low systolic tissue Doppler velocities, reduced cardiac output, and elevated markers of cardiac failure. This may reflect chronicity and severity of hypertension in the recipient fetus. Further research is needed to explore the mechanisms of elongation and long-term implications.

Correlating Prenatal Imaging Findings of Fetal Ventriculomegaly with the Need for Surgical Intervention in the First 3 Months after Birth.

BACKGROUND/AIMS: This study evaluates the predictive value of prenatal imaging measurements regarding the need for cerebrospinal fluid (CSF) diversion for fetal hydrocephalus in the first 3 months after birth. METHODS: We retrospectively reviewed a consecutive case series of patients with fetal hydrocephalus from January 2011 to December 2014 (n = 45). Prenatal measurements included head circumference (HC), biparietal diameter (BPD), and lateral ventricle (LV) width. Patients requiring CSF diversion within 12 weeks of birth were compared to those who did not require CSF diversion using the Wilcoxon rank sum test, and receiver-operating characteristic analysis was used to evaluate threshold values. RESULTS: CSF diversion was required within 12 weeks of birth in 30 of 45 patients. Mean LV width (mm) during the entire pregnancy was greater for the surgery group than the nonsurgery group. Neither BPD nor HC showed differences between the groups. A mean LV size ≥15 mm predicted the need for CSF diversion with a sensitivity of 67% and specificity of 73%. CONCLUSION: LV width is the prenatal imaging measurement that best predicts the need for postnatal CSF diversion.

Survey of Parent Experiences in Prenatal Visits for Infants With Cleft Lip and Palate.

OBJECTIVE: The purpose of this study was to identify the factors that influence the parent's choice of cleft team/surgeon. DESIGN: A 10-question survey was used to elucidate factors that influenced parents in choosing their cleft surgeon. No identifiers of the origin of the study were used to improve parent objectivity. SETTING: The setting for this study was an online survey. PARTICIPANTS: Participants in this study were the parents of children who were born with cleft lip and/or palate. INTERVENTIONS: Parents were contacted anonymously via national, established social media websites that were independently run by the parents themselves. MAIN OUTCOME MEASURES: The main outcome measures were information regarding choice of cleft team/surgeon, source of patient referrals, and use of online media in decision making. RESULTS: A total of 112 responses were received. Of the parents, 77% sought prenatal evaluation with at least one cleft surgeon. Maternal-fetal medicine specialists were the most frequent (42%) referral source, followed by primary obstetricians (14%) and pediatricians (12%). The surgeon/cleft team's experience level and overall personality were ranked as the most important, whereas the least important was distance traveled. Of the parents, 95% used the Internet or social media for research prior to their prenatal visit; 96% of the parents found the prenatal visit helpful, and the most useful topics discussed were treatments (surgical, nonsurgical) and feeding techniques. CONCLUSION: This study identifies factors used to choose a cleft team/surgeon. Parents are more concerned with the experience level, reputation, and environment of the cleft team/surgeon than the distance traveled to get to the center.

Donor Death After Selective Fetoscopic Laser Surgery for Twin-Twin Transfusion Syndrome.

OBJECTIVE: To assess the incidence, timing, and risk factors for death of the donor fetus after fetoscopic laser surgery, we evaluated our cohort of patients who underwent the procedure for twin-twin transfusion syndrome. METHODS: This was a prospective cohort study of 166 consecutive patients with twin-twin transfusion syndrome at a single center. Fetal death was diagnosed by ultrasonography after surgery and before onset of labor. Risk factors for death of the donor twin were identified on univariate analysis and then subjected to multivariate, stepwise, logistic regression analysis. RESULTS: Donor demise occurred in 20 (13%) cases and recipient twin death occurred in four (2.6%). The median procedure to death interval was 4 days (range 1-89 days). Risk factors for donor death were fetal growth discordance greater than 30% (odds ratio [OR] 6.7, 95% confidence interval [CI] 2-23), reverse end-diastolic velocity in the donor umbilical artery (OR 25.0, 95% CI 2-290), a marginal and velamentous cord insertion (OR 4.4, 95% CI 1-19), and an increased number of anastomoses (OR 1.2, 95% CI 1.1-1.5). All four donors with both fetal growth discordance greater than 30% and reverse end-diastolic velocity in the donor umbilical artery resulted in a demise. CONCLUSION: Four risk factors significantly affecting acute and delayed donor demise after fetoscopic laser surgery were identified. The presence of both fetal growth discordance greater than 30% and reverse end-diastolic velocity in the donor umbilical artery was highly predictive of donor demise in our cohort. Knowledge of these risk factors can aid in counseling and assist patients in choosing the most appropriate intervention in the management of twin-twin transfusion syndrome. LEVEL OF EVIDENCE: III.

Twin reversed arterial perfusion sequence: a new method of parabiotic twin mass estimation correlated with pump twin compromise.

OBJECTIVES: The purpose of this study was to test the hypothesis that using the formula of a prolate ellipsoid to estimate parabiotic twin mass correlates better with findings of pump twin compromise than using the sonographic method of Moore et al (Am J Obstet Gynecol 1990; 163:907-912). METHODS: A 10-year retrospective review was performed to identify all cases of the suspected twin reversed arterial perfusion (TRAP) sequence. Parabiotic twin mass was estimated by summing body and extremity volumes calculated using the prolate ellipsoid formula (width × height × length × 0.523). Parabiotic twin mass was also estimated using the sonographic Moore method [1.21 × length(2) - (1.66 × length)]. Parabiotic twin mass estimated by both methods was correlated with sonographic findings associated with increased risk of pump twin compromise. RESULTS: Fifty-nine pregnancies complicated by TRAP were identified. Using the prolate ellipsoid formula, the parabiotic twin mean sizes ± SD (as a percentage of pump twin weight) were 103.0% ± 52.0% and 56.9% ± 44.3% in cases with and without pump twin compromise (P = .0005), respectively. Using the sonographic Moore method, the mean parabiotic twin sizes were 122.9% ± 54.3% and 99.6% ± 62.8% in cases with and without pump twin compromise (P = .14). The median estimated masses of the parabiotic twin were 197 ± 219 g using the prolate ellipsoid formula and 310 ± 212 g using the sonographic Moore method (P = .0001). A parabiotic twin size greater than 70% of the pump twin correlated with findings associated with increased risk of pump twin compromise when using the prolate ellipsoid formula (P = .002) but not the sonographic Moore method (P = .09). CONCLUSIONS: Sonographic findings associated with increased risk of pump twin compromise correlate better with prolate ellipsoid estimates of parabiotic twin mass. The median estimated mass of the parabiotic twin was statistically larger when calculated by the sonographic Moore method than by the prolate ellipsoid formula.

Fetoscopic laser therapy for twin-to-twin transfusion syndrome.

Twin-to-twin transfusion syndrome (TTTS) is a unique and complicated phenomenon occurring in 10-15% of monochorionic gestations. The chronic unbalanced distribution of blood volume across placental anastomoses between the donor and recipient fetuses leads to multisystem organ impairment including maladaptive changes in both fetuses. Fetoscopic selective laser photocoagulation (SLPC) is now established as the primary treatment modality for advanced stages of TTTS. SLPC is also associated with a risk reduction in fetal demise and long-term neurological impairment.

Use of magnetic resonance imaging in prenatal prognosis of the fetus with isolated left congenital diaphragmatic hernia.

OBJECTIVES: To investigate the prognostic value of magnetic resonance-calculated fetal lung volumes (FLV) in fetuses with isolated left congenital diaphragmatic hernia (L-CDH) who receive standardized prenatal and postnatal care at a single institution. MATERIALS AND METHODS: A retrospective review was undertaken to identify fetuses with isolated L-CDH between 2001 and 2010. RESULTS: Eighty-five cases of isolated L-CDH were identified. The overall survival was 65% (55/85). Survival was 45% if there was 'liver up' (23/51) and 94% if there was 'liver down' (32/34). Univariate statistical analysis showed that 'liver up' (p=0.001), lung-to-head ratio (LHR) at diagnosis (p=0.009), observed/expected (O/E) LHR (p=0.01), total FLV (p=0.03), right LV (p=0.04), magnetic resonance imaging (MRI) observed versus expected (O/E) FLV (p=0.002), intrathoracic versus intraabdominal stomach (p=0.002), percentage of herniated liver (p=0.004), and postnatal extracorporeal membrane oxygenation use (p=0.001) are predictive of postnatal survival. Multivariate analysis of only prenatal factors showed that the most important determinants of postnatal outcome are percentage of herniated liver, presence of liver up, and MRI O/E FLV. CONCLUSION: Herniated intrathoracic liver expressed as 'liver up' or as percent herniated liver and MRI measurement of FLV expressed as a O/E ratio are strong prenatal indicators of postnatal survival.

Ex utero intrapartum treatment in the management of giant cervical teratomas.

PURPOSE: The purpose of this study is to present the outcome and technical details of the Ex Utero Intrapartum Treatment (EXIT) procedure performed in the management of the fetus with a giant cervical teratoma. METHODS: A retrospective review of the medical records of patients undergoing the EXIT procedure between September 1995 and September 2010 was performed. RESULTS: Eighty-seven EXIT procedures were performed. In 20% of cases (17/87), the indication was giant cervical teratoma. There were 10 females and 7 males. Polyhydramnios was present in 82%. Median gestational age at EXIT was 35 weeks (range, 30-39 weeks). Median birth weight was 2.5 kg (range, 1.7-3.7 kg). Access to the airway under placental support was established in all cases via direct laryngoscopy/bronchoscopy in 8 patients (47%) and via surgical exploration (tracheostomy or retrograde intubation) in 9 patients (53%). The mortality rate under placental support was zero. Seven patients had the tumors resected immediately after the EXIT, 6 patients had the resection later, and 4 patients died before resection. The neonatal mortality rate was 23% (4/17 patients). Patients who died had severe pulmonary hypoplasia that resulted from the upward traction by the giant cervical mass on the airway and compression of the lungs against the thoracic apex. CONCLUSIONS: We conclude that the EXIT procedure continues to be the optimal delivery strategy for patients with prenatally diagnosed giant cervical teratomas and potential airway obstruction at birth. A thorough evaluation of the prenatal images and an experienced multidisciplinary team are key factors for an effective approach to the obstructed fetal airway.

Fetal MRI-calculated total lung volumes in the prediction of short-term outcome in giant omphalocele: preliminary findings.

OBJECTIVE: To determine the value of fetal MRI-calculated total lung volumes (TLV) in the prediction of short-term outcome in patients with giant omphalocele (GO). MATERIAL AND METHODS: We reviewed all cases of GO undergoing fetal MRI after 21 weeks' gestation and receiving postnatal care at our institution between 2003 and 2010. Observed/expected (O/E) TLV was calculated using age-matched TLV normograms [Radiology 2001;219:236-241]. Postnatal outcomes were stratified based on O/E TLV above or below 50% of expected. RESULTS: Seventeen GO cases fulfilled the entry criteria. The mean age at fetal MRI evaluation was 25.8 ± 4.8 weeks' gestation. The mean GO TLV (21.0 ± 13.2) was lower than age-matched population norms (p < 0.001), resulting in a mean O/E TLV of 52.3 ± 16.8%. The mean gestational age at delivery was 36.8 ± 1.6 weeks. Overall survival was 94%. Fourteen (88%) infants underwent staged reduction, and 2 underwent silver sulfadiazine treatment and delayed repair. Infants with ≤50% of predicted O/E TLV (n = 11, 65%) had lower Apgar scores at birth (p = 0.03), prolonged ventilatory support (p = 0.004), delayed oral intake (p = 0.03), and longer hospitalization (p = 0.03) compared to patients with ≥50% of expected O/E TLV. Two infants (both O/E TLV <50%) required tracheostomy placement. CONCLUSION: In the assessment of GO fetuses, MRI-based O/E TLV of <50% was predictive of increased postnatal morbidity.

Abnormal brain development and maturation on magnetic resonance imaging in survivors of severe congenital diaphragmatic hernia.

PURPOSE: The aim of the study was to evaluate the incidence of abnormal brain maturation in survivors of severe congenital diaphragmatic hernia (CDH). MATERIAL AND METHODS: Between July 2004 and December 2009, 50 CDH survivors underwent detailed brain magnetic resonance (MR) imaging before discharge. Magnetic resonance images were analyzed to evaluate the presence of structural brain abnormalities and to calculate overall brain maturation using the total maturation score (TMS). RESULTS: Thirty-two children (64%) underwent MR imaging between 39 and 43 weeks of gestation, allowing for evaluation of the TMS. Eighteen (36%) underwent MR imaging between 44 and 69 weeks of gestation, allowing for structural analysis of brain maturity only. The mean TMS was 14.1 ± 1.2 and significantly lower than reported age-matched normative data in infants without CDH (15.3 ± 1.0, P = .02). The TMS in 4 patients (12.5%) corresponded to a delay of 1 month in structural brain development. Eight infants (25%) demonstrated a 2-week delay. Periventricular leukomalacia was detected in 9 (18%), incomplete development of the opercula in 7 (14%), various degrees of intracranial hemorrhage in 24 (48%), and prominent extraaxial fluid spaces in 20 (40%) cases. CONCLUSIONS: Brain maturation in infants with severe CDH appears to be delayed. Long-term neurodevelopmental follow-up is needed to determine the significance of a lower-than-expected TMS and the presence of structural brain abnormalities on functional outcomes in this population.

Preschool neurobehavioral outcome following fetal myelomeningocele surgery.

OBJECTIVE: It was the aim of this study to assess the prevalence of preschool neurobehavioral problems in children following fetal myelomeningocele (fMMC) surgery. METHODS: Prior to the Management of Myelomeningocele Study, 30 fMMC patients underwent a standardized neurodevelopmental examination at 5 years of age. The prevalence of behavioral problems was assessed by the Child Behavior Check List (CBCL), which includes a total problem score and 2 broad-band indices (internalizing and externalizing problems). fMMC children were classified as having normal, at-risk or clinically significant scores based on calculated T scores. RESULTS: Twenty-two (73%) fMMC families completed the evaluation. Mean age at delivery following fMMC surgery was 35.5 ± 1.6 weeks. The prevalence of 'at-risk' or 'clinically significant' scores for internalizing, externalizing and total behavioral problems was similar to general population norms. No difference in overall internalizing (p = 0.19), externalizing (p = 0.54) and total behavioral (p = 0.18) scores was found between non-shunted and shunted fMMC children. However, shunted fMMC children were more likely to experience anxiety and depressive (p = 0.02), pervasive developmental (p = 0.03) and withdrawn (p = 0.06) behavior. Oppositional defiant, attention deficit and hyperactivity problems were not found. No correlation was found between overall neurodevelopmental outcome and abnormal CBCL scores. CONCLUSIONS: fMMC surgery and subsequent preterm delivery is not associated with increased behavioral problems, impaired social interactions and restricted behavior patterns. Shunted fMMC children were more likely to have scores in the 'at-risk' or 'clinically significant' range for multiple CBCL subindices. Early detection of behavioral problems following fMMC surgery provides an opportunity for targeted and timely intervention enabling affected fMMC children to be socially successful.

Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas.

BACKGROUND: Large, prenatally diagnosed sacrococcygeal teratomas (SCTs) present a formidable challenge because of their unpredictable growth and propensity for complications. In our experience, even with aggressive serial imaging, many fetuses have died under a policy of "watchful waiting." We propose "early delivery" as the best option for selected cases of high-risk fetal SCT. METHODS: The medical charts of all fetuses with SCT followed up at our institution and delivered before 32 weeks of gestation were reviewed for radiologic findings, fetal interventions, delivery information, perinatal inpatient course, and autopsy or discharge report. RESULTS: Between 1996 and 2009, excluding those that underwent fetal surgery, 9 patients with fetal SCT were delivered before 32 weeks of gestation. Four had type I tumors, and 5 had type II tumors. Of the 9 fetuses, 4 survived the neonatal period. The only surviving patient delivered before 28 weeks underwent an ex utero intrapartum therapy procedure. CONCLUSIONS: A significant number of pregnancies complicated by high-risk SCT will manifest signs of fetal or maternal decompensation, or both, between 27 and 32 weeks of gestation. In the absence of fulminant hydrops, preemptive early delivery can be associated with surprisingly good outcomes in appropriately selected fetuses with high-risk SCT.