Ex Utero Intrapartum Treatment for Prenatally Diagnosed Cervicofacial Lymphatic Malformations.

INTRODUCTION: Cervicofacial lymphatic malformations (cf-LM) may be identified on prenatal ultrasound, prompting consideration of ex utero intrapartum treatment (EXIT) to secure the fetal airway. Furthermore, the recent shift in postnatal management of cf-LM from resection alone toward a multimodal approach including sirolimus and sclerotherapy may impact the neonatal outcomes of cf-LM. This study aims to characterize the neonatal outcomes of patients with prenatally diagnosed cf-LM who underwent EXIT-to-airway. METHODS: Retrospective, single-center review of all patients who underwent EXIT-to-airway for cf-LM (2011-2020) was performed. Demographics, prenatal imaging, intraoperative details, and outcomes were analyzed using descriptive statistics (median [interquartile range]). RESULTS: Six patients with prenatally diagnosed cf-LM underwent EXIT-to-airway at a median gestational age of 36 (33.8-36.9) wk. The median volume on fetal magnetic resonance imaging was 187.5 mL (142.3-237.8) and median tracheoesophageal displacement index was 11 mL (9.25-15). All were successfully intubated on placental support with a median duration of 25 (15.25-91) d. There was one fatality at day of life 10 due to necrotizing enterocolitis totalis. Among survivors, 2 of 5 underwent tracheostomy placement, 4 of 5 underwent gastrostomy tubes placement, and all 5 received sirolimus at day of life of 9 [8-10] d. Four patients underwent debulking or excision of their cf-LM during the initial hospitalization. Patients had a median length of stay of 68 (45-129) d. One patient experi enced a pneumothorax with evidence of barotrauma following EXIT-to-airway requiring chest tube placement (duration 8 d). CONCLUSIONS: EXIT-to-airway procedure remains a feasible strategy for mitigating neonatal hypoxia in cases of prenatally diagnosed cervicofacial lymphatic malformations. However, postnatal outcomes are variable with potential long-term aerodigestive sequelae.

Characteristics and Outcomes of Infants in Texas by Facility Children's Surgery Verification Status.

INTRODUCTION: The American College of Surgeons Children's Surgery Verification (CSV) status recognizes hospitals that deliver high-quality pediatric surgical care. Texas has 5 CSV centers in three cities, which may limit equitable access to care. We explored the characteristics and outcomes of infants admitted in Texas as a function of facility CSV status. MATERIALS AND METHODS: We retrospectively reviewed a state-wide hospital discharge database (2013-2021). All patients <1 y of age were included. Patients transferred to an outside hospital were excluded to avoid double counting. Descriptive statistics and chi-square analysis were performed. RESULTS: We analyzed 3,617,173 admissions, with 211,278 (6%) treated at CSV centers. CSV admissions were less likely to be inborn (46% versus 93%) and more likely to be transfers (16% versus 1%). CSV centers also had sicker patients (32% versus 13% extreme illness severity) with higher mortality rates (1% versus 0%), longer length of stay (9 ± 22 versus 4 ± 9), and higher operative rates (33% versus 20%). However, mortality was lower at CSV centers when matched for illness severity. Proportionately more patients from rural counties (9% versus 4%) and counties along the United States-Mexico border (13% versus 1%), as well as patients of Hispanic ethnicity (39% versus 33%), were treated at non-CSV centers. Meanwhile, proportionately more African Americans (21% versus 11%) were treated at CSV centers. CONCLUSIONS: CSV centers are associated with improved outcomes among patients with high illness severity. Population differences among patients treated at CSV centers compared to non-CSV centers may represent disparities in access to care and warrant further evaluation.

Association of Prenatal Congenital Lung Malformation Volume Ratio to Postnatal Computed Tomography Characteristics.

INTRODUCTION: Congenital lung malformations (CLMs) are diverse and readily diagnosed on prenatal ultrasound. Postnatal computed tomography (CT) characteristics, including volume, are used in centers for the clinical decision-making of asymptomatic CLM. We aim to evaluate the relationship of prenatal CLM volume ratio (CVR) to postnatal CT characteristics by suspected prenatal diagnosis and postnatal radiological diagnosis. METHODS: We performed a single-center retrospective review of all patients evaluated with prenatally diagnosed CLM (May 2015-December 2022). Demographics, prenatal imaging findings at initial evaluation, and postnatal radiological diagnosis/imaging findings were analyzed with descriptive statistics. Pairwise correlation coefficient tests were performed to analyze the correlation between prenatal CVR and postnatal CT lesion size stratified by diagnosis. RESULTS: Of 123 patients referred and evaluated, suspected prenatal diagnoses consisted of 68 bronchial atresia (BA), 20 intralobar bronchopulmonary sequestration (iBPS), 20 extralobar bronchopulmonary sequestration (eBPS), and nine congenital pulmonary airway malformation (CPAM). Postnatal radiological diagnoses consisted of 53 BA, 22 iBPS, 14 eBPS, and 20 CPAM. Overall correlation coefficient of prenatal CVR to postnatal CT lesion size volume was 0.56. By suspected prenatal diagnosis, correlation coefficients were 0.61 (BA), 0.59 (iBPS), 0.29 (eBPS), and 0.51 (CPAM). For postnatal radiological diagnosis, correlation coefficients were 0.58 (BA), 0.56 (iBPS), 0.33 (eBPS), and 0.62 (CPAM). CONCLUSIONS: Our results indicate that initial CVR is overall consistent with the postnatal CT lesion size. This correlation is present in suspected prenatal diagnoses of BA and iBPS and postnatal radiological diagnoses of BA, iBPS, and CPAM. Additional studies analyzing long-term follow-up should be conducted to specify the safety of patients who undergo observation rather than surgical intervention.

Prenatal Prognosis of Omphalocele Using Magnetic Resonance Imaging Measurement of Fetal Lung Volumes.

BACKGROUND: Omphalocele is a congenital midline abdominal wall defect resulting in herniation of viscera into a membrane-covered sac. Pulmonary complications, including pulmonary hypoplasia, pulmonary hypertension, and prolonged respiratory support are a leading cause of neonatal morbidity and mortality. OBJECTIVE(S): This study aimed to assess the role of fetal MRI-derived lung volumes and omphalocele defect size as clinical tools to prognosticate postnatal pulmonary morbidity and neonatal mortality in those with a prenatally diagnosed omphalocele (PDO). STUDY DESIGN: This was a retrospective cohort study of all pregnancies with PDO at our fetal center from 2007-2023. Pregnancies with aneuploidy or concurrent life-limiting fetal anomalies were excluded. Using fetal MRI, observed-to-expected total fetal lung volume (O/E TLV) ratios were determined by a previously published method. The transverse diameter of the abdominal defect was also measured. The O/E TLV ratios and abdominal defect measurements were compared with postnatal outcomes. The primary outcome was death at any time. Secondary outcomes included death in the first 30 days of life or before discharge from birth hospitalization, the requirement of respiratory support with intubation and mechanical ventilation, or development of pulmonary hypertension. RESULTS: Of 101 pregnancies with a PDO, 54 pregnancies (53.5%) with prenatally diagnosed omphalocele met inclusion criteria. There was a significant increase in the rate of death when compared between the three O/E TLV classifications: 1/36 (2.8%) in the O/E≥50% group, 3/14 (21.4%) in the O/E 25%-49.9% group, and 4/4 (100%) in the O/E<25% group (P<.001). The rate of intubation increased with the severity of O/E TLV classification, with 27.8% in the O/E≥50% group, 64.3% in the O/E 25%-49.9% group, and 100% in the O/E<25% group (P=.003). The rate of pulmonary hypertension was also higher in the O/E 25%-49.9% (50.0%) and the O/E<25% (50.0%) groups compared to the O/E≥50% group (8.3%, P=.002). There was no association between the transverse diameter of the abdominal wall defect and the primary outcome of death (OR=1.08 95% CI=[0.65-1.78], P=.77). CONCLUSIONS: In our cohort of patients with PDO, O/E TLV<50% is associated with death, need for intubation, prolonged intubation, and pulmonary hypertension. In contrast, omphalocele size demonstrated no prognostic value for these outcomes. The strong association between low fetal lung volume on MRI and poor neonatal outcomes highlights the utility of fetal MRI for estimating postnatal prognosis. Clinicians can utilize fetal lung volumes to direct perinatal counseling and optimize the plan of care.

Extracorporeal Membrane Oxygenation Before 34 Wks' Gestation: A Single-Center Experience.

INTRODUCTION: Traditionally, gestational age <34 wk and weight <2 kg are considered relative contraindications to extracorporeal membrane oxygenation (ECMO). There is a paucity of information that explains the outcomes in this unique population of premature neonates. The purpose of this study is to examine outcomes of patients who undergo ECMO at <34 wk at a single institution. METHODS: A single-center retrospective review was performed for neonates managed with ECMO in the neonatal intensive care unit from January 2012 to April 2022. Characteristics and outcome data were collected. The primary outcome studied was survival at discharge. Secondary outcomes were intraventricular hemorrhage, ischemic brain injury, and thrombosis. Data were analyzed with descriptive statistics. RESULTS: Following exclusion, 107 patients were included with eight having initiating ECMO at <34 wk. Three (38%) patients, who received ECMO at <34 wk, incurred intraventricular hemorrhages compared to 14 (14%) in the ≥34-wk cohort. Two (25%), who underwent ECMO at <34 wk, exhibited signs of brain ischemia on imaging compared to 9 (9%) in those ≥34 wk, and 3 (38%) patients <34 wk experienced thrombosis compared to 31 (31%) in the ≥34-wk cohort. Five (63%) of those in the <34-wk cohort survived to discharge, similar to 61 (61%) in the ≥34 wk cohort. CONCLUSIONS: Our data suggest that EGA <34 wk may not be a contraindication for ECMO, with appropriate counseling of potential risks.

Racial Inequalities in the Initial Management of Congenital Diaphragmatic Hernia in the Houston Area.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is readily prenatally diagnosed and associated with significant perinatal morbidity and mortality. Delivery at facilities with adequate resources for neonatal resuscitation, such as Children's Surgery Verification (CSV) centers, is recommended; however, disparities have been clinically noted. We aimed to characterize locoregional care of CDH and the impact of race and ethnicity. METHODS: We conducted a population-based study using the Texas Inpatient Public Use Data File to identify infants <1 y-old with CDH based on international classification of diseases 9/10 codes (2013-2021). Only initial birth admissions in the Houston region were included. Data was analyzed using descriptive statistics and chi-squared analysis. RESULTS: We identified 257 newborns with CDH. While births were noted across 29 facilities, the majority were at the 2 CSV centers in Houston. There was no significant difference in illness severity, prematurity or insurance status by race. Black and 'other' patients were less likely to deliver at CSV facilities (Black 32% versus 'Other' 48% versus White 70% versus Asian 81%; P < 0.01), receive ECMO (Black 6% versus 'Other' 5% versus White 19% versus Asian 29%; P < 0.01) or undergo a CDH repair (Black 26% versus 'Other' 33% versus White 51% versus Asian 71%; P < 0.01) on their index admission and had lower average daily costs (Black $10,292 [$3219-25,021] versus 'Other' $9106 [$3617-15,672] versus White $12,906 [$9038-18,550] versus Asian $12,896 [$7469-23,817]; P < 0.05). Additionally, black and 'other' patients were more likely to be transferred (Black 23% versus 'Other' 28% versus White 12% versus Asian 14%; P < 0.05). None of the patients born at CSV centers transferred. CONCLUSIONS: Most Houston-born patients deliver at high-resource centers; however, Black and 'other' patients are less likely to deliver at CSV centers and more likely to require transfer during the critical neonatal period. This suggests a vulnerable population which may benefit from targeted intervention to improve prenatal care and delivery planning.

Characteristics and Outcomes of Patients With Spina Bifida in Texas by Patient Age.

INTRODUCTION: Spina bifida (SB) occurs in 3.5/10,000 live births and is associated with significant long-term neurologic and urologic morbidity. We explored the characteristics and outcomes of pediatric patients with SB and the facilities that treat them in Texas. METHODS: We retrospectively reviewed a statewide hospital inpatient discharge database (2013-2021) to identify patients aged <18 y with SB using International Classification of Diseases 9/10 codes. Patients transferred to outside hospitals were excluded to avoid double-counting. Descriptive statistics and chi-square test were performed. RESULTS: Seven thousand five hundred thirty one inpatient hospitalizations with SB were analyzed. Most SB care is provided by a few facilities. Two facilities (1%) averaged >100 SB admissions per year (33% of patients), while 15 facilities (8%) treat 10-100 patients per year (51% of patients). Most facilities (145/193, 75%) average less than one patient per year. Infants tended to be sicker (17% extreme illness severity, P < 0.001). Overall mortality is low (1%), primarily occurring in the neonatal period (8%, P < 0.001). Most admissions are associated with surgical intervention, with 63% of encounters having operating room charges with an average cost of $25,786 ± 24,884. Admissions for spinal procedures were more common among infants, whereas admissions for genitourinary procedures were more common among older patients (P < 0.001). The average length of stay was 8 ± 16 d with infants having the longest length of stay (19 ± 33, P < 0.001). CONCLUSIONS: Patients have significant long-term health needs with evolving pediatric surgical indications as they grow. Pediatric SB care is primarily provided by a small number of facilities in Texas. Longitudinal care coordination of their multidisciplinary surgical care is needed to optimize patient care.

Treatment Facility Case Volume and Disparities in Outcomes of Congenital Diaphragmatic Hernia Cases.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is a life-threatening, prenatally diagnosed congenital anomaly. We aim to characterize care and outcomes of infants with CDH in Texas and the impact of treating facilities volume of care. METHODS: Retrospective cohort study using a state-wide Hospital Inpatient Discharge Public Use Data File was conducted (2013-2021). Neonates and infants <1 year of age were included using CDH ICD-9/ICD-10 codes. Neonates transferred to an outside hospital were excluded to avoid double-counting. Descriptive statistics, chi-square and logistic regression analysis were performed. RESULTS: Of 1314 CDH patient encounters identified, 728 (55%) occurred at 5 higher volume centers (HVC, >75 cases), 326 (25%) at 9 mid-volume centers (MVC, 20-75 cases) and 268 (20%) at 79 low volume centers (LVC, <20 cases). HVC had lower mortality rates (18%, MVC 22% vs LVC 27%; p = 0.011) despite treating sicker patients (extreme illness severity: HVC 71%, MVC 62% vs LVC 50%; p < 0.001) with longer length-of-stay (p < 0.001). Extracorporeal membrane oxygenation was used in 136 (10%) and provided primarily at HVC. LVC treated proportionately more non-white Hispanic patients (p < 0.001) and patients from counties along the Mexican border (p < 0.001). The predicted probability of mortality in CDH patients decreases with higher treatment facility CDH case volume, with a 0.5% decrease in the odds of mortality for every additional CDH case treated (p < 0.001). CONCLUSIONS: Patients treated in HVC have significantly lower mortality despite increased severity. Our data suggest minority populations may be disproportionately treated at LVC associated with worse outcomes. TYPE OF STUDY: Retrospective Prognosis Study. LEVEL OF EVIDENCE: Level II.

Surfactant Therapy in Congenital Diaphragmatic Hernia and Fetoscopic Endoluminal Tracheal Occlusion.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is a complex pathology with severe pulmonary morbidity. Administration of surfactant in CDH is controversial, and the advent of fetoscopic endoluminal tracheal occlusion (FETO) has added further complexity. While FETO has been shown to improve survival outcomes, there are risks of prematurity and potential surfactant deficiency. We aim to evaluate the characteristics and outcomes of surfactant administration for CDH infants and elucidate potential benefits or risks in this unique population. METHODS: A single-center retrospective cohort review of patients with unilateral CDH from September 2015 to July 2022 was performed. Demographics, prognostic perinatal imaging features, and outcomes were collected. Patients were stratified by surfactant administration and history of FETO. Data were analyzed with descriptive statistics, two-sample t-tests, chi-squared analyses, and logistic regression. RESULTS: Of 105 included patients, 19 (18%) underwent FETO and 25 (24%) received surfactant. Overall, surfactant recipients were born at earlier gestational ages and lower birthweights regardless of FETO history. Surfactant recipients possessed significantly worse prenatal prognostic features such as observed to expected total fetal lung volume, observed to expected lung to head ratio, and percent liver herniation. In CDH patients without FETO history, surfactant recipients demonstrated worse outcomes than nonrecipients. This association is notably absent in the FETO population, where surfactant recipients have more favorable survival and comparable outcomes. When controlling for defect severity or surfactant usage, as a proxy for respiratory status, surfactant recipients that underwent FETO trended toward improved survival and decreased ECMO use. CONCLUSIONS: Surfactant administration is not associated with increased morbidity and mortality and may be beneficial in CDH patients that have undergone FETO.

Hospital Variation in Mortality and Failure to Rescue after Surgery for High-Risk Neonatal Diagnoses.

INTRODUCTION: A large proportion of postoperative mortality after pediatric surgery occurs among neonates with specific high-risk diagnoses. The extent to which there is hospital-level mortality variation among patients with these diagnoses and whether this variation is associated with differences in failure to rescue (FTR) is unclear. METHODS: The Pediatric Health Information System® database (2012-2020) was used to identify patients who underwent surgery for eight high-risk neonatal diagnoses: gastroschisis; volvulus; necrotizing enterocolitis; intestinal atresia; meconium peritonitis; tracheoesophageal fistula; congenital diaphragmatic hernia; and perinatal intestinal perforation. Hospitals were stratified into tertiles of reliability-adjusted inpatient mortality rates (lower than average mortality - tertile 1 [T1]; higher than average mortality - tertile 3 [T3]). Multivariable hierarchical regression was used to evaluate the association between hospital-level, reliability-adjusted mortality and FTR. RESULTS: Overall, 20,838 infants were identified across 48 academic, pediatric hospitals. Adjusted hospital mortality rates ranged from 4.0% (95% CI, 0.0-8.2) to 16.3% (12.2-20.4). Median case volume (range, 80-1,238) and number of NICU beds (range, 24-126) were not significantly different across hospital tertiles. Compared to the hospitals with the lowest postoperative mortality (T1), the odds of FTR were significantly higher in hospitals with the highest (T3) postoperative mortality (odds ratio 1.97 [1.50-2.59]). CONCLUSIONS: Significant variation in neonatal hospital mortality for high-risk diagnoses does not appear to be explained by hospital structural characteristics. Rather, difference in FTR suggests quality improvement interventions targeting early recognition and management of postoperative complications could improve surgical quality and safety for high-risk neonatal care.

Management and Outcomes of Patients With High-Risk (Congenital Lung Malformation Volume Ratio≥ 1.6) Congenital Lung Malformations.

INTRODUCTION: Congenital lung malformations (CLMs) have a variable natural history. Larger lesions with CLM volume ratio (CVR) ≥ 1.6 are associated with hydrops and fetal mortality. The purpose of this study is to describe the management and outcomes of high-risk (CVR ≥ 1.6) CLM patients. METHODS: A retrospective cohort study was performed for all fetuses evaluated between May 2015 and May 2022. Demographics, prenatal imaging factors, prenatal and postnatal treatment, and outcomes were collected. Descriptive statistics were used to compare the cohorts. RESULTS: Of 149 fetal CLM patients referred to our fetal center, 21/149 (14%) had CVR ≥ 1.6. One CLM patient had intrauterine fetal demise, and 2 patients were lost to follow-up. Of the remaining 18 patients, 11/18 (67%) received maternal steroids. Seven out of 18 patients (39%) underwent resection at the time of delivery with 1/7 (14%) undergoing exutero intrapartum treatment (EXIT)-to-resection, 5/7 (71%) undergoing EXIT-to-exteriorization-to-resection, and 1/7 (14%) undergoing a coordinated delivery to resection; among those undergoing resection, there were 2 fatalities (28.5%). Seven out of 18 (39%) patients required urgent neonatal open lobectomies, and the remaining 4/18 (22%) patients underwent elective thoracoscopic lobectomies with no mortality. CONCLUSIONS: The natural history and outcomes of severe CLM patients remain highly variable. The EXIT-to-exteriorization-to-resection procedure may be a safe and effective approach for a subset of CLM patients with persistent symptoms of mass effect and severe mediastinal shift due to the observed decreased operative time requiring placental support observed in our study.

Novel Clinical Algorithm for Prenatal Monitoring of Congenital Lung Malformations.

INTRODUCTION: Congenital lung malformations (CLMs) are readily identified early in pregnancy with a variable natural history. Monitoring for lesion size and mediastinal shift (MS) is recommended following diagnosis. The purpose of this study is to propose a risk-stratified clinical algorithm for prenatal monitoring of CLM. METHODS: After ethical approval, all fetuses with CLMs evaluated at our fetal center from January 2015 to June 2022 were retrospectively reviewed. Patient demographics, imaging characteristics, and fetal interventions were collected. Lesions were stratified by congenital lung malformation volume ratio (CVR) and the presence of MS. Descriptive statistics and receiver operating characteristic curves were employed in the analysis. RESULTS: We analyzed 111 patients with a mean of 23.4 wk gestational age, a median CVR of 0.5 (interquartile range, 0.3-1.2), and MS in 76 of 111(68%) patients on initial evaluation. Among low-risk patients (CVR ≤1.1), 96% remained low-risk on final evaluation. No patients transitioned from low to high risk during the growth period. Patients with CVR >1.1 often had persistent MS (P < 0.001). Hydrops (5/111, 5%) and fetal intervention (4/111, 4%) only occurred in patients with CVR >1.1 (P < 0.001, P = 0.002) and MS (P = 0.144, P = 0.214). On receiver operating characteristic curve analysis, initial CVR >1.1 had 100% sensitivity and negative predictive value for hydrops and fetal intervention. CONCLUSIONS: CLMs with initial CVR ≤1.1 are low risk for hydrops and fetal intervention. We propose a risk-stratified algorithm for the monitoring of CLM during the growth period based on CVR. While our experience suggests that patients with CLM and MS are at higher risk, the current subjective assessment of MS is not adequately predictive. Incorporating an MS grading system may further refine risk stratification in the management of CLM.

Implementation and Assessment of a Laparotomy-Assisted Three-Port Fetoscopic Spina Bifida Repair Program.

Open spina bifida (OSB) is a congenital, non-lethal malformation with multifactorial etiology. Fetal therapy can be offered under certain conditions to parents after accurate prenatal diagnostic and interdisciplinary counseling. Since the advent of prenatal OSB surgery, various modifications of the original surgical techniques have evolved, including laparotomy-assisted fetoscopic repair. After a two-year preparation time, the team at the University of Giessen and Marburg (UKGM) became the first center to provide a three-port, three-layer fetoscopic repair of OSB via a laparotomy-assisted approach in the German-speaking area. We point out that under the guidance of experienced centers and by intensive multidisciplinary preparation and training, a previously described and applied technique could be transferred to a different setting.

Maternal Anxiety and Empowerment in Pregnancies Complicated By Fetal Surgical Anomalies: A Mixed Methods Study.

INTRODUCTION: Fetal surgical anomalies cause significant anxiety. Following the diagnosis, prenatal counseling with shared decision-making occurs. Empowerment is an essential component of shared decision-making. The purpose of this mixed-methods study was to evaluate the association between patient empowerment with depression and anxiety among patients with fetal surgical anomalies. METHODS: An explanatory mixed-methods study was conducted at a large tertiary fetal center among patients with recently diagnosed surgical fetal anomalies from May, 2021 to May, 2022. Validated cross-sectional surveys were used to collect quantitative data regarding patient empowerment, depression, and anxiety. Univariate analysis was used to compare the association of maternal empowerment with depression and anxiety. Qualitative data was obtained from semistructured interviews to explore maternal anxiety and depression relative to the fetal diagnosis. Thematic analysis was performed to identify themes. RESULTS: Seventy-four patients were recruited for the quantitative study. Pregnancy-related empowerment score and patient empowerment score were significantly lower for expectant mothers with high anxiety (P < 0.01). Eighteen patients participated in qualitative interviews. Participants expressed significant anxiety related to their fetal diagnosis. Exacerbating stressors included social determinants, personal history of miscarriage, and changing family dynamics. CONCLUSION: Our results suggest there is an association between increased depression and anxiety with lower empowerment. These findings have important implications for prenatal counseling, as targeted interventions to improve psychosocial support to treat depression and anxiety might also improve empowerment.

Approach and Technique for Cesarean Section to Immediate Resection for High-Risk Sacrococcygeal Teratomas.

INTRODUCTION: Ex-utero intrapartum treatment has been established as an option for fetal and perinatal surgeons to deliver patients with sacrococcygeal teratomas (SCTs) which are causing significant fetal distress and possible in-utero fetal demise. However, ex-utero intrapartum treatment procedures carry significant maternal risk and morbidity. Herein, we report an alternative technique of Cesarean section to immediate resection (CSIR) for managing high-risk SCTs. METHODS: A retrospective institutional review board-approved review was performed on all SCTs evaluated at our fetal center from May 2014 to September 2020. Demographics; prenatal imaging characteristics; prenatal interventions; and postnatal surgery data including operative time, estimated blood loss, pathology, and outcomes were collected. Outcomes of interest included surveillance serum alpha-fetoprotein levels, imaging surveillance, developmental milestones, and the presence or absence of constipation or fecal incontinence. RESULTS: A total of 20 patients with prenatal diagnosis of SCT were evaluated. Mothers who transferred their care to another institution after diagnosis were excluded from this study. Twelve neonates underwent standard postnatal resection. Three neonates underwent emergent CSIR for high output cardiac failure, fetal anemia, or concerns for in-utero hemorrhagic rupture. The median (interquartile range) operative time was 231.5 (113) minutes for the standard operative group versus 156 min in the CSIR group. We present three patients who underwent immediate resection after emergent Cesarean section. We report 100% survival for the three consecutive cases. CONCLUSIONS: CSIR is a safe and feasible approach for managing appropriately selected high-risk SCTs with signs of hydrops, fetal distress, or fetal anemia. Despite patient prematurity, we demonstrated 100% survival of three consecutive cases. We suggest that CSIR be considered an option in the management algorithm for high-risk SCTs.

Fetal malrotation with midgut volvulus: Prenatal diagnosis and planning.

Introduction: Malrotation of the intestinal tract is a congenital malformation commonly found either incidentally or after affected individuals develop signs and symptoms of intestinal obstruction. Malrotation is prone to midgut volvulus that can cause intestinal obstruction and lead to ischemia and necrosis requiring emergent surgical intervention. Rare instances of in utero midgut volvulus have been reported in the literature and carry a high mortality given the difficulty in establishing a diagnosis prior to development of signs of intestinal ischemia and necrosis. Advancements in imaging have made it possible to diagnose in utero malrotation earlier, raising the question of optimal timing of delivery, especially in cases of prenatally diagnosed midgut volvulus. In these cases, the risks of premature birth must be weighed against the risks of fetal intestinal ischemia and potential fetal demise. Case presentation: This case report details an interesting presentation of intestinal malrotation with suspected midgut volvulus found on prenatal imaging at 33 weeks and 4 days' gestation. This prompted delivery of the infant at 34 weeks and 2 days' gestation with urgent operative management, within 3 hours of life, after diagnosis was confirmed postnatally. Intraoperatively, the infant was confirmed to have midgut volvulus without bowel ischemia, the intestines were reduced, and a Ladd procedure was performed without incident. The infant recovered postoperatively without complication, tolerated advancement to full volume feeds and was discharged on day of life 18. Conclusion: Successful management of fetal malrotation with midgut volvulus may be accomplished by early access to a multi-disciplinary team of professionals, prompt postnatal confirmation of diagnosis, and urgent correction to minimize the risk of complications.

Characterization of Suboptimal Responses to Fetoscopic Endoluminal Tracheal Occlusion in Congenital Diaphragmatic Hernia.

INTRODUCTION: The aim of the study was to characterize the changes in fetal lung volume following fetoscopic endoluminal tracheal occlusion (FETO) that are associated with infant survival and need for extracorporeal membrane oxygenation (ECMO) in congenital diaphragmatic hernia (CDH). METHODS: Fetuses with CDH who underwent FETO at a single institution were included. CDH cases were reclassified by MRI metrics [observed-to-expected total lung volume (O/E TLV) and percent liver herniation]. The percent changes of MRI metrics after FETO were calculated. ROC-derived cutoffs of these changes were derived to predict infant survival to discharge. Regression analyses were done to determine the association between these cutoffs with infant survival and ECMO need, adjusted for site of CDH, gestational age at delivery, fetal sex, and CDH severity. RESULTS: Thirty CDH cases were included. ROC analysis demonstrated that post-FETO increases in O/E TLV had an area under the curve of 0.74 (p = 0.035) for the prediction of survival to hospital discharge; a cutoff of less than 10% was selected. Fetuses with a <10% post-FETO increase in O/E TLV had lower survival to hospital discharge [44.8% vs. 91.7%; p = 0.018] and higher ECMO use [61.1% vs. 16.7%; p = 0.026] compared to those with an O/E TLV increase ≥10%. Similar results were observed when the analyses were restricted to left-sided CDH cases. A post-FETO <10% increase in O/E TLV was independently associated with lower survival at hospital discharge (aOR: 0.073, 95% CI: 0.008-0.689; p = 0.022) and at 12 months of age (aOR: 0.091, 95% CI: 0.01-0.825; p = 0.036) as well as with higher ECMO use (aOR: 7.88, 95% CI: 1.31-47.04; p = 0.024). CONCLUSION: Fetuses with less than 10% increase in O/E TLV following the FETO procedure are at increased risk for requiring ECMO and for death in the postnatal period when adjusted for gestational age at delivery, CDH severity, and other confounders.

Association of Socioeconomic Status with Empowerment, Depression, and Anxiety in Pregnancies Complicated by Fetal Surgical Anomalies.

BACKGROUND: Women with pregnancies complicated by fetal surgical anomalies experience significant psychosocial burden. There remains a need to determine the impact that socioeconomic status has on maternal empowerment, anxiety, and depression. METHODS: A survey study was conducted from 5/2021-5/2022 to quantify empowerment, anxiety, and depression in women with fetal surgical anomalies. Surveys administered included Pregnancy Related Empowerment Scale (PRES), Patient Empowerment Scale (PES), State Trait Anxiety Inventory (STAI), and Depression Anxiety and Stress Scale (DASS). Two-sample t-test was used to compare survey scores across socioeconomic groups. RESULTS: Seventy-four patients were recruited. Mothers more commonly preferred English as primary language (n = 61, 82%) and were non-Hispanic (n = 43, 58%). Lower empowerment scores were observed in Hispanic mothers (PRES, p = 0.03; PES, p = 0.04) and mothers who preferred Spanish (PRES, p = 0.04; PES, p = 0.06) as primary language. Both non-Hispanic (p = 0.88) and English speaking (p = 0.75) women had higher STAI scores, but neither was significantly different. DASS was not significantly higher for Hispanic (p = 0.79) or Spanish speaking mothers (p = 0.47). CONCLUSION: Hispanic and Spanish speaking women with pregnancies complicated by fetal surgical anomalies have significantly decreased empowerment scores. These findings suggest a need for development of culturally competent, targeted interventions to improve maternal empowerment in this high-risk population. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Cross-Sectional Survey Study.

Surgical Management of Congenital Lung Malformations.

Congenital lung malformations (CLMs) are commonly diagnosed prenatal lesions with varied natural history. Prenatal diagnosis and monitoring help to guide fetal interventions, delivery planning, and need for urgent perinatal surgical interventions. All prenatally diagnosed CLMs should be evaluated postnatally, typically with cross-sectional imaging, because many lesions persist despite the appearance of complete 'regression' in utero. Management of CLMs in asymptomatic infants weighs the surgical and anesthetic risk of prophylactic resection against the risk of expectant management, including the possibility of infection, malignant degeneration, and more complicated surgical resection later with loss of compensatory lung growth.