Ex Utero Intrapartum Treatment Research Articles

Early detection and management of congenital pulmonary airway malformation in a newborn with stable clinical course

Introduction: Congenital pulmonary airway malformation (CPAM) is a rare developmental lung anomaly characterized by cystic lung lesions, constituting about 25% of congenital pulmonary anomalies. It typically occurs sporadically and is not significantly associated with race, age, or other maternal factors. The prenatal course of CPAM can vary based on factors such as lesion size, mediastinal shift, and associated anomalies. While the overall prognosis is generally favorable in the absence of severe complications like hydrops fetalis, which can adversely affect outcomes, management strategies include corticosteroids, thoracoamniotic shunt (TAS), and, in severe cases, open fetal surgery or the EXIT procedure. Postnatal surgical excision usually offers a curative outcome with an excellent prognosis. Without surgical intervention, there are risks of recurrent infections and, rarely, malignant transformation. Effective prenatal detection significantly impacts clinical decision-making and neonatal outcomes, and the necessity and timing of surgery for asymptomatic infants remain subjects of ongoing debate, underscoring the need for personalized, multidisciplinary care. Case Report: A female infant, delivered at 38 weeks via Cesarean section, was diagnosed with CPAM following routine prenatal ultrasound. Presence of extensive cystic regions in the right lung confirmed the diagnosis and a CPAM volume ratio (CVR) of 0.37 indicated a low risk of severe complications. The pregnancy was largely uneventful, with maternal chronic hypertension managed by Nifedipine and mild intermittent asthma. Postnatal chest radiography corroborated the CPAM diagnosis, while an abdominal ultrasound investigated intrahepatic calcifications. Maternal screening for cytomegalovirus and toxoplasmosis yielded negative results, and postnatally, the infant also tested negative for both infections. The neonate, though asymptomatic and stable, required close monitoring in the neonatal intensive care unit to prevent potential respiratory compromise. Conclusion: Advances in prenatal imaging have greatly improved the early detection and management of CPAM, allowing for targeted neonatal care and strategic planning. This case highlights the importance of a multidisciplinary approach involving obstetricians, maternal-fetal medicine specialists, neonatologists, and pediatric thoracic surgeons in the effective management of CPAM. Early identification and diligent postnatal monitoring are essential for ensuring optimal outcomes and minimizing the risk of complications. The coordinated care and strategic decision-making exemplified in this case underscore the potential for enhanced neonatal health and long-term well-being through comprehensive management of congenital anomalies.

Fetal Teratomas: Advances in Diagnosis and Management.

Fetal teratomas, though rare, represent a significant proportion of tumors arising during fetal development. These tumors arise from pluripotent cells and can present in varying degrees of severity, ranging from incidental findings to life-threatening conditions. Prenatal imaging, via ultrasound and MRI, is necessary for diagnosis and risk assessment. The management of fetal teratomas, particularly those associated with complications like hydrops or airway obstruction, often requires a multidisciplinary approach. Interventions such as ex-utero intrapartum treatment (EXIT) procedures and minimally invasive alternatives have emerged as critical tools to improve neonatal outcomes in severe cases. Despite advances in fetal therapies, careful prenatal monitoring and individualized management remain essential, especially for tumors with high vascularity or those that risk compromising cardiac output. This review explores the diagnostic methods, management strategies, and outcomes associated with fetal teratomas, highlighting recent advancements that contribute to improving survival and reducing morbidity in affected neonates.

Fetal Cystic Lymphatic Malformations: Systematic Review on Pregnancy and Neonatal Outcomes.

Evaluate pregnancy and neonatal outcomes with fetal cystic lymphatic malformations (LMs), excluding those arising from the posterior neck, to facilitate patient counseling. A systematic review was performed in accordance with PRISMA guidance. Case series and case reports published between 2000 and 2022 were included. Sixty-five studies (96 fetuses) met the inclusion criteria. The average gestational age at diagnosis was 25.5 weeks with the commonest location being the anterior neck (28%). All patients were diagnosed with LM using two-dimensional (2D) ultrasound. Prenatal progression in LM size, presence of intralesional bleeding, or fetal hydrops occurred in 70% (41/59), 9% (5/59), and 3% (2/59), respectively. Chromosomal and structural abnormalities were reported in 4% (2/52) and 2% (2/96), respectively. Overall livebirth rate was 94% (79/84); 12/96 resulted in termination and 5/84 in in utero demise. The average gestational age of delivery was 37.7 weeks. Exactly 19% (15/79) had a vaginal birth, of which shoulder dystocia occurred in one infant. Ex utero intrapartum treatment (EXIT) procedure was performed in 13% (10/79). Postnatal treatment commonly involved surgical excision 38% (30/79), sclerotherapy in 21.5% (17/79), or combination of both in 11.4% (9/79). Of those with reported follow-up, 4 died within 1 year, 1 developed heart failure at 2 years of life, and the remaining 44 had normal developmental outcomes. Fetal cystic LMs, excluding those in the posterior neck, are not commonly associated with chromosomal, or additional structural abnormalities. They usually increase in size before delivery with only a minority developing complications. The good developmental outcome was reported in all survivors.

Is conversion the only itinerary to peace with "hostile" laparoscopic cholecystectomy?

Laparoscopic cholecystectomy (LC) remains the most common surgical procedure worldwide. With these unequivocal benefits, it is today the optimal standard of care for symptomatic cholelithiasis. However, this routine operation can also represent one of the most "hostile" situations. In scenarios of uncertain Critical View of Safety display, total cholecystectomy and laparoscopic approach incur a risk of serious complications such as iatrogenic bile duct injury (IBDI). In such situations, it is necessary to improvise an approach different from the traditional concepts used for elective cases and provide a rescue plan that promotes safety. Although the balance between maintaining LC and moving to an exit procedure is resolved, it remains a challenge for providers to navigate the best alternative strategy. The truth in this debate of seeking optimal results is that surgeons are inclined to resort to conversion, as the most usual itinerary. However, it is inconsistent to consider that the simple fact of switching to an open procedure necessarily protects against IBDI, as well as to approve the conversion as a single manifest alternative to cope without knowing the other saviors procedures and their profiles. Therefore, we found it very useful to compile research data regarding "hostile" CL bailout methods to optimize their use in our practice and to answer the question which strategy to choose? And why?

Anesthesia and Multidisciplinary Team Management throughout the Exit Procedure and Excision of a Huge Congenital Cervical Teratoma: A Case Report

Anesthesia and Multidisciplinary Team Management throughout the Exit Procedure and Excision of a Huge Congenital Cervical Teratoma: A Case Report

Prenatally Diagnosed Congenital High Airway Obstruction Syndrome: Perinatal Management and Outcome—A Single Tertiary Care Center Experience

AbstractTo report our experience with the management of prenatally diagnosed cases of congenital high airway obstruction syndrome (CHAOS) and the postnatal outcome of those who underwent an ex utero intrapartum treatment (EXIT) procedure. This is a single center, retrospective observational study of prenatally diagnosed CHAOS cases using two-dimensional ultrasound from December 2017 to December 2022 in a tertiary care facility. Of the total nine fetuses prenatally diagnosed with CHAOS, three (33.3%) were associated with multiple congenital anomalies, seven out of nine (77.8%) developed ascites, and one had fetal hydrops. Five (55.6%) underwent medical termination of pregnancy and two were lost to follow-up (22.2%). The remaining two continued pregnancy and required EXIT tracheostomy at the time of delivery (22.2%). Microarray was performed in both which was normal. Postnatally, both infants are tracheostomy dependent with one requiring frequent ventilator support. CHAOS even when isolated generally has poor prognosis without intervention. Performing an EXIT procedure at birth can significantly improve postnatal survival by minimizing hypoxic damage. However, the long-term medical and surgical challenges for survivors remain numerous especially speech disorders, even after lifesaving fetal intervention and surgical correction. Therefore, an accurate prenatal diagnosis is necessary to give the couple an option of continuing pregnancy after realistic counseling regarding the prognosis and postnatal outcome.

Management of fetal head and neck masses: Evaluation of prenatal factors associated with airway obstruction and decision for definitive airway and ex-utero intrapartum treatment at birth.

Fetal head and neck masses can result in critical airway obstruction. Our study aimed to evaluate prenatal factors associated with the decision for a definitive airway, including ex-utero intrapartum treatment (EXIT), at birth among at-risk fetuses. A single-institution retrospective review evaluated all fetal head and neck masses prenatally diagnosed from 2005 to 2023. The primary outcome was the decision for a definitive airway at birth, including intubation, tracheostomy, or EXIT. Thirty four patients were included, with 23 deliveries occurring at our institution. 8/23 (35%) patients received a definitive airway at birth, six underwent an EXIT procedure, and two required intubation only. Patients who received a definitive airway had higher rates of polyhydramnios (50% vs. 7%, p=0.03), tracheal narrowing on ultrasound (US) (50% vs. 0%, p=0.01), tracheal displacement on US (63% vs. 0%, p<0.01), abnormal fetal breathing on US (50% vs. 0%, p=0.01), tracheal narrowing or displacement on magnetic resonance imaging (MRI) (75% vs. 7%, p<0.01), and larger mass maximum diameter (7.9 vs. 4.3cm, p=0.02). In our series, 100% of patients with polyhydramnios, tracheal narrowing or displacement on either US or MRI, and abnormal fetal breathing on US received a definitive airway at birth. Prenatal findings of tracheal narrowing or displacement, polyhydramnios, and abnormal fetal breathing are strongly associated with the decision for a definitive airway at birth and warrant mobilization of appropriate resources.

Maternal anemia and red blood cell requirements in 72 women undergoing ex-utero intrapartum treatment (EXIT) procedure.

The ex-utero intrapartum treatment (EXIT) allows to ensure fetal airway while keeping uteroplacental circulation. However, EXIT may become a life-threatening procedure due to the increased risk of uterine atony or placenta abruption with increased peripartum blood losses and increased transfusion rates. We aim to review maternal anemia prevalence and transfusion requirements in women undergoing EXIT procedure. Using data from the Federal German Statistical Office hospitalized women undergoing EXIT procedure between January 1st 2006 and December 31st 2021 were included. The prevalence of anemia, peripartum hemorrhage, comorbidities and administration of red blood cells (RBC) were analyzed. In total, 72 women underwent EXIT procedure with a median age of 31 years (26;33.5). In 43.1% EXIT was conducted at 34-36 weeks of gestational age. "Anemia during pregnancy" was present in 47.2%, "anemia due to acute bleeding" in 25.0% and "iron deficiency anemia" in 15.3%. Postpartum hemorrhage occurred in 11.1%. RBCs were transfused in 15.3% of all women. Most women required 1-5 units of RBCs. Despite the rarity of this procedure, anemia management and blood conservation strategies in order to reduce the need for RBC transfusion are highly important in women undergoing EXIT procedure.

The silent storm: a case report of missed antenatal diagnosis leading to fatal respiratory distress and death in a rare case of congenital high airway obstruction syndrome

Congenital high airway obstruction syndrome in neonates is a rare, life-threatening condition characterized by upper airway obstruction. Typically diagnosed prenatally through advanced imaging, CHAOS results from anomalies such as laryngeal atresia or web or tracheal stenosis. Neonates with CHAOS face respiratory distress at birth, necessitating prompt intervention. Management often involves ex-utero intrapartum treatment procedure or tracheostomy to establish a secure airway. Timely diagnosis and multidisciplinary collaboration are crucial for optimizing outcomes in affected infants. Despite its rarity, CHAOS demands heightened clinical awareness to ensure swift, tailored interventions and improve the chances of neonatal survival. We report here a case of CHAOS which was not diagnosed on antenatal ultrasonography.

A Detailed Exploration of the Ex Utero Intrapartum Treatment Procedure with Center-Specific Advancements

The Ex Utero Intrapartum Treatment (EXIT) procedure has long been an invaluable tool in managing complex fetal conditions requiring airway interventions during the transition from intrauterine to extrauterine life. This technical note offers an in-depth examination of the EXIT procedure, emphasizing the refinements and innovations introduced at our center. The technique focuses on meticulous preoperative assessment and uses distinctive techniques and anesthetic methodologies. A multidisciplinary team assembles to plan the EXIT procedure, emphasizing patient communication and risk discussion. Our technique involves atraumatic access to the uterine cavity, achieved through the application of a uterine progressive distractor developed for this purpose. Following the use of this distractor, vascular clamps and a stapling device (Premium Poly Cs-57 Autosuture®, Medtronic) are employed. Our anesthetic approach employs general anesthesia with epidural catheter placement. Maternal operation involves low transverse laparotomy and intraoperative ultrasonography-guided hysterotomy. Fetal exposure includes gentle extraction or external version, ensuring airway access. After securing fetal airway access, umbilical cord clamping and maternal abdominal closure conclude the procedure. By revisiting the core principles of EXIT and incorporating center-specific advancements, we enhance our understanding and technical expertise. To our knowledge, this is the first time a detailed description of the technique has been published.

Case Report: Congenital diaphragmatic hernia in one twin followed by ex-utero intrapartum treatment (EXIT) procedure: Pearls &amp; pitfalls

Cases of congenital diaphragmatic hernia (CDH) in one twin have been reported rarely. This study aimed to report a rare case while also analyzing the pearls and pitfalls in diagnosing and managing this case. A 32-year-old multiparous woman referred to a tertiary hospital with multiple pregnancies and suspected CDH on the 1st baby. The patient underwent further investigation and the first fetus had a severe CDH on the left side. The second fetus was in a transverse lie and no other abnormalities were found. The babies were delivered through caesarean section and the first baby underwent the ex-utero intrapartum treatment (EXIT) procedure without prior antepartum fetal therapy. A large defect measuring 7.5 x 6 centimeters on the left side (Bochdalek type) was found in the first baby during surgery to make repairs. The baby died the next day due to some complications from the previous repair. As a pearl of this case, prenatal diagnosis and multidisciplinary approach hold pivotal roles. In prenatal imaging, CDH is distinguished by the visualization of thorax filled with abdominal contents and the mediastinal shifting from the defect to the contralateral side. EXIT procedures could be performed after the baby with CDH is delivered and the infant will still require post-natal surgical repair and its success rate depends on many factors. In conclusion, proper prenatal diagnosis with ultrasound evaluation from the second trimester, close and regular maternal-fetal monitoring, prenatal intervention, intrapartum ultrasound guidance, and post-natal procedures are all critical.

Feeding and Swallowing Trends in Infants Born via Exit Procedure for Giant Head and Neck Masses

Feeding and Swallowing Trends in Infants Born via Exit Procedure for Giant Head and Neck Masses

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

IntroductionCongenital 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. MethodsA 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. ResultsOf 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. ConclusionsThe 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.

Maternal and obstetric outcomes after Ex-Utero Intrapartum Treatment (EXIT): a single center experience

BackgroundThe Ex-utero Intrapartum Treatment (EXIT) is a procedure developed to manage a range of fetal conditions, aiming to ensure the maintenance of neonatal airway and preserving the feto-placental circulation. Its goal is to enhance the neonatal ability to successfully transition and adapt to postnatal life, thereby reducing perinatal morbidity and mortality. However, EXIT has been associated with a high risk of maternal complications. This paper provides an overview of the indications and characteristics of the EXIT procedure, as well as the obstetric outcomes and maternal complications.MethodsA retrospective analysis was conducted on a cohort of patients undergoing EXIT at our center between January 2007 and December 2022. Maternal outcomes, including demographic information, data related to the surgical procedure, surgical complications, and postoperative complications were analyzed. To assess the severity of the surgical complications, a modified Clavien-Dindo classification was used. Comparative analysis was performed by randomly selecting a sample from elective cesarean deliveries performed at our center.ResultsA total of 34 EXIT procedures were performed. According to the modified Clavien-Dindo classification, we observed no major complications, while minor maternal complications were present in 2.94% of cases. Compared to elective cesarean deliveries (n = 350), there were no significant differences in terms of maternal complications, highlighting the similarity observed in the mean decrease in postoperative hemoglobin (1.15 g/dL in EXIT vs. 1.2 g/dL in elective cesarean deliveries, p = 0.94). In EXIT group, there was a higher rate of polyhydramnios (26.47% vs 6.59%, p < 0.001), as well as the need for amnioreduction (14.71% vs 0%, p = 0.001) and preterm delivery (32.35% vs 6.02%, p = 0.001). There were no cases of endometritis, post-procedural fever, or abruptio placentae following EXIT.ConclusionsEXIT can be considered a safe procedure when performed under adequate conditions, including appropriate uterine access and proper anesthetic management. In our series, EXIT procedure was not associated with a higher incidence of maternal complications when compared to elective cesarean delivery.Trial registrationRetrospectively registered.

Outcome of Sclerotherapy in a Preterm Infant with a Giant Fetal Neck Lymphatic Malformation: A Case Report.

Lymphatic malformation (LM), most commonly present in the neck area, is benign vascular malformations of the lymphatic system. In an infant, however, LM poses a high risk of adverse outcomes. We present a case with a giant fetal LM. Through ultrasonography, at 23+ weeks of gestation, a septate cystic mass 7.2×6.5×6.3 cm in size was found on the right side of the fetus's neck. After extensive counseling by the multidisciplinary team, the parents chose to continue the pregnancy. Severe fetal tracheal compression was observed at 29 weeks by magnetic resonance imaging (MRI). At 31 weeks and 5 days, owing to suspected fetal distress, an emergency cesarean section was performed and a male baby weighing 1720 g was delivered. The mass was 10×16×8 cm in size and ex utero intrapartum treatment (EXIT) was implemented. Due to progressive growth of the mass secondary to intralesional bleeding, an intralesional injection of bleomycin was administered three days later. This injection was repeated at the age of 1 month and 8 days. The baby was followed up and, by a year after his birth, LM had disappeared. The baby has since been in good health. Accurate prenatal diagnosis and regular monitoring of a fetus with LM may improve prognosis. It is essential to have a trained multidisciplinary team to evaluate the condition of the fetus and the neonate and to provide treatment based on the evaluation. Our experience with intralesional bleomycin injection for the treatment of a giant fetal neck LM in a preterm infant had a favorable outcome. Long-term follow-up by a multidisciplinary team is needed in such cases.

Perinatal airway management in neonatal goiter: A healthcare cost and utilization project (HCUP) kids’ inpatient database analysis

IntroductionFetal goiter is a rare congenital disorder that can present with life-threatening neonatal airway obstruction. Lifesaving and function-preserving airway management strategies are available, but routine delivery affords a limited window for intervention. Accordingly, fetal goiter is reported among the most common indications for ex-utero intrapartum treatment (EXIT). While EXIT prolongs the window for airway intervention to benefit the neonate, it elevates the risk to the pregnant person and requires extensive resources; therefore, data to guide ideal treatment selection are essential. This study aims to compare perinatal airway interventions between individuals with a birth hospitalization discharge diagnosis (BHDD) of goiter and the general population. Materials and methodsIndividuals with and without BHDD of goiter were identified in the Healthcare Cost and Utilization Project (HCUP) Kids' Inpatient Database from 2000 to 2019. The frequency of airway interventions on day of life (DOL) 0 or 1 were compared using the Rao-Scott chi-square test. Additionally, gestational age, type of intervention, complications, mortality, birth weight, and length of stay were examined for the goiter cohort. ResultsTwo-hundred eighty-seven weighted cases of goiter were identified in the study period. The population was 61 % male, 55 % White, and median birthweight was 3.3 kg. The median length of stay was 4.3 days, and average total charges were $42,332. Airway intervention on DOL 0 or 1 was performed in 16.9 % of individuals with goiter compared to 1.6 % in neonates without goiter (p < 0.001). Interventions in the goiter cohort included endotracheal intubation in 16 % of cases, laryngoscopy/bronchoscopy in 1–5% of cases, and tracheostomy in <1 % of cases. Fewer than 1 % of individuals undergoing intubation additionally had mass decompression/resection on DOL 0 or 1. No neonates received extracorporeal membrane oxygenation cannulation or cardiopulmonary resuscitation. Hypoxic encephalopathy occurred in <1 % of cases, among which endotracheal intubation was the only airway intervention performed. There were no mortalities among neonates with goiter. ConclusionIndividuals with BHDD of goiter receive significantly higher rates of perinatal airway intervention. In most cases, endoscopic interventions alone were sufficient to avoid hypoxic neurological complications. These findings contribute to data to aid in clinical counseling and empower patients to make informed decisions according to their values and treatment goals.

Management of Congenital Cervical Teratoma with Application of EXIT Protocol - Case Report

Background: Congenital teratomas are relatively rare neoplasms, which occurs in about 1:20,000 to 1:80,000 births, and only 1.5% to 5% of which are neoplasm of the cervical. They can be diagnosed through ultrasound during pregnancy and, if not properly handled, have a high mortality rate. Airway compression is a secondary complication following mortality. Case report: A solid-cystic mass was identified in the anterior cervical region of a 30-week-old fetus during an ultrasound scan. EXIT (Ex-Utero Intrapartum Treatment)-to-airway procedure was performed by a multidisciplinary team composed of obstetricians, anesthesiologists, neonatologists and pediatric surgeons to remove the neoplasm. The procedure occurred upon delivery of the fetus, resulting in a positive outcome with neonatal survival. In this case, the fetus was in breech position, and, differently from the usual EXIT protocol, it had to be completely extracted before guaranteeing airway flow. Conclusion: Although congenital teratomas are a rare condition with complex treatment, it is possible to achieve a satisfactory outcome when adequate planning and protocol are established.

EXIT-to-airway for obstructing fetal neck masses: a singular methodology for monochorionic and dichorionic twin pregnancies

INTRODUCTION: Fetal airway obstruction in one twin of a diamniotic pregnancy presents unique challenges. Very few cases of Ex-Utero-Intrapartum-Treatment (EXIT) procedures for twin pregnancy have been reported and only in dichorionic pregnancies. We report a singular methodology for EXIT-to-airway procedures in two pregnancies involving monochorionic and dichorionic twins. CASE PRESENTATION: Two cases of EXIT-to-airway in twin pregnancies were performed in 2018-2019 at a regional fetal treatment center. Case 1 involved a giant cervical teratoma in a monochorionic-diamniotic twin pregnancy with preterm labor at 29 weeks. Case 2 involved a dichorionic-diamniotic pregnancy with a large cervical lymphatic malformation with preterm labor at 36 weeks. In each case, the polyhydramnios caused the affected twin’s amniotic sac to be the presenting sac for the surgical approach. Bronchoscopy and successful intubation was completed after 22 and 10 minutes of uteroplacental bypass, respectively. The bystander twins were delivered second without intubation and resuscitated without perinatal distress. CONCLUSION: EXIT-to-airway appears to be a reasonable option for twins including monochorionic pregnancies, via delivery of the affected twin first followed by delivery of the bystander twin. Thoughtful preparation and counseling by an experienced multidisciplinary team permits an EXIT-to-airway approach for twin pregnancies even in an emergent setting.

Prenatally Diagnosed Large Lung Lesions: Timing of Resection and Perinatal Outcomes

IntroductionFetuses with large lung lesions including congenital cystic adenomatoid malformations (CCAMs) are at risk for cardiopulmonary compromise. Prenatal maternal betamethasone and cyst drainage for micro- and macrocystic lesions respectively have improved outcomes yet some lesions remain large and require resection before birth (open fetal surgery, OFS), at delivery via an Ex Utero Intrapartum Treatment (EXIT), or immediately post cesarean section (section-to-resection, STR). We sought to compare prenatal characteristics and outcomes in fetuses undergoing OFS, EXIT, or STR to inform decision-making and prenatal counseling. MethodsA single institution retrospective review was conducted evaluating patients undergoing OFS, EXIT, or STR for prenatally diagnosed lung lesions from 2000 to 2021. Specimens were reviewed by an anatomic pathologist. Lesions were divided into “CCAMs” (the largest pathology group) and “all lung lesions” since pathologic diagnosis is not possible during prenatal evaluation when care decisions are made. Prenatal variables included initial, greatest, and final CCAM volume-ratio (CVR), betamethasone use/frequency, cyst drainage, and the presence of hydrops. Outcomes included survival, ECMO utilization, NICU length of stay (LOS), postnatal nitric oxide use, and ventilator days. ResultsSixty-nine percent (59 of 85 patients) of lung lesions undergoing resection were CCAMs. Among patients with pathologic diagnosis of CCAM, the initial, largest, and final CVRs were greatest in OFS followed by EXIT and STR patients. Similarly, the incidence of hydrops was significantly greater and the rate of hydrops resolution was lower in the OFS group. Although the rate of cyst drainage did not differ between groups, maternal betamethasone use varied significantly (OFS 60.0%, EXIT 100.0%, STR 74.3%; p = 0.0378). Notably, all OFS took place prior to 2014. There was no difference in survival, ventilator days, nitric oxide, NICU LOS, or ECMO between groups. In multiple variable logistic modeling, determinants of survival to NICU discharge among patients undergoing resection with a pathologic diagnosis of CCAM included initial CVR <3.5 and need for <3 maternal betamethasone doses. ConclusionFor CCAMs that remain large despite maternal betamethasone or cyst drainage, surgical resection via OFS, EXIT, or STR are viable options with favorable and comparable survival between groups. In the modern era there has been a shift from OFS and EXIT procedures to STR for fetuses with persistently large lung lesions. This shift has been fueled by the increased use of maternal betamethasone and introduction of a Special Delivery Unit during the study period and the appreciation of similar fetal and neonatal outcomes for STR vs. EXIT and OFS with reduced maternal morbidity associated with a STR. Accordingly, efforts to optimize multidisciplinary perinatal care for fetuses with large lung lesions are important to inform patient selection criteria and promote STR as the preferred surgical approach in the modern era. Level of EvidenceLevel IV.

Maternal and Neonatal Outcomes After Ex-Utero Intrapartum Treatment for Congenital Diaphragmatic Hernia: A Case Series.

Despite advances in neonatal intensive care, fetuses with congenital diaphragmatic hernia (CDH) remain to have a poor prognosis. Exclusive postnatal treatment is inadequate in patients with moderate CDH (observed than expected lung-to-head ratio [O/E LHR] 26-45%) and can lead to respiratory failure at birth, requiring extracorporeal membrane oxygenation in 75% of cases. An ex-utero intrapartum treatment (EXIT) procedure may be beneficial in these cases, improving the fetal-neonatal transition. We review all pregnancies with fetal isolated left CDH with moderate O/E LHR delivered by EXIT in our center from January 2007 to December 2022. Maternal and neonatal variables were analyzed. As primary outcomes, we included neonatal survival and mortality rates, surgical and infectious complications, uterine scar dehiscence, and blood loss during EXIT. As secondary outcomes, we studied recurrences of the diaphragmatic defect, long-term evolution, subsequent pregnancies, and mode of delivery. A total of 14 patients were delivered by the EXIT procedure, with a neonatal survival rate of 85.7%. All these children had optimal physical and neurocognitive development and no pulmonary morbidity. We found no major complications and 7.1% of minor maternal complications. There were no cases of surgical wound infection or endometritis. The median decrease in hemoglobin during the EXIT procedure was 1.9 mg/dL, and only one case required postoperative transfusion. Two out of the 14 women became pregnant again, and both pregnancies were uneventful. In our series, the EXIT procedure allows for adequate airway management associated with a high neonatal survival rate in patients with moderate O/E LHR CDH, with a low rate of neonatal and maternal complications.