BACKGROUND: Fetal thoracoamniotic shunts are common lifesaving interventions but frequently require replacement. Needle fetal thoracoscopy is a technique that uses standard thoracoamniotic shunt introducer sheaths to permit direct visualization and even instrument manipulation during shunt deployment to facilitate optimal positioning and primary shunt function in the most challenging cases.
METHODS: In this study, 5 patients who underwent needle fetal thoracoscopy-assisted thoracoamniotic shunt placement were reviewed. Three patients with large, macrocystic congenital pulmonary airway malformations (CPAMs) with evidence of worsening mediastinal shift and/or hydrops and 2 patients with large chylothorax with fetal hydrops were treated. Four cases had previous shunts that failed due to poor sonographic visualization during initial placement, cyst septations, shunt obstruction, or dislodgment. Needle fetal thoracoscopy was used to disrupt cyst walls and septations, clear hematoma, and confirm the optimal initial position of the shunt. In this series, 1 severe CPAM patient with a short cervix developed preterm labor postoperatively resulting in neonatal demise. The remaining 4 patients experienced resolution of hydrops and progressed to successful delivery with excellent neonatal outcomes.
CONCLUSIONS: Needle fetal thoracoscopy is a procedure that may be selectively deployed in challenging thoracoamniotic shunt cases impacted by recurrent failure, poor sonographic windows, and challenging fetal positioning.

A thoracoamniotic shunt was placed in a fetus affected by a right congenital diaphragmatic hernia (RCDH) complicated by voluminous nonimmune hydrops (NIH) at 30 weeks of gestation. The fetus showed congestive cardiac failure with a combined cardiac output (CCO) of 460.7 ml/min (Z-score: -1.2). After seven days, no edema, ascites, or pleural effusion was present. CCO increased significantly, reaching a Z-score of -0.2, as well as right and left cardiac output (Z-scores: -0.3 and -0.8, respectively). Two weeks later, the cardiac function and the ascites got worse despite the correct shunt placement, suggesting a possible occlusion. At 33 weeks, a C-section was performed due to labor in breech presentation. Despite the intensive care provided, the newborn died due to pulmonary hypertension and respiratory insufficiency. The thoracoamniotic shunt\'s effect on fetal circulation and the mechanisms of NIH in the event of RCDH are still unclear. Due to the high mortality rate of this condition and its poorer outcomes compared to left-sided defects, shunting cannot be considered an efficient attempt to improve fetal and neonatal survival rates to date. A close relationship between the amount of lymphatic effacement and cardiac function is clear, but further studies are needed to provide more information about this severe condition and its treatment.

OBJECTIVE: The objective of this study was to assess the predictive performance of preoperative cervical length (CL) for delivery within 1 week after pleuroamniotic shunting (PAS) in fetuses with severe hydrothorax.
METHODS: A prospective cohort of fetuses with severe hydrothorax referred to our fetal surgery center in Querétaro, Mexico from January 2012 to July 2020. Severe fetal hydrothorax was diagnosed as an accumulation of fluid within the fetal pleural space accompanied with severe bilateral lung compression, mediastinal shift, polyhydramnios, and/or hydrops. Transvaginal CL was measured immediately before PAS, and a short cervix was defined as that <25 mm. The interval from fetal intervention to delivery, prevalence of preterm prelabor rupture of membranes (PPROMs), and associations with delivery within the first week after PAS according to a short or a normal CL, were evaluated.
RESULTS: Thirty-five pregnancies with severe fetal hydrothorax treated with PAS were evaluated. Median gestational age at PAS was (weeks + days) 31+2 (range, 26+0-36+1). Two (5.7%) and 7 (20.0%) cases delivered within the first 24 h and 1 week after PAS, respectively. Ten (28.6%) women had a short cervix before PAS, while 25 (71.4%) had normal preoperative CL. Women with a short cervix showed lower mean interval between fetal intervention and delivery (2.4 vs. 5.5 weeks, p = 0.01), and higher prevalence of PPROM (50 vs. 12%, p = 0.01), as compared to women with a nonshort cervix. Preoperative short cervix was associated with significantly higher risk of delivery within the first 24 h (20.0 vs. 0%, respectively, p < 0.05) and 1 week after PAS (50.0 vs. 8.0%, respectively, p < 0.01) compared with pregnancies with normal preoperative CL.
CONCLUSIONS: In pregnancies with severe fetal hydrothorax candidates for pleuroamniotic shunt, identification of a short cervix before fetal intervention can predict delivery within 1 week after the surgical procedure.

Congenital pleural effusion is a rare condition with an incidence of approximately one per 15,000 pregnancies. The development of secondary hydrops is a poor prognostic indicator and such cases can be managed with a thoracoamniotic shunt (TAS). Our objective is to describe postnatal outcomes in survivors after TAS placement for congenital pleural effusions.
A retrospective study of all cases with fetal pleural effusions treated between 2006 and 2016. Patients with dominant unilateral or bilateral pleural effusions complicated by secondary hydrops fetalis received TAS placement. The results are reported as median (range).
A total of 29 patients with pleural effusion with secondary hydrops underwent TAS placement. The gestational age at the initial TAS placement was 27.6 (20.3-36.9) wk. Before delivery, hydrops resolved in 17 (58.6%) patients. The delivery gestational age was 35.7 (25.4-41.0) wk and the overall survival rate was 72.4%. Among the 21 survivors, 19 (90.5%) required admission to the neonatal intensive care unit for 15 (5-64) d. All 21 survivors had postnatal resolution of the pleural effusions. All 21 children were long-term survivors, with a median age of survivorship of 3 y 3 mo (9 mo-7 y 6 mo) at the time of last reported follow-up.
Thoracoamniotic shunting in fetuses with a dominant pleural effusion(s) and secondary hydrops resulted in a 72% survival rate. Nearly all survivors required admission to the neonatal intensive care unit. However, a majority did not have significant long-term morbidity.

Congenital chylothorax (CC) can have devastating consequences for neonates. We sought to determine the outcomes of cases treated at our institution and evaluate the role of fetal intervention.
With Institutional Review Board approval, patients treated at our institution 09/2006-04/2016 with CC were reviewed. History and outcomes were compared between patients undergoing fetal intervention (fetal group) and patients who did not (control group).
Twenty-three patients were identified. Mean gestational age at birth was 35 wk. Overall mortality was 30% (7 patients). Nineteen patients (83%) were prenatally diagnosed, and 10 patients (43%) underwent fetal intervention. Birth weight was significantly lower in the fetal group compared to the control group (median interquartile range [IQR]; 2.5 [2.3-3.0] versus 3.3 [2.6-3.7] kg, P = 0.02). Apgar scores were significantly higher in the fetal group than the control group at 1 and 5 min (median [IQR]; 6 [4-8] versus 1 [1-2], P = 0.005 and 8 [7-9] versus 2 [2-6], P = 0.008, respectively). For those patients with prenatal diagnosis of CC and hydrops fetalis, thrombosis and lymphopenia were both improved in the fetal group (thrombosis 0% versus 40%, P = 0.03; lymphocyte nadir [median {IQR}] 1.5 [0.6-2.9] versus 0.1 [0.05-0.2], P = 0.02). Duration of support with mechanical ventilation was significantly shorter in the fetal group (median [IQR]; 1 [0-40] versus 41 [29-75] d, P = 0.04).
Fetal intervention for CC is associated with improved Apgar scores and decreased ventilator days and complications in patients with hydrops fetalis. Fetuses with chylothorax, especially those with hydrops, should be referred to a fetal center for evaluation.

We report a case of a fetus diagnosed at 28 weeks\' gestation with a spontaneous prenatal hemothorax. Fetal intervention consisted of 2 thoracenteses with analysis of the pleural effusion. The pregnancy was further complicated by recurrence of the hemothorax, with subsequent mediastinal shift, hydrops, and nonreassuring antenatal testing requiring delivery at 31 weeks\' gestation. Postnatal workup established the diagnoses of cutis marmorata telangiectatica congenita (CMTC) and pulmonary lymphangiectasia. The child is currently 4 years old and without any active medical issues or sequelae from the CMTC, pulmonary lymphangiectasia, or prenatal interventions.

BACKGROUND: The aim of this study is to evaluate long-term neurodevelopmental and respiratory outcome after fetal therapy for fetal pleural effusion, congenital cystic adenomatoid malformation, and bronchopulmonary sequestration.
METHODS: Children ≥18 months of age underwent an assessment of neurologic, motor, and cognitive development. Medical records were reviewed to determine respiratory outcome. Behavioral outcome was assessed using the Child Behavioral Checklist.
RESULTS: Between 2001 and 2016, 63 fetuses with fetal hydrops secondary to thoracic abnormalities were treated at our center. Overall perinatal survival was 64% (40/63). Twenty-six children were included for follow-up (median age 55 months). Severe neurodevelopmental impairment (NDI) was detected in 15% (4/26). Three out of 4 children with severe NDI had associated causes contributing to the impairment. Overall adverse outcome, including perinatal mortality or NDI, was 55% (27/49). Fifteen percent (4/26) had severe respiratory sequelae. Parents did not report more behavioral problems than Dutch norms.
CONCLUSIONS: Our results suggest that severe NDI in this specific high-risk cohort occurs in 15%, which is above the range of the incidence of NDI reported in case series treated with other fetal therapies (5-10%). Large multicenter studies and an international web-based registry are warranted to prospectively gather outcome data at fixed time points.

OBJECTIVE: The prenatal natural history of intralobar and extralobar bronchopulmonary sequestrations (BPSs), including lesion growth patterns and need for prenatal intervention, have not been fully characterized. We review our series of BPSs to determine their natural history and outcomes in the context of the need for prenatal intervention.
METHODS: A retrospective review of the pre/postnatal course of 103 fetuses with an intralobar (n=44) or extralobar BPS (n=59) managed at a single institution between 2008 and 2015 was performed. Outcomes included prenatal lesion growth trajectory, presence of hydrops, need for prenatal intervention, survival, and postnatal surgical management.
RESULTS: Most extralobar (71%) and intralobar BPSs (94%) decreased in size or became isoechoic from initial to final evaluation. Peak lesion size occurred at 26-28weeks gestation. Eight fetuses developed hydrothorax, four of which (all extralobar BPSs) also developed hydrops. All four hydropic fetuses received maternal betamethasone, and three hydropic fetuses underwent thoracentesis and/or thoracoamniotic shunt placement with subsequent hydrops resolution. All fetuses survived. Forty-one intralobar (93%) and 35 extralobar BPSs (59%) were resected after birth.
CONCLUSIONS: BPSs tend to decrease in size after 26-28weeks gestation and rarely require fetal intervention. Lesions resulting in hydrothorax ± hydrops can be effectively managed with maternal steroids and/or drainage of the hydrothorax.
METHODS: IV.

Primary hydrothorax is a congenital anomaly affecting 1 in 10,000-15,000 pregnancies. The natural history of this condition is variable with some fetuses having spontaneous resolution and others showing progression. The associated pulmonary hypoplasia leads to increased perinatal morbidity and mortality. Optimal prenatal intervention remains controversial.
After obtaining the Institutional Review Board approval, a retrospective review of all patients evaluated for a fetal pleural effusion in the Fetal Diagnosis and Treatment Center at The University of Michigan, between 2006 and 2016 was performed. Cases with secondary etiologies for an effusion or when families decided to pursue elective termination were excluded.
Pleural effusions were identified in 175 patients. Primary hydrothorax was diagnosed in 15 patients (8%). The effusions were bilateral in 13/15 cases (86%) and 10/15 (66%) had hydrops at presentation. All 15 patients with primary hydrothorax underwent prenatal intervention. Thoracentesis was performed in 14/15 cases (93%). Shunt placement was performed in 10/15 cases (66%). Shunt migration was seen in four patients (40%) and all of these underwent prenatal shunt replacement. Overall survival was 76%. The rates of prematurity and preterm premature rupture of membranes were 69% and 35%, respectively.
Fetal intervention for the treatment of primary hydrothorax is effective, and it appears to confer a survival advantage. Both the fetuses and the mothers tolerated the procedures well. Preterm labor and preterm premature rupture of membranes remain an unsolved problem. Further studies are needed to understand the mechanisms behind the development of fetal hydrothorax.

OBJECTIVE: To evaluate the short-term neonatal outcome after fetal thoracoamniotic shunt placement for isolated hydrothorax.
METHODS: Retrospective evaluation of infants with isolated hydrothorax treated with thoracoamniotic shunt placement at our fetal therapy centre between 2001 and 2016.
RESULTS: In total 48 fetuses were treated with a thoracoamniotic shunt. All fetuses had signs of hydrops at the time of intervention. Median (IQR) gestational age at shunting was 28.7 (24.4-31.3) weeks. Forty-one of 48 (85%) fetuses were born alive at a median (IQR) gestational age of 34.4 (31.1-36.7) weeks. In one child the course of disease after birth was unknown (this child was excluded from further analyses). After birth, 24/40 (60%) children had signs of pleural effusion and 12/40 (30%) needed a thoracic shunt for continuous pleural drainage. Twenty-one (53%) children required mechanical ventilation, of whom 13 (33%) needed high-frequency ventilation as rescue therapy. Overall 30/40 (75%) infants survived the neonatal period. Neonatal survival rate was significantly higher when infants were born ≥32 weeks\' gestation as compared with <32 weeks: 93% (26/28) versus 33% (4/12), p<0.01.
CONCLUSIONS: Postnatal course of hydropic fetuses treated with thoracoamniotic shunt for isolated hydrothorax is often complicated by respiratory failure and persistent pleural effusions. Neonatal survival is good provided delivery occurs at or after 32 weeks\' gestation.