Fetal cardiac teratoma and pericardiocentesis: A case report.

Fetal pericardial teratomas are rare. They present with pericardial effusion and hydrops. The definitive management is postnatal resection of the tumor. The exact antenatal management is not known due to its rarity. We present a case of fetal pericardial teratoma with pericardial tamponade. Pericardiocentesis performed at 31 weeks significantly relieved the venous compression, leading to resolution of hydrops and prolonging the gestational age for the definitive management.

Resection of an immature intrapericardial teratoma from a premature neonate presenting as hydrops foetalis.

Intrapericardial teratoma is a germ-cell tumor that typically arises from the base of the heart. This rare cardiac tumour is the second most common tumor diagnosed in fetuses and newborn. Although benign, it can be massive in size causing direct compression on the heart and associated with significant pericardial effusion resulting life-threatening complications such as cardiac tamponade, heart failure, foetal hydrops, and sudden death. Early antenatal diagnosis and surgical intervention improve the survival. We present a case of immature intrapericardial teratoma diagnosed at 25 weeks of gestation but required multiple foetal pericardiocentesis and premature delivery due to massive pericardial effusion. The importance of multidisciplinary team approach to ensure successful management was highlighted in this case report.

In utero Treatment of Congenital High Airway Obstruction Syndrome via Fetal Laryngoscopy and EXIT Procedure.

INTRODUCTION: Congenital high airway obstruction syndrome (CHAOS) is a rare condition that can progress to fetal hydrops and demise in utero or at birth unless interventions are undertaken to alleviate the tracheal obstruction. While the ex-utero intrapartum treatment (EXIT) procedure for airway stabilization is technically feasible, abnormal pulmonary development as a result of the antenatal obstructive process may result in severe postnatal respiratory complications. CASE PRESENTATION: We describe a case of CHAOS with secondary hydrops treated in utero at 24 0/7 weeks' gestation by fetoscopic tracheal decompression via laser perforation of the airway obstruction. Interval imaging after the fetoscopic operation demonstrated resolution of the fetal hydrops. Tracheostomy for airway stabilization was performed at the time of the EXIT procedure near term (36 0/7 weeks). The patient underwent tracheal reconstruction and decannulation at 3 years of life. DISCUSSION/CONCLUSION: The primary goal of fetoscopic airway evaluation and intervention is not necessarily to perform definitive stabilization of the airway but rather to achieve sufficient decompression of the trachea to reverse fetal hydrops and salvage pulmonary development. In utero fetoscopic treatment may allow for prolongation of the pregnancy with delivery at or near term via EXIT procedure for definitive neonatal airway stabilization.

Innovative Fetal Therapy for a Giant Congenital Pulmonary Airway Malformation with Hydrops.

INTRODUCTION: Congenital pulmonary airway malformations (CPAMs) complicated by hydrops portend significant morbidity and mortality, with fetal survival estimates less than 10%. CASE PRESENTATION: We report successful use of ultrasound-guided radiofrequency ablation at 21-week gestation in a hydropic fetus with CPAM, with subsequent resolution of hydrops. Thirty-two-week MRI noted persistent mediastinal shift, and US at 36 weeks and 5 days noted polyhydramnios. Maternal gestational hypertension prompted delivery at 37 weeks, with a cesarean section performed after a failed trial of labor. The infant required CPAP at 100% and weaned to 21%. Tachypnea persisted, and chest CT on day of life 2 demonstrated multiple large cysts in the right lower lobe with anterior pneumothorax. On day of life 3, she successfully underwent a thoracoscopic right lower lobectomy. Adhesions to the chest wall and rib abnormalities were noted. She was extubated to CPAP at the conclusion of the procedure. She was able to wean to 21% on POD2 and transitioned to oral feeds. Her chest tube was removed with resultant ex vacuo pneumothorax noted. She remained asymptomatic and was discharged home on room air POD11. Pathology confirmed a type 1 CPAM. CONCLUSION: In utero radiofrequency ablation may be an adjunct to the management of large CPAM.

Intrauterine Ultrasound-Guided Laser Coagulation as a First Step for Treatment of Prenatally Complicated Bronchopulmonary Sequestration: Our Experience and Literature Review.

INTRODUCTION: Prenatal ultrasound-guided laser coagulation (USLC) for complicated bronchopulmonary sequestrations has been described but a consensus on the procedure and on the following management is still lacking. We present our experience and provide a literature review. METHODS: Retrospective review of patients treated in our center. Literature review and combined analysis of perinatal data were performed. RESULTS: Five cases were treated at our center, all presenting with severe hydrothorax. Four met the criteria for fetal hydrops. Four cases underwent postnatal computed tomography (CT) scan: in one case, there was no evidence of persistent bronchopulmonary sequestration. The other three underwent thoracoscopic resection, in two, a viable sequestration was found. Including our series, 57 cases have been reported, with no mortality and a success rate of 94.7%. Mean gestational age (GA) at the procedure was 28 ± 3.4 weeks and mean GA at birth and birth weight (BW) were 38.6 ± 2.3 weeks and 3,276 ± 519.8 g, respectively. In 80.6% of the cases investigated postnatally, a residual mass was found, 50% of cases who showed prenatal arterial flow cessation had a persistent sequestration postnatally, and 26.3% of cases underwent postnatal sequestrectomy. Both patients in our series had pathology examination confirming a viable bronchopulmonary sequestration. CONCLUSION: Prenatal USLC seems to be a valid option for bronchopulmonary sequestration complicated by severe hydrothorax and/or fetal hydrops. Authors believe that this procedure should aim to reverse fetal distress and allow pregnancy continuation, and it should not be considered a definitive treatment. The currently available data do not support changes of the common postnatal management.

The Value of Exome Sequencing in Thoracoamniotic Shunt for Severe Pleural Effusion with Fetal Hydrops: A Retrospective Clinical Study.

OBJECTIVE: We aimed to study the value of exome sequencing (ES) in severe pleural effusion with nonimmune hydrops fetalis (NIHF) that underwent thoracoamniotic shunt (TAS). METHODS: It was a retrospective study of NIHF that underwent TAS between 2012 and 2020 at Shanghai First Maternity and Infant Hospital. After a detailed assessment, NIHF cases with aneuploidies, infections, and structural anomalies were excluded, and TAS was offered to cases with severe pleural effusion. Quantitative fluorescence polymerase chain reaction (QF-PCR) was conducted to exclude Trisomy 21, 18, and 13 before fetal therapy, and chromosomal microarray analysis (CMA) was offered to all the cases. Before 2019, ES was retrospectively performed using stored fetal DNA extracted from prenatal samples; from 2019 onward, ES was discussed and offered before intrauterine therapies. RESULTS: A total of 18 NIHF cases underwent TAS with negative CMA and continuing pregnancy were included. Fetal hydrops was relieved in 16 cases (88.9%). The median gestational ages at intervention and at delivery were 31.2 (22.0-33.1) weeks and 34.3 (29.7-38.6) weeks, respectively. The neonatal survival rate was 72.2% (13/18), and no causative gene variants were identified from ES in any survivors. Pathogenic or likely pathogenic variants were detected in 3 out of 5 neonatal deaths. If rapid ES could have been available to guide fetal therapy, the neonatal survival rate after TAS would have increased from 72.2% to 86.7%. CONCLUSIONS: Single-gene disorders were one of the major causes of perinatal death in NIHF cases that underwent fetal therapy. Prenatal rapid ES may be of good promise in NIHF to explore precise etiology and guide fetal therapy.

Fetal aortic valvuloplasty may rescue fetuses with critical aortic stenosis and hydrops.

OBJECTIVE: Critical aortic stenosis (CAS) with a restrictive interatrial septum may lead to fetal congestive heart failure and hydrops, usually culminating in fetal demise if left untreated. The aim of this study was to assess the effects of fetal aortic valvuloplasty (FAV) on hemodynamics and outcome in these patients. METHODS: This was a retrospective review of fetuses with CAS and signs of hydrops that underwent FAV in our center between 2000 and 2020. Echocardiograms and patients' charts were analyzed for ventricular and valvular dimensions and for outcome. RESULTS: Hydrops was present at the time of intervention in 15 fetuses with CAS that underwent FAV at our center during the study period. All but one patient had at least one technically successful procedure. There were no procedure-related deaths, but three intrauterine deaths occurred. Twelve subjects were liveborn, of whom two died within 24 h after birth owing to persistent hydrops. Ventricular function improved and hydrops resolved within 3-4 weeks after FAV in 71.4% (10/14) of fetuses with a technically successful intervention. A biventricular outcome was achieved in 50% of the successfully treated patients. CONCLUSIONS: Fetuses with CAS and hydrops can be successfully treated with FAV. The procedure has the potential to restore sufficient fetal cardiac output, which may lead to resolution of hydrops. Surviving patients seem to be good candidates for a biventricular outcome. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Outcome of Pregnancies Diagnosed with TRAP Sequence Prenatally: A Single-Centre Experience.

INTRODUCTION: Twin reversed arterial perfusion (TRAP) sequence is a phenomenon seen in 1-3% of monochorionic twin pregnancies, where the acardiac fetus is found to have multiple anomalies. The normal pump twin maintains its own perfusion in addition to that of its acardiac co-twin. As a result, if the acardiac twin increases in size, the burden on the pump twin is increased, leading to cardiac failure, hydrops fetalis, polyhydramnios, premature delivery, and perinatal death. The outcome of pregnancy is largely dependent on the growth of the acardiac twin. In view of high perinatal loss, surgical techniques to interrupt the blood flow to the acardiac twin have been evaluated. METHODS: We evaluated interstitial laser therapy, which is the main mode of therapy in our unit, of TRAP pregnancies referred over a 5-year period. Interstitial laser was offered if the blood flow in the acardiac twin was found to be persistent at 2 consecutive examinations or if there were cardiac or hydropic changes in the pump twin at the first examination. RESULTS: A total of 18 cases of TRAP were referred during this period and all were counselled regarding fetal therapy if the situation were to deteriorate; 5 couples (27.7%) opted for termination of pregnancy; of the remaining 13, 7 (53.8%) agreed to perform intervention following confirmation of a normal karyotype. Six (85.7%) and 1 (14.3%) lasers were performed in the second and third trimesters, respectively; all 7 had a normal outcome of the pump twin. There were 6/13 (46.2%) in the expectant group who continued the pregnancy with no intervention, with 2 term live births (33.3%). CONCLUSIONS: Our study confirms that there is a high risk of spontaneous loss in untreated pregnancies with TRAP, primarily due to polyhydramnios and fetal hydrops. In the pregnancies that underwent interstitial laser, there was a more favourable outcome. Interstitial laser is minimally invasive, safe, and feasible in experienced hands.

Fetal giant right cervical cyst causing severe tracheal compression: A case report.

RATIONALE: Fetal giant cervical cyst (FGCC) is a rare congenital anomaly. Sometimes FGCC may extend into the mediastinum, and result in severe tracheal compression, which is a life-threatening event at birth. PATIENT CONCERNS: We present a rare case of FGCC, which extended from the right neck into the superior mediastinum, and resulted in severe tracheal compression. DIAGNOSES: An FGCC was observed by ultrasonography and magnetic resonance imaging (MRI) at 27+4 weeks' gestation (WG). Fetal MRI at 35+1 WG showed that the FGCC was 3.3 × 8.2 × 7.5 cm and extended from the right neck into the superior mediastinum. Severe tracheal compression was observed and the inside diameter of the narrowest section of tracheostenosis appeared thread-like and measured only 0.1 cm. INTERVENTIONS: Cervical cyst reduction was performed prenatally under ultrasound guidance to alleviate the tracheal compression and maximize the chance of fetal survival 2 days before birth. At 36+3 WG, cesarean section was performed, and a female neonate was immediately delivered and intubated (3.5-mm tube) by an experienced anesthesiologist. Neonatal intralesional sclerotherapy and cystic component aspiration as guided by digital subtraction angiography were performed under general anesthesia. Anesthesia was maintained only with sevoflurane 3% in 2 L/min oxygen. Extubation was performed soon after surgery. OUTCOME: The neonate recovered uneventfully and was discharged 2 days postoperatively. After 140 days of follow-up, the neonate had recovered completely. LESSONS: If an FGCC is suspected by abdominal ultrasound, a fetal MRI is recommended to assess the severity of tracheal compression before birth, if feasible. An anesthesiologist should assess the risk of intubation failure at birth according to those results. If fetal severe tracheal compression is detected and it may result in inability of intubation at birth, prenatal cervical cyst reduction under ultrasound guidance may be effective for alleviating tracheal compression at birth, if feasible. This could maximize the chance of fetal survival. Improvement of fetal short- and long-term outcomes is important.

Clinical and biological features in PIEZO1-hereditary xerocytosis and Gardos channelopathy: a retrospective series of 126 patients.

We describe the clinical, hematologic and genetic characteristics of a retrospective series of 126 subjects from 64 families with hereditary xerocytosis. Twelve patients from six families carried a KCNN4 mutation, five had the recurrent p.Arg352His mutation and one had a new deletion at the exon 7-intron 7 junction. Forty-nine families carried a PIEZO1 mutation, which was a known recurrent mutation in only one-third of the cases and private sequence variation in others; 12 new probably pathogenic missense mutations were identified. The two dominant features leading to diagnosis were hemolysis that persisted after splenectomy and hyperferritinemia, with an inconstant correlation with liver iron content assessed by magnetic resonance imaging. PIEZO1-hereditary xerocytosis was characterized by compensated hemolysis in most cases, perinatal edema of heterogeneous severity in more than 20% of families and a major risk of post-splenectomy thrombotic events, including a high frequency of portal thrombosis. In KCNN4-related disease, the main symptoms were more severe anemia, hemolysis and iron overload, with no clear sign of red cell dehydration; therefore, this disorder would be better described as a 'Gardos channelopathy'. These data on the largest series to date indicate that PIEZO1-hereditary xerocytosis and Gardos channelopathy are not the same disease although they share hemolysis, a high rate of iron overload and inefficient splenectomy. They demonstrate the high variability in clinical expression as well as genetic bases of PIEZO1-hereditary xerocytosis. These results will help to improve the diagnosis of hereditary xerocytosis and to provide recommendations on the clinical management in terms of splenectomy, iron overload and pregnancy follow-up.

Long-Term Neurodevelopmental and Respiratory Outcome after Intrauterine Therapy for Fetal Thoracic Abnormalities.

INTRODUCTION: 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. DISCUSSION: 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.

Long-Term Outcomes After Thoracoamniotic Shunt for Pleural Effusions With Secondary Hydrops.

BACKGROUND: 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. MATERIALS AND METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.

[Perinatal outcomes of thoraco-amniotic shunting for severe primary fetal hydrothorax].

Objective: To evaluate the safety and perinatal outcomes of thoracoamniotic shunting in the treatment of fetuses with severe primary hydrothorax. Methods: 22 cases of suspected severe primary fetal hydrothorax which underwent thoraco-amniotic shunting in Shanghai First Maternity and Infant Hospital, Fetal Medicine Unit and Prenatal Diagnosis Center from January 2012 to December 2017 were analyzed retrospectively. Hydrothorax associated with structural or chromosomal abnormalities, infections and immune fetal hydrops were excluded. Results: Totally, 28 shunts were placed in 22 fetuses. The median gestational age at TAS was 31.3 weeks. Preterm membrane rupture within 7 days after the procedure occurred in 9.1% (2/22) cases. Catheter displacement occurred in 18% (4/22) cases. The interval from shunting to delivery was 26.0 days. One fetus ended in induced abortion; 21 (95%, 21/22) babies were born alive, and their median gestational age at delivery was 34.4 weeks. 62% (13/21) newborns required ventilator supports; 4 neonatal deaths were attributed to pulmonary hypoplasia. The overall perinatal survival rate was 81% (17/21) . The perinatal survival rate with hydrops and without hydrops were 10/13 and 7/8 respectively. Conclusion: Thoraco-amniotic shunting is a safe procedure for intrauterine therapy and could improve the perinatal outcomes of severe primary fetal hydrothorax.

Prenatal thoraco-amniotic chest drain insertion to manage a case of fetal hydrops secondary to FOXC2.

Lymphoedema-distichiasis is an inherited autosomal dominant disorder of the lymphatic system. Rarely, it is associated with fetal hydrops; the risk and severity of which increases with successive generations. The causative gene is a member of the forkhead transcription factor family (FOXC2). We describe a fetus presenting with early-onset, rapidly progressing body wall oedema, bilateral pleural effusions and a pericardial effusion in a mother with known FOXC2 mutation. First trimester chorionic villus sampling confirmed FOXC2 mutation in the fetus when there was only a large nuchal translucency. As the phenotype progressed, the couple consented to in utero ultrasound-guided insertion of sequential bilateral pleuro-amniotic chest drains (at 23 weeks) which successfully drained the pleural effusions. The fetus was delivered at 39 weeks gestation by elective caesarean section in good condition. The shunts were removed postnatally, and the baby was discharged after 7 days. This is the first case described of a fetus with severe early-onset fetal hydrops secondary to FOXC2 mutation successfully treated by the prenatal insertion of bilateral pleuro-amniotic shunts.

Fetal lung size after thoracoamniotic shunting reflects survival in primary fetal hydrothorax with hydrops.

AIM: To assess the role of lung size and abnormal Doppler findings in the umbilical artery (UA) in determining the outcomes of fetuses with primary fetal hydrothorax (FHT) associated with hydrops who underwent thoracoamniotic shunting (TAS). METHODS: This was a retrospective study at a single center. We included cases of primary FHT with hydrops who underwent TAS at our hospital between 2004 and 2016. We assessed the relationship between mortality until 28 days after birth and ultrasound findings, including absent or reversed end-diastolic velocity (AREDV) in the UA and the lung-to-thorax transverse area ratio (LTR), before and after TAS. RESULTS: Forty-one cases of primary FHT with hydrops underwent TAS. The median (range) gestational age at TAS was 28.5 (19.3-33.8) weeks. Bilateral pleural effusion was observed in 39 cases (95.1%). Among the 41 cases, 19 (46.4%) survived, 11 (26.8%) died in utero, and 11 (26.8%) died in the neonatal period. AREDV in the UA before and after TAS were not associated with mortality (P = 0.32 and 0.47, respectively). The odds ratio for mortality in LTR 0.2-0.3 before TAS was 0.62 (vs LTR < 0.2, P = 0.45) and that in LTR 0.2-0.3 and > 0.3 after TAS were 0.27 and 0.06, respectively (vs LTR < 0.2, P for trend <0.01). CONCLUSION: A larger LTR after TAS was significantly associated with a better prognosis in hydropic primary FHT. The fetal lung size after the procedure may be a prognostic factor of primary FHT.

EXIT procedure for fetal mediastinal teratoma with large pericardial effusion: a case report with review of literature.

BACKGROUND: Large mediastinal teratomas in the fetus are rare and can present with direct compression of intrathoracic structures as well as pericardial and pleural effusions. Mediastinal fetal teratoma may be detected as a mass in the chest during a routine prenatal ultrasound. Because of the pressure on mediastinal structures it may result in non-immune fetal hydrops (NIFH) and polyhydramnios. The development of hydrops may lead to fetal demise. Timely obstetric and/or surgical intervention is important to improve survival in this patient population. Case review: We report a case of a large mediastinal teratoma in a fetus who presented with a large pericardial effusion at 28 weeks gestation. The fetus developed NIFH at 31 weeks gestation. The fetus was successfully managed with an ex utero intrapartum therapy (EXIT) procedure which involved pericardiocentesis and surgical resection of the large teratoma. Histological examination revealed an encapsulated immature teratoma. DISCUSSION: Mediastinal teratomas are usually detected by routine second- and third-trimester ultrasound. Large teratomas have been described to present with airway compromise and NIFH. Some cases have been managed by postnatal surgery and rare cases by EXIT procedure. CONCLUSIONS: This is the first reported case of a fetal mediastinal teratoma and severe pericardial effusion who developed NIFH, in whom EXIT procedure was successfully employed to simultaneously drain the effusion and resect the tumor.

Thoracoamniotic shunting for fetal pleural effusion with hydropic change using a double-basket catheter: An insight into the preoperative determinants of shunting efficacy.

OBJECTIVES: Although the efficacy of thoracoamniotic shunting (TAS) for fetal hydrothorax is well-recognized, the coexistence of hydrops fetalis is still a clinical challenge. The preoperative determinants of shunting efficacy are not fully understood. In this study, we aimed to investigate the perinatal and postnatal outcomes of hydrops fetalis with pleural effusion treated by TAS using a double-basket catheter, and to discuss the preoperative factors predictive of patients who will benefit from TAS. STUDY DESIGN: We conducted a retrospective study in hydropic fetuses with pleural effusion treated by TAS between 2007 and 2015. We extracted information regarding postnatal survival and pretherapeutic sonographic findings, including skin-edema thickness, pleural-effusion pocket size, and Doppler readings. RESULTS: Twelve subjects underwent TAS at a median gestational age of 29+5 weeks (range, 25+5-33+2 weeks). Skin edema disappeared or regressed in 7. Three experienced early neonatal death and the other 9 ultimately survived after a live birth at a median gestational age of 33+4 weeks (range, 29+1-38+2 weeks). All surviving children, except for 1, had a pretherapeutic pleural-effusion pocket greater than the precordial-edema thickness. All 3 children that died had precordial-edema thickness equal to or greater than the size of the pleural-effusion pocket. CONCLUSIONS: We achieved a high survival rate (75%) using the double-basket technique. A greater pretherapeutic width of skin edema compared with the pleural-effusion pocket is possibly suggestive of a treatment-resistant condition and subsequent poor postnatal outcome.

Prenatal factors associated with neonatal survival of infants with congenital chylothorax.

OBJECTIVES: Congenital chylothorax is a rare disease and prognostic factors are key element in properly informing parents. This study aimed at determining the prenatal factors associated with neonatal survival in a cohort of liveborn infants with congenital chylothorax. STUDY DESIGN: Observational monocentric cohort study including all liveborn neonates consecutively admitted for congenital chylothorax. RESULTS: Neonatal mortality was 32% (16/50). Prematurity (or birth weight), persistence of hydrops at birth and the absence of thoracoamniotic shunt procedure were significantly associated with mortality, whereas prenatal diagnosis of pleural effusion, side of pleural effusion, hydrops fetalis and amniodrainage were not. In case of prenatal diagnosis of hydrops fetalis, the reversal in utero of hydrops fetalis was significantly associated with survival (P=0.001). In case of thoracoamniotic shunting, the interval between thoracoamniotic shunting intervention and delivery was significantly longer for patients who survived (P=0.03). CONCLUSIONS: Thoracoamniotic shunting and reversal of hydrops significantly improves survival, whereas prematurity worsened outcome of liveborn infants with congenital chylothorax. Our data also suggest that the interval between thoracoamniotic shunting and birth appears to be crucial; the longer the interval, the more likely is the reversal of antenatal hydrops and neonatal survival.