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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., (© 2024 John Wiley & Sons Ltd.)

Shunt migration is a rare but significant complication of thoracoamniotic shunting, an intervention widely used for fetal pleural effusion. We describe a case of a term infant noted antenatally to have fetal hydrothorax that was managed with thoracoamniotic shunting but complicated by shunt migration. We also present the current literature on risk factors, complications and management of intrathoracic shunt migration. The majority of shunt migration cases are managed conservatively with no untoward postnatal sequelae, but surgical removal of the migrated shunt has been used for associated clinical complications, if visceral damage is suspected or if postnatal thoracic surgery is indicated for other reasons. We advocate an approach of conservative management for asymptomatic infants, where possible, to avoid unnecessary surgical and anaesthetic risks to very young, often already compromised children. However, further studies are still required to determine optimal management after shunt migration has occurred to ensure the best outcome., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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., Competing Interests: None declared., (Thieme. All rights reserved.)

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., (© 2022 S. Karger AG, Basel.)

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., (© 2020 International Society of Ultrasound in Obstetrics and Gynecology.)

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., (© 2019 S. Karger AG, Basel.)

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.

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., (Copyright© 2019 Ferrata Storti Foundation.)

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., (© 2018 The Author(s) Published by S. Karger AG, Basel.)

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

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.

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., (© 2018 Japan Society of Obstetrics and Gynecology.)

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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

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.

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., (Copyright © 2017 Elsevier B.V. All rights reserved.)

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.

Introduction: Pseudoamniotic band syndrome (PABS) occurs iatrogenically after fetal surgery or amniocentesis due to chorioamniotic membrane separation. Separation of the amnion from the chorion can expand to form fibrous amniotic bands that can envelope fetal limbs or the umbilical cord, with consequences ranging from limb constriction to fetal demise., Case Report: We report a case of bilateral fetal pleural effusions at 27 weeks' gestation treated by bilateral thoracoamniotic shunts. Following shunt placement, the hydrothorax resolved. However, chorioamniotic membrane separation developed resulting in PABS with subsequent umbilical cord strangulation and fetal demise at 32 weeks' gestation., Conclusion: PABS has been previously described in the literature following various fetal interventions. This is the first reported case of pseudoamniotic band syndrome after placement of fetal thoracoamniotic shunts. A high index of suspicion is required to diagnose PABS via postoperative ultrasound. Post intervention chorioamniotic membrane separation warrants close surveillance for sonographic evidence of PABS.

Objective: We presented a fetus affected by macrocystic lung lesions with progressive hydropic changes during the second trimester, but experienced remarkable resolution of hydrops in the third trimester after a series of in utero interventions., Case Report: A 19-year-old women, G1P0, presented with fetal multilocular thoracic mass and hydropic change at 23 +4 weeks of gestation. After non-directive genetic counseling, she opted for intrauterine cyst aspiration followed by intra-cystic OK-432 injection at 24 weeks of pregnancy, as well as sequential thoracoamniotic shunts at 26 weeks and 27 +3 weeks of pregnancy when we observed hydrops developed progressively. Finally, the hydrops resolved in the third trimester and a healthy baby was born at 33 +3 weeks of pregnancy, in which further surgical intervention was performed at five-month old., Conclusion: Thoracoamniotic shunting is a preferred option for all hydropic fetuses resulted from large macrocystic lung lesions to enhance perinatal survival rate., (Copyright © 2017. Published by Elsevier B.V.)

Objectives: This study aims to determine the prognostic factors and outcomes of primary fetal hydrothorax (FHT) and investigate the effects of fetal therapy., Methods: A nationwide survey was conducted on fetuses with primary FHT delivered after 22 weeks of gestation between January 2007 and December 2011 at perinatal centers., Results: Among the 287 cases of primary FHT, the survival rates for those with and without hydrops were 58.0% (113/195) and 97.8% (90/92), respectively. The survival rates in the no-therapy, thoracocentesis, and thoracoamniotic shunting (TAS) groups in the hydropic cases and the non-hydropic cases were 59.7% (40/67), 51.5% (35/68), and 63.3% (38/60) and 98.1% (53/54), 96.3% (26/27), and 100% (11/11), respectively. The crude relative risk for death was 2.1 (p = 0.005) for fetuses diagnosed at 26 to 30 weeks of gestational age (vs ≥30 weeks), 2.3 (p = 0.001) for both skin edema and ascites, and 3.1 (p = 0.02) for bilateral pleural effusion. TAS was associated with a significant risk reduction for death in hydropic cases [adjusted relative risk 0.61, p = 0.01 (vs no fetal therapy)]., Conclusions: Hydrops and an early gestational age at diagnosis (<30 weeks of gestation), skin edema with ascites, and bilateral effusion predicted a poor prognosis in primary FHT cases. TAS was associated with a higher survival rate. © 2016 John Wiley & Sons, Ltd., (© 2016 John Wiley & Sons, Ltd.)

Mirror syndrome is a rare disease with unknown pathophysiology that can be present in different diseases that can cause fetal hydrops. The prognosis is usually bad with a high perinatal mortality. We report an unusual form of mirror syndrome that manifested itself only after a successful treatment for fetal hydrops (caused by twin-twin transfusion syndrome, in Quinteros stage IV) was performed. This syndrome was controlled by medical treatment, and despite the usually bad prognosis seen in these cases, we could extend the pregnancy from the 23rd to the 34th week of gestation, resulting in the birth of 2 live infants., (Thieme Publicações Ltda Rio de Janeiro, Brazil.)

Antenatally, congenital pulmonary airway malformation (CPAM) causing fetal hydrops can be palliated with thoracoamniotic shunts, which may become displaced in utero. We report a case of an infant born at 34 weeks gestational age with an antenatally diagnosed macrocystic lung lesion, fetal hydrops and an internally displaced thoracoamniotic shunt. The infant suffered refractory pneumothoraces despite multiple chest drains, and stabilised only after surgical resection of the lesion. Intraoperatively, the shunt was noted to form a connection between a type I CPAM and the pleural space. As the shunt was displaced internally, this complication was not immediately obvious during the initial resuscitation. In infants with large cystic lung lesions, clinicians should be aware that internally displaced thoracoamniotic shunts could contribute to refractory tension pneumothoraces and anticipate the need for advanced neonatal resuscitation, including early thoracocentesis or chest drain insertion. Furthermore, displaced shunts may require early surgical intervention., (2016 BMJ Publishing Group Ltd.)

Dehydrated hereditary stomatocytosis (DHSt) is an autosomal dominant congenital hemolytic anemia with moderate splenomegaly and often compensated hemolysis. Affected red cells are characterized by a nonspecific cation leak of the red cell membrane, reflected in elevated sodium content, decreased potassium content, elevated MCHC and MCV, and decreased osmotic fragility. The majority of symptomatic DHSt cases reported to date have been associated with gain-of-function mutations in the mechanosensitive cation channel gene, PIEZO1. A recent study has identified two families with DHSt associated with a single mutation in the KCNN4 gene encoding the Gardos channel (KCa3.1), the erythroid Ca(2+) -sensitive K(+) channel of intermediate conductance, also expressed in many other cell types. We present here, in the second report of DHSt associated with KCNN4 mutations, two previously undiagnosed DHSt families. Family NA exhibited the same de novo missense mutation as that recently described, suggesting a hot spot codon for DHSt mutations. Family WO carried a novel, inherited missense mutation in the ion transport domain of the channel. The patients' mild hemolytic anemia did not improve post-splenectomy, but splenectomy led to no serious thromboembolic events. We further characterized the expression of KCNN4 in the mutated patients and during erythroid differentiation of CD34+ cells and K562 cells. We also analyzed KCNN4 expression during mouse embryonic development., (© 2015 Wiley Periodicals, Inc.)

Purpose: The purpose of this study was to review the unique imaging characteristics, prenatal course, and outcomes for fetuses with mainstem bronchial atresia (MBA)., Methods: The records of all patients referred for a fetal lung malformation from 2001 to 2012 and the medical literature were reviewed to identify cases of MBA., Results: Of 129 fetuses evaluated, 3 were diagnosed prenatally with right-sided MBA. The first had a CCAM-volume ratio (CVR) of 9, hydrops, mirror syndrome, and preterm delivery of a nonviable fetus. The second (CVR 2.6) had ascites, preterm delivery at 34-weeks, and neonatal demise. The third fetus (CVR 5.7) presented with hydrops at 21-weeks, prompting fetal pneumonectomy. Postoperatively, hydrops resolved, and the contralateral lung grew dramatically, but preterm delivery occurred 3 weeks later. Ventilation could not be sustained, and the infant died. Four similar cases of MBA were in the literature, all right-sided. Two fetuses with hydrops delivered at 25-weeks and died immediately. One pregnancy was terminated. One fetus underwent pneumonectomy at 24-weeks but died intraoperatively., Conclusion: MBA is a rare and lethal lesion that must be distinguished from other right-sided lung masses. Fetal pneumonectomy can be performed with resolution of hydrops and compensatory contralateral lung growth, but remains limited by complications of preterm birth., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: The aim of the study was to perform an audit the results of fetal therapy in cases of nonimmune hydrops fetalis (NIHF), isolated hydrothorax and isolated ascites., Methods: A total of 38 fetuses (17-35 weeks of pregnancy) were included in the study whereas 6 patients were excluded due to abnormal karyotype. NIHF was diagnosed in 24 cases, hydrothorax in 4 cases, and ascites in 4 cases. Shunts were implanted in 26 (81%) cases and 7 (19%) participants underwent therapeutic cordocentesis., Results: After therapy anterior-posterior diameter of the right and the left lung increased to 9.6 mm (27%) and 12.4 mm (35%), respectively. Early complications were observed in 5 (16%) cases. PROM 2 (40%), fetal death 1 (20%), infection 1 (20%), and preterm delivery 1 (20%). Out of the 27 patients, 65% had a caesarian section without early complications and 35% had a vaginal delivery with 58% at term and 42% pre-term., Conclusions: Preceding results show that intrauterine therapy significantly improves prognosis of fetuses with NIHF.

Objective: Cardiac dysfunction is common in the recipient fetus of twin-twin transfusion syndrome (TTTS). In this study, we aimed to document the severity of fetal cardiac dysfunction in Stage IV TTTS (fetal hydrops) and assess evolution of cardiac function longitudinally after fetoscopic laser surgery., Methods: We reviewed obstetric ultrasound examination data, pre- and postoperative echocardiograms and neonatal outcomes for 22 cases of Stage IV TTTS undergoing fetoscopic laser ablation of placental anastomoses between 1998 and 2011. Myocardial performance index, atrioventricular valve flow patterns, ventricular shortening fraction, ventricular hypertrophy, outflow tract obstruction and venous Doppler waveforms were assessed., Results: Nineteen fetuses (86.4%) had ascites, eight (36.4%) had pleural effusions, nine (40.9%) had a pericardial effusion and 12 (54.5%) had subcutaneous edema at presentation. Preoperatively, cardiac function was grossly abnormal in all. Eight fetuses (36.4%) had functional pulmonary atresia and one (4.5%) had functional aortic atresia. Seventy-seven percent of recipient fetuses survived until birth. Postoperative echocardiographic follow-up (mean, 26 days) showed that indices of fetal cardiac function improved considerably, but never completely normalized. Six of the eight fetuses with functional pulmonary atresia (75.0%), as well as the fetus with functional aortic atresia, survived to birth. In all cases, the functional atresia resolved within 48 h of laser ablation therapy and none had structural valve anomalies at birth. All fetal effusions resolved after the laser., Conclusions: Fetoscopic laser ablation of placental anastomoses reverses cardiac dysfunction and valvulopathy, even in the most severe cases of TTTS. However, recovery takes longer than in early stage disease., (Copyright © 2013 ISUOG. Published by John Wiley & Sons, Ltd.)

A 22-week fetus presented with a large left ventricular aneurysm, 24 × 21 × 18 mm in size, detected by abnormal four-chamber view, and severe fetal hydrops with pericardial effusion, ascites and skin edema. The aneurysm was thin-walled, hypokinetic, and had enlarged with gestational age, causing compression of the lung. Although the left ventricular function had progressively impaired as expressed by increase in Tei index, hydrops had resolved by 32 weeks of gestation, probably because of maternal digoxin therapy and successful compensation by the right ventricle, as represented by retrograde blood flow in the distal aortic arch via the patent arterial duct. Because of the significant risk of severe cardiorespiratory failure, we transported the mother to a neonatal cardiac surgical center at 38 weeks of gestation. Indeed, the baby showed severe cardiopulmonary failure after birth, showing 100% of cardiothoracic ratio on the chest X-ray film, but was saved by the successful Dor procedure, including surgical resection of the aneurysm at 10 h of life. In this case, serial echocardiographic evaluation can allow us to monitor the hemodynamics and lead to successful postnatal management.

Sacrococcygeal teratoma (SCT) is a rare congenital disease and prognostic factors have not been entirely established. We report two cases of fetal SCT with different clinical courses. Case 1 was a cystic, slow growing tumor with mild vascularity. The tumor was removed one week after delivery at 35 weeks, and there was no recurrence at 1.5-year follow-up. Case 2 was a solid, rapid growing tumor with rich vascularity. Cesarean section was performed due to severe fetal hydrops and mirror syndrome in the mother at 27 weeks. The tumor had ruptured and was removed soon after delivery to control bleeding, but the baby died the next day. Our cases suggest that solid component and rich vascularity might correlate with poor prognosis., (© 2012 The Authors. Congenital Anomalies © 2012 Japanese Teratology Society.)

Hydrops fetalis (fetal hydrops) is a serious fetal condition defined as abnormal accumulation of fluid in two or more extravascular compartments, including ascites, pleural effusion, pericardial effusion, and skin edema. Edema is classified as immune or non-immune. Today more than 90% of fetal edema has non-immune cause. This paper presents a case of a pregnant woman who was admitted to the Obstetrics and Gynecology Department because of fetal hydrops with massive pleural effusion and polyhydramnios at 34 weeks gestation. The intrauterine therapy consisted of two treatments. During the first surgery amnioreduction, evacuation of fluid from the pleural cavity of the fetus, and shunts to both pleural cavities were performed. During the second surgery amnioreduction, cordocentesis with albumin administration and pleural shunt were performed. Intrauterine therapy led to a reduction of swelling of the fetus from 7mm up to 1-2 mm and the total evacuation of fluid from the pleural cavity and the fetal lung expansion. We also present the condition of the neonate after birth and after 12 months of life.

Objective: From a single-center retrospective cohort with fetal chylothorax, we evaluated the factors related to the decision to use shunting, poor prognostic factors, and reported shunting outcomes with a new double basket-catheter device., Methods: A retrospective single-center study was performed in 35 cases of fetal chylothorax., Results: There were 35 cases of chylothorax: 23 with hydrops and 12 without hydrops. Twenty-one procedures were performed on 15 fetuses (11 with hydrops) with a single shunt in 11, two shunts in 3 and four shunts in 1. All 12 nonhydropic cases survived. In 23 hydropic cases, overall survival rates with and without thoracoamniotic shunting were 46 and 33%, respectively. The mortality rates of fetal hydropic cases with and without ascites were 93 and 11%, respectively. Fetal ascites, progression of fetal hydrops, and premature delivery at <33 weeks were significant risk factors for a poor prognosis. Progression of polyhydramnios after shunting was also associated with a poor prognosis. Obstruction of the catheter was observed in 38%. There were no direct fetal deaths associated with shunting., Conclusion: Thoracoamniotic shunting should be considered for pleural effusion before development of fetal hydrops, or at least before the appearance of fetal ascites. A double-basket catheter tends to be obstructive, but may be less invasive for fetuses., (Copyright © 2013 S. Karger AG, Basel.)

This is the first report of fetoscopy to successfully treat a case of bilobar bronchial atresia presenting in the form of a massive right lung tumor causing severe thoracic compression with mediastinal shift, pulmonary hypoplasia and eventually fetal hydrops. The presence of an echolucent tubular structure in the pulmonary hilum was identified as the intrapulmonary continuation of the interrupted main bronchus, and led to the diagnosis of main bronchial atresia. After extensive discussion and counseling, a fetoscopic operation was designed and indicated. The procedure consisted of a fetal tracheoscopy and access to the right main bronchus, which was perforated with a diode laser until a communication with the dead end of the intrapulmonary main bronchus was achieved. After the operation a significant reduction in size was observed, hydrops and mediastinal shift disappeared and O/E LHR increased to 85%. The fetus was born at term and lobectomy of two dysplastic pulmonary lobes was performed. The infant is developing well at 14 months of age. This report demonstrates that fetoscopic airway decompression can achieve fetal survival in the rare event of main bronchus atresia., (Copyright © 2012 S. Karger AG, Basel.)

Objective: To evaluate the perinatal outcomes in hydropic fetuses with congenital microcystic pulmonary lesions that underwent percutaneous, invasive, laser therapy., Method: This retrospective study reviews the literature and our experience between 2004 and 2010. Characteristics of the cystic lung lesions, liquor volume (presence of polyhydramnios or not), localization of ablation (vascular vs interstitial) and gestational age at which the procedure was performed were related to outcome (survival)., Results: In total, 16 fetuses with congenital lung lesions underwent 'invasive' percutaneous laser ablation, seven performed in our center and nine published cases. Survival rate was higher in fetuses with a subsequent postnatal diagnosis of bronchopulmonary sequestration (87.5%) compared with congenital adenomatoid malformation (28.6%; p = 0.04). The technique of vascular ablation was more successful (100%) than interstitial ablation (25.0%, p < 0.01)., Conclusion: Percutaneous vascular laser ablation seems to be effective for bronchopulmonary sequestration in hydropic fetuses. Outcomes were worst following interstitial ablation for microcystic congenital adenomatoid with hydrops., (© 2012 John Wiley & Sons, Ltd.)

Objective: To describe the safety and efficacy of thoracoamniotic shunting for fetal pleural effusion using a double-basket catheter with a very small diameter (1.47 mm)., Method: In this 2-year multicenter, prospective single-arm clinical study registered with the University hospital Medical Information Network (UMIN) Clinical Trials Registry (UMIN00001095); shunting was performed between 18w0d and 33w6d of gestation with this catheter in cases of fetal pleural effusions reaccumulating after thoracocentesis. The primary endpoint measures were maternal and fetal adverse effects and survival in the neonatal period., Results: A total of 24 cases were included, of which 17 had hydrops (71%). The median gestational ages at shunting and delivery were 27.4 and 34.8 weeks, respectively. There were no fetal deaths, lung injuries, or severe maternal complications. Preterm rupture of the membranes occurred in 7/24 (29%) cases at a median of 62 days after the shunting. Preterm rupture of the membranes within 28 days of the procedure occurred in 1/24 (4%) cases. Catheter displacement towards the fetal thoracic cavity occurred in 4/42 (10%) cases. The overall survival rate was 79% (19/24), whereas it was 71% (12/17) in the cases with hydrops., Conclusion: Drainage of fetal pleural effusions with a double-basket shunt is safe and effective, and the shunt could be an alternative device., (© 2012 John Wiley & Sons, Ltd.)

Here we describe a case of antenatally diagnosed congenital cystic adenomatoid malformation. Due to increasing cyst size and fetal hydrops an antenatal drain was inserted. The lesion was excised surgically soon after birth due to significant respiratory distress. During histological assessment an area of squamous metaplasia was found within the cyst wall. This developed as a result of the drain placement. At 4-year follow-up the child remains well and has experienced no long-term respiratory sequalae.

Thoracoamniotic shunting is the treatment of choice for management of the fetus with type 1 congenital pulmonary airway malformation. Thoracoamniotic shunting has been performed to reduce life-threatening risks such as fetal hydrops. However, caution is needed because of possible complications. Here, we report that thoracoamniotic shunting can cause histologic changes in the cyst epithelia. In 5 of 8 patients treated prenatally with thoracoamniotic shunting, squamous metaplasia in the cyst epithelia was seen; whereas squamous metaplasia was not found in 6 patients who were not treated with this procedure. Our results reveal that long-term exposure to the intrauterine environment could possibly lead to the change in the nature of cyst epithelium and consequent squamous metaplasia., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: To evaluate fetal thoracoamniotic shunting for isolated large macrocystic congenital cystic adenomatoid malformations (CCAM) of the lung., Methods: This was a retrospective study of 11 fetuses with macrocystic CCAM who underwent thoracoamniotic shunting. This procedure was offered if fetal hydrops or signs of evolving hydrops (such as ascites or polyhydramnios) were present, or when there were very large lesions or lesions rapidly increasing in size. If there were multiple large cysts within the lesion, a single shunt was used, aiming to traverse several cysts., Results: Shunts were inserted at a mean gestational age of 24.6 (range, 17-32) weeks. Marked mediastinal shift was present in all cases. Six fetuses were hydropic and, of the remaining five, one had severe polyhydramnios, three had lesions that were rapidly increasing in size and one had a very large lesion at initial presentation. In total, four cases had polyhydramnios. Shunting one cyst always decompressed the entire lesion and hydrops and/or polyhydramnios resolved in all surviving fetuses. One hydropic fetus that underwent the procedure at 17 weeks died 1 day later. The shunt dislodged in one case and the lesion did not re-expand. No mother went into labor or had ruptured membranes before 35.6 weeks. Mean gestational age at delivery was 38.2 weeks (n = 10). All pregnancies were delivered vaginally, with no maternal complications. All newborns had uneventful lobectomies, and pathology confirmed CCAM in all cases., Conclusion: Fetal thoracoamniotic shunting for large macrocystic CCAM is associated with favorable outcome in most cases, and should be considered in severe cases even before hydrops develops., (Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd.)

Background and Objectives: Fetus prenatally diagnosed with neck tumors, or with any other disease that obstructs the airways, should not be treated conventionally, as the assistant physician has to face two challenges right after the infant's delivery: the limited time to establish the access to the potentially difficult airways and the lack of anesthesia of the neonate in case of instrumentation of the airways. The ex utero intrapartum treatment, i.e., the EXIT procedure consists of maintaining the fetoplacental circulation during the cesarean section, until the airways of the fetus be secured., Case Reports: Female patient, 37 years old, G3P2, 38 weeks pregnant, having polyhydramnios and fetus diagnosed with large cervical masses by prenatal ultrasound. A cesarean section was performed using the EXIT procedure to enable safe access to the infant's airways. After hysterotomy, the fetus was intubated by direct laryngoscopy. The neonate was immediately transferred to another operating room, where cervical tumor resection of the neck tumor and tracheostomy were successfully performed. Female patient, 27 years old, G3P1A1, 32 weeks pregnant, whose fetus was prenatally diagnosed with a large oral tumor. As the tumor obstructed the fetus' airways, a tracheostomy was performed when the fetus underwent EXIT procedure. It was then possible to use direct laryngoscopy for neonate intubation. The fetus underwent tumor resection and was sent to the Neonatal Intensive Care Unit., Conclusions: Reports describe the successful use of general anesthesia with isoflurane for cesarean delivery followed by the EXIT procedure in fetus diagnosed with tumors obstructing the airways., (Copyright © 2012 Elsevier Editora Ltda. All rights reserved.)

Background/purpose: Congenital pulmonary airway malformations (CPAM) are rare lesions often diagnosed during routine prenatal ultrasound. The presence of hydrops fetalis is an indicator of poor prognosis. Here we present a retrospective review of fetuses undergoing either open fetal surgery or steroids for predominantly microcystic CPAM with hydrops fetalis., Method: A retrospective review of patients undergoing open fetal surgery or steroids for CPAM at our institution was performed. The primary outcome was survival., Results: A retrospective review of all patients referred to our institution with the diagnosis of CPAM was performed. Fetuses with predominantly microcystic CPAM and the presence of hydrops fetalis treated with steroid or surgery were included. Thirteen patients were treated with steroids, and 11 patients underwent open fetal surgery. In the steroid group 12 (92%) of 13 fetuses survived to delivery versus 9 (82%) of 11 in the open fetal surgery group. Only 5 (56%) of 9 of the patients in the open fetal surgery group survived to neonatal discharge compared to 10 (83%) of 12 in the steroid group., Conclusions: In the present retrospective study, improved survival was seen in fetuses with hydrops fetalis and predominantly microcystic CPAM treated with steroids when compared with open fetal surgery. Steroids should be considered for first-line therapy in these cases., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: To determine whether the congenital cystic adenomatoid malformation (CCAM) volume ratio (CVR) is associated with fetal and postnatal outcome after prenatal diagnosis and antenatal expectant management in a provincial tertiary referral center that does not offer fetal surgery., Methods: Retrospective cohort of 71 consecutive cases of prenatally diagnosed CCAM meeting study criteria (1996-2004). CVR was calculated on the initial ultrasound at the referral center, and associated with hydrops (Fisher's exact test) and a composite adverse postnatal outcome consisting of death, intubation for respiratory distress, extracorporeal membrane oxygenation, non-elective surgery for symptomatology, or respiratory infection requiring hospital admission (Mann-Whitney test)., Results: A CVR > 1.6 was significantly associated with hydrops (p = 0.003). In addition, the CVR was significantly associated with the composite adverse postnatal outcome (p = 0.004) at a mean age of follow-up of 41 months (range < 1-117 months). For CVR and postnatal outcome, the area-under-the-curve receiver operating characteristic was 0.81 (95% CI 0.69-0.93, p = 0.006), and choosing a CVR cut-off of < 0.56, the negative predictive value was 100% (95% CI 0.85-1.00)., Conclusion: In a provincial referral center with antenatal expectant management of CCAM, the CVR was associated with hydrops and postnatal outcome, with a CVR < 0.56 predictive of good prognosis after birth., (Copyright © 2012 S. Karger AG, Basel.)

Purpose: The aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors., Methods: The records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings of hydrops) and vascular tumor evaluated between July 2001 and March 2011 were reviewed retrospectively. Hydrops was defined as accumulation of fluid in 2 or more compartments., Results: Of fetuses with high-risk lung mass, hydrops was identified in 46% (11/24). Fetuses with hydrops and an abnormal echocardiogram (n = 8) demonstrated poor survival without fetal surgery (13%) compared with 100% survival in fetuses with hydrops and a normal echocardiogram (n = 3; P = .02). Of 21 fetuses with vascular tumor (11 sacrococcygeal and 8 cervical teratomas; 2 hemangioendotheliomas), hydrops was identified in 29% and an abnormal echocardiogram in 57%. All fetuses with hydrops had an abnormal echocardiogram and either died (n = 5) or required fetal surgery (n = 1). However, all fetuses with abnormal echocardiograms alone (n = 7) survived without fetal intervention., Conclusions: For fetuses with lung mass, an abnormal echocardiogram in the setting of hydrops is the best predictor of mortality and need for fetal surgery. For fetuses with vascular tumor, hydrops in the setting of high-output physiology best predicts demise and need for fetal surgery., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Nonimmune hydrops fetalis is observed with the frequency of 1:3000 cases diagnosed pre- and postnatally. In the following paper the authors analyzed the course of pregnancy complicated by fetal ascites and polyhydramnios with the appearance of colonic ileus and they presented the postnatal condition of the baby The preliminary diagnosis was confirmed after birth and the newborn was operated in the second day of his life. The congenital small bowel atresia was qualified as a III B type (Grossfeld qualification), which is called the "pagoda" syndrome [3]. The colonic atresia is located then around the superior mesenteric vessels, which leads to colonic necrosis (Figure 1).

Fetal pleural effusion is a rare occurrence, with an incidence of 1 per 10-15,000 pregnancies. The prognosis is related to the underlying cause and is often poor. There is increasing evidence that in utero therapy with thoraco-amniotic shunting improves prognosis by allowing lung expansion thereby preventing hydrops and pulmonary hypoplasia. This is a review of all cases of fetal pleural effusion managed over an eight year period the National Maternity Hospital Dublin. Over the nine year period there were 21 cases of fetal pleural effusion giving an overall incidence of 1 per 9281 deliveries. Of these, 15 underwent thoraco-amniotic shunting. There were associated anomalies diagnosed in 5 (33%) of cases. The overall survival in our cohort was 53%. The presence of hydrops was a poor prognostic factor, with survival in cases with hydrops of 33% (3/9) compared to 83% (5/6) in those cases without associated hydrops.

Most fetal lung masses present by mid gestation, grow during the canalicular phase of lung development (18-26 weeks of gestation), and plateau in growth or shrink after 26 weeks of gestation. We describe the unique case of a fetal lung mass presenting at 37 weeks of gestation with hydrops and fetal heart failure. The late growth of this lesion and resultant hydrops prompted resection as part of the ex utero intrapartum treatment. Histopathology revealed a rare, recently described fetal lung interstitial tumor. This case demonstrates that a subset of fetal lung masses may continue to grow later in gestation and emphasizes the need for late gestation imaging and close follow-up in this patient cohort., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Twin anemia-polycythemia sequence (TAPS) is an atypical form of twin-twin transfusion syndrome (TTTS) that presents as a large intertwin hemoglobin difference with one twin developing anemia and the other developing polycythemia, without oligohydramnios-polyhydramnios sequence (Lopriore et al., Placenta 2007;28:47-51). The prenatal diagnostic criteria for TAPS require that the middle cerebral artery-peak systolic velocity (MCA-PSV) measure greater than 1.5 multiples of median (MoM) in the donor twin and less than 0.8 MoM in the recipient twin (Robyr et al., Am J Obstet Gynecol 2006;194:796-803; Klaritsch et al., Ultrasound Obstet Gynecol 2009;34:149-154; Mari et al., N Engl Med 2000;342:9-14). The presumed etiology of TAPS involves the presence of small caliber arteriovenous anastomoses, which generate a slow transfusional process allowing for hemodynamic compensation (Lopriore et al., Placenta 2007;28:47-51; Lopriore et al., Placenta 2009;30:223-225; Lewi et al., Am J Obstet Gynecol 2006;194:790-795; Lopriore et al., Am J Obstet Gynecol 2008;112:753-758; Van den Wijngaard et al., Placenta 2007;28:611-615). The resulting polycythemia in the recipient twin is a risk factor for fetal and placental thrombosis (Van den Wijngaard et al., Am J Physiol 2005;288:R799-R814). We present a case of spontaneous TAPS complicated by a large placental vessel thrombosis and hydrops fetalis. Treatment via selective laser photocoagulation of communicating vessels (SLPCV) resulted in normalization of the MCA-PSV discordance.

Aim: The purpose of this study is to evaluate the accuracy of prenatal diagnostic features, particularly congenital cystic adenomatoid malformation volume ratio (CVR), in predicting outcomes for fetuses with lung masses., Methods: The records and imaging features of all fetuses referred to the Texas Children's Fetal Center with a fetal lung mass between July 2001 and May 2010 were reviewed retrospectively. Data collected included gestational age (GA) at diagnosis, fetal magnetic resonance imaging findings, CVR, mass size, nature of fetal treatment, surgical findings, pathology, and outcome. Data were analyzed for predicting development of hydrops or the need for fetal therapy using receiver operating characteristic curves., Results: Of 82 fetuses (41 male) evaluated for a lung mass, 53 (65%) were left-sided (1 bilateral), and the mean (SD) GA at diagnosis was 21.5 (4.3) weeks. Seventy-three fetuses underwent fetal magnetic resonance imaging at a mean (SD) GA of 26.1 (4.6) weeks. Thirteen fetuses (16%) had fetal treatment. Four fetuses with hydrops underwent open fetal surgical resection, and 3 survived. Six fetuses with large lung masses and persistent mediastinal compression near term underwent ex-utero intrapartum therapy-to-resection procedures, and 3 fetuses with hydrops underwent serial thoracentesis. Congenital cystic adenomatoid malformation volume ratio correlated strongly with the development of hydrops and the need for fetal therapy with an area under the receiver operating characteristic curve of 0.96 (P < .0001) and 0.88 (P < .0001), respectively. Of 18 fetuses with a CVR greater than 2.0 compared with 2 (3%) of 60 with a CVR of 2.0 or less, 10 (56%) required fetal intervention (P < .0001)., Conclusion: Congenital cystic adenomatoid malformation volume ratio correlates strongly with the development of fetal hydrops and the need for fetal intervention. A threshold value of 2.0 yields the most powerful statistical results., (Copyright © 2011 Elsevier Inc. All rights reserved.)