Resolution Of Hydrops Research Articles

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

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

The impact of in utero transfusions on perinatal outcomes in patients with alpha thalassemia major: the UCSF registry

AbstractAlpha thalassemia major (ATM) is a hemoglobinopathy that usually results in perinatal demise if in utero transfusions (IUTs) are not performed. We established an international registry (NCT04872179) to evaluate the impact of IUTs on survival to discharge (primary outcome) as well as perinatal and neurodevelopmental secondary outcomes. Forty-nine patients were diagnosed prenatally, 11 were diagnosed postnatally, and all 11 spontaneous survivor genotypes had preserved embryonic zeta-globin levels. We compared 3 groups of patients; group 1, prenatally diagnosed and alive at hospital discharge (n = 14), group 2, prenatally diagnosed and deceased perinatally (n = 5), and group 3, postnatally diagnosed and alive at hospital discharge (n = 11). Group 1 had better outcomes than groups 2 and 3 in terms of the resolution of hydrops, delivery closer to term, shorter hospitalizations, and more frequent average or greater neurodevelopmental outcomes. Earlier IUT initiation was correlated with higher neurodevelopmental (Vineland-3) scores (r = −0.72, P = .02). Preterm delivery after IUT was seen in 3/16 (19%) patients who continued their pregnancy. When we combined our data with those from 2 published series, patients who received ≥2 IUTs had better outcomes than those with 0 to 1 IUT, including resolution of hydrops, delivery at ≥34 weeks gestation, and 5-minute appearance, pulse, grimace, activity, and respiration scores ≥7. Neurodevelopmental assessments were normal in 17/18 of the ≥2 IUT vs 5/13 of the 0 to 1 IUT group (OR 2.74; P = .01). Thus, fetal transfusions enable the survival of patients with ATM and normal neurodevelopment, even in those patients presenting with hydrops. Nondirective prenatal counseling for expectant parents should include the option of IUTs.

Maternal Steroids in High-Risk Congenital Lung Malformations

IntroductionThe purpose of the present study is to evaluate our institutional management of high-risk congenital lung malformations (CLM) with particular consideration of the use of multiple maternal steroid courses and maternal steroids in CLMs with pathologies other than congenital pulmonary airway malformation (CPAM). MethodsA single-center retrospective review was performed for all fetuses evaluated for CLM who received maternal steroids and/or had a CLM volume ratio (CVR) ≥ 1.6 (2015-2020). Fetuses were categorized as receiving no steroids, single steroid, or multiple steroid courses. Outcomes evaluated included CVR growth rate, resolution of early hydrops, and resolution of hydrops. Results are reported with a descriptive analysis. ResultsNineteen patients were identified who had CVR ≥ 1.6 (single steroid course 9/19, multiple steroid courses 6/19, and no steroids 4/19). A majority (n = 13, 68%) of all lesions had a reduction or no change in CVR between initial and final measurements (single steroid course 7/9, 78%; multiple steroid courses 4/6, 67%). When evaluating by pathology, ≥ 50% of each classification had reduction or no growth of CVR (CPAM 7/11, bronchial atresia 2/4, sequestration 3/3, congenital lobar emphysema 1/1). Seventy five percent (3/4) of lesions with early hydrops had resolution following steroid treatment (single steroid course 1, multiple steroid courses 2). Of the four lesions that had hydrops, only one had resolution after receiving multiple steroid courses. ConclusionsOur institutional experience reports the majority of CLM (including pathologies other than CPAM) who received steroids had reduction or no change in CVR. Given the low risk-benefit ratio of maternal steroids, physicians could consider use of multiple steroid courses for CLM refractory to a single course.

Successful Management of Corneal Hydrops and Intrastromal Corneal Ring Segment (ICRS) Migration Following ICRS Implantation for Keratoconus

Patient: Male, 17-year-old Final Diagnosis: Acute corneal hydrops • intrastromal corneal ring segment migration Symptoms: Poor visual acuity • rigid contact lens intolerance Medication: — Clinical Procedure: Intrastromal corneal ring segment implantation • intrastromal corneal ring segment reimplantation Specialty: Ophthalmology Objective: Unusual or unexpected effect of treatment Background:Acute corneal hydrops refers to a rare complication of keratoconus and other ectatic disorders with potentially sight-threatening sequelae. Intrastromal corneal ring segment (ICRS) implantation is a surgical procedure performed as therapy for keratoconus when there is contact lens intolerance. ICRS migration along the tunnel made for its insertion is one of its most frequent complications. We believe this is the first published case of acute corneal hydrops and ICRS migration unfolding shortly after an uneventful ICRS implantation, and successfully managed with medical treatment and ICRS reimplantation, respectively.Case Report:A 17-year-old male, previously diagnosed with bilateral keratoconus 4 years earlier for which he underwent cross-linking surgery for both eyes 3 years prior, presented to our department for a first-time keratoconus assessment. His best-corrected visual acuity (BCVA) was 20/25 OD and 20/40 OS with rigid gas-permeable contact lenses. Due to contact lens intolerance in the OS and lack of custom-fit scleral lenses at the time, 2 ring segments (Ferrara, AJL Ophthalmic, Inc.) with an arc length of 160 degrees and 300 μm in size were implanted at a depth of 320 μm. Within 1 week, severe hydrops and ICRS migration emerged. Medical treatment provided resolution of hydrops and then ICRS reimplantation was performed without further complications, resulting in contact lens tolerance alongside a BCVA of 20/40 OS.Conclusions:Corneal hydrops is a challenging complication in ICRS implantation due to its infrequent occurrence and the severity of its potential sequelae. Prompt medical treatment and close follow-up are essential to obtain the best outcome in these instances.

Use of Corticosteroids in Prenatal Treatment of Congenital Pulmonary Adenomatoid Malformation: Integrative Review.

To review data on the use of corticosteroids for the treatment of fetuses with high-risk congenital pulmonary adenomatoid malformation (CPAM). Integrative review based on the literature available on MEDLINE and LILACS, including articles published until November, 2020. The initial search resulted in 87 articles, 4 of which were selected for analysis, with all of them being retrospective descriptive observational studies. In the group of fetuses that received only a single corticosteroid cycle, the hydrops resolution rate was 70%, and the survival rate was 83.8%. In fetuses treated with 2 or more cycles of corticosteroids, there was an improvement in the condition of hydrops or edema in a single body compartment in 47%, and survival of 81.8% of the fetuses. The use of corticosteroids for the prenatal treatment of high-risk CPAM appears to be associated with an improvement in perinatal outcomes.

Favourable outcome for hydrops or cardiac failure associated with fetal tachyarrhythmia: a 20-year review.

Prognosis of fetuses with hydrops and tachyarrhythmia has been portrayed as poor in most published reports. This might lead to biased counselling, unnecessary caesarean section, preterm delivery, and even termination of pregnancy. To evaluate contemporary fetal and postnatal outcomes of hydropic fetuses with fetal tachyarrhythmia when it is treated effectively and monitored systematically. This is a retrospective review of a single centre experience at the University Hospital of Wales over a 20-year period. All fetuses received high doses of flecainide and digoxin combination treatment. Tachycardia response rate, time to arrhythmia and hydrops resolution, fetal and postnatal morbidity, and mortality rates were analysed. Twenty fetuses were diagnosed with hydrops fetalis and received treatment. The mechanism of fetal tachyarrhythmia was supraventricular tachycardia in thirteen and atrial flutter in eight cases. Among the 20 fetuses treated, the overall tachycardia response rate was 90% (18/20) with the restoration of sinus rhythm in 85% (17/20) of the cases. The median time to restore sinus rhythm or to rate control of the arrhythmia was 1.5 days (range 12 hours to 13 days). Hydrops resolved in 17 of the 20 fetuses, with a median time of 12 days (range 3-21 days). Four fetuses went into spontaneous preterm birth and one fetus was delivered early due to worsening hydrops. No significant neurological morbidity was observed in surviving neonates and infants on clinical examination. There was one postnatal death due to respiratory complications of prematurity in the non-responsive supraventricular tachycardia case. High-dose flecainide and digoxin combination offers effective treatment strategy in fetuses with hydrops and tachyarrhythmia with favourable outcomes. This study may guide more realistic counselling for pregnancies complicated by tachyarrhythmia and hydrops.

In-utero treatment of prenatal thoracic abnormalities by thoraco-amniotic shunts, short and long term neuro developmental outcome: A single center experience

BackgroundSevere primary fetal hydrothorax (PFH) and fetal lung lesions (FLL) such as congenital pulmonary airway malformation (CPAM) and Bronchopulmonary sequestration (BPS) are often treated by thoraco-amniotic shunt (TAS). ObjectivesTo compare short and long-term outcome of fetuses treated by TAS due to FLL to those treated due to PFH. MethodA retrospective analysis was performed for all fetuses treated by TAS, between the years 2004–2015, evaluating the short and long term neurodevelopmental outcome. Long term neurodevelopment was additionally analyzed prospectively by Vineland adaptive behavioral scale (VABS) standardized questionnaires. Results38 fetuses were treated by 52 TAS insertions; of which 13 (35%) due to FLL and 25 due to PFH. Perinatal survival was high (87.9%) with 3 neonatal death and one termination of pregnancy (TOP). High survival rate persisted even in cases requiring recurrent shunt insertion (80% survival). There was no significant difference in short or long term outcome including perinatal survival (84% Vs 90%, P = 0.64) and hydrops resolution (91% Vs 63%, p = 0.19). Long term outcome, including rate of neurodevelopmental abnormalities (23.5% Vs 20%) and VABS score (91.3 ± 13.3 Vs 96.4 ± 14.7), were similar for both groups. ConclusionTAS insertion is effective and resulting in high perinatal survival even in cases when sequential insertion is needed. Short and long- term outcome of neonates with FLL treated by TAS are comparable to neonates treated due to PFH.

Our Experience of Immune Fetal Hydrops: its Clinical Characteristics and Perinatal Outcome.

Fetal hydrops is a serious condition which has high morbidity and mortality. Incidences of immune hydrops have decreased by manifold after introduction of anti-D immunoglobulin. Intra-uterine fetal blood transfusion revolutionized the treatment of these affected fetuses after diagnosis of immune fetal hydrops. In this study we aim to evaluate the clinical characteristics of immune hydropic fetuses and perinatal outcome after institution of intra-uterine transfusions. A retrospective study was carried out in pregnant women with immune fetal hydrops from October 2004 to December 2019 in our tertiary care hospital. After diagnosis of fetal hydrops, all the fetuses received intra-uterine transfusions. All the newborns were followed up till 3months postdelivery. All the fetuses were divided in two groups: hydrops diagnosed below 32weeks (Group A) and in second group hydrops diagnosed after 32weeks gestation (Group B). Total 63 patients were diagnosed to have hydrops during the study period. Group A had 48 fetuses and Group B had 15 fetuses. Average gestational age of diagnosis of hydrops in group A was 24.2weeks and in group B it was 32.5weeks. All the fetuses received intra-vascular intra-uterine transfusion. Pericardial effusion was found to be significantly associated with group A. Successful perinatal outcome was seen in 92% fetuses. 87% fetuses had complete resolution of hydrops before delivery. All the fetuses received phototherapy and intra-venous immunoglobulin after delivery, and 5 fetuses underwent exchange transfusion. Favourable perinatal outcome was achieved in hydropic fetuses with intra-uterine blood transfusions. Complete resolution of hydrops before delivery increases the chances of perinatal survival. The online version contains supplementary material available at 10.1007/s13224-020-01423-4.

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

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. 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. 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. 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.

Perinatal outcome of pregnancies complicated by placental chorioangioma: systematic review and meta-analysis.

To report the perinatal outcome of singleton pregnancies complicated by placental chorioangioma diagnosed on prenatal ultrasound. MEDLINE, EMBASE, CINAHL and ClinicalTrials.gov databases were searched for studies reporting the outcome of pregnancies complicated by placental chorioangioma. Inclusion criteria were singleton pregnancy diagnosed with placental chorioangioma on prenatal ultrasound, with no other associated structural anomaly. The primary outcome was perinatal mortality. Secondary outcomes included associated non-structural anomalies detected on prenatal ultrasound (including fetal hydrops, anemia, polyhydramnios, signs of hyperdynamic circulation and small-for-gestational-age (SGA) fetus), SGA at birth, composite neonatal morbidity and preterm birth. Outcome was assessed separately in pregnancies undergoing and those not undergoing fetal therapy. Subanalyses were performed according to the presence of hydrops and the size of the tumor in all pregnancies diagnosed with chorioangioma. Random-effects meta-analyses of proportions were used to analyze the data. Twenty-eight studies (161 pregnancies) were included. In pregnancies complicated by chorioangioma that did not undergo intervention, intrauterine death occurred in 8.2% (95% CI, 3.8-15.0%), while neonatal death and perinatal death occurred in 3.8% (95% CI, 1.0-8.1%) and 11.1% (95% CI, 5.0-19.4%), respectively. SGA at birth was present in 24.0% (95% CI, 13.5-36.5%) of cases, while preterm birth < 37 weeks complicated 34.1% (95% CI, 21.1-48.3%) of pregnancies. Composite neonatal morbidity occurred in 12.0% (95% CI, 4.5-22.3%) of cases. On ultrasound, signs of fetal hyperdynamic circulation were present in 21.0% (95% CI, 9.6-35.3%) of cases, while peak systolic velocity in the fetal middle cerebral artery was increased in 20.6% (95% CI, 10.9-32.3%). Subanalysis according to the size of chorioangioma, including both pregnancies that did and those that did not undergo intervention, showed a progressive increase in the occurrence of most of the outcomes explored with increasing size of the tumor. Furthermore, the prevalence of adverse perinatal outcome was high in pregnancies complicated by chorioangioma presenting with fetal hydrops. There was no randomized controlled trial comparing intervention vs expectant management in pregnancies complicated by chorioangioma with signs of fetal compromise (hydrops or hyperdynamic circulation). Overall, perinatal mortality occurred in 31.2% (95% CI, 18.1-46.1%) of fetuses undergoing in-utero therapy, and 57.3% (95% CI, 39.2-74.4%) had resolution of hydrops or hyperdynamic circulation after treatment. Placental chorioangioma is associated with adverse perinatal outcome. The size of the mass and presence of fetal hydrops are likely to be the main determinants of perinatal outcome in affected pregnancies. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Nonimmune hydrops fetalis management from the perspective of fetal cardiologists: A single tertiary center experience from Egypt.

Despite advances in managing nonimmune hydrops fetalis (NIHF), perinatal mortality is still significant. Fetal cardiac failure eventually occurs regardless of etiology. However, no previous study has addressed NIHF from fetal cardiologists' perspective. Therefore, we evaluated etiology and management of a NIHF cohort requiring fetal cardiologist consultation in a developing country. A single-center retrospective cohort study of 70 cases with NIHF that were referred to a fetal cardiology unit over four years was performed. Demographics, etiologic diagnosis, and outcomes of the cases were assessed. Antenatal management was evaluated using cardiovascular profile score (CVPS). The most frequent diagnosis was Idiopathic hydrops 42(62.6%), followed by hydrops due to cardiac diseases 19(28.4%), and 3 dead fetuses were detected at the first fetal echocardiography. Treatment of fetal tachyarrhythmia (n = 7) had 100% success rate in terms of antenatal hydrops resolution. Digoxin was used in cases of structural heart diseases, twin- twin transfusion syndrome, and dilated cardiomyopathy with perinatal mortality occurring in all cases (n = 9). In cases of idiopathic hydrops, 14 fetuses received digoxin with intrauterine hydrops resolution in 2/14 (14%) while non-treated cases had intrauterine or early neonatal death. Nonimmune hydrops is the worst complication of diverse etiologies. Limitations in resources for advanced investigations in developing countries increase the possibility of categorizing NIHF as idiopathic. Tachyarrhythmia induced hydrops can be entirely reversed with antenatal therapy while non-tachyarrhythmia fetal cardiac disease outcomes are unfavorable regardless of therapy. On the other hand, idiopathic hydrops shows a limited potential response to digoxin in utero.

Regression of Corneal Vascularization Occurring after Corneal Suturing in a Case of Acute Hydrops Managed with a Combination Therapy of Argon Laser Photocoagulation and Intrastromal Ranibizumab Injection: A Case Report

ABSTRACT Aim To report a case of acute hydrops with a large stromal cleft, in a case of keratoconus, managed with compression sutures and perfluoropropane (C3F8) descemetopexy and subsequent management of corneal vascularization in that eye with combination therapy of laser and anti-vascular endothelial growth factor (anti-VEGF). Background Acute hydrops in keratoconus occurs following a tear or rupture in the Descemet's membrane (DM), resulting in aqueous percolating into the stroma. The presence of large stromal clefts in acute hydrops has been described as a known risk factor for delayed resolution and persistent edema, which in turn can incite inflammation and vascularization and thereby adversely affect the chances of graft survival later. We describe such a case managed effectively with a combination of different treatment modalities. Case description A 12-year-old boy with keratoconus presented with acute hydrops in his left eye. The acute hydrops was managed with compression sutures along with C3F8 descemetopexy. Subsequent development of persistent deep corneal vascularization and recurrence of inflammation after the resolution of hydrops was managed with a combination of argon laser photocoagulation and intrastromal anti-VEGF injections, resulting in complete regression of the deep vascularization and resolution of inflammation. Conclusion In our case, a combination of management modalities to address severe acute hydrops and its subsequent complications resulted in complete regression of the superficial and deep vessels, thus heralding an optimal outcome for a future corneal graft. Clinical significance There are no definite guidelines for the management of acute hydrops in keratoconus. A customized and judicious combination of various treatment modalities described in the literature for acute hydrops and its sequelae can result in an accepted outcome, which can pave the way for an optimum result with elective treatments like keratoplasty later on. How to cite this article Kurian A, Reghunadhan I, Soman M, et al. Regression of Corneal Vascularization Occurring after Corneal Suturing in a Case of Acute Hydrops Managed with a Combination Therapy of Argon Laser Photocoagulation and Intrastromal Ranibizumab Injection: A Case Report. Int J Kerat Ect Cor Dis 2019;8(2):46–49.

Outcome of fetuses with congenital parvovirus B19 infection: systematic review and meta-analysis.

To explore the outcome of fetuses affected by congenital parvovirus B19 (PB19) infection, with or without signs of hydrops on ultrasound. PubMed, EMBASE and CINAHL databases were searched for studies reporting on prenatal diagnosis and outcome of fetal PB19 infection. The outcomes explored were miscarriage, perinatal death (PND), intrauterine death, neonatal death, spontaneous resolution of hydrops or fetal anemia, need for intrauterine transfusion (IUT), resolution of hydrops or anemia after transfusion, fetal loss following transfusion, abnormal brain scan after birth and abnormal neurodevelopmental outcome. Outcomes were reported according to the presence or absence of signs of hydrops on ultrasound. A subgroup analysis was performed including hydropic and non-hydropic fetuses diagnosed at < 20 weeks and ≥ 20 weeks of gestation. Meta-analyses of proportions and meta-analyses using individual-data random-effects logistic regression were performed to analyze the data. Thirty-five observational studies were included, involving 611 fetuses affected by PB19 infection. The risks of miscarriage (odds ratio (OR), 11.5; 95% CI, 2.7-49.7) and PND (OR, 4.2; 95% CI, 1.6-11.0) were higher in fetuses with PB19 infection presenting, compared with those not presenting, signs of hydrops on ultrasound. In fetuses affected by hydrops, spontaneous resolution of the infection, defined as disappearance of hydrops without need for IUT, occurred in 5.2% (95% CI, 2.5-8.8%) of cases whereas, in the group of fetuses not affected by hydrops, infection resolved in 49.6% (95% CI, 20.7-78.6%) of cases. IUT was performed in 78.7% (95% CI, 66.4-88.8%) of hydropic and in 29.6% (95% CI, 6.0-61.6%) of non-hydropic fetuses affected by congenital PB19 infection and resolution of the infection after IUT occurred in 55.1% (95% CI, 34.0-75.3%) and in 100% (95% CI, 57.3-100%) of cases, respectively. The risk of fetal loss after IUT was higher in fetuses affected compared with those not affected by hydrops (OR, 9.8; 95% CI, 2.8-34.6). The prevalence of abnormal brain imaging was 9.8% (95% CI, 2.5-21.0%) in fetuses affected and 0.0% (95% CI, 0.0-7.0%) in those not affected by hydrops, whilst the corresponding figures for abnormal neurodevelopmental outcome were 9.5% (95% CI, 2.6-20.2) and 0.0% (95% CI, 0.0-7.5), respectively; however, statistical power to assess these outcomes was inadequate due to the small number of included cases. Hydrops is the main determinant of mortality and adverse perinatal outcome in fetuses with PB19 infection. Perinatal outcome in non-hydropic fetuses is generally favorable. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Prenatal growth characteristics and pre/postnatal management of bronchopulmonary sequestrations

PurposeThe 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. MethodsA 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. ResultsMost 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. ConclusionsBPSs 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. Level of evidenceIV

Technical aspects and effectiveness of percutaneous fetal therapies for large sacrococcygeal teratomas: cohort study and literature review.

The objectives of this study were to evaluate the efficacy of minimally invasive ablation of high-risk large sacrococcygeal teratomas (SCT) and to compare the efficacy of vascular and interstitial tumor ablation. This was a retrospective multicenter study including a cohort of fetuses with high-risk large SCTs between 2004 and 2010. In addition, we performed a systematic literature review of all cases that underwent tumor ablation in order to compare the survival rates after 'vascular' and 'interstitial' ablation. Statistical analysis was conducted using Bayesian methods. In our cohort, a total of 13 fetuses had high-risk large SCT and five of them underwent tumor ablation. The estimated difference in hydrops resolution rate between the fetal intervention and the no fetal intervention groups was 44.6% (95% credibility interval, 1.5 to 81.0%; Pdiff> 0 = 97.9%). The estimated difference in survival rate between the fetal intervention and the no fetal intervention groups was 31.0% (13.9 to 48.1%; Pdiff> 0 = 99.9%). We analyzed our five cases together with 28 cases from the literature and estimated the difference in survival rate between the vascular and interstitial ablation groups as 19.8% (-13.1 to 50.1%; Pdiff> 0 = 88.3%). The estimated difference in hydrops resolution rate between the vascular and the interstitial ablation groups was 36.7% (-5.7 to 72.7%; Pdiff> 0 = 95.5%). Minimally invasive surgery seems to improve perinatal outcome in cases of high-risk large fetal SCT. Our findings suggest that 'vascular' ablation may improve outcome and may be more effective than 'interstitial' tumor ablation, but this hypothesis needs further investigation in a larger multicenter prospective study. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

Effect of single and multiple courses of maternal betamethasone on prenatal congenital lung lesion growth and fetal survival

PurposeAdministration of maternal betamethasone (BMZ) is a therapeutic option for fetuses with large microcystic congenital lung lesions at risk for, or causing, hydrops. Not all fetuses respond to a single course of BMZ. We review our experience with the use of single and multiple courses of maternal BMZ for the management of these patients. MethodsA retrospective review of fetuses with congenital lung lesions managed with maternal BMZ from 2003 to 2014 was performed. ResultsForty-three patients were managed with prenatal steroids (28 single course, 15 multiple courses). Single course recipients demonstrated a reduction in lesion size and resolution of hydrops in 82% and 88% of patients respectively compared to 47% and 56% in recipients of multiple steroid courses. Survival of multiple course patients (86%) was comparable to that of single course patients (93%) and improved compared to non-treated historical controls. Multiple course recipients demonstrated an increased need for open fetal surgery and postnatal surgery at a younger age. ConclusionFetuses who fail to respond to a single course of BMZ may benefit, as indicated by hydrops resolution and improved survival, from additional courses. However, failure to respond is indicative of a lesion which may require fetal or immediate neonatal resection.

Endolymphatic Hydrops Reversal following Acetazolamide Therapy: Demonstration with Delayed Intravenous Contrast-Enhanced 3D-FLAIR MRI.

Endolymphatic hydrops, the primary pathologic alteration in Menière disease, can be visualized by using delayed intravenous contrast-enhanced 3D-FLAIR MR imaging. It is not known whether MR imaging-demonstrable changes of hydrops fluctuate with disease activity or are fixed. We describe the results of baseline and posttreatment MR imaging studies in a group of subjects with Menière disease with hydrops who were treated with acetazolamide. Seven subjects with untreated Menière disease with MR imaging evidence of hydrops had repeat MR imaging during acetazolamide treatment. Symptoms and imaging findings were assessed at each time point. Five subjects showed symptom improvement, of whom 3 had improvement or resolution of hydrops. One subject had recurrent symptoms with recurrent hydrops after discontinuing therapy. Two had unchanged hydrops despite symptom improvement. Subjects with unchanged symptoms had unchanged hydrops. Hydrops reversal may be seen with acetazolamide treatment in Menière disease. MR imaging may provide an additional biomarker of disease.

Thoracoamniotic shunts for the management of fetal lung lesions and pleural effusions: a single-institution review and predictors of survival in 75 cases

PurposeHydrops and pulmonary hypoplasia are associated with significant morbidity and mortality in the setting of a congenital lung lesion or pleural effusion (PE). We reviewed our experience using in utero thoracoamniotic shunts (TA) to manage fetuses with these diagnoses. MethodsA retrospective review of fetuses diagnosed with a congenital lung lesion or pleural effusion who underwent TA shunt placement from 1998–2013 was performed. ResultsNinety-seven shunts were placed in 75 fetuses. Average gestational age (± SD) at shunt placement and birth was 25±3 and 34±5weeks. Shunt placement resulted in a 55±21% decrease in macrocystic lung lesion volume and complete or partial drainage of the PE in 29% and 71% of fetuses. 69% of fetuses presented with hydrops, which resolved following shunt placement in 83%. Survival was 68%, which correlated with GA at birth, % reduction in lesion size, unilateral pleural effusions, and hydrops resolution. Surviving infants had prolonged NICU courses and often required either surgical resection or tube thoracostomy in the perinatal period. ConclusionTA shunts provide a therapeutic option for select fetuses with large macrocystic lung lesions or PEs at risk for hydrops and/or pulmonary hypoplasia. Survival following shunting depends on GA at birth, reduction in mass size, and hydrops resolution.

The many faces of hydrops

PurposeFetal hydrops arises from multiple disease processes and can portend a grim prognosis. We reviewed our experience with hydropic fetuses to understand relevant antenatal anatomic and physiologic predictors of survival. MethodsWe reviewed fetal ultrasounds and echocardiograms of hydropic fetuses evaluated from 1996 to 2013. ResultsOverall neonatal survival in 167 fetuses was 44% (range, 0–75%) and was influenced by the underlying disease process. The anatomic distribution of fluid varied and was not significantly different between survivors and nonsurvivors. Univariate analysis indicated that resolution of hydrops and delivery at a later gestational age were predictive of survival (OR: 5.7 (95% CI: 2.5–13.2) and OR: 1.3 (95% CI: 1.1–1.4), respectively). Fetal intervention also improved survival in some diseases. Echocardiograms were reviewed to group fetuses with similar cardiac physiology and defined categories with high or low/normal cardiothoracic ratio (CTR). Among patients with a high CTR, the cardiovascular profile score was predictive of survival (p=0.009). ConclusionSurvival in hydrops depends on the underlying disease, available fetal therapies to resolve hydrops, and the gestational age of delivery and not on the specific anatomic manifestations of hydrops. In hydropic fetuses with high CTRs, the cardiovascular profile score may be a useful prognostic indicator.

Giant Chorioangiomas: Perinatal Outcomes and Techniques in Fetoscopic Devascularization

Objective: To evaluate the intrauterine management and perinatal outcome of pregnancies complicated by giant placental chorioangioma (>4 cm) and elaborate on various devascularization techniques. Materials and Methods: Retrospective review of 10 cases of giant placental chorioangioma evaluated between January 2005 and August 2012. Maternal demographics, prenatal imaging, response to fetoscopic treatment, obstetrical complications, and perinatal outcomes were evaluated. Results: Overall survival was 80%. Seven (70%) cases were associated with obstetrical complications, including polyhydramnios (n = 7), non-immune hydrops (n = 3), and high cardiac output state (n = 5). Five patients underwent fetoscopic devascularization at a mean gestational age of 24.03 weeks with 80% survival. The tumors were devascularized by bipolar coagulation (n = 1), combination of bipolar and diode laser (n = 2), bipolar and radiofrequency ablation (n = 1), and surgical clip application (n = 1). Postoperatively, all survivors had resolution of hydrops and high cardiac output states with survival beyond 60 days of life. Discussion: Fetoscopic devascularization is indicated for high cardiac output states or non-immune hydrops and may require multiple techniques including bipolar coagulation, clip application, and/or laser to interrupt arterial inflow and devascularize the mass.