Ultrasound in Obstetrics & GynecologyAccepted Articles Letter to the Editor Ruptured saccular limited dorsal myeloschisis: good indication for fetal repair C. Gine, Corresponding Author C. Gine [email protected] Pediatric Surgery Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainCorrespondence to: Dr. Carlos Giné MD PhD. Vall d'Hebron Barcelona Hospital Campus. Passeig de la Vall d'Hebron 119-129. 08035 Barcelona. Spain., e-mail: [email protected]Search for more papers by this authorN. Maiz, N. Maiz Maternal-Fetal Medicine Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this authorS. Arévalo, S. Arévalo Maternal-Fetal Medicine Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this authorC. Rodó, C. Rodó Maternal-Fetal Medicine Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this authorM. López, M. López Pediatric Surgery Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this authorE. Carreras, E. Carreras Maternal-Fetal Medicine Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this author C. Gine, Corresponding Author C. Gine [email protected] Pediatric Surgery Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainCorrespondence to: Dr. Carlos Giné MD PhD. Vall d'Hebron Barcelona Hospital Campus. Passeig de la Vall d'Hebron 119-129. 08035 Barcelona. Spain., e-mail: [email protected]Search for more papers by this authorN. Maiz, N. Maiz Maternal-Fetal Medicine Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this authorS. Arévalo, S. Arévalo Maternal-Fetal Medicine Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this authorC. Rodó, C. Rodó Maternal-Fetal Medicine Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this authorM. López, M. López Pediatric Surgery Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this authorE. Carreras, E. Carreras Maternal-Fetal Medicine Department, Vall d'Hebron Barcelona Hospital Campus, Barcelona, SpainSearch for more papers by this author First published: 22 August 2023 https://doi.org/10.1002/uog.27457 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1002/uog.27457. AboutPDF ToolsExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat Supporting Information Filename Description uog27457-sup-0001-FigureS1.jpgimage/jpg, 4.6 MB Figure S1 – MRI pre and post: Comparison of MRI before and after surgery. A: MRI at 24 weeks: A1: axial view of lateral ventricles of 9 mm; A2: sagittal view of descended tentorium, collapse of subarachnoid spaces, small Cisterna Magna. B: Post-surgical MRI at 32+1 weeks: B1: axial view of posterior horns of lateral ventricles measuring 6 mm; B2: sagittal view of a normal-looking posterior fossa. uog27457-sup-0002-VideoS1.mp4MPEG-4 video, 23.7 MB Videoclip S1 – US initial.mp4: Video of the transabdominal ultrasound of fetus with a saccular limited dorsal myeloschisis at 21+6 weeks. uog27457-sup-0003-VideoS2.mp4MPEG-4 video, 47.8 MB Videoclip S2 – US broken cyst.mp4: Video of the transabdominal ultrasound of the patient at 23+6 weeks showing the rupture of the cyst. The spinal cord remains well neurulated and descends up to L5 anatomical level. uog27457-sup-0004-VideoS3.mp4MPEG-4 video, 128.5 MB Videoclip S3 – surgical video.mp4: Surgical fetoscopic intervention at 25+5 weeks. Reconstruction in three layers (dural, myofascial and skin). Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article. Accepted ArticlesAccepted, unedited articles published online and citable. The final edited and typeset version of record will appear in the future. RelatedInformation
ABSTRACTObjectivesFirst, to investigate the correlation between prenatal presurgery anatomical and motor levels of the lesion with motor level at birth in cases undergoing prenatal repair of open spina bifida and, second, to identify factors leading to a loss of two or more motor levels between the presurgery and postnatal assessments.MethodsThis was an observational study of singleton pregnancies undergoing prenatal repair of open spina bifida, conducted between March 2011 and May 2022. All fetuses underwent an ultrasound assessment at 20–24 weeks of gestation to determine the motor and anatomical levels of the lesion before surgery. The anatomical level of the lesion was defined as the highest open posterior vertebral arch. The motor level was determined by systematic observation of the lower limb movements and was defined as the most distal active muscle present. Prenatal repair was performed at 23–26 weeks. At birth, motor level was assessed by a rehabilitation specialist by physical examination. Cases of intrauterine death or termination of pregnancy and those delivered at other sites were excluded from the neonatal assessment. The agreement between presurgery motor level and motor level at birth, and between presurgery anatomical level and motor level at birth, was assessed using the weighted kappa index (wκ). Logistic regression analysis was used to assess factors leading to a loss of two or more motor levels between the presurgery and postnatal assessments.ResultsPresurgery motor and anatomical levels were assessed in 61 fetuses at a median gestational age of 22.7 (interquartile range (IQR), 21.6–24.4) weeks. Prenatal repair was performed at a median gestational age of 24.6 (IQR, 23.7–25.7) weeks. Motor level at birth was assessed in 52 neonates after exclusion of nine fetuses due to loss to follow‐up or fetal loss. There was moderate agreement between presurgery motor level and motor level at birth (wκ = 0.42; 95% CI, 0.21–0.63), with a median difference of 0 (IQR, –2 to 9) levels. Factors leading to a loss of two or more motor levels between the presurgery ultrasound assessment and postnatal examination were higher presurgery anatomical level (odds ratio (OR), 0.59 (95% CI, 0.35–0.98); P = 0.04) and larger difference between the anatomical and motor levels before surgery (OR, 1.85 (95% CI, 1.12–3.06); P = 0.017). None of the other ultrasound, surgery‐related or neonatal variables assessed was associated significantly with a loss of two or more motor levels. There was slight agreement between the presurgery anatomical level of the lesion and motor level at birth (wκ = 0.07; 95% CI, –0.02 to 0.15).ConclusionsThere is moderate agreement between fetal motor level of the lesion before prenatal repair of open spina bifida and motor level at birth, as opposed to only slight agreement between presurgery anatomical level and motor level at birth. A loss of two or more motor levels between the presurgery and postnatal assessments is associated with a higher presurgery anatomical level and with a larger difference between the presurgery anatomical and motor levels. Consequently, motor level, rather than the anatomical level, should be used for prenatal counseling. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
The main objective of this study is to assess the prevalence of chromosomal abnormalities in fetuses with cleft lip (CL) and cleft lip and palate (CLP). Secondary objectives were to study the prevalence of chromosomal abnormalities in a) isolated CL/CLP and those with associated anomalies; b) CL versus CLP; c) unilateral, bilateral or central CL/CLP. This is an observational single cohort study between 2010 and 2020. The study population were fetuses diagnosed with CL or CLP, and those without chromosomal study were excluded from the analysis. The chromosomal study included QF-PCR, karyotype and/or array. The result was classified as normal, variant of uncertain significance (VUS) or pathogenic. Risk factors associated with pathogenic results were analysed by logistic regression analysis. 134 fetuses were included for the analysis. In 31 (23.1%) fetuses, pathogenic chromosomal abnormalities were identified, in 3 (2.2%) cases non-pathogenic findings (2 cases of VUS and 1 case of balanced translocation from paternal origin), and in 100 (74.6%), the result was normal. In 78 (58.2%) fetuses, the defect was isolated, and from these, one (1.3%) had a pathogenic copy number variant (CNV), 3 (3.8%) a non-pathogenic finding, and 74 (94.9%) a normal result. In 30 (54%) of the 56 cases with associated anomalies, the result was pathogenic and 26 (46%) were normal. Within the isolated cases, 66 were unilateral (1 pathogenic, 3 VUS and 62 normal), 10 bilateral (10 normal), and 2 central (2 normal). 10 were CL cases (1 VUS and 9 normal), and 68 were CLP (1 pathogenic, 12 VUS and 65 normal). Logistic regression analysis showed that the absence of other abnormalities (isolated CL/CLP) reduced the risk for chromosomal abnormalities (OR 0.011, 95% CI 0.001 to 0.057, p < 0.001), but not laterality (p = 0.189), or CL/CLP (p = 0.170). Overall, 23% of CL/CLP are associated with pathogenic chromosomal abnormalities. The absence of additional fetal abnormalities reduces this risk to 1%.
We aimed to describe predictors of fetal/neonatal loss, neonatal morbidity and the need for surgery in fetuses diagnosed with an abdominal cyst. This is an observational retrospective study performed at Vall d'Hebron Hospital, including pregnant women ≥18 years and diagnosis of fetal abdominal cyst. Primary outcomes were fetal/neonatal loss, neonatal morbidity, and need for postnatal surgery. Univariate logistic regression analysis was used to study factors associated with fetal and neonatal loss, neonatal morbidity, and surgical treatment. 82 cases were included. Median gestational age at diagnosis was 31.1 weeks (12-39 range). 7 (8.5%) cases were diagnosed in the 1st trimester, 28 (34.1%) in the 2nd trimester and 47 (57.3%) in the 3rd trimester. Fetal/neonatal loss was observed in 10/82 (12.2%) cases, neonatal morbidity in 10/75 (13.3%) neonates and 16/75 (21.3%) neonates underwent postnatal surgery. Table 1 shows risk factors associated with these adverse perinatal outcomes. The main risk factors associated with adverse perinatal outcome are associated anomalies, trimester at diagnosis, fetal gender, and bowel location of the cyst.
Objectives: The aim was to describe pregnancy outcomes after Zika virus (ZIKV) infection in a nonendemic region. Methods: According to the Spanish protocol issued after the ZIKV outbreak in Brazil in 2015, all pregnant women who had travelled to high-burden countries were screened for ZIKV. Serological and molecular tests were used to identify ZIKV-infected pregnant women. They were classified as confirmed ZIKV infection when reverse transcription (RT) PCR tested positive, or probable ZIKV infection when ZIKV immunoglobulin M and/or immunoglobulin G and ZIKV plaque reduction neutralization tests were positive. Women found positive using molecular or serological tests were prospectively followed-up with ultrasound scans and neurosonograms on a monthly basis until delivery; magnetic resonance imaging and amniotic fluid testing were performed after signed informed consent. Samples of placenta, and fetal and neonatal tissues were obtained. Results: Seventy-two pregnant women tested positive for ZIKV infection: ten were confirmed by RT-PCR, and 62 were probable cases based on serological tests. The prevalence of adverse perinatal outcomes was 33.3% (three out of nine, 95% CI 12.1-64.6%): two cases of congenital ZIKV syndrome (CZS) and one miscarriage, all born to women infected in the first trimester of gestation. All ZIKV-confirmed women had persistent viraemias beyond 2 weeks (median 61.50 days; IQR 35.50-80.75). Amniotic fluid testing was only positive in the two fetuses with anomalies. Conclusion: The prevalence of perinatal adverse outcomes for women with ZIKV-confirmed infection was 33.3%. Amniocentesis for ZIKV RT-PCR is recommended when fetal abnormalities are found. Intensive prenatal and postnatal follow-up of ZIKV-infected pregnancies is advised in confirmed cases. (C) 2019 European Society of Clinical Microbiology and Infectious Diseases. Published by Elsevier Ltd. All rights reserved.
We describe a case of a pregnant woman with Zika virus (ZIKV) infection and a foetus with severe brain malformations. ZIKV tested positive in amniotic fluid at 19 weeks but was negative at delivery. The newborn did not meet the case definition of congenital ZIKV syndrome because neither ZIKV RNA nor IgM antibodies were detected; however, prenatal brain lesions were confirmed after birth (Graphical Abstract).
ABSTRACTObjectiveFetoscopy for closure of open neural tube defects (NTD) remains controversial, as the use of patches or single‐layer closure is not considered to meet the standards of good neurosurgical reconstruction. In this study, we describe a fetoscopic two‐layer (myofascial and skin) closure technique for the treatment of NTD in five patients and report the preliminary anatomical outcome at birth.MethodsFrom February to September 2017, five pregnant women with a fetus with a NTD, including three cases of myelomeningocele and two cases of myelocele, were operated on using a fetoscopic two‐layer closure technique. In this technique, with the uterus exteriorized and using three 10‐Fr ports, the placode is dissected from the surrounding tissue and detethered, removing the cystic tissue. The skin is undermined by blunt dissection and the defect is sutured to the midline in two layers (myofascial and skin) using a running 4/0 resorbable barbed suture.ResultsMedian gestational age at the procedure was 24 + 3 (range, 23 + 5 to 27 + 3) weeks. Surgery was successful in all cases, without any intraoperative complications. Median time in surgery was 180 (range, 140–180) min and median time for fetoscopy was 105 (range, 65–120) min. In terms of obstetric complications, three cases of premature rupture of membranes and one case of chorioamnionitis were recorded. Median gestational age at delivery was 34 + 1 (range, 25 + 4 to 37 + 2) weeks and two patients delivered vaginally. The closed defect was watertight with good quality tissue in all cases.ConclusionFetoscopic two‐layer closure of NTD may improve the quality of the tissue covering the defect, diminishing the need for postnatal surgical revision, and preserving the well‐documented beneficial effects of prenatal closure on the neural tissue and hindbrain herniation. However, this technique may not be appropriate for those cases with wide diastasis of the myofascial layer or with a low quantity of available tissue. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.
To evaluate prenatal predictors of postnatal survival in fetuses diagnosed with TAPS. In a multicentre retrospective cohort analysis, we reviewed all fetuses with diagnosed with TAPS in three fetal centres (Barcelona, Spain; São Paulo, Brazil and Toulouse, France) between 2009 and 2016. A total of 42 cases (39 prenatally diagnosed and 3 postnatally diagnosed) were included in the present study. All cases were monochorionic diamniotic twin pregnancies. The mean gestational age at the time of diagnosis was 26.5 ± 4.9 weeks. The mean middle cerebral artery peak systolic velocity for ex-recipients and ex-donors were .78 ± .16 MoM (.50-1.10) and 1.83 ± .40 MoM (.80-2.90), respectively. The spontaneous TAPS and post-laser TAPS were present in 27 (64%) and 15 (36%) fetuses, respectively. With regards to fetal therapy, 17 fetuses did not receive any kind of fetal therapy while 22 cases underwent at least a method of fetal intervention before delivery. The mean gestational age at treatment for TAPS was 25.4 ± 4.0 weeks. Interventions included intrauterine transfusion (n = 7), laser ablation (n = 7), amniodrainage (n = 2), and cord occlusion (n = 1). In 9 cases elective preterm delivery was performed due to TAPS. The rate of preterm delivery was not higher in fetuses underwent fetal therapy (P > .05). The mean gestational age at delivery was 32.7 ± 3.2 weeks. Due twin demise, due twin survival and at least one survival occurred in 4 (10%). 30 (70%) and 8 (20%) cases, respectively. The survival rate was not different between treated and untreated fetuses. (OR: 0.29, 95% CI: 0.47-52.0). Because of the small number of reported cases, the natural history, role of fetal therapy, and prognostic factors of TAPS remain not well understood. Further studies are needed to determine different aspects of this condition and minimise the morbidity and mortality of the fetuses and the risk of the mother.
We describe here the first pregnant women described with Zika virus (ZIKV) infection in Europe. One of the cases was a probable coinfection with dengue virus (DENV) and the most prolonged viraemia ever reported. We communicate the protocol established in our setting as a response for the international emergency. The first patient was a Colombian woman in her 40s living in Spain who had travelled to Colombia in the months of December 2015 and January 2016. Three days after her return, at approximately 10 weeks’ gestation, she observed a maculopapular, nonconfluent rash affecting her trunk and limbs, with no fever or other concurrent symptoms. Fifteen days later, she contacted the outpatient service of the International Health Unit at Vall d’Hebron Hospital, Barcelona, where an arboviral infection was suspected and a serum sample was taken. To confirm ZIKV diagnosis, anti-ZIKV immunoglobulin (Ig) M and IgG antibodies (Arboviral Fever Mosaic, Euroimmun, Germany) and specific retrotranscriptase (RT) PCR, RealStar ZIKV RT-PCR Kit and a modification from Balm et al. [[1]Balm M.N. Lee C.K. Lee H.K. Chiu L. Koay E.S. Tang J.W. A diagnostic polymerase chain reaction assay for Zika virus.J Med Virol. 2012 Sep; 84: 1501-1505Crossref PubMed Scopus (146) Google Scholar]) were performed at the Spanish National Microbiology Center at Instituto Carlos III, Madrid. RT-PCR and ZIKV IgG were positive and ZIKV IgM was negative. Anti–chikungunya virus (CHKV) antibodies were negative. Anti-DENV IgM and IgG (Dengue VirClia Monotest, Vircell, Granada, Spain) showed positive results. It was considered a confirmed case of ZIKV infection according to approved diagnostic criteria. The second patient was a Spanish woman in her 30s who had traveled to Colombia during similar dates. Ten days after her arrival to Colombia, at 22 weeks’ gestation, she experienced a rash on the face and trunk that was self-limited in 12 hours, with no other concurrent symptoms. She contacted the Unit of International Health upon her return, 20 days later, and a serum sample was obtained for DENV, CHKV and ZIKV serology. Anti-CHKV IgG/IgM and anti-DENV IgM were negative, but anti-DENV IgG was positive. For ZIKV, both IgM and IgG were positive, and serum neutralization confirmed the positive results, discarding cross-reaction with other viruses. It was considered a case of ZIKV infection according to the approved diagnostic criteria. The same day the results were known, obstetricians assessed both patients. Prenatal ultrasounds, including neurosonography, were performed. The gestational age of the women’s fetuses were 14 and 27 weeks, respectively; no apparent foetal malformations were detected. Both pregnant women are currently under strict supervision. The working group designed and launched a protocol to diagnose and follow up all pregnant women coming from endemic ZIKV areas in order to be able to promptly detect foetal microcephaly and ZIKV infection among women at risk. The protocol is described in Fig. 1. In the first case, specific RT-PCR detected viraemia 15 days after the onset of symptoms; this was confirmed by a second RT-PCR. Viraemia for ZIKV is usually shorter [[2]Hayes E.B. Zika virus outside Africa.Emerg Infect Dis. 2009; 15: 1347-1350Crossref PubMed Scopus (611) Google Scholar]; further studies are needed to assess the likely time ranges. DENV IgM was also positive; coinfection cannot be excluded. Further studies are needed to determine if these circumstances may be risk factors for foetal involvement. In the second case, in which RT-PCR was not performed and DENV IgG was also positive, seroneutralization was performed in order to confirm the case, as serologic cross-reactivity between ZIKV and DENV has been reported [[3]Lanciotti R.S. Kosoy O.L. Laven J.J. Velez J.O. Lambert A.J. Johnson A.J. et al.Genetic and serologic properties of Zika virus associated with an epidemic, Yap State, Micronesia, 2007.Emerg Infect Dis. 2008; 14: 1232-1239Crossref PubMed Scopus (1599) Google Scholar]. A complete follow-up has been set until delivery because the impact on the foetus is not yet well established [[4]Adibi J.J. Marques Jr., E.T. Cartus A. Beigi R.H. Teratogenic effects of the Zika virus and the role of the placenta.Lancet. 2016; 387: 1587-1590Abstract Full Text Full Text PDF PubMed Scopus (124) Google Scholar]. Moreover, these women live in Europe, far from the endemic areas for arbovirosis. These cases provide information about the evolution of the infection without reexposure to the virus. Key points to complete the appropriate diagnosis are a good clinical history adapted to every moment to the changing situation, laboratory capacity to quickly implement new techniques and a timely and appropriate follow-up of the affected women. The authors particularly acknowledge the Spanish Network on Tropical Diseases Research, especially groups RD12/0018/0021 and RD12/0018/0006. All authors report no conflicts of interest relevant to this article.
OBJECTIVE:To investigate the value of fetal stomach position in predicting postnatal outcome in left-sided congenital diaphragmatic hernia (CDH) with and without fetoscopic endoluminal tracheal occlusion (FETO). METHODS:This was a retrospective review of CDH cases that were expectantly managed or treated with FETO, assessed from May 2008 to October 2013, in which we graded, on a scale of 1-4, stomach position on the four-chamber view of the heart with respect to thoracic structures. Logistic regression analysis was used to investigate the effect of management center (Paris, Brussels, Barcelona, Milan), stomach grading, observed-to-expected lung area-to-head circumference ratio (O/E-LHR), gestational age at delivery, birth weight in expectantly managed CDH, gestational ages at FETO and at removal and period of tracheal occlusion, on postnatal survival in CDH cases treated with FETO. RESULTS:We identified 67 expectantly managed CDH cases and 47 CDH cases that were treated with FETO. In expectantly managed CDH, stomach position and O/E-LHR predicted postnatal survival independently. In CDH treated with FETO, stomach position and gestational age at delivery predicted postnatal survival independently. CONCLUSION:In left-sided CDH with or without FETO, stomach position is predictive of postnatal survival.
We read with great interest the article by Ruano and coworkers1 regarding the use of the ‘Solomon technique’ as part of the laser procedure for the treatment of severe twin–twin transfusion syndrome (TTTS). The authors quote an article by Chalouhi et al.2 as the first description of this method. We would like to point out that our esteemed colleagues at Leiden University described this method 3 years earlier3. The nomenclature of the technique was derived from the Hebrew biblical passage (1 Kings 3.16–28) that describes King Solomon's offer to divide a child by the sword in order to settle a dispute between two mothers, each of whom claimed the child to be their own (Enrico Lopriore, pers. comm.). It was proposed that the Solomon method should be applied at the time of laser photocoagulation as a means of preventing residual placental anastomoses, to avert subsequent twin anemia–polycythemia sequence (TAPS) or recurrent TTTS. A multicenter randomized clinical trial was completed recently; we await publication of the results. Ruano et al.1 analyzed their two cohorts for TAPS and recurrent TTTS. Most laser centers in such large countries as the USA and Brazil serve a large referral population base with great geographic distribution. As such, complete perinatal follow-up is difficult. For this reason, we feel it would be valuable to know the answers to a few relevant questions. What percent of their laser patients were lost to follow-up? How many of their six cases of TAPS were diagnosed by discordant middle cerebral artery velocities? Of these cases, how many exhibited discordant neonatal hemoglobins at delivery? Complete ‘Solomonization’ can be difficult, particularly with anterior placentation. In how many cases were the authors unsuccessful in completing this technique? Ruano et al. noted enhanced dual survival when the Solomon method was employed. The dual neonatal survival rate in the control group was 46%, a rate that is lower than that reported by other experienced centers4. The Solomon group was noted to have a dual neonatal survival rate of 84%. Since this technique was not introduced at three of the centers until later in their experience, we wonder whether this difference could be attributed to the learning curve, which has been well documented5. K. J. Moise Jr*, A. Johnson, M. W. Bebbington and R. Papanna Department of Obstetrics, Gynecology and Reproductive Medicine, UT Health - School of Medicine, Texas Fetal Center, Children's Memorial Hermann Hospital, Houston, TX, USA *Correspondence. (e-mail: Kenneth.J.Moise@uth.tmc.edu)
Congenitally corrected transpositions of great arteries (ccTGA) is an uncommon cardiac defect. Clinical presentation can be uncertain and will depend on associated cardiac lesions. The aim of this study is to analyse the prenatal diagnosis and long-term postnatal outcome of ccTGA. It is a retrospective observational study of 53 cases of ccTGA diagnosed in our centre from 1993 to 2012. In 13 cases (24%) ccTGA was diagnosed prenatally. Median gestational age was 20.6 weeks. Among the fetal cases, Isolated ccTGA was present in 6 patients (46%), 2 had a ventricular septal defect (15%), 3 Ebstein's anomaly (23%) and 4 with pulmonary stenosis (31%). One case presented extracardiac abnormalities (diaphragmatic hernia). There weren't any chromosomal abnormalities. In 7 cases parents elected TOP,28% of them were isolated. In all TOP necropsy confirmed the diagnosis. The prenatal outcome of the cases that continued with pregnancy was favorable. About the 46 cases with postnatal evolution, at a median follow-up time of 28 years (0.2 to 66 years), 35% of patients developed a complete heart block, requiring a permanent pacemaker (35% of them before they were 18 years old). 16 patients (35%) underwent various surgical procedures including: 2 Double switch (Senning + Jatene), 1 Senning + Rastelli, 6 repairs of ventricular-septal defect, of whom 4 also needs outflow tract obstruction repair, 1 of them with Blalock-Taussing shunt, 3 univentricular surgeries (Fontan) and 4 valve replacements due to systemic tricuspid regurgitation. The long-term survival was 98%, 45% of patients required drug treatment and 93% were in NYHA functional class I or II. We report a large number of cases of ccTGA, with a long-term follow-up, (0.2 to 66 years). The long-term survival of ccTGA is high (98%), with a good quality of life and an acceptable morbidity. However the amount of cases in which parents elected to terminate pregnancy is high (54%), despite they are isolated cases.
Primary: to evaluate whether placement of a vaginal pessary in twin pregnancy with short cervical length (≤25 mm) measured at 23 weeks leads to a reduction in the incidence of spontaneous delivery before 34 completed weeks compared with expectant management. Secondary: to quantify and compare the needs of admission at hospital and needs of tocolysis and other treatments during pregnancy between the two study groups. To assess morbidity and mortality in newborn twins comparing the two study groups. To evaluate the incidence of maternal adverse effects secondary to the pessary placement. A multicentee, randomized, open-controlled trial (PECEP-TWINS Trial) (1:1) was undertaken to ascertain whether the insertion of a cervical pessary in twin's pregnant women with a short cervix identified by routine transvaginal scanning at 20-23 weeks' gestation reduces the rate of early preterm delivery. The PECEP-TWINS Trial was undertaken in 5 hospitals in Spain. Cervical length was measured in 2,287 women; 137 pregnant women with a cervical length ≤ 25 mm (of 154 detected with a short cervix) were randomly assigned to receive a cervical pessary or expectant management without a cervical pessary (1:1 ratio). Three patients were lost to follow-up. Because of the nature of the intervention, this study was not blinded. The primary outcome was spontaneous delivery before 34 weeks of gestation. Neonatal morbidity and mortality were also evaluated. All analyses were by intention to treat. This study is registered as ClinicalTrials.gov NCT01242410. Spontaneous delivery before 34 weeks of gestation was significantly less frequent in the pessary group than in the expectant management group (11/68 (16.2%) vs 17/66 (25.7%); p 0.0001. No differences were observed in neonatal morbidity or mortality. No serious adverse effects associated with the use of a cervical pessary were observed. The insertion of a cervical pessary reduces the rate of spontaneous early preterm delivery in women with a short cervix.
Prenatal diagnosis of coarctation of the aorta (CoAo) remains a challenge. In order to predict those who will need surgical postnatal approach some sonographic features have been described. We have analyzed the postnatal outcome of all cases with cardiac disproportion in our center using these sonographic markers. Between 2005 and 2014, 95 consecutive fetuses were diagnosed of isolated ventricular and/or great arterial disproportion. (3 underwent TOP, 1 patient incomplete follow-up). The following sonographic features were analyzed: Isthmal diameter Z-score, ratio duct/isthmus diameter, presence of coarctation shelf, Isthmal flow disturbance, PLSCV, small VSD and bicuspid aortic valve. We had also recorded postnatal variables. Logistic regression analysis was used to compare association between surgery and these sonographic predictors. When analysing in 91 fetuses the songraphic features above mentioned we prenatally diagnosed CoAo in 57 fetuses (62%). Among fetuses with disproportion, coarctation was confirmed postnatally in 33 (PPV36.2%) and in 31 of suspected prenatal CoAo (PPV54.4%). 27 patients with disproportion (PPV29,6%) and 24 with suspected CoAo (PPV42%) required surgery. After a median follow-up of 4.3 years, 96,8% surveillance (3 deaths), 12 patients (36.3%) develop severe recoarctation (91,6% with Isthmal diameter Z-score < −2, (p 0.001)). The most predictor value of surgical CoAo was Isthmal diameter Z-score < −2 (p 0.03) and ratio duct/isthmus diameter >1.3, coartation shelf was identified in 23% of patients (86,7% with confirmed postnatally CoA (p 0.001)). We haven't found any statistically significance relation between PLSCV, VSD and CoAo. Only 6 patients had an aortic bicuspid valve and all of them required surgery for CoA. Isthmus Z-Score < −2, ratio duct/isthmus diameter >1.3 and presence of Shelf are useful prenatal sonographic features for prediction of surgical approach and postnatal outcome of prenatal diagnosis of CoA.
ABSTRACTObjectiveTo document perinatal outcomes following use of the ‘Solomon technique’ in the selective photocoagulation of placental anastomoses for severe twin–twin transfusion syndrome (TTTS).MethodsBetween January 2010 and July 2012, data were collected from 102 consecutive monochorionic twin pregnancies complicated by severe TTTS that underwent fetoscopic laser ablation at four different centers. We compared outcomes between subjects that underwent selective laser coagulation using the Solomon technique (cases) and those that underwent selective laser coagulation without this procedure (controls).ResultsOf the 102 pregnancies examined, 26 (25.5%) underwent the Solomon technique and 76 (74.5%) did not. Of the 204 fetuses, 139 (68.1%) survived up to 30 days of age. At least one twin survived in 82 (80.4%) pregnancies and both twins survived in 57 (55.9%) pregnancies. When compared with the control group, the Solomon‐technique group had a significantly higher survival rate for both twins (84.6 vs 46.1%; P < 0.01) and a higher overall neonatal survival rate (45/52 (86.5%) vs 94/152 (61.8%); P < 0.01). Use of the Solomon technique remained independently associated with dual twin survival (adjusted odds ratio (aOR), 11.35 (95% CI, 3.11–53.14); P = 0.0007) and overall neonatal survival rate (aOR, 4.65 (95% CI, 1.59–13.62); P = 0.005) on multivariable analysis. There were no cases of recurrent TTTS or twin anemia–polycythemia sequence (TAPS) in the Solomon‐technique group.ConclusionsUse of the Solomon technique following selective laser coagulation of placental anastomoses appears to improve twin survival and may reduce the risk of recurrent TTTS and TAPS. Our data support the idea of performing a randomized controlled trial to evaluate the effectiveness of the Solomon technique. Copyright © 2013 ISUOG. Published by John Wiley & Sons Ltd.