Perinatal outcome of monochorionic triamniotic triplet pregnancy: multicenter cohort study.

OBJECTIVE: Monochorionic (MC) triplet pregnancies are extremely rare and information on these pregnancies and their complications is limited. We aimed to investigate the risk of early and late pregnancy complications, perinatal outcome and the timing and methods of fetal intervention in these pregnancies. METHODS: This was a multicenter retrospective cohort study of MC triamniotic (TA) triplet pregnancies managed in 21 participating centers around the world from 2007 onwards. Data on maternal age, mode of conception, diagnosis of major fetal structural anomalies or aneuploidy, gestational age (GA) at diagnosis of anomalies, twin-to-twin transfusion syndrome (TTTS), twin anemia-polycythemia sequence (TAPS), twin reversed arterial perfusion (TRAP) sequence and or selective fetal growth restriction (sFGR) were retrieved from patient records. Data on antenatal interventions were collected, including data on selective fetal reduction (three to two or three to one), laser surgery and any other active fetal intervention (including amniodrainage). Data on perinatal outcome were collected, including numbers of live birth, intrauterine demise, neonatal death, perinatal death and termination of fetus or pregnancy (TOP). Neonatal data such as GA at birth, birth weight, admission to neonatal intensive care unit and neonatal morbidity were also collected. Perinatal outcomes were assessed according to whether the pregnancy was managed expectantly or underwent fetal intervention. RESULTS: Of an initial cohort of 174 MCTA triplet pregnancies, 11 underwent early TOP, three had an early miscarriage, six were lost to follow-up and one was ongoing at the time of writing. Thus, the study cohort included 153 pregnancies, of which the majority (92.8%) were managed expectantly. The incidence of pregnancy affected by one or more fetal structural abnormality was 13.7% (21/153) and that of TRAP sequence was 5.2% (8/153). The most common antenatal complication related to chorionicity was TTTS, which affected just over one quarter (27.6%; 42/152, after removing a pregnancy with TOP < 24 weeks for fetal anomalies) of the pregnancies, followed by sFGR (16.4%; 25/152), while TAPS (spontaneous or post TTTS with or without laser treatment) occurred in only 4.6% (7/152) of pregnancies. No monochorionicity-related antenatal complication was recorded in 49.3% (75/152) of pregnancies. Survival was apparently associated largely with the development of these complications: there was at least one survivor beyond the neonatal period in 85.1% (57/67) of pregnancies without antenatal complications, in 100% (25/25) of those complicated by sFGR and in 47.6% (20/42) of those complicated by TTTS. The overall rate of preterm birth prior to 28 weeks was 14.5% (18/124) and that prior to 32 weeks' gestation was 49.2% (61/124). CONCLUSION: Monochorionicity-related complications, which can impact adversely perinatal outcome, occur in almost half of MCTA triplet pregnancies, creating a challenge with regard to counseling, surveillance and management. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Brain Doppler abnormalities in fetuses with open spina bifida.

BACKGROUND: To describe the brain Doppler changes in fetuses with isolated open spina bifida (OSB). STUDY DESIGN: Doppler ultrasound measurements including the umbilical artery (UA) pulsatility index (PI), middle cerebral artery (MCA) PI, and their combination into the cerebroplacental ratio (CPR) and umbilicocerebral ratio (UCR) were performed in a cohort of fetuses with OSB. Included patients were individually matched to a control group without structural anomalies and similar gestational ages. MCA-PI and/or CPR values below the 5th centile were considered abnormal and indicative of cerebral blood redistribution, while UCR and UA-PI above the 95th centile were considered abnormal. RESULTS: During the study period, 266 fetuses were recruited, 133 normally grown fetuses with OSB and 133 matched controls. Doppler recordings were successfully obtained in all fetuses at a median gestational age of 30.9 weeks. All cases showed UA-PI within normal ranges, but fetuses with OSB showed significantly higher frequency of cerebral blood redistribution (15.0 vs. 3.0%, p < .01), MCA-PI <5th centile (10.5% vs. 3.0%, p = .01), decreased CPR (9.8% vs. 2.3%, p = .01) and increased UCR (17.2 % vs 7.5 %, p =.02). The rate of cerebral blood redistribution was significantly higher in the OSB group with ventriculomegaly in comparison to those with OSB and normal lateral ventricle diameter (18.6% vs. 3.2%, respectively; p <.05). CONCLUSION: A proportion of fetuses with isolated open spina bifida showed cerebral blood redistribution.

Doppler changes in umbilical artery, middle cerebral artery, cerebroplacental ratio and ductus venosus during open fetal microneurosurgery for intrauterine open spina bifida repair.

OBJECTIVE: To describe changes in fetal Doppler parameters during a novel technique for open fetal microneurosurgery for open spina bifida (OSB) repair. METHODS: This was a prospective study of 44 fetuses undergoing open fetal surgery for OSB repair using a novel microneurosurgery approach that is characterized by a mini-hysterotomy (diameter of 15 mm), minimal fetal manipulation and maintenance of a constant normal amniotic fluid volume throughout the procedure. Doppler velocimetry of the umbilical artery (UA), fetal middle cerebral artery (MCA) and ductus venosus (DV) was performed before the start of surgery and at prespecified timepoints during fetal surgery. UA pulsatility index (PI) > 95th percentile, DV-PI > 95th percentile, MCA-PI < 5th percentile and cerebroplacental ratio (CPR) < 5th percentile were considered abnormal. RESULTS: Median gestational age at fetal surgery was 25.2 weeks (range, 22.9-27.9 weeks). Doppler recordings were successfully obtained in all cases during all timepoints throughout the surgery. As compared with Doppler values before surgery, there was a significant increase in the proportion of fetuses with MCA-PI < 5th percentile (63.6% vs 13.6%; P < 0.001), CPR < 5th percentile (65.9% vs 15.9%; P < 0.001) and DV-PI > 95th percentile (22.7% vs 0%; P = 0.01) and a non-significant increase in the proportion of fetuses with UA-PI > 95th percentile (11.4% vs 0%; P = 0.12) during fetal surgery. None of the fetuses showed absent or reversed end-diastolic velocity in the UA or absent or reversed DV a-wave at any stage during OSB repair. All abnormal Doppler parameters returned to normal after surgery. CONCLUSIONS: During open fetal surgery for OSB repair, a small hysterotomy, reduced fetal manipulation and maintenance of a normal amniotic fluid volume seem to prevent severe fetal Doppler abnormalities. The mild Doppler changes observed during fetal surgery could be a manifestation of fetal adaptation to the stress of fetal surgery. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Survival outcome in severe left-sided congenital diaphragmatic hernia with and without fetal endoscopic tracheal occlusion in a country with suboptimal neonatal management.

OBJECTIVE: To evaluate the impact of fetal endoscopic tracheal occlusion (FETO) on improving survival of fetuses with severe left-sided congenital diaphragmatic hernia (CDH), as compared with contemporaneous cases managed expectantly during pregnancy, in a country with suboptimal neonatal management. METHODS: In this prospective cohort study, consecutive fetuses with isolated left-sided CDH, normal karyotype and severe pulmonary hypoplasia (defined as liver herniation and observed/expected lung-to-head circumference ratio below 26%) were selected for FETO at less than 32 weeks of gestation in a single tertiary referral center in Queretaro, Mexico. Postnatal outcome (survival up to 28 days after birth) was compared between fetuses treated with FETO and contemporaneous cases with similar lung size managed expectantly during pregnancy. RESULTS: Twenty-five fetuses with isolated severe left-sided CDH treated with FETO were matched individually with 25 cases managed expectantly during pregnancy. Endotracheal placement of the balloon was performed successfully on the first attempt in all cases. The median gestational age (GA) at balloon placement was 29.1 (range, 25.6-31.8) weeks and 34.1 (range, 30.0-36.1) weeks at balloon removal. There were no technical problems with the introduction or removal of the balloon in any cases. The median GA at delivery was significantly lower in the group treated with FETO than in those managed expectantly (35.3 vs 37.7 weeks; P = 0.04). The survival rate was significantly higher in the group treated with FETO than in those without fetal intervention (32% vs 0%; P < 0.001). CONCLUSION: In settings with suboptimal neonatal management, FETO was associated with improved neonatal survival in fetuses with isolated left-sided CDH and severe pulmonary hypoplasia. Copyright © 2020 ISUOG. Published by John Wiley & Sons Ltd.

Resultado de supervivencia en una hernia diafragmática congénita grave del lado izquierdo, con y sin oclusión traqueal endoscópica fetal en un país con un tratamiento neonatal subóptimo OBJETIVO: Evaluar el impacto de la oclusión traqueal endoscópica fetal (OTEF) en la mejora de la supervivencia de los fetos con hernia diafragmática congénita (HDC) grave del lado izquierdo, en comparación con los casos actuales tratados como embarazo gestante, en un país con un tratamiento neonatal subóptimo. MÉTODOS: En este estudio prospectivo de cohortes, se seleccionaron fetos consecutivos con HDC aislada del lado izquierdo, cariotipo normal e hipoplasia pulmonar grave (definida como hernia hepática y una proporción observada/esperada de la circunferencia pulmonar-cabeza inferior al 26%) para una OTEF antes de las 32 semanas de gestación, en un único centro de medicina especializada terciaria en Querétaro (México). El resultado postnatal (supervivencia hasta los 28 días después del nacimiento) se comparó entre fetos tratados con OTEF y los casos contemporáneos con tamaño pulmonar similar, tratados como embarazo gestante. RESULTADOS: Veinticinco fetos con HDC grave aislada del lado izquierdo que habían sido tratados con OTEF fueron emparejados individualmente con 25 casos tratados como embarazo gestante. La colocación endotraqueal del globo se realizó con éxito en el primer intento en todos los casos. La mediana de la edad gestacional (EG) en el momento de la colocación del globo fue de 29,1 (rango, 25,6-31,8) semanas y 34,1 (rango, 30,0-36,1) semanas cuando se retiró el globo. En ningún caso hubo problemas técnicos con la introducción o la retirada del globo. La mediana de la EG en el momento del parto fue significativamente menor en el grupo tratado con OTEF que en el grupo tratado como gestante (35,3 vs 37,7 semanas; P=0,04). La tasa de supervivencia fue significativamente más alta en el grupo tratado con OTEF que en los casos sin intervención fetal (32% vs 0%; P<0,001). CONCLUSIÓN: En los entornos con un tratamiento neonatal subóptimo, la OTEF se asoció con una mejora de la supervivencia neonatal en los fetos con HDC aislada del lado izquierdo y con hipoplasia pulmonar grave. Copyright © 2020 ISUOG. Published by John Wiley & Sons Ltd.

Fetal laser ablation of feeding artery of cystic lung lesions with systemic arterial blood supply.

OBJECTIVE: To assess the effectiveness of laser surgery in fetuses with a cystic lung lesion with systemic arterial blood supply (hybrid lung lesion) at risk of perinatal death. METHODS: A cohort of five consecutive fetuses with a large hybrid lung lesion associated with hydrops and/or pleural effusion with severe lung compression was selected for percutaneous ultrasound-guided fetal laser ablation of the feeding artery (FLAFA) before 32 weeks' gestation in a single tertiary national referral center in Queretaro, Mexico. The primary outcomes were survival and need for postnatal surgery. RESULTS: FLAFA was performed successfully in all cases at a median gestational age of 24.9 (range, 24.4-31.7) weeks. After fetal intervention, dimensions in both lungs increased and fluid effusions resolved in all cases. All cases were delivered liveborn at term at a median gestational age of 39.6 (range, 38.0-39.7) weeks, without respiratory morbidity or need for oxygen support, resulting in perinatal survival of 100%. During follow-up, three (60%) cases showed progressive regression of the entire lung mass and did not require postnatal surgery, whereas in two (40%) cases a progressive decrease in size of the mass was observed but a cystic portion of the lung mass persisted and postnatal lobectomy was required. CONCLUSION: In fetuses with large hybrid lung lesions at risk of perinatal death, FLAFA is feasible and could improve survival and decrease the need for postnatal surgery. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Neurodevelopmental outcome of full-term small-for-gestational-age infants with normal placental function.

OBJECTIVE: To evaluate the 2-year neurodevelopmental outcome of full-term, small-for-gestational-age (SGA) newborns with normal placental function, according to current criteria based on umbilical artery Doppler findings. METHODS: A cohort of consecutive full-term, SGA newborns with normal prenatal umbilical artery Doppler was compared with a group of full-term, appropriate-for-gestational-age (AGA) infants sampled from our general neonatal population. Neurodevelopmental outcome was evaluated at 24 months' corrected age using the Bayley Scales of Infant and Toddler Development, 3(rd) Edition (Bayley-III), which evaluates cognitive, language, motor, social-emotional and adaptive competencies. The effect of the study group on each domain was adjusted with MANCOVA and logistic regression for gestational age at delivery, socioeconomic status, gender, tobacco smoking and breastfeeding. RESULTS: A total of 223 infants (112 SGA and 111 AGA) were included. The groups differed significantly with respect to socioeconomic status and gestational age at delivery. All studied neurodevelopmental domains were poorer in the SGA group, reaching significance for the cognitive (92.9 vs 100.2, adjusted P = 0.027), language (94.7 vs 101, adjusted P = 0.025), motor (94.2 vs 100, adjusted P = 0.027) and adaptive (89.2 vs 96.5, adjusted P = 0.012) scores. Likewise, the SGA group had a higher risk of low scores in language (odds ratio (OR) = 2.63; adjusted P = 0.045) and adaptive (OR = 2.72; adjusted P = 0.009) domains. CONCLUSIONS: Compared with normal-sized babies, full-term SGA infants, without placental insufficiency defined according to currently used criteria, have lower 2-year neurodevelopmental scores. These data challenge the concept that SGA fetuses with normal umbilical artery Doppler are 'constitutionally small' but otherwise completely normal.

Usefulness of lung-to-head ratio and intrapulmonary arterial Doppler in predicting neonatal morbidity in fetuses with congenital diaphragmatic hernia treated with fetoscopic tracheal occlusion.

OBJECTIVE: To explore the potential value of intrapulmonary artery Doppler velocimetry in predicting neonatal morbidity in fetuses with left-sided congenital diaphragmatic hernia (CDH) treated with fetoscopic tracheal occlusion (FETO). METHODS: Observed/expected lung-to-head ratio (O/E-LHR), and intrapulmonary Doppler pulsatility index and peak early-diastolic reversed flow were evaluated within 24 h before FETO in a consecutive cohort of 51 fetuses with left-sided CDH at between 24 and 33 weeks' gestation. Lung Doppler parameters were converted into Z-scores and defined as abnormal if the pulsatility index had a Z-score of > 1.0 or the peak early-diastolic reversed flow had a Z-score of > 3.5. The association of O/E-LHR and Doppler velocimetry with neonatal outcome was assessed using multiple linear or logistic regression analysis adjusted for gestational age at birth. RESULTS: Among the 26 fetuses that survived, 18 (69.2%) had normal and eight (30.8%) had abnormal Doppler values. O/E-LHR was not associated with neonatal morbidity in surviving fetuses. Compared with the group with normal Doppler parameters, cases with abnormal intrapulmonary Doppler were associated with a significant increase in the duration of mechanical ventilation (average increase of 21.2 (95% CI, 9.99-32.5) days; P < 0.01), conventional ventilation (15.2 (95% CI, 7.43-23.0) days; P < 0.01), high-frequency ventilation (6.34 (95% CI, 0.69-11.99) days; P < 0.05), nitric oxide therapy (5.73 (95% CI, 0.60-10.9) days; P < 0.05), oxygen support (36.5 (95% CI, 16.3-56.7) days; P < 0.01), parenteral nutrition (19.1 (95% CI, 7.53-30.7) days; P < 0.01) and stay in neonatal intensive care unit (42.7 (95% CI, 22.9-62.6) days; P < 0.001), and with significantly higher rates of high-frequency ventilation (87.5 vs. 44.4%;P < 0.05), oxygen requirement at 28 days of age (75.0 vs. 11.1%; P < 0.01), gastroesophageal reflux (62.5 vs. 22.2%; P < 0.05) and tube feeding at discharge (37.5 vs. 5.56%; P < 0.05). CONCLUSION: As previously reported, O/E-LHR did not predict neonatal morbidity. In contrast, intrapulmonary artery Doppler evaluation was predictive of neonatal morbidity in CDH fetuses treated with FETO.

Outcome of fetuses with congenital diaphragmatic hernia and associated intrafetal fluid effusions managed in the era of fetal surgery.

OBJECTIVE: Fetuses with congenital diaphragmatic hernia (CDH) and for whom additional ultrasound findings are abnormal typically are considered to have a dismal prognosis. Our aim was to assess the outcome of fetuses with CDH and associated intrafetal fluid effusions. METHODS: This was a retrospective bicentric cohort study on the perinatal management of fetuses with CDH and intrafetal fluid effusions. RESULTS: The incidence of effusions was 5.2% (n = 14) in 269 consecutive cases of left-sided CDH and 29.2% (n = 14) in 48 cases of right-sided CDH. Hydrothorax (n = 19 (68%)) and ascites (n = 16 (57%)) were the most common effusions. A combination of both was present in 11 (39%) fetuses. Of 20 ongoing pregnancies with CDH and fluid effusions, without other anomalies, five with moderate or mild pulmonary hypoplasia were managed without fetoscopic endoluminal tracheal occlusion (FETO). The 15 other cases underwent FETO because of severe pulmonary hypoplasia. Neonatal survival rate was similar in both groups (n = 2/5 and n = 6/15, respectively (P = 1.0)). Survival among those who underwent FETO was similar to previously published results concerning isolated cases undergoing FETO. CONCLUSIONS: Our observations do not support the view that intrafetal effusions are an adverse prognostic factor in fetuses with CDH. In CDH fetuses with effusions and severe pulmonary hypoplasia treated with FETO, neonatal survival is similar to that in isolated cases undergoing the intervention. Whether pleural effusions should be addressed by thoracic drainage procedures remains unproven.

Neurobehavioral outcomes in preterm, growth-restricted infants with and without prenatal advanced signs of brain-sparing.

OBJECTIVE: To evaluate the neurobehavioral outcomes of preterm infants with intrauterine growth restriction (IUGR), with and without prenatal advanced brain-sparing. METHODS: A cohort of IUGR infants (birth weight < 10(th) percentile with abnormal umbilical artery Doppler) born before 34 weeks of gestation was compared with a control group of appropriate-for-gestational age infants matched for gestational age at delivery. MCA pulsatility index was determined in all cases within 72 hours before delivery. Neonatal neurobehavior was evaluated at 40 weeks' ( ± 1) corrected age using the Neonatal Behavioral Assessment Scale. The effect of abnormal MCA pulsatility index (< 5(th) percentile) on each neurobehavioral area was adjusted for maternal smoking status and socioeconomic level, mode of delivery, gestational age at delivery, pre-eclampsia, newborn illness severity score and infant sex by multiple linear and logistic regression. RESULTS: A total of 126 preterm newborns (64 controls and 62 IUGR) were included. Among IUGR fetuses, the proportion of abnormal MCA Doppler parameters was 53%. Compared with appropriate-for-gestational age infants, newborns in the IUGR subgroup with abnormal MCA Doppler had significantly lower neurobehavioral scores in the areas of habituation, motor system, social-interactive and attention. Similarly, the proportion of infants with abnormal neurobehavioral scores was significantly higher in the IUGR subgroup with abnormal MCA Doppler parameters in the areas of habituation, social-interactive, motor system and attention. CONCLUSION: Abnormal MCA Doppler findings are predictive of neurobehavioral impairment among preterm newborns with IUGR, which suggests that this reflects an advanced stage of brain injury with a higher risk of abnormal neurological maturation.

Incidence and clinical implications of early inadvertent septostomy after laser therapy for twin-twin transfusion syndrome.

OBJECTIVE: To evaluate the incidence and clinical outcome of inadvertent septostomy after fetoscopic laser therapy for twin-twin transfusion syndrome (TTTS) and, particularly, to explore its association with the risk of developing pseudoamniotic band syndrome (PABS). METHODS: In a cohort of 414 consecutive monochorionic twin pregnancies with confirmed TTTS treated with laser, the incidence of postoperative septostomy within 1 week of the procedure was recorded prospectively. Rates of preterm delivery, preterm premature rupture of membranes (PPROM), intrauterine fetal demise (IUFD) and PABS were compared among cases with and without septostomy. RESULTS: The mean gestational age at laser therapy was 20.4 (range, 15.3-27.6) weeks. Postoperative septostomy occurred in 30 (7.2%) cases. Pregnancies complicated with septostomy had a significantly higher proportion of preterm delivery before 32 weeks (76.7% vs. 30.2%, P < 0.001), PPROM before 32 weeks (46.7% vs. 19.0%, P < 0.001), IUFD (43.3% vs. 25.8%, P < 0.05) and PABS (13.3% vs. 1.0%, P < 0.001), compared with pregnancies without septostomy. CONCLUSIONS: Inadvertent septostomy occurred in 7% of cases after fetoscopic laser therapy and was associated with a substantially increased risk of adverse perinatal outcome and PABS.