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Congenital diaphragmatic hernia (CDH) can be diagnosed prenatally and its severity assessed by fetal imaging. The prognosis of a fetus with CDH is based on whether or not the hernia is isolated, the measurement of lung volume on ultrasound and MRI, and the position of the liver. The birth of a child with CDH should take place in a center adapted to the care of such children, and in accordance with the recommendations defined by the French National Diagnosis and Care Protocol. It has recently been demonstrated that for moderate and severe forms of CDH, tracheal occlusion using a balloon placed in utero by fetoscopy (FETO) increases survival until discharge from the neonatal unit, but at the cost of an increased risk of prematurity. At the same time, advances in neonatal resuscitation and the standardization of follow-up of these children within the framework of the "Centre de référence maladies rares: hernie de coupole diaphragmatique" have improved the prognosis of these children and young adults., (Copyright © 2024. Published by Elsevier Masson SAS.)

Objectives: To report preoperative data, surgical characteristics, complications and perinatal outcome of twin-twin transfusion syndrome (TTTS) managed with laser ablation surgery, to analyze predictors of neonatal survival and to compare the 100 most recent cases with the older 100., Materials and Methods: Observational cohort moncentric study of 200 cases of TTTS consecutively treated with fetoscopic laser coagulation between January 2004 and December 2014., Results: There were 49 stage I, 88 stage II, 55 stage III and eight stage IV. Median gestation at time of laser was 20.1±3.0 weeks' gestation (WG) whereas median gestation at delivery was 31.6±5.4 WG. Overall perinatal survival rate was 68.0% and 84.0% have one or more surviving twins. Preterm premature rupture of membranes occurred in 39 cases with and the median gestational age for this complication was 28.8±4.6 SA. Predictive factors to have at least one living birth were Quintero stage and gestational age at delivery. In the most recent period, there were significantly more TTTS Quintero stage I treated with laser, more coagulation by the Solomon technique and a larger number of coagulated vessels., Conclusion: The neonatal survival of TTTS is improved by fetoscopic laser coagulation, preferely by using Solomon tecnhique. The use of active management of stage I is currently on research., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Objectives: Study the outcomes of triplet pregnancies (GGG) complicated with twin-to-twin transfusion syndrome (TTTS) treated with laser fetoscopy., Methods: Retrospective study of interventions, outcomes and perinatal follow-up of GGG treated for TTS., Results: Between 2002 and 2013, 25 GGG complicated by TTTS were seen in our center, 20 dichorionic and 5 monochorionic. The mean gestational age (GA) at diagnosis of TTTS was 19.7 GW (±2.4) with 2, 4, 16 and 1 pregnancies at Quintero's stage I, II, III and V, respectively. They had a fetoscopy at an average GA of 19 GW and 6 days. There were 3 (13.0%) late miscarriages. The average GA at delivery was of 29.6 GW overall (26.3 GW and 31.1 GW in monochorionic and dichorionic pregnancies respectively). The overall fetal survival rate was 57.97% (40% and 66.7% in the group of monochorionic dichorionic pregnancies, respectively). However, neonatal mortality (<28 days) is 17.5%., Conclusion: GGG operated by fetoscopy for TTTS have a survival rate of three, at least 2 and at least 1 fetus of 21.7%, 69.6% and 82.6% respectively. The overall fetal survival rate is 59.97%. There is a tendency for better survival rates in dichorionic GGG compared to monochorionic GGG (P=0.079)., (Copyright © 2016. Published by Elsevier Masson SAS.)

Both the improvement of pathophysiological knowledge of major fetal anomalies and the development of therapeutic tools have allowed in some specific cases in utero therapy by foetoscopy. We discuss the state of art and recent advances for four major anomalies: twin-to-twin transfusion syndrome, congenital diaphragmatic hernia, myelomeningocele and lower urinary tract obstruction. Fetoscopic laser surgery for twin-to-twin transfusion syndrome has become the gold standard for treatment of TTS. In terms of fetal surgery, severe congenital diaphragmatic hernia and myelomeningocele are the two main indications, even if open fetal surgery is still the gold standard for management of myelomeningocele. New techniques using fetal cystoscopy are currently under development. Although the maternal morbidity associated with foetoscopy is low, preterm rupture of membranes and preterm delivery remain an important problem. Long-term evaluation of those neonates remains mandatory., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Open spina bifida or myelomeningocele (MMC) is a frequent congenital abnormality (450 cases per year in France) associated with high morbidity. Immediate postnatal surgery is aimed at covering the exposed spinal cord, preventing infection, treating hydrocephalus with a ventricular shunt. MMC surgical techniques haven't achieved any major progress in the past decades. Numerous experimental and clinical studies have demonstrated the MMC "two-hit" hypothetic pathogenesis: a primary embryonic congenital abnormality of the nervous system due to a failure in the closure of the developing neural tube, followed by secondary damages of spinal cord and nerves caused by long-term exposure to amniotic fluid. This malformation frequently develops cranial consequences, i.e. hydrocephalus and Chiari II malformation, due to leakage of cerebrospinal fluid. After 30 years of research, a randomized trial published in February 2011 proved open maternal-fetal surgery (OMFS) for MMC to be a real therapeutic option. Comparing prenatal to postnatal surgery, it confirmed better outcomes of MMC children after a follow up of 2.5 years: enhancement of lower limb motor function, decrease of the degree of hindbrain herniation associated with the Chiari II malformation and the need for shunting. At 5 years of age, MMC children operated prenatally seems to have better neurocognitive, motor and bladder-sphincter outcomes than those operated postnatally. However, risks of OMFS exist: prematurity for the fetus and a double hysterotomy at approximately 3-month interval for the mother. Nowadays, it seems crucial to inform parents of MMC patients about OMFS and to offer it in France. Future research will improve our understanding of MMC pathophysiology and evaluate long-term outcomes of OMFS. Tomorrow's prenatal surgery will be less invasive and more premature using endoscopic, robotic or percutaneous techniques. Beforehand, Achilles' heel of maternal-fetal surgery, i.e. preterm premature rupture of membranes, preterm labor and preterm birth, must be solved., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Congenital cystic adenomatoid malformations (CCAM) of the lung are the most frequent congenital lung malformations. Their diagnosis is based on histological features. CCAM consist of bronchopulmonary cystic lesions which are classified according to the presence and cysts size. Type I CCAM are composed of large cysts (>2 cm) lined by a columnar pseudostratified epithelium. Type II CCAM contain multiple small cystic lesions (<1 cm) lined by a flattened cuboidal epithelium. Type III CCAM are more solid and contain immature structures resembling the pseudoglandular stage of lung development. Ultrasonography (US) allows early detection during the second trimester of pregnancy as cystic, and/or hyperechoic fetal lung lesions. Although most CCAM remain asymptomatic, CCAM can cause polyhydramnios or fetal hydrops, respiratory distress at birth, infections and pneumothoraces during infancy, and may give rise to malignancies. Serial US allow detection of complications, and planification of delivery. Complicated forms require an urgent treatment. In fetuses with a macrocystic life-threatening lesion, a thoraco-amniotic shunt can be placed. Microcystic compressive forms may respond to prenatal steroids. Post-natal symptomatic lesions require early surgery. The treatment of asymptomatic forms remains controversial. Some recommend a non-operative approach with a long-term clinical and radiological following, whereas other favour a preventive surgical excision. The origin of CCAM remains unknown. Recent advances suggest a transient and focal abnormality in lung development which may result from an airway obstruction. This article reviews the diagnosis, treatment, and pathophysiology of CCAM., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Myelomeningocele is characterized by the extrusion of the spinal cord into a sac filled with cerebrospinal fluid. One part of the postnatal disabilities could be related to the spinal damage and to the cerebral repercussion of the leak of cerebrospinal fluid from the defect. Several experimental studies in animals have demonstrated that a surgical repair of the lesion at middle gestation reduced the postnatal disabilities. These results were confirmed in humans by the Management of Myelomeningocele (MOM) Trial. However, the prenatal surgical repair is associated with maternal and fetal morbidity., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Both technical and anatomical features and the need for a long follow-up, usually over several years, explain the specificity of lung surgery in children. Apart from the oncological resections, the main indications for this surgery are congenital lung anomalies (cystic adenomatoid malformation, bronchopulmonary sequestration, lobar emphysema), symptomatic postinfectious lesions (bronchiectasis, atelectasis, abscess) and pneumothorax. Advances in miniaturization of the surgical equipment are used to carry the majority of these interventions by thoracoscopy, except when performed at the neonatal period. In the newborn, for which the indications for lung surgical resections are exceptional, ventilatory conditions and the tightness of the chest lead to still prefer open surgery. Pulmonary malformations can also be managed prenatally when they affect lung development or cause heart failure because of compressive complications. These interventions, performed after the 30th week of gestation, are not intended to remove the lesions, but to release the compression. But these procedures are still under evaluation and, in light of possible fetal and maternal complications, they should only be performed in tertiary centers and in the context of specific protocols., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Twin-to-twin transfusion syndrome (TTTS), also called twin oligoamnios polyhydramnios sequence (TOPS) complicates around 15% of monochorionic pregnancies irrespective of the mode of conception. It is a hemodynamic, and probably hormonal, discordance secondary to imbalanced blood flow through the vascular anastomoses aforementioned. During the last 15 years, a lot of progress has been made in the understanding, diagnosis and treatment of TTTS. Nevertheless, many questions remain unanswered and research is still active in this domain. Fetoscopic laser coagulation of placental vessels is the only treatment addressing the pathophysiology of the syndrome as proven through a randomized controlled study against amnioreduction. This technique has definitely improved overall survival as well as the rate of neurologically intact survival but it still lacks preoperative prognostic markers., (Copyright © 2012. Published by Elsevier SAS.)

Objectives: To evaluate the role of fetal movement counting and amnioscopy in the management of prolonged pregnancies., Materials and Methods: PubMed and the Cochrane library were searched using terms: fetal movement counting, decreased fetal movement, post-term pregnancy, late pregnancy, prolonged pregnancy, postdate pregnancy, amnioscopy, meconium amniotic fluid, surveillance of the fetus., Results: Decreased fetal mouvements (DFM) in prolonged pregnancies is a frequent cause of unplanned visits. For many authors, the best criterion is the self-perception of reduced maternal or any change of fetal movement. Current data is insufficient to show a link between DFM and increased fetal morbidity and mortality. It is reasonable to recommend to the patient to consult in case of DFM and make an assessment of fetal vitality (experts' opinion). Current data are insufficient to show a potential benefit of the establishment of an information given to patients on DFM in prolonged pregnancy (experts' opinion). No fetal movement counting was assessed in the prolonged pregnancy. High negative predictive value of amnioscopy explains it is reassuring only if the amniotic fluid is clear. Amnioscopy showed no neonatal benefit in the survey of prolonged pregnancies (NP4)., Conclusion: In the prolonged pregnancies, no fetal movement counting can reduce the perinatal morbidity and mortality rate. The interest of amnioscopy in the survey of prolonged pregnancies has never been showed. It is not recommended to use it., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Introduction: Foetal lung lesions are uncommon (<1/1000 deliveries). Prenatal ultrasound can distinguish echogenic and cystic lesions., Background: The most frequently diagnosed abnormalities are congenital cystic adenomatoid malformation (echogenic and/or cystic), pulmonary sequestration (echogenic), congenital lobar emphysema (echogenic) and bronchogenic cyst (cystic). Most of them carry a good prognosis but complications, including foetal hydrops, may occur and should be looked for regularly prenatally. Congenital upper airway obstruction syndromes are very rare and carry a very poor prognosis., Viewpoint: The follow-up of these pregnancies should be undertaken in a tertiary centre and information regarding postnatal management should be given prenatally by the doctors who will care for the infant after birth. Serial ultrasound examinations are required to evaluate the natural history and detect complications. Spontaneous regression may occur. The occurrence of foetal hydrops is usually fatal in the absence of treatment and should be managed as an emergency., Conclusions: Congenital malformations of the lung are rare and usually carry a good prognosis. Careful follow-up and delivery should be performed in a tertiary centre and postnatal investigation should include clinical and radiological evaluation., (Copyright © 2011 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Objectives: To report pre- and post-surgical datas of large series of severe twin-to-twin transfusion syndrome (TTTS) managed with laser ablation surgery in our centre, to evaluate the incidence of complications, perinatal outcome and to compare with other cohorts., Patients and Methods: Observational study of 100 cases of TTTS consecutively treated with fetoscopic laser coagulation between January 2004 and April 2010 in CMCO-SIHCUS of Schiltigheim., Results: There are nine stage I, 49 stage II, 38 stage III and four stage 4. Median gestation at time of laser is 20.6 weeks (14-29) whereas median gestation at delivery is 32.6 weeks (16.3-39). Overall perinatal survival rate is 68.5% (137 children over 200). Eighty-five percent have one or more surviving twins. The survival rate is the same for donors and for recipients. Preterm premature rupture of the membranes are observed in 17% of cases and the median gestational age for this complication is 30 weeks (20-34). Cerebral abnormalities are present in 7% of newborns., Conclusion: Our results for the management of severe TTTS are comparable to the other reported series. There are still many questions remaining concerning the optimal management of TTTS., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Monochorionic twin pregnancies (MC) are associated with a higher risk of complications than their bichorionic equivalents. When two fetuses share the same placenta, the two fetal circulations are closely bound by omnipresent vascular anastomoses. The determination of the chorionicity is essential for an adequate management of these particular pregnancies. The twin-to-twin transfusion syndrome (TTTS) is one of the. possible complications and must be considered as an obstetrical emergency. Severe growth restriction of one twin occurs in 11% of MC pregnancies. Before the limit of viability, in some of the cases, selective foeticide using particular methods are mandatory to preserve the wellbeing of the healthy twin. The invasive procedures carried out at the time these complications occur proved their effectiveness, under the condition of being applied by experienced experts. Laser coagulation, TTTS gold standard treatment, is associated with a fetal survival rate equal or higher than 75%. Among MC twins, selective foeticide with bipolar cord coagulation allows an intact survival rate of the other twin in more than 75 to 80% of the cases.

Foetoscopy consists of the introduction into the ovular cavity, at the beginning of the second trimester of pregnancy, of an optical device in order to be able to observe the foetus and the foetal surface of the placenta. It may be used to detect certain foetal malformations which are not seen by echography and to collect foetal red cells by direct puncture of a placental vein. Its major indication is the prenatal diagnosis of homozygous forms of haemoglobinopathies: sickle cell disease and beta-thalassaemia. It is a dangerous investigation which requires great experience and perfect organisation.