Objective To assess the value of antenatally determined observed to expected fetal lung area to head circumference ratio (LHR) in the prediction of postnatal survival in isolated, congenital diaphragmatic hernia (CDH). Methods Two groups of fetuses were examined. The first group included 650 normal fetuses at 12–32 weeks’ gestation, and the data collected were used to establish a normal range of observed to expected LHR with gestational age. The second group included the data of a retrospective multicenter study of 354 fetuses with isolated CDH in which the LHR was measured on one occasion at 18–38 weeks’ gestation. The patients were divided into those with left-sided CDH with and without intrathoracic herniation of the liver and right-sided CDH. Regression analysis was used to determine the significant predictors of postnatal survival. Results In both the normal fetuses and those with CDH the LHR increased but the observed to expected LHR did not change significantly with gestational age. In normal fetuses the mean observed to expected LHR in the left lung was 100% (95% CI, 61–139%) and in the right lung it was 100% (95% CI, 67–133%). In fetuses with CDH the mean observed to expected LHR was 39% (range 7–79%). Regression analysis demonstrated that significant predictors of survival were the observed to expected LHR (odds ratio (OR) 1.09, 95% CI, 1.06–1.12), side of CDH (left side OR 11.14, 95% CI, 3.41–36.39) and gestational age at delivery (OR 1.18, 95% CI, 1.02–1.36).

https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.4052

Observed to expected lung area to head circumference ratio in the prediction of survival in fetuses with isolated diaphragmatic hernia

Background

Congenital diaphragmatic hernia (CDH) with liver herniation and a lung area to head circumference ratio (LHR) > 1 is associated with a high rate of neonatal death due to pulmonary hypoplasia.



Methods

We report the development of a minimally invasive and reversible fetoscopic tracheal occlusion (FETO) with a balloon, carried out in 21 consecutive fetuses with severe CDH.



Results

Endotracheal placement of the balloon was successfully performed in all 21 cases and the mean duration of the operation was 20 (range, 5–54) min. The median gestation at FETO was 26 (range, 25–33) weeks. There were no maternal complications such as hemorrhage, placental abruption or pulmonary edema. In 11 (52.4%) patients there was postoperative prelabor amniorrhexis, which occurred within 2 weeks in five patients and after 2 weeks in six patients. Ultrasound scans after FETO demonstrated an increase in the echogenicity of the lungs within 48 h and improvement in the LHR from a median 0.7 (range, 0.4–0.9) before FETO to 1.8 (range, 1.1–2.9) within 2 weeks following surgery. The median gestation at delivery was 34 (range, 27–38) weeks and in 17 (77.3%) patients delivery occurred after 32 weeks. Nine babies died in the neonatal period due to complications from pulmonary hypoplasia. Surgical repair of the diaphragmatic hernia was carried out in 12 babies and in all but one the defect was extensive and required the insertion of a patch. Ten of these babies survived, and at the time of writing were aged 6–25 (median, 18) months and were developing normally. Survival was 30% in the first group of 10 fetuses and 63.6% in the second group of 11 fetuses. The total number of cases was too small for definite conclusions to be drawn as to the causes of this apparent improvement in survival. Nevertheless, improved survival coincided with a shift in the timing of FETO from the third to the second trimester, the administration of epidural rather than general anesthesia, reduced incidence of postoperative amniorrhexis and a change in the policy on the timing of removal of the balloon from the intrapartum to the prenatal period. During the same period of study there were 17 cases examined in the participating centers that met the criteria for FETO but which declined prenatal therapy. In all cases there was isolated left-sided CDH with liver in the thorax and LHR of 0.4–0.9 (mean, 0.7). In five cases the parents elected to terminate the pregnancy. In the 12 cases with expectant management all babies were liveborn but 11 died in the neonatal period due to pulmonary hypoplasia and only one (8.3%) survived.



Conclusion

Severe CDH can be successfully treated with FETO, which is minimally invasive and may improve postnatal survival. Copyright © 2004 ISUOG. Published by John Wiley & Sons, Ltd.

Fetoscopic tracheal occlusion (FETO) for severe congenital diaphragmatic hernia: evolution of a technique and preliminary results

Objectives To examine operative and perinatal aspects of fetal endoscopic tracheal occlusion (FETO) in congenital diaphragmatic hernia (CDH). Methods This was a multicenter study of singleton pregnancies with CDH treated by FETO. The entry criteria for FETO were severe CDH on the basis of sonographic evidence of intrathoracic herniation of the liver and low lung area to head circumference ratio (LHR) defined as the observed to the expected normal mean for gestation (o/e LHR) equivalent to an LHR of 1o r less. Results FETO was carried out in 210 cases, including 175 cases with left-sided, 34 right-sided and one with bilateral CDH. In 188 cases the CDH was isolated and in 22 there was an associated defect. FETO was performed at a median gestational age of 27.1 (range, 23.0–33.3) weeks. The first eight cases were done under general anesthesia, but subsequently either regional or local anesthesia was used. The median duration of FETO was 10 (range, 3–93) min. Successful placement of the balloon at the first procedure was achieved in 203 (96.7%) cases. Spontaneous preterm prelabor rupture of membranes (PPROM) occurred in 99 (47.1%) cases at 3–83 (median, 30) days after FETO and within 3 weeks of the procedure in 35 (16.7%) cases. Removal of the balloon was prenatal either by fetoscopy or ultrasound-guided puncture, intrapartum by ex-utero intrapartum treatment, or postnatal either by tracheoscopy or percutaneous puncture. Delivery was at 25.7–41.0 (median, 35.3) weeks and before 34 weeks in 65 (30.9%) cases. In 204 (97.1%) cases the babies were live born and 98 (48.0%) were discharged from the hospital alive. There were 10 deaths directly related to difficulties with removal of the balloon. Significant prediction of survival was provided by the o/e LHR and gestational age at delivery. On the basis of the relationship between survival and o/e LHR in expectantly managed fetuses with CDH, as reported in the antenatal CDH registry, we estimated that in fetuses with left CDH treated with FETO the survival rate increased from 24.1% to 49.1%, and in right CDH survival increased from 0% to 35.3% (P < 0.001). Conclusions FETO in severe CDH is associated with a high incidence of PPROM and preterm delivery but a substantial improvement in survival. Copyright  2009 ISUOG. Published by John Wiley & Sons, Ltd.

/pdf/severe-diaphragmatic-hernia-treated-by-fetal-endoscopic-266bprgceo.pdf

Severe diaphragmatic hernia treated by fetal endoscopic tracheal occlusion.

Spontaneous rupture of membranes (ROM) is a normal component of labor and delivery. Premature ROM (PROM) refers to rupture of the fetal membranes prior to the onset of labor irrespective of gestational age. Once the membranes rupture, delivery is recommended when the risk of ascending infection outweighs the risk of prematurity. When PROM occurs at term, labor typically ensues spontaneously or is induced within 12 to 24 hours. The management of pregnancies complicated by preterm PROM (defined as PROM occurring prior to 37 weeks of gestation) is more challenging. Preterm PROM complicates 2% to 20% of all deliveries and is associated with 18% to 20% of perinatal deaths. Management options include admission to hospital, amniocentesis to exclude intra-amniotic infection, and administration of antenatal corticosteroids and broad-spectrum antibiotics, if indicated. This article reviews in detail the contemporary diagnosis and management of preterm PROM.

Contemporary Diagnosis and Management of Preterm Premature Rupture of Membranes

Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia

OBJECTIVE: To investigate the potential value of antenatally determined intrathoracic herniation of the liver and the ratio of fetal lung area to head circumference (LHR) in the prediction of postnatal survival in isolated, left-sided congenital diaphragmatic hernia (CDH). METHODS: In this multicenter study, we searched the antenatal-CDH-Registry for fetuses with isolated left-sided CDH that were liveborn after 30 weeks of gestation and were followed up postnatally for at least 3 months after discharge from hospital. The patients were subdivided into those with and those without intrathoracic herniation of the liver and into subgroups according to the LHR that had been measured between 22 + 0 and 28 + 0 weeks of gestation. The rate of survival in each group and subgroup of patients was determined and compared. RESULTS: The registry search identified 184 patients that fulfilled the entry criteria. Logistic regression analysis demonstrated that significant predictors of survival were the presence or absence of intrathoracic herniation of the liver and the LHR. In 86 cases there was liver herniation (Group A) and in 98 the liver was confined to the abdomen (Group B). In Group A, the survival rate was 50.0% and was significantly associated with LHR; in Group B the survival rate was 76.5% and was unrelated to LHR. CONCLUSION: In isolated, left-sided CDH, the postnatal mortality rate is substantially higher if there is intrathoracic herniation of the liver. In fetuses with liver herniation, measurement of LHR at 22-28 weeks of gestation is useful in the prediction of subsequent survival.

Prenatal prediction of survival in isolated left-sided diaphragmatic hernia.

A histological study of fetoscopic membrane defects to document membrane healing

http://koraszulesmegelozes.hu/sites/default/files/letoltesek/amniopatch2.pdf

Successful patching of iatrogenic rupture of the fetal membranes.

In a limited number of conditions in utero surgery may be life-saving, such as some cases of congenital diaphragmatic hernia, cystic adenomatoid malformation of the lung, sacrococcygeal teratoma and lower urinary tract obstruction. Postoperative premature labour and its extreme invasiveness have been major drawbacks for open fetal surgery. More recently the merger of fetoscopy and advanced video-endoscopic surgery has been the basis of the concept of endoscopic fetal surgery. In order to evaluate the opportunities of 'fetendo' surgery, animal models have been developed to test the safety of the endoscopic approach, and the feasibility of surgical manipulations on the fetus. In the non-human primate, a lesser invasiveness of endoscopy over open surgical approach was demonstrated, by a significant decrease in uterine activity in comparison with hysterotomy. The main application of fetoscopy today is the surgical treatment of complicated or abnormal monochorionic twin gestations. Fetoscopic laser coagulation of chorionic plate vessels is suggested as a causal therapy for severe feto-fetal transfusion syndrome. Survival rates are around 55 per cent with an incidence of five per cent of neurological morbidity. Fetoscopic cord ligation is associated with a 66 per cent survival rate, but unfortunately also with a risk of 30 per cent for PPROM prior to 32 weeks. Although still in its early experimental phase, endoscopy seems to offer new hope for surgical fetal therapy. Though conceptually very tempting, the development of endoscopic fetal surgery should follow the formal guidelines, as earlier formulated for open surgery by the International Fetal Medicine and Surgery Society. The prospective registration of worldwide experience is advocated and a randomized trial of laser therapy versus amniodrainage is announced.

Operative fetoscopy: new perspective in fetal therapy?

Operative fetoscopy may be limited by its relatively high associated risk of preterm prelabour rupture of membranes. The objective of this study was to study closure techniques of the access site for fetoscopy in the mid-gestational rabbit. A total of 32 does (288 amniotic sacs) at 22 days gestational age (GA; term = 32 days) underwent 14 gauge needle fetoscopy, by puncture through surgically exposed amnion. Entry site was randomly allocated to four closure technique groups: myometrial suture (n = 14), fibrin sealant (n = 15), autologous maternal blood plug (n = 13), collagen plug (n = 14); 16 sacs were left unclosed (positive controls), and the unmanipulated 216 sacs were negative controls. Membrane integrity, presence of amniotic fluid and fetal lung to body weight ratio (FLBWR) were evaluated at 31 days GA. Following fetoscopy without an attempt to close the membranes, amniotic integrity was restored in 41% of cases (amniotic integrity in controls 94%; P = 0.00001). When the access site was surgically closed, the amnion resealed in 20-44% of cases, but none of the tested techniques was significantly better than the others or than positive controls. Permanent amniotic disruption was associated with a significantly lower FLBWR in all groups. In conclusion, the rate of fetoscopy-induced permanent membrane defects in this model did not improve by using any of the closure techniques tested here.

Jan A. Deprest

Papers

Prenatal prediction of survival in isolated left-sided diaphragmatic hernia.

A histological study of fetoscopic membrane defects to document membrane healing

Successful patching of iatrogenic rupture of the fetal membranes.

Operative fetoscopy: new perspective in fetal therapy?

Closure techniques for fetoscopic access sites in the rabbit at mid-gestation