Survival in very preterm infants with congenital diaphragmatic hernia and association with prenatal imaging markers: A retrospective cohort study

Horn-Oudshoorn, Emily J J; Russo, Francesca Maria; Deprest, Jan; Kipfmueller, Florian; Geipel, A.; Schaible, Thomas; Rafat, Neysan; Cordier, Anne-Gaël; Benachi, Alexandra; Abbasi, Nimrah; Chiu, Pao Chin; Boode, Willem P. de; Sikkel, Esther; Peters, N.; Hansen, Bettina E.; DeKoninck, Philip

doi:10.1111/1471-0528.17497

Cited by 7 publications

(2 citation statements)

References 37 publications

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“…17 Furthermore, preterm birth before 37 weeks of gestational age has been consistently associated with an unfavorable prognosis in neonates with CDH. 18,19 In contrast, patients with left-sided CDH, an o/e LHR of ≥46%, and an intraabdominal liver position represent a group characterized by a more favorable prognosis. 20 Consequently, we have adopted a combination of these criteria as a basis for considering SBA in CDH neonates.…”

Section: Discussionmentioning

confidence: 99%

Spontaneous breathing in selected neonates with very mild congenital diaphragmatic hernia

Kipfmueller,

Leyens,

Pugnaloni

et al. 2023

Pediatric Pulmonology

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AimsCurrent treatment guidelines recommend immediate postnatal intubation in all neonates with congenital diaphragmatic hernia (CDH). This study aimed to investigate the feasibility and outcomes of a spontaneous breathing approach (SBA) versus immediate intubation in neonates with prenatally diagnosed very mild CDH.MethodsA retrospective study was conducted comparing neonates with very mild CDH (left‐sided, liver‐down, observed‐to‐expected lung‐to‐head ratio ≥45%) undergoing SBA and matched controls receiving standard treatment. Data on early echocardiographic findings, respiratory support, length of hospital stay, and clinical outcomes were analyzed.ResultsOf 151 CDH neonates, eight underwent SBA, while 31 received standard treatment. SBA was successful in six of eight patients. SBA patients had shorter length of stay (14 vs. 30 days, p = .005), mechanical ventilation (3.5 vs. 8.7 days, p = .011), and oxygen supplementation (3.2 vs. 9.3 days, p = .013) compared to matched controls. Echocardiographic evidence of pulmonary hypertension and cardiac dysfunction were significantly lower in SBA neonates after admission but similar before surgical repair. The SBA group tolerated enteral feeding earlier (day of life 7 vs. 16, p = .019).ConclusionsSBA appears feasible and beneficial for prenatally diagnosed very mild CDH. It was associated with a shortened hospital stay supportive therapies. However, larger trials are needed to confirm these findings and determine optimal respiratory support.

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Section: Discussionmentioning

confidence: 99%

Spontaneous breathing in selected neonates with very mild congenital diaphragmatic hernia

Kipfmueller,

Leyens,

Pugnaloni

et al. 2023

Pediatric Pulmonology

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“…The risk of preterm delivery is elevated when CDH is diagnosed in the fetus, estimated to be between 22 and 35%. Cohort studies posit that preterm birth amplifies the mortality rate in CDH-affected fetuses, with a higher risk for RSCDH [17,18]. One potential risk factor for premature delivery is the presence of polyhydramnios due to oesophageal compression and fetal swallowing impairment [8].…”

Section: Left Cdh Right Cdhmentioning

confidence: 99%

Prenatal diagnosis of right-sided congenital diaphragmatic hernia

Sokołowski,

Pałgan,

Strzelecka

et al. 2023

pcard

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This case report details the prenatal diagnosis and management of a right-sided congenital diaphragmatic hernia (CDH) in a 37-year-old pregnant woman. In the 22 nd week of the pregnancy, a screening ultrasound revealed an abnormal mass in the right fetal chest, suspected to be a diaphragmatic hernia. A series of fetal ultrasound and echocardiographic examinations were conducted, revealing a mass of intestines in the right chest displacing the heart (with normal heart anatomy). Fetal magnetic resonance imaging at 27 weeks confirmed the diaphragmatic hernia. Subsequent monitoring revealed unfavourable prognostic indicators, including a decreased lung-tohead ratio (LHR) index. Premature rupture of membranes occurred in the 28th week. An urgent caesarean section was performed at 31 weeks due to the onset of labour and signs of fetal distress, and a female newborn with a birth weight of 1800 g was delivered. Right-sided diaphragmatic hernia was confirmed postnatally. Surgical intervention was undertaken to relocate the intestines and close the diaphragmatic defect. Despite initial efforts, the newborn's respiratory and circulatory function deteriorated, leading to the unfortunate demise on the sixth day of life.

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Observed‐to‐expected lung‐area‐to‐head‐circumference ratio on ultrasound examination vs total fetal lung volume on magnetic resonance imaging in prediction of survival in fetuses with left‐sided diaphragmatic hernia

Dütemeyer,

Schaible,

Badr

et al. 2024

Ultrasound in Obstet & Gyne

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ObjectiveTo assess and compare the value of antenatally determined observed‐to‐expected (O/E) lung‐area‐to‐head‐circumference ratio (LHR) on ultrasound examination vs O/E total fetal lung volume (TFLV) on MRI examination to predict postnatal survival of fetuses with isolated, expectantly managed left‐sided congenital diaphragmatic hernia (CDH).MethodsThis was a multicenter retrospective study including all consecutive fetuses with isolated CDH that were managed expectantly in Mannheim, Germany, and in five other European centers, that underwent at least one ultrasound examination for measurement of O/E‐LHR and one MRI scan for measurement of O/E‐TFLV during pregnancy. All MRI data were centralized, and lung volumes were measured by two experienced operators blinded to the pre‐ and postnatal data. Multiple logistic regression analyses were performed to examine the effect on survival at hospital discharge of various perinatal variables, including the center of management. In left‐sided CDH with intrathoracic herniation of the liver, receiver‐operating‐characteristics (ROC) curves were constructed separately for cases from Mannheim and the other five European centers and were used to compare O/E‐TFLV and O/E‐LHR in the prediction of postnatal survival.ResultsFrom Mannheim, 309 patients were included with a median gestational age (GA) at ultrasound examination of 29.6 (range, 19.7–39.1) weeks and median GA at MRI examination of 31.1 (range, 18.0–39.9) weeks. From the other five European centers, 116 patients were included with a median GA at ultrasound examination of 26.7 (range, 20.6–37.6) weeks and median GA at MRI examination of 27.7 (range, 21.3–37.9) weeks. Regression analysis demonstrated that the survival rates at discharge were lower in left‐sided CDH (odds ratio (OR), 0.349 (95% CI, 0.133–0.918), P = 0.033) and those with intrathoracic liver (OR, 0.297 (95% CI, 0.141–0.628), P = 0.001), and higher with increasing O/E‐TFLV (OR, 1.123 (95% CI, 1.079–1.170), P < 0.001), advanced GA at birth (OR, 1.294 (95% CI, 1.055–1.588), P = 0.013) and when birth occurred in Mannheim (OR, 7.560 (95% CI, 3.368–16.967), P < 0.001). Given the difference in survival rate between Mannheim and the five other European centers, ROC curve comparisons between the two imaging modalities were presented separately. For cases of left‐sided CDH with intrathoracic herniation of the liver, pairwise comparison showed no significant difference between the area under the ROC curves for the prediction of postnatal survival between O/E‐TFLV and O/E‐LHR in Mannheim (mean difference = 0.025, P = 0.610, standard error = 0.050), whereas there was a significant difference in the other European centers studied (mean difference = 0.056, P = 0.033, standard error = 0.056).ConclusionsIn fetuses with left‐sided CDH and intrathoracic herniation of the liver, the predictive value for postnatal survival of O/E‐TFLV on MRI examination and O/E‐LHR on ultrasound examination was similar in one center (Mannheim), but O/E‐TFLV had better predictive value compared to O/E‐LHR in the five other European centers. Hence, in these five European centers, MRI should be included in the diagnostic process for left‐sided CDH. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

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Survival in very preterm infants with congenital diaphragmatic hernia and association with prenatal imaging markers: A retrospective cohort study

Cited by 7 publications

References 37 publications

Spontaneous breathing in selected neonates with very mild congenital diaphragmatic hernia

Spontaneous breathing in selected neonates with very mild congenital diaphragmatic hernia

Prenatal diagnosis of right-sided congenital diaphragmatic hernia

Observed‐to‐expected lung‐area‐to‐head‐circumference ratio on ultrasound examination vs total fetal lung volume on magnetic resonance imaging in prediction of survival in fetuses with left‐sided diaphragmatic hernia

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