Diaphragmatic hernia in the fetus: Prenatal diagnosis and outcome in 94 cases

Adzick, N. Scott; Harrison, Michael R.; Glick, Philip L.; Nakayama, Donald K.; Manning, Frank A.; deLorimier, Alfred A.

doi:10.1016/s0022-3468(85)80219-0

Cited by 349 publications

(122 citation statements)

References 12 publications

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“…8 Many sonographic parameters have been proposed to predict the prognosis of CDH. Polyhydramnios in the third semester, 10 early diagnosis (less than 25 weeks' gestation), 4 presence of an intrathoracic stomach, 11 small lung-thorax transverse area ratio, 12 underdevelopment of the left heart, 13 and visceral herniation 14 have all been evaluated, but none of these has been widely accepted or applied clinically.…”

Section: Discussionmentioning

confidence: 99%

Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival?

Sbragia

Paek

Filly

et al. 2000

Journal of Ultrasound in Medicine

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The purpose of the present study was to determine the ability of lung‐to‐head ratio to predict survival and need for extracorporeal membrane oxygenation support in fetuses with left congenital diaphragmatic hernia without herniation of the liver into the chest. The perinatal records of 20 fetuses with isolated left congenital diaphragmatic hernia without herniation of the left lobe of the liver into the chest were reviewed. Fetuses were stratified into two groups depending on lung‐to‐head ratio: those with a ratio of less than 1.4 (historically a poor prognosis group) and those with a ratio of greater than 1.4. The outcome of both groups was compared with chi‐square analysis. Eight of 11 fetuses with a lung‐to‐head ratio greater than 1.4 survived, whereas 8 of 9 fetuses with a ratio of less than 1.4 survived. No differences were noted in the need for extracorporeal membrane oxygenation support or survival between the two groups. Fetuses with a prenatally diagnosed left congenital diaphragmatic hernia without herniation of liver into the chest have a favorable prognosis even in the presence of a low lung‐to‐head ratio.

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Section: Discussionmentioning

confidence: 99%

Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival?

Sbragia

Paek

Filly

et al. 2000

Journal of Ultrasound in Medicine

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“…Other groups have shown a mortality of 60% to 80% in patients with CDH. 11,12 Several investigators have reported a significant impact of ECMO on the survival of infants born with CDH. [13][14][15] In a single institution study, the post-ECMO survival was 69% compared with only 5% in pre-ECMO historical controls.…”

Section: Discussionmentioning

confidence: 99%

Survival, Intracranial Lesions, and Neurodevelopmental Outcome in Infants With Congenital Diaphragmatic Hernia Treated With Extracorporeal Membrane Oxygenation

et al. 1999

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OBJECTIVE:Before the use of extracorporeal membrane oxygenation (ECMO), infants with a severe form of congenital diaphragmatic hernia (CDH) had a high mortality and morbidity. Recent studies have shown an improvement in the survival of these infants after ECMO treatment; however, the existing data do not provide sufficient information regarding the quality of survival and developmental outcome of these infants. The objective of this study was to evaluate survival, intracranial lesions, and the neurodevelopmental outcome of infants with CDH treated with ECMO. METHODS:We retrieved data for 51 (n ϭ 51) infants with CDH who were treated with ECMO at Huntington Memorial Hospital between 1985 and 1994. Their mean gestational age was 38.5 Ϯ 2.4 weeks (mean Ϯ SD); their mean birth weight was 3170 Ϯ 620 gm. Vital signs, arterial blood gases, chest radiographs, cranial and cardiac ultrasonography were routinely obtained before ECMO treatment. Cranial ultrasounds were performed daily on all infants while on ECMO; computerized tomography scans were obtained on all infants after completion of ECMO treatment. The surviving infants were followed at our neonatal follow-up clinic for neurodevelopmental assessment. RESULTS:A total of 39 infants were placed on venoarterial ECMO and 12 infants were placed on venovenous ECMO; a total of 35 infants had CDH repair before ECMO, whereas 16 infants had delayed surgery. A total of 31 infants (61%) survived. The infants who survived had a mean pH of 7.33 Ϯ 0.20, mean airway pressure of 19.6 Ϯ 5.8 cm H 2 O, and an oxygenation index (OI) of 87 Ϯ 55 before ECMO intervention. The infants who expired (n ϭ 20) had a mean pH of 7.31 Ϯ 0.15, mean airway pressure of 23.1 Ϯ 5.5 cm H 2 O, and a mean oxygenation index of 127 Ϯ 56 before ECMO treatment. Before ECMO, survivors had a significantly lower oxygenation index and a higher PaO 2 compared with nonsurvivors (p Ͻ 0.01). A total of 18 infants (35%) had abnormal central nervous system findings. Of the 51 infants, 10 had ventricular dilatation, 6 had intracranial hemorrhage, and 11 had focal or diffuse cerebral atrophy diagnosed by computerized tomography scan or at autopsy (1 patient had an infarct). Eight infants had more than one central nervous system abnormality. A total of 16 survivors had a neurodevelopmental evaluation at 12 months, and 11 of these survivors were evaluated at 24 months of age (Bayley Scales of Infant Development). The developmental progress of these infants falls within the low-average range of cognitive and motor abilities. Their mean Bayley Mental Developmental Index was 85 Ϯ 25 (50 to 145) at 24 months; their Psychomotor Developmental Index was 89 Ϯ 21 (50 to 113) at 24 months of age. Follow-up at 4 and 6 years of age is in progress. CONCLUSION:Our preliminary findings indicate that 35% of infants with severe CDH requiring ECMO had central nervous system abnormalities (intracranial lesions, including ventricular dilatation). The survival rate in our study population is consistent with recent reports. As a group, infants w...

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“…ongenital diaphragmatic hernia (CDH) diagnosed in utero has a neonatal mortality rate of 50% to 80%, [1][2][3][4][5][6][7] death being mainly due to pulmonary hypoplasia. Predicting the neonatal outcome prenatally remains a challenge.…”

mentioning

confidence: 99%

Evaluation of Fetal Pulmonary Vasculature by Power Doppler Imaging in Congenital Diaphragmatic Hernia

Michelet

Aubry

Sayed

et al. 2004

J of Ultrasound Medicine

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Objective. To evaluate the feasibility of prenatal power Doppler imaging of pulmonary arteries in congenital diaphragmatic hernia and to study its potential to predict outcome. Methods. A prospective observational study was conducted. Forty-two cases of congenital diaphragmatic hernia (32 left and 10 right) without associated anomalies were analyzed. Qualitative evaluation of pulmonary vasculature was based on power Doppler imaging performed at 26 to 38 weeks. The pulmonary arteries were studied in the lung contralateral to the hernia. Pulmonary Doppler angiography was considered satisfactory when 3 levels of bifurcation defining 3 distinct segments of the pulmonary arteries were imaged and was otherwise considered poor. We also recorded the gestational age at diagnosis, side of the hernia, abdominal circumference below the third percentile, amniotic fluid volume, lung/thoracic area ratio, left/right ventricle ratio, and, in left-sided hernias, stomach position, and we carried out a multivariate analysis to determine the contribution of each factor to predict neonatal mortality. Results. More than 3 divisions of the fetal pulmonary arteries were imaged in 20 cases; 1 or 2 divisions or none were imaged in 22. Neonatal mortality was significantly greater when fewer than 3 divisions of the pulmonary arteries were imaged (18 [82%] of 22) than when 3 divisions could be identified on power Doppler imaging (5 [25%] of 20; P = .0005). However, the lung/thoracic area ratio was the only factor that remained significantly associated with mortality in the multivariate analysis. Conclusions. An altered pulmonary power Doppler image is associated with neonatal mortality, but estimation of the lung area remains the best predictor of neonatal outcome. Key words: congenital diaphragmatic hernia; lung/head ratio; lung/thorax ratio; power Doppler imaging; prenatal diagnosis; pulmonary hypoplasia; sonography. ongenital diaphragmatic hernia (CDH) diagnosed in utero has a neonatal mortality rate of 50% to 80%, 1-7 death being mainly due to pulmonary hypoplasia. Predicting the neonatal outcome prenatally remains a challenge. Moreover, prenatal staging of CDH is becoming increasingly important as new intrauterine therapeutic interventions emerge, including minimally invasive fetal surgery.8 So far, research on prenatal prediction of pulmonary hypoplasia has focused on using sonography or magnetic resonance imaging to assess the size of the lungs, 7,9-14 the degree of herniation of abdominal organs, 6,15,16 and the consequences of fetal hemodynamic changes on left ventricular size. 17,18 In CDH, however, pulmonary hypoplasia is associated with alterations of the pulmonary vasculature, accounting for potentially lethal postnatal pulmonary hypertension.19

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Diaphragmatic hernia in the fetus: Prenatal diagnosis and outcome in 94 cases

Cited by 349 publications

References 12 publications

Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival?

Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival?

Survival, Intracranial Lesions, and Neurodevelopmental Outcome in Infants With Congenital Diaphragmatic Hernia Treated With Extracorporeal Membrane Oxygenation

Evaluation of Fetal Pulmonary Vasculature by Power Doppler Imaging in Congenital Diaphragmatic Hernia

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