Outcome in fetuses with isolated congenital diaphragmatic hernia with increased nuchal translucency thickness in first trimester
AP-HP, Department of Obstetrics and Gynecology, Necker Enfants-Malades Hospital, Paris, France. Prenatal Diagnosis
(Impact Factor: 3.27).
03/2012; 32(3):268-71. DOI: 10.1002/pd.3819
To examine the possible association between increased nuchal translucency (NT) thickness in the first trimester and perinatal outcome in isolated congenital diaphragmatic hernia (CDH).
We conducted a retrospective study between January 2004 and June 2010. The database was searched to identify all consecutive cases of CDH referred to the fetal medicine center of Necker Hospital in Paris. Enlarged NT was defined above the 95th centile. Only children born alive with an isolated CDH were selected for the analysis of prognostic factors. We also studied the correlation between NT thickness in the first trimester and lung-to-head ratio, observed to expected lung area-to-head ratio, lung volume estimated by magnetic resonance imaging, and other prenatal features of intrathoracic compression.
Seventy-one cases of isolated CDH were available. The fetal NT was above the 95th centile in 9 of the 71 cases. Neonatal death occurred in 7/9 (78%) cases with enlarged NT, compared with 24/62 (38%) with normal NT (P = 0.035). Enlarged NT was significantly associated with prenatal features of intrathoracic compression.
Enlarged NT thickness in CDH is associated with a poor outcome and is related to an early intrathoracic compression.
Available from: Jong Kwan Jun
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ABSTRACT: sAimThe aim of this study was to evaluate the usefulness of abdomen-to-thorax ratio (ATR) measured by antenatal ultrasonography for predicting neonatal survival of fetuses with congenital diaphragmatic hernia (CDH).Material and Methods
We identified 75 fetuses who were prenatally diagnosed with CDH and delivered in Seoul National University Hospital from 1998 to 2012, retrospectively. Of these, 40 cases who met the following criteria were included: (i) singleton pregnancy; (ii) a case that had available ultrasonographic images; and (iii) a neonate in whom follow-up until discharge from the hospital was available. ATR was measured as follows: each anteroposterior distance (APD) in the abdominal and thoracic cavity was measured in the same midsagittal plane with the fetal neutral position. A thoracic APD was measured from the back to the distal end of the sternum and an abdominal APD from the back to the most protruding abdominal surface. ATR is the ratio of the abdominal APD versus the thoracic APD.ResultsSurvival rates were 57.5%. There were significant differences in ATR, lung–head ratio (LHR), observed/expected-LHR, quantitative lung index and intrathoracic liver between survivors and non-survivors. Regression analysis demonstrated that only ATR and intrathoracic position of the liver were independent predictors of survival. ATR by multivariate analysis had the most influence on survival rate (P = 0.002). The area under the receiver–operator curve for prediction of survival from ATR was 0.770 (P = 0.004). The diagnostic cut-off value for ATR was 0.96.Conclusions
Because ATR is effective to predict neonatal survival in CDH fetuses and is easy to measure, it can be used as another powerful parameter for managing CDH fetuses.
Available from: Angelos Daniilidis
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ABSTRACT: Increased nuchal translucency (NT) thickness is present in 40% of fetuses with diaphragmatic hernia, including 80% of those that result in neonatal death and in 20% of the survivors. A 33-year-old nulliparous woman had first trimester scan at 12 weeks. The fetus had a NT of 2.3 mm, normal ductus venosus (DV), and tricuspid doppler and present nasal bone. Pregnancy-associated plasma protein A (PAPP-A) was 0.59 MoM and beta-human chorionic gonadotropin (b-hCG) 2.56 MoM. The couple did not opt for chorionic villous sampling (CVS) and repeat ultrasound examination was advised. At 18 weeks, ultrasound revealed left sided diaphragmatic hernia. The couple consented for termination of the pregnancy. The molecular test showed normal karyotype and male gender. In such cases with intrathoracic herniation of abdominal viscera, the increased NT may be the consequence of venous congestion due to mediastinal compression. The prolonged compression of the lungs causes pulmonary hypoplasia. Increased NT with normal fetal karyotype is associated with structural fetal anomalies like diaphragmatic hernia and screening at 16-18 weeks is imperative.
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ABSTRACT: To study the relationship between gestational age at diagnosis and mortality and morbidity in fetuses with an isolated congenital diaphragmatic hernia.
Between January 2008 and November 2013, 377 live births with isolated congenital diaphragmatic hernia diagnosed antenatally at a known gestational age were recorded in the database of the French National Center for Rare Diseases. The primary outcome studied was mortality estimated at 28 days and at 6 months. The secondary outcome was morbidity evaluated by pulmonary arterial hypertension at 48 hours, oxygen therapy dependence at 28 days, oral disorders, enteral feeding and prosthetic patch repair. Analyses were adjusted for the main factors of congenital diaphragmatic hernia severity (side of the hernia, thoracic herniation of the liver, gestational age at birth, Lung-to-Head Ratio and prenatal treatment by tracheal occlusion.
Mortality rates at 28 days decreased significantly (p<0.001) when gestational age at diagnosis increased: 61.1%, 39.2 % and 10.4% for a diagnosis in the first, second and third trimester, respectively. Adjusted odds ratios were 3.12 [1.86-5.25] and 0.35 [0.18-0.66] for a diagnosis in the first and third trimester, respectively, compared with a diagnosis in the second trimester. Similarly, morbidity decreased significantly when gestational age at diagnosis increased and the trend remained significant after adjustment for the main factors of congenital diaphragmatic hernia severity (p<0.001).
Gestational age at diagnosis is an independent predictor of postnatal prognosis for children presenting an isolated congenital diaphragmatic hernia and should be taken into account when estimating postnatal morbidity and mortality.
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