ABSTRACT: Congenital diaphragmatic hernia (CDH) is an anomaly that results in lung hypoplasia and pulmonary hypertension. The lungs of the CDH fetus have an abnormal architecture, with fewer bronchial branches and decreased number of arteries and veins, factors which result in pulmonary compromise postnatally. The goal of this review is to evaluate prenatal prognostic factors in the fetus with isolated left CDH, with particular emphasis on fetal MRI. These imaging indicators may be used to provide health professionals and the parents with the most accurate information about fetal prognosis.
Seminars in Pediatric Surgery 02/2013; 22(1):30-6. · 2.93 Impact Factor
ABSTRACT: To investigate the prognostic value of magnetic resonance-calculated fetal lung volumes (FLV) in fetuses with isolated left congenital diaphragmatic hernia (L-CDH) who receive standardized prenatal and postnatal care at a single institution.
A retrospective review was undertaken to identify fetuses with isolated L-CDH between 2001 and 2010.
Eighty-five cases of isolated L-CDH were identified. The overall survival was 65% (55/85). Survival was 45% if there was 'liver up' (23/51) and 94% if there was 'liver down' (32/34). Univariate statistical analysis showed that 'liver up' (p=0.001), lung-to-head ratio (LHR) at diagnosis (p=0.009), observed/expected (O/E) LHR (p=0.01), total FLV (p=0.03), right LV (p=0.04), magnetic resonance imaging (MRI) observed versus expected (O/E) FLV (p=0.002), intrathoracic versus intraabdominal stomach (p=0.002), percentage of herniated liver (p=0.004), and postnatal extracorporeal membrane oxygenation use (p=0.001) are predictive of postnatal survival. Multivariate analysis of only prenatal factors showed that the most important determinants of postnatal outcome are percentage of herniated liver, presence of liver up, and MRI O/E FLV.
Herniated intrathoracic liver expressed as 'liver up' or as percent herniated liver and MRI measurement of FLV expressed as a O/E ratio are strong prenatal indicators of postnatal survival.
Prenatal Diagnosis 06/2012; 32(8):715-23. · 2.11 Impact Factor
ABSTRACT: To determine the value of fetal MRI-calculated total lung volumes (TLV) in the prediction of short-term outcome in patients with giant omphalocele (GO).
We reviewed all cases of GO undergoing fetal MRI after 21 weeks' gestation and receiving postnatal care at our institution between 2003 and 2010. Observed/expected (O/E) TLV was calculated using age-matched TLV normograms [Radiology 2001;219:236-241]. Postnatal outcomes were stratified based on O/E TLV above or below 50% of expected.
Seventeen GO cases fulfilled the entry criteria. The mean age at fetal MRI evaluation was 25.8 ± 4.8 weeks' gestation. The mean GO TLV (21.0 ± 13.2) was lower than age-matched population norms (p < 0.001), resulting in a mean O/E TLV of 52.3 ± 16.8%. The mean gestational age at delivery was 36.8 ± 1.6 weeks. Overall survival was 94%. Fourteen (88%) infants underwent staged reduction, and 2 underwent silver sulfadiazine treatment and delayed repair. Infants with ≤50% of predicted O/E TLV (n = 11, 65%) had lower Apgar scores at birth (p = 0.03), prolonged ventilatory support (p = 0.004), delayed oral intake (p = 0.03), and longer hospitalization (p = 0.03) compared to patients with ≥50% of expected O/E TLV. Two infants (both O/E TLV <50%) required tracheostomy placement.
In the assessment of GO fetuses, MRI-based O/E TLV of <50% was predictive of increased postnatal morbidity.
Fetal Diagnosis and Therapy 04/2012; 31(4):248-53. · 1.05 Impact Factor