Congenital diaphragmatic hernias are complex and life-threatening lesions that are not just anato... more Congenital diaphragmatic hernias are complex and life-threatening lesions that are not just anatomic defects of the diaphragm, but represent a complex set of physiologic derangements of the lung, the pulmonary vasculature, and related structures. Imaging plays an increasingly important role in the care of these infants. Prenatal sonography and MRI have allowed early and accurate identification of the defect and associated anomalies. These tools have also been the key to defining the degree of pulmonary hypoplasia and to predicting neonatal survival and need for aggressive respiratory rescue strategies. In the postnatal period, conventional radiography supplemented by cross-sectional imaging in selected cases can be very useful in sorting out the differential diagnosis of intrathoracic masses, in the detection of associated anomalies, and in the management of complications. Understanding the pathogenesis of diaphragmatic defects, the underlying physiologic disturbances, and the strengths and limitations of current imaging protocols is essential to the effective and accurate management of these complex patients.
We evaluated a subset of infants with bilateral markedly hyperechoic "bright&... more We evaluated a subset of infants with bilateral markedly hyperechoic "bright" kidneys noted prenatally in association with normal amniotic fluid volume during gestation. Prenatal ultrasound showed increased renal parenchymal echogenicity bilaterally with preservation of the medullary pyramid architecture. These children were followed for up to 3 years to determine potential changes in the sonographic appearance of the echogenic renal parenchyma and to assess renal function. In 3 years 8 cases of bilateral hyperechoic fetal kidneys were identified. During postnatal followup renal echogenicity resolved in 4 cases, diminished in 1 and remained the same in 3. The serum creatinine and electrolytes were normal in all cases followed for more than 3 months. Other renal findings included vesicoureteral reflux in 2 of 7 cases, mild pelvic ectasia in 1 and tiny medullary calcifications in 1. The specific etiology of increased echogenicity is unknown, although 1 infant appeared to have a form of autosomal recessive disease with liver hyperechogenicity as well. We conclude that fetuses with bilateral hyperechoic kidneys associated with normal amniotic fluid volume have a favorable outcome. Ultrasonographic finding of marked parenchymal hyperechogenicity appears to improve with time; in our experience renal function is normal and the infants thrive. Continued followup is necessary to determine the long-term natural history of this phenomenon, as is subsequent prenatal and postnatal evaluation of future siblings to assess genetic risk.
Background A mother’s circulating estrogen increases over the third trimester, producing physiolo... more Background A mother’s circulating estrogen increases over the third trimester, producing physiological effects on her newborn that wane postnatally. Estrogenization might be prolonged in newborns exposed to exogenous estrogens, such as isoflavones in soy formula. Objective We evaluated ultrasonography for monitoring growth of multiple estrogen-responsive organs in healthy infants and developed organ-growth trajectories. Materials and methods We studied 38 boys (61 visits) from birth to age 6 months and 41 girls (96 visits) from birth to age 1 year using a partly cross-sectional, partly longitudinal design. We measured uterus and ovaries in girls, testes and prostate in boys, and kidneys, breasts, thymus, and thyroid in all children. We imaged all organs from the body surface in one session of < 1 h. Results Uterine volume decreased from birth (P < 0.0001), whereas ovarian volume increased sharply until age 2 months and then decreased (P < 0.001). Testicular volume increased with age (P < 0.0001), but prostatic volume showed minimal age trend. Breast bud diameter showed no age trend in girls but declined from birth in boys (P = 0.03). Conclusion US examination of multiple estrogen-responsive organs in infants in a single session is feasible and yields volume estimates useful for assessing potential endocrine disruptor effects on organ growth.
Fetal MR imaging is complementary to obstetric ultrasonography. The additional information provid... more Fetal MR imaging is complementary to obstetric ultrasonography. The additional information provided by in utero MR imaging may alter prenatal, perinatal, or immediate postnatal management. For example, the MR imaging findings may affect the decision to continue the pregnancy, change the mode, timing, or location of delivery, or modify decisions regarding the necessity of immediate postnatal surgery. Finally, the information contributed by MR may permit a better assessment of the risk of recurrent defects in subsequent pregnancies.
To elucidate the appearance of fetal thoracic abnormalities at prenatal magnetic resonance (MR) i... more To elucidate the appearance of fetal thoracic abnormalities at prenatal magnetic resonance (MR) imaging and determine whether MR imaging yields information additional to that obtained with ultrasonography (US). US and MR imaging data from 83 MR examinations of 74 fetuses with thoracic abnormalities and confirmatory US performed within 1 week before MR imaging were compared with respect to resulting changes in patient counseling and/or care. Lung parenchyma and lesion signal intensities and vascularity, airway, esophagus, and diaphragm appearances were reviewed retrospectively on MR images. Student t tests and analyses of variance were performed. MR imaging yielded information additional to that acquired with US in 28 (38%) of 74 fetuses. The additional findings were confirmed in 19 of the 28 fetuses at postnatal follow-up; no follow-up data were available for the other nine fetuses. Thoracic MR information affected care with regard to six (8%) of 74 fetuses. Mean gestational age of 15 fetuses with lung signal intensity (SI) slightly lower than that of amniotic fluid (28.4 weeks +/- 6.8 [SD]) at T2-weighted MR imaging was significantly older than that of 18 fetuses with intermediate SI (21.3 weeks +/- 4.3) (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;.05). Mean SI of 13 congenital cystic adenomatoid malformations (CCAMs) and/or sequestrations (1.74 +/- 1.05) at T2-weighted MR imaging was significantly higher than that of the normal lungs of 33 fetuses (2.63 +/-.63) (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;.001). Among nine studies in which vessels were visualized in CCAMs and/or sequestrations, six involved a normal vascular branching pattern. Portions of the esophagus were seen in 31 (36%) of 85 fetuses. Nonvisualization of a major airway was not sufficient for diagnosis of pulmonary atresia. Visualization of a portion of the esophagus did not correlate with esophageal atresia. In all except one fetus, who had anhydramnios and pulmonary hypoplasia, and the fetuses with congenital diaphragmatic hernia, at least a portion of the diaphragm was visualized at MR imaging. MR imaging yields information additional to that yielded with US in fetuses with thoracic abnormalities.
We aimed to identify risk factors for neonatal surgical airway intervention among fetuses with pr... more We aimed to identify risk factors for neonatal surgical airway intervention among fetuses with prenatally diagnosed cervical masses. An 8-year retrospective review identified 23 consecutive patients with a prenatal diagnosis of a neck mass, managed at a single tertiary center. Variables analyzed included anticipated diagnosis, extent of the mass, need for any surgical airway intervention in the neonatal period, final histopathology data, and survival. Statistical analysis was based on the Fisher and Fisher-Freeman-Halton exact tests (significance set at P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; or = .05) and exact 95% confidence intervals for risk differences. Eight patients underwent termination of pregnancy or were lost to follow-up. The imaging-based prenatal diagnosis was confirmed postnatally in 93% (14/15) of the remaining patients. Final diagnoses included lymphatic malformation (8), teratoma (6), and esophageal duplication (1). Teratomas were associated with a significantly higher risk for neonatal airway intervention than lymphatic malformations (67% vs 11%, P = .02). The majority of such procedures were performed under ex utero intrapartum treatment. Survival was 93% (14/15). Cervical teratomas are significantly more likely to demand surgical airway intervention in the neonate, typically under ex utero intrapartum treatment, than cervical lymphatic malformations. These findings should be considered in the prenatal counseling for fetal cervical masses.
Congenital cystic adenomatoid malformation (CCAM), intralobar sequestration (ILS), extralobar seq... more Congenital cystic adenomatoid malformation (CCAM), intralobar sequestration (ILS), extralobar sequestration (ELS), and lobar emphysema (LE) are well-accepted entities; however, certain findings are common to all, particularly the parenchymal maldevelopment characterizing CCAM. Isolated reports have described bronchial atresia (BA) in some specimens in all 4 entities, but this finding has not been evaluated in a prospective manner. With the aid of a dissecting microscope, we prospectively examined 47 lung specimens resected during the past 4 years and submitted with the clinical impression of ELS (n=11), ILS (n=11), CCAM (n=20), LE (n=4), and airway-esophageal communication (n=1). Most lesions were detected by prenatal ultrasound and were resected during infancy. The clinical impression and pathologic findings were compared. Pathologic examination revealed atresia of a lobar, segmental, or subsegmental bronchus in 100% of ELS, 82% of ILS, 70% of CCAM, and 50% of LE (those clinically recognized to have BA or minor CCAM) cases. Parenchymal maldevelopment that characterizes CCAM was present in 100% of CCAM cases (as expected by definition) as well as in 91% of ELS, 91% of ILS, and 50% of LE (those with BA) cases. Bronchial atresia is present in all ELS, most ILS and CCAM, and some LE cases, and its detection is greatly enhanced with the dissecting microscope. Bronchial atresia and CCAM nearly always coexist. It may be that both have the same etiopathogenesis with anatomic differences accounted for by aberrant genetic programs or other insults, perhaps modified by time of onset or duration.
Congenital diaphragmatic hernias are complex and life-threatening lesions that are not just anato... more Congenital diaphragmatic hernias are complex and life-threatening lesions that are not just anatomic defects of the diaphragm, but represent a complex set of physiologic derangements of the lung, the pulmonary vasculature, and related structures. Imaging plays an increasingly important role in the care of these infants. Prenatal sonography and MRI have allowed early and accurate identification of the defect and associated anomalies. These tools have also been the key to defining the degree of pulmonary hypoplasia and to predicting neonatal survival and need for aggressive respiratory rescue strategies. In the postnatal period, conventional radiography supplemented by cross-sectional imaging in selected cases can be very useful in sorting out the differential diagnosis of intrathoracic masses, in the detection of associated anomalies, and in the management of complications. Understanding the pathogenesis of diaphragmatic defects, the underlying physiologic disturbances, and the strengths and limitations of current imaging protocols is essential to the effective and accurate management of these complex patients.
We evaluated a subset of infants with bilateral markedly hyperechoic &amp;amp;quot;bright&... more We evaluated a subset of infants with bilateral markedly hyperechoic &amp;amp;quot;bright&amp;amp;quot; kidneys noted prenatally in association with normal amniotic fluid volume during gestation. Prenatal ultrasound showed increased renal parenchymal echogenicity bilaterally with preservation of the medullary pyramid architecture. These children were followed for up to 3 years to determine potential changes in the sonographic appearance of the echogenic renal parenchyma and to assess renal function. In 3 years 8 cases of bilateral hyperechoic fetal kidneys were identified. During postnatal followup renal echogenicity resolved in 4 cases, diminished in 1 and remained the same in 3. The serum creatinine and electrolytes were normal in all cases followed for more than 3 months. Other renal findings included vesicoureteral reflux in 2 of 7 cases, mild pelvic ectasia in 1 and tiny medullary calcifications in 1. The specific etiology of increased echogenicity is unknown, although 1 infant appeared to have a form of autosomal recessive disease with liver hyperechogenicity as well. We conclude that fetuses with bilateral hyperechoic kidneys associated with normal amniotic fluid volume have a favorable outcome. Ultrasonographic finding of marked parenchymal hyperechogenicity appears to improve with time; in our experience renal function is normal and the infants thrive. Continued followup is necessary to determine the long-term natural history of this phenomenon, as is subsequent prenatal and postnatal evaluation of future siblings to assess genetic risk.
Background A mother’s circulating estrogen increases over the third trimester, producing physiolo... more Background A mother’s circulating estrogen increases over the third trimester, producing physiological effects on her newborn that wane postnatally. Estrogenization might be prolonged in newborns exposed to exogenous estrogens, such as isoflavones in soy formula. Objective We evaluated ultrasonography for monitoring growth of multiple estrogen-responsive organs in healthy infants and developed organ-growth trajectories. Materials and methods We studied 38 boys (61 visits) from birth to age 6 months and 41 girls (96 visits) from birth to age 1 year using a partly cross-sectional, partly longitudinal design. We measured uterus and ovaries in girls, testes and prostate in boys, and kidneys, breasts, thymus, and thyroid in all children. We imaged all organs from the body surface in one session of < 1 h. Results Uterine volume decreased from birth (P < 0.0001), whereas ovarian volume increased sharply until age 2 months and then decreased (P < 0.001). Testicular volume increased with age (P < 0.0001), but prostatic volume showed minimal age trend. Breast bud diameter showed no age trend in girls but declined from birth in boys (P = 0.03). Conclusion US examination of multiple estrogen-responsive organs in infants in a single session is feasible and yields volume estimates useful for assessing potential endocrine disruptor effects on organ growth.
Fetal MR imaging is complementary to obstetric ultrasonography. The additional information provid... more Fetal MR imaging is complementary to obstetric ultrasonography. The additional information provided by in utero MR imaging may alter prenatal, perinatal, or immediate postnatal management. For example, the MR imaging findings may affect the decision to continue the pregnancy, change the mode, timing, or location of delivery, or modify decisions regarding the necessity of immediate postnatal surgery. Finally, the information contributed by MR may permit a better assessment of the risk of recurrent defects in subsequent pregnancies.
To elucidate the appearance of fetal thoracic abnormalities at prenatal magnetic resonance (MR) i... more To elucidate the appearance of fetal thoracic abnormalities at prenatal magnetic resonance (MR) imaging and determine whether MR imaging yields information additional to that obtained with ultrasonography (US). US and MR imaging data from 83 MR examinations of 74 fetuses with thoracic abnormalities and confirmatory US performed within 1 week before MR imaging were compared with respect to resulting changes in patient counseling and/or care. Lung parenchyma and lesion signal intensities and vascularity, airway, esophagus, and diaphragm appearances were reviewed retrospectively on MR images. Student t tests and analyses of variance were performed. MR imaging yielded information additional to that acquired with US in 28 (38%) of 74 fetuses. The additional findings were confirmed in 19 of the 28 fetuses at postnatal follow-up; no follow-up data were available for the other nine fetuses. Thoracic MR information affected care with regard to six (8%) of 74 fetuses. Mean gestational age of 15 fetuses with lung signal intensity (SI) slightly lower than that of amniotic fluid (28.4 weeks +/- 6.8 [SD]) at T2-weighted MR imaging was significantly older than that of 18 fetuses with intermediate SI (21.3 weeks +/- 4.3) (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;.05). Mean SI of 13 congenital cystic adenomatoid malformations (CCAMs) and/or sequestrations (1.74 +/- 1.05) at T2-weighted MR imaging was significantly higher than that of the normal lungs of 33 fetuses (2.63 +/-.63) (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;.001). Among nine studies in which vessels were visualized in CCAMs and/or sequestrations, six involved a normal vascular branching pattern. Portions of the esophagus were seen in 31 (36%) of 85 fetuses. Nonvisualization of a major airway was not sufficient for diagnosis of pulmonary atresia. Visualization of a portion of the esophagus did not correlate with esophageal atresia. In all except one fetus, who had anhydramnios and pulmonary hypoplasia, and the fetuses with congenital diaphragmatic hernia, at least a portion of the diaphragm was visualized at MR imaging. MR imaging yields information additional to that yielded with US in fetuses with thoracic abnormalities.
We aimed to identify risk factors for neonatal surgical airway intervention among fetuses with pr... more We aimed to identify risk factors for neonatal surgical airway intervention among fetuses with prenatally diagnosed cervical masses. An 8-year retrospective review identified 23 consecutive patients with a prenatal diagnosis of a neck mass, managed at a single tertiary center. Variables analyzed included anticipated diagnosis, extent of the mass, need for any surgical airway intervention in the neonatal period, final histopathology data, and survival. Statistical analysis was based on the Fisher and Fisher-Freeman-Halton exact tests (significance set at P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; or = .05) and exact 95% confidence intervals for risk differences. Eight patients underwent termination of pregnancy or were lost to follow-up. The imaging-based prenatal diagnosis was confirmed postnatally in 93% (14/15) of the remaining patients. Final diagnoses included lymphatic malformation (8), teratoma (6), and esophageal duplication (1). Teratomas were associated with a significantly higher risk for neonatal airway intervention than lymphatic malformations (67% vs 11%, P = .02). The majority of such procedures were performed under ex utero intrapartum treatment. Survival was 93% (14/15). Cervical teratomas are significantly more likely to demand surgical airway intervention in the neonate, typically under ex utero intrapartum treatment, than cervical lymphatic malformations. These findings should be considered in the prenatal counseling for fetal cervical masses.
Congenital cystic adenomatoid malformation (CCAM), intralobar sequestration (ILS), extralobar seq... more Congenital cystic adenomatoid malformation (CCAM), intralobar sequestration (ILS), extralobar sequestration (ELS), and lobar emphysema (LE) are well-accepted entities; however, certain findings are common to all, particularly the parenchymal maldevelopment characterizing CCAM. Isolated reports have described bronchial atresia (BA) in some specimens in all 4 entities, but this finding has not been evaluated in a prospective manner. With the aid of a dissecting microscope, we prospectively examined 47 lung specimens resected during the past 4 years and submitted with the clinical impression of ELS (n=11), ILS (n=11), CCAM (n=20), LE (n=4), and airway-esophageal communication (n=1). Most lesions were detected by prenatal ultrasound and were resected during infancy. The clinical impression and pathologic findings were compared. Pathologic examination revealed atresia of a lobar, segmental, or subsegmental bronchus in 100% of ELS, 82% of ILS, 70% of CCAM, and 50% of LE (those clinically recognized to have BA or minor CCAM) cases. Parenchymal maldevelopment that characterizes CCAM was present in 100% of CCAM cases (as expected by definition) as well as in 91% of ELS, 91% of ILS, and 50% of LE (those with BA) cases. Bronchial atresia is present in all ELS, most ILS and CCAM, and some LE cases, and its detection is greatly enhanced with the dissecting microscope. Bronchial atresia and CCAM nearly always coexist. It may be that both have the same etiopathogenesis with anatomic differences accounted for by aberrant genetic programs or other insults, perhaps modified by time of onset or duration.
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Papers by Judy Estroff