Prenatal tracheal obstruction due to double aortic arch: a potential mimic of congenital high airway obstruction syndrome.
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Biomedical subjects
Publications and source records attributed to Ruth B Goldstein.
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Familial choledochal cysts are extremely rare. High-resolution ultrasound now allows for the antenatal diagnosis of these anomalies. After delivery, elective surgical resection should be planned; however, increases in size, deterioration of liver function, and ascending cholangitis frequently force early intervention. We report an unusual occurrence of siblings with type I choledochal cysts and review the existing literature on cause, genetics, classification, diagnosis, and management of this disease.
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OBJECTIVE: Although fetal magnetic resonance imaging (MRI) is being increasingly used to evaluate sonographically suspected abnormalities, its utility in the evaluation of the spinal canal is not well studied. Because it is not susceptible to the limitations of fetal position, oligohydramnios, and shadowing from bony structures, we hypothesize that fetal MRI is better suited to assess the contents of the spinal canal compared with prenatal sonography. The purpose of this investigation was to determine whether fetal MRI could detect spinal abnormalities in cases in which they had not been originally suspected on prenatal sonography. METHODS: Fetal spine MR images were retrospectively reviewed over a 42-month period. Corresponding sonographic images were then rereviewed to determine whether there were findings in retrospect that might have suggested the cord abnormalities. Cases of myelomeningocele were counted as a spinal cord abnormality only if fetal MRI showed a cord anomaly other than the myelomeningocele. RESULTS: Of 33 cases referred for bony anomalies of the spine, fetal MRI showed additional abnormalities involving the spinal cord in 3 patients. These included diastematomyelia in 2 cases and segmental spinal dysgenesis in the third case. One case of diastematomyelia occurred in association with a lumbosacral myelomeningocele. The spinal cord anomalies were not visible on any of the prenatal sonograms, even in retrospect. CONCLUSIONS: Additional spinal cord anomalies were detected in 10% of cases reviewed. Fetal MRI can be useful in assessing the spinal cord in fetuses with bony spinal anomalies. Our findings suggest that fetuses with sonographically diagnosed bony abnormalities of the spine may benefit from further evaluation with fetal MRI.
OBJECTIVE: Fetal magnetic resonance imaging (MRI) has been shown to be useful in assessing the developing central nervous system. However, its utility in specific brain disorders has not been well investigated. We hypothesized that fetal MRI can better assess the integrity of the brain in cases with sonographically suspected callosal abnormalities. METHODS: We retrospectively reviewed fetal MRI and prenatal sonographic studies of 10 fetuses referred for MRI for sonographically suspected callosal abnormalities. RESULTS: An abnormal corpus callosum was identified on fetal MRI in 80% of cases. The type of callosal abnormality (complete or partial agenesis) was similar on both prenatal sonography and fetal MRI in all cases. All sonographically identified additional brain abnormalities were detected on fetal MRI, with the exception of choroid plexus cysts. Furthermore, in 63% (5 of 8) of cases with a callosal abnormality on both sonography and fetal MRI, additional brain abnormalities were detected on fetal MRI that were not apparent on sonography. These sonographically occult findings were confirmed on postnatal MRI or autopsy in 3 of 5 patients. CONCLUSIONS: Fetal MRI is an important adjunct to sonography in assessing the corpus callosum and other aspects of brain development when agenesis of the corpus callosum is suspected. It can identify frequent additional findings that are not visible on sonography such as abnormal sulcation. In light of the association between additional brain abnormalities and worse neurodevelopmental outcome, the potential of fetal MRI as an important adjunctive prognostic imaging test in fetuses with callosal agenesis can now be tested.
A congenital diaphragmatic hernia (CDH) occurs in approximately 1 per 2000 pregnancies. Most CDHs that are recognized in utero are of the Bochdalek type, resulting from a posterior defect in the diaphragm. Morgagni hernias result from an anterior defect and are the least common type of CDH (5%). They are rarely seen in utero. Furthermore, when they are seen in utero, they may be mischaracterized. We present a case of an unusual type of Morgagni hernia diagnosed in utero. The type of Morgagni hernia reported here is particularly uncommon. Two cases with similar findings have been reported previously. The shared findings between these cases indicate that, although rare, this may be a recognizable type of CDH and one that is distinguishable from the Bochdalek type.
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BACKGROUND AND PURPOSE: The accurate identification of white matter injury in premature neonates is important for counseling parents and for targeting these high risk neonates for appropriate rehabilitation services. The objective of this study was to compare the diagnosis of white matter injury detected by serial MR imaging and ultrasonography of a contemporary cohort of premature neonates. METHODS: Each of the 32 consecutively enrolled neonates was studied with MR imaging at a median postconceptional age of 31.9 weeks (range, 27.6-38.1 weeks) and again at a median postconceptional age of 36.5 weeks (range, 33.4-42.9 weeks) and with serial ultrasonography according to a clinical protocol. Because periventricular echogenicity shown on ultrasonograms evolves over time, both the highest grade of echogenicity and the grade of echogenicity shown on the last neonatal ultrasonogram were used in the analysis to determine the predictive values and correlation (Spearman's rho) of ultrasonography for predicting white matter abnormalities shown on MR images. RESULTS: White matter abnormalities were diagnosed in 18 (56%) neonates based on MR imaging, consisting of foci of scattered T1 hyperintensity in the periventricular white matter, and in 22 (69%) neonates based on ultrasonography, consisting of abnormal periventricular echogenicity. The severity of white matter abnormalities shown by MR imaging was not correlated with the highest grade of white matter abnormalities detected with ultrasonography (rho=0.18, P=.3) or with the grade of white matter abnormalities shown on the last ultrasonogram (rho = 0.16, P=.4). CONCLUSION: Although ultrasonography is commonly used to screen premature neonates for white matter injury, it was not a sensitive predictor of the milder spectrum of MR imaging-defined white matter abnormalities.
Cystic mesenchymal hamartoma is an extremely rare, benign tumor. Rapid growth to a giant size can pose a threat not only in early childhood but also during fetal life. The experience with 2 antenatally diagnosed giant hepatic cysts with widely disparate approaches to management, treatment, and outcome is presented. A giant hepatic cyst was diagnosed on routine screening ultrasound scan. Because of its extremely massive size, the cyst was treated in utero with repeated aspirations, primarily for obstetric considerations. The infant did well, and the lesion was excised laparoscopically during the neonatal period. A second fetus with a giant hepatic cyst was not treated in utero, and the pregnancy continued to term. Nonimmune hydrops fetalis developed, and the fetus was delivered prematurely at 34 weeks. At birth, the infant was noted to have diffuse neurologic injury and no urine output despite normal-appearing kidneys. The lesion was excised during the neonatal period by open laparotomy. Observations at the time of surgery and pathologic studies of the placenta showed aneurysmal dilatation of the placental veins suggesting in utero compression of the fetal intraabdominal umbilical vein. The infant died shortly after birth. The experience with these 2 cases suggests the possibility that giant mesenchymal hamartoma diagnosed in utero may cause umbilical venous obstruction leading to ischemia during fetal life. Decompression of giant hepatic cysts may reverse this phenomenon and allow normal fetal development.
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