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Fetal Magnetic Resonance Imaging of Malformations Associated with Heterotaxy.

Loomba R, Shah PH, Anderson RH - Cureus (2015)

Bottom Line: One such syndrome is so-called "visceral heterotaxy", in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs.Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations.In this review, we discuss how these findings can be demonstrated using fetal MRI.

View Article: PubMed Central - HTML - PubMed

Affiliation: Cardiology Dept., Children's Hospital of Wisconsin.

ABSTRACT
Magnetic resonance imaging (MRI) is increasingly used as an investigation during fetal life, particularly for assessment of intracranial masses, congenital diaphragmatic hernia, myelomeningocele, and abdominal masses. As the number of scans increases, so is the variety of congenital malformations being recognized. It is axiomatic that interpretation of the findings is enhanced when attention is paid to the likely findings in the setting of known syndromes, this information then dictating the need for additional acquisition of images. One such syndrome is so-called "visceral heterotaxy", in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs. Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations. In this review, we discuss how these findings can be demonstrated using fetal MRI.

No MeSH data available.


Related in: MedlinePlus

Horseshoe kidneyT2-weighted HASTE imaging of a 33-week gestational age fetus. Panel A consists of a sagittal image demonstrating a horseshoe kidney (arrow). Panel B is a sagittal slice also demonstrating a horseshoe kidney and a pouch in the upper esophagus (arrow) consistent with a diagnosis of tracheoesophageal fistula. Image reprinted without change from Martin, et al. under the creative commons license.
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FIG16: Horseshoe kidneyT2-weighted HASTE imaging of a 33-week gestational age fetus. Panel A consists of a sagittal image demonstrating a horseshoe kidney (arrow). Panel B is a sagittal slice also demonstrating a horseshoe kidney and a pouch in the upper esophagus (arrow) consistent with a diagnosis of tracheoesophageal fistula. Image reprinted without change from Martin, et al. under the creative commons license.

Mentions: Apart from abnormal lateralization of the major organs, other gastrointestinal malformations are to be anticipated. While not purely gastrointestinal malformations, tracheoesophageal fistulas (Figure 16) and congenital diaphragmatic hernias (Figure 17) can be associated with heterotaxy [14, 37, 46-48]. Perhaps the most frequent gastrointestinal manifestation, nonetheless, is malrotation [49]. Routine screening for malrotation in the setting of heterotaxy is currently under debate, since screening studies in asymptomatic infants likely offer no benefit [50]. Prophylactic Ladd’s procedures carried out in asymptomatic patients may be harmful, particularly in patients who have had cardiac palliation with a shunt, since the procedure increases the risk of shunt thrombosis [51]. Omphalocele (Figure 18) has also been noted in those with heterotaxy and is always associated with a degree of malrotation [14, 48, 52-53]. Biliary atresia, duodenal atresia, agenesis of the dorsal pancreas, and anal atresia are also found [54-58]. Heterotaxy, furthermore, is known to carry a higher risk of portosystemic shunts, known as Abernethy malformations [59-61].


Fetal Magnetic Resonance Imaging of Malformations Associated with Heterotaxy.

Loomba R, Shah PH, Anderson RH - Cureus (2015)

Horseshoe kidneyT2-weighted HASTE imaging of a 33-week gestational age fetus. Panel A consists of a sagittal image demonstrating a horseshoe kidney (arrow). Panel B is a sagittal slice also demonstrating a horseshoe kidney and a pouch in the upper esophagus (arrow) consistent with a diagnosis of tracheoesophageal fistula. Image reprinted without change from Martin, et al. under the creative commons license.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4494530&req=5

FIG16: Horseshoe kidneyT2-weighted HASTE imaging of a 33-week gestational age fetus. Panel A consists of a sagittal image demonstrating a horseshoe kidney (arrow). Panel B is a sagittal slice also demonstrating a horseshoe kidney and a pouch in the upper esophagus (arrow) consistent with a diagnosis of tracheoesophageal fistula. Image reprinted without change from Martin, et al. under the creative commons license.
Mentions: Apart from abnormal lateralization of the major organs, other gastrointestinal malformations are to be anticipated. While not purely gastrointestinal malformations, tracheoesophageal fistulas (Figure 16) and congenital diaphragmatic hernias (Figure 17) can be associated with heterotaxy [14, 37, 46-48]. Perhaps the most frequent gastrointestinal manifestation, nonetheless, is malrotation [49]. Routine screening for malrotation in the setting of heterotaxy is currently under debate, since screening studies in asymptomatic infants likely offer no benefit [50]. Prophylactic Ladd’s procedures carried out in asymptomatic patients may be harmful, particularly in patients who have had cardiac palliation with a shunt, since the procedure increases the risk of shunt thrombosis [51]. Omphalocele (Figure 18) has also been noted in those with heterotaxy and is always associated with a degree of malrotation [14, 48, 52-53]. Biliary atresia, duodenal atresia, agenesis of the dorsal pancreas, and anal atresia are also found [54-58]. Heterotaxy, furthermore, is known to carry a higher risk of portosystemic shunts, known as Abernethy malformations [59-61].

Bottom Line: One such syndrome is so-called "visceral heterotaxy", in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs.Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations.In this review, we discuss how these findings can be demonstrated using fetal MRI.

View Article: PubMed Central - HTML - PubMed

Affiliation: Cardiology Dept., Children's Hospital of Wisconsin.

ABSTRACT
Magnetic resonance imaging (MRI) is increasingly used as an investigation during fetal life, particularly for assessment of intracranial masses, congenital diaphragmatic hernia, myelomeningocele, and abdominal masses. As the number of scans increases, so is the variety of congenital malformations being recognized. It is axiomatic that interpretation of the findings is enhanced when attention is paid to the likely findings in the setting of known syndromes, this information then dictating the need for additional acquisition of images. One such syndrome is so-called "visceral heterotaxy", in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs. Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations. In this review, we discuss how these findings can be demonstrated using fetal MRI.

No MeSH data available.


Related in: MedlinePlus