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Prenatal MRI findings of fetuses with congenital high airway obstruction sequence.

Guimaraes CV, Linam LE, Kline-Fath BM, Donnelly LF, Calvo-Garcia MA, Rubio EI, Livingston JC, Hopkin RJ, Peach E, Lim FY, Crombleholme TM - Korean J Radiol (2009)

Bottom Line: Associated abnormalities were found in 4 of 7 (genetic syndromes in 2).Two fetuses were terminated and one fetus demised in utero.Four fetuses were delivered via ex utero intrapartum treatment procedure.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA. carolina.guimaraes@cchmc.org

ABSTRACT

Objective: To define the MRI findings of congenital high airway obstruction sequence (CHAOS) in a series of fetuses.

Materials and methods: Prenatal fetal MR images were reviewed in seven fetuses with CHAOS at 21 to 27 weeks of gestation. The MRI findings were reviewed. The MRI parameters evaluated included the appearance of the lungs and diaphragm, presence or absence of hydrops, amount of amniotic fluid, airway appearance, predicted level of airway obstruction, and any additional findings or suspected genetic syndromes.

Results: All the fetuses viewed (7 of 7) demonstrated the following MRI findings: dilated airway below the level of obstruction, increased lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, massive ascites, centrally positioned and compressed heart, as well as placentomegaly. Other frequent findings were anasarca (6 of 7) and polyhydramnios (3 of 7). MRI identified the level of obstruction as laryngeal in five cases and tracheal in two cases. In four of the patients, surgery or autopsy confirmed the MRI predicted level of obstruction. Associated abnormalities were found in 4 of 7 (genetic syndromes in 2). Postnatal radiography (n = 3) showed markedly hyperinflated lungs with inverted or flattened hemidiaphragms, strandy perihilar opacities, pneumothoraces and tracheotomy. Two fetuses were terminated and one fetus demised in utero. Four fetuses were delivered via ex utero intrapartum treatment procedure.

Conclusion: MRI shows a consistent pattern of abnormalities in fetuses with CHAOS, accurately identifies the level of airway obstruction, and helps differentiate from other lung abnormalities such as bilateral congenital pulmonary airway malformation by demonstrating an abnormally dilated airway distal to the obstruction.

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Congenital high airway obstruction sequence with decreased imaging findings on follow up fetal MR imaging.A. Sagittal T2-weighted single shot fast spin echo image obtained at 23-weeks of gestation shows markedly enlarged lung volumes (L) and increased lung signal, marked ascites (A), and inverted diaphragms (arrows). Note gap seen in fluid-filled, high-signal airway (arrowheads) at level of larynx, consistent with level of obstruction.B. Sagittal T2-weighted single shot fast spin echo image obtained at 33-weeks of gestation shows marked interval reduction in degree of ascites (A), marked decrease in lung volume with resolution of inverted diaphragm (arrows). Gap seen in fluid-filled, high-signal airway at level of larynx is still seen. However, airway distal to level of obstruction (arrowhead) remains dilated.
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Figure 3: Congenital high airway obstruction sequence with decreased imaging findings on follow up fetal MR imaging.A. Sagittal T2-weighted single shot fast spin echo image obtained at 23-weeks of gestation shows markedly enlarged lung volumes (L) and increased lung signal, marked ascites (A), and inverted diaphragms (arrows). Note gap seen in fluid-filled, high-signal airway (arrowheads) at level of larynx, consistent with level of obstruction.B. Sagittal T2-weighted single shot fast spin echo image obtained at 33-weeks of gestation shows marked interval reduction in degree of ascites (A), marked decrease in lung volume with resolution of inverted diaphragm (arrows). Gap seen in fluid-filled, high-signal airway at level of larynx is still seen. However, airway distal to level of obstruction (arrowhead) remains dilated.

Mentions: All fetuses (7 of 7) demonstrated MRI findings of a dilated airway below the level of obstruction, and included an increase in the lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, centrally positioned and compressed heart, ascites, and placentomegaly (Figs. 1-3). The trachea and distal airway were dilated in six of the seven fetuses, whereas airway dilatation was isolated to the bronchi in the remaining fetus of the group (1 of 7).


Prenatal MRI findings of fetuses with congenital high airway obstruction sequence.

Guimaraes CV, Linam LE, Kline-Fath BM, Donnelly LF, Calvo-Garcia MA, Rubio EI, Livingston JC, Hopkin RJ, Peach E, Lim FY, Crombleholme TM - Korean J Radiol (2009)

Congenital high airway obstruction sequence with decreased imaging findings on follow up fetal MR imaging.A. Sagittal T2-weighted single shot fast spin echo image obtained at 23-weeks of gestation shows markedly enlarged lung volumes (L) and increased lung signal, marked ascites (A), and inverted diaphragms (arrows). Note gap seen in fluid-filled, high-signal airway (arrowheads) at level of larynx, consistent with level of obstruction.B. Sagittal T2-weighted single shot fast spin echo image obtained at 33-weeks of gestation shows marked interval reduction in degree of ascites (A), marked decrease in lung volume with resolution of inverted diaphragm (arrows). Gap seen in fluid-filled, high-signal airway at level of larynx is still seen. However, airway distal to level of obstruction (arrowhead) remains dilated.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2651442&req=5

Figure 3: Congenital high airway obstruction sequence with decreased imaging findings on follow up fetal MR imaging.A. Sagittal T2-weighted single shot fast spin echo image obtained at 23-weeks of gestation shows markedly enlarged lung volumes (L) and increased lung signal, marked ascites (A), and inverted diaphragms (arrows). Note gap seen in fluid-filled, high-signal airway (arrowheads) at level of larynx, consistent with level of obstruction.B. Sagittal T2-weighted single shot fast spin echo image obtained at 33-weeks of gestation shows marked interval reduction in degree of ascites (A), marked decrease in lung volume with resolution of inverted diaphragm (arrows). Gap seen in fluid-filled, high-signal airway at level of larynx is still seen. However, airway distal to level of obstruction (arrowhead) remains dilated.
Mentions: All fetuses (7 of 7) demonstrated MRI findings of a dilated airway below the level of obstruction, and included an increase in the lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, centrally positioned and compressed heart, ascites, and placentomegaly (Figs. 1-3). The trachea and distal airway were dilated in six of the seven fetuses, whereas airway dilatation was isolated to the bronchi in the remaining fetus of the group (1 of 7).

Bottom Line: Associated abnormalities were found in 4 of 7 (genetic syndromes in 2).Two fetuses were terminated and one fetus demised in utero.Four fetuses were delivered via ex utero intrapartum treatment procedure.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA. carolina.guimaraes@cchmc.org

ABSTRACT

Objective: To define the MRI findings of congenital high airway obstruction sequence (CHAOS) in a series of fetuses.

Materials and methods: Prenatal fetal MR images were reviewed in seven fetuses with CHAOS at 21 to 27 weeks of gestation. The MRI findings were reviewed. The MRI parameters evaluated included the appearance of the lungs and diaphragm, presence or absence of hydrops, amount of amniotic fluid, airway appearance, predicted level of airway obstruction, and any additional findings or suspected genetic syndromes.

Results: All the fetuses viewed (7 of 7) demonstrated the following MRI findings: dilated airway below the level of obstruction, increased lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, massive ascites, centrally positioned and compressed heart, as well as placentomegaly. Other frequent findings were anasarca (6 of 7) and polyhydramnios (3 of 7). MRI identified the level of obstruction as laryngeal in five cases and tracheal in two cases. In four of the patients, surgery or autopsy confirmed the MRI predicted level of obstruction. Associated abnormalities were found in 4 of 7 (genetic syndromes in 2). Postnatal radiography (n = 3) showed markedly hyperinflated lungs with inverted or flattened hemidiaphragms, strandy perihilar opacities, pneumothoraces and tracheotomy. Two fetuses were terminated and one fetus demised in utero. Four fetuses were delivered via ex utero intrapartum treatment procedure.

Conclusion: MRI shows a consistent pattern of abnormalities in fetuses with CHAOS, accurately identifies the level of airway obstruction, and helps differentiate from other lung abnormalities such as bilateral congenital pulmonary airway malformation by demonstrating an abnormally dilated airway distal to the obstruction.

Show MeSH
Related in: MedlinePlus