Korean J Radiol.  2009 Apr;10(2):129-134. 10.3348/kjr.2009.10.2.129.

Prenatal MRI Findings of Fetuses with Congenital High Airway Obstruction Sequence

• Guimaraes
• Linam LE
• Kline-Fath BM
• Donnelly L
• Calvo-Garcia MA
• Rubio EI
• Livingston JC
• Hopkin RJ
• Peach E
• Lim FY
• Crombleholme TM

• KMID: 1088723
• DOI: http://doi.org/10.3348/kjr.2009.10.2.129

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.

Keyword

Fetal MRI; Congenital high airway obstruction sequence

MeSH Terms

Abnormalities, Multiple
Airway Obstruction/congenital/*pathology
Ascites/pathology
Diaphragm/abnormalities
Female
Fetal Diseases/*pathology
Humans
Lung/pathology
*Magnetic Resonance Imaging
Placenta Diseases/pathology
Pregnancy
*Prenatal Diagnosis
Retrospective Studies

Figure

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  Fig. 1 27-week-old fetus with congenital high airway obstruction sequence.A. Coronal T2-weighted single shot fast spin echo image shows markedly enlarged lung volumes (L) and increase in lung signal, marked ascites (A), and dilated bronchi bilaterally (arrows).B. Sagittal T2-weighted single shot fast spin echo image once again shows inverted diaphragm (arrowheads), ascites (A), dilated bronchus (arrow) and subcutaneous edema (black arrows).C. Axial T2-weighted single shot fast spin echo image shows centrally positioned and compressed heart (H), dilated bronchi (arrows), large lung volumes (L), and subcutaneous edema (black arrow).

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  Fig. 2 22-week-old fetus with congenital high airway obstruction sequence.A. Axial T2-weighted single shot fast spin echo image shows marked placentomegaly (P). Note that fetus has centrally positioned and compressed heart (H), and large lung volumes (L).B. Coronal T2-weighted single shot fast spin echo image shows markedly enlarged lung volumes (L) and increase in lung signal, marked ascites (A), and dilated airway (arrow). Note inverted diaphragms.

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  Fig. 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.

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  Fig. 4 Radiograph of chest and abdomen in 1-day-old child with congenital high airway obstruction sequence, delivered by ex utero intrapartum treatment to tracheostomy. Note markedly hyperinflated lungs with flattened hemidiaphragms, strandy perihilar opacities, pneumothoraces, tracheotomy, and enlarged abdomen, which is likely due to ascites. Child expired at 2-months of age.

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