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Postmortem magnetic resonance appearances of congenital high airway obstruction syndrome.

Arthurs OJ, Chitty LS, Judge-Kronis L, Sebire NJ - Pediatr Radiol (2014)

Bottom Line: In both cases, postmortem plain radiographs demonstrated gross abdominal distension with distortion and splaying of the rib cage.One foetus had an associated limb abnormality.Postmortem MRI in foetuses suspected of having CHAOS allows confirmation of the diagnosis, determination of the anatomical level of the atresia or stenosis, and identification of associated abnormalities without the need for invasive autopsy.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK, owen.arthurs@gosh.nhs.uk.

ABSTRACT

Background: Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening condition characterised by complete or near-complete developmental obstruction of the foetal airway. Although antenatal imaging findings have been described, the postmortem MRI findings have not been reported.

Objective: To present postmortem MRI features of CHAOS.

Materials and methods: We retrospectively reviewed our hospital pathology and imaging databases for cases of CHAOS over a 2-year period.

Results: We identified two cases of CHAOS. In both cases, postmortem plain radiographs demonstrated gross abdominal distension with distortion and splaying of the rib cage. Both foetuses had characteristic postmortem MRI findings including large-volume fluid-filled lungs on T2-weighted imaging, diaphragmatic eversion, fluid-filled airway dilatation below the level of obstruction, centrally positioned and compressed heart, and massive ascites. One foetus had an associated limb abnormality.

Conclusion: Postmortem MRI in foetuses suspected of having CHAOS allows confirmation of the diagnosis, determination of the anatomical level of the atresia or stenosis, and identification of associated abnormalities without the need for invasive autopsy.

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Related in: MedlinePlus

Case 1. Imaging in a male foetus (same as in Fig. 1) with congenital high airway obstruction syndrome (CHAOS), including antenatal US at 20 weeks’ gestation (a, c) and postmortem coronal (b) and axial (d) high-resolution T2-weighted postmortem MRI following termination at 24 weeks’ gestation. Postmortem MRI shows disproportionately gross ascites (relative to subcutaneous oedema) on coronal (b) and axial (d) abdominal imaging, marked distension of both lungs with eversion of the diaphragm and a centrally placed heart, typical features of CHAOS. The heart and lungs are indicated on axial MR image (d). Coronal MR image (b) demonstrates the fluid-filled airway (white arrow) below the level of the obstruction, as well as the eventration of the diaphragm (black arrow)
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Fig2: Case 1. Imaging in a male foetus (same as in Fig. 1) with congenital high airway obstruction syndrome (CHAOS), including antenatal US at 20 weeks’ gestation (a, c) and postmortem coronal (b) and axial (d) high-resolution T2-weighted postmortem MRI following termination at 24 weeks’ gestation. Postmortem MRI shows disproportionately gross ascites (relative to subcutaneous oedema) on coronal (b) and axial (d) abdominal imaging, marked distension of both lungs with eversion of the diaphragm and a centrally placed heart, typical features of CHAOS. The heart and lungs are indicated on axial MR image (d). Coronal MR image (b) demonstrates the fluid-filled airway (white arrow) below the level of the obstruction, as well as the eventration of the diaphragm (black arrow)

Mentions: Postmortem skeletal radiography showed gross soft-tissue swelling of thorax and abdomen, with splaying of the ribcage (Fig. 1) but no bone abnormality. Postmortem MRI 5 days after death showed dilatation of the trachea below the level of the obstruction, with bilateral lung expansion causing diaphragmatic eversion, corresponding to the prenatal sonographic findings (Fig. 2). The heart was centrally placed but otherwise structurally normal (Fig. 2). The abdominal organs were normal but there was gross ascites with minor septations (Fig. 2). The intracranial appearances were unremarkable. Three-dimensional multiplanar reconstructed images were used to delineate the level of the obstruction (Fig. 3). Histology is presented in (Fig. 4).Fig. 1


Postmortem magnetic resonance appearances of congenital high airway obstruction syndrome.

Arthurs OJ, Chitty LS, Judge-Kronis L, Sebire NJ - Pediatr Radiol (2014)

Case 1. Imaging in a male foetus (same as in Fig. 1) with congenital high airway obstruction syndrome (CHAOS), including antenatal US at 20 weeks’ gestation (a, c) and postmortem coronal (b) and axial (d) high-resolution T2-weighted postmortem MRI following termination at 24 weeks’ gestation. Postmortem MRI shows disproportionately gross ascites (relative to subcutaneous oedema) on coronal (b) and axial (d) abdominal imaging, marked distension of both lungs with eversion of the diaphragm and a centrally placed heart, typical features of CHAOS. The heart and lungs are indicated on axial MR image (d). Coronal MR image (b) demonstrates the fluid-filled airway (white arrow) below the level of the obstruction, as well as the eventration of the diaphragm (black arrow)
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4381089&req=5

Fig2: Case 1. Imaging in a male foetus (same as in Fig. 1) with congenital high airway obstruction syndrome (CHAOS), including antenatal US at 20 weeks’ gestation (a, c) and postmortem coronal (b) and axial (d) high-resolution T2-weighted postmortem MRI following termination at 24 weeks’ gestation. Postmortem MRI shows disproportionately gross ascites (relative to subcutaneous oedema) on coronal (b) and axial (d) abdominal imaging, marked distension of both lungs with eversion of the diaphragm and a centrally placed heart, typical features of CHAOS. The heart and lungs are indicated on axial MR image (d). Coronal MR image (b) demonstrates the fluid-filled airway (white arrow) below the level of the obstruction, as well as the eventration of the diaphragm (black arrow)
Mentions: Postmortem skeletal radiography showed gross soft-tissue swelling of thorax and abdomen, with splaying of the ribcage (Fig. 1) but no bone abnormality. Postmortem MRI 5 days after death showed dilatation of the trachea below the level of the obstruction, with bilateral lung expansion causing diaphragmatic eversion, corresponding to the prenatal sonographic findings (Fig. 2). The heart was centrally placed but otherwise structurally normal (Fig. 2). The abdominal organs were normal but there was gross ascites with minor septations (Fig. 2). The intracranial appearances were unremarkable. Three-dimensional multiplanar reconstructed images were used to delineate the level of the obstruction (Fig. 3). Histology is presented in (Fig. 4).Fig. 1

Bottom Line: In both cases, postmortem plain radiographs demonstrated gross abdominal distension with distortion and splaying of the rib cage.One foetus had an associated limb abnormality.Postmortem MRI in foetuses suspected of having CHAOS allows confirmation of the diagnosis, determination of the anatomical level of the atresia or stenosis, and identification of associated abnormalities without the need for invasive autopsy.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK, owen.arthurs@gosh.nhs.uk.

ABSTRACT

Background: Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening condition characterised by complete or near-complete developmental obstruction of the foetal airway. Although antenatal imaging findings have been described, the postmortem MRI findings have not been reported.

Objective: To present postmortem MRI features of CHAOS.

Materials and methods: We retrospectively reviewed our hospital pathology and imaging databases for cases of CHAOS over a 2-year period.

Results: We identified two cases of CHAOS. In both cases, postmortem plain radiographs demonstrated gross abdominal distension with distortion and splaying of the rib cage. Both foetuses had characteristic postmortem MRI findings including large-volume fluid-filled lungs on T2-weighted imaging, diaphragmatic eversion, fluid-filled airway dilatation below the level of obstruction, centrally positioned and compressed heart, and massive ascites. One foetus had an associated limb abnormality.

Conclusion: Postmortem MRI in foetuses suspected of having CHAOS allows confirmation of the diagnosis, determination of the anatomical level of the atresia or stenosis, and identification of associated abnormalities without the need for invasive autopsy.

Show MeSH
Related in: MedlinePlus