Fetal laryngeal stenosis/atresia and congenital high airway obstructive syndrome (CHAOS): a case report.
Congenital high airway obstructive syndrome (CHAOS) is a rare but fatal disease with predictably characteristic features including stenotic or atretic upper airway, hyperplastic lungs, elevated diaphragm, massive fetal ascites and fetal hydrops. Diagnosis of CHAOS by ultrasound scan is possible and clinically important since advanced intrauterine surgery to correct the defect is possible. We report a case of fetus of CHAOS with massive ascites, pulmonary hyperplasia and laryngeal stenosis/atresia. We feel that it is important to recognize the entity both by ultrasound scan and by the pathologist so that some cases can be corrected by intrauterine fetal surgery.
Zhang
Peilin
P
Department of Pathology, Obstetrics and Gynecology, West Virginia University, Morgantown, USA.
Herring
Dan
D
Cook
Linda
L
Mertz
Heather
H
D000293:Adolescent; D000402:Airway Obstruction / Q000151:congenital* / Q000196:embryology; D005260:Female; D006801:Humans; D007829:Laryngostenosis / Q000151:congenital* / Q000196:embryology; D011247:Pregnancy; D013577:Syndrome