Mainstem bronchial atresia: a lethal anomaly amenable to fetal surgical treatment.
OBJECTIVE
The purpose of this study was to review the unique imaging characteristics, prenatal course, and outcomes for fetuses with mainstem bronchial atresia (MBA).


METHODS
The records of all patients referred for a fetal lung malformation from 2001 to 2012 and the medical literature were reviewed to identify cases of MBA.


RESULTS
Of 129 fetuses evaluated, 3 were diagnosed prenatally with right-sided MBA. The first had a CCAM-volume ratio (CVR) of 9, hydrops, mirror syndrome, and preterm delivery of a nonviable fetus. The second (CVR 2.6) had ascites, preterm delivery at 34-weeks, and neonatal demise. The third fetus (CVR 5.7) presented with hydrops at 21-weeks, prompting fetal pneumonectomy. Postoperatively, hydrops resolved, and the contralateral lung grew dramatically, but preterm delivery occurred 3 weeks later. Ventilation could not be sustained, and the infant died. Four similar cases of MBA were in the literature, all right-sided. Two fetuses with hydrops delivered at 25-weeks and died immediately. One pregnancy was terminated. One fetus underwent pneumonectomy at 24-weeks but died intraoperatively.


CONCLUSIONS
MBA is a rare and lethal lesion that must be distinguished from other right-sided lung masses. Fetal pneumonectomy can be performed with resolution of hydrops and compensatory contralateral lung growth, but remains limited by complications of preterm birth.
Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX.
Irving J
IJ
Zamora
Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX.
Fariha
F
Sheikh
Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX; Department Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX.
Oluyinka O
OO
Olutoye
Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Department of Radiology, Baylor College of Medicine, Houston, TX.
Christopher I
CI
Cassady
Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX.
Timothy C
TC
Lee
Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Department Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX.
Rodrigo
R
Ruano
Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX; Department Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX. Electronic address: dcass@bcm.edu.
Darrell L
DL
Cass
D000293:Adolescent; D000328:Adult; D001980:Bronchi / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D005333:Fetus / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D015160:Hydrops Fetalis / Q000209:etiology / Q000601:surgery; D007231:Infant, Newborn; D007234:Infant, Premature; D008168:Lung / Q000196:embryology; D008279:Magnetic Resonance Imaging; D008297:Male; D011013:Pneumonectomy; D011247:Pregnancy; D011296:Prenatal Diagnosis
CCAM; Congenital lung malformation; Fetal lung mass; Fetal surgery; Mainstem bronchial atresia