Prenatal diagnosis of lobar bronchial atresia

Maternal Fetal Medicine Unit, CHU Clermont-Ferrand, CHU Estaing, Clermont-Ferrand Cedex 1, France.
Ultrasound in Obstetrics and Gynecology (Impact Factor: 3.14). 01/2011; 37(1):110-2. DOI: 10.1002/uog.8838
Source: PubMed

ABSTRACT We report three cases of fetal lobar bronchial atresia referred to our Fetal Medicine Center during the mid-trimester of pregnancy over the last 15 years. Lobar bronchial atresia can mimic a main stem bronchial atresia on mid-trimester ultrasound examination as it induces extensive lobar enlargement, major mediastinal shift and eversion of the diaphragm. It was associated with severe pulmonary hypoplasia in all three cases, even though polyhydramnios and ascites were absent in two. Termination of pregnancy was performed at parental request after extensive counseling in each of the cases and necropsy confirmed one or two enlarged lung lobes leading to major compression of the remaining lobe(s) of the ipsilateral lung, the contralateral lung and the heart. No other anomalies were observed and the karyotype was normal in all cases.


Available from: Pierre Blanc, Oct 20, 2014
  • [Show abstract] [Hide abstract]
    ABSTRACT: This is the first report of fetoscopy to successfully treat a case of bilobar bronchial atresia presenting in the form of a massive right lung tumor causing severe thoracic compression with mediastinal shift, pulmonary hypoplasia and eventually fetal hydrops. The presence of an echolucent tubular structure in the pulmonary hilum was identified as the intrapulmonary continuation of the interrupted main bronchus, and led to the diagnosis of main bronchial atresia. After extensive discussion and counseling, a fetoscopic operation was designed and indicated. The procedure consisted of a fetal tracheoscopy and access to the right main bronchus, which was perforated with a diode laser until a communication with the dead end of the intrapulmonary main bronchus was achieved. After the operation a significant reduction in size was observed, hydrops and mediastinal shift disappeared and O/E LHR increased to 85%. The fetus was born at term and lobectomy of two dysplastic pulmonary lobes was performed. The infant is developing well at 14 months of age. This report demonstrates that fetoscopic airway decompression can achieve fetal survival in the rare event of main bronchus atresia.
    Fetal Diagnosis and Therapy 07/2012; 33(1). DOI:10.1159/000339681 · 2.30 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Mainstem or lobar bronchial atresia is associated with massive pulmonary hyperplasia, contralateral pulmonary hypoplasia, non-immune hydrops and a fatal fetal prognosis. Open fetal surgery currently provides a potential therapeutic option for management of a fetus with this diagnosis. We present 2 cases of open fetal surgery for bronchial atresia at the level of the mainstem and lobar bronchus. The first case involved a left pneumonectomy performed at 26 weeks' gestation. After successful intraoperative resuscitation for fetal cardiac arrest, the fetus survived until 32 weeks' gestation when preterm premature rupture of membranes prompted delivery. The neonate expired on day of life (DOL)1. The second case involved a left upper lobectomy performed at 24(3)/7 weeks' gestation. The fetus was delivered at 34(3)/7 weeks' gestation and, after a prolonged NICU course including a completion pneumonectomy and tracheostomy, was discharged home at 8 months of life. This second case is the first report of survival following open fetal surgery for central bronchial atresia. This report highlights the potential benefit of fetal lung resection for central bronchial atresia while describing the many challenges to achieve the successful management of a fetus with this condition. © 2014 S. Karger AG, Basel.
    Fetal Diagnosis and Therapy 01/2014; 35(2). DOI:10.1159/000357497 · 2.30 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Massive microcystic congenital cystic adenomatoid malformation (CCAM) and bronchial atresia are associated with a high perinatal mortality secondary to lung hypoplasia and cardiac dysfunction, and fetal intervention should be considered to improve prognosis. Therapeutic options include open fetal surgery with pulmonary resection, fetal sclerotherapy and fetoscopy. We present a case with a severely enlarged left lung without ultrasound signs of dilated airways compatible with the diagnosis of microcystic CCAM, hydrops and severe contralateral lung hypoplasia that was treated successfully at 30 weeks of gestation by fetal bronchoscopy, through which bronchial atresia was identified at the end of the left mainstem bronchi and permeabilized by laser ablation. After fetal surgery, weekly follow-up showed a progressive decrease in the affected lung size and an increase in the contralateral hypoplastic lung size, demonstrating normal dimensions of both lungs at 34 weeks of gestation, reversal of the mediastinal shift, and complete disappearance of hydrops. A healthy neonate was delivered uneventfully at term with no need for respiratory support, and the boy is now doing well at 15 months of age. This report demonstrates that in cases with prenatal diagnosis of large microcystic CCAM, fetal bronchoscopy can be used to refine the diagnosis of bronchial atresia and as a therapeutic tool with good outcome. © 2014 S. Karger AG, Basel.
    Fetal Diagnosis and Therapy 08/2014; 37(1). DOI:10.1159/000361015 · 2.30 Impact Factor