Open Fetal Surgery for Central Bronchial Atresia

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 24³/₇ weeks' gestation. The fetus was delivered at 34³/₇ 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.

Imaging of fetal tumors and other dysplastic lesions: A review with emphasis on MR imaging

“…BA may be central or peripheral. If central, these lesions are usually lethal and may be associated with massive ipsilateral pulmonary hyperplasia, contralateral hypoplasia, and the development of hydrops . If peripheral, they may be associated with microcystic changes usually seen in CPAM lesions …”

Right mainstem bronchial atresia successfully corrected with slide tracheobronchoplasty

“…Only ten cases of mainstem bronchial atresia (MBA) have been reported to date, with universally poor outcomes. [2][3][4][5][6] Unlike peripheral bronchial atresia, MBA presents a unique challenge with expansion of the involved lung, mediastinal compression and contralateral shift, fetal pneumonectomy, fetal thoracoamniotic shunt placement, observation, and elective termination. [2][3][4][5][6] Only 3 of 10 reported cases survived to delivery but none survived past day one of life.…”

“…[2][3][4][5][6] Unlike peripheral bronchial atresia, MBA presents a unique challenge with expansion of the involved lung, mediastinal compression and contralateral shift, fetal pneumonectomy, fetal thoracoamniotic shunt placement, observation, and elective termination. [2][3][4][5][6] Only 3 of 10 reported cases survived to delivery but none survived past day one of life. One had undergone fetal thoracoamniotic shunt at 23 weeks GA, and was delivered at 25 weeks GA. 3 Another had a pneumonectomy at 21 weeks GA and was delivered at 24 weeks GA. 2 The third underwent fetal pneumonectomy at 26 weeks GA, and was delivered at 32 weeks GA. 5 Fetal lung growth is dependent on the transpulmonary pressure gradient generated by lung fluid production and absorption, fetal breath movements and the resistance of the glottis.…”

“…[2][3][4][5][6] Only 3 of 10 reported cases survived to delivery but none survived past day one of life. One had undergone fetal thoracoamniotic shunt at 23 weeks GA, and was delivered at 25 weeks GA. 3 Another had a pneumonectomy at 21 weeks GA and was delivered at 24 weeks GA. 2 The third underwent fetal pneumonectomy at 26 weeks GA, and was delivered at 32 weeks GA. 5 Fetal lung growth is dependent on the transpulmonary pressure gradient generated by lung fluid production and absorption, fetal breath movements and the resistance of the glottis. However, an obstruction in the airway will elevate the distal trans-…”

Sequential Amniotic Fluid Thyroid Hormone Changes Correlate with Goiter Shrinkage following in utero Thyroxine Therapy

“…Surgical therapies for conditions such as diaphragmatic hernia and obstructive uropathies represented therapies in ‘life or death' situations. As experience built and we developed an understanding of the risks and benefits in given situations, we could shift to ‘quality of life' concerns such as meningomyelocele [2,3,4,5,6,7,8,9,10,11]. Although not gaining much public attention, medical therapies such as routine folic acid supplementation of bread and grains for neural tube defects and, to a much lesser extent, dexamethasone for congenital adrenal hyperplasia, as well as a few others have successfully prevented anatomic abnormalities or at least altered fetal physiology [12,13].…”