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.
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 two cases with a severely enlarged left lung without ultrasound signs of dilated airways compatible with diagnosis of microcystic CCAM, hydrops and severe contralateral lung hypoplasia that was treated successfully at 29 and 30 weeks of gestation, respectively 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. Both neonates were delivered uneventfully at term with no need for respiratory support, and both are now doing well at 3 and 15 months of age. This report demonstrates that in cases with prenatal diagnosis of large microcystic CCAM, fetal bronchoscopy can achieve diagnosis of bronchial atresia with good outcome.Supporting information can be found in the online version of this abstract Hypoplastic left heart syndrome (HLHS) with restrictive or closed foramen ovale (Fo) is a fatal condition. In our experience the mortality among such neonates was 100%. In other series it was about 90% if the immediate surgery was performed after delivery, but the further treatment had also very high risk due to the severe damage of the pulmonary circulation. Knowing this, in an era of fetal interventions, fetal balloon atrioseptostomy or interatrial stent placement should be considered. Between June 2011 and April 2014 we performed 4 balloon atrioseptostomies and 6 stent placements in 9 fetuses. In one fetus the stent was placed after ineffective atrioseptostomy. All procedures were performed in anesthetized fetuses and mothers. There were 6 cases of HLHS and 3 cases of secondary closure of the Fo in fetuses with severe aortic stenosis and mitral insufficiency. All procedures were performed after 24 weeks.They were technically successful and improved pulmonary venous flow. There were 2 intrauterine deaths related to the procedure and 2 premature deliveries (not related to the procedure) with neonatal death. One fetus is still in utero. 4 neonates were born at term in good general condition but deteriorated quickly due to increased pulmonary flow after delivery. 3 died in spite of intensive treatment few weeks after delivery. All of them were immediately operated on. One was operated on in the 5th hour of life and his condition is stable during the first week. Atrioseptostomy and stent placement can be successfully performed in fetuses. They are potentially lifesaving ...
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