Fetal Bronchoscopy as a Useful Procedure in a Case with Prenatal Diagnosis of Congenital Microcystic Adenomatoid Malformation

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.

Fetal Endoscopic Tracheal Intubation: A New Fetoscopic Procedure to Ensure Extrauterine Tracheal Permeability in a Case with Congenital Cervical Teratoma

“…However, it has been demonstrated that in experienced hands, fetal tracheoscopy for the treatment of fetuses with congenital diaphragmatic hernia can be achieved in a median surgical time of 10 min [15]. Similarly, we and others have demonstrated the feasibility of fetal airway endoscopy for cases with congenital high airway obstruction syndrome [16,17], bronchial atresia [18] and congenital microcystic adenomatoid malformation [19,20]. In this regard, it would be expected that fetal tracheoscopy in cases with neck masses may be more challenging due to severe distortion of the glottis, laryngeal and tracheal anatomy.…”

Prenatal Diagnosis and Evaluation of Sonographic Predictors for Intervention and Adverse Outcome in Congenital Pulmonary Airway Malformation

“…In cases of non-hydropic fetuses with CPAM the prognosis is excellent with high survival rates and low postnatal morbidity, whereas fetuses with hydrops managed expectantly usually die either before or after delivery [ 2 , 3 , 35 ]. Therefore numerous attempts of fetal therapy have been made such as thoraco-amniotic shunting or thoracocentesis [ 5 , 22 , 36 ], prenatal open surgery with hysterotomy and lobectomy [ 3 , 6 , 37 ] and ultrasound-guided laser ablation [ 22 , 38 , 39 ], recently described by fetal bronchoscopy[ 40 ], or percutaneous radiofrequency [ 2 ]. Finally the treatment of fetuses with maternal steriod application is described in small retrospective trials and seems to be effective in microcystic lesions in presence of hydrops, where survival rates with expectant management are described as very low [ 41 , 42 , 43 , 44 ].…”

The Use of Fetal Bronchoscopy in the Diagnosis and Management of a Suspected Obstructive Lung Mass

“…In summary, the collective evidence from previously published fetal tracheobronchoscopy cases 6 9 24 25 and ours adds to the current understanding of prenatal diagnosis and management of congenital lung lesions. Under the proper circumstances, fetal tracheobronchoscopy can serve as a diagnostic tool, as well as a possible therapeutic measure if an obstructive lesion is identified.…”

“…If an obstruction, such as bronchial atresia is encountered in utero, laser energy can be used to perforate the obstruction and re-establish luminal patency. 6 24 This not only allows the stored pulmonary mucus secretions to be expelled, but also normalization of the fetal lung tissue, correction of the mediastinal shift, and resolution of the hydrops fetalis. 6 24 …”