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.

1.
Metkus AP, Filly RA, Stringer MD, Harrison MR, Adzick NS: Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg 1996;31:148-151.
2.
Jani J, Nicolaides KH, Keller RL, Benachi A, Peralta CF, Favre R, Moreno O, Tibboel D, Lipitz S, Eggink A, Vaast P, Allegaert K, Harrison M, Deprest J: Observed to expected lung area to head circumference ratio in the prediction of survival in fetuses with isolated diaphragmatic hernia. Ultrasound Obstet Gynecol 2007;30:67-71.
3.
Crombleholme TM, Coleman B, Hedrick H, Liechty K, Howell L, Flake AW, Johnson M, Adzick NS: Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg 2002;37:331-338.
4.
Adzick NS: Management of fetal lung lesions. Clin Perinatol 2009;36:363-376.
5.
Stocker JT: Sequestrations of the lung. Semin Diagn Pathol 1986;3:106-121.
6.
Duncombe GJ, Dickinson JE, Kikiros CS: Prenatal diagnosis and management of congenital cystic adenomatoid malformation of the lung. Am J Obstet Gynecol 2002;187:950-954.
7.
Wilson RD, Hedrick HL, Liechty KW, Flake AW, Johnson MP, Bebbington M, Adzick NS: Cystic adenomatoid malformation of the lung: review of genetics, prenatal diagnosis, and in utero treatment. Am J Med Genet A 2006;140:151-155.
8.
Bonnefoy C, Blanc P, Coste K, Delabaere A, Dechelotte PJ, Laurichesse-Delmas H, Labbe A, Jacquetin B, Lemery D, Sapin V, Gallot D: Prenatal diagnosis of lobar bronchial atresia. Ultrasound Obstet Gynecol 2011;37:110-112.
9.
Keswani SG, Crombleholme TM, Pawel BR, Johnson MP, Flake AW, Hedrick HL, Howell LJ, Wilson RD, Davis GH, Adzick NS: Prenatal diagnosis and management of mainstem bronchial atresia. Fetal Diagn Ther 2005;20:74-78.
10.
Vidaeff AC, Szmuk P, Mastrobattista JM, Rowe TF, Ghelber O: More or less CHAOS: case report and literature review suggesting the existence of a distinct subtype of congenital high airway obstruction syndrome. Ultrasound Obstet Gynecol 2007;30:114-117.
11.
Cavoretto P, Molina F, Poggi S, Davenport M, Nicolaides KH: Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol 2008;32:769-783.
12.
Wilson RD, Baxter JK, Johnson MP, King M, Kasperski S, Crombleholme TM, Flake AW, Hedrick HL, Howell LJ, Adzick NS: Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations. Fetal Diagn Ther 2004;19:413-420.
13.
Adzick NS: Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med 2010;15:1-8.
14.
Bruner JP, Jarnagin BK, Reinisch L: Percutaneous laser ablation of fetal congenital cystic adenomatoid malformation: too little, too late? Fetal Diagn Ther 2000;15:359-363.
15.
Bermúdez C, Pérez-Wulff J, Arcadipane M, Bufalino G, Gómez L, Flores L, Sosa C, Bornick PW, Kontopoulos E, Quintero RA: Percutaneous fetal sclerotherapy for congenital cystic adenomatoid malformation of the lung. Fetal Diagn Ther 2008;24:237-240.
16.
Lee FL, Said N, Grikscheit TC, Shin CE, Llanes A, Chmait RH: Treatment of congenital pulmonary airway malformation induced hydrops fetalis via percutaneous sclerotherapy. Fetal Diagn Ther 2012;31:264-268.
17.
Stocker J: Congenital pulmonary airway malformation: a new name for and an expanded classification of congenial cystic adenomatous malformation of the lung. Histopathology 2002;41(suppl 2):424-431.
18.
Quintero R, Hale-Burnett E, Bornick PW, Gilbert-Barness E: Fetal laryngoscopy and lung biopsy in a case of bilateral lethal congenital cystic adenomatoid malformation of the lung. Fetal Pediatr Pathol 2007;26:229-234.
19.
Quintero RA, Kontopoulos E, Reiter J, Pedreira WL, Colin AA: Fetal bronchoscopy: its successful use in a case of extralobar pulmonary sequestration. J Matern Fetal Neonatal Med 2012;25:2354-2358.
20.
Martínez JM, Prat J, Gómez O, Crispi F, Bennasar M, Puerto B, Castañón M, Gratacós E: Decompression through tracheobronchial endoscopy of bronchial atresia presenting as massive pulmonary tumor: a new indication for fetoscopic surgery. Fetal Diagn Ther 2013;33:69-74.
21.
Curran PF, Jelin EB, Rand L, Hirose S, Feldstein VA, Goldstein RB, Lee H: Prenatal steroids for microcystic congenital cystic adenomatoid malformations. J Pediatr Surg 2010;45:145-150.
22.
Peranteau WH, Wilson RD, Liechty KW, Johnson MP, Bebbington MW, Hedrick HL, Flake AW, Adzick NS: Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival. Fetal Diagn Ther 2007;22:365-371.
23.
Balci S, Altinok G, Ozaltin F, Aktaş D, Niron EA, Onol B: Laryngeal atresia presenting as fetal ascites, olygohydramnios and lung appearance mimicking cystic adenomatoid malformation in a 25-week-old fetus with Fraser syndrome. Prenat Diagn 1999;19:856-858.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.