Journal Mobile Options
Table of Contents
Vol. 23, No. 4, 2008
Issue release date: May 2008
Fetal Diagn Ther 2008;23:250–253
(DOI:10.1159/000123609)

Does Congenital Diaphragmatic Hernia Associated with Bronchopulmonary Sequestration Portend a Better Prognosis?

Grethel E.J. · Farrell J. · Ball R.H. · Keller R.L. · Goldstein R.B. · Lee H. · Farmer D.L. · Harrison M.R. · Nobuhara K.K.
To view the fulltext, log in and/or choose pay-per-view option

Individual Users: Register with Karger Login Information

Please create your User ID & Password





Contact Information











I have read the Karger Terms and Conditions and agree.

To view the fulltext, please log in

To view the pdf, please log in

Abstract

Introduction: Congenital diaphragmatic hernia (CDH) continues to be a devastating disease in the newborn population, with well-documented morbidity and mortality. Bronchopulmonary sequestration is a separate congenital defect that has been associated with CDH. While the association of sequestration with CDH has been reported to be as high as 30–40%, the prognosis associated with the two simultaneous defects is unknown. We reviewed our experience to evaluate if prognosis was better in the CDH infants with associated bronchopulmonary sequestration. Methods: Institutional approval was obtained. Our institutional database was examined from August 1995 to August 2005, identifying all mothers carrying fetuses with pulmonary masses and/or CDH and all neonates treated with bronchopulmonary sequestration and/or CDH. Patients who had both CDH and sequestration were identified by prenatal ultrasound reports, postnatal radiographs, and operative and pathology reports. Results: 16 patients were identified in the fetal or neonatal period with concomitant diagnoses of CDH and bronchopulmonary sequestration. Of those proceeding to delivery, 6 expired and 6 survived. The presence of liver herniation and low lung-to-head ratio on antenatal ultrasound correlated with mortality. However, 2 patients survived with very low lung-to-head ratio that would usually be associated with 100% mortality at our institution. Two diagnoses of bronchopulmonary sequestration were reversed after final pathology revealed liver tissue. Conclusion: Given the limited series, we cannot conclude that bronchopulmonary sequestration confers an anatomic advantage to patients that have CDH. We did observe survivors in this group that, given their antenatal predictors of CDH severity, would ordinarily have dismal prognosis. The presence of a sequestration may be protective in a subset of patients with severe CDH, or may confound our antenatal predictors of disease severity in these patients.



Copyright / Drug Dosage

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 or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
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 goverment 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.

References

  1. Langham MR Jr, Kays DW, Ledbetter DJ, Frentzen B, Sanford LL, Richards DS: Congenital diaphragmatic hernia: epidemiology and outcome. Clin Perinatol 1996;23:671–688.
  2. Savic B, Birtel FJ, Tholen W, Knoche R: Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979;34:96–101.
  3. Corbett HJ, Humphrey GM: Pulmonary sequestration. Paediatr Respir Rev 2004;5:59–68.
  4. Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ: Fetal lung lesions: management and outcome. Am J Obstet Gynecol 1998;179:884–889.
  5. Harris K: Extralobar sequestration with congenital diaphragmatic hernia: a complicated case study. Neonatal Network 2004;23:7–24.
  6. Luet’ic T, Crombleholme TM, Semple JP, D’Alton M: Early prenatal diagnosis of bronchopulmonary sequestration with associated diaphragmatic hernia. J Ultrasound Med 1995;14:533–535.

    External Resources

  7. Hamrick SEG, Brook MM, Farmer DL: Fetal surgery for congenital diaphragmatic hernia and pulmonary sequestration complicated by postnatal diagnosis of transposition of the great arteries. Fetal Diagn Ther 2004;19:40–42.
  8. Urushihara N, Nakagawa Y, Kawashima K, Fukazawa T, Watanabe Y, Todani T: Congenital right diaphragmatic hernias through posterolateral and anterolateral defects associated with extralobar pulmonary sequestration: a case report. J Pediatr Surg 1997;32:101–102.
  9. Ankola PA, Morkos A: Pathology teach and tell: neonatal ELS. Pediatr Pathol Mol Med 2003;22:175–179.
  10. DeParedes CG, Pierce WS, Johnson DG, Waldhausen JA: Pulmonary sequestration in infants and children: a 20-year experience and review of the literature. J Pediatr Surg 1970;5:136–147.
  11. Aideyan U: Pulmonary Development Anomalies: Pulmonary Sequestration. Virtual Hos- pital. Retreived August 26, 2005 from http://www.vh.org/pediatric/provider/radiology/PedsChest/04PulmonarySequestration.html.
  12. Jani J, Keller RL, Benachi A, Nicolaides KH, Favre R, Gratacos E, Laudy J, Eisenberg V, Eggink A, Vaast P, Deprest J: Prenatal prediction of survival in isolated left-sided diaphragmatic hernia. Ultrasound Obstet Gynecol 2006;27:18–22.
  13. Albanese CT, Lopoo J, Goldstein RB, Filly RA, Feldstein VA, Calen PW, et al: Fetal liver position and perinatal outcome for congenital diaphragmatic hernia. Prenat Diagn 1998;18:1138–1142.
  14. Townsend TC, Cortes RA, Farmer DL, et al: Predictors of survival for congenital diaphragmatic hernia: comparison of lung-head ratio (LHR) to the congenital diaphragmatic hernia study group equation. Presented at the American Academy of Pediatrics National Conference, San Francisco, Calif., October 9–13, 2004.
  15. 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.
  16. Lipshutz GS, Albanese CT, Feldstein VA, Jennings RW, Housley HT, Beech R, et al: Prospective analysis of lung-to-head ratio predicts survival for patients with prenatally diagnosed congenital diaphragmatic hernia. J Pediatr Surg 1997;32:1624–1626.


Pay-per-View Options
Direct payment This item at the regular price: USD 33.00
Payment from account With a Karger Pay-per-View account (down payment USD 150) you profit from a special rate for this and other single items.
This item at the discounted price: USD 23.00