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Vol. 23, No. 4, 2008
Issue release date: May 2008
Section title: Paper
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.
Departments of aPediatric Surgery, bObstetrics and Gynecology, cPediatrics, and dRadiology, University of California, San Francisco, Calif., USA

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Article / Publication Details

First-Page Preview
Abstract of Paper

Received: 8/30/2006
Accepted: 11/14/2006
Published online: 4/14/2008

Number of Print Pages: 4
Number of Figures: 1
Number of Tables: 1

ISSN: 1015-3837 (Print)
eISSN: 1421-9964 (Online)

For additional information: http://www.karger.com/FDT

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.


  

Author Contacts

Erich Grethel
Fetal Treatment Center, University of California, San Francisco
513 Parnassus Ave, HSW-1601, Box 0570
San Francisco, CA 94143-0570 (USA)
Tel. +1 415 476 0555, Fax +1 415 476 2314, E-Mail grethele@surgery.ucsf.edu

  

Article Information

Received: August 30, 2006
Accepted after revision: November 14, 2006
Published online: April 14, 2008
Number of Print Pages : 4
Number of Figures : 1, Number of Tables : 1, Number of References : 16

  

Publication Details

Fetal Diagnosis and Therapy (Clinical Advances and Basic Research)

Vol. 23, No. 4, Year 2008 (Cover Date: May 2008)

Journal Editor: Holzgreve W. (Basel), Evans M.I. (New York, N.Y.), Uzan S. (Paris), Michejda M. (Washington, D.C.), Pringle K.C. (Wellington)
ISSN: 1015–3837 (Print), eISSN: 1421–9964 (Online)

For additional information: http://www.karger.com/FDT


Article / Publication Details

First-Page Preview
Abstract of Paper

Received: 8/30/2006
Accepted: 11/14/2006
Published online: 4/14/2008

Number of Print Pages: 4
Number of Figures: 1
Number of Tables: 1

ISSN: 1015-3837 (Print)
eISSN: 1421-9964 (Online)

For additional information: http://www.karger.com/FDT


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