Subscribe to RSS
DOI: 10.1055/s-0029-1246129
© Georg Thieme Verlag KG Stuttgart · New York
Congenital Segmental Emphysema: An Evolving Lesion
Publication History
received October 15, 2009
accepted after revision November 14, 2009
Publication Date:
15 April 2010 (online)
![](https://www.thieme-connect.de/media/ejps/201002/lookinside/thumbnails/10.1055-s-0029-1246129-1.jpg)
Abstract
Introduction: Congenital segmental emphysema (CSE) is a newly-recognised sub-type of congenital parenchymal lung anomaly. It is characterised by antenatal detection and post-natal evolution from an initially solid segmental appearance to a hyperlucent and hyperinflated segment.
Methods: A retrospective review of a single-centre tertiary referral database between Jan 1994 and Dec 2007 was performed.
Main Results: 130 infants had antenatally detected lung anomalies, and of these 12 (9.2%) infants (initially labelled as congenital cystic adenomatoid malformation (CCAM)), showed features better defined as CSE. The lesions were described antenatally as non-progressive microcystic (n=6), hyperechogenic (n=2) or both (n=2). Early post-natal CT scans showed areas of solid segmental parenchyma, initial hyperlucency or microcysts. Subsequent CT imaging, however, showed evolution to segmental hyperlucency in areas previously solid and in 2 cases a central bronchocele was noted. Ten children underwent resectional surgery (segmentectomy n=4, lobectomy n=6) at a median age of 1 (range 0.4–5.2) year and the gross appearance of the resected specimen confirmed hyperinflated (not cystic) segments. Histological review showed localised abnormally dilated alveolar spaces in 7 cases. Adjacent areas consistent with type 2 CCAM were also seen (n=3).
Conclusion: CSE lies within the spectrum of both CCAM and sequestration but there is a definite post-natal evolution and volume change which presage symptoms. This may be associated with segmental bronchial atresia and progressive air trapping via collateral airways such as the interalveolar pores of Kohn.
Key words
congenital lung anomalies - cystic adenomatoid malformation - bronchial atresia
References
- 1 Adzick NS, Harrison MR, Crombleholme TM. Fetal lung lesions: management and outcome. Am J Obstet Gynecol. 1988; 179 884-889
- 2 Adzick NS, Harrison MR, Glick PL. Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg. 1985; 20 483-488
- 3 Cass DL, Crombleholme TM, Howell LJ. Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg. 1997; 32 986-990
- 4 Davenport M, Warne SA, Cacciaguera S. Current outcome of antenatally diagnosed cystic lung disease. J Pediatr Surg. 2004; 39 549-556
- 5 Karnak I, Senocak ME, Ciftci AO. Congenital lobar emphysema: diagnostic and therapeutic considerations. J Pediatr Surg. 1999; 34 1347-1351
- 6 Kohn HN. Zur Histologie der indurierenden fibrinösen Pneumonie. Münchener Medicinische Wochenschrift. 1893; 40 42-45
- 7 Mani H, Suarez E, Stocker JT. The morphologic spectrum of infantile lobar emphysema: a study of 33 cases. Paediatr Respir Rev. 2004; 5 (Suppl A) 313-320
- 8 Mei-Zahav M, Konen O, Manson D. Is congenital lobar emphysema a surgical disease?. J Pediatr Surg. 2006; 41 1058-1061
- 9 Nobuhara KK, Gorski YC, La Quaglia MP. Bronchogenic cysts and oesophageal duplication: common origins and treatment. J Pediatr Surg. 1997; 32 1408-1413
- 10 Olutoye OO, Coleman BG, Hubbard AM. Prenatal diagnosis and management of congenital lobar emphysema. J Pediatr Surg. 2000; 35 792-795
- 11 Peranteau WH, Merchant AM, Hedrick HL. Prenatal course and postnatal management of peripheral bronchial atresia: association with congenital cystic adenomatoid malformation of the lung. Fetal Diagn Ther. 2008; 24 190-196
- 12 Riedlinger WF, Vargas SO, Jennings RW. Bronchial atresia is common to extralobar sequestration, intralobar sequestration, congenital cystic adenomatoid malformation and lobar emphysema. Pediatr Develop Pathol. 2006; 9 361-373
- 13 Schuster SR, Harris GB, Williams A. Bronchial atresia: a recognizable entity in the pediatric age group. J Pediatr Surg. 1978; 13 682-689
- 14 Stanton M, Davenport M. Management of congenital lung lesions. Early Human Development. 2006; 82 289-295
- 15 Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung: classification and morphologic spectrum. Hum Pathol. 1977; 8 155-172
- 16 Tsai AY, Liechty KW, Hedrick HL. Outcomes after postnatal resection of prenatally diagnosed asymptomatic cystic lung lesions. J Pediatr Surg. 2008; 43 513-517
Correspondence
Prof. Mark Davenport
King's College Hospital
Department of Paediatric
Surgery
SE5 9RS Denmark Hill
London
United Kingdom
Phone: +44 (0)20 3299 3350
Fax: +44 (0)20 3299 4021
Email: markdav2@ntlworld.com