Congenital lobar emphysema in neonates:

Anaesthetic challenges.
Nath MP, Gupta S, Kumar A,

Chakrabarty A.

Source
Department of Anaesthesiology and Critical Care, Gauhati Medical College Hospital, Guwahati, Assam, India.

Abstract
Congenital lobar emphysema (CLE) is a potentially reversible, though possibly life-threatening, cause of respiratory distress in the neonate. It poses dilemma in diagnosis and management. We are presenting a 6-week-old baby who presented with a sudden onset of respiratory distress related to CLE affecting the left upper lobe. Lobectomy was performed under general anaesthesia with one lung ventilation. The details of anaesthetic challenges and management are described here.

http://www.ncbi.nlm.nih.gov/pubmed/21808403

Preoperative MDCT evaluation of congenital lung anomalies in children: comparison of axial, multiplanar, and 3D images.
(PMID:21512069)

Abstract Citations BioEntities Related Articles

Lee EY, Tracy DA, Mahmood SA, Weldon CB, Zurakowski D, Boiselle PM
Department of Radiology, Children's Hospital Boston, Harvard Medical School, MA 02115, USA. Edward.Lee@childrens.harvard.edu AJR. American Journal of Roentgenology [2011, 196(5):1040-6] Type: Journal Article, Comparative Study DOI: 10.2214/AJR.10.5357 Abstract Highlight Terms
Diseases(1)

OBJECTIVE: The objective of our study was to compare the preoperative diagnostic accuracy of axial, multiplanar, and 3D MDCT images for evaluating congenital lung anomalies in pediatric patients and to assess the potential added diagnostic value of multiplanar and 3D MDCT images in this setting. MATERIALS AND METHODS: We used our hospital information system to identify all consecutive pediatric patients younger than 18 years who had undergone preoperative MDCT angiography and had a pathologically proven congenital lung anomaly between June 2005 and February 2010. Each MDCT examination was reviewed independently by two experienced pediatric radiologists for the types, location, associated mass effect, and associated anomalous vessels of congenital lung anomalies on axial, multiplanar, and 3D MDCT images. The final diagnosis was determined by surgical and pathologic findings. Diagnostic accuracy, confidence level of diagnosis (scale of 1-3: 1 = highest confidence and 3 = lowest), perceived added diagnostic value of multiplanar or 3D MDCT images (scale of 1-5: 5 = highest added diagnostic value and 1 = lowest), and interobserver kappa agreement were evaluated. RESULTS: The final study cohort consisted of 46 pediatric patients (28 males and 18 females; mean age, 5.6 6 [SD] months; range, 1 day-50 months). Histopathologic diagnoses included congenital pulmonary airway malformation (n = 19, 41%), sequestration (n = 15, 33%), congenital lobar emphysema (n = 7, 15%), and

bronchogenic cyst (n = 5, 11%). Both independent reviewers correctly diagnosed types, location, associated mass effect, and associated anomalous arteries of all congenital lung anomalies with high accuracy (100%) and confidence level (mean confidence level < 1.2) on each type of image display (axial, multiplanar, and 3D). However, for the detection of anomalous veins, multiplanar and 3D images were associated with greater diagnostic accuracy and higher confidence level than axial images alone. Specifically, diagnostic accuracy for the detection of anomalous veins (n = 15; 33%) was 60% (9/15 cases) for axial MDCT images, 80% (12/15) for multiplanar MDCT images, and 100% (15/15) for 3D MDCT images (Friedman test, p = 0.011). Confidence levels for the detection of anomalous veins were significantly higher with 3D MDCT images (mean level = 1.0) and multiplanar MDCT images (mean level = 1.5) compared with axial MDCT images alone (mean level = 2.6) (Friedman test, p < 0.01). Both multiplanar and 3D MDCT images were found to provide added diagnostic value for accurately detecting anomalous veins associated with congenial lung anomalies (paired Student t tests, p < 0.012). CONCLUSION: Axial MDCT images allow accurate diagnosis of the types, location, associated mass effect, and anomalous arteries of congenital lung anomalies, but supplemental multiplanar and 3D MDCT images add diagnostic value for the evaluation of congenital lung lesions associated with anomalous veins.

http://ukpmc.ac.uk/abstract/MED/21512069
Pediatr Surg Int. 2001 Mar;17(2-3):88-91.

Congenital lobar emphysema: experience with 21 cases.

Thakral CL, Maji DC, Sajwani MJ.

Source
Department of Pediatric Surgery, Royal Hospital, P.O. Box 1331, Postal Code 111, Muscat, Sultanate of Oman.

Abstract
A retrospective study of all cases (n = 21) of congenital lobar emphysema (CLE) treated at the Royal Hospital, Muscat, from September 1988 to August 1999 was performed. The presentation, diagnosis, treatment, and outcome are reviewed. All 21 patients were nonwhite. Thirteen had left-upper-lobe, 7 right-middle-lobe, and 1 right-upper-lobe involvement. Of the 14 upperlobe cases, 10 had severe symptoms whereas only 2 of the 7 middle-lobe cases had severe symptoms. Mildly symptomatic cases on conservative treatment invariably needed surgery if infected. It is concluded that CLE is not limited to white infants, upper-lobe disease is more severe than middle-lobe disease, and nonoperative measures are likely to be useful in mildly symptomatic cases involving the middle lobe.
PMID:

11315310

[PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed?term=11315310

Medline Abstract for Reference 10

of 'Congenital lobar emphysema'
10 PubMed TI Prenatal diagnosis and management of congenital lobar emphysema. AU Olutoye OO, Coleman BG, Hubbard AM, Adzick NS SO J Pediatr Surg. 2000;35(5):792.

BACKGROUND: Congenital lobar emphysema (CLE) is a rare anomaly of lung development that usually presents in the neonatal period with respiratory distress and pulmonary lobar hyperinflation. The routine use of prenatal ultrasonography has resulted in the early identification and serial evaluation of congenital lung lesions. CLE can be distinguished from other congenital lung lesions on ultrasonography by the differences in echogenicity and reflectivity. METHODS: Two cases of CLE diagnosed at midgestation by ultrasonography and ultrafast fetal magnetic resonance imaging (MRI), along with serial sonographic documentation of their prenatal course were reviewed. RESULTS: The CLE lesions decreased in size over the course of the pregnancy, similar to that seen with other congenital lung lesions such as cystic adenomatoid malformation and bronchopulmonary sequestration. However, these neonates with CLE showed marked air-trapping and respiratory distress requiring lobectomy in the early neonatal period. CONCLUSIONS: These cases provide insight into the prenatal course of CLE and underscore the need for continued postnatal evaluation of fetuses even those in whom the lesions appear to have resolved in utero. These patients should have ready access to postnatal surgical intervention. AD Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, PA, USA. PMID 10813352

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Congenital lobar emphysema

A severe case of bullous emphysema

CLE results in overexpansion of a pulmonary lobe and resultant compression of the remaining lobes of the ipsilateral lung, and possibly also the contralaterallung. There is bronchial narrowing because of weakened or absent bronchial cartilage.[4] There may be congenital extrinsic compression, commonly by an abnormally large pulmonary artery. This causes malformation of bronchial cartilage, making them soft and collapsible.[4] CLE is potentially reversible, yet possibly life-threatening, causing respiratory distress in the neonate.[4]
^ a b c eMedicine Specialties > Radiology > Pediatrics --> Congenital Lobar Emphysema Author: Beverly P Wood, MD, MS, PhD, University of Southern California. Updated: December 1, 2008

Overview
Congenital lobar emphysema (CLE) is a potentially reversible though possibly life-threatening cause of respiratory distress in the neonate. See the images below of congenital lobar emphysema.

A frontal radiograph of the chest in a neonate shows marked overdistention of

the left upper lobe with mediastinal shift to the right.

Histopathology of congenital

lobar emphysema with marked overdistention of all alveoli. overexpanded and shows no other intrinsic abnormality.

Resected lobe is

Congenital lobar emphysema is most often detected in neonates or identified during in utero ultrasound. Anomalies are infrequent and usually present at birth. Lobar distention can be visible during in utero ultrasound as an overinflated, fluid-filled lobe; in less severe cases, the diagnosis is made in infancy or childhood.[1, 2, 3, 4, 5, 6] CLE almost always involves one lobe, with rates of occurrence as follows: Left upper lobe - 41% Right middle lobe - 34% Right upper lobe - 21% Congenital lobar emphysema has 2 forms: Hypoalveolar (fewer than expected number of alveoli) Polyalveolar (greater than expected number of alveoli)

The thorax on the involved side is hyperresonant with decreased or absent breath sounds and transillumination. Progressive respiratory distress from birth reflects the degree of emphysema; symptoms are at their worst in the first month. Occasionally, patients present in later childhood or adulthood. Congenital lobar emphysema (ie, congenital lesion) should be differentiated from Swyer-James syndrome (ie, acquired pulmonary abnormality secondary to infection). In Swyer-James syndrome, infection results in the following: Vascular compromise "Pruning" of peripheral pulmonary vasculature Small but hyperlucent lung (as the opposite, normal lung grows, the involved lung does not grow and appears more radiolucent)

http://emedicine.medscape.com/article/407635-overview

Bibliography

http://en.wikipedia.org/wiki/Emphysema#cite_note-Wood-3 http://www.uptodate.com/contents/congenital-lobar-emphysema/abstract/10