H. Frimmel (Halle/S.)  

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21 Months  

Male  

Thorax-Lung  

congenital  

1 9/12 year-old boy. Reoccurring respiratory infections. In the X-ray (sent to us from another clinic), segmental, basal opacities on the left side.  

Pulmonary sequester = "Rokitansky-lobe"
Congenital malformation of the lung.
A mass of non-aerated pulmonary tissue that is not connected to the normal bronchial tree. It receives its own blood supply from a systemic artery.
In 65% of the cases in the left lobe, otherwise in the right, posterior lower lobular segment.
Two forms:
Intralobular (75-85%) - without own pleura, drainage into the pulmonary veins.
Extralobular (15-25%) - with own pleura, drainage into the vena cava/azygos vein.
 

<- view MRI 1

MRI 1: T2-HASTE coronal: conically configurated, ca. 4x2cm large structure in the left dorsobasal lung, In the T2-weighted image, an obvious signal intensity increase is seen.







<- view MRI 2

MRI 2: T1-FLASH coronal with contrast: strong, homogenic, aortic synchronized enhancement of the sequestration after application of contrast. No aerated areas within this structure.







<- view MRI 3

MRI 3: MRA (MIP-Projections): Proof of the systemic arterial vasculature from the aotra and the venous drainage in the hemiazygos vein.













<- view MRI 4

MRI 4: MRA (MIP-Projections): Proof of the systemic arterial vasculature from the aotra and the venous drainage in the hemiazygos vein.









<- view MRI 5

MRI 5: MRA (MIP-Projections): Proof of the systemic arterial vasculature from the aotra and the venous drainage in the hemiazygos vein.










 

Imaging including endoscopy  

In obvious sequester-vascular supply: none.  

Congenital deformity.
Often secondary hematogenic infection with delayed healing and sustained inflammatory symptoms due to missing aeration (no self-cleaning).
Mostly, surgical resection. Sometimes also prophylactically due to the danger of infection.  

The presented case is the classical example of a lung sequester. Here, probably an extralobular sequester.  

N/A  

1. Medline:
M. Galanski, M. Prokop
Ganzkörper-CT
Georg-Thieme-Verlag 1998

2. Medline:
Psychrembel
Klinisches Wörterbuch, 256. Auflage
de Gruyter-Verlag 1990  

Lung sequester, extralobular, Rokitansky lobe, Rokitansky, lung sequestration, recurrent respiratory infections, child, childhood, pediatric radiology  

H Frimmel. Lung sequestration, extralobar. PedRad [serial online] vol 2, no. 8.
URL: www.PedRad.info/?search=20020815182432