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Year : 2003  |  Volume : 13  |  Issue : 3  |  Page : 271-273
Case report : Extra lobar pulmonary sequestration

Department of Radiology & Imaging, V. S. General Hospital, Smt, N.H.L Municipal Medical College, Ahmedabad-380 006, India

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Keywords: Pulmonary Sequestration

How to cite this article:
Shah M S, Bhatt C B, Modi J M. Case report : Extra lobar pulmonary sequestration. Indian J Radiol Imaging 2003;13:271-3

How to cite this URL:
Shah M S, Bhatt C B, Modi J M. Case report : Extra lobar pulmonary sequestration. Indian J Radiol Imaging [serial online] 2003 [cited 2020 Nov 28];13:271-3. Available from:

   Introduction Top

Pulmonary sequestration is an uncommon congenital usually cystic mass of non-functioning primitive lung tissue that does not communicate with the tracheobronchial tree or the pulmonary arteries. It is supplied by an anomalous artery arising from aorta & venous drainage is via the azygos system, pulmonary veins or the inferior vena cava [1],[2].

The two forms of pulmonary sequestration include, intrapulmonary which is surrounded by normal lung tissue & extra pulmonary which has its own pleural investment. Other congenital malformation may be present. We report a case of extra lobar pulmonary sequestration [2].

   Case report Top

A woman with 8-month amenorrhoea came to hospital for Antenatal USG for the first time. She was second gravida with previous history of full term normal delivery. Sonography performed with a 3.5 MHz Curvilinear probe, showed a cystic lesion of 17x16 mm in size to the, right of and inferior to the heart, situated mainly between left lobe of liver, heart & right lung field [Figure - 1],[Figure - 2]. Spine & brain appeared normal. All other organs did not reveal any abnormality. There was no evidence of poly or oligohydraminos. On the basis of sonography findings a diagnosis of Neurenteric cyst or Bronchogenic cyst was made.

After full term normal delivery of male child, chest x-ray showed welldefined cystic lesion in posterior mediastinum on right side [Figure - 3].

CT scan of the Thorax performed on third day after birth, confirmed the previous sonography findings. CT scan showed a well-defined cystic lesion of fluid density in posterior mediastinum on right side. The lesion did not show any enhancement on post contrast study. [Figure - 4],[Figure - 5]. On the basis of CT scan findings a diagnosis of Neurenteric cyst (? Bronchogenic cyst) was made.

Subsequently Barium study was done to see communication with oesophagus. Barium study showed an impression on lower part of oesophagus on the right side.

Thoracotomy done on the 25th day of life showed a cystic lesion in posterior mediastinum on right side, separate from the bronchi.

Histopathology external surface pinkish; cut surface revealed a cyst of 2x1.2 cm on size, containing clear fluid. Sections on microscopic examination reveal dilated pulmonary alveoli & bronchi with one large cyst with a lining of bronchial epithelium, smooth mucus & dilated lymphatics in the wall. Blood vessels were dilated & congested; diagnosis of extra lobar pulmonary sequestration was made [Figure - 6].

   Discussion Top

A pulmonary sequestration is an uncommon cystic mass of nonfunctioning primitive lung tissue with no communication with tracheobronchial tree or pulmonary arteries. Out of the two forms, in extra pulmonary form males are affected 4 times more frequently than female [1].

Most frequently supported theory of how a sequestration arises is that an accessory lung bud develops from the ventral aspect of the primitive gut. The pleuropotential tissue from this additional lung bud migrates in a caudal direction with the normally developing lung. It receives its blood supply from vessels that connect to the aorta & that cover the primitive gut. The attachments to the aorta remain to term resulting in the systemic arterial supply of the sequestration.

Early embryonic development of the accessory lung bud results in formation of sequestration within normal lung tissue & encasement within the same pleural covering. This is intrapulmonary variant. In contrast later development of the accessory lung bud results in the extra pulmonary type. Both types have arterial supply from the thoracic or abdominal aorta. Rarely, the celiac axis, internal mammary, subclavian or renal artery may be involved [1].

Extra pulmonary sequestration is enclosed completely in its own pleural sac. It may occur above, within or below the diaphragm & nearly all appear in left lung. Venous drainage is via the systemic venous system. Foregut communication & associated anomalies such as diaphragmatic hernia, are more common.

More than half of the cases of extra pulmonary sequestrations are diagnosed when patients are younger than 1 year [1]. Associated congenital anomalies, include congenital diaphragmatic hernia, cardiac malformations & gastrointestinal malformations [2]. Many patients present in infancy with respiratory distress & chronic cough.[3] Lesions are commonly diagnosed coincidently during investigation of, or surgery for, an associated congenital anomaly. Therefore clinical symptoms may be absent or minor. Rarely it presents with gastrointestinal symptoms if communications with the gastrointestinal tract is present. As a result, feeding difficulties may be present in infants.[4]

The differential diagnosis of an extra pulmonary sequestration includes consolidation. But here in view of cystic lesion a diagnosis of bronchogenic cyst was kept. So, this is cystic variety of extra lobar pulmonary sequestrum.

The treatment of an extra pulmonary sequestrum is mainly surgical & total excision of the sequestrum to reduce the risk of repeated infection.

   References Top

1.Bruce M. Schnapf, DO, Pulmonary sequestration, E- Medicine Journal, Aug. 2003 topic - 2628.   Back to cited text no. 1    
2.Felker R.E. Tonkin ILD, 1990 Imaging of pulmonary sequestration, Am J Roentgenol. 154 : 241-249.   Back to cited text no. 2    
3.Bliek. A. J., and Mulholland, D.J Extralobar lung sequestration associated with foetal neonatal respiratory distress. Thorax 26 : 125, 1971   Back to cited text no. 3    
4.Gerce; R. D., Jarefzki, A.I I I , Ashley,C.A; and Berne, A.S. : congenital broncho pulmonary foregut malformation : Pulmonary sequestration communicating with the gastrointestinal tract. New Eng. J. Med. 278 : 1413,1968.  Back to cited text no. 4    

Correspondence Address:
M S Shah
14, Vibhuti society, near Baliakaka Mandir, Old Dhor Bazaar, Ahmedabad-380028
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Source of Support: None, Conflict of Interest: None

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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]


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