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Neonatal Presentation of Extralobar Pulmonary Sequestration
Nitin P Dhende*, SB Mane**, Santosh B Kurbet***, Samir Gupta****, Suresh Basarkod***
Antenatally diagnosed as congenital cystic adenomatoid malformation presented on day one of life with respiratory distress. Clinically no abnormality was detected but Contrast CT thorax revealed extralobar pulmonary sequestration supplied by a separate artery arising from aorta. The neonate underwent thoracotomy and excision of the sequestrated lobe successfully.

Pulmonary sequestration is a rare congenital malformation of lung tissue which receives blood supply from aorta and has no communication with tracheobronchial tree. There are two forms of pulmonary sequestration, intralobar the commoner one and extralobar.We report a case of neonatal presentation of extralobar pulmonary sequestration.

Case Report
Antenatally suspected to have left lower lobar congenital cystic adenomatoid malformation by ultrasonography was referred after delivery with complaints of transiently increased respiratory activity. The neonate was examined and found to have no positive findings. Routine haemogram was normal, chest X-ray (Fig. 1) revealed hazy left cardiac border and, lateral view showed no abnormality. CT scan with contrast study showed pulmonary sequestration left side posteromedial region (Fig. 2) with demonstrable single arterial twig arising from abdominal aorta.

Patient underwent left posterolateral transpleural thoracotomy and found to have extralobar sequestration (Fig. 3). The sequestrated lobe was supplied by single arterial twig coming from abdominal aorta (Fig. 4). Excision of extralobar sequestration with ligational division of the artery coming from aorta. Histopathology of specimen revealed diagnosis of extralobar pulmonary sequestration. Post-operative period was uneventful, chest drain removed on day 3 and patient discharged on day 5. On follow up the infant had gained weight and doing well.

Pulmonary sequestration is a rare congenital anomaly, it is a nonfunctioning lung tissue with no tracheobronchial

Fig. 1 : Chest X-ray showing hazy left cardiac border.

Fig. 2 : CT-Scan thorax showing left posteromedial extralobar
pulmonary sequestration.

Fig. 3 : Operative photograph showing sequestrated

Fig. 4 : Sequestrated lobe being supplied by a branch
from abdominal aorta.

communication and separate blood supply from aorta. Of the two types, intralobar presents with recurrent respiratory infections and extralobar is asymptomatic or presents with respiratory distress at birth. Embryologically pulmonary sequestration is due to an accessory lung bud arising from foregut in extralobar type hence invested by separate visceral pleura, and from normal lung buds in intralobar type.2 They receive blood supply from aorta and drain usually into pulmonary veins and rarely into azygous veins or inferior vena cava.2 The communication with foregut rarely persists and there is no communication with tracheobronchial tree. Extralobar sequestration is lined by separate visceral pleura. Commonest site is left side medial posterobasal region of thorax, can also occur posterolateral right side when communication with foregut is common and rarely is found in left adrenal region.4 Most commonly males are affected in the ratio of 4:1.2 Associated anomalies include congenital diaphragmatic hernia, eventration of diaphragm, ectopia cordis, pericardial defects in extralobar pulmonary sequestration.5

Diagnosis of extralobar pulmonary sequestration is made incidentally when being investigated for other conditions or when being operated for condition such as Congenital diaphragmatic hernia, Eventration of diaphragm or cardiac malformations. Some patients present during late infancy and early childhood with chronic cough, feeding difficulties and respiratory distress. The differential diagnosis include posterior mediastinal lesions as ganglioneuromas, neuroblastoma lung conditions as pneumonias. Antenatally extralobar pulmonary sequestration can be diagnosed by Doppler study. Other diagnostic modalities include chest X-ray, CT scan, 3D Contrast CT scan, M R angiography. Demonstration of separate vessel arising from aorta is the definitive diagnosis. With the advent of these new minimally noninvasive investigations, angiography is not done routinely these days.

Treatment is early excision of the sequestration to prevent repeated respiratory infections, adhesions and risk of high surgical morbidity and mortality, which we followed with a good outcome.


  1. Bliek AJ, Mulholland DJ. Extralobar lung sequestration associated with foetal neonatal respiratory distress. Thorax 1971; 26 : 125.
  2. Bruce M, Schnapf DO. Pulmonary sequestration. E-Medicine Journal 2003 Topic -2628.
  3. Felkar RE, Tonkin ILD. Imaging of Pulmonary Sequestration. American J Roentgenology 1990; 154 : 241-9.
  4. Grece RD, Jarefzki A III, Ashley CA, et al. Congenital bronchopulmonary foregut malformation: Pulmonary sequestration communicating with gastrointestinal tract. New England J Medicine 1968; 278 : 1413.
  5. Shah MS, Bhatt CB, Modi JM. Case Report: Extralobar Pulmonary Sequestration. Ind J Radiol Imaging 2003; 13 (3) : 271.

‘Members of the staphylococci are getting together to share genetic information, widening the range of hosts that can be infected by its most aggressive species’
Binh An Diep and colleagues publish the complete genome sequence of USA300-a community acuired meticillin-resistant species of Staphylococcus aureus. In their comparisons of the genome of one particular strain of USA300 (FPR3757) with those of other staphylococcal strains they noted various similarities, suggesting sharing of mobile genetic elements. This finding is especially important because of the nature of the shared material - it encodes resistance and virulence determinants that could enhance fitness and pathogenicity. In a Comment, Marc Enright sees history repeating itself: “Hospital and community MRSA were first formed by the transfer of resistance elements of S aureus from other staphylococci, and it now seems that this gene-pool sharing did not stop there”. All hope is not lost though; as noted in one of this week’s Editorials, the mapping of genomes of major pathogens is an essential step in the identification of mutations that lead to multidrug resistance and its subsequent prevention.

Lancet, 2006; 704, 705, 731.



*Lecturer; **Asst Professor; ***Senior Resident; ****Chief Resident, Department of Paediatric Surgery, Grant Medical College, Mumbai-400 008.