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Year : 2017  |  Volume : 6  |  Issue : 1  |  Page : 52-54

Extralobar pulmonary sequestration in adults

Post-graduate Student in Radiology, St. John's Medical College, Bengaluru, Karnataka, India

Date of Web Publication20-Mar-2017

Correspondence Address:
Ravikanth Reddy
Post-graduate Student in Radiology, St. John's Medical College, Bengaluru - 560 034, Karnataka
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Pulmonary sequestration is defined as a nonfunctioning mass composed of dysplastic lung parenchyma, embryologically detached from the tracheobronchial tree and receiving its own blood supply from a systemic artery, usually the thoracic or abdominal aorta. It may be intralobar or extralobar depending on the presence of an independent pleural envelope. Extralobar sequestration is a much rarer entity, and diagnosis is usually made antenatally or in early life. Here, we present a rare case of extralobar pulmonary sequestration in an adult.

Keywords: Anomalous long bud, CECT, extralobar pulmonary sequestration

How to cite this article:
Reddy R. Extralobar pulmonary sequestration in adults. J NTR Univ Health Sci 2017;6:52-4

How to cite this URL:
Reddy R. Extralobar pulmonary sequestration in adults. J NTR Univ Health Sci [serial online] 2017 [cited 2022 Jan 17];6:52-4. Available from: https://www.jdrntruhs.org/text.asp?2017/6/1/52/202578

  Introduction Top

Pulmonary sequestration is an uncommon congenital anomaly of the primitive foregut. Extralobar pulmonary sequestration is rare and often found between the lower lobe and diaphragm. The sequestered segment of the lung is completely separated and enclosed in its own pleural investment. The definite diagnosis of pulmonary sequestration depends on the demonstration of the systemic arterial supply and venous drainage. Here, we present a rare case of extralobar pulmonary sequestration in an adult.

  Case Report Top

A 32-year-old woman presented to the emergency department with a 2-month history of left thoracic pain, cough, anorexia, and weight loss. Preoperative chest X-ray done showed a wedge-shaped opacity in the left lower zone causing obliteration of the left costophrenic angle and loss of clarity of the adjacent hemidiaphragm [Figure 1]. Multidetector contrast-enhanced computed tomography (CECT) of the thorax was performed, which revealed a wedge-shaped heterogeneously enhancing soft tissue attenuation mass [Figure 2] and [Figure 3], measuring 6.0 × 3.0 × 5.5 cm (AP × ML × CC) with a central nonenhancing hypodense area. The lesion was noted to derive arterial supply from the thoracic aorta at the level of D9 vertebra. Imaging did not reveal any evidence of air bronchograms communication with the tracheobronchial tree or left pleural effusion. In addition, CECT of the abdomen and pelvis was done to screen for associated congenital anomalies, and a gastric duplication cyst measuring 6 × 5 cm was visualized [Figure 4]. The patient underwent surgery for the extralobar lung sequestration, and a hard mass located above the left hemidiaphragm was resected. A 1-cm diameter arterial vessel supplied the lesion, and venous drainage via the pulmonary veins to the left atrium was confirmed. Pathological examination revealed a mass composed of dysplastic pulmonary tissue with an independent pleural envelope, thus confirming the diagnosis of pulmonary sequestration of the extralobar type. Postoperative chest X-ray done 2 weeks apart showed mild hypovascularity in the left lower lobe following resection of the extralobar pulmonary sequestration [Figure 5].
Figure 1: Chest radiograph PA view showing a wedge shaped opacity in the left lower zone (arrow) with obliteration of the left costophrenic angle and silhouetting of the left hemidiaphragm

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Figure 2: Axial CECT MIP image showing heterogeneously enhancing soft tissue attenuation mass (arrow) above the left hemidiaphragm demonstrating arterial supply from the thoracic aorta at the level of D9 vertebra

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Figure 3: Coronal reformatted 3D image demonstrating an abnormal vessel arising from the thoracic aorta (arrow) feeding the pulmonary mass at the left inferior lobe

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Figure 4: CECT image of the abdomen demonstrating a gastric duplication cyst (arrow)

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Figure 5: Post-operative chest radiograph PA view done 2 weeks following resection of the mass lesion showing mild hypovascularity in the left lower lobe (arrow)

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  Discussion Top

Pulmonary sequestration is a nonfunctioning lung tissue that is not in normal continuity with the tracheobronchial tree deriving its blood supply from systemic circulation. It constitutes 0.15–6.4% of all congenital pulmonary malformations.[1] Extralobar pulmonary sequestration occurs more frequently in males than females with a male-to-female ratio of 4:1.[2] Extralobar sequestration is the result of an anomalous or supernumerary lung bud that derives its blood supply from the primitive splanchnic vessels that surround the foregut. The anomalous arterial supply of the sequestration is usually from the dorsal aorta.[3] Clinical manifestations usually occur in the newborn period or early infancy with symptoms of respiratory distress, repeated infections, high output congestive heart failure, and spontaneous pulmonary or pleural hemorrhage. Treatment consists of complete surgical excision of the extralobar sequestration. Congenital anomalies occur more frequently in patients with extralobar sequestration than intralobar lung sequestration. Associated anomalies include congenital cystic adenomatoid malformation, congenital diaphragmatic hernia, vertebral anomalies, congenital heart disease, pulmonary hypoplasia, colonic duplication, tracheoesophageal fistula, bronchogenic cyst, pericardial defects, and congenital megacolon. Approximately 50–60% of the patients have associated malformations, the most common being congenital diaphragmatic hernia with the esophagus or stomach.[4] Type II cystic adenomatoid malformation of the lung has been reported in 15–25% of patients with extralobar sequestration. Our case demonstrated a gastric duplication cyst.

  Conclusion Top

In conclusion, the diagnosis of pulmonary sequestration relies on the demonstration of aberrant systemic arterial supply to a pulmonary or juxtapulmonary mass on imaging. Differential diagnosis of extralobar pulmonary sequestration is consolidation, intrathoracic kidney, and hepatic herniation through the diaphragm. Complete surgical excision of the sequestrum is the treatment of choice to reduce the risk of repeated infections.

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  References Top

Lin CH, Chuang CY, Hsia JY, Lee MC, Shai SE, Yang SS, et al. Pulmonary sequestration-differences in diagnosis and treatment in a single institution. J Chin Med Assoc 2013;76:385-9.  Back to cited text no. 1
Wei Y, Li F. Pulmonary sequestration: A retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg 2011;40:e39-42.  Back to cited text no. 2
Andrade CF, Ferreira HP, Fischer GB. Congenital lung malformations. J Bras Pneumol 2011;37:259-71.  Back to cited text no. 3
Sulhyan KR, Ramteerthakar NA, Gosavi AV, Anvikar AR. Extralobar sequestration of lung associated with congenital diaphragmatic hernia and malrotation of gut. Lung India 2015;32:381-3.  Back to cited text no. 4
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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