Extralobar pulmonary sequestration: a case report

Abstract

Pulmonary sequestration is defined as a portion of abnormal lung tissue that does not communicate with the tracheobronchial tree through a normally located bronchus and has an abnormal vascular supply [1]. It was first described by Rektorzik in 1861, but the term "sequestration" was introduced by Pryce in 1946 in a 7-case report [2,3]. This extremely rare disorder accounts for 0.16% to 6.4% of all pulmonary congenital malformations [3]. Depending on its location, pulmonary sequestration is generally subdivided into intralobar and extralobar sequestration [4]. Extralobar pulmonary sequestration accounts for 14%-25% of cases [2]. It is most frequently diagnosed in the first 6 months of life, but is seen incidentally, though rarely, in older children and adults [5]. We present an adult case with extralobar pulmonary sequestration. Case ReportA 36-year-old Turkish female, a non-smoker, was admitted to our department with complaints of chest and back pain for five years. Her past history was unremarkable. A chest X-ray (CxR) showed an area of opacity behind the cardiac silhouette in the lower area of the left hemithorax (figure 1). On admission, her resting pulse rate was 86 beats per min., blood pressure 100/70 mmHg, and respiratory rate 18 breaths per min. Her respiratory system and general examinations revealed no significant abnormalities. Full blood count and biochemical tests were normal. Erythrocyte sedimentation rate was 20 mm/h. During hospital stay, the patient was given a course of antibiotics, but opacity in the left lower area persisted. A Computed Tomography (CT) scan of the thorax with intravenous contrast showed a 9 x 7 cm in size ovoid mass with necrosis in the lower left lobe. It revealed two aortic branches directed toward the pulmonary opacity (figure 2), which is consistent with a suspicion of pulmonary sequestration. Bronchoscopic examination revealed a normal endobronchial appearance. Bronchial lavage examination was negative for acid-fast bacilli. Her thoracic Magnetic Resonance (MR) angiography showed an arterial supply from descending thoracic aorta. Pulmonary functional tests were within normal limits. The patient was referred to surgery, and a left-sided thoracotomy was performed. The lesion was located within the pleural space between the diaphragm and the lower lobe. Four arteries arising from the descending aorta were identified. The anomalous pulmonary tissue was removed by mass excision. Pathologic examination of surgical material obtained diagnosis of extralobar pulmonary sequestration. The patient still remains in excellent condition after nearly two years from the operation. We described the case of a 36-year-old Turkish female with an extralobar pulmonary sequestration who suffered from chest and back pain for five years without any evidence of pulmonary infection. A chest X-ray showed an area of opacity behind the cardiac silhouette in the lower area of the left hemithorax. A CT scan of the thorax with intravenous contrast showed a 9 x 7 cm in size ovoid mass with necrosis in the...

A rare tale of an extralobar pulmonary sequestration as a cause of hemoptysis not often contemplated: a case report

“…The pathogenesis of BPS, though not completely understood, has an embryological basis to many theories ( 2 , 8 ). The concept of sequestrations originating from recurrent infections causing an angiogenic process of aberrant arterial vessels arising from the aorta have also been suggested ( 9 ).…”

Contemporary Management of Pulmonary Sequestration

“…Antenatal, ultrasound is a useful tool for diagnosis of PS starting at 18 weeks of gestational age. It has been recommended as the first diagnostic modality to evaluate a suspected supraphrenic pulmonary mass, because it is simple, non-invasive, non-radioactive and can be done as a bed-side procedure [6,7]. The substantial or complete regression of the lesion on sequential scanning has been reported through pregnancy.…”

“…The substantial or complete regression of the lesion on sequential scanning has been reported through pregnancy. The postnatal persistence of the lesion requires further imaging procedures to delineate the aberrant blood supply [6]. However, antenatal PS may disappear on ultrasound but still present by postnatal CT, as it becomes isoechogenic with adjacent normal lung parenchyma [7].…”

“…Extralobar PS is frequently located between the left lower lobe and diaphragm, and for rare instances it may locate intraadiaphragamtic, infradiaphragmatic, intraabdominal, intrapericardial, mediastinal or in the upper thoracic region. In contrast to ILS, more than 50% of ELS cases have other pulmonary anomalies such as bronchogenic cysts, lung hypoplasia, congenital cystic adenomatoid malformation, and congenital diaphragmatic hernia [5,6].…”

Case report: Diagnosis and treatment of pulmonary choristoma in a newborn calf

“…Pulmonary choristoma (PC) is a congenital anomaly characterized by the formation of mass lesions due to reduplication during embryonic development or derived from supernumerary lung buds ( 9 ). This disease is referred to as an ectopic or accessory lung, bronchopulmonary foregut malformation, and extralobar pulmonary sequestration ( 9 , 10 ). Bronchogenic cysts are also etiologically associated with pulmonary sequestration ( 11 ).…”