Intra-abdominal pulmonary sequestration: a benign suprarenal mass

A 34-year-old white woman presented with an 8.5-cm left suprarenal mass. Evaluation revealed normal adrenal hormone function. Pathologic examination after surgical removal revealed a rare benign condition, intra-abdominal extralobar pulmonary sequestration (accessory lung).     

Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation

BACKGROUND: Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favourable natural history than that of sequestration without associated cystic adenomatoid malformation. METHODS: An outline of the postnatal work up leading to the management of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation diagnosed antenatally as pulmonary malformation is presented and the indications for surgical intervention are discussed. RESULTS: In five infants in whom an antenatal ultrasound scan had detected a congenital lung malformation at 18-19 weeks gestation a final diagnosis of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation was made postnatally. Postnatal ultrasound and computerised axial tomographic scans confirmed the diagnosis of sequestration by delineating anomalous vascular supply. Cystic changes were also observed in the basal area of the sequestration in all patients. Four children remained asymptomatic and one infant presented at 10 months of age with pneumonia. The mean age at surgical resection was 6.8 months (range 2-10). Histopathological examination confirmed intralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in two patients and extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean period of follow up was four years (range 1-8). The children remain well and are developing normally. CONCLUSIONS: The importance of seeking an anomalous blood supply in children with congenital lung lesions is emphasised. Pulmonary sequestration and congenital cystic adenomatoid malformation probably share a common embryogenesis despite diverse morphology. The natural history of antenatally diagnosed lung masses is variable. Early postnatal surgical resection of pulmonary sequestration with cystic adenomatoid malformation is recommended. Surgical excision should be conservative, sparing the normal lung parenchyma.     

Extralobar pulmonary sequestration

Extralobar sequestration of pulmonary tissues is relatively rare and seldom produces symptoms. An incidental finding is described of extralobar sequestration of the lung in a Nigerian child, presenting as an anterior mediastinal polycystic mass. The mass was attached to the right pulmonary artery by a fibrovascular pedicle which contained a small elastic artery, veins, and nerve bundles. Sequestrated lobes are usually situated in the posterior basal lobes of the lungs and may be associated with other congenital abnormalities, but the case reported here differs in these respects. Death was due to enterocolitis unrelated to the congenital abnormality.     

Intralobar Bronchopulmonary Sequestration with Mixed Venous Drainage in a 10-Year-Old Girl

Bronchopulmonary sequestration consists of a mass of abnormal lung tissue that has no normal connection with the bronchial tree and is supplied with blood from an aberrant artery mostly originating in the thoracic aorta. Two forms are recognized: intralobar and extralobar sequestration. The first is localized within the normal visceral pleura and has a venous drainage into the pulmonary system; the latter is localized without the normal lung in its own pleura with venous drainage into the systemic venous system. Intralobar sequestration is the most common form accounting for 75% of the cases. Intralobar sequestration usually presents in adolescence or adulthood with signs of recurrent pneumonia. Extralobar sequestration presents early in life with respiratory distress or feeding difficulties and is frequently associated with other congenital malformations. The diagnosis is confirmed by CT scan of the lungs and magnetic resonance angiography as demonstration of the aberrant vascular supply is essential for the diagnosis. Therapy consists in surgical removal.

We present a case of intralobar sequestration in a 10-year-old girl. The clinical symptomatology was typical. Arterial supply with two aberrant arteries and mixed venous drainage into the pulmonary and systemic systems were particular features.     

Malignant spindle cell tumour (fibrosarcoma) in sequestration of lung—a case report

Pulmonary sequestration is an uncommon congenital anomaly of respiratory system and usually diagnosed in childhood but can present at any at age. It frequently presents as a mediastinal mass with symptoms from vascular shunting, or associated anatomic defects. This case report describes an adult who presented with chest pain due to extralobar sequestration of right lung extending into the mediastinum. Per-operatively it was found to be an extra pulmonary mass lesion with a peduncle attached to right lung suggestive of extralobar sequestration. Histopathological examination of the specimen showed it to be spindle cell tumor (fibrosarcoma) in sequestrated lung. The patient had an uneventful recovery and is under regular follow up without any further problems.     

Infarction of extralobar pulmonary sequestration after blunt trauma

Pulmonary sequestration is a rare congenital lung malformation that occurs in either an intralobar or extralobar location and is generally asymptomatic. We report an unusual case of a child who presented with infarction of a previously asymptomatic extralobar pulmonary sequestration after blunt trauma, eventually requiring surgical excision.     

Video-assisted thoracoscopic lobectomy in the treatment of intralobar pulmonary sequestration

Pulmonary sequestration is a congenital malformation characterised by cystic, non-functioning embryonic lung tissue with vascularisation of an abnormal systemic artery. They are classified as intralobar (75%) and extralobar (25%) and are more common in the left lung and lower lobes (60-90%). We report two cases of intralobar pulmonary sequestration located in the lower lobe of the left lung which were subjected to video-assisted thoracoscopic surgery (VATS). Both patients had recurrent infections for which, after performing imaging tests, they were diagnosed with intralobar pulmonary sequestration in the left lower lobe, with an afferent arterial branch to the malformation from the aorta. A lower lobectomy was performed by video-assisted surgery, dividing the aberrant aortic artery with an endostapler. A single thoracic chest tube was placed and removed on postoperative day 2 and the patients were discharged on the same day. In both cases, the pathology examination revealed intralobar pulmonary sequestration. Pulmonary sequestrations are uncommon malformations that can be operated on using minimally invasive techniques, thereby permitting early discharge and a low rate of complications.     

Extralobar pulmonary sequestration with aberrant lobulation of the lower lobe; report of a case

An adult case of extralobar pulmonary sequestration with aberrant lobulation of the right lower lobe is reported. A 32-year-old woman admitted to our hospital with a right intrathoracic tumor in the chest computed tomography (CT). Chest CT showed a mass lesion on the right diaphragm of a diameter 5 cm and abnormal fissure of the right lower lobe. Under the diagnosis of pulmonary sequestration without clearly thick vessels, video-assisted thoracoscopic surgery was performed. The mass existed between the right diaphragm and posterior lower lobe with a thin stalk, and the lower lobe had aberrant lobulation. The resected mass was histopathologically diagnosed as extralobar pulmonary sequestration.     

Upper thoracic extralobar pulmonary sequestration with anomalous blood supply from the subclavian artery

The authors report on a newborn boy with extralobar pulmonary sequestration in the right upper thoracic region. Preoperative angiography showed an anomalous large vessel from the right subclavian artery, supplying the sequestrated lobe. Right thoracotomy was performed to resect the sequestrated lobe, which was diagnosed as extralobar form. This is an extremely rare case of extralobar pulmonary sequestration in which anomalous blood supply from the subclavian artery was seen preoperatively on radiographs. The authors recommend angiographic examination, particularly in case of diagnostic difficulty.     

Treatment of pulmonary sequestration by video-assisted thoracic surgery (VATS)

Pulmonary sequestration is a rare malformation of the respiratory tract that accounts for 0.15–6.4% of all congenital lung anomalies. Treatment requires resection of the lesion, provided that there is no technical contraindication. The lession should first be evaluated using video thoracoscopy and then resected whenever possible by video-assisted thoracic surgery (VATS). We report a case of extralobar pulmonary sequestration in a 48-year-old woman. She underwent lobectomy by VATS and achieved an excellent outcome.     

Pulmonary Sequestration and Pterygium Colli Syndrome

A rare case of a male pterygium colli syndrome associated with an extralobar pulmonary sequestration is reported. The patient had webbed neck, low-set ears, low-set hair line, aplasia of the left kidney and hypoplastic mesenterial vessels referring to Turner's syndrome, but a normal XY sex chromosome constitution. The pulmonary sequestration was resected and the patient made an uneventful recovery.     

Pulmonary sequestration in older child and in adults.

Pulmonary sequestration is often found in children with recurrent pneumonia, but is not rare in adults. During the last 6 years (1985-1990), 6 patients with pulmonary sequestration were surgically treated. One 40-year-old patient had an extralobar sequestration, and 5 patients ranging in age from 14 to 26 years had an intralobar sequestration. The patient with extralobar sequestration was asymptomatic. In cases of intralobar sequestration, 4 patients were symptomatic. All 6 patients had an abnormal chest roentgenogram. Preoperative arteriography revealed anomalous blood supply came from a systemic artery in 4 patients. All cases with intralobar sequestration had a lower lobectomy, and one case with extralobar sequestration had an extirpation. Even in cases without any symptoms, there was the possibility of a microscopic infection in the sequestrated lesion with the occurrence of later symptoms. Therefore, patients diagnosed with pulmonary sequestration should be considered for surgical resection.     

Intra-abdominal pulmonary secuestration as an exceptional cause of abdominal mass in the adult

INTRODUCTIONPulmonary sequestration (PS) is an extremely rare malformation defined as a portion of lung tissue isolated from the pulmonary system. PSs are classified into intralobar type and intra-abdominal PS that represents only 2.5% of cases. There are 20 cases of PS reported in adults and only two were managed by laparoscopic approach. We report a case of intra-abdominal PS mimicking a gastroesophageal duplication cyst in an adult. Besides its rarity, this is the first intra-abdominal PS in an adult managed by an anterior laparoscopic approach.PRESENTATION OF CASEA 60-year-old female patient had had epigastric and left upper quadrant pain for several days. Physical examination was normal. Image test were consistent with a gastroesophageal duplication. The patient was taken to the operating room for laparoscopic exploration and resection. The pathological diagnosis was extralobar pulmonary sequestration.DISCUSSIONLess than 20 cases of PS have been reported in adults and only two cases were managed by a lateral laparoscopic approach. In contrast to these reports, we used an anterior approach due to the GEJ suspected origin of the mass.CONCLUSIONExtralobar intra-abdominal PS is an extremely rare condition during adulthood but this diagnosis should be included in the differential diagnosis of a left-sided suprarenal mass. Due to the difficulty in achieving a definitive preoperative diagnosis, surgery is recommended. Laparoscopic resection is safe and effective but careful preoperative imaging studies are recommended in order to plan the most suitable approach.     

Thoracoscopic resection of an apical extralobar pulmonary sequestration in an infant

Pulmonary sequestration is a form of bronchopulmonary-foregut malformation that is treated with surgical resection, either via a thoracotomy or the thoracoscopic approach. Apical extralobar pulmonary sequestrations are rare. We report a case of an apical pulmonary sequestration in an infant that was amenable to thoracoscopic resection. An 8-month-old girl, weighing 10.3 kg, with a left apical extralobar pulmonary sequestration underwent thoracoscopic resection. The pulmonary sequestration was identified and dissected free from surrounding tissue. The vascular peduncle was secured with an endo-GIA loaded with 2-mm vascular staples. The infant was discharged on postoperative day two without complications. In spite of the patient's small size and the apical nature of the sequestration, the operation was easily performed via a thoracoscopic approach. Thoracoscopic resection of an apical extrapulmonary sequestration in a small infant is feasible and may minimize the morbidity of this procedure.     

Pulmonary sequestration

The author discusses pulmonary sequestration which is a rare developmental anomaly difficult for diagnosis. Eleven patients were under observation and underwent operation for pulmonary sequestration (8 with intralobar and 3 with extralobar) which accounted for 2.4% of patients with congenital developmental anomalies of the lungs and for 0.48% of those with chronic unspecific disease of the lung. Complete clinical radiological examination, including aortography, made it possible to recognize the anomaly before the operation in 6 patients. In the remaining patients sequestration of the pulmonary tissue was an operative finding. Preoperative functional study of pulmonary circulation revealed a marked left-to-right shunt of the blood, increased oxygenation of blood in the superior vena cava and saturation of arterial and venous blood with oxygen (to 94 and 91%, respectively, on the average). Analysis of clinical, radiological, and morphological data allowed three main forms of pulmonary sequestration to be distinguished: cystic-abscessed, pseudotumorous, and fibrous-atelectatic.     

Pulmonary sequestration presenting as a prenatally detected suprarenal lesion in a neonate

An echogenic suprarenal mass was detected antenatally in the fetus by routine ultrasound scan. Resection of the mass after delivery confirmed an extralobar pulmonary sequestration with features of a type II congenital cystic adenomatoid malformation of the lung. The lesion should be considered in all neonates presenting with a suprarenal or upper abdominal mass particularly when the mass has been detected antenatally. Ultrasound-guided needle biopsy if feasible could provide a reasonably confident diagnosis. Congenital anomalies in other organs should be looked for carefully.     

Pulmonary sequestration. A report of an unusual case and a review of the literature

A case of extralobar sequestration with findings indicating a pulmonary origin is reported. The literature has been reviewed and evidence presented that intra- and extralobar sequestration are variants of the same abnormality and that the systemic artery is an associated anomaly. From the evidence available at present the pathogenesis of this anomaly is best explained as an abnormality of bronchial and foregut budding resulting from failure of normal embryonic organizer control.     

Diaphragmatic hernia associated with double pulmonary sequestration

The association of pulmonary sequestration with diaphragmatic hernia is an infrequent but well known situation. We report a rare case in which the diaphragmatic malformation was accompanied by two extralobar pulmonary sequestrations.     

Hybrid minimally invasive treatment of intralobar pulmonary sequestration: a single-centre experience

Pulmonary sequestrations are rare congenital malformations. They are often located in the lower lobes, and they are supplied by an aberrant systemic vessel arising from the thoracic aorta or abdominal arteries. These pulmonary malformations are divided into intra- and extralobar sequestrations, depending on the respective lack or presence of an independent pleural covering. Pulmonary sequestration can be asymptomatic or lead to recurrent pulmonary infections. The goal of this study was to analyse the feasibility and safety of a hybrid sequential approach. We report a small series of intralobar pulmonary sequestrations, from November 2017 to December 2018, successfully treated with a hybrid minimally invasive approach consisting of endovascular embolization of the aberrant arterial branch followed by video-assisted thoracoscopic lobectomy the day after. Thoracic pain following endovascular embolization was noted in all cases. Patients were discharged early in the absence of major postoperative complications. Prolonged air leak was observed in only 1 case. Despite the presence of sequestration-related pulmonary inflammation, in our experience, hybrid treatment for intralobar pulmonary sequestration is a safe and reproducible approach in terms of postoperative complications and hospital stay.     

Extralobar Pulmonary Sequestration Mimicking an Adrenal Tumor

Pulmonary sequestration is a rare cystic malformation composed of bronchopulmonary tissue that is discontinuous from the tracheobronchial tree and has an anomalous systemic blood supply. We present a case of a 40-y-old male who presented with an extralobar pulmonary sequestration and underwent a laparoscopic retroperitoneal mass excision. Preoperative imaging revealed a large 11.3-cm retroperitoneal tumor consisting of a multiloculated cystic lesion. The patient was discharged home, and at 3-mo follow-up no complaints were reported.