Spectrum of Pulmonary Sequestration

Bronchopulmonary sequestration was diagnosed in 17 patients ranging in age from newborn to 64 years. The sequestration was intralobar in 14 patients and extralobar in 3. The spectrum of symptoms could be divided into three patterns: no symptoms (6 patients), respiratory problems (8 patients), and cardiovascular problems (3 patients). Cardiovascular problems usually manifest themselves in the first few weeks or months of life and often have a respiratory component. In older patients the sequestration is first manifested by recurrent pulmonary infections or, if it remains uninfected, an asymptomatic density on chest roentgenogram.The definitive diagnostic study is arteriography. Operative treatment for the intralobar variety consists of segmental resection or, if the inflammatory process is more extensive, lobectomy. An extralobar sequestration may simply be excised.     

Diagnosis and management of congenital cystic disease of the lung in children

Pulmonary sequestration, congenital adenomatoid malformation (CAM), congenital lobar overinflation (CLO), and bronchogenic cysts are four congenital lesions that may present as abnormal cystic areas within the pleural cavity in early life. They share similar clinical and embryologic characteristics, are frequently difficult to diagnose, and all require surgical treatment. From December 1974 to January 1985, 22 patients were operated upon on the Pediatric Surgical Service for congenital cystic disease of the lung. There were eight females and 14 males, ranging in age from 1 day to 18 years; 12 were under 6 months of age, and 14 were under one year. There were 7 CAMs, 5 extralobar pulmonary sequestrations, 5 intralobar pulmonary sequestrations, 3 bronchogenic cysts, and 3 cystic lobes caused by CLO. One patient had bilateral pulmonary sequestrations, intralobar on one side and extralobar on the other, with both sharing a common systemic artery arising from the infradiaphragmatic aorta. All except two asymptomatic patients presented with either progressive respiratory distress or recurrent pulmonary infections, and had cystic changes noted on chest roentgenograms. The differentiation of these congenital lesions from resolvable inflammatory cysts is important. Diagnosis was aided by selective utilization of barium contrast studies, ultrasonography, computed tomography, and arteriography. Operation consisted of resection alone of the extralobar pulmonary sequestrations and bronchogenic cysts, and total lobectomy in CAM, intralobar sequestration, and CLO. One infant with CAM died 1 day postoperatively from bilateral hypoplastic lungs. The other 21 patients are alive and well with follow-up ranging from 1 month to 9 years, with a mean of 3.5 years.(ABSTRACT TRUNCATED AT 250 WORDS)     

Thoracoscopic treatment of pulmonary sequestration.

OBJECTIVE: Pulmonary sequestration is a rare congenital malformation and may be the cause of recurrent infections or hemoptysis. It has been shown in case reports that resection by video-assisted thoracic surgery (VATS) is feasible despite the possible technical difficulties due to inflammatory changes, but its role has not been evaluated yet in a larger series of consecutively treated patients. METHODS: Retrospective analysis of all consecutively thoracoscopically treated patients (between January 1991 and January 2005) with pulmonary sequestration in a single center. We included 14 patients in the study who fulfilled the criteria; seven were women. Median age was 33 years (20-64 years). The following data were analyzed for all patients: major symptoms, diagnostic procedures, treatment, and outcome. Operative parameters and findings including operating time, blood loss, anatomical location of the sequestration, and feeding vessels were evaluated. RESULTS: Leading symptoms were recurrent infections (10), hemoptysis (3), and chest discomfort (1). The diagnosis was made by CT scan. Additionally, an arteriography or an angio-MRI was done in three patients and one patient, respectively. Thirteen intralobar (all lower lobes, eight on the right) and one left-sided extralobar pulmonary sequestration were resected. We performed eight lobectomies, four atypical segmentectomies, one extralobar resection, and one occlusion of the aberrant artery. One case had to be converted to a thoracotomy due to bleeding from the aberrant artery. There was no mortality. Complications included pneumonia in three cases, one hemothorax, one pneumothorax after removing the chest tube, and one wound infection. All were treated conservatively. CONCLUSION: Thoracoscopic treatment of pulmonary sequestration is feasible in experienced hands. The aberrant systemic artery can be freed and dissected safely despite the frequently occurring inflammatory changes. Conversion rate to thoracotomy is low.     

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.     

Thoracoscopic resection of intra- and extralobar pulmonary sequestration in the first 3 months of life

Background

Thoracoscopic techniques have gained increasing acceptance in pediatric surgery, but experience with newborns and small children is limited. To our knowledge, a series of minimally invasive resection of pulmonary sequestration in newborns has not yet been reported in the literature. We report on 5 patients with pulmonary sequestration thoracoscopically.

Methods

From November 2000 to November 2002, 5 patients underwent thoracoscopic resection of pulmonary sequestration. Ages ranged from 4 to 91 days. Two patients had postnatal pulmonary symptoms. Preoperative diagnosis was dubious in 4 children. There were 4 extralobar and 1 intralobar pulmonary sequestrations.

Results

Thoracoscopy was performed with 3-mm instruments and 3 to 5 ports. All procedures were completed successfully. The median duration of the operation was 95 minutes (range, 63-117 minutes), and visualization was excellent. Anomalous blood vessels were clipped and/or ligated. Four patients were extubated immediately after the operation, 1, the day after. The postoperative course was uneventful in all children. At follow-up after 14 months (mean; range, 10-19 months), all patients were free of symptoms and had normal chest x-rays.

Conclusion

Thoracoscopy is feasible for resection of intra- and extralobar pulmonary sequestrations during the first 3 months of life.     

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 in a 4-months infant

Aim-Background

To highlight the rarity of a congenital condition of Pulmonary sequestration. This condition is presenting in two forms, intralobar and extralobar.

Case report

An extralobar sequestration in a 4-month-old infant is reported. A mass located in the left lower pulmonary lobe was diagnosed by a prenatal ultrasound examination at the sixth month of the pregnancy had set the possible diagnosis of cystic adenomatoid dysplasia of the left lower pulmonary lobe. No distress or other symptoms were observed. Surgical intervention with a left posterolateral thoracotomy over the fifth intercostal space allowed approach to the sequestration. The clinical presentation, differential diagnosis and therapeutic approach are discussed herein.

Conclusions

It is significant for the medical team to consider pulmonary sequestration in the differential diagnosis for any patient that presents with respiratory distress and a chest radiograph showing a mediastinal shift.     

Congenital cystic adenomatoid malformation associated with pulmonary sequestration

The association of congenital cystic adenomatoid malformation with pulmonary sequestration is infrequent. Has been reported less than 60 cases. We describe two patients with this association. One of this with extralobar pulmonary sequestration, and the other with an intralobar one.     

Intralobar pulmonary sequestration

For the period of 21 years the intralobar pulmonary sequestration was revealed in 6 patients aging from 11 to 47 years old. In all the observations of the intralobar sequestration the only affection of lower pulmonary lobes was noted. In 4 patients the symptomless course of the disease was present, while in 2 it was manifested by the recurrent pneumonia. The correct diagnosis before the operation was established in one patient only. All the patients were operated on, in 2 two aberrant vessels to the sequestrated pulmonary region were revealed and in all the patients--bronchogenic cyst in the affected region.     

Intralobar sequestration. A missed diagnosis

Intralobar pulmonary sequestration is an uncommon but distinct clinical entity that may be the unrecognized cause of recurrent pulmonary infections. Between 1967 and 1987, 10 patients, ranging in age from 5 to 39 years, were found to have an intralobar sequestration. Nine patients (90%) had a history of recurrent pulmonary infections, chronic cough, and intermittent fevers. One patient was asymptomatic. Many patients had been treated with antibiotics on numerous occasions. The delay in diagnosis varied between 3 months and 7 years (mean delay, 1.5 years). The chest roentgenogram was abnormal in all patients. The intralobar sequestration was present in the left lower lobe in 7 patients and the right lower lobe in 3 patients. Bronchography was abnormal in 4 patients in whom it was done. Bronchoscopy was performed in 7 patients, but it was only helpful in excluding other diagnoses. Preoperative thoracic arteriography in 9 patients visualized the systemic arterial supply from the thoracic or abdominal aorta to the intralobar sequestration and helped prevent any catastrophic surgical bleeding. A lobectomy was performed in 9 patients and a segmentectomy in 1 patient without morbidity or mortality. In patients with recurrent infections in the same lower lobe, a high index of suspicion for an intralobar sequestration should prompt early diagnostic arteriography and, if confirmed, early operative intervention.     

Late revelation of a subphrenic extralobar pulmonary sequestration as a suprarenal mass

Pulmonary sequestrations are some rare congenital anomalies. The incidence was estimated of 0.15% to 1.7%. They are characterized by a mass of non functioning pulmonary tissue that has no communication to the normal bronchial tree. The vascularisation is supplied by systemic arteries. They are classified further as intralobar and extralobar types. Extralobar sequestration, so-called accessory lung, is separated from the normal lung. We present a rare case of subphrenic extralobar pulmonary sequestration in a 57 years old patient. The lesion was initially presented as a non-typical suprarenal mass discovered on CT scan. The approach by laparatomy permitted the resection and the definitive diagnosis.     

Diagnosis and surgical treatment of pulmonary sequestration

The article reported 7 cases of pulmonary sequestration diagnosed by B-mode ultrasound and retrograde arteriography preoperatively, and was proved by operation. 6 cases were intralobar type & 1 case extralobar type. 7 cases underwent ultrasound examination and 6 cases retrograde arteriography before operation. The 7 patients underwent operation in which lobectomy was done in 6 cases and cystectomy in one. The clinical roentgenological and ultrasound characteristics and the key points of operative technique were discussed in the article. The author stressed that B-mode ultrasound could make an important contribution to preoperative diagnosis of pulmonary sequestration by demonstrating the nature of the mass and its vascular supply.     

Emergency surgical intervention after unsuccessful percutaneous transluminal angioplasty and stenting of aortic coarctation

Two adult patients were diagnosed with extralobar and intralobar pulmonary sequestration. One patient presented with haemoptysis. Both patients suffered from recurrent episodes of severe pulmonary infections. Both patients were treated by means of endovascular embolization using Amplatzer(?) vascular plugs (AVPs). They were discharged from hospital after 48 and 24 h and then followed up for 24 and six months, respectively. No recurrence of symptoms was observed. Computed tomography scans were obtained every six months. Persistent occlusion of vascular supply and moderate regression of the sequestered lung tissue are evident after 24 and six months in both patients. Just one case of an adult patient affected by pulmonary sequestration and treated by endovascular embolization has been reported to date. The present report is the first on the use of the AVPs in adults for this condition. The potential advantages and drawbacks of this treatment modality in adults are discussed, as well the specific benefit represented by the AVPs.     

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.     

Pulmonary sequestration: a comparison between pediatric and adult patients.

OBJECTIVE: Modern large single institutional reports on pulmonary sequestration (PS) are extremely rare. We were interested in comparing patients with PS referred by our pediatric versus adult pulmonologists. METHODS: Hospital notes of all patients operated on between 1978 and 1997 for a congenital broncho-pulmonary malformation were reviewed. In 28 patients, the parenchymal lesion was vascularized by a systemic artery and was separated from the bronchial tree, thus matching the strict definition of PS. Patient characteristics and outcome were analyzed comparing the pediatric group (< or =16 years: n=13; mean age, 3+/-5 years) versus the adult group (>16 years: n=15; mean age, 33+/-13 years). RESULTS: No significant differences between both groups were observed in sex, side, type of sequestration, pulmonary venous drainage, associated anomalies, hospital and late outcome, and patient's overall score. Patients (n=21) with the intralobar type of sequestration presented significantly more often with an infection when compared with patients (n=7) with the extralobar type (91 versus 14%; P=0.0033). When compared with the pediatric group, patients in the adult group had significantly more respiratory infections (87 versus 38%; P=0.016), and also required a lobectomy more often (67 versus 31%; P=0.056). CONCLUSIONS: The extralobar type of sequestration often remains asymptomatic, and is usually an incidental finding during infancy. The intralobar type mostly presents with recurrent infections in adulthood resulting in more lobectomies. We believe these findings support our current policy to remove any pulmonary malformation whenever diagnosed in order to: (1), prevent infection and other potentially serious late complications which may compromise the surgical outcome; and (2), enhance the chance of a parenchymal-sparing resection.     

Lung sequestration: report of seven cases and review of 540 published cases

We present an analysis of 400 intralobar and 133 extralobar sequestrations, six cases presenting intra- and extralobar sequestration simultaneously, five sequestrations of one whole lung, and two bilateral intralobar sequestrations.     

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.     

Distribution,diagnosis, and treatment of pulmonary sequestration: Report of 208 cases

ObjectiveThis study was performed to explore the clinical features, typing, distribution, and treatment of pulmonary sequestration (PS), with the aim of improving the awareness and treatment of this condition.MethodsClinical data regarding surgical procedures, outcomes, and prognosis of 208 pediatric patients with PS who were treated in our center from January 2005 to October 2017 were retrospectively analyzed.ResultsPS was confirmed by ultrasonography, enhanced computed tomography (CT), and/or magnetic resonance imaging (MRI) before surgery, and the surgeries were smoothly performed in all 208 patients (138 males, 70 females; age, 1 month to 14 years; mean age, 19.70 ± 48.82 months). The operative time ranged from 10 to 230 min (mean, 70 ± 48.75 min), and the intraoperative blood loss volume ranged from 1 to 200 ml (mean, 5 ± 18 ml). PS was located in multiple sites of the thoracic cavity and was also found in some rare locations such as the neck and abdomen. The feeding arteries of the PS mainly arose from the thoracic aorta or abdominal aorta, and a few of them originated from other vessels in the systemic circulation. The venous drainage differed between intralobar and extralobar PS: in patients with intralobar PS, the venous drainage was mainly via the pulmonary veins, especially the lower pulmonary veins; in patients with extralobar PS, the venous drainage was via the azygos vein and hemiazygos vein or reached the right atrium via the vena cava. The infection rate in children with intralobar sequestration was 71.17% (79/111), and that in children with extralobar sequestration was 31.37% (16/51).ConclusionPS has increasingly been detected by prenatal ultrasonography, and enhanced CT and MRI are the main techniques for diagnosing PS. Once confirmed, PS should be surgically resected. We choose an age of 6 to 12 months for surgical resection. Minimally invasive video-assisted thoracic surgery has many advantages in the treatment of PS and can be the treatment of choice for this condition.Type of studyTreatment Study.Level of evidenceLevel III.     

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.     

以咯血为主要症状的叶内型肺隔离症的栓塞治疗：4例报道及文献复习

目的探讨以咯血为主要症状的叶内型肺隔离症的血管造影特点及栓塞治疗效果,并复习相关文献。方法回顾性分析4例以顽固性咯血为主要症状的叶内型左下肺隔离症患者,咯血时间1周～4年,内科保守治疗无效,造影后采用明胶海绵、Cook弹簧圈或PVA颗粒对责任血管进行栓塞。结果 2例无左下肺组织实变者为单支异常动脉供血的动脉性肺隔离症,2例伴有左下肺组织实变者有3支异常动脉供血。栓塞后咯血即刻停止,随访2～8年,1例多支责任血管明胶海绵栓塞者2年后咯血复发。结论介入栓塞对以顽固性咯血为主要表现的叶内型肺隔离症的治疗是一种可供选择的方法,其远期效果尚有待观察。