Pulmonary sequestration. A broad spectrum of bronchopulmonary foregut abnormalities.

We believe the broad clinical, embryological, and radiological spectrum of pulmonary sequestration has not been adequately emphasized. In order to gain clearer understanding of these foregut abnormalities, all cases from the files of three Army Medical Centers were reviewed. Thirty-two patients, the largest single series in the literature, met the criteria for a diagnosis of bronchopulmonary sequestration. Clinical manifestations varied from no symptoms to recurrent pulmonary infection, hemoptysis, and intrapleural hemorrhage. The presence of symptoms strongly correlated (23/32) with air-containing cystic sequestrations. The embryological and radiological spectra as well as appropriate operative therapy are reviewed.     

肺隔离症4例诊治体会及文献复习

目的 探讨肺隔离症的诊断和治疗策略。方法 2017年7月至2019年6月收治的4例肺隔离症患者,3例患者行胸腔镜手术治疗,1例行介入下血管塞封堵迷走供血动脉,对其诊治过程和临床资料进行回顾性分析,总结诊治过程中的体会。结果 4例肺隔离症患者均接受治疗,1例患者接受介入下血管塞封堵迷走供血动脉后恢复顺利;1例患者胸腔镜下肺叶切除术后恢复顺利;2例患者接受胸腔镜下隔离肺叶切除术,其中1例恢复顺利,1例因胸腔进行性出血行胸腔镜下开胸止血术,后恢复顺利。术后3月复查胸部螺旋计算机体层摄影血管造影显示,3例行胸腔镜下隔离肺叶或肺叶切除手术患者的异常肺叶及供血动脉消失,行介入下血管塞封堵异常血管的1例患者的迷走供血动脉被栓塞,栓塞血管远端已无血流供应,隔离肺组织充血表现较前明显好转,4例患者随访7~31个月,未见复发。在手术时间、术中出血量、术后疼痛、术后胸腔闭式引流量、术后并发症、住院时间及住院费用等方面,介入栓塞治疗均优于胸腔镜手术治疗。结论 胸腔镜手术是目前处理肺隔离症的主要方式,介入栓塞治疗肺隔离症同样是一种安全、有效、微创的治疗方法,尤其对以咯血为主要症状,凝血功能异常且病情较重者效果佳。     

Pulmonary sequestration.

Pulmonary sequestration is a congenital anomaly in which an aberrant systemic artery arising from the thoracic or upper abdominal aorta supplies part of the lungs, usually the lower lobe. The sequestered lung may be anatomically distinct from the remainder of the lobe (extralobar), or may be included in the substance of the lobe, in which case it may or may not have bronchial communication with the rest of the bronchial tree. The patients present, often in the first two decades of life, with recurrent and severe bronchopulmonary infections. Associated anomalies are present, especially in the extralobar variety. Nine cases of sequestration are reviewed, stressing significant clinical, radiological, and arteriographic findings. Preoperative demonstration of the anomalous vessel by aortography has contributed significantly to the planning and safety of the surgical procedure, which was generally a lower lobectomy. Eight out of nine patients survived the procedure.     

Taussig-Bing畸形合并大动脉侧侧位的外科治疗

目的总结外科手术一期治疗Taussig—Bing畸形合并大动脉侧侧位的临床经验。方法2000年5月～2007年9月手术治疗26例（手术年龄3.1±2．2个月）Taussig—Bing畸形合并大动脉侧侧位患者，其中合并主动脉弓病变13例。25例患者采用动脉转位术（arterial switch operation，ASO），1例采用Kawashima手术治疗。合并主动脉弓病变的矫治方法主要是切除缩窄段后，将胸主动脉与主动脉弓下缘行端端吻合，或与升主动脉行端侧吻合。结果住院死亡3例（11.5％，3／26）。2005年6月以后连续手术治疗的15例Taussig—Bing畸形伴大动脉侧侧位，无住院死亡。随访21例，随访时间1～5年；患者生长发育均明显改善，心脏较术前缩小，肺血明显减少。2例患者因肺动脉瓣上狭窄而再次行手术修补。结论Taussig—Bing畸形合并大动脉侧侧位的病理解剖比较复杂，必须根据不同的解剖条件选择不同的手术方法，才能降低手术死亡率。     

Pulmonary sarcoidosis: alterations in bronchoalveolar lymphocytes and T cell subsets.

Peripheral blood and bronchoalveolar lavage lymphocyte subpopulations have been evaluated in 14 patients with pulmonary sarcoidosis and eight normal subjects, monoclonal antibodies of the leu series being used. No significant alterations of T lymphocyte subpopulations were found in the peripheral blood of sarcoidosis patients. There was, however, a significantly greater proportion of T suppressor-cytotoxic cells (36.0 (SD 17.6%] in the bronchoalveolar lavage fluid of patients than of normal subjects (15% (5.6%); p less than 0.01), but a decrease in the proportion of T helper-inducer cells (51.1% (18%) v 79.3% (9%). These changes correlated with the duration of the disease but not with other clinical, radiological, physiological, or biochemical criteria. Patients were followed up for six to 20 months and five patients had a repeat bronchoalveolar lavage and lymphocyte subpopulation evaluation after three to 14 months. The initial pulmonary T lymphocyte subset proportions were not predictive of clinical, physiological, or radiological alterations during follow up. There was also no consistent pattern in the relationship between change in T subset proportions and change in clinical physiological, and radiological features in the five patients having a repeat lavage. Lymphocyte surface marker studies may indicate immunopathogenetic mechanisms in sarcoidosis but do not appear to be good predictors of clinical outcome.     

Anomalous systemic arterial supply to normal basal segments of the left lower lobe.

BACKGROUND: Anomalous arterial supply to the normal basal segments of the lower lobe without sequestration is a rare congenital abnormality, and whether it belongs to the broad spectrum of sequestration disorders remains controversial. METHODS: The cases of all 4 patients who were treated surgically by us were reviewed together with 8 previously reported cases. RESULTS: The anomalous artery originated from the descending thoracic aorta, distributed to the basal segments of the left lower lobe, and drained to a normal inferior pulmonary vein in each case. The anomalous artery was thick and elastic walled. From the review of all 12 cases, male gender, left side, descending thoracic aorta as the aberrant arterial origin, absence of pulmonary blood flow to the basal segments, and normal pulmonary venous drainage were predominant. Despite some differences, the findings seemed closely related to intralobar sequestration. Surgical treatments were lung resection, anastomosis, and ligation of the anomalous artery. CONCLUSIONS: This anomaly is probably one type of sequestration complex. Both aortic and pulmonary arterial angiographic studies are needed to plan the definitive surgical procedure.     

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.     

单操作孔胸腔镜手术治疗肺隔离症的临床体会

目的 总结单操作孔胸腔镜手术治疗肺隔离症的临床经验,评价其安全性、有效性。方法 回顾性分析2015年1月至2019年5月共21例单操作孔胸腔镜手术治疗的肺隔离症患者临床资料,其中男性10例,女性11例,年龄21~57（39.5±9.2）岁,21名患者均为下肺病变,包括左下肺13例,右下肺8例;叶外型1例,叶内型20例,术前均行增强CT扫描和三维重建;双腔气管插管全麻下单操作孔胸腔镜行肺叶或肺段切除20例,中转开胸1例。结果 胸腔严重粘连者8例,行下肺叶切除术20例,下肺后基底段切除1例;手术均顺利完成,手术时间（167.3±61.5）min,术中出血量（157.3±30.2）mL,术后带管时间（5.3±1.6）天,术后住院时间（6.2±1.5）天,围手术期无死亡及严重并发症发生;随访21例无失访,随访时间7~59个月,21例患者复查胸片或CT无复发。结论 单操作孔胸腔镜手术治疗肺隔离症安全可行,术前合理的检查评估和术中对隔离肺异常血管的合理处理是手术成功的关键。     

Utility of preoperative visualization for intrapulmonary sequestration in video-assisted thoracoscopic surgery

We herein report the case of a 59-year-old male suffering from severe cough. The patient had previously experienced several episodes of pneumonia beginning in childhood. A three dimensional multidetector computed tomography (3D-MDCT) scan revealed pulmonary sequestration with a left gastric artery blood supply, and clearly revealed the other pulmonary vessels. The patient underwent video-assisted thoracoscopic surgery, in which the anomalous tissue was resected safely. Preoperative visualization of the vessels may be helpful for the safe treatment of a pulmonary sequestration, and the VATS approach is a suitable operation under corrective surgical planning.     

The rare congenital anomaly of pulmonary sequestration experience and review of literature

Objective  

Pulmonary sequestration is rare lung anomaly which had very major theories of origin. The aim of this study was to treat this rare congenital anomaly, pulmonary sequestration, which was not diagnosed of preoperatively, in our centre.     

The spectrum of pulmonary sequestration

An intralobar sequestration with the unusual anatomical finding of both pulmonary and systemic (azygos) venous drainage is presented in detail. A review of the literature pertaining to pulmonary sequestration revealed a continuum of lung anomalies in which nearly every combination of pulmonary and systemic arterial supply, pulmonary and systemic venous drainage, normal and abnormal pulmonary tissue, gastrointestinal fistula, and defective diaphragm was described. Since no single anatomical mechanism can account for all these anomalies, the spectrum can best be explained at this time as a defect or defects of morphogenesis in the embryonic thorax. The surgeon must be aware of this spectrum of anomalies in order to remain alert to the possibility of unusual blood vessels and gastrointestinal fistula during operation for any cystic or suppurative lesion of the lungs.     

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.     

肺隔离症（附14例报告）

目的 探讨肺隔离症（PS）的临床特点及X线特征，指导术前诊断及治疗方法。方法 回顾分析10年来14例肺隔离症患者资料。结果 14例患者术前误诊率高达71％（10／14），均经手术后病理证实，CT及X线的特征性表现可提高诊断率，手术治疗后均痊愈出院。结论 肺隔离症虽有相应的临床表现及X线特征，但术前鉴别诊断困难，手术治疗即可明确诊断，亦可获得满意疗效，手术切除应注意异常动脉的处理。     

Assessment of Blalock-Taussig shunts in children with complex cardiac anomalies associated with reduced pulmonary blood flow and total anomalous pulmonary venous drainage]

Eleven patients with syndromes of asplenia and polysplenia associated with total anomalous pulmonary venous drainage (TAPVD) were underwent Blalock-Taussig (B-T) shunt operation for reduction of pulmonary blood flow. The age of patients at operation were 6 days to 5 years (average 19.5 +/- 18.7 month). There were seven patients in supracardiac type and four in cardiac type of TAPVD. All patients didn't present obstruction to pulmonary venous return (PVO) before B-T shunt operation. There were one early (9%) and two late deaths (20%) after surgery. Although the hospital death was related to perioperative errors. Two late deaths were not due to the PVO. One of infants had moderately pulmonary congestion and cardiac failure after shunt procedure. Two patients were measured pressure gradient (3 to 4 mmHg) between common pulmonary vein to atrium chamber before shunt procedure. Repeated catheterization revealed that 6 of them could be measured pressure gradient, 3 to 7 mmHg, and no patients had clinical sign of the PVO. Our results demonstrated that B-T shunt operation could be satisfactory for syndromes of asplenia and polysplenia associated with reduced pulmonary blood flow and TAPVD.     

Intralobar pulmonary sequestration supplied by an anomalous aneurysmal artery

Pulmonary sequestration is a relatively rare condition in which a systemic artery supplies blood to an abnormal lung tissue. Pulmonary sequestration with an aneurysmal systemic artery is extremely rare. We describe the case of a 52-year-old man with intralobar pulmonary sequestration supplied by an aneurysmal systemic artery. Because the nomenclature of pulmonary sequestration is still not clear, we propose that type 1 intralobar pulmonary sequestration be called "systemic arterial supply to the normal lung," as named by many professionals, and for this to be distinguished from pulmonary sequestration.     

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.     

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.     

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.     

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.     

Video-assisted thoracic surgery for bronchopulmonary sequestration

Bronchopulmonary sequestration is a rare congenital lung anomaly for which surgical resection is the definitive treatment. Open thoracotomy is the conventional approach, yet associated with considerable morbidity. We report one of the largest series of major lung resection for bronchopulmonary sequestration using the video-assisted thoracic surgery (VATS) approach that could reduce such morbidity. Six cases of VATS anatomical lobectomy for intrapulmonary sequestration performed between January 1996 and January 2005 were reviewed. The six patients included two males and four females, with a mean age of 43.3 years (range: 27-64 years). Anatomical lobectomy without conversion to open was achieved in all cases. The mean operating time was 112.8 min (range: 90-140 min), the mean blood loss was 283.3 ml (range: 100-500 ml), and the mean length of post-operative hospital stay was 8.8 days (range: 7-24 days). There was no mortality. Three patients had minor wound infection. The results were comparable patients receiving lung resections for bronchopulmonary sequestration by an open approach. VATS major lung resection for bronchopulmonary sequestration is safe and feasible. Further studies are warranted to define the role of VATS in the management of bronchopulmonary sequestration.