Intra-abdominal pulmonary sequestration: a case report and literature review

Pulmonary sequestration is a rare congenital malformation mostly located in the thorax, while intra-abdominal pulmonary sequestration is an extremely rare type of pulmonary sequestration usually diagnosed during the first 6 months of life. Only 1 case of intra-abdominal pulmonary sequestration in a patient older than 60 years has been reported in the current literature. It is difficult to differentiate an intra-abdominal pulmonary sequestration from other retroperitoneal tumors. A definitive diagnosis is always made by histological examination. Intra-abdominal pulmonary sequestration commonly responds well to surgical resection and is associated with excellent results and prognosis. The authors present the case of a 74-year-old asymptomatic man with a retroperitoneal mass which was completely excised and revealed by histopathological study to be an intra-abdominal pulmonary sequestration.     

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.     

Intralobar pulmonary sequestration and mediastinal bronchogenic cyst.

A patient with a bronchogenic cyst and intralobar pulmonary sequestration is presented. Chest radiography showed a posterior mediastinal mass and a computed tomographic scan of the chest revealed, in addition, an abnormality suggestive of pulmonary sequestration. This was confirmed by an aortogram. It is important to be aware of the coexistence of these anomalies to make a diagnosis preoperatively.     

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。     

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.     

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.     

Surgical treatment of infected intralobar pulmonary sequestration: a collective review of patients older than 50 years reported in the literature.

We report on the rare and surgical treatment of a senile patient of infected intralobar pulmonary sequestration. A 56-year-old male who had complained of headache, vomiting, cough, sputum production, and high fever was admitted to our hospital. Chest computed tomography (CT) showed an infected intralobar pulmonary sequestration as an 8x6 cm cystic mass with multiple air-fluid cavities in the left lower basal segment and severe pneumonia in the left upper and lower lobes around the mass. A 3-D CT showed an aberrant artery entering the consolidation from the descending aorta. A standard lower lobectomy was performed with a ligation of the aberrant artery with a diameter of 1 cm supplying the posterior segment of the left lower lobe. A histological examination of the lung revealed acute and chronic broncho-bronchiolitis with cystic dilatation consistent with intralobar pulmonary sequestration. We discuss the characters of senile patients compared with juvenile patients, with reference to a collective review of patients older than 50 reported in the literature.     

A case of intralobar pulmonary sequestration associated with abnormal connection between the aberrant artery and the pulmonary artery

The patient was a 5-year-old girl and had a history of recurrent pneumonia. Angiogram demonstrated that the aberrant arteries arising from descending aorta communicated with left pulmonary A10 where the multi-lobulated cystic lesion was seen by chest X-P. The right heart catheterization revealed the step-up of oxygen saturation in distal part of left pulmonary artery A10. Diagnosis was the intralobar pulmonary sequestration with the aberrant arteries communicating with pulmonary artery. The connection between aberrant arteries and pulmonary artery in this case might be caused by the chronic inflammation in the sequestrated lung. We found 4 case reports in which the communication between aberrant arteries and pulmonary artery was evident.     

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.     

A case report of subdiaphragmatic pulmonary sequestration

A 32-year-old man with extralobar pulmonary sequestration under diaphragm is reported. The patient was admitted because of abnormal shadow on chest X-ray film without any complaints. Chest roentgenogram showed a homogenous density at the left cardiophrenic angle. Aortogram demonstrated an abnormal artery arising from the abdominal aorta to left subphrenic mass shadow. On laparotomy a large cystic mass connected with diaphragm was found behind the stomach and removed successfully. The postoperative course was uneventful. The histological diagnosis was subphrenic pulmonary sequestration. The frequency, localisation, pathogenesis of pulmonary sequestration are discussed.     

Pulmonary sequestration with myocardial ischemia caused by vasospasm and steal

We describe patient with a rare pulmonary sequestration with myocardial ischemia in the left coronary artery caused by vasospastic angina and stealing from coronary circulation. The anterior atrial branch from the left circumflex artery gave rise to a large and anomalous vessel in the right posterior lung field. Both the surgical and medical treatment of this pulmonary sequestration improved the control of angina attacks.     

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.     

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.     

Anomalous systemic arterial supply to the basal segment of the left lung; report of a case

A 35-year-old female was admitted to our hospital for hysteromyoma. Chest X-ray showed a mass shadow in the left lung field. A thorough examination revealed anomalous systemic arterial supply to the basal segment of the left lung with 2 abnormal arteries. Because the patient had no symptoms, no operation was performed. After 9 months however, the patient had bloody phlegm and chest pain and received an operation. Thus, we propose that the operation for the pulmonary sequestration is to be performed when such a diagnosis is made.     

Pulmonary sequestration complicated by anomalies of pulmonary venous return

Five anomalies of pulmonary venous drainage were seen among 12 children operated for lung sequestration. In two children, venous drainage from the sequestrated lobe and the rest of the right lung was via a single channel into the inferior vena cava ("scimitar syndrome"). In one of these children, the sequestrated lobe was resected and repair of the scimitar syndrome was delayed; in the second patient, the anomalous pulmonary venous drainage was not recognized preoperatively and the vein was ligated, resulting in acute hemorrhagic infarction of the right lung and death of the patient. Three patients had less severe anomalies of pulmonary venous drainage. We recommend very careful evaluation of patients with lung sequestration with special reference to pulmonary venous drainage.     

Simultaneous intra- and extralobar lung sequestration and its intermediate stages

We operated upon a patient with a simultaneous intra- and extrapulmonary sequestration in relationship to the same left lower lobe. The rarity of this form of pulmonary sequestration and its contribution to the comprehension of the pathogenesis of this malformation justifies the present publication. Most of the distinctive characteristics between the two different sequestration form found in the literature can be questioned. The unit of the lesion in our patient with the stalk connected intra- and extrapulmonary part, the identical histology, the arterial supply and venous drainage they have in common, points impressively to one single coherent structure - an organoid.     

Video-assisted thoracic resection for intralobar pulmonary sequestration

We present a case in which video-assisted thoracic resection for intralobar pulmonary sequestration (ILPS) was successfully performed. A 36-year-old woman had repeated pneumonia. Chest computed tomography (CT) showed a round mass in the right lower lobe of the lung. Subsequent three-dimensional CT revealed that a large anomalous artery arising from the descending thoracic aorta was distributing to the posterior basal segment containing the lesion and was draining into the inferior pulmonary vein. The patient was diagnosed with ILPS and underwent surgery. The anomalous artery was divided, and the sequestered segment was completely resected by video-assisted thoracic surgery (VATS). We think that VATS resection for ILPS is feasible and is a major therapeutic option as noninvasive surgery.     

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.     

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.     

Colonisation with Aspergillus of an intralobar pulmonary sequestration.

Pulmonary sequestration is a term used to describe an area of embryonic lung tissue supplied by an anomalous systemic artery. Two forms are recognised-extralobar and intralobar-with different clinical presentations. A patient is reported with intralobar pulmonary sequestration in the left lung and colonisation with Aspergillus which was successfully treated by lower lobectomy.