Hidden in Plain Sight: A Rare Encounter With Pulmonary Sequestration

Intralobar pulmonary sequestration associated with an aneurysmal aberrant aortic branch

Subdiaphragmatic Extralobar Pulmonary Sequestration with Granulomatous Necrotizing Reaction Simulating a Tuberculous Infection: A Case Report

Pulmonary sequestration is a congenital lung malformation, defined by dysplastic and non-functioning lung tissue lacking normal tracheobronchial connections and accompanied by an anomalous systemic blood supply. Recognition of anomalous arteries and veins in pulmonary sequestration is paramount to making the correct diagnosis. In contrast to intralobar pulmonary sequestration, where anomalous venous drainage is usually into the pulmonary venous system, the pattern of anomalous venous drainage is more varied in extralobar pulmonary sequestration. To the best of our knowledge, anomalous venous drainage to the internal mammary vein in extralobar sequestrations has not been reported. We report an anomalous venous drainage into the internal mammary vein in an extralobar sequestration which was evaluated with 3D multidetector CT angiography.

ABSTRACTINTRODUCTIONCoexisting bilateral intralobar and extralobar pulmonary sequestration has rarely been reported, with no previous reports indicating that simultaneous resection is necessary because of a common drainage vein.CASE PRESENTATIONA 1-year-old boy was prenatally suspected to have right pulmonary sequestration. A preoperative contrast-enhanced CT scan revealed coexisting right intralobar and left extralobar pulmonary sequestrations. The left extralobar sequestration drained into the right inferior pulmonary vein, which was shared by the right intralobar sequestration. The patient had a history of infection with right intralobar pulmonary sequestration during infancy, so a right lower lobectomy was considered. Although an isolated right lower lobectomy would have resulted in congestion of the left sequestrated lung, simultaneous bilateral thoracoscopic surgery was performed. First, thoracoscopic resection of the left sequestrated lung and ligation of the right feeding artery of the right sequestrated lung were performed with right differential lung ventilation, followed by thoracoscopic right lower lobectomy with left differential lung ventilation after repositioning the patient. The intra- and postoperative courses were uneventful.CONCLUSIONSSimultaneous thoracoscopic surgery for bilateral pulmonary sequestration in infants with a common drainage vein is a reasonable strategy, as unilateral lesion resection may lead to postoperative complications.

Bronchopulmonary sequestration (BPS) is a rare congenital anomaly due to disturbed embryogenesis with a surplus budding of the lungs. BPS has no communication with the bronchial system. In most cases the blood supply of the sequestration is systemic via the aorta. There are two types of BPS: intralobar sequestration without its own pleural covering and extralobar sequestration with a separate pleura. They are clinically manifested in different ways. Sequestration normally occurs in the left lower lobe. The venous drainage often occurs via the pulmonary veins, producing a large shunt volume. The clinical symptoms are caused by recurrence of infection because of extensive therapy resistance, and rarely by haemoptysis.

<b>Introduction:</b> Pulmonary sequestration (PS) is a rare congenital pulmonary malformation, often found incidentally and can cause repeated respiratory infections. Aberrant vessels from the systemic circulation provide blood supply, commonly the descending aorta. PS can be intra-lobar (ILS) {more common} or extra-lobar (ELS). Outcomes from unresected PS is unknown. <b>Methods:</b> With Caldicott approval, a retrospective review of all CT scans performed between 2005 and Nov 2020 for the words ‘pulmonary or lung or intra-lobar or extra-lobar sequestration’ was performed.&nbsp; 56,247&nbsp;CTs were performed in total. 8 CTs were matched with the search words and 6 had PS. Basic demographics, clinical details and outcomes were collected. Descriptive statistical methodology was applied. <b>Results:</b> There were 4 male and 2 female patients. Mean age was 56.5 years (range 35-82). 3 were incidental findings and 3 were found after investigation for pain, haemoptysis and recurrent infections. 5 out of 6 were ILS and all of those were in the left lower lobe. The 1 ELS was in the right lower lobe. All had their blood supply from the descending aorta. 1 patient requested to have surgery and the ILS was excised with no complication. The 5 others did not require any intervention. 2 have died at the time of analysis due to unrelated medical issues. The incidence based on the total number of scans is 0.0084%. <b>Conclusions:</b> The above is a review of the incidence and outcome of PS in a large district general hospital in the North East of England. The results confirm the existing literature. We have reached out to regional hospitals so that a database can be built and encourage other centres to do the same. A crude incidence rate is 0.011% (11/100,000).

1. William T. Scouten, MD* 2. Jeanette White, MD† 1. *National Capital Consortium Pediatric Residency Program, National Naval Medical Center/Walter Reed Army Medical Center, Washington, DC 2. †Pediatric Critical Care, Children’s National Medical Center, Washington, DC A previously healthy 17-year-old girl complains of a “dry and intermittent” cough for the past 1.5 months. The cough worsens when she is recumbent and does not respond to over-the-counter cough medications. The cough is not associated with fever, chills, nausea, vomiting, night sweats, or weight loss. She reports several episodes of posttussive chest pain. Recently prescribed treatments of albuterol and fexofenadine do not alleviate her symptoms, despite her clinical diagnoses of exercise-induced bronchospasm and allergic rhinitis. She is an accomplished basketball player; her cough does not diminish her athletic performance. The patient’s past medical history is remarkable for a cardiac murmur noted at birth that resolved by 2 years of age and for three episodes of pneumonia by 6 months of age. She has no drug or environmental allergies, her immunizations are up-to-date, and her growth and development are normal. She denies drug, alcohol, or tobacco use. Further review of a psychosocial history reveals minimal adolescent risk characteristics. For the past 6 years, she has lived in the southwest United States and spent the prior summer in northern California. She denies exposure to others who had chronic cough, tuberculosis, or lung diseases. She admits to watching bats on one occasion during vacation in the past year. On physical examination, the patient appears healthy and is in no apparent distress. Vital signs are: temperature, 36.8°C (98.2°F); respiratory rate, 22 breaths/min; pulse, 88 beats/min; blood pressure, 117/73 mm Hg; and pulse oximetry, 100% hemoglobin-oxygen saturation while breathing room air. The patient’s respirations are unlabored, and she has adequate air movement. Auscultation of the chest reveals decreased breath sounds over the left base and fine, end-inspiratory crackles within the midposterior left chest. There is no appreciable egophony. The remainder of the physical findings are normal. A complete blood count reveals a white …

Paediatric torsed extralobar sequestration containing calcification: Imaging findings with pathological correlation

Intralobar pulmonary sequestration: A clinical and pathological spectrum

This discussion is concerned with the roentgen diagnosis of an anomalous systemic artery to the lung associated with congenital cystic pulmonary abnormalities. Seven cases proved by surgical resection and subsequent pathologic examination form the basis of the study. Although there are numerous reports in the literature by surgeons who have encountered anomalous arteries arising from either the thoracic or abdominal portions of the aorta and entering the lower lobes (1, 3, 4, 5, 7, 8, 11, 13, 16, 17, 18), very little reference has been made to the preoperative diagnosis, and particularly to the roentgen findings in these cases. Because a number of deaths have resulted from inadvertent section of such an artery during lobectomy or pneumonectomy (14), the vital importance of recognition of the condition prior to surgery is emphasized. The artery is “pulmonary” in type histologically, being elastic rather than muscular (19, 20) and is frequently athero-sclerotic (2, 8, 19). It may supply only normally connected lung, only sequestered lung, or both; it usually anastomoses with normal pulmonary arteries (6). In intralobar sequestration, venous drainage is to the normal pulmonary venous system; in extralobar sequestration, drainage is to systemic veins. The pulmonary abnormality, sequestration of lung, consists in the presence of lung tissue which does not connect with the bronchial tree in the usual fashion. Lung so sequestered may lie within the lower lobe (intralobar) (19) or adjacent to the lower lobe (extralobar) (10, 12, 19), forming an accessory lobe or lung. The cases presented here are all of the intralobar type. The extralobar type will not be discussed. The sequestered portion of the lung may be composed chiefly of normally differentiated lung, a system of branching bronchi, one or more bronchogenic cysts, or a polycystic mass. The fact that such cysts sometimes contain air suggests the probability of communication with the bronchial tree, although no communication was demonstrated in the cases presented. The embryologie events responsible for the anomalous distribution of the arteries and the accompanying deformity of the lung, as well as the frequent association of hiatus hernia, have been the subject of discussion by many authors, notably Cockayne and Gladstone (9) and Pryce (19). It has been the opinion of most writers that the condition is of congenital origin. Although it cannot be disproved in any case that the cyst or cysts have been acquired, this possibility is believed to be remote (15). In all of the cases presented, the pulmonary abnormality consisted essentially of bronchogenic cyst formation within a lower lobe. In none was there demonstration of any direct communication between abnormal lung and the bronchial tree of the involved lobe. In all instances the vascular anomaly consisted of a pulmonary artery arising from the thoracic aorta or from below the diaphragm.

Revising the classification of lung sequestrations

Pulmonary sequestration is a rare congenital anomaly of the lower respiratory tract, usually diagnosed in childhood. It involves lung parenchyma that has abnormal or absent communication with the tracheobronchial tree. The blood supply is not connected to the pulmonary circulation but comes from the systemic circulation. Intralobar pulmonary sequestration refers to pulmonary sequestration that shares a common pleura with normal parenchyma. In contrast, extralobar sequestration has its own visceral pleura that is not continuous with the normal lung. Extralobar pulmonary sequestration, unlike intralobar pulmonary sequestration, typically does not present with infectious manifestations due to the absence of a connection to the tracheobronchial tree. Instead, it may manifest as hypoxia, cardiac failure, or rarely, torsion of the sequestrum. However, it is more often an incidental asymptomatic finding. Computed tomography currently plays a leading role in diagnosis, enabling visualization of the feeding vessels. Surgical resection is the mainstay of the treatment, primarily to prevent hemorrhage or malignant transformation of the tissue. Resection also provides material for biopsy examination. A 68-year-old patient with nephropathy was referred by her nephrologist for abdominal ultrasound, which incidentally detected a tumor in the left retroperitoneum. A CT scan was performed, and the patient was then referred for tumor excision due to suspicion of a neurogenic tumor. Postoperatively, she was monitored in the intensive care unit with oxygen therapy for hypoxemia. The further course was uncomplicated. She was discharged home on postoperative day 5. We present a case of this very rare diagnosis, which is even rarer in an adult patient.

Bronchopulmonary sequestration is a rare congenital or acquired malformation of the lower respiratory tract. Sequestrations are classified anatomically as intralobar sequestration, located within the normal lobe, and extralobar sequestration, located outside the normal lobe and with its own visceral pleura. Pulmonary infection, mainly bacterial, is the most common complication of bronchopulmonary sequestration. Nocardiosis is characteristically thought as an opportunistic infection in immuno-compromised patients. Sex hormones can influence cell-mediated immunity and predispose to such infection. We report the case of a middle aged woman with a concurrent history of symptomatic endometriosis under long-term treatment with medroxyprogesterone acetate, and in whom an extralobar sequestration complicated by Nocardia asteroides infection was diagnosed by video-assisted thoracoscopic surgery.

Intralobar sequestration a missed diagnosis

Pulmonary sequestrations are non-functional masses of lung tissue lacking normal communication with the tracheobronchial tree and systemic-circulation-derived blood supply. The disconnection between the normal airway and pulmonary arterial supply prevents pulmonary sequestrations from participating in respiration while their aberrant circulation increases the potential for hemodynamic imbalance. Extralobar sequestrations are prenatally diagnosed, congenital lesions that may become symptomatic before or after birth, whereas intralobar sequestrations are usually identified during adulthood. This report describes a 2-month-old boy with respiratory distress caused by an anomaly characterized by a systemic artery feeding a large segment of the left lower lobe lacking normal ventilation due to absent bronchial connections. This segment that was surrounded by its own visceral pleura, and separated from the normal lower lobe by a fissure, drained through a large vessel into the left inferior pulmonary vein, causing left-to-left shunting and high output failure. Symptoms disappeared immediately following thoracoscopic division of the aberrant vessels and excision of only the sequestered segment. This case accentuates the hemodynamic imbalance set off by a left-to-left shunt across an atypical sequestration that although intralobar was separated by an accessory fissure from the normal parenchyma and also the benefits of video-assisted thoracoscopic surgery.