Secuestro pulmonar: caracterización y tratamiento en pacientes adultos y pediátricos / Pulmonary sequestration: characteristics and treatment in adult and pediatric patients

Resumen Introducción: El secuestro pulmonar (SP) es una malformación congénita caracterizada por tejido pulmonar con vascularización de una arteria sistémica anómala. Objetivo: Analizar las características y tratamiento de pacientes adultos y pediátricos con secuestro pulmonar. Materiales y Método: Estudio descriptivo transversal. Periodo: enero de 1988 a diciembre de 2018. La información se obtuvo de fichas clínicas y registros de anatomía patológica. Se describen edad, sexo, características clínicas, diagnóstico, tratamiento quirúrgico y hallazgos anatomopatológicos. Se realizó análisis estadístico mediante SPSS25® y se usó la prueba Mann-Whitney y X2, considerándose significativo p < 0,05. Resultados: Total 33 pacientes, 25 (75,8%) mujeres. Edad promedio 30,2 años, rango: 0-68. Adultos 23 (69,7%) pacientes y pediátricos (< 15 años) 10 (30,3%) pacientes. La presentación clínica fue sintomatología pulmonar en 23 (69,7%) casos y 9 (27,3%) eran asintomáticos. Tres (9,1%) presentaron malformación congénita asociada. Diagnóstico preoperatorio en 15 (45,5%) pacientes. La ubicación más frecuente fue lóbulo inferior izquierdo. El tipo intralobar fue el más frecuente en 23 (69,7%) casos. La cirugía más frecuente fue la lobectomía con identificación y ligadura del vaso sistémico. El vaso aberrante se originó en aorta torácica en 27 (81,8%) casos e infradiafragmático (no precisado) en 3 (9,1%) casos. Vaso único en 26 (78,8%) y doble en 5 (15,2%) casos. No hubo mortalidad. Existen diferencias en las características entre los secuestros en pacientes adultos y pediátricos. Discusión y Conclusión: Los SP son infrecuentes, se presentan principalmente en adultos jóvenes como neumopatías a repetición, se distinguen diferencias en las características entre los pacientes adultos y pediátricos, y tienen excelente pronóstico posoperatorio.

Background: Pulmonary sequestration (PS) is a congenital malformation characterized by lung tissue with vascularization from anomalous systemic arteries. Aim: To analyze characteristics and treatment of adult and pediatric patients with pulmonary sequestration. Materials and Method: Transversal descriptive study. Period: January-1988 to December-2018. Information was obtained from clinical files and pathological anatomy records. Age, sex, clinical characteristics, diagnosis, surgical treatment and pathological findings are described. Statistical analysis was performed using SPSS25® and the Mann-Whitney and Chi square test were used, considering p < 0.05 to be significant. Results: Total 33 patients, 25 (75.8%) women. Average age 30.2 years, range: 0-68. Adults 23 (69.7%) patients and pediatric (< 15 years) 10 (30.3%) patients. The clinical presentation was pulmonary symptoms in 23 (69.7%) cases and 9 (27.3%) were asymptomatic. Three (9.1%) presented another congenital malformation. Preoperative diagnosis in 15 (48.4%) patients. The most frequent location was the left lower lobe. The intralobar type was the most frequent: 23 (69.7%) cases. The most frequent surgery was lobectomy with identification and ligation of the systemic vessel. The systemic vessel originated in the thoracic aorta in 27 (81.8%) cases and infradiaphragmatic (not specified) in 3 (9.1%) cases. Single vessel in 26 (78.8%) and double in 5 (15.2%) cases. There was no mortality. Differences were found in characteristics between adult and pediatric patients. Conclusion: SP are infrequent, they mostly appear in young adults as recurrent lung diseases, differences in characteristics are distinguished between adult and pediatric patients and they have an excellent postoperative prognosis.

A diver's dilemma - a case report on bronchopulmonary sequestration.

BACKGROUND: An asymptomatic SCUBA (Self-contained underwater breathing apparatus) diver was discovered to have an intralobar bronchopulmonary sequestration during routine pre-course screening. This is the first reported case of a diver who, having previously completed several recreational and military diving courses, was subsequently diagnosed with a congenital lung condition, possibly contraindicating diving. Presently, there is no available literature providing guidance on the diving fitness of patients with such a condition. CASE PRESENTATION: An asymptomatic 26-year-old male diver was nominated to attend an overseas naval diving course. Prior to this, he had been medically certified to participate in, and had successfully completed other military and recreational diving courses. He had also completed several hyperbaric dives up to a depth of 50 m and 45 recreational dives up to a depth of 30 m. He did not have a history of diving-related injuries or complications. He had never smoked and did not have any medical or congenital conditions, specifically recurrent respiratory infections. As part of pre-course screening requirements, a lateral Chest X-ray was performed, which revealed a left lower lobe pulmonary nodule. This was subsequently diagnosed as a cavitatory left lower lobe intralobar bronchopulmonary sequestration on Computed Tomography Thorax. The diver remains asymptomatic and well at the time of writing and has been accepted to participate in another overseas course involving only dry diving in a hyperbaric chamber, with no prerequisites for him to undergo surgery. CONCLUSION: Although bronchopulmonary sequestrations lack communication with the tracheobronchial tree, they may still contain pockets of air, even if not radiologically visible. This can be attributed to anomalous connections which link them to other bronchi, lung parenchyma and/or pores of Kohn. As such, there is a higher theoretical risk of pulmonary barotrauma during diving, leading to pneumothorax, pneumomediastinum, or cerebral arterial gas embolism. Taking these into consideration, the current clinical consensus is that bronchopulmonary sequestrations and all other air-containing lung parenchymal lesions should be regarded as contraindications to diving. Patients who have undergone definitive and uncomplicated surgical resection may be considered fit to dive.

Intralobar pulmonary sequestration with cystic degeneration mimicking a bronchogenic cyst in an elderly patient: A case report and literature review.

INTRODUCTION: Pulmonary sequestration (PS) is a rare congenital malformation defined as nonfunctioning lung tissue supplied by systemic circulation. It is uncommonly diagnosed in adults. Herein, we describe a clinical case of PS with cystic degeneration mimicking a bronchogenic cyst in an elderly patient. PATIENT CONCERNS: A huge cystic mass was incidentally found in a 65-year-old man on chest computed tomography (CT) scans during preoperative workup for a hand laceration. A 15-cm-sized round cystic mass was detected in the right lower lobe. DIAGNOSIS: After reviewing the chest CT scan, we decided to perform contrast-enhanced chest magnetic resonance imaging (MRI) and CT-guided lung aspiration biopsy. On MRI, the lesion had the appearance of a cystic mass with hemorrhagic clots, such as an intrapulmonary bronchogenic cyst. The aspirated specimen was nondiagnostic; thus, we decided to surgically remove the mass. INTERVENTIONS: Upon right lower lobectomy, the mass was diagnosed as a PS. A thin systemic artery supplying the cystic mass was visualized during surgery. OUTCOMES: The patient is undergoing regular follow-up at the outpatient clinic. CONCLUSIONS: PS should be considered as a differential diagnosis in patients with a cystic lung mass. Identification of a systemic artery on radiologic imaging is important in the diagnosis of PS before preoperative workup to prevent unpredicted massive bleeding during surgery.

Extralobar Sequestration Complicated by a Cystic Hamartoma in an Adult.

A 49-year-old woman presented after a respiratory infection with an abnormal chest roentgenogram demonstrating a cystic calcified mass at the base of the right lung. A chest computed tomographic angiogram demonstrated that the blood supply arose from the abdominal aorta. This extralobar sequestration was surgically resected using video-assisted thoracoscopy without complication. The pathology report showed a cystic hamartoma. This case highlights the importance of preoperative evaluation of the blood supply of suspected sequestrations and the very rare disorder that was found.

Bronchopulmonary sequestration: Improving practice by evaluating for a missed diagnosis.

Bronchopulmonary sequestration (BPS) is a lung mass that does not communicate with the tracheobronchial tree or the pulmonary arterial vasculature, and thus does not play a role in oxygenation. This article discusses the etiology of BPS, as well as its pathophysiology, signs and symptoms, imaging studies used to diagnose, and treatment options in both pediatric and adult patients.

[Management of the Therapy of Pulmonary Sequestration: A Retrospective Multicentre Study]. / Management in der Therapie adulter Lungensequester - eine retrospektive multizentrische Analyse.

BACKGROUND: Pulmonary sequestration is a rare pulmonary malformation whose complex pathogenesis is not completely understood. Extrapulmonary sequestrations are always hereditary malformations and are usually diagnosed during childhood. Some intralobar sequestrations in adults, by contrast, seem to be acquired. The clinical presentation is non-specific and often misinterpreted, which results in delayed diagnosis. Surgical resection continues to be the gold standard. Despite the low incidence, new technologies developed in the past few decades, e.g. preoperative interventional angiology procedures and video-assisted lung resection, have changed the management of the disease. METHODS: A prospective data collection was performed on adult patients who had undergone surgical resection of a pulmonary sequestration in four different centres during a period of 23 years. These data were retrospectively analysed. RESULTS: A total of 14 patients with intralobar sequestrations (n = 11, 79 % left lower lobe) underwent surgical resection. The male/female ratio was 8/6 (median age 50 years). Non-specific pulmonary symptoms such as recurrent infections had a high prevalence (n = 6, 42 %). Two patients had haemoptysis. Three patients were asymptomatic. All patients had a chest CT, 7 patients had an additional abdominal CT, while 10 underwent angiography. Preoperative embolization was performed in 5 of these patients (branches of the thoracic aorta: in 2 cases, branches of abdominal aorta: in 2 cases, and branches of both: in 1 case). Resection was mostly performed with an open surgical approach (n = 12, 86 %). Most patients had a non-anatomic pulmonary resection (n = 8, 57 %). In 7 patients, a microbiologic examination revealed a superinfection with Streptococcus pneumoniae, while 1 patient had an invasive mycosis with Candida albicans. CONCLUSION: In patients with non-specific pulmonary symptoms and undetermined lesions, especially in the lower lobes, pulmonary sequestrations must be considered in the differential diagnostic evaluation even in adult and elderly patients. If there are no contraindications, surgical removal is basically recommended and may be minimally-invasive in selected cases. The planning of the resection can be facilitated by preoperative evaluation of the arterial supply (branches of the thoracic or abdominal aorta, or both). In cases with haemoptysis or blood supply over the abdominal aorta, preoperative embolization is indicated.

[Pulmonary Sequestration Associated with Increased Serum Tumor Markers;Report of a Case].

A 51-year-old woman visited our hospital with chief complaints of cough and fever. A chest X-ray detected an abnormal shadow in the right lung field. A chest computed tomography scan showed solid consolidation at S10 of the right lung. A blood test revealed elevated levels of the tumor markers, CEA(12.1 ng/ml), SLX (134 U/ml) and CA19-9 (76.2 U/ml). Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed abnormally increased 18F-FDG uptake with an SUV max of 11.29. Lung cancer was strongly suspected, and the surgery was performed. Abnormal blood vessels were found within the pulmonary ligament. Intraoperative rapid pathology indicated no malignancy, and the final diagnosis was pulmonary sequestration.

[Scimitar syndrome: about a case and review of the literature]. / Syndrome de cimeterre: à propos d'un cas et revue de la literature.

Scimitar syndrome or veino-lobaire syndrome (term coined by Felson) is a very rare disease characterized by the combination of cardiopulmonary anomalies, in particular an anomalous right pulmonary venous return, located mostly in the inferior vena cava. We here report the original case of a 6-month-old female infant presenting with acute dyspnea. The diagnosis was suspected on the basis of thoracic radiograph and was confirmed by tomodensitometry which showed a large single right pulmonary vein draining into the right atrium associated with dextrocardia and pulmonary sequestration. The prognosis was based on the size of the left-right shunt and related malformations.

Congenital cystic lesions of the lung.

Congenital cystic lesions of the lung are present in 1 in 10,000-35,000 births and present as a spectrum of anomalies. Majority of these cystic lesions comprise congenital cystic adenomatoid malformations, pulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts. Most of these lesions are nowadays detected antenatally, however some will present either in the newborn or during later childhood. A review of the aetiology, classification, natural history, investigations, and treatment of congenital cystic lung lesions is discussed.

Bilateral intralobar sequestration of the lung with a bridging isthmus: pathologic and radiologic findings.

A 4-year-old boy presented with pneumonia. Computed tomography demonstrated a multicystic mass at the posteromedial bilateral lower lobe segments, which were connected by a bronchus behind the heart. Enhanced computed tomography revealed that an anomalous artery arose from the left gastric artery and inserted into the left sequestrated lung and branched to the right one. The diagnosis of bilateral intralobar sequestration (ILS) with a bridging isthmus was made. After removal of the bilateral ILS, radiologic and pathologic approaches were undertaken to reconstruct the vascular and bronchial architectures. The following observations were made: (1) histologically, the region near the anomalous artery insertion site contained bronchial structures, which looked like an ectopic pulmonary hilus. This bronchial structure was continuously observed in the isthmus and its opening of the right sequestrated lung; (2) radiologically, the shape and course of the bronchi within the ILS indicated a distinct bronchial origin that arose from the pulmonary hilus-like structure, and the anomalous artery that ran along with those bronchi, resembled a pulmonary artery. These features suggested that this bilateral ILS might have originated from an accessory lung tissue.

Incidental finding and management of intralobar sequestration of the lung in a 24-year-old man.

Pulmonary sequestration is described as a dysplastic mass of lung tissue that lacks communication with the tracheobronchial tree and receives systemic rather than pulmonary arterial blood supply. Two distinct classifications, intralobar and extralobar, have been described. The present article discusses the etiology, clinical and radiographic features of pulmonary sequestration as well as the management of this condition when it is discovered incidentally.

Massive hemoptysis and hemothorax: a rare but fatal complication of intralobar sequestration.

Intralobar sequestration (ILS) is an uncommon abnormality that accounts for 75% of all pulmonary sequestrations. Over the years there have been several reports of various presenting signs of which hemoptysis was commonly described, however, massive hemoptysis and hemothorax is extremely rare in literature. We present a case of a 45-year-old man who died of fatal complication from an ILS. This case report shows an uncommon presentation of ILS with massive hemoptysis and hemothorax resulting in a dramatic course of disease and a fatal outcome, and for this reason in the absence of trauma or other causes for massive hemoptysis, hemothorax, or lung hematoma, this possibility should be kept in mind so as to avoid misdiagnosis, and resection of the sequestered tissue should be considered in all patients.