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.

[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.

Left diaphragmatic eventration associated with ipsilateral pulmonary sequestration and intrathoracic kidney in a fetus: reviewing the prenatal diagnosis and etiopathogenesis.

The prenatal diagnosis of congenital diaphragmatic eventration, pulmonary extralobar sequestration and intrathoracic ectopic kidney in a single fetus is not reported yet according to the pubmed. Congenital diaphragmatic eventration is an abnormal elevation of the diaphragm. Differential diagnosis from hernia is essential for the perinatal management. Extralobar sequestrations are usually asymptomatic and detected incidentally. Intrathoracic kidney is an extremely rare congenital anomaly. Genitourinary and cardiac anomalies should be searched as common co-existing malformations. Besides prenatal ultrasound, fetal magnetic resonance imaging has a substantial support in counselling the family, planning the follow-up of the pregnancy and decision-making for the perinatal management.

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.

Intralobar sequestration associated with cystic adenomatoid malformation: diagnostic and thoracoscopic pitfalls.

BACKGROUND: Intralobar sequestration (ILS) associated with congenital cystic adenomatoid malformation (CCAM) is a well-known entity. This hybrid form has many considerations for an appropriate management. This report aims to analyze pitfalls in the diagnosis, treatment, and thoracoscopic procedure pertaining to this association. METHODS: Between May 2004 and September 2007, 23 fetal lung lesions were diagnosed prenatally and managed at the authors' institution. Seven of these lesions were the hybrid form of ILS-CCAM. The records for these lesions were reviewed retrospectively. RESULTS: In all seven fetuses, prenatal ultrasound diagnosed congenital lung abnormalities. In three cases, fetal magnetic resonance imaging (MRI) demonstrated a macrocystic lesion supplied by an aberrant vessel. In all cases, the intrauterine evolution remained uncomplicated. All the newborns were asymptomatic at birth. Chest computed tomography (CT) performed at the age of 1 month showed the aberrant vessel in five cases. A regression of the lesion was noted in one patient and that of the aberrant vessel in another patient. Elective thoracoscopic lobectomy of the affected lobe was performed for six patients. The average age at the time of intervention was 5 months. At this writing, one patient is under observation due to resolution of the lesion. The operating time ranged from 80 to 160 min. One conversion to thoracotomy was required. All surgically treated patients had an uneventful hospital course. Histopathologic examination confirmed the association of intralobar sequestration with Stocker type 2 CCAM in five cases and with CCAM type 1 in one case. CONCLUSIONS: Prenatal diagnosis of the ILS-CCAM association was possible, as was resolution of one or both components of this lesion. Preoperative identification of the aberrant vessel is important for prevention of operative morbidity. This should be controlled before the lobectomy is begun.

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.

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.

[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.

Secuestro pulmonar en varón joven con episodio de hemoptisis / Pulmonary sequestration in a young man with hemoptisis episode

Se describe el caso de un paciente de 37 años, que a raíz de un episodio de hemoptisis diagnosticamos un secuestro pulmonar, malformación congénita infrecuente del pulmón, diagnosticada principalmente durante los primeros años de vida. Habitualmente cursa de manera asintomática, pudiendo debutar de manera puntual en forma de hemoptisis en la edad adulta (AU)

We describe a case of a 37-year-old patient who, as a result of an episode of hemoptisis, we find out a lung malformation, a rare cause of adult hemoptisis. Pulmonary sequestration is a rare congenital malformation of the lung, diagnosed mainly during the early years of life and childhood, which usually takes place asymptomatically, but which can start on time in the form of hemoptisis in adulthood (AU)

Intralobular bronchopulmonary sequestrations associated with bronchogenic cysts.

We present three cases of intralobar bronchopulmonary sequestrations with associated congenital bronchogenic cysts. As congenital abnormalities tend to be found together, these cases question the notion that intralobar sequestrations only occur secondary to chronic inflammation or infection, and suggest they can be congenital lesions.

Pulmonary intralobar sequestration in a patient with cystic fibrosis.

We report a case in which pulmonary Intralobar Sequestration (ILS) was an incidental finding at autopsy in an adult with Cystic Fibrosis. Two aberrant arteries from the descending thoracic aorta supplied a bronchial cystic lesion in the right lower lobe. Termination of the segmental bronchus and scar formation proximal to the cyst suggested prior bronchial obliteration. The elastic configuration of the aberrant aortic-derived vessels of the sequestration contrasted sharply with massively hypertrophied, muscular, bronchial arteries which supplied the bronchiectatic upper lobe. Sections of inferior pulmonary ligament were studied in five additional patients with CF but without ILS. Small muscular arteries were consistently noted within the inferior pulmonary ligament. These histologic findings support the concept that the vascular portion of ILS is congenital, whereas the bronchocystic component, in some cases, may be acquired.

Extralobar sequestration presenting as massive hemothorax.

Pulmonary extralobar sequestration is a rare anomaly, usually diagnosed during the first months of life. A case of extralobar pulmonary sequestration in an adult, manifesting itself as massive hemothorax, is presented.

A study of pulmonary ligament arteries. Relationship to intralobar pulmonary sequestration.

Intralobar pulmonary sequestration (ILS) has generally been considered a congenital malformation, mainly because of the presence of one or more systemic arteries to the sequestered portion of lung. We performed a study of the pulmonary ligaments in children without congenital pulmonary or vascular disease that demonstrated systemic arteries in ten of 11 cases, with as many as five arteries present in a single case. These arteries arose from the thoracic aorta, contributed to the esophageal plexus, and traversed the pulmonary ligament to ramify in the visceral pleura. A sequence of events including bronchial obstruction, pneumonia, pulmonary artery occlusion, pleuritis, and parasitization of pulmonary ligament (or diaphragmatic) arteries leading to the "creation" of an ILS is proposed.

Intralobar pulmonary sequestration: a clinical and pathological spectrum.

Pulmonary sequestration is a mass of abnormal pulmonary tissue that does not communicate with the tracheobronchial tree and is supplied by an anomalous systemic artery. Whereas extralobar sequestration is clearly congenital, intralobar sequestration, which frequently presents in older children with pathological findings showing acute and chronic inflammation, may have an acquired origin secondary to frequent infections. Several large autopsy series support an acquired etiology of intralobar sequestration. Four cases of intralobar sequestration are presented that demonstrate a spectrum of inflammatory change that support its congenital, rather than acquired origin. Case 1 was a newborn who presented with tachypnea and a right lower lobe density. Resection at 3 weeks of age showed no inflammation in the sequestration specimen. Case 2 presented as a newborn infant with congestive heart failure. Pulmonary sequestration was confirmed by arteriogram. Resection at 3 months of age showed chronic inflammation. Case 3 presented at 7 months of age with chronic pneumonia. The resected specimen demonstrated moderately severe acute and chronic inflammation. Case 4 presented as a 6 year old. The operative specimen showed extensive bronchiectatic changes with marked acute and chronic inflammation. These cases support the congenital origin of intralobar sequestration and suggest a temporal progression from no inflammation to severe acute and chronic inflammation.

Acute chest syndrome in the postoperative sickle cell patient.

BACKGROUND/PURPOSE: Acute chest syndrome (ACS), a phenomenon of pulmonary sequestration in sickle cell disease (SCD) patients, is frequently missed in the postoperative SCD child. The constellation of symptoms range from fever and respiratory distress to abdominal discomfort. In its most fulminate state, the syndrome has been reported in some series to carry almost a 25% to 50% mortality rate in the postoperative patient. The incidence in pediatric patients in the era of minimally invasive surgery is unknown. METHODS: Since December 1995, 63 episodes of ACS have been documented in the nearly 500 SCD children seen at our institution. Six of 63 episodes occurred within 2 weeks after a surgical procedure under general anesthesia. During this period, 59 operations were performed by the pediatric surgery service on SCD patients with an ACS incidence of 10.2%. Careful review of the preoperative, intraoperative, and postoperative management of these patients was performed. RESULTS: All six received preoperative oxygen saturation monitoring and intravenous fluid (IVF) hydration. One half of these patients required transfusion to achieve a hemoglobin level of greater than 10 mg/dL. Documentation of intraoperative temperature, hypoxia, volume status, and hypercarbia as well as any atypical perioperative events were monitored and reviewed. All patients received postoperative oxygen supplementation and IVF hydration. Onset of ACS ranged from 1 hour to 7 days postoperatively. Only one of six was thought to be of microbial etiology (elevated mycoplasma titers), and all patients received prophylactic antibiotic and aggressive pulmonary therapy. Overall length of hospitalization was increased with an average stay of 6.1 days. There were no postsurgical ACS deaths. CONCLUSIONS: Despite close attention and avoidance of known risk factors for development of postoperative SCD complications, ACS occurred with an incidence much higher than previously reported in the literature (0.4% v 10.2%). Interestingly, five of six cases were after laparoscopic procedures suggesting that the advantages of laparoscopy, such as reduced postoperative pain, do not extrapolate to decreased incidence of ACS.

Pneumonectomy in infants and children: the use of a prosthesis to prevent mediastinal shift and its complications.

Twenty cases of pneumonectomy in infants and children performed over a 35-yr period have been reviewed. Pneumonectomy may be necessary for inflammatory, congenital, neoplastic, and traumatic conditions. The postoperative complications of progressive mediastinal shift can be minimized by the use of a pleural prosthesis.