Pulmonary Sequestration

Bronchopulmonary sequestrations (BPSs) are rare congenital anomalies characterized by non-functioning embryonic lung tissue receiving anomalous blood supply. They are most commonly located within the thorax (supradiaphragmatic) or into the abdominal cavity (infradiaphragmatic). Intradiaphragmatic extralobar BPs (IDEPS) are an exceptionally rare finding, representing a diagnostic and operative challenge. We report three cases of IDEPS and their surgical management, describing our experience and approach to such rare clinical entities. From 2016 to 2022, we treated 3 cases of IDEPS. Surgical techniques, histopathological findings and clinical outcomes were retrospectively evaluated for each case and compared. Three different surgical techniques were used to approach each lesion, from open thoracotomy to a combined laparoscopic and thoracoscopic approach. Histopathological analysis of the specimens revealed hybrid pathological features, proper of both congenital pulmonary airway malformation (CPAM) and extralobar pulmonary sequestration. IDEPS represent a surgical challenge for pediatric surgeons, given their complex surgical planning. In our experience, the thoracoscopic approach is safe and feasible when performed by trained surgeons, even though a combined thoracoscopic-laparoscopic approach allows for optimal vessels control. The presence of CPAM elements within the lesions supports their surgical removal. Additional studies are required to better characterize IDEPS and their management.

O seqüestro pulmonar é um segmento ou cisto pulmonar com suprimento arterial anômalo proveniente, na maioria dos casos, da aorta torácica descendente. Suas manifestações clínicas mais comuns são decorrentes das freqüentes co-infecções bacterianas piogênicas, assemelhando-se a diversas supurações broncopulmonares. Os autores pretendem, com tal relato, mostrar a investigação de uma supuração pulmonar de evolução insidiosa, cujo diagnóstico foi de seqüestro pulmonar associado à tuberculose pulmonar.

Extralobar pulmonary sequestrations (ELS) are masses of non-functioning lung tissue that are supplied by an anomalous systemic artery and do not have a bronchial connection to the native tracheobronchial tree. On prenatal ultrasonography, an ELS appears as a well-defined echodense, homogeneous mass. Detection by color flow Doppler ultrasonography of a systemic artery from the aorta to the fetal lung lesion is a pathognomonic feature of fetal ELS. MR imaging may help in the diagnosis of pulmonary sequestration by demonstrating a solid, well-defined mass, and the feeding artery. In this case report, we describe the sonographic and MR diagnosis of an ELS in a fetus at 22 weeks gestation with a review of the available literature.

We present a child with a rare finding of an extralobar pulmonary sequestration localized in the upper mediastinum. Findings of the prenatal screening enabled early postnatal diagnostic measures that revealed a heterogeneous mass situated next to the thymus. Based on the localization and inconclusive computed tomography images, the preoperative prediagnoses included enterogenic cyst, thymus cyst and teratoma. Intraoperative features of the vascular supply and structure of the mass drew the surgeons' suspicion to extralobar pulmonary sequestration, which was confirmed in the histopathological examination. The scans were reevaluated after the surgery. Surgical management was implemented prior to the occurrence of any symptoms, which led to a positive general outcome. The presented case should raise the awareness of radiologists, pediatric surgeons and other consultants involved in the diagnostic process of mediastinal lesions in children. The rare localization and lack of visualiz...

We present a child with a rare finding of an extralobar pulmonary sequestration localized in the upper mediastinum. Findings of the prenatal screening enabled early postnatal diagnostic measures that revealed a heterogeneous mass situated next to the thymus. Based on the localization and inconclusive computed tomography images, the preoperative prediagnoses included enterogenic cyst, thymus cyst and teratoma. Intraoperative features of the vascular supply and structure of the mass drew the surgeons' suspicion to extralobar pulmonary sequestration, which was confirmed in the histopathological examination. The scans were reevaluated after the surgery. Surgical management was implemented prior to the occurrence of any symptoms, which led to a positive general outcome. The presented case should raise the awareness of radiologists, pediatric surgeons and other consultants involved in the diagnostic process of mediastinal lesions in children. The rare localization and lack of visualization of a systemic feeding artery can divert suspicion away from extralobar pulmonary sequestration in the preoperative differential diagnosis.

OBJECTIVES: The aim of the study was to evaluate pulmonary sequestration (PS). We report on location, blood supply, histology, clinical manifestation, and surgical treatment of PS, as well as on postoperative course in patients with PS. BACKGROUND: PS is a rare congenital defect of the lower respiratory tract, it represents locus minoris resistentiae of the body. Occasionally, PS is diagnosed for the first time in adulthood. METHODS: We evaluated 7 cases of PS treated at the Centre of Thoracic Surgery in Vyšné Hágy, Slovakia, between years 2013 and 2020. RESULTS: Four of our seven patients were asymptomatic; the PS was found incidentally upon chest imaging. Three patients had recurrent bronchopneumonia related specifically to the intralobar type of sequestration. The most significant complication, observed in a singular patient, was a life-threatening episode of haemoptysis, requiring urgent surgical intervention. In the other 6 cases, the sequestra were surgically resected during the period when they were asymptomatic. and their sputum was confirmed negative upon microbiological examination. Anatomical resection of the affected pulmonary lobe by thoracotomy was the most common type of operation performed (4 cases, n = 7). There was no surgical mortality. CONCLUSION: To prevent complications, it is crucial to perform surgical treatment for pulmonary sequestration in patients who have sufficient functional capacity (Tab. 2, Fig. 4, Ref. 30).

In the pathology of respiratory tract, sequestration presents as a non-functional lung tissue with no communication with tracheobronchial tree. It represents a rare congenital bronchopulmonary and vascular malformation, which occurs at a frequency of 0.1 to 6%. Intralobar and extralobar sequestrations are extremely rare congenital anomalies, especially if they occur at the same time in a patient. Proper diagnosis and appropriate surgical therapy, in the absence of associated anomalies, provide an excellent prognosis. In this paper, we are describing the simultaneous presence of intralobar sequestration (ILS) in the lower lobe of the left lung and extralobar sequestration (ELS) which is positioned on the aortic arch, in a 53 years old man. Two years post surgery, there is no recurrence or any patomorphological and functional disorders in the respiratory tract.

The purpose of our study was to evaluate the diagnostic performance of fetal magnetic resonance imaging (MRI)-based texture analysis (TA) to differentiate the two most common lung malformations, congenital pulmonary airway malformation (CPAM) and pulmonary sequestration (PS). This retrospective single-center study included 24 patients with CPAM and 8 patients with PS who had a fetal MRI examination between January 2015 and December 2020. T2-weighted coronal images were used for TA. One reader designated the malformation borders and drew a region-of-interest for TA. The differences in values of the texture features between the groups were assessed and receiver operating characteristic curves were calculated for each statistically significant feature. P-value<0.05 was considered statistically significant. Results: Forty-eight texture features were calculated for each malformation. Twenty features on T2-weighted images were significantly different between the CPAMSs and PSs. Among these, short-run high gray-level emphasis and long-run emphasis, which are gray-level runlength matrix features parameters, had the largest area under the curves: 0.956 (sensitivity 87%, specificity 95%) and 0.943 (sensitivity 87%, specificity 85%), respectively. Our results suggest that fetal MRI-based TA may be used to distinguish CPAMs from PSs in fetuses with uncertain pulmonary findings prior to birth.

The purpose of our study was to evaluate the diagnostic performance of fetal magnetic resonance imaging (MRI)-based texture analysis (TA) to differentiate the two most common lung malformations, congenital pulmonary airway malformation (CPAM) and pulmonary sequestration (PS). This retrospective single-center study included 24 patients with CPAM and 8 patients with PS who had a fetal MRI examination between January 2015 and December 2020. T2-weighted coronal images were used for TA. One reader designated the malformation borders and drew a region-of-interest for TA. The differences in values of the texture features between the groups were assessed and receiver operating characteristic curves were calculated for each statistically significant feature. P-value<0.05 was considered statistically significant. Results: Forty-eight texture features were calculated for each malformation. Twenty features on T2-weighted images were significantly different between the CPAMSs and PSs. Among these, short-run high gray-level emphasis and long-run emphasis, which are gray-level runlength matrix features parameters, had the largest area under the curves: 0.956 (sensitivity 87%, specificity 95%) and 0.943 (sensitivity 87%, specificity 85%), respectively. Our results suggest that fetal MRI-based TA may be used to distinguish CPAMs from PSs in fetuses with uncertain pulmonary findings prior to birth.

Fundamento: las malformaciones broncopulmonares son un grupo heterogéneo de enfermedades producidas por una alteración en algún momento del desarrollo embrionario de los pulmones y bronquios. Objetivo: describir las malformaciones broncopulmonares más frecuentes en la edad pediátrica y su nomenclatura actual. Métodos: la búsqueda de la información se realizó en el periodo de dos meses (noviembre y diciembre de 2017). A partir de la información obtenida se realizó una revisión bibliográfica de un total de 74 artículos publicados en las bases de datos PubMed, Hinari, SciELO, Ecumed y Medline; mediante el gestor de búsqueda y administrador de referencias EndNote. Desarrollo: los avances en los métodos diagnósticos de imagen y la mayor experiencia de los especialistas que las realizan hacen que sea una enfermedad de detección principalmente prenatal o intraútero, lo que va a permitir realizar un seguimiento más estrecho del paciente. Conclusiones: según la consulta realizada las malformaciones más frecuentes son: malformaciones de las vías aéreas bajas, enfisema lobar congénito, secuestro pulmonar intralobar y quiste broncogénico. En las malformaciones broncopulmonares con mayor riesgo de complicaciones poste- 149 riores o que estén dando síntomas, la opción terapéutica más clara es la cirugía.

El neumatocele es una lesion redondeada de contenido aereo y pared fina que se produce tras infeccion, trauma o inflamacion pulmonares. Puede ser confundida con otras imagenes quisticas pulmonares (malformacion adenomatosa, bronquiectasia). En ausencia de clinica respiratoria importante, el seguimiento radiologico evolutivo podria bastar, evitando procedimientos diagnosticos o terapeuticos lesivos para el paciente.

Pulmonary sequestrations are congenital malformations of the lung of mixed origin -bronchial and arterial-, which are characterized by a zone of non-functioning cystic embryonic lung tissue whose vascularization is through an anomalous systemic artery. The case of a patient who receives a confirmation of the diagnosis of abdominal tumor by ultrasound, at birth, is reported. A contrasted computerized axial tomography was conducted and the patient underwent surgery. A left supraumbilical transverse incision was performed and the retroperitoneal space was accessed through the opening of the line of Toldt. The pathology report showed extralobar pulmonary sequestration (abdominal) with a cystic adenomatoid malformation.

OBJECTIVES: Few experiences comparing paediatric and adult patients treated for pulmonary sequestration (PS) have been reported. Surgical treatment is considered the best choice, but the time of surgery is still controversial. We present our experience in this setting, comparing characteristics, histological results and outcome of paediatric and adult patients undergoing PS resection. METHODS: Between 1998 and 2017, a total of 74 patients underwent lobectomy or sublobar resection for PS. Sixty patients were children (group A: < _16 years old) and 14 were adults (group B: >16 years old). Preoperative diagnosis was radiological. PS was intralobar (42 cases) and extralobar (32 cases). The operation was a muscle-sparing lateral thoracotomy or video-assisted thoracoscopic surgery. Preoperative characteristics, histological results and short-/long-term results of the 2 groups were retrospectively analysed and compared.

Majority of sequestrations fall into two categories: Intra-Lobar (ILS) and Extra-Lobar (ELS). Rarely the abnormal lung could be attached to the gastrointestinal tract, Bronchopulmonary Foregut Malformation (BPFM). We described a case of a girl of 3-years-old with antenatal diagnosis of left intrathoracic mass of the inferior lobe. Postnatal Computed-Tomography (CT) revealed a bilateral ELS with an isthmic bridge crossing the vertebral spine. She follows a MRI follow-up at 18months/30months confirming the lesion. Before surgery, a three-dimensional-CT-angiography was performed to study the mass, its blood supply and to plan surgery. She underwent to thoracoscopic resection. Two aberrant blood vessels were dissected from the thoracic aorta and ligated. The postoperative course was uneventful. She was discharged after 3 days. The rarity of our case is due to the bilateral extension. An appropriate preoperatory imaging study is necessary for the success of surgery while thoracoscopy is ...

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 pre- sentations. A patient is reported with intralobar pulmonary sequestration in the left lung and colonisation with Aspergillus which was successfully treated by lower lobectomy.

We present an unusual case of right lower lobe intralobar pulmonary sequestration in a previously healthy and physical active 27-year-old U.S. Marine, who presented with new onset hemoptysis after doing push-ups. Diagnosis was obtained by chest X-ray and contrast computed tomography scan. Preoperatively the patient underwent fluoroscopy-guided embolization of the aberrant systemic artery supplying the sequestration. The segmental resection of the sequestration was then successfully performed through a muscle-sparing thoracotomy.

Pulmonary sequestration (PS) is a rare congenital broncho-pulmonary malformation. The main feature of this disease is that partial lung tissues separate from the main lung during the embryonic period, receiving blood supply from systemic circulation arteries. Pathogenesis of PS is not clear, and categorized into congenital and acquired PS. We report a case of a 38 year old woman smoker with medical history characterized by difficult to treat asthma with frequent exacerbations and infections since childhood. CT scan showed a partial PS of left lower lobe, supplied by an abnormal artery arising from supradiaphragmatic aortic diverticulum. Surgical treatment through a lung sequestrectomy and laterobasal segment resection was performed.

O seqüestro pulmonar é um segmento ou cisto pulmonar com suprimento arterial anômalo proveniente, na maioria dos casos, da aorta torácica descendente. Suas manifestações clínicas mais comuns são decorrentes das freqüentes co-infecções bacterianas piogênicas, assemelhando-se a diversas supurações broncopulmonares. Os autores pretendem, com tal relato, mostrar a investigação de uma supuração pulmonar de evolução insidiosa, cujo diagnóstico foi de seqüestro pulmonar associado à tuberculose pulmonar.

Pulmonary sequestration occurs when a portion of lung tissue receives its blood supply from an anomalous systemic artery. Three main presentations, intralobar, extralobar and communicating bronchopulmonary foregut malformations, have been described. It is the second most common congenital lung anomaly. The intralobar variant is the most common type seen in 75% of cases, especially in late childhood. Imaging of choice for diagnosis are computed tomography scan and magnetic resonance imaging. Management involves surgical resection with ligation of the aberrant blood supply via thoracotomy or thoracoscopy. Endovascular therapy with coil embolization of the aberrant anomalous systemic artery as a standard therapy or as a hybrid therapy is an option. We present our successful surgical management of an infant diagnosed prenatally with congenital lung abnormality and confirmed postnatally as intralobar pulmonary sequestration.

Congenital Cystic Adenomatoid Malformation (CCAM) is a rare development abnormality of the lung occurring in 1-4 / 100,000 live birth. In most cases the outcome of a fetus with CCAM is very good whereas in some cases it can be life threatening for the fetus. We report here a case of 40 dayold-female baby with features of respiratory distress since birth and X-ray chest suggestive of large cystic lesion of left lung CCAM.

Communicating bronchopulmonary foregut malformation (CBPFM) is a communica tion between the respiratory and gastrointestinal tracts that can be difficult to differen tiate from pulmonary sequestration or Htype tracheoesophageal fistula (TEF) because of the similarities in clinical features. A female neonate born at full term had been ex periencing respiratory difficulty during feeding from the third day of life. The esopha gography performed to rule out Htype TEF revealed that the esophageal bronchus directly communicated with the left lower lobe (LLL) of the lung. Lobectomy of the LLL, fistulectomy of the esophagobronchial fistula, and primary repair of the esopha gus were performed. Finally, CBPFM type III with pulmonary sequestration was con firm ed on the basis of the postoperative histopathological finding. We report the first newborn case of CBPFM type III with pulmonary sequestration in Korea.

A 9-month-old male infant was admitted for surgery because of a prenatally detected lower left intralobar pulmonary sequestration. Computed tomography performed at 3 months of age had confirmed prenatal diagnosis showing a multisegmental posterobasal left lobar consolidation and a peripheral dysplastic area with microcystic lesions (hybrid lesion) (Figures and). The pulmonary sequestration was richly and extensively vascularized by a large artery deriving from the thoracic aorta (Figures and) and drained through four anomalous veins into the inferior vena cava. At excision, performed thoracoscopically, a previously undetected exophytic nodule resembling a hemangioma (see video in online supplement) was found on the pleural surface of the Author Contributions: L.A., F.S., P.R., and G.P. performed surgical procedures on the reported patient. L.A. and F.S. prepared the manuscript and figures. G.A.R. performed radiological study and P.M. performed histological study. This article has a video supplement, which is accessible from this issue's table of contents at www.atsjournals.org

Surgical resection in mmspected intralobar sequestration can result in fatal hemorrhagic complications. The roentgen criteria for establishing tbis diagnosis are: 1) chronic and recurrent pulmonary Wtrate; 2) most often located in b a s k segments of the lower lobes; 3) multiple cystic areas; 4) failure of contrast media to enter the cystic area during broncbography. We studied two cases of intralobar sequestration where on bronchography the contrast medium entered the cystic areas. To our knowledge only four other similar cases are reported in the literature. In our cases the preoperative diagnosis was confirmed by aortogmphy. Since the roentgenographic and bronchographie findings alone would have suggested the possibility of cystic broncbiectasis, the importance of aortography is stressed.

The intra-abdominal localization of extralobar pulmonary sequestration (EPS) is an uncommon entity, although there are an increasing number of publications in literature on EPS over recent years. There seems to be a predominance of left-sided suprarenal positioning of the sequester and so far resection has been undertaken by way of laparotomy. This paper describes the laparoscopic resection of EPS in two patients. In both instances the procedure was successful and the postoperative course was uneventful. It is concluded that EPS should be included in the differential diagnosis of suprarenal masses, particularly on the left side. Laparoscopic resection is the method of choice for EPS.

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 intralob...

Congenital abnormalities of the lung can manifest in any component of the bronchopulmonary system. Congenital lobar emphysema, bronchogenic cyst, congenital cystic adenoid malformation (CCAM) or pulmonary sequestration (PS) are just some of the many kinds of malformations of the lung. The coexistence of CCAM and PS is a very rare condition. Due to such complications as dyspnea or recurrent infection, most malformations are diagnosed antenatally or in the immediate postnatal period. The present case was not diagnosed until late adolescence, and the patient was subsequently referred to our clinic after undergoing a previous operation. After examinations and a preoperative evaluation, a second operation was performed. We present to literature a very rare case of co-existing CCAM and PS (hybrid lung malformation).

Background High-dose radioactive iodine-131 (I-131) is generally used post-total or subtotal thyroidectomy in differentiated thyroid cancer (DTC) patients. I-131 whole-body scan (WBS) is performed to localize I-131 accumulation in the body and for restaging after therapy. Nonetheless, there are numerous causes of false-positive uptake in I-131 WBS in the absence of residual thyroid tissue or thyroid cancer metastasis. Case presentation We present a case of 51-year-old lady with false-positive uptake in I-131 WBS mimicking lung metastasis. Patient underwent total thyroidectomy, and histopathological examination showed well-differentiated papillary thyroid microcarcinoma. Diagnostic I-131 WBS followed by two subsequent high-dose radioiodine ablation showed persistent I-131 uptake in the left lower lung field. However, the serum thyroglobulin (Tg) is incessantly undetectable (

Pulmonary sequestration is a relatively rare malformation. Infection with common pyogenes is a frequent feature in the evolution of this disease. We report a case of intralobar sequestration infected with Mycobacterium tuberculosis in the absence of any other site of tuberculous infection. The patient underwent surgical removal of the affected lobe and subsequent antituberculous chemotherapy. At 1-year follow-up his clinical status is excellent.

Background Controversy still exists in the indication and timing of surgical treatment of pulmonary hamartoma (PH). The objective of this study is to summarize the experience and the outcome of the surgical treatment for pulmonary hamartomas, and to assess the effectiveness and necessity of surgical therapy administered in patients with pulmonary hamatoma as well as clinical and pathological features and long-term follow-up results. Methods This retrospective report has reviewed a 20-years clinical history of surgical treatment for 39 patients with PH from 1985 to 2006. These thirty-nine patients underwent 40 operations as follows: wedge resection (23), enucleation (10), segmentectomy (3), lobectomy (3), and pneumonectomy (1). Results The PH occurred most frequently (78.1%) in the patients aged 40 to 60 years and the sex ratio (male/female) was 2.25/1 in our series. No postoperative death was encountered. One patient with pleural effusion was cured after thoracentesis. All of these ...

An asymptomatic 6-week-old female infant presented to the clinic with a prenatal history of a right-sided chest mass. Physical examination revealed no abnormalities, and laboratory data were essentially normal. Prenatal ultrasonography (US) had demonstrated slight cardiac compression and mild hydrops associated with the chest mass, but the findings were not severe; therefore, no fetal intervention was performed. The patient had no birth complications or postnatal respiratory symptoms. © RSNA, 2011 • radiographics.rsna.org

L'hétérotopie pulmonaire gliale est une entité extrêmement rare dont la physiopathologie demeure incertaine. Nous rapportons pour la première fois un cas survenu chez un bébé de 1 mois, indemne de toute malformation. Elle est issue d'une grossesse gémellaire monochoriale monozygote où l'autre jumelle atteinte d'anencéphalie exencéphalie a bénéficié d'une interruption médicale de grossesse. La maladie s'est manifestée par une détresse respiratoire transitoire néonatale, récidivante à un mois de vie, avec découverte sur la radiographie thoracique d'images kystiques pulmonaires plus marquées à droite. Devant la suspicion de malformation adénomatoïde kystique pulmonaire (MAKP), elle a été opérée à l'âge de quatre mois d'une lobectomie supérieure et inférieure droite. L'étude anatomopathologique retrouvait une lésion multi-kystique dont les parois étaient constituées de tissu glial bien différencié et peu cellulaire, par endroit, bordé d'épithélium bronchique. Il n'y avait pas d'argument morphologique en faveur d'une MAKP. L'évolution a été favorable avec un recul de cinq mois après l'intervention. Il s'agit d'un des très rares cas où la maladie n'a pas conduit à un décès rapide in utéro ou en périnatal immédiat. Cela suggère qu'une prise en charge efficace par chirurgie pourrait être un élément décisif dans la survie de ces patients.

Introduction: We describe the rare case of endobronchial tuberculosis (EBTB) and chronic pulmonary atelectasis with mediastinal distortion. Finding of the concomitant venous anomaly of inferior vena cava revealed the diagnosis of bronchopulmonary sequestration. Case Report: A 22-year-old Caucasian woman presented with a history of chronic cough, initially treated as bronchial asthma for a year. Chest X-ray showed fibrocaseous cavernous tuberculosis on the right lung. Acid Fast Bacilli (AFB) were found in sputum samples. Patient was treated for 6 months with usual antituberculous regiment. Control chest X-ray showed subatelectasis of the upper right lobe. Six months later the first thorax computed tomography (CT) showed complete atelectasis of the right lung. Patient was admitted to the hospital again after 6 years due to the persistent fever and cough. Endoscopic finding and histopathological analysis confirmed EBTB. Thoracic CT scan revealed duplication of inferior vena cava which ...

A 10-month-old boy was referred for tachypnea and heart murmur. An echocardiogram showed unexplained left heart dilation without evidence of an intracardiac shunt. A 64-slice computed tomographic contrast-enhanced angiography showed a large tortuous anomalous artery arising from the descending thoracic aorta and supplying the lower lobe of the left lung. The venous return into the left atrium was normal. The affected lobe had normal lung parenchyma, and its bronchial tree was connected normally with the left main bronchus. Hence, it was not a sequestrated lobe. The boy underwent surgical lobectomy of the left lower lobe and improved. Anomalous arterial supply of a lobe without sequestration of its bronchial tree is a rare pathologic entity. It also is a very rare cause of congestive heart failure in children. Computed tomographic angiography was a useful tool for evaluation of the intrathoracic anomalous vessel in this case.

Intralobar pulmonary sequestration is a rare congenital malformation characterized by the presence of dysplastic lung that does not communicate with the tracheobronchial tree, and has aberrant systemic arterial supply. While most are asymptomatic, they rarely can present with hemoptysis, which has been traditionally managed with surgical resection of the sequestration. We report a case of an 18-year-old male who presented with acute large-volume hemoptysis on a background of recurrent minor episodes of hemoptysis, due to intralobar sequestration. He was successfully treated with transarterial embolization with a combination of polyvinyl alcohol (PVA) particles, gelfoam and coils. Transarterial embolization can be effective in managing emergent hemoptysis from pulmonary sequestration.

Congenital diaphragmatic hernia (CDH) has an incidence of approximately 1:4000 live births. Most frequently the diaphragmatic defect is a left and posterolateral (Bochdalek) one. Prenatal diagnosis is made at ultrasonography; the relevant sonographic features will be described in the paper. Cystic adenomatoid malformation of the lung (CAML), pulmonary sequestration, bronchogenic cysts, pulmonary hypoplasia/agenesia need to be considered in differential diagnosis. In some cases, diagnosis of CDH is not possible "in utero": in such cases, herniation of abdominal viscera into the thorax takes place presumably just at delivery through a small diaphragmatic defect. CDH may be associated with intrauterine growth retardation (IUGR), chromosomal abnormalities (3%) and/or other malformations (10-50%): such as Central Nervous System, digestive, cardiac and urogenital anomalies. Therefore, search of associated malformations and amniocentesis with analysis of fetal karyotype are mandatory, whenever a CDH is diagnosed. CDH is still at present characterised by a high mortality (reportedly, about 45%). Many prognostic factors have been correlated to postnatal outcome of CDH: some of them are valuable prenatally by ultrasonography. However, the role of sonography in the prediction of neonatal outcome is still controversial: in particular, although many ultrasonographic parameters have been proposed, prenatal evaluation of pulmonary hypoplasia (a crucial factor related to postnatal survival) has not proved to be very accurate so far. Nevertheless, it is undisputable that prenatal diagnosis itself represents a crucial prognostic factor for CDH, since it allows birth of the affected fetuses in 3d level Perinatologic Centres provided with a Neonatal Intensive Care Unit and Neonatal Surgery.

Congenital malformations are significant causes of mortality and morbidity in both term and preterm infants 1 and, among them, congenital lung malformations, that is, congenital pulmonary airway malformation (CPAM) and pulmonary sequestration (PS) represent a rare yet significant entity. The CPAM is diagnosed in 1:10,000 to 1:35,000 births. 2 It is defined as a multicystic mass of pulmonary tissue in which there is proliferation of immature bronchial structures at the expense of alveolar development. 3 Most cases are diagnosed prenatally by ultrasound scan. The clinical presentation after birth may vary from asymptomatic cases to neonates presenting with severe respiratory distress or respiratory failure due to the mass effect of a large lesion or to secondary pulmonary hypoplasia. 4 CPAM are usually classified according to either Stocker (Stocker et al 5) or Adzick (Adzick et al 6) classification. PS are the second most common prenatally diagnosed lung malformations. They represent up to 6% of congenital lung anomalies. 7 They are characterized by a portion of nonfunctional lung tissue with no connection with the

Objective This study was aimed to evaluate effectiveness of lung ultrasound (LUS) in the management of congenital pulmonary airway malformation and pulmonary sequestration in NICUs. Study Design This is a nonconsecutive case series of neonates admitted to the academic NICU of Policlinico of Bari, Italy, from 2010 to 2018, for suspected lung malformations and examined by LUS. Results Seven neonates were admitted for suspected pulmonary malformations, four neonates were diagnosed with pulmonary sequestration and three with congenital pulmonary airway malformation either type I (two cases) or type II (one case) according to Adzick classification. Prenatal scans had described lung malformations in six patients. Two underwent surgical intervention during the 1st month of life. All were successfully discharged home and their follow-up has been uneventful thereafter. In all the seven neonates, LUS easily detected the lesion showing a significantly high correspondence with computed tomograp...

Congenital pulmonary airway malformation (CPAM) is a rare congenital lung lesion, that usually remains asymptomatic during the fetal and neonatal period. However, it can occasionally cause prenatal cardiocirculatory failure and fetal hydrops, requiring a thoraco-amniotic shunt (TAS) placement. In other cases, it can also cause symptoms at birth (such as respiratory distress), and may require urgent surgical intervention. Thoracoscopic lobectomy for neonates is rarely reported. Here, we report a case of right macrocystic CPAM causing fetal hydrops at 27 weeks of gestation. The fetus was treated with a TAS placement that successfully resolved the hydrops. At 39 weeks of gestation a male neonate was born (weight 2850g). The TAS spontaneously displaced during delivery, causing an open pneumothorax (PNX), initially treated with a drainage. His condition gradually worsened, requiring ventilatory support. TC scan showed different giant cysts in the context of the right lower lobe, left mediastinal shift and compression of the rest of the lung. An urgent surgical management was required. A thoracoscopic right lower lobectomy was performed at 10 days of life (weight 2840g). The postoperative course was uneventful, the child remained totally asymptomatic and showed a good recovery. To the best of our knowledge this is the first reported case of open iatrogenic PNX following TAS positioning and the second of neonatal thoracoscopic lobectomy in a newborn weighting less than 3kg. The purpose of this report is to indicate that minimally invasive surgery is feasible, safe and effective for the resection of congenital pulmonary airway malformation, even in small newborns

Bronchopulmonary sequestration is a rare disease in which a non-functional region of pulmonary tissue receives an aberrant vascular supply and lacks normal communication with the tracheobronchial tree. We present the case of a 30-year-old female with a primary complaint of unexplained weight loss and no other additional signs or symptoms. In view of this, computed tomography imaging was ordered, showing a 33HU mass in the right upper lobe. A specialist radiologist reviewed the images and concluded that the most likely differentials were mediastinal lymphoma or thymic malignancy. Video-assisted thoracoscopic surgery was performed, when it was seen that no malignancy was present, but rather a bronchopulmonary sequestration. Histology confirmed the diagnosis; the patient fared well post-operatively. Bronchopulmonary sequestration is a rare pathology, with most cases occurring in the lower lung lobes. This case is highly atypical, due to the lack of clinical features and the lesion radiologically mimicking the appearance of malignancy.

Bronchopulmonary sequestration is a rare disease in which a non-functional region of pulmonary tissue receives an aberrant vascular supply and lacks normal communication with the tracheobronchial tree. We present the case of a 30-year-old female with a primary complaint of unexplained weight loss and no other additional signs or symptoms. In view of this, computed tomography imaging was ordered, showing a 33HU mass in the right upper lobe. A specialist radiologist reviewed the images and concluded that the most likely differentials were mediastinal lymphoma or thymic malignancy. Video-assisted thoracoscopic surgery was performed, when it was seen that no malignancy was present, but rather a bronchopulmonary sequestration. Histology confirmed the diagnosis; the patient fared well post-operatively. Bronchopulmonary sequestration is a rare pathology, with most cases occurring in the lower lung lobes. This case is highly atypical, due to the lack of clinical features and the lesion radi...

Pulmonary sequestration is a rare anomaly, which does not have a connection with the bronchial system and gets its blood supply, generally, from the aorta or its branches. Anatomically, two forms were described: extralobar and intralobar which is the commoner type of pulmonary sequestration accounting for 75% of all sequestrations. Although 74% of intralobar pulmonary sequestrations get their blood supply from the descending thoracic aorta, they may get their blood supply from different arteries. Furthermore, there is more than one arterial anomaly in 14.8% of cases. We report an intralobar pulmonary sequestration, in, which arterial blood supply is from celiac trunk, with a cardiomegaly at the expense of the right atrium.