Extralobar Sequestration Research Articles

Infradiaphragmatic extralobar pulmonary sequestration asossiated with duplication cyst of the gaster in newborn

Introduction. The biggest difficulties of pulmonary sequestration treatment appear when they are combined with other congenital malformations.
 Materials and methods. This article describes a rare case of combination of infradiaphragmatic extralobar sequestration of the lung and duplication cyst of the gaster. The antenatal ultrasound investigation at 21 week of gestation visualized a retroperitoneal mass that had a cystic-solid structure and a vessel that departed directly from the aorta. CT-scan that was performed after the birth, confirmed an existence of infradiaphragmatic cystic mass that had a vessel that departed directly from the aorta. We couldnt exclude the neoplastic process because of structure and topography of mass. Tumor markers were without pathology. Surgical treatment was performed: laparotomy and removal of the cyst and extrapulmonary sequestration. The histological investigation confirmed a pulmonary sequestrum and duplication cyst of the gaster.
 Conclusion. A nowadays method of visualization allows to find congenital malformation in earliest stages of gestation. The tradition surgical treatment and also minimal invasive surgical treatment are the main option of cure such congenital malformations. Also literature review of rare clinical forms of pulmonary sequestration presented in this article.

Thoracoscopic Surgery for Congenital Lung Malformation Using Miniaturized 3-mm Vessel Sealing and 5-mm Stapling Devices: Single-Center Experience.

Aim: To evaluate the outcomes of thoracoscopic resection of congenital lung malformations (CLM) by using JustRight® (Bolder Surgical) 3-mm vessel sealing system and 5-mm stapler. Methods: Patients who underwent thoracoscopic resection of CLM in our Center from January 2016 to July 2019 were selected and divided into two groups: G1 (2018-2019), treated with the new JustRight instruments, and G2 (2016-2017), treated with Ligasure® and Hem-o-lok. Surgical outcomes in term of length of surgery and complications were compared between groups, stratifying the groups for type of resection (lobectomy, extralobar sequestration [ES] resection, and bronchogenic cyst [BC] resection). Results: Thirteen patients were included in G1, and 16 patients were included in G2. Seven lobectomies, 5 ES resections, and 1 BC resection were performed in G1; whereas 12 lobectomies, 3 ES resections, and 1 BC resection were performed in G2. Mean age at the time of surgery was 7.8 ± 6 months (G1) and 6.8 ± 3.3 months (G2) (P = .57). The average length of surgery was shorter for G1 (lobectomies: 120.7 ± 28.2 versus 171.7 ± 37.5, P = .006; ES resection: 63 ± 21.4 versus 91.7 ± 29.3, P = .15; BC 40 minutes versus 100 minutes). No significant difference was found for length of stay (4 ± 1 days versus 5 ± 2 days, P = .18). Neither the need for conversion nor major complications were observed in either group. Conclusion: In our experience, thoracoscopic resections of CLM with the new JustRight instruments were revealed to be safe and effective. On account of having adequate dimensions for small cavities, these instruments can facilitate the procedure and help to reduce the length of surgery.

Non-operative management of extralobar pulmonary sequestration: a safe alternative to resection?

This retrospective cohort study compares the natural history of patients with extralobar sequestrations (ELS) who do not undergo intervention with those who undergo resection to assess the safety of non-operative management. 126 patients with pulmonary sequestrations or congenital pulmonary airway malformations born between 1999 and 2016 were identified. 49 patients had ELS on postnatal imaging, but two were excluded for associated congenital diaphragmatic hernia. Demographic and clinical data were retrospectively reviewed, with phone follow-up for non-operative patients with no records for > 1year. Statistical analysis was by Fisher's exact test or Wilcoxon signed-rank test (two-tailed p < 0.05). 40% (19/47) were managed non-operatively and 60% (28/47) underwent resection. Non-operative patients were less likely to have an intrathoracic ELS: 47% (9/19) vs. 75% (21/28), p = 0.07. No symptoms were attributable directly to the ELS. Non-operative patients had median follow-up 3.2years, during which time 88% (15/17) of ELS decreased in size on serial imaging. For patients who underwent resection, there was 100% concordance between imaging and intraoperative findings. There was no evidence of inflammation, infection or malignancy on final pathology, though 57% (16/28) of resected lesions had foci of non-aerated cysts. Although further longitudinal study is required, this study supports the safety of non-operative ELS management.

Extralobar pulmonary sequestration with absence of pericardium and atrial septal defect in a woman

BackgroundExtralobar sequestration is a rare congenital malformation of lung tissue, which can be combined with other foregut and cardiac abnormalities. Our case is the first to report extralobar sequestration, absence of pericardium and atrial septal defect in the same patient.Case presentationA 22-year-old female with atrial septal defect came for her recent atypical symptom of intermittent palpitation and shortness of breath. Her computed tomography showed a cystic mass located in left superior anterior mediastinum near the pulmonary trunk. With specious of cystic teratoma prior to video-assisted thoracoscopic surgery, she finally was diagnosed as extralobar sequestration, while incidentally found with congenital absence of pericardium during surgery.ConclusionsExtralobar sequestration, absence of pericardium and atrial septal defect can occur in the same patient. The preoperative diagnostic rate of extralobar sequestration and asymptomatic absence of pericardium remains low attributed to atypical imaging features. A cardiac magnetic resonance imaging is highly recommended if necessary. Regular follow-up is essential to asymptomatic absence of pericardium and atrial septal defect patients. To patients with extralobar sequestration, an operation could be performed.

Congenital Cystic Lung Lesions: Redefining the Natural Distribution of Subtypes and Assessing the Risk of Malignancy.

Asymptomatic cystic lung lesions-congenital pulmonary airway malformations (CPAMs), sequestrations, and bronchogenic cysts-are commonly diagnosed prenatally. Indications to resect are to eliminate risk of malignancy or infection. CPAMs consist of a spectrum of malformations, with type 1 historically considered the most common. Mucinous cell clusters, seen almost exclusively in type 1, are premalignant lesions at risk for progression to mucinous adenocarcinoma. We reviewed and classified 2.5 years of consecutive, prenatally diagnosed lesions as extralobar sequestration, intralobar sequestration, type 1 CPAM, type 2 CPAM/bronchial atresia, or "other" to determine the distribution of lesion types and risk of malignancy. One hundred eighty-four lesions in 174 patients showed type 1 CPAM to be least common subtype. Type 1 CPAMs had more severe presentation, infrequently had features of obstruction, and usually had cysts ≥2 cm. Fifteen of eighteen type 1 CPAMs had mucinous cell clusters (total risk, 8%), with mucous cells outside main cyst in 12/15. No pleuropulmonary blastomas were identified. Additional historic cases were reviewed to further evaluate risk of malignancy. Over 14 years, 28 infants with fetal/type 1 lesions were identified, with clusters of mucinous cells in 75% of cases. A total of 9 pleuropulmonary blastomas were diagnosed in 6 patients over 16 years. Contrary to historical studies, type 1 CPAMs are much less common than type 2, likely related to detection of asymptomatic lesions prenatally. A majority of type 1 CPAMs contain mucinous cell clusters. This data is useful in management of patients in centers that do not resect asymptomatic lesions.

Гетеротопия легкого в брюшную полость у новорожденной девочки

Heterotopia is the spatial displacement of an organ during prenatal development. The literature data describe rare cases of the extralobar sequestration of the lung in different locations, such as neck soft tissues, chest wall, endocardium of the left ventricle, skin, rectum, eye orbit as well as the subretinal space. The paper presents a clinical case of the rare lung heterotopia in the abdominal cavity diagnosed 6 days after birth in a girl. The baby was born after a full-term complicated pregnancy by Caesarean section. Body weight at birth was 3680 g, height 50 cm, 7/8 points on the Apgar scale. The condition of the newborn was severe. On the third day after delivery, an attack of tachycardia up to 205 beats per minute and a drop in blood pressure was recorded and stopped within 15 minutes. On the 6th day after birth, an ultrasound revealed an echogenic inhomogeneous tumor-like formation 22 × 28 × 20 mm in size with multiple aneechogenic cavities with a diameter of up to 6 mm, which was removed laparoscopically on the 10th postpartum day in the neonatal surgery department of the Scientific Center of Children Health of the Ministry of Health of the Russian Federation. Pathological examination of the surgical material revealed that the formation was represented by lung tissue. The girl was discharged in satisfactory condition and fully conscious. This heterotopia could probably appear due to complications of pregnancy in the first and third trimesters, since a fetal malformation, not otherwise specified, was diagnosed in the first trimester, and the threatened miscarriage, increased blood pressure and edema were recorded in the third trimester. Keywords: heterotopia of the lung, high-risk pregnancy.

Distribution, diagnosis, and treatment of pulmonary sequestration: Report of 208 cases

ObjectiveThis study was performed to explore the clinical features, typing, distribution, and treatment of pulmonary sequestration (PS), with the aim of improving the awareness and treatment of this condition. MethodsClinical data regarding surgical procedures, outcomes, and prognosis of 208 pediatric patients with PS who were treated in our center from January 2005 to October 2017 were retrospectively analyzed. ResultsPS was confirmed by ultrasonography, enhanced computed tomography (CT), and/or magnetic resonance imaging (MRI) before surgery, and the surgeries were smoothly performed in all 208 patients (138 males, 70 females; age, 1 month to 14 years; mean age, 19.70 ± 48.82 months). The operative time ranged from 10 to 230 min (mean, 70 ± 48.75 min), and the intraoperative blood loss volume ranged from 1 to 200 ml (mean, 5 ± 18 ml). PS was located in multiple sites of the thoracic cavity and was also found in some rare locations such as the neck and abdomen. The feeding arteries of the PS mainly arose from the thoracic aorta or abdominal aorta, and a few of them originated from other vessels in the systemic circulation. The venous drainage differed between intralobar and extralobar PS: in patients with intralobar PS, the venous drainage was mainly via the pulmonary veins, especially the lower pulmonary veins; in patients with extralobar PS, the venous drainage was via the azygos vein and hemiazygos vein or reached the right atrium via the vena cava. The infection rate in children with intralobar sequestration was 71.17% (79/111), and that in children with extralobar sequestration was 31.37% (16/51). ConclusionPS has increasingly been detected by prenatal ultrasonography, and enhanced CT and MRI are the main techniques for diagnosing PS. Once confirmed, PS should be surgically resected. We choose an age of 6 to 12 months for surgical resection. Minimally invasive video-assisted thoracic surgery has many advantages in the treatment of PS and can be the treatment of choice for this condition. Type of studyTreatment Study. Level of evidenceLevel III.

Proximity to the Diaphragm Predicts the Presence of Rhabdomyomatous Dysplasia in Congenital Pulmonary Airway Malformations.

Rhabdomyomatous dysplasia (RD) is a pathologic finding in CPAMs that was incorrectly attributed to their malignant potential. The increasing recognition of extrathoracic (intradiaphragmatic and intraabdominal) congenital pulmonary airway malformations (CPAMs) offers a clue to the origin of RD. We hypothesize that the presence of RD is related to the CPAM's anatomic location. Retrospective review was performed of all children who underwent resection of a CPAM during a 10-year period. The age at the time of operation, location of the CPAM, and pathologic findings were collected. Peridiaphragmatic location was defined as within the inferior pulmonary ligament, deep to the diaphragmatic portion of the parietal pleura ("intradiaphragmatic") or adjacent to the abdominal side of the diaphragm. Statistical analysis was performed using Fisher's exact test for 2 × 2 tables. Twenty-six patients with CPAM were identified. Preoperative imaging was performed by computed tomography (CT) scan (16/26), ultrasound (5/26), magnetic resonance imaging (MRI) (1/26), and chest radiograph (4/26). The median age at resection was 15 months. Of these, 16 were pure cystic adenomatoid malformations, 4 were extralobar sequestrations, 4 were intralobar sequestrations, and 2 were bronchogenic cysts. Nine lesions were peridiaphragmatic with four being intradiaphragmatic (44%). Eight of the nine resected peridiaphragmatic lesions contained histologic evidence of rhabdomyomatous changes (89%, confidence interval [CI] 52-99%). None of the other lesions contained RD (CI 0-19%, p < 0.001). RD was seen exclusively, and in virtually all peridiaphragmatic CPAMs. While the exact significance of RD remains unclear, it may represent incorporation of striated muscle tissue associated with the developing diaphragm.

Pulmonary sequestration in children. postnatal course, diagnosis and surgical treatment

The aim — to optimize the diagnostic and treatment of pulmonary sequestration in children on the basis of clinical and diagnostic data and treatment results study.Materials and methods. The study included 14 patients aged 8 days to 17 years, 3 of which were newborns, 4 — from 1 to 12 months, 3 — from 1 to 3 years, 2 — 7­8 years, and 2 — 15 — 17 years old. There were 8 (57.1 %) boys and 6 (42.9 %) girls. Postnatal diagnosis was based on contrast computed tomography or angiography. In all cases the surgical treatment was performed. In 5 cases, the diagnosis of pulmonary sequestration was established intraoperatively during the surgery for mediastinal tumor, congenital diaphragmatic hernia or polycystic lung.Results and discussion. Ten (71.4 %) patients had intralobar sequestration and another 4 (28.6 %), extralobar one (p = 0.099), left­side localization was in 9 (64.3 %) cases, right­side in 5 (35.7 %). Associated malformations were found in 7 (50.0 %) patients and included polycystic lung (n = 3), lung hypoplasia (n = 2), diaphragmatic hernia (n = 2), pericardial defect (n = 1), thoracic dystopia of kidney (n = 1), Waardenburg syndrome (n = 1). Twelve (85.7 %) patients had a symptomatic disease presented with respiratory or hemodynamic disorders, signs of inflammation and hemophtysis. Lobectomy (n = 4), segmental lung resection (n = 4) or division of aberrant vessels (n = 2) were used in cases of intralobar disease depending on the extent of lesion while sequesterectomy (n = 4) was performed in extralobar sequestration. Simultaneous diaphragmoplasty, including that with use of bounded autopericardial flap (n = 1) was performed in 2 cases of associated diaphragmatic hernia. There were no postoperative complications and mortality. Late follow­up examination within the period from 4 to 22 years after the operation showed positive results in all 100 % of cases.Conclusions. Pulmonary sequestration, since the diagnosis is established, is subject to compulsory surgical correction, the effectiveness of which reaches 100 %. Correction of intralobar sequestration consists of lobectomy or segmental resection of the lungs, in the case of extralobar disease sequesterectomy is indicated. Associated congenital diaphragmatic hernia can be effectively corrected by diaphragmoplasty, including that with use of autopericardium.

Concurrent extrapulmonary bronchopulmonary sequestration and bronchogenic cyst

Abstract A prenatal ultrasound (US) at 21 weeks estimated gestational age (EGA) identified a left intrathoracic homogenously echogenic microcystic mass with mediastinal shift and a CPAM volume ratio (CVR) of 0.78 (Fig. 1). A fetal magnetic resonance imaging (MRI) study confirmed the US findings. MRI and US were repeated at 35 weeks EGA demonstrating a CVR of 0.36 and a spontaneous decrease in size of the previously identified mass. Moreover, an intralesional, fluid filled dilated bronchus was identified that connected to the esophagus and showed mild branching. This finding was thought to represent an esophageal bronchus. Prenatal echocardiogram demonstrated normal cardiac anatomy and function. The patient was delivered vaginally at 38 weeks EGA with no respiratory distress after delivery. The patient was discharged at this time with planned outpatient follow-up. Outpatient contrast CT at three months of age demonstrated a left BPS supplied by the celiac axis and a fluid filled branching structure within the sequestration suspicious for esophageal bronchus. At four months of age, the patient underwent a left, muscle sparing thoracotomy, where the bronchopulmonary sequestration was excised and an adjacent cyst was enucleated. Pathology demonstrated extralobar bronchopulmonary sequestration with a separate foregut malformation most consistent with bronchogenic cyst.

Surgical chylothorax in neonates: management and outcomes.

Postoperative chylothorax occurs due to trauma to lymphatic vessels and can occur after any thoracic procedure. This study reviewed recent literature to evaluate the management and outcomes of surgical chylothorax in neonates. PubMed database was searched for articles in English, Portuguese and Spanish from 2000 to 2016. Data were collected for surgery, chylothorax management, complications, mortality and length of hospital stay (LOS). Twenty studies offered 107 neonates: congenital diaphragmatic hernia (CDH)(n = 76,71%), cardiac malformations(n = 25,23.4%), esophageal atresia(n = 5,4.7%) and CDH+extralobar sequestration(n = 1,0.9%). Medium-chain triglycerides (MCT) was the initial treatment in 52 neonates (48.6%), prednisolone+MCT in one (0.9%), total parenteral nutrition in 51 patients (47.7%), and three patients (2.8%) did not require any treatment. Octreotide and somatostatin were used as second or third line treatment in 25 neonates (23.4%), and 15 neonates (14%) underwent 17 surgeries, including thoracic duct ligation (TDL)(n = 9); pleurodesis(n = 3) (2 patients required TDL); TDL+pleurodesis(n = 2), and TDL+placement of hemostat(n = 1). Complications due to the chylothorax were reported in 27 neonates (25.2%): hypoalbuminemia+hyponatremia(n = 18), hypoalbuminemia(n = 4), hypoalbuminemia with cutaneous flushing as colateral effect of somatostatin(n = 1), loose stool after somatostatin use(n = 1), pneumonia(n = 1), congestive heart failure+hypernatremia(n = 1), and hyponatremia(n = 1). There were 21 deaths (19.6%) and median LOS was 53.4days (30-93.1days). Conservative management is appropriate as initial treatment for neonatal postsurgical chylothorax. Octreotide and somatostatin are safe in neonates and surgical approach should be considered in prolonged leaks.

Patient 2 Uniportal VATS resection of extralobar sequestration

Patient 2 Uniportal VATS resection of extralobar sequestration

Prenatal growth characteristics and pre/postnatal management of bronchopulmonary sequestrations

PurposeThe prenatal natural history of intralobar and extralobar bronchopulmonary sequestrations (BPSs), including lesion growth patterns and need for prenatal intervention, have not been fully characterized. We review our series of BPSs to determine their natural history and outcomes in the context of the need for prenatal intervention. MethodsA retrospective review of the pre/postnatal course of 103 fetuses with an intralobar (n=44) or extralobar BPS (n=59) managed at a single institution between 2008 and 2015 was performed. Outcomes included prenatal lesion growth trajectory, presence of hydrops, need for prenatal intervention, survival, and postnatal surgical management. ResultsMost extralobar (71%) and intralobar BPSs (94%) decreased in size or became isoechoic from initial to final evaluation. Peak lesion size occurred at 26–28weeks gestation. Eight fetuses developed hydrothorax, four of which (all extralobar BPSs) also developed hydrops. All four hydropic fetuses received maternal betamethasone, and three hydropic fetuses underwent thoracentesis and/or thoracoamniotic shunt placement with subsequent hydrops resolution. All fetuses survived. Forty-one intralobar (93%) and 35 extralobar BPSs (59%) were resected after birth. ConclusionsBPSs tend to decrease in size after 26–28weeks gestation and rarely require fetal intervention. Lesions resulting in hydrothorax ± hydrops can be effectively managed with maternal steroids and/or drainage of the hydrothorax. Level of evidenceIV

Imaging of Nonmalignant Adrenal Lesions in Children.

The adrenal glands in children can be affected by a variety of benign lesions. The diagnosis of adrenal lesions can be challenging, but assessment of morphologic changes in correlation with the clinical presentation can lead to an accurate diagnosis. These lesions can be classified by their cause: congenital (eg, discoid adrenal gland, horseshoe adrenal gland, and epithelial cysts), vascular and/or traumatic (eg, adrenal hemorrhage), infectious (eg, granulomatous diseases), enzyme deficiency disorders (eg, congenital adrenal hyperplasia [CAH] and Wolman disease), benign neoplasms (eg, pheochromocytomas, ganglioneuromas, adrenal adenomas, and myelolipomas), and adrenal mass mimics (eg, extralobar sequestration and extramedullary hematopoiesis). Multimodality cross-sectional imaging helps to define the origin, extent, and relationship of these lesions to adjacent structures, as well as to guide treatment management. The anatomic and functional imaging modalities used to evaluate pediatric adrenal lesions include ultrasonography, computed tomography (CT), magnetic resonance imaging, and iodine 123 metaiodobenzylguanidine scintigraphy. Identifying the imaging features of nonmalignant adrenal lesions is helpful to distinguish these lesions from malignant adrenal neoplasms. Identifying characteristic imaging findings (eg, enlarged adrenal glands, with cerebriform surface, and stippled echogenicity in CAH; a T2-hyperintense mass with avid contrast enhancement in pheochromocytoma; low CT attenuation [<10 HU] and signal intensity drop on opposed-phase chemical shift images in adenoma; and enhancing suprarenal mass supplied by a systemic feeding artery in extralobar sequestration) can aid in making the correct diagnosis. In addition, clinical features (eg, ambiguous genitalia in CAH and hypertension in pheochromocytoma) can also guide the radiologist toward the correct diagnosis. ©RSNA, 2017.

Intralobar Bronchopulmonary Sequestration With Unusual Presentation of Recurrent Abdominal Pain

SESSION TITLE: Genetic and Developmental Disorders SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Bronchopulmonary sequestration (BPS) is a rare congenital malformation characterized by non-functioning lung tissue that does not communicate with the bronchial tree or pulmonary arteries, but typically has its own arterial supply arising from the aorta. It can be divided into extralobar sequestration (ELS), which is outside of the lung, has its own visceral pleura, and manifests earlier in life; and intralobar sequestration (ILS), which is within the pulmonary lobe, shares a visceral pleura, and often manifests later in life. Usually BPS in patients is unidentified unless infected or diagnosed incidentally on imaging. When symptomatic, patients can present with fever, cough, chest pain or hemoptysis. Here, we report a rare case of a young male who presented with recurrent left upper quadrant (LUQ) pain, diagnosed with ILS. CASE PRESENTATION: A 29 year old male with no significant history presented to ED with recurrent LUQ pain. He had 2 prior ED visits spanning 6 months where no specific diagnosis was made. He denied chest pain, cough, and fever. Vital signs, physical exam, and labs including CBC, LFTs and lipase were unremarkable. Chest x-ray and EKG were within normal limits. On the third ED visit, CT abdomen was performed. This showed no abnormalities in the abdomen but a mass in the left lower lobe, concerning for infection, atelectasis, congenital malformation, or malignancy. To clarify the mass, a CT chest with IV contrast was performed that showed a 6x4x7cm mass in the left lower lobe supplied by an aberrant artery off the descending thoracic aorta, highly suspicious for BPS. Patient was referred to thoracic surgery, had a pre-operative angiogram and embolization of the feeding artery, and underwent left thoracotomy with complete resection of BPS, which resolved his abdominal pain. Pathology confirmed the diagnosis of intralobar sequestration with inflammation. DISCUSSION: BPS is very rare, comprising only 0.15-6.4% of all pulmonary malformations. ELS is often identified on prenatal ultrasound and becomes symptomatic early in life. ILS often presents later during the second decade of life, and is found due to recurrent infections, mimicking pneumonia symptoms. It is typically within the lower lobe, on the left side, involving the posterior basal segment1. However, clinical presentation can vary depending on its type, size, and location, and sometimes presentation can be unusual, making it difficult to diagnose, as seen in our case where inflamed BPS caused diaphragmatic irritation resulting in referred abdominal pain. CONCLUSIONS: We report a rare case of ILS presenting with recurrent abdominal pain later in life. Screening for thoracic etiologies should be considered when no obvious abdominal cause is found to explain for the presenting abdominal pain. Reference #1: Wei Y, Li F. Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg. 2011;40(1):39-42. DOI: 10:1016/j.ejcts.2011.01.080. Epub 2011. DISCLOSURE: The following authors have nothing to disclose: David Attalla, Ji Hyun Rhee No Product/Research Disclosure Information

Hydrops fetalis secondary to supradiaphragmatic extrapulmonary sequestration with congenital cystic adenomatoid malformation

Congenital cystic adenomatoid malformation is a hamartomatous lesion. A 30 year old woman, G2P1L1, in the 29th week of gestation presented with pain abdomen, chest pain, cough with expectoration, fever and inability to appreciate fetal movements of 2 days duration. Clinically, she had pneumonia. An ultrasound revealed a single fetus in breech presentation with features of hydrops fetalis, hypoplastic left lung, mediastinal shift to the left and poor diastolic and systolic flow in the umbilical artery.A still born male baby delivered subsequently was found at autopsy to have hydrops fetalis, supradiaphragmatic right sided extralobar sequestration with associated congenital cystic adenomatoid malformation.The right lung also showed congenital cystic adenomatoid malformation. There were no other associated anomalies. We present a rare case of extralobar sequestrationwith congenital cystic adenomatoid malformation.

Rare localized extralobar sequestration with congenital cystic adenomatoid malformation: a case report

Extralobar sequestrations constitute a rare form of congenital pulmonary airway malformations that are difficult to diagnose. Here, we report a rare case of a localized extralobar sequestration in the right superior portion of the mediastinum accompanied by congenital cystic adenomatoid malformation.A 19-year-old man presented with a right upper mediastinal mass that was detected using chest radiography, had a history of left spontaneous pneumothorax, and had undergone a bullectomy 4 years previously.The initial diagnosis included a mature teratoma and a bronchogenic cyst in the mediastinum; however, the presence of a cystic mass in the right upper lobe of the lung prompted further examination. A preoperative diagnosis of extralobular sequestration was finally determined using contrast-enhanced computed tomography. The aberrant artery was connected to the brachiocephalic artery, and its drainage vein was connected to the right pulmonary artery, uniquely behind the pericardium. Despite the unique location, right mediastinal extralobular sequestration with a congenital cystic adenomatoid malformation in the right upper lobe was confirmed pathologically. Examination of contrast-enhanced chest computed tomography (CT) and three-dimensional computed tomography images enabled a correct diagnosis. It is very important for surgeons to correctly diagnose and identify an aberrant artery and drainage vein to prevent uncontrolled hemorrhage.

Thoracoscopic Segmentectomy for Extralobar Sequestration with Atypical Incomplete Fissures

Abstract Introduction: Pulmonary sequestration is a rare congenital developmental malformation of the lung. It represents a piece of nonfunctioning aberrant lung tissue, with its own blood supply arising from the systemic circulation.1 Intralobar sequestration is more common, comprising about 75% of the cases, and is usually treated by lobectomy. Extralobar sequestration is less common (25%) and usually has its visceral pleura. Segmentectomy is the procedure of choice.2 We hereby present a patient with extralobar sequestration but with associated atypical incomplete fissures associated with adjacent normal pulmonary parenchyma. Materials and Methods: This is the 2-year-old boy who was found to have congenital pulmonary mass antenatally at an outside hospital. He had been asymptomatic. Chest CT, with angiography, showed a pulmonary mass in the left lower lung region, with systemic artery arising from the thoracic aorta. One should always look for blood supply arising from the abdominal aorta, which may pas...

Extralobar pulmonary sequestration in adults

Pulmonary sequestration is defined as a nonfunctioning mass composed of dysplastic lung parenchyma, embryologically detached from the tracheobronchial tree and receiving its own blood supply from a systemic artery, usually the thoracic or abdominal aorta. It may be intralobar or extralobar depending on the presence of an independent pleural envelope. Extralobar sequestration is a much rarer entity, and diagnosis is usually made antenatally or in early life. Here, we present a rare case of extralobar pulmonary sequestration in an adult.

Subsegmental consolidation masquerading as extralobar bronchopulmonary sequestration presenting as pyrexia of unknown origin

Acinetobacter baumannii infection is common among inpatients and is a major cause of hospital-acquired or ventilator-associated pneumonia. It is rare for this pathogen to cause community-acquired pneumonia. Eighty percent of the patients with community-acquired Acinetobacter present with lobar consolidation. Bronchopulmonary sequestration is a rare congenital disorder with incidence of 1 in 10,000–35,000 live births. Intralobar sequestration is more common than extralobar. Affected individual generally presents with respiratory distress at an early age. Late manifestation with the lower respiratory tract infection has also been reported. We report an interesting case of subsegmental consolidation masquerading as extralobar sequestration of the lung in an adult who presented with pyrexia of unknown origin.