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congenital lung lesions

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https://www.readbyqxmd.com/read/28891233/fetoscopic-tracheal-occlusion-for-treatment-of-non-isolated-congenital-diaphragmatic-hernia
#1
Viola Seravalli, Eric B Jelin, Jena L Miller, Aylin Tekes, Luca Vricella, Ahmet A Baschat
Fetoscopic endotracheal occlusion (FETO) is a prenatal treatment that may increase survival in severe congenital diaphragmatic hernia (CDH). In the USA, FETO is offered for isolated severe left-sided CDH in the context of an FDA-approved feasibility study. FETO in non-isolated cases of severe CDH is only performed with a compassionate use exemption from US regulatory bodies. Anomalies frequently associated with CDH include congenital cystic lesions of the lung and cardiac defects. We describe two cases of non-isolated severe left-sided CDH that underwent prenatal FETO, survived after birth and underwent postnatal surgical repair...
August 11, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28884206/neonatal-congenital-lung-tumors-the-importance-of-mid-second-trimester-ultrasound-as-a-diagnostic-clue
#2
Stephan L Waelti, Laurent Garel, Dorothée Dal Soglio, Françoise Rypens, Michael Messerli, Josée Dubois
BACKGROUND: The differential diagnosis for primary lung masses in neonates includes a variety of developmental abnormalities; it also consists of the much rarer congenital primary lung tumors: cystic pleuropulmonary blastoma (cystic PPB), fetal lung interstitial tumor (FLIT), congenital peribronchial myofibroblastic tumor (CPMT), and congenital fibrosarcoma. Radiologic differentiation between malformations and tumors is often very challenging. OBJECTIVE: The objective was to establish distinctive features between developmental pulmonary abnormalities and primary lung tumors...
September 7, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28868608/incidental-imaging-findings-of-congenital-rib-abnormalities-a-case-series-and-review-of-developmental-concepts
#3
Anda Maria Aignătoaei, Cristina Elena Moldoveanu, Irina Draga Căruntu, Simona Eliza Giușcă, Simona Partene Vicoleanu, Alin Horațiu Nedelcu
BACKGROUND: Congenital rib abnormalities are found in approximately 2% of the general population. Usually, they occur in isolation, when they are rarely symptomatic, but they can also associate with other malformations. MATERIALS AND METHODS: We reviewed imaging examinations performed over a period of two years (2014-2015), enabling us to identify isolated rib abnormalities in six adult patients. RESULTS: The case series consisted in three cases with bilateral cervical ribs and one case each with bifid rib, costal fusion and rib pseudarthrosis...
September 4, 2017: Folia Morphologica (Warsz)
https://www.readbyqxmd.com/read/28862244/isolated-left-sided-pulmonary-artery-agenesis-with-left-lung-hypoplasia-a-report-of-two-cases
#4
V Govindaraj, J Joseph, B Nagamalli Kumar, R Soman
Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA) is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation...
August 31, 2017: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/28856451/recurrent-and-metastatic-congenital-mesoblastic-nephroma-where-does-the-evidence-stand
#5
Susan Jehangir, Jujju J Kurian, Dharshini Selvarajah, Reju J Thomas, Andrew J A Holland
PURPOSE: Fifty years ago, Bolande described Congenital Mesoblastic Nephroma (CMN) as a benign lesion. Unexpected aggressive clinical behaviors prompted a sub-classification based on histology. Recent molecular genetic evidence has identified the aggressive cellular variant to be the renal manifestation of congenital infantile fibrosarcoma. We submit a reappraisal and analysis of the available literature on recurrent and metastatic CMN. METHODS: An electronic search of PubMed, MEDLINE, EMBASE, and Scopus yielded 38 children with local recurrence and/or metastases...
August 30, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28812458/congenital-cystic-lung-lesions-evolution-from-in-utero-detection-to-pathology-diagnosis-a-multidisciplinary-approach
#6
Steven Hardee, Lea Tuzovic, Cicero T Silva, Robert A Cowles, Joshua Copel, Raffaella A Morotti
Congenital cystic lung lesions are a group of rare pathologies that are usually diagnosed in the prenatal period. The majority of these lesions are diagnosed at pathology examination as congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestration (BPS). These lesions are typically managed by surgical intervention within the first year of life and have an excellent prognosis. We examined the evolution of imaging appearances from prenatal diagnosis to postnatal work-up of these lesions and correlate imaging and pathological findings...
September 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28805595/prenatal-diagnosis-of-congenital-lobar-fluid-overload
#7
REVIEW
Pei-Shan Tsai, Chih-Ping Chen, Dao Chen Lin, Yu-Peng Liu
Prenatal congenital lobar fluid overload (CLFO), which was first described by Ramsay and Byron, is identical to postnatal congenital lobar overinflation. It is characterized by progressive lobar overexpansion that compresses the other adjacent lung lobes. The underlying cause can be an intrinsic cartilaginous abnormality or an extrinsic airway compression. It may be associated with cardiovascular anomalies in 12%-14% of cases and affects males more frequently than females. Most cases are diagnosed postnatally, but early antenatal diagnosis and sequential follow-up are attempted for early treatment, if clinically indicated...
August 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28757838/anesthetic-management-for-lobectomy-of-a-2-month-old-infant-with-bronchogenic-cyst-case-report-along-with-review-of-literature
#8
T Bansal, S Kiran, K Kamal, N Bangarwa
Bronchogenic cyst, a benign congenital cystic lesion of the lung, is a rare cause of respiratory distress in children comprising 7.5% of all mediastinal masses. A thorough preoperative evaluation is crucial to plan for definitive intra- and post-operative management. All patients should be thoroughly evaluated for the presence of compression, deviation or distortion of airways and great veins. The easiest means of providing one lung ventilation in pediatrics is to intubate the main stem bronchus of the nonoperated lung...
July 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/28753246/outcome-of-prenatally-diagnosed-congenital-lung-anomalies-in-the-north-of-england-a-review-of-228-cases-to-aid-in-prenatal-counselling
#9
Lesley Walker, Kelly Cohen, Judith Rankin, David Crabbe
OBJECTIVE: To describe data on congenital lung anomalies identified on prenatal ultrasound from two centres in the North of England. METHOD: This retrospective case series includes all cases notified to the Northern Congenital Abnormality Survey from 1990 to 2010 and to Leeds Regional Fetal Medicine Unit 2000-2015. RESULTS: There were a total of 228 cases, 101 from Northern Congenital Abnormality Survey and 127 from Leeds Regional Fetal Medicine Unit...
July 28, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28683589/infantile-ntrk-associated-mesenchymal-tumors
#10
Jessica L Davis, Christina M Lockwood, Catherine M Albert, Karen Tsuchiya, Douglas S Hawkins, Erin R Rudzinski
Pediatric fibroblastic/myofibroblastic lesions are a relatively common group of tumors with varying morphologies, for which the molecular mechanisms are becoming increasingly well characterized. Congenital infantile fibrosarcoma (CIFS), perhaps the most well studied of these lesions is characterized by a recurrent ETV6-NTRK3 gene fusion. However, a notable subset of locally aggressive congenital/infantile soft tissue lesions with similar morphologic features to CIFS, have not to-date, shown evidence of any canonical molecular aberration...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28638495/an-atypical-presentation-of-congenital-pulmonary-airway-malformation-cpam-a-rare-case-with-antenatal-ultrasound-findings-and-review-of-literature
#11
Munnangi Satya Gautam, Srinivas M Naren Satya, Ivvala Sai Prathyusha, K Hema Chandra Reddy, Kamala Retnam Mayilvaganan, Deepthi Raidu
BACKGROUND: Congenital pulmonary airway malformation (CPAM) is a relatively rare congenital anomaly with a wide spectrum of ultrasound features depending on the specific variety of CPAM. Antenatal ultrasound is a valuable, safe, nonionizing, cost-effective, widely available and easily reproducible imaging tool and is indispensable in the diagnosis of CPAM. In this paper, we aimed to report an atypical imaging presentation of CPAM type II in the second trimester, extensively involving all lobes of the left lung...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28589256/treatment-of-congenital-pulmonary-airway-malformations-a-systematic-review-from-the-apsa-outcomes-and-evidence-based-practice-committee
#12
Cynthia D Downard, Casey M Calkins, Regan F Williams, Elizabeth J Renaud, Tim Jancelewicz, Julia Grabowski, Roshni Dasgupta, Milissa McKee, Robert Baird, Mary T Austin, Meghan A Arnold, Adam B Goldin, Julia Shelton, Saleem Islam
PURPOSE: Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients. METHODS: Questions regarding the management of a pediatric patient with a CPAM were generated. English language articles published between 1960 and 2014 were compiled after searching Medline and OvidSP...
June 6, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28586146/locating-the-level-and-extent-of-congenital-high-airway-obstruction-fluid-in-the-airway-tract-as-reference-points
#13
Mamatha Gowda, Shilpa Gupta, Ashraf Ali, Shanthi Paranthaman
Described here are a series of four cases of congenital high airway obstruction in the fetus. All of the patients presented in the second trimester and all had hydrops fetalis. Three cases had bilateral hyperinflated lungs, midline shift of heart, flattening or inversion of the diaphragm, and fetal ascites. Autopsy was performed in one of these three and showed laryngeal atresia. In one fetus, there was only a unilateral huge enlargement of the lung with mediastinal shift. On autopsy, this fetus had atresia of right main bronchus...
October 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28584070/the-natural-history-of-prenatally-diagnosed-congenital-cystic-lung-lesions-long-term-follow-up-of-119-cases
#14
James Cook, Lyn S Chitty, Paolo De Coppi, Michael Ashworth, Colin Wallis
BACKGROUND: A paucity of evidence regarding the natural history of congenital pulmonary airway malformations (CPAMs) and pulmonary sequestration (PS) has resulted in a divergence in management strategy of asymptomatic cases. METHODS: We describe the long-term clinical course of 119 children diagnosed with these lesions treated at Great Ormond Street Hospital (GOSH). Cases were identified via the GOSH patient database. Study entry required the identification of a cystic lung lesion on prenatal ultrasound and confirmation of CPAM/PS on postnatal CT imaging...
September 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28535027/arsenite-binds-to-the-ring-finger-domain-of-fancl-e3-ubiquitin-ligase-and-inhibits-dna-interstrand-crosslink-repair
#15
Ji Jiang, Marina Bellani, Lin Li, Pengcheng Wang, Michael M Seidman, Yinsheng Wang
Human exposure to arsenic in drinking water is known to be associated with the development of bladder, lung, kidney, and skin cancers. The molecular mechanisms underlying the carcinogenic effects of arsenic species remain incompletely understood. DNA interstrand cross-links (ICLs) are among the most cytotoxic type of DNA lesions that block DNA replication and transcription, and these lesions can be induced by endogenous metabolism and by exposure to exogenous agents. Fanconi anemia (FA) is a congenital disorder manifested with elevated sensitivity toward DNA interstrand cross-linking agents, and monoubiquitination of FANCD2 by FANCL is a crucial step in FA-mediated DNA repair...
July 21, 2017: ACS Chemical Biology
https://www.readbyqxmd.com/read/28493304/cpam-type-2-derived-mesenchymal-stem-cells-malignancy-risk-study-in-a-14-month-old-boy
#16
Gloria Pelizzo, Maria A Avanzini, Marco Folini, Rossana Bussani, Melissa Mantelli, Stefania Croce, Gloria Acquafredda, Paola Travaglino, Graziella Cimino-Reale, Marina Boni, Irene Dambruoso, Valeria Calcaterra
INTRODUCTION: The association between congenital pulmonary airway malformations (CPAM) and malignancy is reported in the literature. Interactions between the tumor, immune, and mesenchymal stromal/stem cells (MSCs) have been recognized as crucial for understanding tumorigenesis. We characterized MSCs isolated from CPAM lesions in order to define potential malignancy risks. METHODS: CPAM II pulmonary tissue was used for MSC expansion; a "healthy" lung section from the same child was used as a comparator...
August 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28438750/intradiaphragmatic-hybrid-lesion-surgical-decision-making-and-value-of-minimal-invasive-surgery
#17
Blanca Schuster, Stefan Gerhard Holland-Cunz, Peter Zimmermann
Hybrid lesions (HLs) have elements of congenital pulmonary airway malformation and extrapulmonary sequestration (EPS) and belong to the congenital lung lesions. EPS usually arises in the thorax or the abdomen but rarely in the diaphragm. The preoperative diagnostic work-up based on chest radiograph, ultrasound (US) and CT often shows imprecise results. Therefore, the exact localisation of the lesion can only be ascertained intraoperatively. Here we present a patient, with an intradiaphragmatic HL, and demonstrate the difficulties of surgical decision making regarding the localisation of the lesion and discuss the value of minimal invasive surgery...
April 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28391172/large-bronchogenic-cyst-of-stomach-a-case-report
#18
Amine Chhaidar, Houssem Ammar, Nihed Abdessayed, Mohamed Azzaza, Rahul Gupta, Nefis Abdennaceur, Ahlem Bdioui, Moncef Mokni, Ali Ben Ali
INTRODUCTION: Bronchogenic cysts are congenital cysts arising as an abnormal budding from primitive tracheobronchial tree. They are lined by pseudostratified columnar or cuboidal ciliated epithelium and contain smooth muscle fibers, submucosal bronchial glands and/or cartilage. They are most frequently located in the mediastinum or the lung parenchyma. Intramural occurrence of bronchogenic cyst in the gastric wall is very rare. PRESENTATION OF CASE: We present a case of 65-year-old lady with a 7×8cm lesion in the gastric cardia suspicious of gastrointestinal stromal tumor...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28293001/-congenital-bronchial-atresia-requiring-differentiation-from-intralobar-sequestration
#19
Yusuke Tanaka, Isao Matsumoto, Daisuke Saito, Syuhei Yoshida, Seiichi Kakegawa, Masaya Tamura, Hirofumi Takemura, Kaori Yoshimura, Hiroko Ikeda
We reported a case of bronchial atresia requiring differentiation from the intralobar sequestration. A 42-year-old man was referred to our institution with suspicion of intralobar sequestration, based on a 3-dimensional computed tomography (CT) angiography that showed abnormal blood vessels from the right inferior phrenic artery flowing into the right lower lobe. CT revealed a lesion between S9 and S10 wherein there were refluxed blood vessels from A9 without an accompanying bronchus, with polycysts and emphysematous changes...
March 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28243740/atypical-pulmonary-sequestration-causing-respiratory-distress-in-a-2-month-old-male-infant
#20
Julio Bauza Rossi
Pulmonary sequestrations are non-functional masses of lung tissue lacking normal communication with the tracheobronchial tree and systemic-circulation-derived blood supply. The disconnection between the normal airway and pulmonary arterial supply prevents pulmonary sequestrations from participating in respiration while their aberrant circulation increases the potential for hemodynamic imbalance. Extralobar sequestrations are prenatally diagnosed, congenital lesions that may become symptomatic before or after birth, whereas intralobar sequestrations are usually identified during adulthood...
February 27, 2017: Pediatric Surgery International
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