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congenital lung lesions

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https://www.readbyqxmd.com/read/29328793/novel-molecular-and-phenotypic-insights-into-congenital-lung-malformations
#1
Daniel T Swarr, William H Peranteau, Jennifer Pogoriler, David B Frank, N Scott Adzick, Holly L Hedrick, Mike Morley, Su Zhou, Edward E Morrisey
RATIONALE: Disruption of normal pulmonary development is a leading cause of morbidity and mortality in infants. Congenital lung malformations are a unique model to study the molecular pathogenesis of isolated structural birth defects as they are often surgically resected. OBJECTIVES: To provide insight into the molecular pathogenesis of congenital lung malformations through analysis of cell-type and gene expression changes in these lesions. METHODS: Clinical data, and lung tissue for DNA, RNA, and histology were obtained from 58 infants undergoing surgical resection of a congenital lung lesion...
January 12, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29284507/a-pediatric-disease-to-keep-in-mind-diagnostic-tools-and-management-of-bronchiectasis-in-pediatric-age
#2
REVIEW
Marcella Gallucci, Emanuela di Palmo, Luca Bertelli, Federica Camela, Giampaolo Ricci, Andrea Pession
Bronchiectasis in pediatric age is a heterogeneous disease associated with significant morbidity.The most common medical conditions leading to bronchial damage are previous pneumonia and recurrent lower airway infections followed by underlying diseases such as immune-deficiencies, congenital airway defects, recurrent aspirations and mucociliary clearance disorders.The most frequent symptom is chronic wet cough. The introduction of high-resolution computed tomography (HRCT) has improved the time of diagnosis allowing earlier treatment...
December 29, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29197847/perforated-neuroenteric-cyst-masquerading-as-congenital-pulmonary-airway-malformation
#3
Sreekar Gundapaneni, Vishesh Jain, Shilpa Sharma, Devendra Kumar Gupta
A 3-month-old child was presented with haemoptysis with respiratory distress. Imaging was suggestive of a cavitary lesion in the lung with surrounding consolidation. Diagnosis of a primary lung pathology like congenital pulmonary airway malformation was considered. Based on clinical suspicion and prior experience, a Tc-99m pertechnetate radionuclide study was performed, which clinched the diagnosis of foregut duplication cyst. Intraoperative findings confirmed the presence of a neuroenteric cyst. The child remains asymptomatic on follow-up awaiting neurosurgical intervention for the intraspinal component of the cyst...
December 2, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29127960/thoracoscopic-lobectomy-for-congenital-lung-lesions
#4
REVIEW
Jarrett Moyer, Hanmin Lee, Lan Vu
Congenital lung lesions (CLLs) comprise a heterogeneous group of developmental and histologic entities often diagnosed on screening prenatal ultrasound. Most fetuses with CLL are asymptomatic at birth; however, the risk of malignancy and infection drives the decision to prophylactically resect these lesions. The authors describe their approach to minimally invasive lobectomy in children with CLLs, postoperative care, and management of procedure-specific complications.
December 2017: Clinics in Perinatology
https://www.readbyqxmd.com/read/29110827/long-term-outcomes-of-congenital-lung-malformations
#5
Nigel J Hall, Michael P Stanton
Congenital lung malformations comprise a group of anatomical abnormalities of the respiratory tree including congenital cystic malformations, bronchopulmonary sequestrations, bronchogenic cyst, bronchial atresia, and congenital lobar emphysema. These anomalies are detected with increasing frequency by pre-natal sonography, but may also present for the first time with symptoms in childhood or later life. When symptomatic, there is little controversy that resection is indicated, which is usually curative. When a lesion is asymptomatic there is greater debate regarding the benefit of resection versus continued observation...
October 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29046943/congenital-pulmonary-airway-malformations-state-of-the-art-review-for-pediatrician-s-use
#6
REVIEW
Claire Leblanc, Marguerite Baron, Emilie Desselas, Minh Hanh Phan, Alexis Rybak, Guillaume Thouvenin, Clara Lauby, Sabine Irtan
Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are diagnosed prenatally, improving the prenatal and immediate postnatal care and ultimately the knowledge on CPAM pathophysiology. CPAM natural evolution can lead to infections or malignancies, whose exact prevalence is still difficult to assess. The aim of this "state-of-the-art" review is to cover the recently published literature on CPAM management whether the pulmonary lesion was detected during pregnancy or after birth, the current indications of surgery or surveillance and finally its potential evolution to pleuro-pulmonary blastoma...
December 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29026365/incidental-finding-of-bronchopulmonary-sequestration-in-a-64-year-old-female
#7
Pichapong Tunsupon, Ayesha Arshad, Sumit Patel, M Jeffery Mador
BACKGROUND: Bronchopulmonary sequestration is a congenital abnormality of the primitive foregut. In adults, the typical age at presentation is 20-25 years. CASE REPORT: A 64-year-old female was referred for evaluation of an 8 × 6-cm right lower lobe cystic lesion. Her medical history was significant for recurrent right lower lobe pneumonia requiring multiple hospitalizations. Her physical examination was significant for crackles at the right lung base. Computed tomography (CT) of the chest with contrast showed cystic changes with thickened septation of the medial segment of the right lower lobe lacking distinct visceral pleura and with arterial supply from the anomalous branch of the thoracic aorta arising near the celiac trunk...
2017: Ochsner Journal
https://www.readbyqxmd.com/read/29023302/complete-resolution-of-lymphoid-interstitial-pneumonia-in-a-patient-with-juvenile-myelomonocytic-leukemia-treated-with-allogeneic-bone-marrow-transplant-killing-2-birds-with-1-stone
#8
Anant Vatsayan, Ravi Talati, Kristen Nagle, Linda Cabral, Sloane Cammock, Amy Dimarino, Rachel Egler, Shahrazad Saab, Jignesh Dalal
Lymphoid interstitial pneumonia (LIP) is a rare disease characterized by benign reactive polyclonal proliferation of bronchus-associated lymphoid tissue after exposure to inhaled or circulating antigen(s), leading to a disease symptomatology similar to idiopathic interstitial pneumonia. Its association with diseases that are caused due to immune dysregulation (autoimmune diseases, congenital/acquired immunodeficiency, and allogeneic bone marrow transplant) and response to immunomodulatory/suppressive medications suggests an immunologic pathophysiology...
October 11, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28974162/antenatal-assessment-and-postnatal-outcome-of-fetal-echogenic-lung-lesions-a-decade-s-experience-at-a-tertiary-referral-hospital
#9
Stefan C Kane, Fabrício Da Silva Costa, Joseph A Crameri, Karen L Reidy, Helen Kaganov, Ricardo Palma-Dias
INTRODUCTION: Fetal echogenic lung lesions (ELL) are the commonest pulmonary pathology diagnosed on antenatal sonography, and include congenital pulmonary airway malformations (CPAMs) and bronchopulmonary sequestrations. This study aimed to evaluate the predictive utility of the CPAM volume ratio (CVR) at presentation in a series of fetuses with ELLs at a tertiary Australian referral hospital. MATERIAL AND METHODS: Retrospective cohort study of all pregnancies with a prenatal diagnosis of an isolated fetal echogenic lung lesion managed at the Royal Women's Hospital, Victoria, Australia, between 2005 and 2015...
October 16, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28891233/fetoscopic-tracheal-occlusion-for-treatment-of-non-isolated-congenital-diaphragmatic-hernia
#10
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...
October 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28884206/neonatal-congenital-lung-tumors-the-importance-of-mid-second-trimester-ultrasound-as-a-diagnostic-clue
#11
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...
December 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28868608/incidental-imaging-findings-of-congenital-rib-abnormalities-a-case-series-and-review-of-developmental-concepts
#12
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
#13
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
#14
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...
November 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
#15
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
#16
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
#17
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
#18
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...
October 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28683589/infantile-ntrk-associated-mesenchymal-tumors
#19
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
#20
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
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