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congenital lung lesions

Akamin Raymond, Ettore Pedretti, Giuseppina Privitera, Cristina Cicero, Giacomo Biasucci
BACKGROUND: Unilateral absence of the pulmonary artery (UAPA) is a rare congenital malformation often associated with other cardiac anomalies; however it may occur as an isolated lesion. Isolated absence of the right pulmonary artery is twice more frequent than that of the left pulmonary artery. Patients with isolated UAPA are usually asymptomatic at birth; thereafter they may develop a progression of symptoms such as exercise intolerance, dyspnea, chest pain, hemoptysis and recurrent pulmonary infections...
February 20, 2018: Italian Journal of Pediatrics
Enrico Danzer, Casey Hoffman, Jo Ann D'Agostino, Matthew M Boelig, Marsha Gerdes, Judy C Bernbaum, Hannah Rosenthal, Lindsay N Waqar, Natalie E Rintoul, Lisa M Herkert, Michael J Kallan, William H Peranteau, Alan W Flake, N Scott Adzick, Holly L Hedrick
BACKGROUND: To evaluate neurodevelopmental outcome in high-risk congenital lung lesions (CLLs) survivors who underwent prenatal intervention or postnatal surgery within the first month of life. METHODS: Forty-five high-risk CLLs survivors underwent assessment using the Bayley Scales of Infant Development, 3rd Edition between 07/2004 and 12/2016. Scores were grouped as average, at-risk, and delayed based on standard deviation intervals. Correlations between outcome and risk factors were analyzed by Fisher's exact test or two-sided t-test as appropriate, with significant p-values <0...
February 10, 2018: Annals of Thoracic Surgery
Felipe Aluja Jaramillo, Fernando R Gutierrez, Federico G Díaz Telli, Sebastian Yevenes Aravena, Cylen Javidan-Nejad, Sanjeev Bhalla
Pulmonary hypertension (PH) is a condition characterized by increased pressure in the pulmonary circulation. It may be idiopathic or arise in the setting of other clinical conditions. Patients with PH tend to present with nonspecific cardiovascular or respiratory symptoms. The clinical classification of PH was recently revised at the World Health Organization symposium in Nice, France, in 2013. That consensus statement provided an updated classification based on the shared hemodynamic characteristics and management of the different categories of PH...
February 12, 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Ji Eun Choi, Soyeoun Lim, Chang Ryul Park, Hee Jeong Cha, Woon-Jung Kwon
RATIONALE: A foregut duplication cyst (FDC) is an uncommon congenital disease. This report presents a case of mediastinal foregut duplication cyst that mimicked a diaphragmatic small bowel hernia. PATIENT CONCERN: A 27-month-old girl was first referred for a mediastinal lesion found incidentally on a chest radiograph. At that time, our impression was cystic lung lesion such as congenital pulmonary airway malformation or pulmonary sequestration. At the age 6 years, she presented with recurrent vomiting...
December 2017: Medicine (Baltimore)
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
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
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
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
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
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
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
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
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
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
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
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)
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
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
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
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
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