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Congenital pulmonary airway malformation

K Schneider
The radiographic technique of pediatric chest X‑rays is substantially different from that in adults. In nearly all cases ap/pa X‑rays are sufficient and lateral radiographs are rarely needed. In the first years of life the thymus may overshadow the heart, the great vessels and the lung hila. The most important anatomical structures essential for diagnosing pathological findings of the lungs and mediastinum are the trachea with the bifurcation and the main bronchi with the adjacent great vessels. For the assessment of distended lungs and intrathoracic consolidations, fundamental knowledge of the anatomy in childhood and malformations which can involve the airways, the lungs, the heart, as well as systemic and pulmonary vessels are indispensable...
March 19, 2018: Der Radiologe
Eric B Jelin, Elizabeth M O'Hare, Tim Jancelewicz, Isam Nasr, Emily Boss, Daniel S Rhee
PURPOSE: We sought to determine optimal timing for CPAM resection within the first year of life. METHODS: We queried the National Surgical Quality Improvement Program pediatric database from 2012 to 2015 for elective CPAM resections on patients less than 1year of age. Patients were divided by age in months: 1-3 (n=57), 4-6 (n=135), and 6-12 (n=214). Patient operative variables and 30-day postoperative outcomes were compared. RESULTS: A total of 406 patients were included with no differences in demographics or comorbidities...
February 10, 2018: Journal of Pediatric Surgery
Ashley Robinson, Rodrigo Romao, Jessica Mills, Dafydd A Davies
PURPOSE: The purpose of this study was to determine practice patterns of Canadian surgeons managing congenital pulmonary airway malformations (CPAMs) and factors influencing practice. METHODS: Pediatric surgeons in Canada were surveyed regarding their experience, evaluation, and management CPAMs, and what factors they feel qualify patients for observation vs resection. Data were summarized, and Fisher's-Exact and Kruskal-Wallis Tests applied where appropriate. RESULTS: Sixty eight percent (n=46) of surgeons responded...
February 10, 2018: Journal of Pediatric Surgery
Christoph Zoeller, Benno M Ure, Jens Dingemann
INTRODUCTION:  Video-assisted thoracoscopic surgery (VATS) has gained broad acceptance among pediatric surgeons. Today, VATS can be regarded as a routine approach for various conditions in neonates and infants. However, there is a lack of information concerning the complications of thoracoscopic pulmonary surgery in neonates and infants. We aimed to review the available data. PATIENTS AND METHODS:  A systematic review of the literature was performed using PubMed...
March 6, 2018: European Journal of Pediatric Surgery
Laurence Collin-Lévesque, Yosra El-Ghaddaf, Madeleine Genest, Martin Jutras, Grégoire Leclair, Etienne Weisskopf, Alice Panchaud, Ema Ferreira
BACKGROUND: Duloxetine and methylphenidate are commonly prescribed for the management of depression and attention-deficit/hyperactivity disorder (ADHD), respectively. However, little information is available concerning their safety during lactation. The purpose of this case series was to provide additional information to the medical literature concerning infant exposure to methylphenidate and duloxetine through breast milk. METHOD: Bioanalytical method (liquid chromatography coupled to mass spectrometry) was developed and validated before its use to determine the concentrations of both medications in breast milk samples...
February 27, 2018: Breastfeeding Medicine: the Official Journal of the Academy of Breastfeeding Medicine
Juneyoug Koh, Euiseok Jung, Se Jin Jang, Dong Kwan Kim, Byong Sop Lee, Ki-Soo Kim, Ellen Ai-Rhan Kim
Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 (GGT → GAT), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia...
January 2018: Korean Journal of Pediatrics
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)
Hiroyuki Kubo, Koichiro Kenzaki, Yasuhide Ishikawa
No abstract text is available yet for this article.
January 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
Abigail Elizabeth Cole, Daniel Heaton, Ahmed Chekairi
Laryngeal tuberculosis (TB) is a rare condition, occurring in less than 1% of patients infected with pulmonary TB. We present a case of a 57-year-old male patient, who presented in extremis with audible stridor, increased work of breathing and cyanosis. In addition, the patient had a complex medical history, including a recent diagnosis of congenital malformation of the epiglottis. Emergency intervention was required to secure the airway, and after initial attempts at intubation were unsuccessful, an emergency tracheostomy was performed...
January 17, 2018: BMJ Case Reports
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
Katerina Dukleska, Erin Teeple, Scott Cowan, Charles Vinocur, Loren Berman
BACKGROUND: Treatment of congenital pulmonary airway malformations (CPAMs) is generally surgical resection; however, there is controversy regarding the optimal timing of surgical intervention, especially in asymptomatic patients. STUDY DESIGN: Using the American College of Surgeons National Surgical Quality Improvement Program-Pediatric Participant Use Files from 2012-2015, children who underwent lung resection for CPAM were identified. Outcomes in children who underwent lung resection during the neonatal period were compared with those who underwent resection beyond the neonatal period, but during the first year of life (non-neonates)...
December 21, 2017: Journal of the American College of Surgeons
Loren Berman, Jordan Jackson, Kristen Miller, Rebecca Kowalski, Paul Kolm, Francois I Luks
BACKGROUND: Pediatric surgeons frequently offer prenatal consultation for congenital pulmonary airway malformation (CPAM) and congenital diaphragmatic hernia (CDH); however, there is no evidence-based consensus to guide prenatal decision making and counseling for these conditions. Eliciting feedback from experts is integral to defining best practice regarding prenatal counseling and intervention. METHODS: A Delphi consensus process was undertaken using a panel of pediatric surgeons identified as experts in fetal therapy to address current limitations...
November 28, 2017: Journal of Pediatric Surgery
Rogelio Cruz-Martínez, Belén Nieto-Castro, Miguel Martínez-Rodríguez, Alma Gámez-Varela, Edgar Ahumada-Angulo, Jonahtan Luna-García, Maria José Pineda-Pérez, Carlos Rebolledo-Fernández
OBJECTIVE: To assess longitudinal intrathoracic changes after fetal laser surgery in fetuses with bronchopulmonary sequestration (BPS) with hydrops and/or hydrothorax. STUDY DESIGN: The presence of intrafetal fluid effusions, the lung mass volume ratio (congenital pulmonary airway malformation volume ratio [CVR]), and the observed/expected lung-to-head circumference ratio (O/E-LHR) of both lungs were evaluated in a cohort of BPS fetuses with hydrops and/or hydrothorax treated with full laser ablation of the feeding artery (FLAFA)...
December 6, 2017: Fetal Diagnosis and Therapy
Andrew H Chon, Moe R Takeda, Juan C Felix, Ramen H Chmait
INTRODUCTION: A congenital pulmonary airway malformation (CPAM) type III may become large enough to cause hydrops fetalis. In such circumstances, the fetus can be treated with open fetal resection, maternal betamethasone administration, or percutaneous sclerotherapy. CASE REPORT: A 24 week gestation fetus with a CPAM type III was treated by percutaneous sclerotherapy using ethanolamine oleate (EO). The EO inadvertently entered the left atrium and ventricle with subsequent fetal bradycardia and demise...
December 2017: Fetal and Pediatric Pathology
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
Toshiaki Takahashi, Florian Friedmacher, Julia Zimmer, Prem Puri
PURPOSE: Congenital diaphragmatic hernia (CDH) and associated pulmonary hypoplasia are thought to be caused by a malformation of the underlying diaphragmatic and airway mesenchyme. GATA binding protein 6 (Gata-6) is a zinc finger-containing transcription factor that plays a crucial role during diaphragm and lung development. In the primordial diaphragm, Gata-6 expression is restricted to mesenchymal compartments of the pleuroperitoneal folds (PPFs). In addition, Gata-6 is essential for airway branching morphogenesis through upregulation of mesenchymal signaling...
March 2018: Pediatric Surgery International
Hyejin Shi, Sungmin Sohn, SungHo Wang, Sungrock Park, SangKi Lee, Song Yi Kim, Sun Young Jeong, Changhwan Kim
Congenital cardiovascular anomalies, such as dextrocardia, persistent left superior vena cava (SVC), and pulmonary artery (PA) sling, are rare disorders. These congenital anomalies can occur alone, or coincide with other congenital malformations. In the majority of cases, congenital anomalies are detected early in life by certain signs and symptoms. A 56-year-old man with no previous medical history was admitted due to recurrent wide QRS complex tachycardia with hemodynamic collapse. A chest radiograph showed dextrocardia...
December 2017: Journal of Korean Medical Science
Brian C Gulack, Harold J Leraas, Brian Ezekian, Jina Kim, Christopher Reed, Obinna O Adibe, Henry E Rice, Elisabeth T Tracy
PURPOSE: Resection of congenital pulmonary airway malformations (CPAMs) is often performed to reduce the risk of recurrent infection and malignant transformation. However, there is substantial variation in the timing of resection. This study was performed to determine the association of age and weight on outcomes following elective resection of CPAMs. METHODS: The American College of Surgeons National Surgical Quality Improvement Program-Pediatric database from 2012 to 2014 was queried for infants undergoing elective resection of a CPAM...
October 9, 2017: Journal of Pediatric Surgery
Chin Tung Lau, Kenneth K Y Wong, Paul Tam
INTRODUCTION: Congenital pulmonary airway malformation (CPAM) is a major indication of lobectomy in children. Early lobectomy had been proposed for the advantage of compensatory lung growth. Despite the increasing use of thoracoscopic lobectomy its effect on postoperative lung function was still not well established in the literature. This study was therefore performed to study the result of postoperative pulmonary function test (PFT) on a medium term basis. MATERIALS AND METHODS: All patients who underwent thoracoscopic lobectomy for CPAM between 2006 and 2010 were recruited into the study...
November 3, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
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
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