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Congenital pulmonary lymphangiectasia

Maxim Itkin
Pulmonary lymphatic diseases have been recognized for many years and have been referred as pulmonary lymphangiectasia, pulmonary lymphangiomatosis, plastic bronchitis, and idiopathic chylothorax or chylopericardium. The lymphatic etiology of these conditions has been determined by detection of cystic lymphatic structures on biopsy or postmortem examination. Development of new imaging techniques such as dynamic contrast-enhanced magnetic resonance lymphangiography has allowed better understanding of pathophysiology of these conditions...
December 2016: Techniques in Vascular and Interventional Radiology
M Kardos, P Martanovic, J Masura
No abstract Keywords.
2016: Bratislavské Lekárske Listy
Cristiane Rúbia Ferreira, Verônica Sibre, Regina Schultz, Ana Maria Andrello Gonçalves Pereira de Melo, Silvia Maria Ibidi, Jackeline Della Torre
Firstly described by Rudolf Virchow in the 19th century, congenital generalized lymphangiectasia is a rare entity characterized by dilation of lymphatic vessels, and was recently classified in primary or secondary lymphangiectasia. Generalized forms may be diagnosed during pre-natal follow-up with ultrasound examination, and, depending on its severity, the newborn outcome is very poor. The authors report the case of a female newborn with a previous diagnosis of fetal hydropsy who was born after a full-term gestation with respiratory failure due to bilateral voluminous pleural effusion and ascites...
October 2015: Autopsy & case reports
David Saul, Karl Degenhardt, Suzanne D Iyoob, Lea F Surrey, Ann M Johnson, Mark P Johnson, Jack Rychik, Teresa Victoria
BACKGROUND: Hypoplastic left heart syndrome (HLHS) is the third most common cause of critical congenital heart disease in newborns, and one of the most challenging forms to treat. Secondary pulmonary lymphangiectasia has been recognized in association with HLHS, an appearance described on fetal MRI as the "nutmeg lung." OBJECTIVE: To investigate the association of fetal nutmeg lung with HLHS survival. MATERIALS AND METHODS: A retrospective search of the fetal MRI database was performed...
April 2016: Pediatric Radiology
Jennifer E Pogoriler, Thomas J Kulik, Alicia M Casey, Christopher W Baird, Mary P Mullen, Kathy J Jenkins, Sara O Vargas
Pulmonary vein stenosis is a rare progressive narrowing of the extrapulmonary pulmonary veins, presenting predominantly in infancy and virtually always lethal. It typically arises following repair of congenital heart disease, particularly anomalous pulmonary venous return. Histologic characterization of pediatric pulmonary vein stenosis, not previously well described, may provide insight into the disease pathobiology. We retrieved archival lung specimens (biopsy, explant, or autopsy) from patients with pediatric pulmonary vein stenosis...
May 2016: Pediatric and Developmental Pathology
Xiaoxia An, Jimei Wang, Xiaolei Zhuang, Jiale Dai, Chengqiu Lu, Xiaotian Li, Yingliu Yan
OBJECTIVE: The objective of this study was to evaluate the clinical characteristics of neonates with hydrops fetalis to improve recognition of the disease. PATIENTS AND METHODS: The clinical data of 10 neonates with hydrops fetalis were retrospectively studied. Prenatal characteristics, causes, clinical features, and prognosis were explored. RESULTS: Eight neonates presenting abnormal nonstress test suffered from severe neonatal asphyxia at birth and were resuscitated by endotracheal intubation...
November 2015: American Journal of Perinatology
Moti M Chowdhury, Subhasis Chakraborty
Congenital lung malformations are a heterogeneous group of anomalies that involve the lungs and tracheobronchial tree (congenital airway pulmonary malformation, bronchial atresia, bronchogenic cyst, congenital lobar overinflation, pulmonary cyst, hamartoma, pulmonary isomerism and azygous lobe), vascular abnormalities (arteriovenous malformations, anomalous pulmonary venous return, pulmonary artery sling, interrupted pulmonary artery, pulmonary varix, pulmonary vein stenosis and pulmonary lymphangiectasia), or frequently both entities (pulmonary sequestration, pulmonary maldevelopment and scimitar syndrome)...
August 2015: Seminars in Pediatric Surgery
D T Malleske, B A Yoder
Congenital chylothorax (CC) can result from a congenital malformation or an acquired obstruction or disruption of the thoracic duct. Recently, oral administration of the phosphodiesterase-5 inhibitor, sildenafil, was reported to be effective in resolving non-pulmonary lymphatic malformations in infants and young children. We report a case of CC in a late preterm infant with congenital pulmonary lymphangiectasia where octreotide was not effective, but management with oral sildenafil was successful.
May 2015: Journal of Perinatology: Official Journal of the California Perinatal Association
Jariya Waisayarat, Chinnawut Suriyonplengsaeng, Chaiyos Khongkhatithum, Mana Rochanawutanon
INTRODUCTION: Nemaline myopathy is a rare genetic muscle disorder defined by the presence of nemaline rods in the muscle fibre sarcoplasm. Congenital nemaline myopathy is the most serious form of the disease's spectrum. CASE PRESENTATION: The affected newborn has no spontaneous movement, fractures at birth and respiratory insufficiency. The present case was a Thai male, floppy at birth with fractures of both humeri and femurs and ventilator-dependent respiration...
2015: Diagnostic Pathology
Giovanni Ruggeri, Francesca Destro, Michela Maffi, Giulio Gregori, Mario Lima
Pulmonary lymphangiectasia (PL) is a rare condition characterized by dilatation of the lymphatic vessels. Post-neonatal PL is usually associated with pleural effusion and should therefore be suspected in the presence of chylothorax. We describe a post-neonatal manifestation of PL in a 7-year-old boy presenting chylothorax. Radiological examinations included thorax X-ray, ultrasound, and computed tomography scans. After the failure of conservative management (maintenance of the chest tube, total parenteral nutrition, administration of somatostatin synthetic analogues) we performed a thoracoscopic massive ligation of the thoracic duct's collateral along with a lung biopsy...
June 2013: European Journal of Pediatric Surgery Reports
Kh Lomauri
Neonatal chylothorax results from the accumulation of chyle in the pleural space and may be either congenital or an acquired condition. Congenital chylothorax is most likely due to abnormal development or obstruction of the lymphatic system. It is often associated with hydrops fetalis. It can be idiopathic or may be associated with various chromosomal anomalies including Trisomy 21, Turner syndrome, Noonan syndrome, and other genetic abnormalities. Congenital pulmonary lymphangiectasia and generalized lymphangiomatosis have also been reported to be associated with congenital chylothorax...
November 2014: Georgian Medical News
Scott Bickel, Nemr Eid, Edward Kim, Russell Chowning, Robert Debski, Adrian O'Hagan, Ronald Morton
Pediatric Student/Resident Case Report PostersSESSION TYPE: Medical Student/Resident Case ReportPRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PMINTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare entity characterized by the diffuse proliferation of capillaries throughout the pulmonary interstitium, bronchioles, and vascular structures. We report on a premature neonate with complex heart disease with persistent radiographic features at several days of life which was ultimately determined to be PCH...
October 1, 2014: Chest
M Gray, K Z Kovatis, T Stuart, E Enlow, M Itkin, M S Keller, H M French
Historically, congenital pulmonary lymphangiectasia (CPL) has had poor treatment outcomes despite aggressive therapy. There are recent reports of ethiodized oil (Lipiodol) lymphangiography successfully treating lymphatic leakage in adults. In this report, we describe two infants with CPL complicated by chylothoraces successfully treated by instillation of ethiodized oil into the lymphatic system. Congenital atresia of the thoracic duct was demonstrated on the lymphangiogram in both patients before treatment...
September 2014: Journal of Perinatology: Official Journal of the California Perinatal Association
Havva Serap Toru, Cem Yasar Sanhal, Gulden Tasova Yilmaz, Irem Hicran Ozbudak, Inanc Mendilcioglu, Gulay Ozbilim
Congenital pulmonary lymphangiectasia (CPL) is a rare congenital disorder that typically presents with intractable respiratory failure in the first few days of life. There is an association non-immun hydrops and CPL. In this study we reviewed four CPL cases between January 2006 and January 2014 among 684 fetal-pediatric autopsies. All cases were in the second trimester. In light microscopy there were marked dilatated channels in the subpleural -peribronchial-subseptal region of the lungs. The channels were lined with flattened cells which were expressing CD 31 and D2-40, negative for CD34...
August 2015: Journal of Maternal-fetal & Neonatal Medicine
Teresa Victoria, Savvas Andronikou
BACKGROUND: A pattern of abnormal signal at fetal MRI may be seen in the setting of primary or secondary congenital lymphangiectasia, manifested as a heterogeneous appearance of the lung parenchyma and the presence of subtle T2-hyperintense branching tubular structures that emanate from the hila. We have named this pattern the nutmeg lung. OBJECTIVE: We describe the nutmeg lung appearance seen in fetal MRI scans, with discussion of possible etiologies and outcomes in a series of eight fetuses...
October 2014: Pediatric Radiology
Joon Ho Hwang, Joo Heon Kim, Jung Ju Hwang, Kyu Soon Kim, Seung Yeon Kim
Congenital pulmonary lymphangiectasia (CPL) is a rare lymphatic pulmonary abnormality. CPL with respiratory distress has a poor prognosis, and is frequently fatal in neonates. We report a case of pneumonectomy for CPL in a newborn. An infant girl, born at 39 weeks' after an uncomplicated pregnancy, exhibited respiratory distress 1 hr after birth, which necessitated intubation and aggressive ventilator care. Right pneumonectomy was performed after her symptoms worsened. Histologic examination indicated CPL. She is currently 12 months old and developing normally...
April 2014: Journal of Korean Medical Science
Zoltán Jakus, Jason P Gleghorn, David R Enis, Aslihan Sen, Stephanie Chia, Xi Liu, David R Rawnsley, Yiqing Yang, Paul R Hess, Zhiying Zou, Jisheng Yang, Susan H Guttentag, Celeste M Nelson, Mark L Kahn
Mammals must inflate their lungs and breathe within minutes of birth to survive. A key regulator of neonatal lung inflation is pulmonary surfactant, a lipoprotein complex which increases lung compliance by reducing alveolar surface tension (Morgan, 1971). Whether other developmental processes also alter lung mechanics in preparation for birth is unknown. We identify prenatal lymphatic function as an unexpected requirement for neonatal lung inflation and respiration. Mice lacking lymphatic vessels, due either to loss of the lymphangiogenic factor CCBE1 or VEGFR3 function, appear cyanotic and die shortly after birth due to failure of lung inflation...
May 5, 2014: Journal of Experimental Medicine
A Legras, P Mordant, F Le Pimpec-Barthes, M Riquet
Primary thoracic lymphatic diseases are both infrequent and probably under diagnosed. The two major forms are lymphangiomas and lymphangiectasias. Lymphangiomas are focal proliferation of well-differentiated lymphatic vessels. Childhood lymphangiomas may follow embryologic disorders. Adult lymphangiomas are more likely secondary to lymphatic obstruction. When associated with typical CT and MRI features, their surgical resection is not mandatory, whereas in case of diagnostic difficulties or related complications, surgical resection is the rule...
October 2013: Revue de Pneumologie Clinique
Sule Yalcin, Arbay Ciftci, Ibrahim Karnak, Saniye Ekinci, Feridun Cahit Tanyel, Mehmet Şenocak
PURPOSE: We aimed to review the pre- and postoperative characteristics of children undergoing pneumonectomy, with special emphasis on the management of surgical complications, in comparison with the current literature findings. PATIENTS AND METHODS: A total of 20 patients who underwent pneumonectomy for various etiologies from 1988 to 2011 were investigated retrospectively with respect to the presenting findings, preoperative evaluation, surgical information, postoperative follow-up, and outcome...
April 2013: European Journal of Pediatric Surgery
Patricia Mele, Shanthy Sridhar
Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder of the lung. CPL can present antenatally as nonimmune hydrops with pleural effusions, which are frequently associated with polyhydraminos. CPL presents in the neonatal period with severe respiratory distress and can be complicated by chylothorax. Diagnosis is difficult, as radiographic findings are nonspecific. Lung biopsy is indicated in some severe cases to diagnose CPL. The majority of infants with this condition rarely survive the neonatal period and are often diagnosed at autopsy...
June 2012: Advances in Neonatal Care: Official Journal of the National Association of Neonatal Nurses
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