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Congenital thoracic malformation

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https://www.readbyqxmd.com/read/29042329/rare-giant-prevertebral-thoracic-myelomeningocele
#1
Bryan D Choi, Katie P Fehnel, William E Butler
Here we report a 72-year-old man who presented with complaint of sudden onset weakness and impaired sensation in the left lower extremity. Radiographic evaluation revealed a congenital malformation with multiple formation defects including a giant thoracic prevertebral myelomeningocele. Following microsurgical dethetering of the spinal cord the patient recovered ambulation with assist. While thoracic myelomeningoceles are themselves rare, in this case the patient presented at a late age, and responded well to conservative management...
October 14, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29033291/controversies-in-poland-syndrome-alternative-diagnoses-in-patients-with-congenital-pectoral-muscle-deficiency
#2
Martijn Baas, Elise B Burger, Dimitri Sneiders, Robert-Jan H Galjaard, Steven E R Hovius, Christianne A van Nieuwenhoven
PURPOSE: Poland syndrome was first described as a deficiency of the pectoral muscle with ipsilateral symbrachydactyly. Currently, numerous case reports describe variations of Poland syndrome in which pectoral muscle deficiency is often used as the only defining criterion. However, more syndromes can present with pectoral muscle deficiency. The aim of this review is to illustrate the diversity of the phenotypic spectrum of Poland syndrome and to create more awareness for alternative diagnoses in pectoral muscle deficiency...
October 13, 2017: Journal of Hand Surgery
https://www.readbyqxmd.com/read/29026365/incidental-finding-of-bronchopulmonary-sequestration-in-a-64-year-old-female
#3
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/28962659/polysplenia-syndrome-with-duodenal-and-pancreatic-dysplasia-in-a-holstein-calf-a-case-report
#4
Daisuke Kondoh, Tomomi Kawano, Tomoaki Kikuchi, Kaoru Hatate, Kenichi Watanabe, Motoki Sasaki, Norio Yamagishi, Hisashi Inokuma, Nobuo Kitamura
BACKGROUND: Laterality disorders of the abdominal organs include situs inversus totalis that mirrors the arrangements of all internal organs and heterotaxy syndrome (situs ambiguus) in which the thoracic or abdominal organs are abnormally arranged. Heterotaxy is often accompanied by multiple congenital malformations, and it generally comprises asplenia and polysplenia syndromes. To our knowledge, polysplenia syndrome has been reported in only three cattle, and computerized tomographic (CT) images of these animals were not obtained...
September 29, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28904674/-a-rare-cause-of-exertional-dry-cough-agenesis-of-the-left-pulmonary-artery-associated-with-pulmonary-hypoplasia
#5
Azzeddine Laaraje, Naima El Hafidi, Chafik Mahraoui
Agenesis of the left pulmonary artery associated with hypoplasia of the ipsilateral lung is a rare congenital malformation in children; it can be discovered fortuitously or because of the presence of recurrent respiratory infections. Diagnosis is based on thoracic angioscanner. Treatment is essentially conservative. We report the case of a 6-year old child with agenesis of the left pulmonary artery associated with hypoplasia of the ipsilateral lung detected because of exertional dry cough.
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28868608/incidental-imaging-findings-of-congenital-rib-abnormalities-a-case-series-and-review-of-developmental-concepts
#6
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/28845003/-diagnosis-of-right-ventricular-outflow-tract-and-intraoperative-lesion-in-patients-with-right-coronary-artery-sinus-rupture-by-transthoracic-echocardiography
#7
Xiongfei Liang, Yifeng Yang, Wei Lin
To investigate the role of thoracic echocardiography in diagnosis and differential diagnosis of the right ventricular outflow tract into the right aorta with right ventricular aneurysm rupture. 
 Methods: A total of 19 patients with sinus rupture caused by ventricular septal defect in the right ventricular outflow road were reviewed. These patients were diagnosed as aortic right coronary sinus by preoperative transthoracic echocardiography from February 2010 to September 2016 in Xiangya Second Hospital and Xiangya Medical College Affiliated Haikou Hospital...
July 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28759082/persistent-left-superior-vena-cava
#8
Kamil W Tyrak, Jakub Holda, Mateusz K Holda, Mateusz Koziej, Katarzyna Piatek, Wieslawa Klimek-Piotrowska
Persistent left superior vena cava (PLSVC) is the most common congenital malformation of thoracic venous return and is present in 0.3 to 0.5% of individuals in the general population. This heart specimen was dissected from a 35-year-old male cadaver whose cause of death was determined as non-cardiac. The heart was examined and we found a PLSVC draining into the coronary sinus. The right superior vena cava was present with a small-diameter ostium. An anomalous pulmonary vein pattern was observed; there was a common trunk to the left superior and left inferior pulmonary veins (diameter 17...
May 23, 2017: Cardiovascular Journal of Africa
https://www.readbyqxmd.com/read/28737279/exercise-capacity-is-not-decreased-in-children-who-have-undergone-lung-resection-early-in-life-for-congenital-thoracic-malformations-compared-to-healthy-age-matched-children
#9
Ashlee Dunn, Kasey Pearce, Robin Callister, Adam Collison, Matthew Morten, Payal Mandaliya, Lauren Platt, Ben Dascombe, Rajendra Kumar, Hiran Selvadurai, Paul D Robinson, Joerg Mattes
PURPOSE: The purpose of this study was to compare (i) the exercise capacity and (ii) lung function prior to and immediately post cardiopulmonary exercise tests (CPET) of children who underwent early life lung resection for Congenital Pulmonary Airway Malformations (CPAM) to healthy control children. METHOD: Eight children with CPAM (four males, age 9.6 ± 1.8 years) and eight control children without respiratory disease (three males, age 9.4 ± 1.4 years) performed a CPET on a cycle ergometer, during which maximal oxygen consumption (V̇O2max ) and heart rate were measured...
July 24, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28709660/effect-of-gastrointestinal-malformations-on-the-outcomes-of-patients-with-congenital-heart-disease
#10
Carlos M Mery, Luis E De León, J Rubén Rodriguez, R Michael Nieto, Wei Zhang, Iki Adachi, Jeffrey S Heinle, Lauren C Kane, E Dean McKenzie, Charles D Fraser
BACKGROUND: The goal of this study was to assess the effect of associated gastrointestinal malformations (GI) on the outcomes of patients undergoing congenital heart operations. METHODS: Neonates and infants with thoracic (esophageal atresia, tracheoesophageal fistula) and abdominal (duodenal stenosis/atresia, imperforate anus, Hirschsprung disease) GI malformations undergoing congenital heart operations between 1995 and 2015 were included. Two control groups were created, one for each group...
July 11, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28683719/microbubbles-in-macrocysts-contrast-enhanced-ultrasound-assisted-sclerosant-therapy-of-a-congenital-macrocystic-lymphangioma-a-case-report
#11
Carlos Menendez-Castro, Maren Zapke, Fabian Fahlbusch, Heiko von Goessel, Wolfgang Rascher, Jörg Jüngert
BACKGROUND: Congenital cystic lymphangiomas are benign malformations due to a developmental disorder of lymphatic vessels. Besides surgical excision, sclerosant therapy of these lesions by intracavitary injection of OK-432 (Picibanil®), a lyophilized mixture of group A Streptococcus pyogenes, is a common therapeutical option. For an appropriate application of OK-432, a detailed knowledge about the structure and composition of the congenital cystic lymphangioma is essential. SonoVue® is a commercially available contrast agent commonly used in sonography by intravenous and intracavitary application...
July 6, 2017: BMC Medical Imaging
https://www.readbyqxmd.com/read/28637939/surgery-of-thymic-tumor-with-persistent-left-superior-vena-cava
#12
Masahiro Yanagiya, Jun Matsumoto, Hirotsugu Hashimoto, Yoshio Suzuki, Hajime Horiuchi
Because a persistent left superior vena cava (PLSVC) is a rare congenital malformation in the thoracic venous system, surgery of the thymus in such patients has rarely been reported. We herein present a case involving a 68-year-old woman who was treated for a thymic tumor adhering to a PLSVC. She underwent complete resection of the thymic tumor through median sternotomy with left-sided video-assisted thoracic surgery. Although the tumor was close to the left phrenic nerve, this nerve was safely preserved. The pathological diagnosis was mucosa-associated lymphoid tissue (MALT) lymphoma of the thymus...
June 22, 2017: Annals of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28626825/phace-s-syndrome-report-of-a-case-with-new-ocular-and-systemic-manifestations
#13
Raheleh Assari, Vahid Ziaee, Sasan Moghimi, Mohammad Reza Akbari, Arash Mirmohammadsadeghi
PURPOSE: To describe an infant with PHACE(S) syndrome [posterior fossa anomalies (P), hemangiomas (H), arterial anomalies (A), cardiac abnormalities and coarctation of aorta (C), eye abnormalities (E), and the sternal defects (S)] with unusual strabismus, congenital glaucoma, and new systemic manifestations. METHODS: A 6-month-old girl was referred with large hemangiomas on the left side of the face. RESULTS: In the ocular examination, right esotropia and hypotropia, and limitation of elevation in adduction in the right eye were seen...
June 2017: Journal of Current Ophthalmology
https://www.readbyqxmd.com/read/28607884/rigid-bronchoscopic-placement-of-fogarty-catheter-as-a-bronchial-blocker-for-one-lung-isolation-and-ventilation-in-infants-and-children-undergoing-thoracic-surgery-a-single-institution-experience-of-27-cases
#14
Sunil Kant Kamra, Ashwin Ashok Jaiswal, Amrish Kumar Garg, Manoj Kumar Mohanty
One-lung ventilation (OLV) is a challenging task in infants and children as few techniques are possible because of narrow anatomy. The aim of this study is to evaluate and experience lung isolation with Fogarty catheters as a bronchial blocker placed by rigid bronchoscope for OLV in infants and children with lung pathologies requiring surgical management in an industrial hospital. This study is a prospective study carried out in J.L.N. Hospital and Research Centre, Bhilai (CG), from January 2011 to December 2014...
June 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28566826/isomerism-in-the-setting-of-the-so-called-heterotaxy-the-usefulness-of-computed-tomographic-analysis
#15
Shumpei Mori, Robert H Anderson, Tatsuya Nishii, Kensuke Matsumoto, Rohit S Loomba
The most complex combinations of congenital cardiac malformations are found in the setting of bodily isomerism. The question remains, however, as to whether evidence of cardiac isomerism is always to be found in the setting of bodily isomerism, also known as "heterotaxy." We have previously shown that, when assessed on the basis of the extent of the pectinate muscles relative to the atrioventricular junctions, there is always isomerism of the atrial appendages in this setting. Doubt has been remained, however, as to whether these cardiac features can accurately be recognized during life...
May 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28450991/-poland-syndrome-about-a-case-and-review-of-the-literature
#16
REVIEW
Meriam Benzalim, Laila Berghalout, Sophia Elfakir, Hicham Jalal
Poland syndrome is a rare congenital malformation associated with various degrees of thoracic and homolateral upper limb abnormalities. We report the case of a 7-year old girl who underwent exploration for depression of the left hemithorax associated with homolateral subclavicular mass. CT scan showed that the deformation of the thoracic wall was related to the absence of left pectoralis major muscle sterno-costal heads insertion associated with agenesis of the pectoralis minor muscle and hypoplasia of the anterior arches of the first six corresponding ribs...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28387106/-when-an-encircling-aortic-arch-anomaly-hides-behind-respiratory-and-digestive-symptoms-in-children
#17
M Solowianiuk, C Soulatges, N Farhat, J Holzki, M-C Seghaye
Aortic arch anomalies are relatively frequent and account for 15 to 20 % of all congenital cardiovascular malformations. They can be discovered in case of symptoms of airway or esophageal compression such as dysphagia, chronic cough, stridor, wheezing and recurrent respiratory infections. We report the cases of two children with encircling aortic arch anomaly. The 3-year-old boy had complained of chronic respiratory symptoms such as laryngitis and asthma since the age of 4 months. The 20-month-old boy presented with inter-mittent stridor, dysphagia and frequent vomiting...
November 2016: Revue Médicale de Liège
https://www.readbyqxmd.com/read/28376682/insertion-of-a-totally-implantable-venous-access-port-in-a-patient-with-persistent-left-superior-vena-cava-plsvc
#18
Julie Van Walleghem, Sofie Depuydt, Stijn Schepers
Persistent left superior vena cava (PLSVC) is a rare congenital variant, but it is the most common venous thoracic malformation, occurring in 0.3-0.5% of the general population. PLSVC is caused by the persistence of the embryological left anterior cardinal vein which normally obliterates and persists as the Marshall ligament. We present the case of a 74-year-old male lung cancer patient, who already underwent an uncomplicated right-hand sided pacemaker insertion in 2006. During implantation of a subcutaneous central venous access port through puncture of the left subclavian vein, a PLSVC was discovered...
April 5, 2017: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/28364011/spectrum-of-spinal-cord-spinal-root-and-brain-mri-abnormalities-in-congenital-zika-syndrome-with-and-without-arthrogryposis
#19
M F V V Aragao, A M Brainer-Lima, A C Holanda, V van der Linden, L Vasco Aragão, M L M Silva, C Sarteschi, N C L Petribu, M M Valença
BACKGROUND AND PURPOSE: Arthrogryposis is among the malformations of congenital Zika syndrome. Similar to the brain, there might exist a spectrum of spinal cord abnormalities. The purpose of this study was to explore and describe in detail the MR imaging features found in the spinal cords, nerve roots, and brains of children with congenital Zika syndrome with and without arthrogryposis. MATERIALS AND METHODS: Twelve infants with congenital Zika syndrome (4 with arthrogryposis and 8 without) who had undergone brain and spinal cord MR imaging were retrospectively selected...
March 31, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28363624/surgical-management-of-hydatid-bilio-bronchial-fistula-by-exclusive-thoracotomy
#20
REVIEW
Sani Rabiou, Marouane Lakranbi, Yassine Ouadnouni, Fabrizio Panaro, Mohamed Smahi
In the industrialized countries, most of Bilio-bronchial fistula are secondary to hepatobiliary trauma, hepatic resection surgery or in the case of congenital malformation of the biliary tract, Bilio-bronchial fistula is recognized as the complication of a number of infectious pathologies such as hydatidosis and hepatic amoebiasis. Among the causes, the Bilio-bronchial fistula of hydatic origin is by far the most frequent especially in the zones of hydatid endemic as Morocco. It is a serious complication of liver hydatid cysts...
May 2017: International Journal of Surgery
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