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Vascular malformations of the thoracic wall

Amalia Forte, Umberto Galderisi, Marilena Cipollaro, Marisa De Feo, Alessandro Della Corte
The term 'epigenetics' refers to heritable, reversible DNA or histone modifications that affect gene expression without modifying the DNA sequence. Epigenetic modulation of gene expression also includes the RNA interference mechanism. Epigenetic regulation of gene expression is fundamental during development and throughout life, also playing a central role in disease progression. The transforming growth factor β1 (TGF-β1) and its downstream effectors are key players in tissue repair and fibrosis, extracellular matrix remodelling, inflammation, cell proliferation and migration...
August 1, 2016: Clinical Science (1979-)
Ismail Shaik, Anil Karapurkar, Shekhar Bhojraj, Premik Naggad
STUDY DESIGN: A case report. OBJECTIVE: To describe the presentation of compressive paraparesis as a result of thoracic rib hemangioma in a young adult and its nonsurgical management. SUMMARY OF BACKGROUND DATA: Hemangiomas are rare bone tumors and those arising from rib are rarer. Only about 50 such cases have been reported in literature so far. METHODS: A 21-year-old male student, presented to us with a 6-week history of progressive weakness in both lower limbs and loss of bowel bladder control...
November 2015: Spine
L Lampl
In order to achieve a minimal complication rate there is a need for a comprehensive strategy. This means in the first line preventive steps which include patient positioning, suitable approaches and access, an appropriately qualified surgical team as well as a carefully planned dissection and preparation. Furthermore, a supply of additional instrumentation, such as thrombectomy catheters, special vascular clamps and even extracorporeal membrane oxygenation (ECMO) and a heart-lung machine (HLM) in cases of centrally located lesions should be on stand-by...
May 2015: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
Gary Rajah, Chiu Yuen To, Sandeep Sood, Steven Ham, Deniz Altinok, Janet Poulik, Abilash Haridas
Blue rubber bleb nevus syndrome (BRBNS) can present with vascular malformations throughout the body, especially in the gastrointestinal tract. Spinal cord compression from these lesions is rare, particularly in the pediatric population. The authors report a case of BRBNS involving an 18-year-old female patient who presented with back pain and an epidural thoracic mass with cord compression. She underwent an uncomplicated thoracic laminectomy and decompression, with removal of what appeared to be a venous malformation...
November 2014: Journal of Neurosurgery. Pediatrics
Maria Nataatmadja, Jennifer West, Sulistiana Prabowo, Malcolm West
BACKGROUND: The expression of transforming growth factor beta (TGF-β) and Smad3 regulates extracellular matrix homeostasis and inflammation in aortic aneurysms. The expression of Smad3 depends on signaling by angiotensin II (AngII) receptor pathways through TGF-β receptor-dependent and -independent pathways. METHODS: To determine the expression of AngII type 1 (AT1R) and type 2 receptors (AT2R), TGF-β, and Smad3 in thoracic aortic aneurysms, we performed immunohistochemistry testing on tissue and cultured cells derived from subjects with Marfan syndrome (MFS) and bicuspid aortic valve (BAV) malformation and from normal aortas of subjects who were organ donors...
2013: Ochsner Journal
Hiroshi Hoshijima, Risa Takeuchi, Masanori Tsukamoto, Saori Ogawa, Yoshinori Iwase, Nobuyuki Matsumoto
Klippel-Trenaunay syndrome (KTS) is a rare disorder associated with the triad of 1) capillary vascular malformation, 2) varicose veins and/or venous malformation, 3) and soft tissue and/or bony hypertrophy. A six-month old, 6.0-kg-weight male pediatric patient was scheduled for ventriculo-peritoneal shunt operation for hydrocephalus caused by obstructive aqueductus cerebri. At the age of three months, he was diagnosed as KTS by extensive capillary vascular malformation and soft tissue hypertrophy of the right leg...
December 2012: Masui. the Japanese Journal of Anesthesiology
Keisuke Takai, Masaaki Usui
A 49-year-old male presented with a rare case of acute lower extremity paresis caused by spontaneous thrombosis of a spinal conus perimedullary arteriovenous fistula (AVF) after a subacute myelopathic course. Magnetic resonance imaging obtained after deterioration showed that the flow voids around the conus medullaris had changed from hypointense to hyperintense lesions. Surgery with thoracic laminoplasty was performed to determine the nature of the lesion because angiography was negative. During surgery, thrombosed abnormal vessels were observed, consistent with thrombosis of a spinal conus perimedullary AVF...
2012: Neurologia Medico-chirurgica
José Aderval Aragão, José Marcone de Oliveira Pacheco, Larissa Azevedo Silva, Francisco Prado Reis
PURPOSE: The aim of the present study was to determine the frequency of occurrence of multiple renal arteries in human fetuses. METHODS: Sixty kidneys from human fetuses (32 males and 28 females) were used, fixed and kept in 10% formol solution. The fetal age was estimated from the hallux-calcaneus length and ranged from 20 to 37 weeks of gestation, with a mean of 25.63 weeks. The renal arteries were dissected without the aid of optical instruments, and their number, length, topographical layout in relation to their origins in the aorta walls and distribution according to gender and kidney (left or right) were determined...
March 2012: Surgical and Radiologic Anatomy: SRA
Zs Kertész, G Bălă, S Bancu, H Gozar, G Virgil, E Horváth, Z Pávai
Lymphangiomas are uncommun congenital malformations of the lymphatic system, that involve the skin and subcutaneous tissues. Of the several types of treatment, surgical excision has been the preferred. There is a high recurrence rate because lymphangiomas tend to infiltrate the surrounding tissues. The bleomycin is a cytotoxic antitumoral antibiotic, that causes modifications of DNA. It has been also successfully used in intralesional injection treatment of cystic hygromas and haemangiomas, based specifically on a high sclerosing effect on vascular endothelium...
January 2011: Chirurgia
Gioel G Secco, Paolo N Marino, Alessandro Carriero, Giuseppe De Luca
We describe a case of an elderly man known for coronary artery disease (previous bypass surgery) hospitalized for ischemia in inferior wall. Since the operation, the patient underwent coronary angiographies. Due to the impossibility of selective engagement of left subclavian artery from femoral access, LIMA was always visualized through a right radial approach. Despite the suspicious of abnormal origin of left subclavian artery, aortic angiography was never performed. During the third angiography, the double aortic arch was coincidentally visualized by using a left Amplatz catheter...
January 2011: Congenital Heart Disease
G K Paraskevas, A Raikos
We report on a unique combination of multiple variations concerning the pectoral muscles and the left external jugular vein. Specifically, a bilateral hypoplasia of the medial clavicular portion of the pectoralis major muscle was noticed along with the coexistence of total right pectoralis minor aplasia, substituted by loose connective and fatty tissue. Simultaneously, a supernumerary anterior-placed external jugular vein was found, which, after its supraclavicular course, pierced the interval between the left clavicular and the sternocostal head, and drained into the left jugular junction...
August 2010: Folia Morphologica (Warsz)
Sevda Yilmaz, Cansel Atinkaya, Aykut Aktas, Bora Peynircioglu
Congenital arteriovenous malformations are usually found in the lower extremities, but a chest wall location is extremely rare. Extensive vascular malformations present difficulties for patients because of severe unsightliness and life-threatening bleeding. Surgical planning and therapeutic indications in vascular malformations are still a difficult problem. This report describes the case of a 27-year-old woman with a congenital giant arteriovenous malformation of the left chest wall. Preoperative embolization was planned prior to surgical intervention because of the increased risk of massive bleeding, and the malformation was completely embolized with absolute alcohol...
December 2010: Surgery Today
Hiroshi Yamamoto, Fumio Yamamoto, Fuminobu Tanaka, Kazuyuki Ishibashi, Gembu Yamaura, Keisuke Shiroto, Mamika Motokawa, Hiroshi Nanjo
We describe a rare case of an arteriosclerotic aneurysm in the right-sided descending thoracic aorta with a left-sided aortic arch and concomitant aberrant right subclavian artery. A 76-year-old woman, who was found to have an aneurysm of the right-sided descending thoracic aorta, was referred to our hospital for surgical treatment. Contrast computed tomography scan revealed a left-sided aortic arch with an aberrant right subclavian artery, a descending thoracic aorta passing downward behind the esophagus, and an aneurysm of the right-sided and distal (level between the 8th and 10th vertebral bodies) descending thoracic aorta...
August 2010: Annals of Vascular Surgery
Kwang-Jong Lee, Shoichiro Kamagata, Seiichi Hirobe, Miki Toma, Takuo Furukawa, Naoki Fukushima, Yukihiro Inomata
We report a rare case of coexisting pulmonary artery sling, congenital tracheal stenosis, and dextrocardia caused by right lung hypoplasia. Successful treatment of severe postoperative tracheomalacia was achieved by aortopexy, aiming displacement of the aortic arch across orthogonally in front of the trachea due to dextrocardia. The aim of this surgery was different from the usual aortopexy for tracheomalacia, which lifts the tracheal wall with the aorta. The three-dimensional evaluation considering the patient's associated malformations led to a successful result...
October 2009: Annals of Thoracic Surgery
Satoshi Tsutsumi, Yukimasa Yasumoto, Masanori Ito, Hidenori Oishi, Hajime Arai
A 62-year-old female suffered severe occipitalgia followed by progressive tetraparesis and bulbar symptoms, although tinnitus in the left ear persisting for more than 1 year resolved spontaneously after the onset. Cerebral magnetic resonance (MR) imaging revealed swelling in the lower brainstem, and cervical T(2)-weighted MR imaging showed diffuse intramedullary hyperintensity in the medulla oblongata extending downward to the upper thoracic cord. Rim-like enhancement was localized at the C2 level after gadolinium administration...
April 2008: Neurologia Medico-chirurgica
António Amador, Isabel Martins, Rosário Massa, J Oliveira Santos
INTRODUCTION: The XYY male has characteristicaly tall stature, behavior problems and speech delay. There may may be an association with nephro-urologic malformations but cardiovascular anomalies are usually not present. CASE REPORT: It is reported a case of a boy with a 47 XYY karyotype with persistent respiratory distress and swallowing difficulties since two months old. On diagnosis workup the fiberoptic bronchoscopy showed a significant pulsatil tracheal obstruction at its right lateral wall and the magnetic resonance imaging of the mediastinum comproved the presence of a vascular ring (right aortic arch with left ligamentum arteriosum)...
November 2007: Acta Médica Portuguesa
F Prefumo, A M Ierullo, E Fulcheri, P L Venturini, P De Biasio, B Thilaganathan
OBJECTIVES: To report the association between thoracic vascular malformations observed in the first trimester of pregnancy and Down syndrome. METHODS: The clinical features were reviewed of seven fetuses undergoing chorionic villus sampling (CVS) for increased nuchal translucency (NT) thickness, in which color Doppler ultrasonography revealed a vascular malformation in the fetal thorax. RESULTS: The crown-rump length of the fetuses ranged from 58 to 78 mm and NT measurements ranged from 2...
September 2005: Ultrasound in Obstetrics & Gynecology
Young Sam Nam, Choong Hyun Lee, Dae Won Chung, Yeo Sung Yoon, Heungshik S Lee, In Se Lee
This report describes coexistence of anomalous branches of the aortic arch and the costocervical vein malformation in a German shepherd dog. The first branch of the aortic arch was a bicarotid trunk that divided into the left and right common carotid arteries. The next branch to leave the aortic arch was a common trunk for the right and left subclavian arteries, a bisubclavian trunk, which was immediately bifurcated. The right subclavian artery passed over the esophagus forming a deep groove, so-called incomplete vascular ring on the dorsal wall of the esophagus...
December 2003: Journal of Veterinary Science
Paul W M Fedak, Mauro P L de Sa, Subodh Verma, Nafiseh Nili, Pedram Kazemian, Jagdish Butany, Bradley H Strauss, Richard D Weisel, Tirone E David
BACKGROUND: Patients with bicuspid aortic valve malformations are at an increased risk of aortic dilatation, aneurysm formation, and dissection. Vascular tissues with deficient fibrillin-1 microfibrils release matrix metalloproteinases, enzymes that weaken the vessel wall by degrading elastic matrix components. In bicuspid aortic valve disease a deficiency of fibrillin-1 and increased matrix metalloproteinase matrix degradation might result in aortic degeneration and dilatation. METHODS: Samples of the pulmonary artery and aorta were obtained from surgical patients with bicuspid aortic valves (n = 21) and tricuspid aortic valves (n = 16)...
September 2003: Journal of Thoracic and Cardiovascular Surgery
C Colpaert, J Bogers, K Hertveldt, P Loquet, J Dumon, P Willems
Limb-body wall complex (LBWC) is a rare, sporadic, congenital defect defined as a combination of at least two of three characteristics: 1. limb defects, 2. anterior body wall defects, and 3. exencephaly or encephalocoele with/without facial clefts. Three pathogenic mechanisms have been proposed: early amnion rupture, vascular disruption and embryonic dysgenesis. In this study we carried out the pathological evaluation of four fetuses with LBWC and their placentas. None of the cases had craniofacial defects...
2000: Pathology, Research and Practice
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