Vascular malformations of the thoracic wall

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...

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...

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...

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...

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...

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)...

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...

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...

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)...

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...

In order to inform the pathologic features and their associated anomalies twenty five hearts with common trunk were studied with the segmental sequential system. An anatomico-embryological correlation was made to understand the pathological complex of this malformation. The results were: type I truncus (96%), infundibular ventricular septal defect (96%), displastic truncal valve (28%), ventriculo infundibular fold (92%), left coronary artery arising from the posterior wall of the truncus (75%), right coronary artery arising from their anterior wall (96%), coronary arteries arising from opposite Valsalva sinuses in the tetracuspid valves; the biventricular conection of the truncus was balanced in 60%, prevailing on the right (16%) or on the left (16%) ventricles...

BACKGROUND: The high incidence of aortic disease in subjects with congenital aortic valve malformations suggests a causative relationship between these 2 conditions. The histological observation in aortic dilatation/aneurysm/dissection is Erdheim cystic medial necrosis (CMN), a noninflammatory loss of smooth muscle cells (SMCs), fragmentation of elastic fibers, and mucoid degeneration. METHODS AND RESULTS: To examine whether apoptosis is 1 of the mechanisms underlying CMN and aortic medial layer SMC loss, ascending aortic wall specimens from 32 patients were collected at cardiothoracic surgery and examined by histochemical staining and terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end labeling...

Soft tissue hematomas generally resolve but may persist and develop into slow-growing, organized masses. These chronic expanding hematomas are characterized by a pseudocapsule and a predominantly necrotic central cavity, with foci of newly formed capillaries. These have been called chronic expanding hematomas or Masson's papillary endothelial hyperplasia. These lesions can mimic vascular neoplasms and must be considered in the evaluation of expanding soft tissue vascular malformations.

The term "Limb Body Wall Defect" (LBWD) refers to a variable group of congenital defects having in common abdomino- or thoraco-schisis and limb deficiency. Three general pathogenic mechanisms have been proposed for this disorder: amnion rupture, vascular disruption, and embryonic malformation. We hypothesize that there are subsets of "Limb Body Wall Defect," which have similar structural abnormalities and a common pathogenesis. We report on five cases of LBWD that were selected by using more restrictive criteria...

Congenital anomalies in the adult thorax are frequently asymptomatic and may remain undetected for many years. Whether they then become symptomatic or are discovered as an incidental finding on an imaging study obtained for unrelated reasons, thoracic anomalies may appear as masses or contour abnormalities that mimic other pathology, particularly neoplastic disease. The aim of this review is to discuss and illustrate a wide variety of congenital thoracic anomalies that can potentially present as a mass in the thorax...

BACKGROUND: Vascular rings are a classical cause of tracheal and esophagus compression. We report the case of such an abnormality in an infant with neurofibromatosis. CASE REPORT: A 1 week-old male infant with a familial neurofibromatosis presented a stridor with severe respiratory distress. A vascular ring was demonstrated and operated on. The stridor persisted after surgery. A postoperative oesophagogram and tracheobronchoscopy showed an irregular compression of the oesophageal lumen, thought to be due to a residual extrinsic compression...

The contour of the azygoesophageal recess (AER) as seen with computed tomography (CT) is an important indicator of mediastinal disorders. Radiologists must recognize, however, that the AER contour varies with patient age. The configuration of the AER is dextroconvex in children younger than 6 years, nonconcave (ie, equally divided between convex and straight) in children aged 6-12 years, and concave (or adult-like) in adolescents and young adults (aged 12-20 years). The cause of this variation is not certain; however, chest wall configuration does not seem to be an important factor...

Embolization is well suited to the treatment of a range of vascular abnormalities found only in the thorax. This includes congenital arteriovenous fistulas or malformations affecting the chest wall and the pulmonary and coronary arteries. Acquired bronchial artery anomalies and some types of congenital heart disease are also suitable for embolization. Embolization in the thorax presents problems related to the need to work through or in the heart or to the risk to important branches of the intrathoracic aorta, such as the carotid arteries...

To assess the magnetic resonance (MR) imaging findings of chest wall lesions, images from 45 patients were reviewed by two experienced chest radiologists. The study included 14 benign and 31 malignant lesions. Images were obtained on 1.5-T imagers with use of conventional T1-, proton-density, and T2-weighted sequences in transverse, coronal, and sagittal imaging planes. Where appropriate, motion artifact was minimized with respiratory compensation, spatial presaturation, flow compensation, and cardiac gating...

Percutaneous, nonsurgical interventions using angiographic catheter techniques and radiologic guidance were used in the management of seven cases of various lesions of the chest and lungs. Successful catheter therapy included the embolization of a large, acquired, postinflammatory vascular malformation causing massive hemoptysis and a cavernous hemangioma of the chest wall. Sixteen pulmonary arteriovenous fistulas (one patient), an iatrogenic internal mammary artery-to-innominate vein fistula, and a persistent, postbiopsy bronchopleural fistula were successfully closed...