Soft tissue aneurysmal bone cyst of left hemithorax with review of literature

Soft tissue aneurysmal bone cysts (STABCs) are extremely rare extraosseous counterpart of aneurysmal bone cyst (ABC), with close resemblance to histo-morphologic characteristics of ABC. Here we would like to report a 13-year-old female patient, who presented with a large mass, occupying the entire left hemithorax. Patient underwent resection of the thoracic mass. On histopathological examination, it was found to be a soft tissue ABC. It is a very rare tumor and until date 28 cases have been reported in English literature, to the best of our knowledge. On review of the literature, we found this to be the first case of STABC reported in thoracic cavity. The objective of this case presentation is to provide information regarding clinical presentation, radiological and pathological features, and course of management for this rare disease. Soft tissue ABCs are a new class of tumors, so more extensive research is required to establish standard guidelines for their diagnosis and management, to yield better prognosis.     

HTAD patient pathway: Strategy for diagnostic work-up of patients and families with (suspected) heritable thoracic aortic diseases (HTAD). A statement from the HTAD working group of VASCERN

Heritable thoracic aortic diseases (HTAD) are rare pathologies associated with thoracic aortic aneurysms and dissection, which can be syndromic or non-syndromic. They may result from genetic defects. Associated genes identified to date are classified into those encoding components of the (a) extracellular matrix (b) TGFβ pathway and (c) smooth muscle contractile mechanism. Timely diagnosis allows for prompt aortic surveillance and prophylactic surgery, hence improving life expectancy and reducing maternal complications as well as providing reassurance to family members when a diagnosis is ruled out. This document is an expert opinion reflecting strategies put forward by medical experts and patient representatives involved in the HTAD Rare Disease Working Group of VASCERN. It aims to provide a patient pathway that improves patient care by diminishing time to diagnosis, facilitating the establishment of a correct diagnosis using molecular genetics when possible, excluding the diagnosis in unaffected persons through appropriate family screening and avoiding overuse of resources. It is being recommended that patients are referred to an expert centre for further evaluation if they meet at least one of the following criteria: (1) thoracic aortic dissection (<70 years if hypertensive; all ages if non-hypertensive), (2) thoracic aortic aneurysm (all adults with Z score >3.5 or 2.5–3.5 if non-hypertensive or hypertensive and <60 years; all children with Z score >3), (3) family history of HTAD with/without a pathogenic variant in a gene linked to HTAD, (4) ectopia lentis without other obvious explanation and (5) a systemic score of >5 in adults and >3 in children. Aortic imaging primarily relies on transthoracic echocardiography with magnetic resonance imaging or computed tomography as needed. Genetic testing should be considered in those with a high suspicion of underlying genetic aortopathy. Though panels vary among centers, for patients with thoracic aortic aneurysm or dissection or systemic features these should include genes with a definitive or strong association to HTAD. Genetic cascade screening and serial aortic imaging should be considered for family screening and follow-up. In conclusion, the implementation of these strategies should help standardise the diagnostic work-up and follow-up of patients with suspected HTAD and the screening of their relatives.     

脊柱有关角度的观测及机能意义

对30具男性成人干燥椎骨的体根角、横突间夹角、弓根间夹角及上关节面与横截面冠状面成角进行了测算,并观察了上关节面的方向、形态及横突与上关节突、椎弓根间的相互位置关系。通过综合分析,讨论了不同节段有关角度的差异与重力线,脊柱曲度大小,方向的关系以及在活动节段中的机能意义。     

Gas movement in the nonventilated lung at the onset of single-lung ventilation for video-assisted thoracoscopy

To assess the potential for atmospheric nitrogen to enter the nonventilated lung following the initiation of single-lung ventilation, the nonventilated lung of 10 patients undergoing video-assisted thoracoscopy was connected to the air in a water-filled spirometer, and gas movement out of and back into the lung was measured. Airway pressure from both lungs and pleural pressure from the nonventilated side were also measured. With each breath of positive-pressure ventilation to the ventilated lung prior to the thoracic cavity being opened to the atmosphere, the pressure transmitted to the opposite hemithorax generated a mean (range) tidal movement of gas in the nonventilated lung of 134 (65-265) ml. In addition, ongoing gas exchange resulted in a progressive influx of gas from the spirometer over the 110-120 s measurement period of a mean (range) volume of 155 (70-320) ml. This easily preventable influx of atmospheric nitrogen could, in theory, predispose to arterial desaturation and to delayed lung collapse after the parietal pleura is opened.     

Unusual fractures and dislocations of the axis vertebra

Seven percent of 400 patients with cervical spine fractures and/or dislocations had unusual lesions of the axis. The authors have analyzed axis injuries by review of radiographs and clinical data and have derived a classification of traumatic conditions. Uncommon traumatic axis abnormalities are discussed with reference to incidence, causes, clinical findings, mechanism of injury, and roentgen characteristics.Picker Scholar, James Picker Foundation