Acute Cardiac Tamponade Secondary to Cardiac Vascular-Type Pleomorphic Leiomyosarcoma: Case Report

Soft tissue sarcomas represent <1% of all neoplasms. Leiomyosarcomas comprise only 5–7% of cases, and only 2% of these are vascular. Vascular leiomyosarcomas are extremely rare and represent only 0.001% of all neoplasms, the venous type being up to 5 times more frequent. Arterial leiomyosarcomas most frequently affect the great vessels, being fatal in most cases. In the reported cases of arterial leiomyosarcomas, the most frequently affected site is the pulmonary artery. We present the clinical case of 2 patients (a 42-year-old woman and a 36-year-old man) with a diagnosis of arterial pleomorphic leiomyosarcoma that conditioned cardiac tamponade as the initial manifestation. As it is an exceptionally rare neoplasm and with few cases reported in the literature, it is important to identify and describe this pathology which, due to the impossibility of offering surgical treatment, represents a therapeutic challenge.

Subaxial cervical Castleman’s disease: A rare cause of myelopathy

Background: Castleman’s disease (CD) is a rare lymphoproliferative disease of unknown origin which rarely affects the spine. Here, we present CD involving a lytic, destructive C3 lesion with extension into the spinal canal contributing to upper cervical cord compression. Notably, the lesion mimicked other primary bone lesions, metastatic tumors, and/or lymphoma. Case Description: A 52-year-old male presented with progressive quadriparesis (i.e. weakness, instability of gait) and loss of dexterity in both hands over 2 weeks. The MRI, X-ray, and CT scans revealed a destructive lytic lesion involving the C3 vertebral body (i.e. including both anterior and posterior elements). The patient underwent a C3 total and C4 partial laminectomy followed by a C2-C4/5 instrumented fusion (i.e. included C2 pedicle screws/laminar screws, and C4/C5 lateral mass fixation). Histopathology showed a lymphoproliferative disorder with follicles of different sizes, central abnormal germinal structures, and a Mantle zone (i.e. expanded germinal centre with concentric layering with an “onionskin” appearance). These findings were all consistent with the diagnosis of CD (i.e. hyaline-vascular type). Conclusion: CD, a rare lymphoproliferative disease of unknown origin rarely affects the spine. Here, we presented a 52-year-old male with a C3 lytic lesion resulting in C3/4 cord compression that favorably responded to a C3/4 laminectomy with posterior instrumented fusion.

Early and Late Outcomes of Cardiovascular Surgery in Patients With Ehlers-Danlos Syndrome

Background Cardiovascular surgical outcomes reports are few for vascular type IV of Ehlers- Danlos Syndrome (vEDS) compared to non-vascular types I-III (nEDS). Methods To define cardiovascular surgical outcomes among adult patients (≥18 years) with EDS types, a review of our institution's in-house STS Adult Cardiac Surgery Database-compliant software and electronic medical records from Mayo Clinic (1993–2019) was performed. Outcomes were compared for vEDS patients and nEDS patients. Demographics, baseline characteristics, operative, in-hospital complications and follow-up vital status were analyzed. Results Over the study time frame, 48 EDS patients underwent surgery (mean age 52.6 ± 14.6 years; 48% females). Of these, 17 patients had vEDS and 31 patients had nEDS. Six patients (12.5%) underwent prior sternotomy. Urgent or emergent surgery was performed in 10 patients (20.8%). Aortic (vEDS 76.5% vs. nEDS 16.1%) and mitral procedures (vEDS 11.8% vs. nEDS 48.4%) were the two most common cardiovascular surgeries performed (p < .01 and p = .007, respectively). Cardiopulmonary bypass time (CPB) (165 ± 18 vs. 90 ± 13 min; p = .015) and aortic cross clamp times (140 ± 14 vs. 62 ± 10 min; p < .001) were longer for vEDS patients. There was 1 (2.1%) early and 7 (14.6%) late deaths; 6 among vEDS and 2 among nEDS patients. Survival at 5 (80% vs. 93%), 10 (45% vs. 84%) and 15 years (45% vs. 84%) was lower in patients with vEDS (p = .015 for each comparison). Conclusion Cardiovascular surgeries are significantly more complex with longer bypass and cross clamp times for type IV vEDS compared to nEDS patients. Reduced overall survival underscores the complexity and fragility of vEDS patients.

Extend aortic repair for acute type A aortic dissection with rupture and malperfusion complicated with Ehlers-Danlos syndrome

Background Ehlers-Danlos syndrome (EDS) is a disorder in the metabolism of fibrillary collagen. Its vascular type (vEDS) frequently develops arterial rupture and aortic dissection. Case presentation The patient was a 54-year-old gentleman who suffered from cardiac tamponade, malperfusion of the left carotid artery and the right lower extremity due to acute type A aortic dissection. Rupture of the aortic root and a huge entry located from the transverse arch to the proximal descending aorta were found. There were no significant deformities at aortic valve, so we tried extended repairs of valve sparing root replacement (VSRR) and total arch replacement (TAR) with frozen elephant trunk (FET) and were successfully performed. He was finally diagnosed as vascular Ehlers-Danlos syndrome (vEDS) by genetic examination. Conclusion Emergency extensive repairs of VSRR and TAR with FET for critical AAAD with rupture and malperfusion was successfully performed for a vEDS patient.

Vascular Ehlers-Danlos syndrome presenting in the ICU as aneurysmal subarachnoid haemorrhage

Vascular Ehlers-Danlos syndrome is caused by mutations of COL3A1 gene coding for type III collagen. The main clinical features involve a propensity to arterial tears leading to several life-threatening conditions and intensive care unit admission. We, herein, report the case of a 34-year-old woman presenting with an aneurysmal subarachnoid haemorrhage. Endovascular coil treatment was attempted; however, the procedure was complicated by dissection of the left iliac artery and abdominal aorta. Hospital management was marked by a series of vascular and haemorrhagic complications. These events, together with some distinctive physical features and medical history, raised the suspicion of vascular type of Ehlers-Danlos syndrome. Neurological evolution was not favourable, and the patient evolved to brain death. Genetic testing was available postmortem and identified a mutation in the COL3A1 gene. This case illustrates the importance of medical history and clinical suspicion for diagnosis, which often goes unnoticed until major complications occur.

Influence of physical exercise on cardiovascular parameters in students with different types of blood flow autoregulation

Objective: to assess cardiovascular parameters in medical students depending on the type of blood flow autoregulation during physical exercise.Material and methods. We examined 58 students whose average age was 19 ± 1.13 years. During the examination, we recorded electrocardiograms (ECG), registered the parameters of central hemodynamics and determined systolic and diastolic blood pressure and heart rate. We performed the comparison of the parameters of the cardiovascular system (CVS) in students with different types of blood flow autoregulation (BFA) (cardiac, cardiovascular and vascular) during physical exercise.Results. The study has identified the dynamics of electrocardiographic and rheographic parameters during physical exercise expressed in excessive activation of energy resources and strain of the CVS functioning in young men with the cardiac type of BFA, which provides adaptation to short-term effects of disturbing environmental factors. On the contrary, the vascular type of BFA is the most economical, and this type of CVS has a wide range of the function mobilization to long-term physical activity.Conclusion. The study has found statistically significant differences in the cardiovascular parameters in the students with different types of blood flow autoregulation during physical activity.

Clinical Case of Castleman’s Disease Located at the Mesorectum

Introduction: A rare benign lymphoid proliferative disorder called Castleman’s disease is the most commonly presented as a solitary mass in the mediastinum, although sometimes with sites at the neck, axilla, mesentery, pancreas, pelvis and retroperitoneum. We report a case of Castleman’s disease in the mesorectum. According to the literature, such localization was visualized just in few cases all over the world. Case History: The patient was a 48-year-old man, who complained of lumbar pain. MRI was performed in 2014 on which the formation in the presacral mass of mesorectal fat (size up to 4,9*5,2*4,0 cm) on the level of S2 vertebrae was found, which is considered as a lymph node with the tendency to increase during 3, 5 years (+1cm). Upon admission to the clinic, differential diagnosis with lymphoma and malignant tumor were carried out. According to the colonoscopy, there were no evidence about any malignancies in the bowel. After laparoscopic removing of the mesorectal tumor and histological examination of the specimen immunohistochemistry assay was recommended. Following the analysis, the hyaline-vascular type of Castleman's disease was confirmed. Chemotherapy wasn’t recommended, because of radical surgical treatment. According to the results of monitoring in two years, no data of recurrence were identified. Conclusion: Castleman’s disease should be taken into consideration in the differential diagnosis of lymphoid formations in mesorectum.

Castleman’s disease: an entity forgotten in ENT

<p class="abstract">Castleman’s disease (CD) usually presents as localized or systemic lymphadenopathy or as an extra nodal mass. The usual site of presentation are mediastinum, retroperitoneum, axilla and mesentery. Only 3 cases of CD have been reported in retro pharyngeal space. We report a case of 20 year old male patient with retropharyngeal mass. He presented with difficulty in swallowing, change in voice and respiratory distress. The mass was removed in-toto transorally after performing elective tracheostomy. The histopathological findings were consistent with hyaline vascular type of CD. He was decannulated after two day and postoperative period was uneventful. Postoperative CT imaging confirmed the complete excision of tumor and patient is on follow up, with no signs of recurrence. The presentation of tumour in the retropharyngeal space which is a rare site of occurrence add to the uniqueness of this case. Unicentric CD has an excellent prognosis and surgery is the management of choice. Its clinical features, histological subtypes, treatment modalities and prognosis are discussed.</p>

Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report

Introduction: Ehlers-Danlos syndrome is a well classified connective tissue disorder recognized by its features of hyperextensibility of joints and hyperelasticity of the skin. However, the rare vascular type (Ehlers-Danlos type IV) is more difficult to identify in the absence, rarity, or subtlety of the classical physical features. Patients presenting to the emergency department (ED) with acute complications of vascular Ehlers-Danlos syndrome may be critically ill, requiring accurate diagnosis and tailored management. Case Report: This report details a case of spontaneous innominate artery pseudoaneurysm rupture in a pediatric patient with previously undiagnosed Ehlers-Danlos syndrome. Initial ED evaluation was followed by urgent operative intervention and subsequent genetic testing to confirm final diagnosis. Conclusion: Due to its high morbidity and mortality, vascular type Ehlers-Danlos syndrome should be considered in the differential for otherwise unexplained spontaneous vascular injury.