Spontaneous Cerebro-Spinal Fluid Rhinorrhoea Caused by Sustained Intracranial Hypertension.

Cerebrospinal Fluid (CSF) leakage results from a defect in the skull base, which communicates the subarachnoid space with the nasal cavity. The most common cause of CSF leakage is traumatic, and non-traumatic causes are less common. This case report illustrates a case of a woman who presented to the emergency department with clear fluid pouring from her nose for three weeks with a fever. The patient had pneumococcal meningitis and Idiopathic Intracranial Hypertension (ICH) seven years ago. Computed Tomography (CT) sinuses showed the defect seen on the right side of the cribriform plate, and the Magnetic Resonant Imaging (MRI) confirmed the CSF leakage. The CSF leakage was diagnosed by positive B transferrin.  This case highlights a rare condition that needs early detection and treatment to prevent complications such as ascending meningitis.

Ruptured functioning adrenal tumour, atypical presentation with renal colic and hypertension.

Pheochromocytomas are uncommon tumours that originate in chromaffin cells. They are a representation of 0.1%-1% of all cases of secondary hypertension. Most pheochromocytomas are unilateral and benign, featuring catecholamine production, as well as the production of other neuropeptides. Pheochromocytomas are mostly located in the adrenal gland; the frequency of occurrence is highest between 30 and 50 years of age; however, up to 25% of cases may be linked to multiple endocrine neoplasia type 2, Von-Hippel-Landau disease and type 1 neurofibromatosis in the young.We present a case of ruptured left adrenal pheochromocytoma with an atypical presentation. A 30-year-old male patient presented with severe left flank pain and hypertension. The CT scan of the abdomen showed bleeding from the left adrenal mass, where resuscitation and angioembolisation were done. Embolisation of the inferior and superior arteries was done, but the middle failed. The patient experienced a significant drop in haemoglobin and a haemorrhagic shock post angioembolisation, which called for emergency laparotomy. The patient is currently doing well with an uneventful postoperative course.

Emotional and Clinical Challenges While Dealing With a Blind-Deaf-Mute Patient.

A 43-year-old male, known to be deaf, mute, and blind, presented to the ED accompanied by his daughter. She said that he had multiple episodes of diarrhea and vomiting for two days. At first, it gave the impression of gastroenteritis because the patient also had upper abdomen pain, but later a series of investigations were carried out, including blood tests, electrocardiogram (ECG), chest X-ray, ultrasound abdomen (USG), and CT of the kidney and the urinary bladder (CT KUB), to reach a diagnosis. It was confirmed on CT KUB that the patient had a stone in his ureter, which was causing dysuria and increased urinary frequency leading to multiple bathroom visits and vomiting due to severe pain. The patient was given analgesia, and the stone was removed via ureteroscopy. It was challenging to make a diagnosis due to a lack of communication, and it was an emotionally distressing case for the clinical team.

The usefulness of Veno-Arterial Extracorporeal Membranous Oxygenation in Patients with Cardiogenic Shock.

BACKGROUND: Venoarterial extracorporeal membranous oxygenation is a form of temporary mechanical circulatory support that gets as a salvage technique in patients with cardiogenic shock, we intended to evaluate the effect of (VA ECMO) support on hemodynamics and lactate levels in patients with cardiogenic shock. AIM: The aim of our study is to detect the ability to introduce veno-arterial extracorporeal membranous oxygenation (VA ECMO) as a temporary extracorporeal life support system (ECLS) in our unit, demonstrate the role of ECMO in cardiogenic shock patients regarding improving hemodynamics and microcirculation, and demonstrate the complications and drawbacks in our first center experience regarding VA ECMO. MATERIAL AND METHODS: This was a single-centre observational study that included 10 patients admitted with cardiogenic shock for which VA ECMO was used as mechanical circulatory support. RESULTS: The MAP increased after initiation of the support. It was 41.8 ± 9.3 mmHg and 59.5 ± 6.8 mmHg (P = 0.005). The use of VA ECMO support was associated with a statistically significant decrease in the base deficit (-10.6 ± 4.2 and -6.3 ± 7.4, P = 0.038). The serum lactate declined from 5.9 ± 3.5 mmoL/L to 0.6 ± 4.4 mmoL/L by the use of VA ECMO; a statistically significant change (P = 0.005). CONCLUSIONS: We concluded that VA ECMO as mechanical support for patients with cardiogenic shock might improve mean arterial blood pressure, base deficit and lactate clearance.

Intima-media thickness of the descending aorta in patients with bicuspid aortic valve.

OBJECTIVE: A bicuspid aortic valve (BAV) is associated with accelerated aortic valve disease (AVD) and abnormalities in aortic elasticity. We investigated the intima-media thickness of the descending aorta (AoIMT) in patients with AVD with or without an ascending aortic aneurysm (AscAA), in relation to BAV versus tricuspid aortic valve (TAV) phenotype, type of valve disease, cardiovascular risk factors, and single-nucleotide polymorphisms (SNPs) with a known association with carotid IMT. METHODS AND RESULTS: 368 patients (210 with BAV, 158 with TAV,); mean age 64 ± 13 years) were examined using transesophageal echocardiography (TEE) before valvular and/or aortic surgery. No patient had a coronary disease (CAD). The AoIMT was measured on short-axis TEE images of the descending aorta using a semi-automated edge-detection technique. AoIMT was univariately (P < 0.05) related to age, blood pressure, smoking, creatinine, highly sensitive C-reactive protein, HDL, valve hemodynamics and BAV. In the TAV subgroup it was also associated with the rs200991 SNP. Using multivariate regression analysis, age was the main determinant for AoIMT (P < 0.001), followed by male gender (P = 0.02), BAV was no longer a significant predictor of AoIMT. AoIMT was still related to the rs200991 SNP in TAV (P = 0.034), and to creatinine in BAV (P = 0.019), when other variables were accounted for. CONCLUSIONS: Intima-media thickness of the descending aorta is not affected by aortic valve morphology (BAV/TAV); age is the main determinant of AoIMT. Genetic markers (SNPs) known to influence IMT in the carotid artery seem to correlate to IMT in the descending aorta only in patients with TAV.

Aortic valve type and calcification as assessed by transthoracic and transoesophageal echocardiography.

INTRODUCTION: Aortic valve calcification (AVC) may predict poor outcome. Bicuspid aortic valve (BAV) leads to several haemodynamic changes accelerating the progress of aortic valve (AV) disease. AIMS: To compare the diagnostic accuracy of transoesophageal echocardiography (TEE) and transthoracic echocardiography (TTE) in the assessment of aortic valve phenotype and degree of AVC, with intra-operative evaluation as a reference. METHODS: We examined 169 patients (median age 65 years, 51 women) without significant coronary artery disease undergoing AV and/or aortic root surgery. TTE was performed within a week prior to surgery and TEE at the time of surgery. RESULTS: Compared with surgical AVC assessment, visual evaluation using a 5-grade scoring system and real-time images showed a higher correlation (TTE r = 0·83 and TEE r = 0·82) than visual (TTE r = 0·64 and TEE 0·63) or grey scale mean (GSMn) (TTE r = 0·63 and TEE r = 0·52) assessment of end-diastolic still frames. AVC assessment using real-time images showed high intraclass correlation coefficients (TTE 0·94 and TEE 0·93). With regard to BAV, TEE was superior to TTE with a higher interobserver agreement, sensitivity and specificity (0·86, 92% and 94% versus 0·57, 77% and 82%, respectively). CONCLUSION: Semi-quantitative AVC assessment of real-time cine loops from both TEE and TTE correlated well with intra-operative evaluation of AVC. Applying a predefined scoring system for AVC evaluation assures a high interobserver correlation. TEE was superior to TTE for evaluation of valve phenotype and should be considered when a diagnosis of BAV is clinically important.

Valvular osteoclasts in calcification and aortic valve stenosis severity.

BACKGROUND: Bone remodeling in calcified aortic valves is thought to originate from microfractures at multiple sites of the valve, at which osteoclasts and osteoblasts are recruited. The aim of the present study was to assess circulating mediators of bone homeostasis, correlate them to the severity of stenosis and explore the spatio-temporal distribution of bone turnover in different parts of calcified aortic valve tissue. METHODS AND RESULTS: Plasma and explanted aortic valves were obtained from 46 patients undergoing aortic valve replacement surgery. Plasma levels of tartrate-resistant acid phosphatase (TRAP), receptor activator of nuclear-κB (RANK) ligand and Runt-related transcription factor 2 (Runx2/Cbfa1) exhibited a significant correlation to the severity of aortic stenosis. mRNA levels in normal, thickened and calcified parts of aortic valves assessed by quantitative real-time PCR were significantly elevated in calcified parts of valves for TRAP (5.08 ± 1.6-fold, P<0.001) RANK ligand (8.6 ± 4.2-fold, P<0.001) and RANK (1.98 ± 0.78-fold, P=0.015). In an age, gender and aortic valve anatomy-adjusted multivariable regression analysis the local transcript levels of TRAP correlated significantly with echocardiographic parameters quantifying stenosis severity in early stages, whereas the expression level of Runx2/Cbfa1 was a predictor of the stenosis severity in advanced stages. CONCLUSIONS: These findings suggest a critical role of bone turnover as a determinant of aortic stenosis severity.

Real-time imaging required for optimal echocardiographic assessment of aortic valve calcification.

INTRODUCTION: Aortic valve calcification (AVC), even without haemodynamic significance, may be prognostically import as an expression of generalized atherosclerosis, but techniques for echocardiographic assessment are essentially unexplored. METHODS: Two-dimensional (2D) echocardiographic recordings (Philips IE33) of the aortic valve in short-axis and long-axis views were performed in 185 consecutive patients within 1 week before surgery for aortic stenosis (n = 109, AS), aortic regurgitation (n = 61, AR), their combination (n = 8) or dilation of the ascending aorta (n = 7). The grey scale mean (GSMn) of the aortic valve in an end-diastolic short-axis still frame was measured. The same frame was scored visually 1-5 as indicating that the aortic valve was normal, thick, or had mild, moderate or severe calcification. The visual echodensity of each leaflet was determined real time applying the same 5-grade scoring system for each leaflet, and the average for the whole valve was calculated. Finally, a similar calcification score for the whole valve based on inspection and palpation by the surgeon was noted. RESULTS: Visual assessment of real-time images using the proposed scoring system showed better correlation with the surgical evaluation of the degree of valve calcification (r = 0·83, P<0·001) compared to evaluation of stop frames by visual assessment (r = 0·66, P< 0·001) or the GSMn score (r = 0·64, P< 0·001). High inter- and intra-observer correlations were observed for real-time visual score (both intraclass correlation coefficient = 0·93). CONCLUSION: Real-time evaluation of the level of AVC is superior to using stop frames assessed either visually or by dedicated computer grey scale measurement software.

The feasibility of velocity vector imaging by transesophageal echocardiography for assessment of elastic properties of the descending aorta in aortic valve disease.

BACKGROUND: Velocity vector imaging (VVI) is a novel two-dimensional speckle-based imaging technique for evaluation of tissue deformation. The aim of this study was to determine the feasibility and variability of VVI for the assessment of aortic strain, distensibility, and stiffness in patients with aortic valve disease. METHOD: Eighty-five patients (mean age 66 +/- 11 years) with aortic stenosis (AS) or aortic regurgitation (AR) were examined in the operating room before the operation using transesophageal echocardiography (TEE). The two-dimensional short-axis images and M-mode recordings of the descending aorta were acquired simultaneously with the invasive blood pressure measurement in the radial artery. The TEE images were analyzed off-line using VVI software. RESULTS: In comparison with patients with AS, patients with AR displayed significantly higher circumferential strain (7.6% +/- 4.5% vs. 3.7% +/- 1.9%, P < .001) and distensibility (27.1 +/- 12.8 kPa(-1)10(-3) vs. 17.2 +/- 7.2 kPa(-1)10(-3), P < .001) by VVI and distensibility (32.8 +/- 16.7 kPa(-1)10(-3) vs. 21.7 +/- 10.6 kPa(-1)10(-3), P < .004) by M-mode. Stiffness was higher in AS than AR, as measured by VVI (13.3 +/- 6.0 vs. 10.5 +/- 6.0, P < .01) and M-mode (11.2 +/- 6.1 vs. 10.4 +/- 9.1, P < .048). The correlations between VVI and M-mode distensibility (r = 0.84) and stiffness (r = 0.84) were both highly significant (P < .0001). The VVI strain measurements showed low inter- and intraobserver variability with intraclass correlations greater than 0.95 and coefficients of variation less than 10%. CONCLUSION: VVI-derived strain, distensibility, and stiffness differ significantly between AR and AS and correlate strongly with the corresponding M-mode-derived parameters. VVI is a feasible method for the assessment of the elastic properties of the descending aorta with low variability and has the advantage of incorporating the entire aortic wall circumference in the analysis, consequently accounting for local variations in the elastic properties of the aorta.