Ectopic Pituitary Neuroendocrine Tumors/Adenomas Around the Sella Turcica

Functional or non-secretory ectopic pituitary neuroendocrine tumors (PitNET) can form around the sella turcica during the development of the adenohypophysis by differentiating and detaching from the pharyngeal roof. These tumors usually appear in the sphenoid sinus, clivus, cavernous sinus, infundibulum, and suprasellar cistern. Ectopic PitNETs typically display the characteristic magnetic resonance imaging findings of pituitary adenomas. However, preoperative diagnosis of PitNETs is usually challenging because of the variety of clinical and imaging presentations, locations, and sizes. Ectopic suprasellar PitNETs resemble mass lesions in the pituitary stalk. Ectopic cavernous sinus of PitNETs are typically microadenomas in the medial wall. Ectopic sphenoclival tumors are characterized by more aggressive tumor activity than the other ectopic PitNETs. Although ectopic PitNETs are exceedingly rare, they should be considered as a differential diagnosis for masses around the sella turcica. Treatment of the disease should be individualized and may include medical care, surgical resection, gamma-knife radiosurgery, and radiotherapy.

Clinical features and arrhythmic complications of patients with pediatric-onset arrhythmogenic right ventricular dysplasia.

OBJECTIVE: Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial genetic disease that occurs primarily in the right ventricle. Patients with ARVD may present with severe ventricular arrhythmias, syncope, and cardiac arrest. The purpose of this study is to evaluate the clinical features and arrhythmic complications of patients with pediatric-onset ARVD. METHODS: Patients diagnosed with ARVD between January 2010 and January 2019 were included in this study. RESULTS: A total of 19 patients with ARVD were evaluated. Of them, 15 patients were male, and their mean age was 12±4 years. The most common symptoms were palpitations (n=6), syncope (n=4), and heart failure symptoms (n=2). Five patients were asymptomatic. Thirteen patients had an epsilon wave; all patients ≥14 years had a T wave inversion in V1-3. Premature ventricular contractions (PVCs) were observed in 15 patients, and ventricular tachycardia (VT) was observed in 9 patients. All patients underwent cardiac magnetic resonance imaging (MRI). Echocardiography and cardiac MRI of two patients were normal at the time of admission; patients were in the concealed phase, and the diagnosis was made by ECG, Holter monitoring, and genetic findings. We administered a beta-blocker in all patients. Two patients underwent an electrophysiological study and ablation because of PVC/VT. An implantable cardiac defibrillator was implanted in 8 patients. The mean follow-up period was 21.5±11 months. Two patients were deceased with incessant VT and heart failure, and one patient was deceased with multiorgan dysfunction after biventricular assist device implantation (n=3). CONCLUSION: Diagnosis of pediatric-onset ARVD might be much more difficult in children. Sudden cardiac death might be prevented in the early period by raising the awareness of physicians about the disorder. Prevention of sudden death with implantable cardiac defibrillators is crucial in the management of these patients. It should be kept in mind that children with structurally normal hearts may present with an earlier concealed phase and can be diagnosed with ARVD.

The role of the angle of the ascending aortic curvature on the development of type A aortic dissection: ascending aortic angulation and dissection.

OBJECTIVES: Type A aortic dissection (TAD), which consists of an intimal tear in the aorta, necessitates emergency surgery. Various risk factors related to aortic dissection have been defined in the literature. According to our hypothesis, a narrower angle of ascending aortic curvature (AAAC) may be an additional risk factor in relation to aortic dissection due to the increased force applied to the aortic wall. METHODS: Patients undergoing ascending aortic surgery due to an ascending aortic aneurysm (AsAA) (n = 105) and patients undergoing such surgery because of the occurrence of TAD (n = 101) were enrolled in this study. The AAAC was measured using Cobb's method; the measurements were made on all patients by just 1 cardiovascular radiologist using 3-dimensional computerized tomographic imaging. This measurement was made indirectly by using the aortic valve and brachiocephalic artery to avoid obtaining misleading data as a result of distortions due to dissection. A statistical comparison was also performed relating the traditional risk factors for TAD to other clinical and echocardiographic parameters: the diameter of the ascending aorta and the AAAC. RESULTS: The AAAC was found to be narrower statistically in the TAD group (α = 76.2° ± 17.5°) than it was in the AsAA group (α = 92.9° ± 13°) (P < 0.001). Furthermore, mean ascending aortic diameter (P = 0.019), the presence of a bicuspid aorta (P = 0.007) and aortic valve stenosis (P = 0.005) were higher in the AsAA group. According to multivariable analyses, a narrower AAAC is a significant predictor for the development of TAD (odds ratio 0.93, 95% confidence interval 0.91-0.95; P < 0.001). Overall hospital mortality from various causes including stroke, myocardial infarction, bleeding or renal failure was 13% in the TAD group and 7% in the AsAA group. CONCLUSIONS: According to this study, the AAAC was significantly smaller in aortic dissection patients than in aortic aneurysm patients. This may be related to higher shear stress and elevated pressure on the ascending aorta in patients with a narrower AAAC. Thus, a narrower AAAC may be an additional risk factor in the development of TAD. Therefore, we may need to be more careful in terms of looking for the development of aortic dissection in patients with narrower AAAC.

Three-dimensional transesophageal echocardiography vs cardiac magnetic resonance in the assessment of planimetric mitral valve area in rheumatic mitral stenosis.

AIM: Rheumatic heart disease is a common cause of valvular disease, especially in developing countries. Echocardiography is the gold standard investigation modality for cardiac valves. In rheumatic mitral stenosis (MS), three-dimensional transesophageal echocardiography (3D TEE) provides better alignment of the image plane at the mitral tips and more accurate and reproducible planimetric measurement of mitral valve area (MVA). Cardiac magnetic resonance (CMR) is a new method that provides evaluation of cardiac anatomy and function noninvasively. Previous studies showed strong correlation between planimetric MVA measured by two-dimensional transthoracic echocardiography and CMR. We aimed to compare the planimetric MVAs assessed by 3D TEE and CMR in rheumatic MS patients. To best of our knowledge, this is the first study that compares 3D TEE and CMR for the assessment of the planimetric MVA in rheumatic MS. METHODS: We retrospectively evaluated 28 rheumatic MS patients who underwent 3D TEE and ECG-gated CMR. 3D TEE planimetric MVAs were measured manually by multiplanar reconstruction (MPR) method and CMR planimetric MVAs were measured manually on short-axis cine images. Then, 3D TEE and CMR measurements were compared. RESULTS: A total of 28 patients' (mean age 44 ± 12, 82.1% female) planimetric 3D TEE MVAs (1.00 ± 0.20 cm2 ) and CMR MVAs (1.04 ± 0.17 cm2 ) were found to be highly correlated (P < 0.0001, r: 0.744) with Pearson correlation analysis. Bland-Altman analysis showed strong agreement between two techniques. CONCLUSION: For the diagnosis and the follow-up of rheumatic MS, planimetric CMR MVA is an alternative noninvasive method which highly correlates with planimetric 3D TEE MVA.

An unusual cause of chest pain: An isolated huge cardiac hydatid cyst.

Hydatid disease is a human parasitic infection caused by the larval stage of Echinococcus granulosus. The most common locations for hydatid cysts are the liver and lungs. Cardiac involvement is rare, and isolated cardiac hydatid cysts are even more unusual. We report the case a 48-year-old female patient with an isolated huge cardiac hydatid cyst involving both the left ventricular free wall and the pericardium, and presenting with atypical chest pain. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 46:262-264, 2018.

Effect of Proximal Anastomotic Diameter on Venous Bypass Graft Patency in Patients Undergoing Coronary Artery Bypass Grafting.

BACKGROUND: In this study, the relationship between patency of saphenous vein (SV) graft and different sizes of aorta wall punches was investigated during the follow-up period after coronary artery bypass graft surgery. We also evaluated the other possible factors affecting SV graft patency. METHODS: This study consisted of 266 consecutive and symptomatic patients with postoperative angiography. The primary endpoint was at least one saphenous graft failure observed from coronary computed tomography angiography (cCTA) and/or invasive angiography after surgery. Groups were created as SV occluded and patent group. Survival curves of patients in groups were estimated using Kaplan-Meier method and compared by log-rank test. Multivariate analysis was performed using the Cox proportional hazard model. RESULTS: Cox-regression analysis demonstrated influence of older age (P = .023) and Diabetes Mellitus (DM) (P = .002) on SV graft failure. However, increasing ejection fraction (P = .011) was a protective factor against SV graft failure. There was no significant difference between the two groups in terms of usage rate of the punches with different diameters (P = .296). CONCLUSION: The incidence of SV graft patency does not seem to increase in patients whose 4.8-mm aortic punch was used during proximal anastomosis compared to the reference group in which a punch of 4.0 mm was used. Also, the final proximal anastomosis graft size that was measured using cCTA was similar between patients with 4.8-mm punch and patients with 4-mm punch. Results from this study could help to determine which size for aortosaphenous anastomosis is clinically optimal.

Posttraumatic infrarenal abdominal aortic pseudoaneurysm treated with bifurcated endovascular graft stent.

Posttraumatic infrarenal aortic pseudoaneurysms are rare and potentially lethal lesions. We report the case of a 32-year-old man presenting with infrarenal aortic pseudoaneurysm eight months after being stabbed in the back and right flank. His pseudoaneurysm was close to the iliac bifurcation, so we decided to deploy a bifurcated endovascular graft stent, the TriVascular Ovation endovascular stent. Imaging one month after the procedure revealed no endoleak and slight shrinkage of the pseudoaneurysm.

Arch-first technique via clamshell incision: successful surgical reoperation for aortic arch dissection.

We report a case of successful reoperation for aortic arch dissection with use of the "arch-first" technique in a patient who had Marfan syndrome. Extracorporeal circulation was initiated via right subclavian artery cannulation, and the chest was entered through a clamshell incision for the best exposure. When the patient was cooled to 18 degrees C, the perfusion was stopped. After the 1st aortic arch anastomosis to a 30-mm Dacron graft, cerebral perfusion was reestablished via the right subclavian artery. The aortic repair was then completed. The cerebral ischemic time was 18 minutes, the aortic cross-clamp time was 69 minutes, and the total extracorporeal circulation time was 334 minutes. The patient was discharged from the hospital on postoperative day 10 with no neurologic impairment. The arch-first technique shortens the duration of brain ischemia. When combined with a clamshell incision, the technique is particularly helpful for reoperation of the aortic arch and thoracic aorta.

Primary cerebellar glioblastoma multiforme.

Primary glioblastoma multiforme of cerebellar hemispheres in adults is a rare condition. Most of them result from dedifferentiation of astrocytoma to glioblastoma. We present two cases of unusual de novo cerebellar glioblastomas, one of which is the giant-cell variant. We review their clinical behaviour with conventional MR imaging features and discuss the key findings that can lead to the correct diagnosis in sight of new MR imaging technologies.