Quadricuspid Aortic Valve: A Case Report and Review of the Literature

The quadricuspid aortic valve (QAV) is a rare malformation; often isolated, sometimes associated with other heart diseases. Before the era of echocardiography, the diagnosis was made incidentally at autopsy or during surgery of valve replacement. The extensive use of echocardiography has allowed an early and accurate diagnosis of this malformation. In many cases, the transthoracic approach is suitable for the diagnosis but, transesophageal echocardiography is a tool for the accurate definition of the valve anatomy. This review analyzes, after the presentation of a clinical case, the current knowledge on embryogenesis, classification, diagnosis and clinical course of QAV.     

Locked-in Syndrome Due to Meningovascular Syphilis: A Case Report and Literature Review

We herein report a 46-year-old man presenting with locked-in syndrome secondary to meningovascular syphilis. Brain magnetic resonance imaging (MRI) demonstrated multiple acute infarctions in the left ventromedial pons, right basis pontis, and left basal ganglia. His locked-in syndrome was hypothesized to have been caused by thrombosis of the small paramedian branches of the basilar artery due to syphilitic arteritis. This is a unique case of bilateral ventromedial pontine infarction caused by meningovascular syphilis that presented as locked-in syndrome. Meningovascular syphilis should be included in the differential diagnosis of uncommon stroke, particularly in young men.     

Atherosclerotic Aneurysms of the Profunda Femoris Arteries—Case Report and Literature Review

The case of a 71-year-old man with aneurysms of bilateral profunda femoris arteries associated with so-called “arteriomegaly” is presented. Both aneurysms were resected and only the left lateral femoral circumflex artery was reconstructed. Atherosclerotic aneurysms of the profunda femoris artery are rarely encountered, but once diagnosed, surgical repair is generally recommended to avoid complications. However, there are no cases presenting with complications of diameters less than 2 cm; therefore, we suggest that a profunda femoris aneurysm over 2 cm in diameter is an indication for surgical treatment. If the superficial femoral artery is patent, ligation without reconstruction of the profunda femoris artery does not cause severe leg ischemia; so that in such cases, reconstruction of the profunda femoris artery is not necessary.     

Case Report: Aortoesophageal Fistula Case Report and Review of the Literature

Dubrueil (1) described the first case ofaortoesophageal fistula in 1818. Chiari (2) in 1914described the syndrome of aortoesophageal fistula as atriad of: midthoracic pain or dysphagia followed by a herald hemorrhage and fatalhematemesis. Carter et al (3), in an autopsy review of24 cases of aortoesophageal fistula, found that 80% hada sentinal hemorrhage prior to fatal exsanguination. Wereport a case of an aortoesophageal fistula due to asaccular aneurysm of the descending aorta and review thecauses, clinical manifestations, diagnostic modalities,and treatment options of this uncommon but often fatal cause of upper gastrointestinalhemorrhage.     

系统性红斑狼疮合并主动脉夹层1例及文献复习

目的:探讨系统性红斑狼疮(SLE)合并主动脉夹层(AD)患者的临床特点,夹层形成的病理生理机制及本病的治疗.方法:分析我院收治的1例SLE合并AD以及1969年至今的国内外文献报道共29例.结果:29例中大多为中青年,长期服用激素治疗,大多数伴有高血压.按Stanford分型A型19例,手术治疗8例,全部存活;保守治疗11例,均死亡.B型10例,手术治疗5例(包括1例介入治疗),存活3例;保守治疗5例,存活1例.结论:长期激素治疗、SLE相关的高血压、动脉粥样硬化共同促进了AD的发生.手术治疗的存活率明显高于保守治疗者.     

Mitral Valve Stenosis after Transcatheter Aortic Valve Replacement: Case Report and Review of the Literature

Mitral stenosis is a rare and potentially severe complication of transcatheter aortic valve replacement (TAVR). Given the anatomic coupling and interdependence of the aortic and mitral valves, it comes by itself that procedures (either surgical or percutaneous) involving the aortic valve imply the risk of altering mitral valve function. Indeed, transcatheter aortic prostheses may impair adequate anterior mitral leaflet (AML) opening, especially when implanted in a “low” position, thus resulting in high transvalvular gradients.Hereby, we report the case of a 71-year-old male with symptomatic severe aortic stenosis and a history of previous surgical mitral valve repair who underwent TAVR with a self-expandable prosthesis. Notwithstanding an acceptable angiographic position, the prosthetic frame was shown to interfere with the AML, as evidenced by augmented transmitral gradients; nonetheless, pulmonary artery pressures remained unchanged, and the patient experienced symptomatic improvement. Therefore, a conservative approach was chosen and the patient was discharged home after medical therapy optimization.Moreover, we provide a review of the available literature regarding the incidence, predictors and possible management of this infrequent complication.     

Case Report: Adult Nesidioblastosis A Case Report and Review of the Literature

George F. Laidlaw first described a pancreatic abnormality now known to be the most common cause of persistent hyperinsulinemic hypoglycemia in infants in 1938 (1, 2). The term he coined, nesidioblastosis, is derived from the Greek words for islets (nesidia) and germ (blastos) (3). It accurately describes the characteristic feature of nesidioblastosis, islet cells differentiating and budding from ductal epithelium. In adults, hyperinsulinemic hypoglycemia is rarely caused by nesidioblastosis and is usually caused by insulinoma or exogenous insulin treatment (4, 5). The first case series of adult nesidioblastosis was reported by Harness et al in 1981 (6). Since this case series of six patients, there have been only sporadic literature reports of adult nesidioblastosis, documenting fewer than 20 cases of adult nesidioblastosis over the past 15 years (3, 7-10). This paper presents an adult patient with hyperinsulinemic hypoglycemia due to nesidioblastosis and provides a guide to the diagnosis and treatment of this rare disorder in the adult population.     

Refractory Asthma Coexisting with a Double Aortic Arch: A Case Report and Literature Review

Asthma that is poorly controlled despite adequate treatment should be investigated for medication compliance, treatment adherence, environmental control, asthma mimics, and comorbidities especially in infants and young children. Here, the case of a 2-year-old child with refractory asthma coexisting with a double aortic arch is reported.     

Onset of Aortic Dissection Complicated with Cushing's Disease: A Case Report and Review of the Literature

Cushing''s syndrome and Cushing''s disease cause various metabolic disorders associated with high cortisol levels. Some reports have shown that Cushing''s syndrome is complicated with dissecting aortic aneurysm and aortic dissection after long-term exposure to high cortisol levels. We herein report a rare case of aortic dissection complicated with Cushing''s disease. Aortic dissection may occur even under relatively short periods of high cortisol conditions. This case suggests that hypercortisolemia should be treated as soon as possible in order to prevent aortic dissection in subjects with Cushing''s disease.     

Pregnancy in Marfan Syndrome after Aortic Root Replacement: A Case Report and Review of the Literature

Objective. Marfan syndrome is an inheritable connective tissue disorder with multisystem involvement and variable expression of signs and symptoms, caused by mutations within the fibrillin gene on chromosome 15q21. The reported incidence of severe cardiovascular events during pregnancy in Marfan patients’ series is 3–7%, and the overall risk of fatal complication is approximately 1%. Although acute aortic dissection is a rare complication, an association between pregnancy and aortic dissection has been reported even in the absence of preconception aortic root dilatation. Design. Case report and literature review. Results. A 29‐year‐old pregnant woman with Marfan syndrome after replacement of ascending aorta with aortic valve conservation. She had a normal pregnancy and was delivered at term by cesarean section. Conclusion. Successful pregnancy and delivery can be achieved in Marfan’s patients after aortic root replacement.     

Neuroleptic-Induced Dysphagia: Case Report and Literature Review

Neuroleptic medication may cause extrapyramidal symptoms (EPS) that can affect swallowing as well. This report describes a case of drug-induced dysphagia in a 53-year-old man receiving haloperidol for treatment of schizophrenia. The diagnosis was established by fiberoptic endoscopic evaluation of swallowing. After changing medication to fluphenazin, dysphagic symptoms resolved. The literature on EPS-related dysphagia, its demographic features, typical symptoms, diagnostic modalities, clinical course, and treatment options is reviewed. Rainer Dziewas and Tobias Warnecke contributed equally to this work.     

Endobronchial Lipoma: Case Report and Literature Review

Endobronchial lipomas are rare benign tumors that can cause bronchial obstruction resulting in significant symptoms and post-obstructive parenchymal damage. Accurate diagnosis and treatment are essential to avoid unnecessary morbidity and mortality in these patients. We describe one case of endobronchial lipoma at our institution and include a literature review of endobronchial lipoma cases reported during the time period 2003–2018. Treatment has shifted towards bronchoscopic management and away from surgery for the majority of patients; 64.3% of patients in this review had their lipoma resected bronchoscopically, compared to 30% or less in reviews as recent as 2003. Notably, in cases reported since 2010, 72.7% of cases were managed bronchoscopically. Recurrence rates are low following both bronchoscopic and surgical resection.     

Terbinafine Hepatotoxicity: Case Report and Review of the Literature

We report a patient who developed significant liver dysfunction following therapy with terbinafine. At the end of a 31/2-wk course of terbinafine, he developed progressive jaundice and pruritus. His serum bilirubin peaked at 30.9 mg/dl 3 wk after discontinuing terbinafine. A liver biopsy revealed mild to moderate mixed cellular infiltrate in the portal tracts, and hepatocellular and canicular cholestasis. His liver tests normalized 100 days after stopping terbinafine.     

Familial Acromegaly: Case Report and Review of the Literature

Familial acromegaly is an exceptional clinical entity when not associated with features of multiple endocrine neoplasia type 1 (MEN1). We report here 3 pedigrees in each of which 2 patients have been shown to develop acromegaly. In 5/6 patients, clinical follow-up, and biological screening allowed to confidently exclude MEN1. Absence of mutation in the MEN1 gene after direct DNA analysis in 2 pedigrees reinforces the conviction that the families do not have MEN1. In families 1 and 2, diagnosis