Pancreatic pseudotumors associated with multifocal idiopathic fibrosclerosis

Two patients with multifocal idiopathic fibrosclerosis and sclerosing cholangitis developed biliary obstruction due to a fibrotic pancreatic pseudotumor. The masslike fibrosis mimicked pancreatic carcinoma on sonography and cholangio-pancreatography. In one patient sonography was successfully used to assess the response of the pseudotumor to corticosteroid therapy.     

Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis

BACKGROUND: Autoimmune pancreatitis is a unique clinical entity proposed recently, and is sometimes associated with inflammation of other organs. AIMS: To examine the pathophysiology of the pancreas and other organs in patients with autoimmune pancreatitis. PATIENTS AND METHODS: We evaluated clinicopathological findings in six resected and one autopsied patient with autoimmune pancreatitis. The pancreas, peripancreatic tissue, bile duct, and gall bladder were examined histologically and immunohistochemically. Biopsied salivary gland and cervical lymph node of one patient were also examined. We also performed similar immunohistochemical examinations in pancreatectomy specimens from 10 patients with alcoholic chronic pancreatitis and biopsied salivary glands from five patients with Sj?gren's syndrome. RESULTS: Stenosis of the extrahepatic bile duct was detected in all patients. Histological findings were characterised by diffuse lymphoplasmacytic infiltration with marked interstitial fibrosis and acinar atrophy, obliterated phlebitis of the pancreatic veins, and involvement of the portal vein. Immunohistochemically, diffusely infiltrating cells consisted predominantly of CD4 or CD8 positive T lymphocytes and IgG4 positive plasma cells. Similar inflammatory processes also involved the peripancreatic tissue, extrahepatic bile duct, gall bladder, and salivary gland. Lymph nodes were swollen with infiltration of IgG4 positive plasma cells. None of these findings was seen in alcoholic chronic pancreatitis or Sj?gren's syndrome. CONCLUSIONS: The development of the specific inflammations in extensive organs as well as the pancreas in patients with autoimmune pancreatitis strongly suggests a close relationship between autoimmune pancreatitis and multifocal fibrosclerosis.     

A case of IgG4-related multifocal fibrosclerosis complicated by central diabetes insipidus

A 55-years-old man was admitted to our hospital with a 6-month history of general fatigue, purulent nasal discharge, polyuria, and polydipsia. Endocrinological findings revealed central diabetes insipidus (CDI) with mild anterior pituitary dysfunction. Imaging studies revealed thickening of the proximal end of the pituitary stalk just below the third ventricle, a mass in the paranasal sinus, and a mass encompassing the abdominal aorta. Histopathology of the mass in the paranasal sinus revealed abundant IgG4-positive plasma cells, and the IgG4 serum level was markedly elevated. Thus, he was diagnosed with IgG4-related multifocal fibrosclerosis. Therapy with prednisolone resulted in complete resolution of clinical symptoms and reduction in size of the masses in the affected organs. However, CDI remained unchanged. This is the first case in which the cause of CDI was IgG4-related multifocal fibrosclerosis. IgG4-related sclerosing disease should be included in the differential diagnosis of thickening of the pituitary stalk with CDI, and a search for extra-pituitary involvement is essential.     

Idiopathic fibrosclerosis

Idiopathic fibrosclerosis (IF) is an acute and chronic inflammatory cellular infiltration of tissue associated with varying amounts of fibrosis. Clinical prototypes of IF include retroperitoneal fibrosis, mediastinal fibrosis and Reidel's struma of the thyroid. IF has been associated with numerous other diseases in the literature including systemic lupus erythematosus, scleroderma, polyarteritis nodosa and reactions to several drugs, especially, methysergide and several antihypertensive. However, no clear etiologic factor for either the inflammatory component or the fibrosis has been found. We present 2 cases of IF that exemplify the protean nature of the disease and its potential response to immunosuppressive therapy.     

Aortic root abscess--echocardiographic image

Autopsy findings point to a high prevalence of perivalvular abscesses in patients with infective endocarditis. Diagnosis of this complication, which may have therapeutical implications, is difficult. The authors present echocardiographic image obtained in a patient with bacterial endocarditis in whom an aortic root abscess was subsequently found at operation.     

Aortic root subadventitial hematoma

A 51-year-old woman was operated on for aortic valve regurgitation 3 months after experiencing chest tightness awakening her from sleep. Intraoperative findings included turbid dark brown pericardial fluid and a nipple-shaped protrusion on the external aspect of the noncoronary sinus of Valsalva. Histologically, the lesion was enclosed by intact media and adventitia, and represented an organized hematoma. Dilated venules noted adjacent to the lesion were suggestive of an intramural hemangioma. The etiology of this lesion is unclear, but it might be an unusual type of intramural hematoma (IMH) and gives us a hint of an origin of IMH.     

Aortic root dilatation in athletic population

Remodeling of the aortic root may be expected to occur in athletes as a consequence of hemodynamic overload associated with exercise training; however, there are few data reporting its presence or extent. This review reports the current knowledge regarding the prevalence, upper limits, and clinical significance of aortic remodeling induced by athletic training. Several determinants impact aortic dimension in healthy, nonathletic individuals, including height, body size, age, sex, and blood pressure. Of these factors, anthropometric variables have the greatest impact. In athletes, the effect of exercise training appears to have only a modest additional influence on aortic dimension, although previous studies have produced some conflicting results. Specifically, data derived from the largest available athletic cohort suggest that the most hemodynamically intense endurance disciplines (eg, cycling and swimming) are associated with a significant but mild increase in aortic dimensions. Power disciplines, instead, (eg, weight lifting, throwing events) have only trivial, if any, impact. In contrast, selected data from a different athlete population suggest a more significant dimensional aortic remodeling in strength-trained individuals. In our experience, the 99th percentile value of aortic root diameter corresponds to 40 mm in males and 34 mm in females, which can reasonably be considered the upper limits of physiologic aortic root remodeling. However, a small proportion of apparently healthy male athletes (approximately 1%) show aortic enlargement above the upper limits, in the absence of systemic disease (ie, Marfan syndrome). Athletes presenting with aortic enlargement may demonstrate a further dimensional increase in midlife leading to clinically relevant aortic dilatation. Occasionally, dilation may be severe enough to warrant consideration for surgical treatment. Therefore, serial clinical and echocardiographic evaluations are recommended in athletes when aortic root exceeds the sex-specific thresholds.     

Aortic root dynamics are asymmetric

BACKGROUND AND AIM OF THE STUDY: The presence of conformational changes in the aortic root during the cardiac cycle is well known, but precise information on time-related changes at each level of the root is lacking. METHODS: High-resolution, 3D sonomicrometry (200 Hz) was applied in an acute sheep model. Twelve crystals were implanted in eight sheep at each base (n = 3), commissure (n = 3), sinotubular junction (n = 3) and ascending aorta (n = 3). Under stable hemodynamic conditions, geometric changes of the perimeter of each sinus of Valsalva, sinus height, and twist and root tilt angles were time-related to left ventricluar (LV) and aortic pressures. RESULTS: Expansion of the perimeter of the three sinuses of Valsalva was homogeneous, but in significantly different proportions (p < 0.001): the right sinus expanded (+32.4 +/- 2.4%) more than the left (+29.3 +/- 3.2%), and more than the non-coronary (NC) sinus (+25.8 +/- 1.7%). A similar pattern was found for aortic root height: right greater than left, and left greater than NC sinus (p < 0.001). This asymmetry resulted in changes of the root's twist and tilt angles. Although the twist deformation was consistent for each sheep, no general pattern was found. The aortic root tilt angle (between the basal plane and the commissural plane) was 16.3 +/- 1.5 degrees at end-diastole (angle oriented posteriorly and to the left). During systole, it was reduced by 6.6 +/- 0.5 degrees, aligning the LV outflow tract with the ascending aorta. This tilt angle returned to its original value after valve closure. CONCLUSION: Aortic root expansion is asymmetric, generating precise changes in its tilt angle. During systole, tilt angle reduction resulted in a straight cylinder that probably facilitates ejection; during diastole, the tilt angle increased, probably reducing leaflet stress. These findings should impact upon surgical procedures and the design of new prostheses.     

Aortic root disease in tetralogy of Fallot

PURPOSE OF REVIEW: Progressive aortic root dilatation is a recognized feature of tetralogy of Fallot even in patients following initial reparative surgery. The underlying pathophysiology was initially attributed to altered hemodynamics resulting from longstanding volume overloading and stretching of the aortic root from increased right to left shunting. This review explores the pathophysiology and possible mechanisms for the aortic dilatation, and whether these changes are a reflection of the initial hemodynamic stress or a cellular expression of an unrecognized gene associated with conotruncal defects. RECENT FINDINGS: The recent publication of two case reports of aortic aneurysm and dissection in tetralogy of Fallot patients re-emphasized the fact that aortic root dilatation can no longer be regarded as a benign problem in tetralogy of Fallot patients. Findings of intrinsic histological abnormalities in the aortic root and ascending aorta of tetralogy of Fallot patients suggest that intrinsic abnormalities may also play an important causative role. SUMMARY: A better understanding of the pathophysiology will help to formulate future treatment and management strategies in the subgroup of tetralogy of Fallot patients with progressive aortic dilatation.     

主动脉根包裹手术治疗主动脉根部动脉瘤

目的:开发一个相对简单有效、手术风险较小的主动脉根部包裹术,以治疗主动脉根动脉瘤。方法:自2008年6月至2014年12月,25例主动脉根部瘤患者接受主动脉根部包裹术,其中合并主动脉瓣狭窄和/或反流者23例。主动脉瓣环的直径22~32mm,平均(26.30±3.45)mm,主动脉窦的直径45~62mm,平均(52.23±6.87)mm。所有患者进行主动脉根部包裹术,同时合并主动脉瓣成形术或置换术23例。结果:25例围手术期无死亡。CBP时间为55~128min,平均(81.56±15.43)min。出院前超声心动图检查结果显示:5例主动脉瓣轻度反流,20例主动脉瓣功能正常,与术前超声心动图结果对比,术后主动脉环直径21~25mm,平均(22.43±1.35)mm,较术前显著减小(P0.01),主动脉窦直径30~52mm,平均(40.56±4.21)mm,显著减小(P0.001)。随访结果:所有25例患者生存良好,心脏功能(NYHA)均恢复I级,近中期无死亡病例。结论:主动脉根部包裹术结合主动脉瓣成型或置换术是一种外科手术治疗主动脉窦动脉瘤和主动脉瓣疾病新方法。     

升主动脉根部和主动脉弓替换的外科治疗

目的总结并讨论我科15例升主动脉和主动脉弓替换手术的临床体会。方法在中低温体外循环下,应用带瓣人工管道行升主动脉根部替换手术10例。用人工血管和人工瓣自行缝制带瓣管道3例,预制带瓣人工管道6例。1例行主动脉瓣替换加升主动脉补片术,1例行腔内人工血管移植术。冠状动脉移植应用纽扣法6例,直接移植3例,Cabrol法1例。在深低温停循环脑顺行灌注下行人造主动脉弓(用Medox24mm和8mm人工血管自行缝制)替换3例。其中1例DeBakeyⅢ型术后6年的患者复发,DeBakeyI同时行升主动脉根部替换。结果行主动脉根部替换平均主动脉阻断时间130min。行主动脉弓替换主动脉阻断时间为112～240min(平均170min),停循环30～65min(平均46min),脑选择顺行灌注63～92min(平均80min)。无脑及脊髓并发症。声音嘶哑1例。术后早期死亡1例。随访2～8年,病情稳定,术后心脏功能NYHAⅠ级11例,Ⅱ级3例。结论升主动脉根部手术,特别是联合主动脉弓替换,手术复杂,风险大。为确保手术成功,各吻合口要精确吻合,无张力,无扭转,对头臂干的吻合宜采取分支吻合的方法。应用深低温停循环联合选择性脑顺行灌注进行脑保护。     

Lympho-nodal fibrosclerosis in primary lymphedema. Part one: Considerations on lympho-nodal fibrosclerosis in primary lymphedema

Segments of an inferior inguinal ganglion and of an external iliac (upper inguinal) ganglion were microscopically examined in 46 cases of bilateral primary lymphedema and 26 cases of unilateral primary lymphedema. The examination was performed bilaterally and comparatively to a set of ganglions unaffected by lymphedema, during the years 1974-1978. In all the lymph nodes originating from the patients with lymphedema important morphopathological alternations were noticed, chiefly consisting in fibrosis, fibrosclerosis, fat loading, hyalinization processes, giganto-cellular responses, etc., leading even to an aspect of cirrhosis, lympho-nodal pseudo-cirrhosis. These alterations were also found on the healthy side of the patients with unilateral primary lymphedema at the time of the microscopical examination. In the same patient clinical edema appeared in the following years. The degree of the morphopathological alterations was greater in the side of the greater edema and more peculiar in the cases of bulkier edema.