Saccular Dilatation Research Articles

Pulmonary Artery Sarcoma Mimicking a Pulmonary Artery Aneurysm

Pulmonary artery sarcoma is an uncommon neoplasm, and its clinical and radiological presentation usually simulates chronic thromboembolic disease. We present the case of a 77-year-old woman admitted with dyspnea, chest pain, and hemoptysis. A chest computed tomographic scan showed moderate right-sided pleural effusion and a saccular dilatation of the interlobar portion of the right pulmonary artery, which was filled with contrast and surrounded by an irregular soft-tissue attenuation mass, suggesting a ruptured pulmonary artery aneurysm. The patient was operated on. Intraoperatively, a pseudoaneurysm and a solid mass were identified within the oblique fissure around the interlobar artery. Therefore, a right pneumonectomy was performed. Definitive pathologic examination was consistent with pulmonary artery sarcoma. The patient had a good outcome and is free of disease 2 years after surgery.

Gastrointestinal stromal tumours: CT findings

The aim of this study was to identify the predictors of malignancy and to demonstrate the uncommon radiological features of gastrointestinal stromal tumours (GIST) using CT. 30 patients participated in this retrospective study. CT findings were evaluated by two radiologists, and categorical variables were compared using chi(2) or Fisher's exact tests. These tests were used to assess any possible relationships between mitotic index and (i) tumour location, size, contour and growth status, (ii) presence of calcifications, necrosis or lymphadenopathy, (iii) enhancement pattern, (iv) metastasis to the liver or other distant sites and (v) invasion of the mesentery or adjacent organs. Tumours were located in the stomach (n = 22), small bowel (n = 6) and colon (n = 2). The size, localization, enhancement pattern, metastasis to the liver or other distant sites and the cystic-necrotic component of the tumours were all associated with a high mitotic index in statistical analyses (p<0.05). Uncommon radiological findings seen on CT were saccular aneurysmal dilatation, malignant lymphadenopathy and satellite nodules. In conclusion, CT features of GISTs were helpful in identifying predictors of malignant behaviour.

Recurrent sarcoma originating from the pulmonary artery 6 years after extensive thoracic resection

sided aortic arch and Kommerell diverticulum. Kommerell diverticulum (Figure 2) is a saccular aneurysmal dilation at the origin of an aberrant right or left subclavian artery. It is a rare anomaly that occurs in association with a double or left aortic arch and an aberrant right subclavian artery (0.5% of the population) or with a right aortic arch and an aberrant left subclavian artery (0.1%). Embryologically, it represents the persistent distal end of the interrupted fourth aortic arch, between the carotid and subclavian arteries. The question arises as to whether this congenital anomaly increases the likelihood of development of an AEF in response to disease or disruption. Kommerell diverticulum represents a likely area of weakening in the aortic wall that can also compress the esophagus and, in theory, could cause erosion of the structure into each other, especially when disruption of adventitial layers has occurred, such as with trauma. In summary, long-term survival can be achieved with early diagnosis and prompt surgical management of AEF. Because this condition presents in a catastrophic manner, younger and healthier patients are more likely to have better outcomes. In our case, the patient’s young age and the relatively small size of his AEF, as well as prompt diagnosis and surgical treatment, proved the ultimate determinants of success and are why he remains alive to this day.

Patients with Cartilage-Hair Hypoplasia Have an Increased Risk for Bronchiectasis

To assess the prevalence, predictors, and course of bronchiectasis in patients with cartilage-hair hypoplasia. Patients who had undergone high-resolution computed tomography of the lungs or bronchography were included in the study. Hospital records were coorelated for clinical features, respiratory symptoms, and laboratory variables. Imaging studies were correlated for changes suggestive of bronchiectasis and findings correlated with clinical and immunological measurements. The study included 15 patients (5 male; median height Z-score, -7.7) aged from 2 to 39 years (median, 10 years). Cell-mediated immunity was impaired in 79% of the subjects, and humoral immunity was impaired in 71% of the subjects. 8 patients (52%) had bronchiectasis, diagnosed by means of high-resolution computed tomography (n = 6) or bronchography (n = 2). The findings ranged from localized mild dilatation of the airways to severe bronchiectasis with saccular airway dilatation. Bronchiectasis progressed during follow-up in 2 patients. Patients with bronchiectasis tended to have more severe growth failure and more often had defective humoral immunity than the general cartilage-hair hypoplasia population. Patients with cartilage-hair hypoplasia are at risk of the development of bronchiectasis.

Caroli's Disease

Caroli's disease is a rare congenital disorder of the liver characterized by saccular or fusiform dilatation of the intrahepatic biliary ducts. In majority the transmission is autosomal recessive [1] but autosomal dominant inheritance has also been reported [2]. The main clinical features are intermittent abdominal pain, hepatomegaly and recurrent cholangitis [1, 3]. We report an unusual case where in spite of advanced cystic dilations of biliary tree, the patient did not suffer a single episode of cholangitis.

Laringocele: aspecto ultra-sonográfico - relato de caso

Laringoceles são lesões relativamente raras definidas como dilatações anômalas dos sáculos dos ventrículos laríngeos. A classificação usual divide a laringocele em interna, externa e combinada ou mista. Laringoceles internas são as que se localizam medialmente à cartilagem tireóidea e geralmente causam compressão nas bandas ventriculares levando a rouquidão e sintomas compressivos na via aérea. As externas se estendem através da membrana tireóidea, apresentando-se como massas cervicais, e as mistas são as que ocupam as duas regiões, podendo causar ambos os sintomas. O diagnóstico é geralmente feito por tomografia computadorizada e/ou laringoscopia. Apresentamos um caso de laringocele mista em que o diagnóstico foi sugerido no exame de ultra-sonografia, num paciente encaminhado com história de massa cervical.

Caroli Disease

Caroli disease is a rare autosomal recessive disorder characterized by multiple segmental communicating dilatations of the intrahepatic bile ducts affecting all or part of the liver that is often associated with renal disorders. It occurs in 2 forms: the rare isolated variety (type I) characterized by recurrent episodes of cholangitis and absence of periportal fibrosis and the more common variety (type II) associated with congenital hepatic fibrosis and consequently occurs in patients with cirrhosis and portal hypertension with esophageal varices.1Caroli J Diseases of the intrahepatic biliary tree.Clin Gastroenterol. 1973; 2: 147-161PubMed Google Scholar A 27-year-old, otherwise healthy man was admitted to our hospital because of septic fever of 7 days' duration associated with mild pain in the right upper abdominal quadrant and anorexia. Physical examination findings were normal except for tenderness of the right hypochondrium above an enlarged liver. Laboratory test results (reference ranges shown parenthetically) were suggestive of cholangitis: serum alkaline phosphatase, 192 U/L (35-110 U/L); γ-glutamyl transpeptidase, 102 U/L (10-75 U/L); C-reactive protein, 21 mg/dL (<0.50 mg/dL); and white blood cell count, 15.3 × 109/L (3.5-5.0 × 109/L). Magnetic resonance imaging of the abdomen showed hepatomegaly and multiple communicating, irregular, tubular (white arrows on left-hand figure), and cystic (black arrows on left-hand figure) dilatations of the intrahepatic bile ducts mainly involving the right lobe. Splenomegaly and other findings suggestive of portal hypertension were absent, but bilateral renal tubular ectasia was evident. Magnetic resonance cholangiopancreatography also showed communications between saccular dilatations of the intrahepatic biliary tract and normal bile ducts (white arrows on right-hand figure). The gallbladder (black star on right-hand figure) was dilated but without thickened walls, and the extrahepatic bile duct was normal (white arrowhead on right-hand figure). These findings were indicative of type I Caroli disease.2Levy AD Rohrmann Jr, CA Murakata LA Lonergan GJ Caroli's disease: radiologic spectrum with pathologic correlation.AJR Am J Roentgenol. 2002; 179: 1053-1057Crossref PubMed Scopus (118) Google Scholar The patient recovered after a course of ceftriaxone, but during the following 4 months, he experienced at least 5 other episodes of cholangitis. His clinical status stabilized, and he is currently awaiting liver transplantation.

Clinical characteristics of Caroli’s disease

Caroli's disease is a rare congenital condition chara-cterized by non-obstructive saccular or fusiform dilatation of larger intrahepatic bile ducts. Cholangitis, liver cirrhosis, and cholangiocarcinoma are its potential complications. The diagnosis of Caroli's disease depends on demonstrating that the cystic lesions are in continuity with the biliary tree which can be showed by ultrasonography, computerized tomography, endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography or magnetic resonance cholangiopancreatography. Treatment of Caroli's disease relies on the location of the biliary abnormalities. While localized forms confined to one lobe can be treated with surgery, liver transplantation is the only effective modality for diffuse forms. Although a rare disorder; Caroli's disease should always be considered in the differential diagnosis of chronic cholestasis of unknown cause.

Clinical characteristics of Caroli’s syndrome

Caroli's syndrome is characterized by multiple segmental cystic or saccular dilatations of intrahepatic bile ducts associated with congenital hepatic fibrosis. The clinical features of this syndrome reflect both the characteristics of congenital hepatic fibrosis such as portal hypertension and that of Caroli's disease named as recurrent cholangitis and cholelithiasis. The diagnosis depends on both histology and imaging methods which can show the communication between the sacculi and the bile ducts. Treatment consists of symptomatic treatment of cholangitis attacks by antibiotics, some endoscopic, radiological and surgical drainage procedures and surgery. Liver transplantation seems the ultimate treatment for this disease. Prognosis is fairly good unless recurrent cholangitis and renal failure develops.

Intravitreal bevacizumab (avastin) for radiation retinopathy 53 years after treatment of retinoblastoma.

To report the successful treatment of rubeosis and an atypical polypoidal vascular lesion in a case of radiation retinopathy treated with intravitreal bevacizumab. A 56-year-old woman with a history of retinoblastoma treated with radiation in the right eye was followed up for a progressively enlarging lesion in the macula that was associated with subretinal fluid. The patient was examined with serial ultrasound and clinical examinations and underwent pupilloplasty on the right eye to improve the view to her fundus. Fluorescein angiography revealed a vascular lesion with prominent saccular dilations. Her subsequent course was complicated by a retinal vein occlusion, rubeosis, and decreased vision. A single treatment with intravitreal bevacizumab resulted in regression of the rubeosis and atypical polypoidal vascular lesion and subjective improvement in vision, which persisted for 3 months. Rubeosis and atypical polypoidal neovascularization are known complications of radiation retinopathy, and treatment with intravitreal bevacizumab may be beneficial.

Multi-Focal Colonic Follicular Lymphoma

The gastrointestinal tract is the most common site of primary extranodal involvement of non-Hodgkin's lymphoma (NHL). However, follicular lymphoma, the most common form of low-grade NHL, rarely involves the colon. This case describes a rare occurrence of an uncommon type of follicular lymphoma discovered in the colon on screening colonoscopy. Case: An apparently healthy 61-year-old male who presented to the endoscopy center for screening colonoscopy. The patient was asymptomatic upon presentation. Family history revealed a grandfather who was diagnosed with colon carcinoma. Physical exam was normal. The colonoscopy showed a 2.5-cm flat colon polyp in the proximal ascending colon, which was removed with snare electrocautery; a 4-mm sessile colon polyp in the sigmoid colon; and five 3–5-mm submucosal polyps at the rectosigmoid junction and rectum, which were biopsied with cold biopsy forceps. Biopsies at each site revealed multi-focal colonic follicular lymphoma, grade II/III. Immunohistochemical staining revealed a CD5+, CD10+, and BCL2+ neoplasm. Heme profile with differential and basic metabolic panel were normal. The patient underwent a CT of the abdomen which showed thickening of the small bowel with saccular dilation suspicious for small bowel lymphoma, numerous mesenteric and para-aortic lymph nodes, and a 1.3-cm splenic granuloma. A capsule enteroscopy was performed and revealed polyps in the proximal jejunum, and erythematous areas of uncertain etiology in the distal small bowel. A PET scan revealed a focal area of increased uptake in the rectum. Bilateral bone marrow aspirations and biopsies were obtained which revealed no evidence of disease. The patient is currently under the care of an oncologist. Discussion: The most common site of primary extranodal involvement of non-Hodgkin's lymphoma is the gastrointestinal tract, with the stomach being the usual site and the colon comprising only 10%. Of this subset of neoplasms, follicular lymphomas, or follicle center lymphomas, represent a mere 1–3%. Pathology of these neoplasms are most commonly CD5-, CD10+, and BCL2+ (t14;18). The preceding case represents a rare occurrence of CD5+ and CD10+ multi-focal primary extranodal lymphoma discovered in the colon of an asymptomatic individual without physical exam findings (specifically lymphadenopathy or organomegaly) or bone marrow involvement.

3483: Internal jugular vein aneurysm with thrombosis diagnosed on US and CT: A case report

Introduction: Jugular venous malformations are rare and only sporadic cases have been reported in the literature. They are divided into phlebectasias and aneurysms. Jugular phlebectasias are fusiform dilatations, which are usually congenital and are seen most commonly in childhood. Jugular aneurysms are saccular dilatations, which are generally acquired and mostly seen in adults.

ADP-Ribosylation Factor-Like 3 Is Involved in Kidney and Photoreceptor Development

ADP-ribosylation factor-like 3 (Arl3) is a member of a small subfamily of G-proteins involved in membrane-associated vesicular and intracellular trafficking processes. Genetic studies in Leishmania have shown that the Arl3 homolog is essential for flagellum biogenesis. Mutations in a related human family member, Arl6, result in Bardet-Biedl syndrome in humans, which is characterized by genital, renal, and retinal abnormalities, obesity, and learning deficits. As part of our large-scale phenotypic screen, mice deficient for the Arl3 gene were generated and analyzed. Arl3 (-/-) mice were born at a sub-Mendelian ratio, were small and sickly, and had markedly swollen abdomens. These mutants failed to thrive, and all died by 3 weeks of age. The (-/-) mice exhibited abnormal development of renal, hepatic, and pancreatic epithelial tubule structures, which is characteristic of the renal-hepatic-pancreatic dysplasia found in autosomal recessive polycystic kidney disease. Absence of Arl3 was associated with abnormal epithelial cell proliferation and cyst formation. Moreover, mice lacking Arl3 exhibited photoreceptor degeneration as early as postnatal day 14. These results are the first to implicate Arl3 in a ciliary disease affecting the kidney, biliary tract, pancreas, and retina.

Recurrent vulval congenital lymphangioma circumscriptum-a case report and literature review

Lymphangioma circumscriptum (LC) is a defect of lymphatics in deep dermis. This abnormal saccular dilatation of lymphatics occurs in dermal papillae visible as vesicles clinically. The entity may be acquired or congenital. The occurrence of congenital variety of LC in vulva is extremely rare, with only few cases reported in the literature. We communicate one such case presenting as recurrent vulval growth along with a review of literature and treatment modalities.

Incidental Sonographic Finding of Bilateral Ureteroceles

An ureterocele is a congenital saccular dilatation of the terminal aspect of the ureter, arising from abnormal embryogenesis and affecting the early development of the intravesicular ureter, the ipsilateral kidney, and its collecting system. This is one of the more challenging urologic abnormalities facing pediatric and adult urologists, posing a diagnostic and therapeutic dilemma because of the many variations of anatomic abnormalities and nonspecific clinical symptoms.

Role of estrogen deficiency in the formation and progression of cerebral aneurysms. Part II: experimental study of the effects of hormone replacement therapy in rats

The increased incidence of cerebral aneurysms in postmenopausal women appears to be related to low levels of circulating estrogen. Using a rat model of aneurysm induction, the authors found that oophorectomy increased the incidence of experimental cerebral aneurysms (Part I in this issue). In the current study they examined the effects of hormone replacement therapy (HRT) on the formation of cerebral aneurysms in rats. Forty-five female Sprague-Dawley rats were divided into three equal groups. The animals in Groups A and B were subjected to a cerebral aneurysm induction procedure (renal hypertension and right common carotid artery ligation) followed 1 month later by bilateral oophorectomy. After an additional week the rats in Group A received 17beta estradiol continuous-release pellets. The rats in Group C served as controls. Three months after the aneurysm induction procedure, all the rats were killed and vascular corrosion casts of their cerebral arteries were prepared and checked for aneurysmal changes. Using a scanning electron microscope, the authors recorded aneurysmal changes as endothelial changes alone (Stage I), endothelial changes with intimal pad elevation (Stage II), and saccular aneurysm formation (Stage III). Aneurysmal changes (Stages I, II, and III) occurred in one third of rats that had undergone oophorectomy and were receiving HRT (Group A), compared with 87% of the rats that had undergone oophorectomy but did not receive HRT (Group B). Although most of the aneurysmal changes identified in Group A rats were limited to Stage I or II, most changes in Group B animals were identified as saccular dilation (Stage III). The findings demonstrated the significant protective role of estrogen against the formation and progression of cerebral aneurysms. It appears to be related to the beneficial effects of estrogen on the function and growth of endothelial cells, which play a major role in preserving the integrity of the vascular wall.

An inelastic multi-mechanism constitutive equation for cerebral arterial tissue

Intracranial aneurysms (ICA) are abnormal saccular dilations of cerebral arteries, commonly found at apices of arterial bifurcations and outer walls of curved arterial segments. Histological evidence suggests the stages in ICA development include the deformation of a segment of arterial wall into a "bleb" with no identifiable neck region followed by the development of an aneurysm with a clear neck. Afterwards, the aneurysm may undergo further enlargement, possibly with significant biological response including calcification and thrombosis. Past studies of the biomechanics of cerebral aneurysm tissue have been directed at modeling elastic deformations of pre-existing aneurysms. Taking this approach, the aneurysm wall is treated as a different entity than the arterial tissue from which it developed. In the current work, a nonlinear, inelastic, dual-mechanism constitutive equation for cerebral arterial tissue is developed. It is the first to model the recruitment of collagen fibers and degradation of the internal elastic lamina, two important characteristics of early stage aneurysm formation.

Intrauterine Device Related Hepatic Abscess

Hepatic Actinomycosis is a rare, but well documented cause of liver abscess. Recent literature has demonstrated the relationship of intrauterine devices (IUD) and infection with Actinomycoses. Infections caused by Capnocytophaga species occur infrequently but usually present in patients that are immunocompromised. A 42 year old Polish female with no significant past medical history, presented with a month of upper abdominal fullness, loss of appetite, nausea, and weight loss of 30 lbs. On exam she was cachectic with right upper quadrant tenderness and hepatomegaly. Lab values were notable for WBC's of 12.1 with 27% bands, an alkaline phosphatase of 493 U/L, AST of 48 U/L, ALT of 45 U/L and albumin of 2.5 g/dL. A CT of the abdomen showed dilatation of the intrahepatic ducts, 2 large subcapsular fluid collections in the right lobe of the liver, a large fluid collection in the pelvis and the presence of an IUD. An ERCP demonstrated multiple saccular dilatations of the intrahepatic ducts and two cystic areas in the liver communicating with the biliary tree. Papillotomy was performed with drainage of purulent fluid. The patient underwent CT-guided drainage of the fluid collections in the right lobe of the liver and the pelvis, with removal of 700 ml of pus. The patient was started on piperacillin/tazobactam and the IUD was removed. The patient improved and cultures of the hepatic abscess eventually grew A. israelii and C. ochracea and the pelvic abscess grew A. israelii also. However the patient had a seizure 7 days into antibiotic therapy and CT of the head revealed 3 ring-enhancing lesions, consistent with metastatic abscesses. The patient was started on phenytoin and the antibiotics changed to ceftriaxone and metronidazole. Repeat CT of the abdomen showed resolution of the abscesses. The patient subsequently did well and eventually went home on long-term antibiotic therapy. Actinomyces and Capnocytophaga species are both endogenous to the oral flora and occasionally can be found in the gastrointestinal and vaginal tracts. Hepatic Actinomycosis accounts for 5% of all infections with Actinomyces species. Risk factors include poor dentition, appendicitis, prior abdominal surgery, immunosuppression and IUD use. Most infections with Capnocytophaga occur in immunocompromised patients with poor dentition and clinically manifest as bacteremia, endocarditis, osteomyelitis or empyema. An extensive literature review revealed only one case report of liver abscess due to Capnocytophaga.

Successful Preoperative Diagnosis and Complete Resection of Biliary Intraductal Papillary-Mucinous Neoplasm of the Liver

CASE REPORT A 59-year-old man was admitted to our hospital for further examination of an asymptomatic cystic tumor in the liver. On admission, he was not jaundiced, and the mass was not palpable in his abdomen. Laboratory evaluation did not demonstrate any abnormalities. Serum carcinoembryonic antigen level was within the normal range, but the carbohydrate antigen 19-9 level was elevated at 221 U/ml (normal range 3 to 25 U/ml). Abdominal ultrasonography and CT demonstrated a multilocular cystic tumor in the left lobe of the liver measuring 5 cm without mural nodules (Fig. 1). Endoscopic retrograde cholangiography showed a widely opened Vater’s ampulla and dilated common bile duct measuring 25 mm with filling defects containing mucus. The left hepatic and intrahepatic bile ducts in the left hepatic lobe were not visualized. No apparent communication was present between the cystic tumor and the bile ducts. Cytology of the biliary juice was examined three times, and diagnosed as showing borderline malignancy. Under a diagnosis of biliary intraductal papillarymucinous neoplasm localized in the left lobe of the liver with possible malignant transformation, left hemihepatectomy with lymphadenectomy in the hepatoduodenal ligament was performed. The resected specimen macroscopically showed multiple saccular dilatations of the intrahepatic biliary tree containing mucous fluid in the left lobe of the liver (Fig. 2). Subsequent pathological examination demonstrated that the tumor was a biliary intraductal papillarymucinous adenocarcinoma. The internal surface of the dilated biliary tree consisted of high columnar epithelium with mucin-producing cells and a partly

Vascular corrosion casts mirroring early morphological changes that lead to the formation of saccular cerebral aneurysm: an experimental study in rats

The formation of cerebral aneurysms involves complex processes and little is known about the mechanisms by which they originate, grow, and rupture. The purpose of this study was to identify early ultrastructural morphological changes that lead to the formation of experimental cerebral aneurysms. Twenty male Sprague-Dawley rats were subjected to cerebral aneurysm induction (renal hypertension and right common carotid artery ligation); 10 intact rats served as the control group. The animals were killed after 2 months, and a vascular corrosion cast of their cerebral arteries was prepared and screened for aneurysm development by using a scanning electron microscope. Sequential morphological changes observed at the cerebral artery bifurcation in response to hemodynamic shear stress included endothelial changes, intimal pad elevation, and saccular dilation. Endothelial cell changes were the first observed morphological changes; they were followed by various degrees of artery wall dilation. No aneurysmal changes developed in any of the control rats. Of the 20 surgically treated rats, 11 displayed aneurysmal changes. In five of these animals only changes in the endothelial cell imprints could be identified. In the other six rats morphological changes in endothelial cells were associated with different stages of aneurysmal dilation. This is the first study to demonstrate in vivo early morphological changes that lead to the formation of cerebral aneurysms. The morphological findings indicate the principal role of endothelial cells in the pathogenesis of cerebral aneurysms and suggest that hemodynamic shear stress and blood flow patterns may precipitate these early changes.