Cases reported "Fibrosis"

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1/21. Free vascularized appendix transfer for reconstruction of penile urethras with severe fibrosis.

    Despite the development of newer techniques with a free radial forearm tube flaps for phallus reconstruction, severe urethral strictures are still seen in such cases after irradiation or repeated infection because of the paucity of healthy, well-vascularized tissue. For urethral reconstruction in cases with poorly vascularized tissue as well as for total penile creation, a new technique involving a free vascularized appendix transfer combined with a radial forearm osteocutaneous flap was successfully used in two cases. The appendix provides a normal tube structure composed of a muscular tubular layer lined with mucosal epithelium. It has no hair and has rich vascularization. This results in little stricture at the junction with the original urethra, no occurrence of urethral stones, and possible postoperative enlargement of the diameter with changes in catheters. This method will allow a patient with severe fibrosis around the urethra to undergo one-stage phallus reconstruction with minimal complications.
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2/21. Histopathologic effects of radiofrequency catheter ablation in previously infarcted human myocardium.

    INTRODUCTION: The use of catheter-based radiofrequency (RF) ablation for the treatment of ventricular tachyarrhythmias due to previous myocardial infarction has been steadily increasing. The histopathologic changes caused by this technique are not well described in humans. methods AND RESULTS: Three patients with hemodynamically tolerated ventricular tachycardias (VTs) due to previous myocardial infarction underwent endocardial mapping and catheter based RF ablation. All patients received between 5 and 11 RF lesions each of 60-second duration. One patient underwent myocardial resection of a left ventricular aneurysm 1 day following RF ablation, one expired 7 days after RF ablation, and one expired 9 months after RF ablation. None of the deaths occurred as a result of RF ablation. Pathologic specimens obtained early after RF ablation revealed areas of focal acute inflammation and fibrin deposition. Later specimens revealed several focal areas of fibrosis and granulation tissue. Specimens obtained late after RF ablation revealed a dense band of fibrosis, measuring 17 x 17 x 5 mm (1,250 mm3). CONCLUSION: Catheter-based RF ablation of ischemic VT in humans causes lesions that initially resemble coagulation necrosis. This is followed by the development of an inflammatory infiltrate and, finally, the development of fibrosis. Repeated application of RF ablation may result in much larger lesions than have been previously reported.
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3/21. endomyocardial fibrosis (Davies disease) coincidental with systemic lupus erythematosus.

    This is the case of a 27 years-old woman with signs and symptoms of severe untreatable congestive heart failure, anemia, gingival mucosa ulcers, photosensitivity and alopecia. The electrocardiographic, echocardiographic, angiographic and hemodynamic data oriented the diagnosis of restrictive cardiomyopathy, mitral insufficiency secondary to mitral prolapse and bi-atrial dilation. The histologic study of the endomyocardial biopsy, performed during catheterization, showed signs of endomyocardial fibrosis, and immunological analysis was compatible with systemic lupus erythematosus. As far as we know, this is the first case of endomyocardial fibrosis (Davies disease) associated with systemic lupus erythematosus published in the medical literature. The etiology of Davies disease remains unrevealed and its association with systemic lupus erythematosus suggest a probable autoimmune origin.
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4/21. Proof of the patent subcutaneous fibrous tract in children with V-P shunt malfunction.

    A connective tissue sheath that forms around the peritoneal catheter of silicone ventriculo-peritoneal (V-P) shunt tubing is quite often observed in children with V-P shunts. However, proof of the passage of cerebrospinal fluid (CSF) through these sheaths has been reported in only one published study to date. We present four cases associated with chronic malfunction of the V-P shunt peritoneal catheter. In these cases, CSF passage through the subcutaneous fibrous tract, which had a pericatheter connective tissue sheath, was demonstrated around the V-P shunt peritoneal catheter. In the first case the patient suffered intermittent headache attacks over a long period of time; abdominal migration of the peritoneal catheter was detected. The second patient, who had been asymptomatic in the follow-up period with an outgrown peritoneal catheter, was admitted with acute hydrocephalus symptoms. A peritoneal catheter disconnection was detected in another patient, who had had multiple shunt revisions previously. In the last case, an obstruction of the peritoneal catheter was detected. The existence of the subcutaneous fibrous tract and its function were demonstrated by radio-opaque shuntogram in two cases and radionuclide shuntogram in the other two cases. In all four cases V-P shunt revision was performed. Within this study, the possible passage of CSF through a fibrous tract in cases of migration, outgrowth, disconnection or obstruction of the peritoneal shunt catheter was demonstrated. In conclusion, patients with shunt malfunction with a well-grown pericatheter fibrous sheath who are either asymptomatic or minimally symptomatic and show no evidence of active ventricular dilatation on their cranial CT scan should not be regarded as having arrested hydrocephalus until radio-opaque or radionuclide shuntogram studies have been done.
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5/21. Percutaneous pulmonary artery and vein stenting: a novel treatment for mediastinal fibrosis.

    Mediastinal fibrosis is a rare consequence of infection with the fungus histoplasma capsulatum that can lead to occlusion of large pulmonary arteries and veins and mainstem bronchi. Medical and surgical treatments for this disorder have been ineffective. We describe successful treatment for central pulmonary arterial and venous obstruction due to mediastinal fibrosis in four patients using percutaneously placed intravascular stents. patients were severely limited, world health organization functional class III or IV. At the time of right and left heart catheterization, stents were placed in pulmonary arteries (n = 1), veins (n = 2), or both (n = 1) to relieve vascular obstruction resulting from mediastinal fibrosis. Immediate hemodynamic and clinical improvement was observed in all patients. Three of the four patients have had sustained improvement in exercise tolerance, from 3.5 mo to 4.5 yr after stent placement. The only complication was a self-limited pulmonary hemorrhage in one patient. Our initial experience suggests that percutaneous stent placement to relieve central pulmonary arterial or venous obstruction due to mediastinal fibrosis is an effective new treatment modality.
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6/21. Post-mortem observations of a recent radiofrequency catheter ablation site.

    The acute and chronic gross and microscopic morphologic changes present in myocardium after radiofrequency catheter ablation have been previously described in animal experiments. Acute changes have also been described in four cadaveric human specimens. We describe post-mortem observations of a recent radiofrequency catheter ablation site in a patient who underwent successful ablation for refractory ventricular tachycardia. Our gross and microscopic observations are similar to those previously described in animal experiments and confirm that the animal experimental results can be extrapolated to human hearts. As the use of radiofrequency becomes more prevalent as an alternative treatment for refractory cardiac tachycardias, pathologists will be called upon to identify post-mortem the lesions described. These lesions can be specifically identified, which can serve as a useful verification for this procedure.
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7/21. Re-entry circuit in ventricular tachycardia due to focal fatty-fibrosis in a patient with myotonic dystrophy.

    A 69-year-old man with a recurrent ventricular tachycardia (VT) was admitted. The patient was diagnosed as myotonic dystrophy type 1 (DM1) and dna analysis revealed 1,800 CTG-repeat expansion in the myotonic dystrophy protein kinase (DMPK) gene. Ultrasonic cardiogram (UCG), left ventriculogram (LVG) and magnetic resonance imaging (MRI) did not show any abnormal sign including fatty infiltration. But, endomyocardial biopsy obtained from ventricular outflow tract revealed severe fatty infiltration and interstitial fibrosis. Radiofrequency catheter ablation at the biopsy site could eliminate VT, so it was strongly suggested that the re-entry circuit was formed by focal fatty-fibrosis. Careful observation should be continued for a long period.
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8/21. Advantages of oxygen inhalation therapy for postoperative pulmonary hypertension.

    Total correction was performed in a case of complete transposition of the great arteries (TGA) with severe pulmonary vascular obstructive disease (PVOD). Although severe pulmonary hypertension remained after surgery, oxygenation was continued for 15 months, which included a shift to at-home oxygen inhalation therapy (HOT). cardiac catheterization 15 months after surgery demonstrated that pulmonary hypertension was greatly improved. For patients in whom the palliative Mustard operation is considered due to severe PVOD on the basis of lung biopsy diagnosis, total correction of TGA is possible by employing HOT after surgery.
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9/21. Tethered hemodialysis catheter with retained portions in central vein and right atrium on attempted removal.

    A case of tunneled Tesio (Medcomp, Harrisburg, PA) twin hemodialysis catheters (silicone) extensively tethered subcutaneously and intravascularly is reported. Attempted removal resulted in significant portions of both catheters being retained in the superior vena cava and right atrium.
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10/21. Iatrogenic deaths following treatment for hypertrophic obstructive cardiomyopathy: case reports and an approach to the autopsy and death certification.

    Hypertrophic cardiomyopathy (HCM) is a disease process which results in a large, heavy heart, with hypertrophy of the interventricular septum (IVS) and left ventricle. HCM accounts for a significant number of cases of sudden cardiac death each year, most infamously in young athletes. The prevalence of the disease has increased over the past several years due to advances in clinical diagnosis and molecular genetic studies. Over this same period, new forms of treatment also have emerged. One such treatment is alcohol septal ablation (ASA). ASA is a procedure performed by a cardiologist, via cardiac catheterization, by injecting pure ethanol into selected arteries which supply the IVS, resulting in a targeted myocardial infarction. This infarct then retracts and forms a scar, decreasing the outflow obstruction and improving the patient's clinical symptoms.The authors report 2 cases of death following ASA treatment of HCM. The first, a 56-year-old male, had his ASA procedure 10 days prior to death. The second decedent, a 76-year-old female, had her procedure only 30 hours before death. These case reports are followed by a discussion about HCM, including pathology, treatments, and treatment-related pathology, before closing with a discussion about death certification in the cases presented and therapy-related deaths in general.
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