Cases reported "Fibrosis"

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11/52. Paramacular Von Hippel angioma with tractional macular detachment.

    A rare case of paramacular angioma with tractional macular detachment that was managed successfully with laser, followed by surgical intervention with good visual outcome, is presented. A 23-year-old female patient with paramacular Von Hippel angioma was treated with argon laser photocoagulation. Three-months following photocoagulation, vitrectomy with epiretinal tractional membrane removal was attempted successfully. visual acuity improved from a preoperative level of 20/200 to 20/50 at the 6 months postoperative period. No recurrence of epiretinal membrane or exudation was observed during the follow-up period. Photocoagulation, followed by surgical intervention for removal of epiretinal traction membranes, resulted in relieving the tractional macular detachment and recovering useful visual acuity in paramacular Von Hippel angioma.
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12/52. Bilateral corneal fibrosis in homocystinuria: case report and transmission electron microscopic findings.

    PURPOSE: To report the unusual occurrence of bilateral, superficial, corneal fibrosis with pannus formation in a young woman with homocystinuria, and to describe the light and transmission electron microscopic findings of these deposits. methods: Excision of the corneal lesion and amniotic membrane transplantation was performed in both eyes. The excised material was studied using light and transmission electron microscopy. RESULTS: Whitish, elevated, irregular masses with superficial vascularization in the peripheral cornea were noted in both eyes. Histopathologic evaluation of the excised corneal tissue revealed variable epithelial thickness with melanin pigment in the basal layer. A fibrovascular pannus, fibrosis and disruption of the Bowman's layer, and fibrosis of anterior stroma were evident. Transmission electron microscopy revealed numerous empty intracytoplasmic vacuoles in the corneal epithelial cells and intracytoplasmic inclusions containing fibrillogranular material in the cytoplasm of keratocytes. CONCLUSION: We report the unusual association of corneal fibrosis and scarring in a young woman with homocystinuria. The deposits revealed the presence of membrane-bound inclusions containing fibrillogranular material in the corneal epithelium and keratocytes. Although the etiology of these deposits is not clear, the condition improved following excision of these lesions and amniotic membrane transplantation.
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13/52. The abdominal cocoon: a case report.

    The abdominal cocoon is a rare disease that is characterized by a total or partial encasement of the small bowel by a thick and fibrotic membrane. Thirty-five cases were reported since it was first described. It occurs primarily in females. Preoperative diagnosis is a matter of challenge and usually made at laparotomy. We report a patient with partial intestinal obstruction and abdominal cocoon which was diagnosed peri-operatively. We review the literature and discuss the etiology of this disease.
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14/52. Postmenopausal frontal fibrosing alopecia.

    Recently a new entity, postmenopausal frontal fibrosing alopecia, was added to the established subtypes of scarring alopecias affecting postmenopausal women. This condition is characterized by a progressive frontal hairline recession associated with scarring. We studied the clinical and histopathologic features in four women with this disorder. Of note, a history of bilateral oophorectomy in two of them appears to be a new association. All four cases had frontoparietal recession of the hairline and two of them also had loss of their eyebrows. None of our four patients had any mucous membrane or other skin lesions. Histological examination showed perifollicular fibrosis and lymphocytic inflammation around the isthmus and infundibular areas of the follicles. No effective treatments have emerged for this type of postmenopausal alopecia, but progression of the hair loss and scarring appears to be self-limiting. We believe that this condition is a distinct clinicopathological variant of lichen planopilaris.
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15/52. Refractory medial conjunctival inflammation associated with jones tubes.

    PURPOSE: To report an uncommon complication of refractory medial conjunctival inflammation associated with Jones tubes in 2 patients. methods:" Complete ophthalmic and nasal examinations were performed on 2 patients in whom medial conjunctival injection developed after conjunctivodacryocystorhinostomy (cDCR) with Jones tube placement that persisted despite aggressive medical treatment. RESULTS: The medial conjunctival inflammation, which resembled episcleritis, persisted despite removal of the Jones tube in the first patient. The other patient had a "pterygium-like" lesion and ultimately underwent excision of the medial granulation tissue with amniotic membrane graft and exchange of the Jones tube. Histopathologic examination revealed conjunctival tissue with inflammation and fibrosis.CONCLUSIONS: Medial conjunctival inflammation is an uncommon complication of cDCR with Jones tubes. Based on our experience and a thorough review of the literature, medial conjunctival injection and thickening usually resolves once Jones tubes are removed. There have been no previous reports indicating the persistence of medial conjunctival inflammation after Jones tube removal, as was the case in our first patient. These cases suggest that some chronic effect initiated by the Jones tubes and/or surgical manipulation may induce inflammation and scarring of the medial conjunctiva. Multiple surgeries may also predispose to this inflammatory response.
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16/52. Phototherapeutic keratectomy with amniotic membrane for severe subepithelial fibrosis following excimer laser refractive surgery.

    We report 2 patients who developed subepithelial opacities and myopic regression after photorefractive keratectomy (PRK) and laser-assisted subepithelial keratectomy. Both cases were treated successfully with manual debridement of the epithelium, phototherapeutic keratectomy, and PRK with amniotic membrane application.
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17/52. Distinction between aleukaemic prodrome of childhood acute lymphoblastic leukaemia and aplastic anaemia.

    AIMS: To document the features of the so-called aplastic presentation of childhood acute lymphoblastic leukaemia (ALL) and to determine whether this prodrome can be distinguished from aplasia. methods: The peripheral blood and bone marrow appearances of all cases of childhood ALL presenting in one health region of england in 13 years and eight months were reviewed. All cases presenting with cytopenia without circulating blasts and marrow aspirates with no infiltrate of blasts were studied in detail. RESULTS: Four of 305 (1.3%) children presented in this way. All four had reticulin fibrosis and increased cellularity in all or part of the marrow biopsy specimen. All were girls. Three had common and one surface membrane immunoglobulin positive ALL. Reassessment of this prodrome, by combining the features of four previously reported series of similar cases with the present one, highlighted the female preponderance (19 of 22 cases), bone marrow fibrosis (10 of 11 evaluable cases), prominent bone marrow lymphocytes (14 of 22 cases) and temporary recovery (all 12 evaluable cases). Six of 14 evaluable cases had bone marrow biopsy specimen appearances of apparently uniform hypocellularity, but only one of these did not have fibrosis. CONCLUSIONS: If, in addition to an aspirate, a bone marrow trephine biopsy is carried out the prodrome can be distinguished from aplasia in most cases. The similarity of this prodrome to aplastic anaemia is merely superficial. Clinicians and morphologists may fail to appreciate the implications of this mode of presentation if the term "aplastic" continues to be used to describe this aleukaemic prodrome of ALL.
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18/52. Aristolochic acid nephropathy and the peritoneum: Functional, structural, and molecular studies.

    BACKGROUND: Aristolochic acid nephropathy (AAN) is a rapidly progressive interstitial nephropathy linked to the exposure to aristolochic acid (AA) and characterized by extensive fibrosis and urothelial atypia. Although the fibrotic process has been documented in extrarenal tissues, the involvement of the peritoneum, as well as the efficacy of peritoneal dialysis in AAN patients, remain uncertain. methods: The structure of the peritoneal membrane and the expression of basic fibroblast growth factor (bFGF), collagen type iii, endothelial nitric oxide synthase (eNOS), and aquaporin-1 (AQP1) were investigated in peritoneal biopsies from an index AAN patient, four other AAN patients, four regular peritoneal dialysis patients, and two controls. Similar methods were used to investigate a rabbit model of AAN after intraperitoneal exposure to high-dose AA. AA-dna adducts were screened by 32P-postlabeling analysis. RESULTS: The AAN patients had renal failure, renal fibrosis, and urothelial atypia. The peritoneum of AAN patients had a normal structure, lacked cellular atypia, and, in comparison with regular peritoneal dialysis patients and controls, did not show abnormal regulation of fibrotic and endothelial markers. Furthermore, specific AA-dna adducts were not identified in the peritoneum of AAN patients. In contrast, AA-dna adducts were detected in peritoneal and kidney tissues of all exposed rabbits, and one of them developed a malignant mesothelioma. CONCLUSION: These data demonstrate the lack of fibrotic and vascular alterations and the absence of cellular atypia in the peritoneum from AAN patients. Thus, peritoneal dialysis should not be discouraged in these patients. Nevertheless, studies in a rabbit model of high-dose AA exposure may suggest a potential risk of peritoneal malignancy.
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19/52. Immunohistopathologic evaluation of choroidal neovascular membranes following verteporfin-photodynamic therapy.

    PURPOSE: To evaluate the vascularization and proliferative activity in choroidal neovascular membranes due to age-related macular degeneration after verteporfin photodynamic therapy and submacular removal. DESIGN: Interventional case series. methods: In a retrospective review of seven patients who underwent removal of subfoveal classic choroidal neovascular membranes after treatment with photodynamic therapy 3 to 146 days earlier, membranes were stained for CD 34, CD 105, and Ki-67 and correlated with clinical pictures and fluorescein angiography. RESULTS: fluorescein angiography performed on the day of surgery disclosed nonperfusion of the treated area 3 days after photodynamic therapy, but perfusion and leakage were seen at greater post-photodynamic therapy intervals. membranes excised 3 days after photodynamic therapy showed CD34 and CD105 positive, mostly occluded vessels. The endothelial cells appeared damaged. Ki-67 activity was low. In membranes excised 34 to 146 days after photodynamic therapy, all vessels appeared patent and were lined by healthy endothelial cells with strong expression of CD34 and CD105. Ki-67 expression was elevated after 34 days but decreased thereafter. CONCLUSION: Photodynamic therapy did not cause a general or complete occlusion of vessels within the choroidal neovascular membranes, as suggested by fluorescein angiography 3 days postintervention, but the endothelial cells appeared to be severely damaged. Proliferative activity within these specimens was reduced. At longer intervals after photodynamic therapy, the fibrovascular tissue seemed to recover; perfusion, hyperfluorescence, and leakage of the choroidal neovascular membranes could be detected by fluorescein angiography. The clinical appearance showed a correlation with the immunohistologic characteristics of an increased proliferative activity and patent vascularization.
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20/52. Complete occlusion of the anterior capsular opening by fibrocellular membrane associated with retained silicone oil: a clinicopathologic correlation.

    BACKGROUND: To report the clinicopathologic features of a fibrocellular membrane in a pseudophakic eye with retained silicone oil in the absence of any capsular contraction syndrome, necessitating repeat anterior capsulorhexis. methods: Clinicopathologic report of a case. RESULTS: Histopathologic study of the membrane showed absence of any true periodic acid Schiff-positive capsule within the specimen. Clinically, there was no evidence of any anterior capsular contraction. The fibrocellular membrane may have occurred as a result of inflammatory and mechanical effects of silicone oil in the anterior chamber or as a result of the intraocular lens design. CONCLUSION: The authors report the clinicopathologic features of a fibrocellular membrane occluding the anterior capsular opening in a pseudophakic eye with retained silicone oil, in the absence of any capsular contraction. The stimulus for its formation may have been the inflammatory and mechanical effects of retained silicone oil in the anterior chamber.
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