Giovanni Giancipoli

LDL-Apheresis Accelerates the Recovery of Nonarteritic Acute Anterior Ischemic Optic Neuropathy

Abstract:  Nonarteritic acute anterior ischemic optic neuropathy (NAION) is a disabling disease which impairs visual function. It is presumed to result from disturbances of microcirculation in the anterior portion of the optic nerve head due to hemodynamic factors derived from excessive blood viscosity, or restriction of the vasal lumen in hypertensive, hypercholesterolemic, diabetic patients. We aimed to determine whether acute reduction of plasma fibrinogen and serum low‐density lipoprotein (LDL) cholesterol is effective for treatment of NAION. We recruited 11 patients (7 females, 4 males) with a mean age of 57.2 ± 19.6 years. All except one of them presented risk factors for atherosclerosis. The mean values of LDL‐cholesterol and fibrinogen before treatment were 144 ± 32 mg/dL and 341 ± 80 mg/dL, respectively. All were treated with standard therapy (prednisone, salicylate, pentoxiphyllin) and underwent three sessions of LDL‐apheresis (HELP system—B Braun) that can reduce plasma LDL‐cholesterol and fibrinogen by more than 50% in a very short time. In all patients we observed a drastic reduction of LDL cholesterol and fibrinogen and a clear improvement in the visual functional data. In fact, mean values of corrected vision increased from 3.7/10 ± 3/10 to 7.9/10 ± 2.2/10 (P = 0.002) after the third session, while the scotomatous portion of the visual field regressed after the first session, and in 5 patients further regressed after the third session. This improvement had remained stable after 3 months. Thanks to its effect of antagonizing hemorheologic disorders of the ocular microcirculation, fibrinogen/LDL‐apheresis seems to be an efficacious treatment of NAION.

Solitary eyelid Kaposi sarcoma in an HIV-negative patient.

Purpose: To describe a case of localized Kaposi sarcoma (KS) of the eyelid in an HIV-seronegative patient. Methods: An 80-year-old man developed an ulcerated nodular tumor-like mass that grew rapidly on his left upper eyelid. There were no similar lesions elsewhere. The eyelid lesion was completely excised and histopatologically examined. Serological analyses and molecular biologic techniques, including polymerase chain reaction, were used. Results: Laboratory examinations were within normal limits, and serology for HIV was negative. Histological sections revealed a vascular proliferation composed predominantly of small slit-like blood vessels and epithelioid spindle cells, supporting the diagnosis of KS. Polymerase chain reaction was positive for human herpesvirus 8. During a 2-year follow-up, no recurrences, development of new lesions, or HIV seroconversions were observed. Conclusion: This is a classic KS involving only the eyelid in an HIV-negative patient. Location in the eyelid is a possible, albeit rare, initial solitary manifestation of KS in elderly HIV-negative patients. Surgery is both safe and effective.

Retinal circulation after carotid artery revascularization.

Doubts still exist concerning the mechanisms involved in ocular ischemic syndrome (OIS) and its dependence on carotid disease. We report findings from 14 surgical patients undergoing carotid artery reconstruction for symptomatic cerebrovascular disease. All of them had fluorescein angiography (FA) of the eye ipsilateral to the carotid operation before surgery and 3 months after to provide information regarding retinal circulation time. Before the surgical procedure, the mean circulation time was 29.4 ± 9.4 seconds (CI 95%: 24.5-34.3). After 3 months, a significant (P < .001) decrease in the circulation time was observed: 18.9 ± 8.4 seconds (CI 95%: 14.5-23.4). The present series demonstrates that carotid revascularization surgery improved retinal flow in approximately 80% of the patients.

A Rare Case of Primitive Epithelioid Leiomyosarcoma of the Conjunctiva

Purpose: To describe a rare case of conjunctival leiomyosarcoma initially diagnosed as a poorly differentiated squamous cell carcinoma. Methods: Clinical, light microscopic, immunohistochemical, and ultrastructural findings are reported. Results: A 56-year-old Caucasian woman was referred with a history of a progressive, rapidly growing mass in her left eye. Biopsy of the mass and histology yielded a first diagnosis of a poorly differentiated conjunctival squamous cell carcinoma. Orbital exenteration was performed 2 weeks later. Macroscopically, the exenteration specimen showed a soft mass completely involving the conjunctiva and extending to the eyelids and orbital structures. Histological examination revealed a malignant tumour composed of atypical, predominantly epithelioid large cells. Immunohistochemical and ultrastructural studies combined with the light microscopic findings contributed to clarify the diagnosis of epithelioid leiomyosarcoma. The patient was started on chemotherapy and radiotherapy, but died a few months later from widespread metastases. Conclusions: primary involvement of the orbit by a leiomyosarcoma is rare, but this eventuality should be considered in the differential diagnosis of rapidly growing orbital and conjunctival masses.

Orbital Granular Cell Tumor in a Patient with Churg Strauss Syndrome: The Importance of Biopsy

A 65-year-old woman presented with a long standing, progressive exophthalmos of the right eye. Her medical history was significant for Churg Strauss syndrome, and was treated with immunosuppressive therapy. She had undergone two previous orbital biopsies showing inflammatory reactive lymphoid hyperplasia. A diagnosis of orbital inflammation in Churg-Strauss syndrome was suspected, and the immunosuppressive therapy was increased. Because of the lack of response to therapy, a further biopsy was performed, by lateral orbitotomy approach. Biopsy of the mass revealed a granular cell tumor composed of S-100 positive cells with an acidophilic granular cytoplasm and peripheral lymphocytic infiltration. A granular cell tumor, which is very rare in the orbit, should be considered in the differential diagnosis of orbital tumors, and if suspected, an excisional biopsy must be undertaken. Typical hystopathological aspect of the granular cell tumor is characterized by the presence of S-100 positive closely packed polygonal cells with a granular cytoplasm.

Bilateral sixth nerve palsy as a manifestation of Wernicke's encephalopathy in a patient with refractory vomiting

PURPOSE To report a case of Wernickes encephalopathy in a nonalcoholic woman with secondary hypoadrenalism. METHODS A 58-year-old Italian woman developed Wernickes syndrome secondary to recurrent vomiting due to secondary hypoadrenalism. RESULTS Recurrent vomiting and resulting malnutrition caused a depletion of the patients body stores of thiamine and the development of mental confusion and an oculomotor deficit. On the diagnostic suspicion of encephalitis in this patient with immunosuppression due to prolonged cortisone-based therapy, she underwent magnetic resonance imaging, which showed typical bilateral abnormal lesions pathognomonic of Wernickes encephalopathy. The patient improved after parenteral administration of vitamin B1. CONCLUSIONS Wernickes encephalopathy must be regarded as a medical emergency that should be suspected in all cases presenting with a sudden deficit or lack of coordination of the ocular movements associated with ataxia and general degeneration of the mental faculties, in patients who have suffered recurrent vomiting or malnutrition for different reasons.