Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review.

BACKGROUND/AIMS: Adult xanthogranulomatous disease involving the ocular tissues is rare and poorly understood. Adult onset xanthogranuloma (AOX), adult onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX), and Erdheim-Chester disease (ECD) are the four syndromes within this disorder, which is diagnosed by characteristic histopathology. Experience with eight cases prompted a multi-institutional effort to study the histopathology, immunohistochemistry, clinical findings, and systemic associations in this disorder. METHODS: 22 cases, including histopathological slides, were compiled. Published reports were identified by an English language Medline search (1966-2005) and review of reference citations. Each case in this series and the literature was classified as one of four syndromes and then analysed for age onset, sex, skin xanthoma, orbital location, immune dysfunction, internal organ and bone lesions, treatment, and outcome. The histopathology in each of these cases was reviewed by two pathologists. Immunhistochemical stains (CD3, CD4, CD8, L26) were performed in 14 cases where unstained slides were available. RESULTS: 137 cases were compiled. There was no sex or age difference between syndromes. AOX, AAPOX, NBX affect the anterior orbit, ECD tends to be diffuse and intraconal. Skin lesions are found in all the syndromes. Immune dysfunction was noted in all cases of AAPOX and NBX; 11% of NBX and all ECD patients had internal organ disease. Treatment included surgery, corticosteroids, other chemotherapeutic agents, radiotherapy, and combinations of these. No AOX or AAPOX deaths occurred; 66% of ECD patients died. All 22 cases had xanthoma cells; most had Touton giant cells. Lymphocytes were present in all cases and occurred as aggregates (mostly in AAPOX) or diffuse populations mixed with fibroblasts (mostly in ECD). Immunohistochemistry revealed the majority of these to be CD8+. Necrosis was most marked in NBX. CONCLUSION: Adult xanthogranuloma of the orbit is rare, making prospective evaluation or meta-analysis impossible. The best treatment is unknown but seems to be with multiagent chemotherapy guided by histopathological, immunohistochemical, and systemic findings.

Localised invasive sino-orbital aspergillosis: characteristic features.

BACKGROUND/AIM: To describe the characteristic constellation of historical, clinical, radiographic, and histopathological findings of localised invasive sino-orbital aspergillosis based on the authors' recent experience of four consecutive cases presenting over a 6 month period. Treatment and outcome are reviewed. METHODS: A case series of four patients with review of the English language literature. RESULTS: There have been 17 reported cases of invasive sino-orbital aspergillosis in healthy individuals over the past 33 years. The authors report four patients who presented during a 6 month period with persistent and significant pain followed by progressive ophthalmic signs-clinical histories reflecting the literature. Similar imaging findings were also noted: focal hypodense areas within apical infiltrates on contrasted computed tomography correspond to abscesses seen at surgery, and sinus obliteration or involvement of the adjacent sinus lining was noted on magnetic resonance imaging. Bone erosion (often focal) was also seen. There is frequently a delay in making the correct diagnosis, and often disease progression occurs despite treatment. CONCLUSIONS: The authors encountered four cases of invasive sino-orbital aspergillosis, three of which occurred in otherwise healthy individuals. The clinician must be aware of the characteristic presentation so that earlier diagnosis, management, and improved outcomes can be achieved.

Latanoprost-induced iris heterochromia and open-angle glaucoma: a clinicopathologic report.

PURPOSE: To report the histopathologic and immunohistochemical findings from the iridectomy specimen of a patient with acquired unilateral iris heterochromia due to latanoprost. PATIENT AND METHODS: A 45-year-old woman with open-angle glaucoma and unilateral iris heterochromia was evaluated for uncontrolled intraocular pressure increase. Subsequently, the patient underwent trabeculectomy with mitomycin C and an iridectomy specimen was obtained for analysis. RESULTS: The histopathologic analysis of the iridectomy specimen did not reveal any nuclear atypia, nuclear crowding, or mitotic figures. Immunohistochemical studies showed that the iris melanocytes were negative for HMB45 and S-100, and weakly positive for Melan A. CONCLUSION: Latanoprost-associated iris color change may exhibit a diffuse, uniform, dark velvet-brown appearance, thereby simulating diffuse iris melanoma. Histopathologic and immunohistochemical analysis confirmed the benign characteristics of the affected iris melanocytes.

Evidence-based recommendations for the diagnosis and treatment of neovascular glaucoma.

PURPOSE: To succinctly update information on the pathogenesis, etiology, diagnosis, and treatment of neovascular glaucoma based on a systematic review of available literature and to provide summary recommendations rated for their importance to clinical outcome. CLINICAL RELEVANCE: Neovascular glaucoma is a devastating ocular disease that often results in loss of vision. The current standard of care includes retinal ablation and control of increased intraocular pressure with medical and surgical therapy. LITERATURE REVIEW METHODOLOGY: The authors conducted a MEDLINE literature search of articles published in English from 1966 to the present. Each article reviewed was rated as to the strength of evidence it provided, and summary ratings for the strength of evidence supporting clinical recommendations were generated. RESULTS: Level A (most important to patient outcome) recommendations for the diagnosis of neovascular glaucoma include a high index of suspicion, a full ocular examination including undilated gonioscopy, and pupil examination. In regard to treatment, Level A recommendations include treatment of the underlying disease origin, complete panretinal photocoagulation (if retinal ischemia is a factor), and medical control of both elevated intraocular pressure and inflammation. Level B recommendations (moderately important to patient outcome) encompass glaucoma surgery to control intraocular pressure when medical therapy is unsuccessful, although the ideal surgical procedure is unknown. Currently, trabeculectomy with antimetabolite therapy, aqueous shunt implants, and diode laser cyclophotocoagulation are the preferred surgical treatment options. CONCLUSIONS: The current literature on neovascular glaucoma has few articles that provide strong evidence in support of therapy recommendations (level I). Future research studies are needed to address areas in which the current evidence is moderately strong (level II) or weak, consisting only of a consensus of expert opinion (level III). Whenever practicable, these studies should be prospective, randomized clinical trials.