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.

Use of demineralized bone implants in orbital and craniofacial reconstruction and a review of the literature.

A retrospective study and review of the literature was performed on the use of demineralized bone implants for the correction of orbital and craniofacial defects. Demineralized bone implants heal by endochondral osteogenesis, inducing a transformation of local cells, as well as by osteoconduction, similar to autogenous grafts. They induce the chemotaxis and transformation of mesenchymal cells into chondroblasts, followed by ossification. They also act as a scaffold, with bone resorption taking place simultaneous with bone formation. This study reviewed 21 patients and 31 orbits, in which demineralized bone was used for orbitocranial reconstruction for congenital deformities (nine patients), after surgery for orbital fractures (seven patients: four floor, three roof), and orbital tumors (five patients). The surgical technique is described, and the results are discussed. The follow-up period averaged 15 months (6 months to 33 months). The overall resorption rate of the demineralized bone implants was estimated based on follow-up radiologic studies (facial films, computed tomography scans, and magnetic resonance imaging scans), as well as clinical examinations. All patients had a satisfactory to excellent result. The demineralized bone and Grafton (Osteotech, Shrewsbury, NJ, U.S.A.) was obtained from the Musculoskeletal Transplant Foundation of Holmdel, New Jersey, and it was all processed and reconstituted in a standard manner with Alloprep System. Surgical complications were cerebrospinal fluid leaks (one patient) with infection, transient chemosis, enophthalmos, and hypophthalmos. There were no complications related to demineralized bone alone. Properly prepared demineralized bone is a safe material for orbital reconstruction that eliminates the need for a second operative site to harvest a bone graft.

Graves orbitopathy: correlation of CT and clinical findings.

The clinical and high-resolution computed tomographic (CT) findings in 71 patients (142 orbits) with Graves orbitopathy and 20 healthy patients (40 orbits) were retrospectively reviewed. The orbits with orbitopathy were subgrouped at clinical examination into those with (n = 18) and those without (n = 124) optic neuropathy. Mean extraocular muscle diameters and the calculated muscle diameter index were significantly increased in all orbits with ophthalmopathy, particularly in those with optic neuropathy. Graves orbitopathy affected the superior muscle group (63.4%) more than the medial (61.3%) or inferior (57%) recti. The most common pattern of muscle involvement involved all five measured extraocular muscles. Solitary muscle involvement most frequently involved the superior muscle group (6.3%). Significant enlargements of the retrobulbar optic nerve sheath and superior ophthalmic vein were noted only in orbits with optic neuropathy. Anterior displacement of the lacrimal gland at CT correlated with clinical palpability and occurred more frequently in patients with optic neuropathy. Severe apical crowding was the most sensitive indication of optic neuropathy at CT.

Infestation of extraocular muscle by Cysticercus cellulosae.

Reports of orbital cysticercosis are uncommon despite the high incidence of brain and ocular involvement. Infestation of extraocular muscle is exceedingly rare. Two cases of cysticercosis of the extraocular muscles are reported here. Surgical removal of the encysted parasites successfully resolved the infestation in both cases.

Dysthyroid optic neuropathy. The crowded orbital apex syndrome.

The authors have reviewed the clinical presentation, visual fields, color vision testing, visual-evoked potentials, and computed tomographic (CT) findings of 58 patients (95 eyes) with dysthyroid optic neuropathy. The authors compared these findings to a control group of 60 patients (119 eyes) with thyroid eye disease who underwent CT scanning and did not exhibit evidence of optic neuropathy. Clinically, dysthyroid optic neuropathy is an insidious disease; when compared with the usual Graves' orbitopathy patient, the optic neuropathy group presented at a later age and with a later onset of thyroid eye disease. The patients in this group were more likely to be male and/or diabetic, and often presented with desaturation of color vision. Asymmetrical extraocular muscle restriction and vertical tropias were more frequent in the optic neuropathy group. The most sensitive indicators of optic nerve dysfunction appeared to be visual-evoked potentials and color vision. Computed tomographic studies confirmed that apical orbital crowding was a characteristic feature of optic neuropathy. These findings should alert the clinician to a more aggressive approach to these patients.

The Tolosa-Hunt syndrome: computed tomographic changes and reversal after steroid therapy.

Painful ophthalmoplegia, or Tolosa-Hunt syndrome, is due to nonspecific inflammation of the cavernous sinus. We report two cases of the Tolosa-Hunt syndrome, which, on high resolution fourth-generation CT scan, showed an abnormality in the cavernous sinus consisting of an enhancing soft tissue mass. Both the clinical findings and the CT lesion resolved after high-dosage corticosteroid therapy.