Lymphoproliferative disorders of the ocular adnexa

PURPOSE OF REVIEW: To describe recent advances in clinical features, diagnostic tools, and treatment modalities for lymphoproliferative diseases affecting the ocular adnexa. RECENT FINDINGS: A close relationship of non-Hodgkin lymphoma with long-term use of aspirin and nonaspirin nonsteroidal antiinflammatory drugs has recently been documented. Some infectious agents have also been implicated in the pathogenesis of lymphomas. The advent of immunohistochemistry and polymerase chain reaction have helped to distinguish between benign lymphoproliferation and malignant lymphoma. New techniques for radiation therapy are proposed to decrease complication rates associated to this treatment. SUMMARY: Recent studies demonstrated that ocular adnexal lymphomas may be masked by a number of different diseases and clinical features. Prognosis depends on the stage and histology of lymphoma as well as the location.     

Anaphylaxis of ocular adnexa induced by infection of anti-IgE antibody

To examine the role in ocular tissue injury of anaphylaxis alone (as distinct from other mechanisms possibly present in a model involving immunization), a model of anaphylaxis was produced by injection of rabbit anti-rat IgE antibody into ocular adnexal tissues of rats. Adnexal swelling, seen within 15 min, disappeared by 6 hr. Vascular permeability and weight of adnexal tissues were increased at 0.5 hr and had returned to near normal levels by 6 hr. Histologic study revealed extensive degranulation of mast cells at 0.5 hr after injection and a return to normal by 24 hr. Neutrophils reached a level of 7400/mm3 at 6 hr and returned to normal by 24 hr. Macrophages accumulated by 24 hr in all tissues, including control eyes injected with normal serum. The similarity of results from this 'pure' model of anaphylaxis to results from a model produced by injecting antigen into ocular adnexal tissues of immunized rats suggests that anaphylactic mechanisms are responsible for the changes seen in the antigen-injected model.     

Oncocytoma of the ocular adnexa

The authors report on the clinical and pathological findings in 8 oncocytic lesions of the ocular adnexa. These tumors may involve the caruncle, the conjunctiva, the lacrimal gland and the lacrimal sac. They are often clinically mistaken for haemangiomas, nevi or cysts. In the present authors series the tumor involved the caruncle in 4 cases, the lacrimal gland in 3 and the conjunctiva in one. The clinical and histopathological findings are presented. The occurrence of a malignant oncocytoma (oxyphilic adenocarcinoma) in a 58-year-old man, an oncocytoma (oxyphilic adenoma) in 18-month-old girl and a cystic oncocytoma in a 76-year-old woman are particularly remarkable findings. They are compared, on the basis of a review of the literature, with 50 previously reported oncocytic lesions of the ocular adnexa, of which only 3 involved the lacrimal gland.     

Burns of the ocular adnexa

Ocular and periocular burns continue to be very complicated problems to manage clinically. Significant contributions have been made over the last year regarding the care and rehabilitation of the patient with this type of injury. N-acetylglucose aminidase activity in burned corneoscleral tissues may prove to be a good clinical indicator to assess the severity of ocular burns. Tenonplasty and split-thickness dermal grafts offer alternatives to conjunctival flaps when the latter is not practical or possible. Split-thickness dermal grafts are useful for treating persistent corneoscleral defects when an environment conducive to free grafts exists. Tenonplasty is a useful procedure in patients with persistent corneoscleral defects in whom no conjunctiva is available and a free graft is not advisable. Oversized soft contact lenses may prove useful in the care of the burn patient predisposed to forniceal foreshortening. An algorithm has been presented to aid in the management of the patient with periocular burns. High-density porous polyethylene can be successfully utilized in the reconstruction of the facial skeleton in burn patients. Useful guidelines have been proposed for the use of tissue expanders in the lower face and neck in pediatric burn patients.     

Sebaceous carcinoma of the ocular adnexa

Sebaceous carcinoma accounts for 1–5.5% of all eyelid malignancies. This lesion, traditionally considered among the most lethal of all tumors of the ocular adnexa, occurs more commonly in women and in the elderly and has a predilection for the upper lid. Both clinically and histologically, sebaceous carcinoma may masquerade as benign or less invasive conditions such as chalazion, blepharitis, conjunctivitis, meibomitis, superior limbic keratoconjunctivitis, basal cell carcinoma and carcinoma in situ, resulting in delayed diagnosis and treatment. In this review the incidence, clinical presentation, and prognostic factors (both clinical and histopathologic), and treatment modalities are discussed.     

Stem cell therapy for ocular disorders

Cell injury or degeneration occurs in a number of blinding diseases. Therapy has classically consisted of preventing the initial injury or increasing the resistance of cells to injury (cytoprotection). Recently, it has become possible to repopulate tissue compartments with stem cells. This article presents a current summary of ocular stem cell research and applications to disease. It is based on presentations and discussions from the July 2002 international conference "Stem Cells and Glaucoma" sponsored by the Glaucoma Foundation. This meeting, the first of its kind, brought together ophthalmologists, geneticists, immunologists, and developmental biologists working on stem cell development and applications in both human and animal models.     

Lymphomas involving the eye and the ocular adnexa

PURPOSE OF REVIEW: To describe recent advances in the understanding of the pathogenesis of the most common malignant lymphomas that occur as primary and secondary tumors in ocular tissues. RECENT FINDINGS: Advances have been made in the understanding of the genetic alterations in mucosa-associated lymphoid tissue lymphomas, including various chromosomal translocations, such as the most recently described t(3;14)(p14.1;q32) involving the FOXP1 gene. Further, the development of ocular adnexal mucosa-associated lymphoid tissue lymphomas has been associated with Chlamydia psittaci in some geographic areas. Subdivision of diffuse large B-cell lymphoma into clinically prognostic groups had been achieved on the basis of gene expression profiles using complementary DNA microarrays. Tumor-infiltrating cells, such as macrophages, have been demonstrated to be of prognostic significance in follicular lymphoma. SUMMARY: Understanding of the ocular adnexal and intraocular lymphomas has advanced with progress in lymphoma classification systems, namely the World Health Organization lymphoma classification. This knowledge is being fine tuned with advances in technology, such as complementary DNA microarrays. The clinical significance of this scientific progress has yet to be determined.     

Metastatic tumors of the orbit and ocular adnexa

PURPOSE OF REVIEW: The management of cancer metastatic to the orbit and ocular adnexa (eyelid and periocular structures) has changed in recent decades. The purpose of this article is to review the incidence, presentation, and clinical features of metastatic tumors of the orbit and ocular adnexa and discuss their multidisciplinary care. RECENT FINDINGS: The improved survival of patients with common cancers such as breast cancer and prostate cancer, together with aging of the population has led to a higher incidence of patients living with metastatic disease in unusual sites such as the orbit and ocular adnexa. Furthermore, vigilant surveillance and advances in diagnostics have led to increased detection of orbital metastases. Treatment of metastatic lesions in the orbit and ocular adnexa is usually palliative and may include radiotherapy, chemotherapy, hormonal therapy, surgery, or a combination of these modalities. SUMMARY: Breast carcinoma continues to account for the majority of metastatic lesions of the orbit and ocular adnexa. Although the overall prognosis for patients with such lesions remains poor, the longer survival time for patients with breast carcinoma, the availability of novel targeted treatment options and new investigational agents, and advances in radiotherapy techniques may lead to better quality of life and preservation of ocular function for patients with metastatic orbital tumors.     

Histopathologic, immunophenotypic and genotypic analyses in ocular adnexal lymphoproliferative disorders

This study reports on fourteen biopsies from patients presenting to our orbital and oncology service with ocular adnexal lymphoid proliferations between November 1988 to September 1991. The biopsies were studied using histologic, immunophenotypic and genotypic analyses. By histologic criteria, there were two reactive, five indeterminate and seven lymphomatous lesions. On immunophenotypic analysis, there were two monoclonal and 10 polyclonal lesions in the 12 specimens analysed. Genotypic analysis confirmed the histopathologic diagnoses for the reactive lesions by showing them to be germline. It also confirmed that both the histopathologic lymphomas and immunophenotypically monoclonal lesions were clonally rearranged. Genotypic analysis was able to separate the histologically indeterminate group into two subsets: clonally rearranged, of which there was one, and germline, of which there were four. In addition, it demonstrated that immunophenotypic polyclonality cannot always be equated with genotypic polyclonality as was the situation in four out of 10 lesions in our series. The significance of clonal arrangements in the histologically indeterminate and immunophenotypically polyclonal groups can only be determined by prospective study.     

Low-dose radiotherapy for lymphoid lesions of the orbit and ocular adnexa

PURPOSE: There is no agreement within the radiation oncology and ophthalmic communities regarding the treatment of lymphoid lesions of the orbit and ocular adnexa. The authors report their experience with the use of low-dose radiation therapy for malignant and benign lymphoid masses of the orbital region in a series of 54 patients treated between 1985 and 1993. METHODS: All patients received 2 Gy per day for a total of 24 Gy, except when the lesion was extensive, in which case the therapy was 1.5 Gy per day for a total of 25.5 Gy. A diagnosis was established by incisional surgical biopsy in 26 patients and aspiration cytology in 28 patients. Those with a malignant or an indeterminate diagnosis were evaluated with a modified Ann Arbor staging system. RESULTS: Low-dose radiation therapy produced a complete response in 100% of the orbital lymphoid lesions. This local control was maintained in 52 patients (96%) for the first year and in 51 patients (95%) for 5 or more years with a mean follow-up of 7 years. One patient died of causes unrelated to the malignant lymphoma after the first year of observation. The mean age of the 54 patients was 67 years, and the range was 37 to 90 years. The mean ages of presentation for each location were: orbit, 67 years; conjunctiva, 68 years; lacrimal gland, 66 years; and eyelids, 72 years. The female-to-male ratio was 1.25:1 (34 women and 20 men). In this series, 9 patients had benign processes, 38 patients had non-Hodgkin's lymphoma, and 7 patients had abnormalities of indeterminate cause. All histologic subtypes of non-Hodgkin's lymphoma involving the orbit responded equally well to therapy. Forty-five patients had clinically staged disease as follows: stage I, 21 patients; stage II, 4 patients; stage III, 2 patients; and stage IV, 18 patients. Benign disease, diagnosed in 9 patients, was not staged. CONCLUSION: Low-dose radiation therapy proved effective in treating lymphoid lesions of the orbital area. No treatment-limiting complications occurred. The only early side effects were mild xerophthalmia and chemosis in 50% of patients, and the only chronic side effect was mild xerophthalmia in 33% of patients. Cataracts, corneal ulcerations, and retinal injury were not observed.     

Dog bite injuries of the eye and ocular adnexa

Dog bites result in a diverse range of injuries and complications in the periocular region, particularly in school aged children. It is therefore incumbent on the oculoplastic surgeon to be well versed in both acute and long-term management. The intent of this review is to provide a systematic evaluation of the epidemiology, principles of dog bite wound care, and specific considerations related to common patterns of ophthalmic injury. Review of clinical literature from 1976 to 2014. The majority of periocular injuries result from seemingly benign interactions between young children and familiar dogs. Aggressive saline lavage combined with selective debridement of devitalized tissue is essential. High-risk wounds and vulnerable patient groups may benefit from preventive antibiotic coverage as well as appropriate rabies and tetanus prophylaxis. While the nuances of surgical repair are variable given the heterogeneity of presentation, systematic examination and an algorithm-driven approach underlie the optimal management of these complex injuries.     

Kimura disease of the orbit and ocular adnexa.

Kimura disease (KD) is a distinct clinicopathologic entity that has been the subject of considerable confusion and debate. Although common in Asia, KD rarely occurs in non-Asian patients. Kimura disease shares both clinical and histopathologic features with angiolymphoid hyperplasia with eosinophilia (ALHE). Because of this overlap and the rarity of KD in Europe and the United States, KD and ALHE have been used synonymously in the Western medical literature, as they were thought to represent variations of the same disease. Some pathologic reports have called for distinguishing KD and ALHE as two separate entities, based on their histologic features. Kimura disease occurs most commonly in the head and neck region and has been described in the orbit, eyelids, and lacrimal gland more frequently than ALHE. Because both diseases can cause proptosis, lid swelling, ocular dysmotility, or a palpable mass, they should be considered in the differential diagnosis of orbital lesions occurring in adults. We report two cases of KD involving the orbit and ocular adnexa, and review additional cases reported in the literature. The ophthalmic literature does not clearly reflect the current understanding that KD and ALHE are best considered two separate clinicopathologic entities.     

Histopathologic, immunophenotypic and genotypic analyses in ocular adnexal lymphoproliferative disorders.

This study reports on fourteen biopsies from patients presenting to our orbital and oncology service with ocular adnexal lymphoid proliferations between November 1988 to September 1991. The biopsies were studied using histologic, immunophenotypic and genotypic analyses. By histologic criteria, there were two reactive, five indeterminate and seven lymphomatous lesions. On immunophenotypic analysis, there were two monoclonal and 10 polyclonal lesions in the 12 specimens analysed. Genotypic analysis confirmed the histopathologic diagnoses for the reactive lesions by showing them to be germline. It also confirmed that both the histopathologic lymphomas and immunophenotypically monoclonal lesions were clonally rearranged. Genotypic analysis was able to separate the histologically indeterminate group into two subsets: clonally rearranged, of which there was one, and germline, of which there were four. In addition, it demonstrated that immunophenotypic polyclonality cannot always be equated with genotypic polyclonality as was the situation in four out of 10 lesions in our series. The significance of clonal arrangements in the histologically indeterminate and immunophenotypically polyclonal groups can only be determined by prospective study.     

Telemedical evaluation of ocular adnexa and anterior segment

PURPOSE: To report the accuracy of telemedical slit-lamp evaluation in examination of ocular adnexa and anterior segment. METHODS: By means of contingency tables, slit-lamp findings by live examination and by real-time telemedicine were compared in 50 eyes of 25 patients. RESULTS: Sensitivity percentages (proportion with findings correctly identified by telemedicine)/specificity percentages (proportion without the finding that was correctly identified by telemedicine) were 100/64 for eyelid mass, 100/85 for conjunctival pigment, 100/100 for posterior synechiae, 80/0 for blepharitis, 83/93 for iridotomy, 70/93 for pinguecula, 75/93 for iris lesions, 56/98 for corneal scar, 0/100 for chamber inflammation, 57/93 for nuclear cataract, and 37/100 for intraocular lens presence. CONCLUSIONS: By means of monocular slit-lamp telemedical evaluation, clinical findings with high contrast cues for color and depth have the highest sensitivity and specificity.