Brett O'Donnell

Orbital Metastasis: Clinical Features, Management and Outcome

Purpose: To review the clinical features, treatment, outcome and survival of metastatic tumors of the orbit. Materials and Methods: Retrospective, non-comparative, chart review of 80 patients with orbital metastasis treated in four tertiary orbital centres in Australia. Results: The study included 80 patients of which, 44 were male with a mean age of 60 years. Orbital involvement commonly presented late in a multisystemic disease; however, the orbit was the first presentation in 15% of the cases. Diplopia (48%), pain (42%), and visual loss (30%) were the commonest symptoms at presentation; whereas proptosis (63%), strabismus (62%), and visual loss (41%) were the most frequent clinical signs. Computed tomography commonly showed a solid enhancing mass (42 cases) located within the orbital fat (43%), or enlarging an extraocular muscle (28%). Breast carcinoma (29%), melanoma (20%), and prostatic cancer (13%) were the most frequent histological types. Treatment was often multi-disciplinary and modalities included radiotherapy, chemotherapy, hormone therapy, surgery, and immunotherapy. Survival was limited to 1.5 years after diagnosis independent of the histological type, with 29% of patients alive after 17 months follow-up. Conclusions: A high index of suspicion and appropriate intervention with histological diagnosis can help in the management and quality of life in patients with metastatic orbital disease. Overall survival is limited and we encountered statistical limitations proving differences in the survival based on the sub-type of primary tumour involved. Metastatic orbital melanoma presented a higher incidence when compared with previous studies, probably due to the increase frequency of skin found in the Australian population.

Dacryocystorhinostomy for epiphora in the presence of a patent lacrimal system.

Purpose: To evaluate the success rate of dacryocystorhinostomy (DCR) for epiphora in patients with a clinically patent lacrimal drainage system.

Clinical features and management of tumors affecting the lacrimal drainage apparatus

Purpose: To report the clinical features of a series of patients with lacrimal drainage apparatus tumors and present guidelines for management based on histopathology. Methods: A noncomparative retrospective chart review of the clinical, imaging, and pathologic findings of 37 patients presenting to four regional orbital surgery departments with tumors affecting the lacrimal drainage apparatus between 1990 and 2004. Results: There were 37 patients, of whom 62% were male. The mean age at referral was 54 years. Epiphora, a palpable mass, and dacryocystitis were the most common presentations. Two thirds of the tumors were epithelial, with carcinomas being the most frequent (38%), followed by papillomas (27%). Lymphomas were the most common nonepithelial malignancy (30%). Epithelial tumors were more common in men (87%), whereas lymphomas were more common in women (57%). Treatment modalities included surgery, in addition to radiotherapy and/or chemotherapy and immunotherapy. Mean follow-up was 38 months. Thirty-three patients (89%) remain alive without evidence of disease and 4 patients died of recurrence and/or metastases. Conclusions: Lacrimal drainage apparatus tumors require careful initial management to ensure adequate local and systemic disease control. Atypical mucosa encountered during dacryocystorhinostomy should be biopsied and small papillomas or pedunculated tumors excised and analyzed with frozen sections. If a diffuse or infiltrative mass is encountered, it should be biopsied and managed on the basis of histopathology and extent of disease. Lymphomas should be treated according to protocols, whereas noninvasive carcinoma and extensive papillomas require complete excision of the system. Invasive disease requires en bloc excision. Long-term follow-up is essential for early detection of recurrence.

The ophthalmic side-effects of imiquimod therapy in the management of periocular skin lesions

Aim To describe the ophthalmic side-effects of topical imiquimod for periocular actinic keratoses, squamous cell carcinoma in situ and basal cell carcinoma. Method A retrospective study was carried out in two centres of all patients who underwent topical imiquimod therapy between January 2004 and January 2009. Imiquimod was applied three times weekly for 4–6 weeks. Diagnosis of the lesions, complications, clinical resolution and long-term ophthalmic side-effects was recorded. Patients on therapy were reviewed fortnightly and then every 6 weeks following completion of treatment. Results 47 patients were identified; the mean age was 74 years. 37 patients had actinic keratoses, seven patients had Bowen disease, and three patients had BCC. The lower lid was the commonest site involved (68%). Application site erythema occurred in all patients. Conjunctivitis occurred in 15 patients, and six patients complained of ocular stinging on application of imiquimod. One patient had a staphylococcal keratitis, which responded to topical antibiotic and steroid therapy. Two patients required oral antibiotics for preseptal cellulitis. Three patients had delayed conjunctivitis at a mean of 2.3 weeks. Nine patients discontinued imiquimod due to ocular irritation and conjunctivitis, of whom four patients recommenced and finished the treatment after a rest period. At a mean follow-up of 16 weeks, 34 patients had clinical resolution of the periocular lesions and no patient had any residual ophthalmic side-effects from imiquimod. Conclusion Conjunctivitis and ocular stinging were the commonest ophthalmic side-effects encountered with the application of imiquimod for periocular skin lesions. These effects were temporary and resolved on terminating the imiquimod therapy.

The failed probing

The management of failed probing for congenital nasolacrimal duct obstruction is given by a panel of authors. Treatment options examined are repeat probing, inferior turbinate infracture, closed lacrimal intubation and dacryocystorhinostomy. There is considerable variation as to the timing of these interventional techniques.

Malignant fibrous histiocytoma of the eyelid: differential diagnosis and management.

Purpose: Malignant fibrous histiocytoma (MFH) is a pleomorphic soft tissue sarcoma that occurs rarely in the periocular region. The purpose of this study was to present a case series of periocular MFH and to discuss the differential diagnosis and management. Methods: This is a retrospective case review of patients diagnosed with periocular MFH from tertiary hospitals and private practices. Results: Four patients, two women and two men with periocular MFH, had a mean age of 81 years (range, 72 to 85 years). All tumors were <5 cm in diameter and of storiform pleomorphic histologic subtype. One was located deep and 3 presented superficially. The initial diagnoses were sarcomatoid carcinoma, atypical fibroxanthoma, and leiomyosarcoma that were subsequently reclassified as MFH. One patient had a frozen section, 1 had fast track paraffin section margin control, and 2 had no frozen section margins at the initial excisions. Histology showed 2 negative margins, 1 close margin, and 1 positive margin that were cleared with wide local excision. Local recurrence occurred in 3 cases, and of these, 1 required orbital exenteration. Adjuvant radiotherapy was given to 3 patients. One had regional node metastases. There were no distant metastases or tumor-related deaths. Mean follow-up period was 36 ± 35 months (median, 30; range, 1 to 84 months). Conclusions: This study highlights the difficulties in the clinicopathologic diagnosis of periocular MFH and in particular the distinction of more superficial tumors from atypical fibroxanthoma. The mainstay of treatment is complete surgical excision with wide margins, and consideration should be given to histologic margin control in addition to adjuvant radiotherapy.

External ophthalmic findings in multiple endocrine neoplasia type 2B

Purpose: To identify external ophthalmic abnormalities in multiple endocrine neoplasia type 2B (MEN2B), which may facilitate early detection and prophylactic thyroidectomy to prevent medullary thyroid carcinoma.

Lesions mimicking lacrimal gland pleomorphic adenoma.

Aim To report a series of patients with lacrimal gland lesions simulating the clinicoradiological features of lacrimal gland pleomorphic adenoma (LGPA). Methods Multicentre retrospective, interventional case series. Clinical records of all patients with lesions mimicking LGPA seen in five orbital units were reviewed. Results The study included 14 patients (seven men and seven women) with a mean age of 50.9 years. The diagnosis of LGPA was made in all cases by experienced orbital surgeons, based on clinicoradiological features, and lacrimal gland excision was performed. Postoperative histology revealed lymphoma (four patients), chronic dacryoadenitis (three patients), adenoid cystic carcinoma (two patients), Sjogrens syndrome (two patients), cavernous haemangioma (one patient), benign lymphoid hyperplasia (one patient) and granulomatous dacryoadenitis (one patient). Comparison with the total number of histologically confirmed LGPA cases seen during the study period revealed that 22.6% of cases of suspected LGPA were misdiagnosed based on clinicoradiological criteria. Conclusions Many different lesions may mimic the clinicoradiological features of LGPA. The accepted clinicoradiological criteria used for the diagnosis of LGPA have a high false-positive rate, even in experienced hands. Based on this study, the authors believe that fine-needle aspiration biopsy or intraoperative biopsy and frozen section diagnosis may help reduce unnecessary lacrimal gland excision.

Enucleation versus evisceration

The choice of enucleation and evisceration for removal of an eye remains controversial in certain circumstances. An international panel was asked to give their surgical management of two clinical cases that require either enucleation or evisceration. Case one follows multiple vitreoretinal procedures, and the risk of sympathetic ophthalmia is considered in the surgical management. Case two has had postoperative endophthalmitis, and the possibility of implant infection following insertion of an orbital implant with evisceration and enucleation is discussed.

Orbital apex syndrome secondary to Pseudallescheria boydii fungal sinusitis in an immunocompetent patient.

Orbital apex syndrome secondary to mucormycosis in immunocompromised patients is well described; however, few reports exist of a paranasal sinus mycetoma resulting in this presentation in the immunocompetent patient. The case is reported of a 92‐year‐old man who developed orbital apex syndrome secondary to a sphenoidal sinus mycetoma of Pseudallescheria boydii.