Clinico-radiological findings of neuroendocrine tumour metastases to the orbit.

PURPOSE: We present the clinico-radiological findings of neuroendocrine tumour metastases to the orbit. METHODS: This was a multicentre, retrospective study of patients with neuroendocrine tumour metastases to the orbit. Data was collected from medical records across five different sites within Australia and the United Kingdom. RESULTS: Nine patients (eleven lesions) were identified. The most common presenting complaint was diplopia (5/9, 56%). Disease occurred bilaterally in two patients. Seven patients (78%) had extraocular muscle involvement. The lateral recti (4/9, 44%) and superior recti (2/9, 22%) were the most commonly affected. Ocular presentation preceded primary tumour diagnosis in three patients (33%). On orbital imaging, metastases were most commonly reported as well circumscribed, ovoid or round, heterogeneous, contrast-enhancing masses. Features of intralesional haemorrhage and bony invasion are uncommonly reported. CONCLUSIONS: Neuroendocrine tumour metastasis to the orbit is uncommon. Metastases have a propensity for the extraocular muscles, commonly presenting as heterogeneous, well circumscribed, contrast-enhancing lesions on neuroimaging. New ocular symptoms, a history of neuroendocrine tumours, and these radiological findings, should lead to high clinical suspicion of metastatic disease. Atypical findings warrant biopsy to exclude other causes of orbital lesions.

The 2017 Doyne Lecture: the orbit as a window to systemic disease.

A very large number of disorders affect the orbit, and many of these occur in the setting of systemic disease. This lecture covers selected aspects of orbital diseases with systemic associations in which the author has a particular clinical or research interest. Spontaneous orbital haemorrhage often occurs in the presence of bleeding diatheses. Thrombosis of orbital veins and ischaemic necrosis of orbital and ocular adnexal tissues occur with thrombophilic disorders, vasculitis, and certain bacterial and fungal infections. Non-infectious orbital inflammation commonly occurs with specific inflammatory diseases, including Graves' disease, IgG4-related disease, sarcoidosis, Sjögren's syndrome and granulomatosis with polyangiitis, all of which have systemic manifestations. IgG4-related ophthalmic disease is commoner than all these except Graves' orbitopathy. Some of these orbital inflammatory diseases are associated with an increased risk of B-cell lymphoma, usually marginal zone lymphoma of MALT type. Ocular adnexal lymphoma also has an association with infectious agents including Helicobacter pylori and Chlamydia psittaci. Orbital metastasis may be the first presentation of systemic malignancy. A number of orbital neoplasms occur in the setting of familial cancer syndromes, including Neurofibromatosis types 1 and 2. Study of the genetics and molecular biology of orbital diseases such as Graves' orbitopathy and idiopathic orbital inflammatory disease will yield useful information on their diagnosis and management.

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.

Orbital decompression in Graves' orbitopathy: efficacy and safety.

BACKGROUND: Vision-threatening compressive optic neuropathy occurs in a minority of patients with Graves' orbitopathy. Surgical orbital decompression, systemic glucocorticoids and orbital irradiation are treatment options. Orbital decompression is being performed on an increasing number of patients for other indications such as corneal exposure and disfiguring proptosis. AIMS: To examine the outcomes of surgical orbital decompression for Graves' orbitopathy by one surgeon. METHODS: An analysis of a retrospective case series of 88 consecutive patients (151 orbits) who underwent orbital decompression for Graves' orbitopathy between April 1991 and November 2002. RESULTS: The indication for surgery was compressive optic neuropathy for 57 orbits; 94 orbits had an indication other than optic neuropathy. Of those with optic neuropathy, 94% had improvement or maintenance of visual acuity and 93% had improvement in colour vision after decompression. The overall mean reduction in proptosis was 4.7 mm. Of all patients, 30% had new or worsened diplopia postdecompression. Patients with optic neuropathy were more likely to develop new or worsened diplopia than those without optic neuropathy. CONCLUSIONS: Orbital decompression is a safe procedure and effective in improving vision in compressive optic neuropathy. It is effective in reducing proptosis, therefore improving exposure keratopathy and cosmesis. However, new or worsened diplopia is a significant postoperative complication, and subsequent strabismus surgery might be required. This is an important consideration, especially for patients undergoing surgery for non-optic neuropathy indications.

Scarring in external dacryocystorhinostomy: fact or fiction?

PURPOSE: To determine patients' long-term satisfaction with external dacryocystorhinostomy (DCR) with respect to the visibility of the scar and the resolution of symptoms. METHOD: A single postal questionnaire with stamped addressed envelope for reply was sent to 204 sequential external primary DCR patients operated on by AAM from January 1999 to April 2002. RESULTS: Of the 79% who replied, 67% rated the scar as invisible with 97% happy with the scar. In 92% the epiphora was either improved or cured and 85% would recommend the operation. Younger patients were significantly more likely to rate the scar as visible but equally likely to rate the scar as good or better. Women were no more likely than men to rate the scar as visible, but if visible, were more likely to consider the scar poor. CONCLUSIONS: There was an excellent response rate for a single questionnaire. Patient satisfaction with the DCR scar is very high, with the majority considering the scar invisible. Only 3% remained unhappy with the scar. Overall patient satisfaction with external DCR is very high, for the majority of patients the procedure is effective, and 85% would recommend the operation.

Orbitofrontal cholesterol granuloma.

Cholesterol granuloma of the orbital bones is a rare but readily recognisable condition. It is an osteolytic lesion with a granulomatous reaction surrounding cholesterol crystals, old haemorrhage and a fibrous capsule. There is a male preponderance and it usually occurs in young or middle-aged men. It is treatable with drainage and curettage via an orbitotomy, and craniotomy or wide bone removal is almost never required. Six cases of this condition were reviewed to highlight the typical clinical presentation, computed tomography and magnetic resonance results, and surgical management.

A brief history of punctoplasty: the 3-snip revisited.

PURPOSE: To determine when and how to perform punctoplasty, review the history, and determine the efficacy of posterior ampullectomy. METHOD: Retrospective analysis of 53 cases of punctal stenosis, operated by a single surgeon by means of posterior ampullectomy via 3-snips. A review of the history of the procedure from papers found with a Medline search for 1-snip, 2-snip, 3-snip, and punctoplasty. RESULTS: In all, 102 sets of notes were reviewed. The age range was from 9 to 89 years with a mean age of 56 years. A total of 74% of patients were female. We excluded 22 patients who had additional surgery and 16 patients who elected not to have surgery. There was no comment as to success or failure in four sets of notes and seven notes could not be found; these cases were also excluded. Of the remaining 53 patients, success was documented in 49 cases, or 92%. CONCLUSIONS: The historical review helps explain the debate about the procedure. The retrospective review confirms that posterior ampullectomy via 3-snips is an effective procedure.

Upper lid loading with gold weights in paralytic lagophthalmos: a modified technique to maximize the long-term functional and cosmetic success.

PURPOSE: To describe a modified fixation technique to improve the long-term functional and cosmetic success of upper lid loading with gold weights. METHOD: A retrospective single surgeon study of combined upper pre-tarsal and direct levator fixation of the gold weight. The minimum follow-up period was 12 months. RESULTS: Gold weights were implanted with the modified technique into 29 patients. The gold weight was removed in two cases due to infection and in one case due to ptosis. There was no loss of function over the follow-up period and the cosmetic outcome was good or very good in all cases. The follow-up ranged from 12 to 84 months with a mean of 40 months. CONCLUSIONS: Combined upper pre-tarsal fixation and direct levator fixation is effective in maximizing both the long-term functional and cosmetic success of upper lid loading with gold weights in paralytic lagophthalmos.

A technique for reconstruction of upper lid marginal defects.

BACKGROUND/AIMS: Reconstruction of large full thickness upper lid defects that cannot be closed directly often rely on utilising the lower lid. An example is the Cutler Beard procedure. A one stage technique for repair of large horizontal upper lid defects utilising local posterior and anterior lamella advancement flaps is described and the results reported. METHOD: Eight cases with upper lid defects repaired utilising this technique were reviewed retrospectively. The procedures were carried out by one surgeon. The upper lid lesions were removed under frozen section control. The mean follow up time was 35 months. RESULTS: All patients had a good cosmetic result. One patient had a recurrence of the upper lid lesion. Two patients complained of corneal irritation from lanugo hairs. The technique was modified to prevent this complication. CONCLUSIONS: Large upper lid marginal defects can be readily repaired using the technique described with local advancement flaps with no significant complications.

A prospective randomized study comparing division of the pedicle of modified hughes flaps at two or four weeks.

PURPOSE: To compare the incidence of upper and lower eyelid malposition or other complications when the pedicle in modified Hughes flap repair of the lower eyelid is divided at 2 versus 4 weeks. METHODS: A prospective randomized study. Data were collected on patient age and sex, horizontal tumor dimension, upper and lower eyelid position before surgery and 3 months after division of the pedicle, and any complications. RESULTS: Complete data from 60 patients were collected, with 32 patients in the 2-week group and 28 patients in the 4-week group. The two groups were not significantly different in terms of sex, age, or tumor dimensions. In the 2-week group, the upper eyelid was within 1 mm of its preoperative position at 3 months in 25 of 32 patients (78.1%) and in 26 of 28 (92.8%) in the 4-week group. The lower eyelid was within 1 mm of its normal position in 27 of 32 patients in the 2-week group (84.4%) versus 25 of 28 (89.3%) in the 4-week group. Statistical analysis showed no significant difference between the 2- and 4-week groups for upper and lower eyelid position at 3 months. Other complications were uncommon and evenly spread between the two groups. CONCLUSIONS: A modified Hughes flap pedicle may be divided at 2 weeks with no increased incidence in upper or lower eyelid malposition or other complication.

Orbital and optic nerve trauma.

The ability to measure orbital volume expansion and correlate this with later enophthalmos has advanced radiological assessment of patients with orbital floor fractures. Attempts have also been made to classify orbital floor fractures on the basis of their configuration on CT scan and to correlate this with outcome in terms of ocular motility. A small subset of relatively undisplaced floor fractures with tight entrapment of tissues occurring most commonly in young patients has been identified, and early surgical intervention has been recommended for these. The range of alloplastic materials available for orbital rim and wall fractures has increased, and complications related to various alloplastic implants continue to be reported. Optic nerve trauma has received considerable attention but remains a difficult management area. Recent literature on this subject shows no definite benefit for optic nerve decompression or high dose corticosteroids, although no prospective randomized study has been possible.

Fulminant orbital cellulitis with complete loss of vision.

Bacterial orbital cellulitis is a condition that rarely presents with complete loss of vision. A case is reported of a 69-year-old man who presented with fulminant onset of proptosis, significant ophthalmoplegia and no perception of light. Computed tomography showed no evidence of paranasal sinus disease. Despite treatment with intravenous flucloxacillin, ceftriaxone and metronidazole, and later, penicillin after Streptococcus pyogenes was grown from tissue culture, there was no improvement in vision; however ocular motility returned to normal.

Australasian orbital and adnexal Wegener's granulomatosis.

OBJECTIVE: To report a retrospective case series of 29 Australian and New Zealand patients with orbital and adnexal Wegener's granulomatosis (WG). DESIGN: Retrospective case series. PARTICIPANTS: Twenty-nine cases of orbital and adnexal WG were identified. METHODS: A number of oculoplastic surgeons and other clinicians in Australia and New Zealand was asked about their experience with orbital and adnexal WG. Clinical data regarding these cases were conveyed by means of a questionnaire. Cases of ophthalmic WG without features of orbital or adnexal disease were excluded. MAIN OUTCOME MEASURES: Data obtained from the questionnaire includes age, gender, limited or generalized disease, antineutrophil cytoplasmic antibody (ANCA) status, symptoms and signs: nasolacrimal obstruction, sinusitis, fistula/orbital bone erosion, orbital mass/proptosis, extraocular muscle/diplopia, visual acuity reduction caused by optic nerve compression, orbital pain, lid edema/erythema, biopsy status, and treatment status. RESULTS: Twenty-nine patients with orbital and adnexal WG were identified and described. Symptoms included awareness of an orbital mass, epiphora, orbital pain and diplopia. Signs included an orbital mass or proptosis (69%), nasolacrimal duct obstruction (52%), limited ocular rotations (52%), lid erythema and edema (31%), bony destruction (21%), and reduced visual acuity (17%). Two patients had a persistent nasolacrimocanthal fistula. Cytoplasmic pattern antineutrophil cytoplasmic antibodies (c-ANCA) were present in 52% of patients, and in 9 of 10 patients with generalized disease. However, c-ANCA was positive in only 32% (6 of 19) of patients with limited WG. Perinuclear pattern antineutrophil cytoplasmic antibodies (p-ANCA) was positive in 10% of cases. CONCLUSIONS: To diagnose and treat ophthalmic WG effectively, the clinician must be aware of its protean orbital and adnexal manifestations. WG may occur with or without systemic involvement, and c-ANCA was negative in approximately half our cases. Our cases also demonstrated two orbital fistulae, an observation previously believed to be rare.

Nontraumatic subperiosteal orbital hemorrhage.

PURPOSE: Nontraumatic subperiosteal orbital hemorrhage (NTSOH) has been reported rarely in association with sudden elevation of cranial venous pressure, generalized diseases with bleeding diatheses, and paranasal sinusitis. To define more clearly the clinical and imaging characteristics of NTSOH, we evaluated nine cases seen by the authors and reviewed previous case reports. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients (10 eyes) with NTSOH. INTERVENTION: All patients underwent computed tomography scans of the orbits. Patients with typical clinical and imaging features and normal visual function were observed. Those with an uncertain diagnosis or visual compromise underwent surgical drainage of the hematoma. MAIN OUTCOME MEASURES: Resolution of proptosis, diplopia, lid swelling, and ptosis. RESULTS: Nine patients ranging from birth to 73 years of age were identified. All were females. The lesions were located superiorly in eight patients (one patient had bilateral lesions) and medially in one patient. Most were associated with sudden elevation in venous pressure (vomiting, strangulation, straining), and most required no surgical intervention. The bilateral case occurred in the setting of disseminated intravascular coagulation and was the only case associated with visual loss possibly resulting from ischemic optic neuropathy. CONCLUSIONS: Nontraumatic subperiosteal orbital hemorrhage may occur at any age, usually secondary to sudden elevation in venous pressure. It is nearly always superior. The clinical and radiologic features are sufficiently characteristic to allow conservative treatment in the absence of visual compromise.

Sino-orbital fistula: two case reports.

A fistula between the paranasal sinuses and the orbit as a late complication of orbital fractures is rare and may present with intermittent symptoms due to air passing into the orbit. A case note review of two patients with sino-orbital fistula is presented. Two patients, 23- and 30-year-old males, presented with intermittent symptoms of globe displacement, diplopia or discomfort months after repair of an orbital floor fracture with a synthetic orbital floor implant. The symptoms occurred after nose blowing. They were both cured by removal of the implant and partial removal of the tissue surrounding the implant. A sino-orbital fistula may complicate the otherwise routine repair of an orbital floor fracture, but may be cured by removal of the implant and part of the surrounding pseudocapsule.

Reversal of floppy eyelid syndrome with treatment of obstructive sleep apnoea.

PURPOSE: To describe the beneficial effects of treatment of obstructive sleep apnoea on the symptoms and signs of floppy eyelid syndrome. METHOD: A case of sleep apnoea with associated floppy eyelid syndrome is reported. RESULTS: A 32-year-old man presented with left floppy eyelid syndrome and a known diagnosis of obstructive sleep apnoea. He underwent treatment for 4 years with continuous positive airways pressure by mask during sleep and the symptoms and signs of his floppy eyelid syndrome disappeared. CONCLUSION: Treatment of obstructive sleep apnoea may reverse the changes of floppy eyelid syndrome.

Secondary haemorrhage after dacryocystorhinostomy.

OBJECTIVE: To describe the incidence, clinical features, possible risk factors and outcome of patients suffering secondary haemorrhage after dacryocystorhinostomy (DCR). METHODS: Records of all patients undergoing external DCR by one surgeon (AAM) over a 6-year period were analysed retrospectively. RESULTS: After 293 DCR operations in 260 patients, 10 patients had a secondary haemorrhage (one after bilateral DCR), usually between days 4 and 10 post-operatively (mean 6.8 days). Six of the 10 were hospitalized for 2-5 days (mean 2.8 days), all of whom had nasal packing, intravenous antibiotics and intravenous fluids; none required blood transfusion. Three patients had immune system defects, three had used non-steroidal anti-inflammatory drugs (NSAID) and four had no obvious risk factors. All 10 had a successful outcome following DCR (compared to an overall failure rate of 25/293 (8.5%): 16 anatomical and nine functional). CONCLUSIONS: Secondary haemorrhage after DCR was found to occur after 3.8% of DCR. Risk factors include being immunocompromised and taking NSAID prior to surgery. The haemorrhage did not adversely affect surgical outcome.

Subconjunctival fat prolapse.

PURPOSE: Subconjunctival orbital fat prolapse has been rarely described in the literature and is frequently confused with other conjunctival tumours, such as dermolipoma. The purpose of the present study was to describe the clinical features and treatment of a series of patients with subconjunctival orbital fat prolapse. METHODS: Review of clinical records. RESULTS: Twelve patients with subconjunctival orbital fat prolapse were identified. Eight had a superotemporal prolapse and five of these were bilateral. All but one were male and most were elderly and tended to be obese. Five of these patients underwent surgical excision of the prolapsed fat for symptoms of discomfort or for cosmesis. Four atypical unilateral cases were seen in younger patients with fat prolapse occurring superonasally in three and inferiorly in one. In one case there was a history of trauma CONCLUSIONS: Subconjunctival orbital fat prolapse typically occurs superotemporally in obese elderly males and is easily differentiated from other lesions, such as dermolipoma. The prolapsed orbital fat may be excised via the conjunctiva.