- [Show abstract] [Hide abstract] ABSTRACT: Orbital Langerhans cell histiocytosis (LCH) without bone involvement is rare. Isolated involvement of an extraocular muscle without bone change, to the best of the authors' knowledge, has not been previously reported. They describe a unique case of unifocal LCH of the superior oblique muscle with no bone involvement. A 16-year-old girl presented with a 2-week history of diplopia and headache. CT and MRI showed an enlarged right superior oblique muscle with no associated bone involvement. Biopsy with partial debulking revealed LCH and staging confirmed unifocal disease. The lesion underwent complete radiologic resolution following surgery. There was no recurrence after 16 months follow up.
- [Show abstract] [Hide abstract] ABSTRACT: Importance The literature on Merkel cell carcinoma (MCC) of the eyelid remains scarce, and there has yet to be a study using the most up-to-date TNM staging system for this rare but aggressive tumor.Objective To analyze the TNM stage, management, and outcomes of patients with MCC of the eyelid.Design, Setting, and Participants Retrospective case series of 21 patients from 5 tertiary referral centers in the United Kingdom and Australia with primary MCC of the eyelid presenting at a median age of 77 years, with median follow-up of 54 months. Tumors were staged according to the American Joint Committee on Cancer, 7th edition, TNM criteria for eyelid carcinoma and MCC.Main Outcomes and Measures TNM stage, treatment modalities, and clinical outcome.Results The eyelid carcinoma TNM stages were T2aN0M0 for 5 patients, T2bN0M0 for 7 patients, T3aN0M0 for 4 patients, T3bN0M0 for 3 patients, T2bN1M0 for 1 patient, and T3aN1M0 for 1 patient. The MCC TNM stages were T1N0M0 for 12 patients, T2N0M0 for 7 patients, T1N1M0 for 1 patient, and T2N1M0 for 1 patient. One patient had a sentinel lymph node biopsy, and 8 patients underwent head/neck imaging. Eighteen patients underwent a wide local excision, 12 with a paraffin section and 6 with a frozen section. Two patients underwent Mohs surgery, 1 of whom required an orbital exenteration. Twelve patients (57%) received adjuvant radiotherapy, and 2 patients received chemotherapy. The local recurrence rate was 10%, the regional nodal recurrence rate was 10%, and the distant metastatic recurrence rate was 19%. The lowest T category tumor metastasizing to both regional nodes and distant locations was a T2a (eyelid TNM)/T1 (Merkel TNM) tumor measuring 8 mm. Two patients with T3a (eyelid TNM)/T2 (Merkel TNM) tumors died of metastatic MCC.Conclusions and Relevance The majority of patients with MCC of the eyelid present with localized eyelid disease of T category T2 (eyelid TNM)/T1 (Merkel TNM). A wide local excision with margin control remains the mainstay of treatment, whereas the use of radiotherapy is institution specific. Tumors with a low T category are associated with regional nodal and distant metastatic disease. It may therefore be reasonable to consider a sentinel lymph node biopsy or strict regional lymph node surveillance for all MCCs of the eyelid, regardless of T category or size.
- [Show abstract] [Hide abstract] ABSTRACT: ABSTRACT We present a case report where hepatocellular carcinoma metastasis to the sphenoid wing was the initial presentation of an occult primary.
- [Show abstract] [Hide abstract] ABSTRACT: Background/aims: Immunohistochemical characterisation of orbital cavernous haemangiomas (CHs) with respect to proliferative capacity, hormone receptor status and vascular differentiation. Methods: Eleven cases of orbital CHs were reviewed. Immunohistochemical stains for Mib-1, proliferating cell nuclear antigen (PCNA), Bcl-2, estrogen and progesterone receptors (ER & PR), CD31, D2-40, and VEGF were investigated in 11 specimens. Results: Immunohistochemical staining revealed positivity for PCNA in ten of the 11 cases (91%). Bcl-2 was positive in 8 cases (73%). VEGF and PR were each weakly positive in 3 cases. All cases were negative for Mib-1, ER and D2-40. The staining was localized around the endothelium. Conclusion: This is the first study to characterise in detail the immunohistochemical features of orbital CHs. The proliferative markers PCNA and Mib-1 show discordant expression in these lesions and the expression of PCNA and Bcl-2 in the absence of Mib-1 is indicative of low proliferative potential. Small subsets of these tumors express PR and VEGF, which may partly explain the proliferative capacity of some orbital CHs.
- [Show abstract] [Hide abstract] ABSTRACT: Orbitofrontal cholesterol granuloma is a rare entity that has a predilection for men in their fourth or fifth decade and occurs almost exclusively within the frontal bone overlying the lacrimal fossa. Surgery by drainage and curettage is virtually curative in all cases and recurrence is very rare. We describe the case of a 77-year-old man with recurrent orbitofrontal cholesterol granuloma 11 years after surgery, presumably due to subtotal curettage. The potential use of an endoscope to aid complete removal of lesion is discussed.
- [Show abstract] [Hide abstract] ABSTRACT: The use of endoscopic orbital and optic nerve decompression for traumatic optic neuropathy and dysthyroid orbitopathy have been well documented; however, reports on endoscopic decompression for benign orbital apex lesions are scarce. The records of two patients who underwent endoscopic decompression of the bony orbit for progressive visual loss were reviewed. Patient 1 had fibrous dysplasia and presented with headache and visual field defects. Patient 2 had sphenoid wing meningioma and multiple previous attempts of transcranial tumor resection and orbital decompression. Both had progressive visual deterioration and ultimately underwent transnasal endoscopic orbital decompression. Post-operatively, both patients had subjective and objective improvement in visual function and compressive symptoms. No complications from the endoscopic decompression were observed in both patients. Transnasal endoscopic approach may be a viable option for decompression of benign orbital apex lesions.
- [Show abstract] [Hide abstract] ABSTRACT: Fibromas of the tendon sheath are slow-growing, benign tumors most commonly found on the hands and wrist. A fibromas of the tendon sheath arising from the medial canthal tendon presented as an enlarging nodule that had been present for 40 years. The fibroma was identified by microscopy and immunohistochemistry, and surgical resection appears to have been curative.
- [Show abstract] [Hide abstract] ABSTRACT: To describe a case series of patients with anterior orbital invasion by medial canthal basal cell carcinoma (BCC) managed with non-exenterating surgery. International, multicenter, retrospective, noncomparative, consecutive case series. Twenty patients identified from the individual institutions' databases with histologically confirmed orbital invasion by periocular BCC. Examination of charts, relevant imaging, and histopathologic data. Demographics; clinical characteristics and radiologic features; histopathologic features; surgical techniques for excision, reconstruction, and subsequent procedures; complications; visual acuity; and recurrence. Twenty patients were identified. Twelve of 20 patients (60%) had recurrent BCCs, with 1 patient having had prior radiotherapy for previously incomplete excision. Eighteen of 20 patients (90%) had a palpable mass, 16 of 20 patients (80%) had clinical involvement of the nasolacrimal system, and 1 of 20 patients (5%) had limited extraocular movements. Preoperative radiologic evidence of orbital invasion was found in 10 of 20 patients (50%). Histologic evidence of orbital invasion was present in every patient, the subtypes being infiltrative (9/20, 45%), nodular (4/20, 20%), micronodular (2/20, 10%), multifocal (1/20, 5%), and mixed (4/20, 20%); extratumoral perineural invasion was present in 1 patient (5%). Final margins were clear in 18 of 20 patients (90%), positive in 1 of 20 patients (5%), and unclear in 1 of 20 patients (5%). Reconstruction was by direct closure in 1 patient and by a variety of standard oculoplastic flaps and grafts in 19 of 20 patients (95%). Twelve of 20 patients (60%) had postoperative extraocular muscle movement restriction, and 15 of 20 patients (75%) had epiphora. Subsequent revision procedures were needed in 12 of 20 patients (60%), including insertion of a lacrimal bypass tube and revision of medial canthal position. At a mean follow-up of 38 months, 18 of 20 patients (90%) were still alive (2 deaths due to other causes) with 1 recurrence (exenterated). Postoperative visual acuity was within 2 Snellen lines of preoperative visual acuity in 17 of 20 patients (85%). With careful planning and margin control, conservative surgery in this highly selected group proved possible with a low rate of disease recurrence, albeit with a relatively short follow-up. Postoperative complications, such as epiphora and ophthalmoplegia, were largely expected; most patients underwent subsequent revision procedures to address these and other complications. The author(s) have no proprietary or commercial interest in any materials discussed in this article.
- [Show abstract] [Hide abstract] ABSTRACT: To report a series of patients with lacrimal gland lesions simulating the clinicoradiological features of lacrimal gland pleomorphic adenoma (LGPA). Multicentre retrospective, interventional case series. Clinical records of all patients with lesions mimicking LGPA seen in five orbital units were reviewed. 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), Sjogren's 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. 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.
- [Show abstract] [Hide abstract] ABSTRACT: Primary orbital intraosseous hemangioma represents a rare, histopathologically benign, vascular tumor of the bony orbit. Only 41 cases have been documented in the literature to date. The authors present 4 new cases of the disease and review the relevant literature. Retrospective, multicenter case note analysis of 4 patients with histopathologically confirmed primary orbital intraosseous hemangioma and a systematic review of the English-language literature. Four new cases of cavernous haemangiomata are presented with varying clinical manifestations, radiologic appearances, and treatments. Literature review (including the present 4 cases) yielded 45 cases in total. Presentation is often in the fourth and fifth decades (42% cases), the frontal bone being most commonly affected, followed by the zygoma, sphenoid, and maxilla. Intracranial extension occurred in 4 cases. Median duration of symptoms before presentation was 12 months (range, 1 month to 15 years) and the most frequent presentation was a painless mass, often on the orbital rim. The radiologic findings are reviewed. Histopathologically, the lesions were cavernous in 80%, capillary in 17%, and mixed in 3%; the capillary subtype seemed to be associated with more aggressive disease. Treatment was mainly by surgical excision and occasionally complicated by significant blood loss; preoperative embolization of lesions may reduce bleeding. Primary orbital intraosseous hemangioma is a rare vascular tumor that typically presents with a mass effect in the orbits of patients in the fourth and fifth decades of life. Preoperatively, it is important to be cognizant of the possible diagnosis as surgery can be complicated by life-threatening hemorrhage.
Article: Orbital Arteriovenous Malformations[Show abstract] [Hide abstract] ABSTRACT: To present the clinical features, management, and outcomes in a series of patients with orbital arteriovenous malformations (AVMs). Clinical records of patients with orbital AVMs confirmed using angiography were reviewed as a retrospective, noncomparative, interventional case series. Eight patients (3 women and 5 men) with unilateral AVMs and a mean age of 39 years (median, 36.5 years; range, 26-70 years) were reviewed. Findings existed for an average of 11.2 years before diagnosis and included periocular mass (7 patients, 88%); periocular edema, pulsation/bruit, proptosis, episcleral congestion, and previous trauma (4 patients each, 50%); elevated intraocular pressure (3 patients, 38%); pain and reduced visual acuity (2 patients each, 25%); and restriction of extraocular movements, and diplopia (1 patient each, 12%). All of the patients except 1 underwent surgical resection, with 3 (38%) receiving preoperative embolization of feeder vessels; all of the patients had initial resolution of manifestations after treatment. Angiography is essential for diagnosis and for planning the management of orbital AVMs. Treatment depends on patient-specific features and includes observation, embolization, and surgical excision or combined preoperative embolization/excision. Given their vascular nature, the main cause of poor management outcomes is perioperative hemorrhage. Outcomes after a multidisciplinary approach are good, with few recurrences reported at follow-up.
- [Show abstract] [Hide abstract] ABSTRACT: Cerebrospinal fluid leakage is an uncommon but significant complication of orbital and rarely lacrimal surgery which may have serious consequences including death. In a retrospective review of four orbital units, we report an incidence of cerebrospinal fluid leak (diagnosed intraoperatively) during exenteration, orbital decompression, and dacryocystorhinostomy of 1/154 (0.6%), 4/397 (1%), and 0/3,504 (0%), respectively. We found two additional cases of cerebrospinal fluid leaks associated with excision of orbital masses involving the orbital roof. In the literature, the incidence of cerebrospinal fluid leaks associated with orbital exenterations and decompressions was 1.6-16.7% and 0-10%, respectively. Cerebrospinal fluid leaks occur very rarely in dacryocystorhinostomies with only a few case reports found in the literature. Preventative measures, diagnosis, and management of this complication are discussed. Knowledge of anatomy and thorough preoperative assessment may predict areas at high risk for encountering cerebrospinal fluid leaks. Proper surgical technique further minimizes the risk for this complication. If a cerebrospinal fluid leak occurs, however, prompt diagnosis and management usually results in uncomplicated recovery.
- [Show abstract] [Hide abstract] ABSTRACT: Velocardiofacial syndrome (VCFS, or Shprintzen syndrome) is the most common syndrome associated with palatal anomalies and is characterized by the following major features: cleft palate, cardiac anomalies, typical facies, and learning disabilities. Ophthalmologic abnormalities are seen in 70% of cases and include posterior embryotoxon, bilateral cataracts, tortuous retinal vessels, and small optic disks. Congenital absence of the nasolacrimal duct is a rare condition with only a few cases reported, most of which describe complete bony occlusion at the distal end of the lacrimal sac. We report a patient with VCFS with congenital absence of the membranous nasolacrimal duct, a finding confirmed at endoscopic dacryocystorhinostomy. The patient was successfully treated with dacryocystorhinostomy and insertion of Jones tubes. To our knowledge, this is the first report documenting absence of the membranous nasolacrimal duct and also the first time this finding has been described in association with VCFS.
- [Show abstract] [Hide abstract] ABSTRACT: To report a series of patients who developed late complications secondary to silicone implants used in orbital fracture repairs and review the literature. Retrospective interventional case series. Analysis of records of patients who developed complications following repair of orbital wall fractures with silicone implants. Over a 5-year period, 4 patients were seen with complications arising from a silicone orbital implant. There were 3 males and 1 female whose ages ranged from 41-73 years. The time interval between initial insertion of implant and development of complications was 1.5, 6, 10, and 20 years. The complications noted were worsening diplopia, recurrent orbital cellulitis, lower lid retraction with restricted upgaze, and orbital abscess. Computed tomographic scans demonstrated the implant in all cases. Histologic examination revealed nonspecific chronic inflammation and fibrosis in all cases and foci of squamous epithelium in one case. Treatment included surgical removal of the implant, resulting in complete resolution or significant improvement in symptoms and signs in all cases. The use of silicone implants is associated with a wide range of complications, which may occur many years following the original surgery. Surgical removal of the implant usually leads to resolution of symptoms. Given the potential of silicone implants to cause delayed complications, their use in orbital fracture repair is not recommended.
- [Show abstract] [Hide abstract] ABSTRACT: To present findings of a pilot study on intraorbital corticosteroid therapy in the management of idiopathic orbital inflammation. This prospective, noncomparative, interventional case series included patients with clinically, radiologically, and histologically confirmed idiopathic orbital inflammation with an anterior orbital mass. Twenty to 40 mg/mL of triamcinolone acetonide was injected intraorbitally (intralesionally or perilesionally) in all patients. The injection was repeated at 4-week intervals if complete resolution was not achieved. Patients were assessed for local and systemic complications of corticosteroid injection. Visual acuity, fundus examination, intraocular pressure, blood pressure, and serum glucose levels were measured at each visit. Ten patients (5 men and 5 women; mean age, 49.8 years [age range, 25-82 years]) received treatment. In 4 patients, an orbital mass was noted; in 6 patients, the lacrimal gland was involved (dacryoadenitis). Substantial improvement (1 patient) or complete resolution (8 patients) was noted during a follow-up of 9.8 months (range, 3-24 months). Intraorbital injection of a corticosteroid is an effective treatment for idiopathic orbital inflammation and may be considered first-line treatment in selected patients.
Article: Orbital and Adnexal Sarcoidosis[Show abstract] [Hide abstract] ABSTRACT: To present the clinical features and management in a series of patients with orbital and adnexal sarcoidosis. This multicenter retrospective study included patients with biopsy-proven noncaseating granuloma involving the orbit or adnexa and evidence of systemic sarcoidosis. Clinical records were reviewed for initial examination findings, radiological findings, treatment modalities, and outcome. The study included 26 patients (19 female, 7 male; mean age, 52 years). The most common feature at the first examination was a palpable periocular mass followed by discomfort, proptosis, ptosis, dry eye, diplopia, and decreased vision. The disease affected the lacrimal gland (42.3%), orbit (38.5%), eyelid (11.5%), and lacrimal sac (7.7%). Among orbital lesions, the antero-inferior quadrant was preferentially involved. Treatment modalities included steroids, surgical debulking, and methotrexate. During a mean follow-up of 18.75 months, 84.6% of patients showed a complete response to the treatment, but 19.2% of patients developed further signs of sarcoidosis. Orbital soft tissue involvement is more common in patients older than 50 years and in women. The anterior inferior quadrants of the orbits appear to be preferentially affected. Although a good response to treatment with oral steroids is seen, long-term follow-up is recommended because active systemic disease can develop months to years later.
- [Show abstract] [Hide abstract] ABSTRACT: Choroidal melanoma is a rare occurrence in pigmented races. Low clinical suspicion of choroidal melanoma may result in diagnostic delay. We present a rare case of choroidal melanoma with extrascleral orbital extension in an Australian Aboriginal man. We are unaware of previous reports of uveal melanoma in Aboriginal Australians and could find no reference to it in a computerized search utilizing Medline. © 2007 The Authors; Journal compilation © 2007 Royal Australian and New Zealand College of Ophthalmologists.
Royal Adelaide HospitalTarndarnya, South Australia, Australia
South Australian Institute of OphthalmologyTarndarnya, South Australia, Australia