Ameloblastic Carcinoma With Orbital Invasion.

Ameloblastic carcinomas are malignant tumors arising from the odontogenic epithelium and defined as having features of ameloblastic differentiation in addition to cytological features of malignancy. Orbital involvement is rare and generally involves invasion of the orbital floor, apex, or soft tissue. This report describes an advanced presentation of ameloblastic carcinoma with orbital invasion and provides a review of the literature. A 58-year-old male presented with a 2-year history of a mid-facial mass, causing intracranial invasion and distortion of most skull foramina, nasopharynx, nasal cavity, and both orbits. Notably, there was an en-plaque pattern of circumferential tracking of the tumor along both orbital walls without invasion beyond the extraconal space, causing compression of the orbital apex and proptosis. Histology demonstrated nests of ameloblastic carcinoma and the advanced tumor was deemed nonresectable, with treatment being palliative.

A Brief History of Lacrimal Bypass Techniques.

PURPOSE: Lacrimal bypass is the creation of a fistula connecting the conjunctiva with the lacrimal sac or nasal cavity. Bypass is indicated in canalicular obstruction or agenesis; sac absence, destruction or prior excision; lacrimal pump failure; or dacryocystorhinostomy failure. We aim to review the various techniques that have been developed over the last century for lacrimal bypass. METHODS: We conducted a comprehensive literature review of techniques which have focused on creating a conduit extending from the conjunctiva or canaliculi to the lacrimal sac, or extending that bypass to the nasal cavity bypass. RESULTS: The main techniques reviewed include canaliculodacryocystorhinostomy, conjunctivodacryocystostomy, conjunctivorhinostomy, conjunctivodacryocystorhinostomy, and conjunctivoductivodacryocystorhinostomy. CONCLUSION: Lacrimal bypass surgery has evolved due to innovation in microsurgical techniques, instruments and materials. Conjunctivodacryocystorhinostomy with Jones tube insertion is the predominant bypass technique, reflecting a culmination of historical developments. Understanding the variety of lacrimal bypass techniques is important for exploring alternative options when necessary.

Corneal Neurotization for Neurotrophic Keratopathy: A Multicentre Experience.

PURPOSE: Corneal neurotization is an emerging technique that offers potential for visual rehabilitation in neurotrophic keratopathy. This study reports on a multicenter experience and outcomes for both direct and indirect methods of corneal neurotization. METHODS: Retrospective case series. Sixteen patients with neurotrophic keratopathy who underwent corneal neurotization across 5 centers in Australia and Israel were identified for inclusion. Corneal neurotization was performed via direct neurotization from the ipsilateral or contralateral supraorbital/supratrochlear nerve or by the use of an interpositional sural nerve graft. Change in corneal sensitivity (measured in millimeters by the Cochet-Bonnet aesthesiometer), visual acuity, and corneal health. RESULTS: Over a mean follow-up period of 31.3 months (range: 3 months-8 years), mean corneal sensitivity improved from 3.6 mm (range: 0-25 mm) to 25.3 mm (range: 0-57 mm). Visual acuity improved on average from 20/380 to 20/260. Twelve of 16 patients (75.0%) improved in at least 2 out of the 3 main outcome measures. Nine patients (56.3%) showed an improvement in visual acuity; 13 (81.3%) showed an improvement in average corneal sensitivity; and 11 (68.8%) showed an improvement in corneal health. There were no intraoperative or postoperative complications. CONCLUSIONS: Corneal neurotization is an emerging surgical treatment option for the management of neurotrophic keratopathy. With appropriate case selection, outcomes are favorable and complication rates are low, for a condition that is otherwise challenging to manage. Patients with severe neurotrophic keratopathy should be considered for this surgical treatment option.

IgG4-related Orbital Disease Presenting as an Orbital Apex Lesion.

IgG4-related disease is an uncommon multisystem inflammatory disease characterized by tissue infiltration by IgG4 plasma cells, extensive fibrosis, and sclerosing inflammation. Diffuse orbital involvement extending to the orbital apex and cavernous sinus has been reported, but a solitary well-circumscribed lesion at the apex has not been previously reported. Herein, we report a unique case of IgG4-related orbital disease presenting as a solitary well-circumscribed orbital apex lesion causing subacute visual decline in a 45-year-old male. MRI demonstrated bilateral lacrimal gland and infraorbital nerve enlargement and an 18 × 7 mm left orbital apex lesion demonstrating homogeneous contrast enhancement and isointense signal on T2. He was initially treated with tapering systemic corticosteroid therapy with improvement in his vision; however, he experienced visual deterioration 3 months later with recurrent inflammation. He subsequently commenced high-dose systemic corticosteroid therapy and rituximab infusions with improvement of left visual acuity to 6/7.5 + 2.

Magnetic Resonance Imaging of Idiopathic Orbital Myositis.

PURPOSE: To characterize the qualitative and quantitative features of idiopathic orbital myositis (IOM) on MRI. METHODS: This was a multicenter retrospective study of patients with active IOM with MRI. Patients with incomplete clinical records, poor-quality or interval scans without active myositis, and specific orbital myositis (i.e., orbital myositis secondary to an identified condition) were excluded. An enlargement ratio was calculated by dividing the diameters of the affected extraocular muscle (EOM) by the contralateral unaffected EOM. RESULTS: Twenty-four patients (mean age: 44.4 ± 17.8 years-old, male: 11) between 2011 and 2022 were included. One case (4.2%) was pediatric (17 years old), and 6 cases presented with recurrence. Active IOM was characterized by fusiform EOM enlargement, high T2 signal, and contrast enhancement. Average maximal EOM diameters ranged from 4.6 to 7.7 mm (enlargement ratio: 1.4-2.2). Eighteen (75%) patients had single EOM involvement, most commonly the medial rectus. Other ipsilateral structures affected included focal orbital fat (16/24, 66.7%) and lacrimal gland (8/24, 33.3%). Contralateral changes in the EOM and/or lacrimal gland were observed in 7 patients (29.2%). Patients presenting with recurrence were likely to develop ongoing recurrent episodes (p = 0.003). CONCLUSIONS: Various radiological patterns of involvement described including EOM enlargement, contrast enhancement, abnormal signal, and involvement of other orbital structures are indicative of active IOM. IOM remains a heterogeneous spectrum of acute and chronic clinico-radiological presentations. Inflammation may involve other ipsilateral or contralateral orbital structures or may be bilateral despite presenting clinically as unilateral disease. Quantitative measurements may have utility in differentiating IOM from other causes of orbital myositis.

Recurrent inferior oblique myositis and its outcomes.

This report presents a unique case of recurrent idiopathic inferior oblique myositis (IOM) with a focus on clinico-radiological characteristics and histological features. A woman in her early 40s presented with a third episode of IOM following a 12-year period of quiescence. The first two episodes were characterised by unilateral IOM with rapid resolution following oral prednisone treatment. MRI revealed anterior focal enlargement of the left inferior oblique muscle with ipsilateral lacrimal gland enlargement. An inferior oblique muscle and lacrimal gland biopsy demonstrated significant inflammatory infiltrate. An intraorbital injection of triamcinolone acetonide was administered with complete resolution of symptoms within 1 week.

Inferior orbital fissure release to access the inferolateral orbital apex.

OBJECTIVE: To describe release of the inferior orbital fissure (IOF) as a novel surgical technique that will improve access to the inferior and inferolateral orbital apex. DESIGN: Laboratory investigation. PARTICIPANTS: Human cadaver heads. METHODS: Cadaveric dissection study whereby the technique of an IOF release was performed. A swinging eyelid preseptal approach was used to enable a subperiosteal dissection to the orbital floor without obstruction from orbital fat prolapse. A plane was dissected between periorbita and Muller's muscle, the smooth muscle that overlies the entire length of the IOF. Measurements were taken in the sagittal plane from a point on the inferior orbital rim directly above the infraorbital foramen to the most posterior aspect of the orbital floor. RESULTS: Fifteen orbits from 8 cadaver heads were dissected with the aid of a rigid 0-degree endoscope and microsurgical instruments. This study demonstrated that an additional 10.93 ± 2.10 mm of access to the orbital apex was gained after the release of periorbita from the IOF. CONCLUSION: We propose that the IOF release can be adopted as an adjunctive technique to orbital surgery of the inferior and inferolateral orbital apex.

Sinonasal Chondrosarcoma With Unusual Orbital Invasion.

A case is presented of a 43-year-old male with a chronic history of progressive nasal obstruction and epiphora. MRI confirmed a heterogeneous mass involving the middle and superior turbinates with T2 hyperintense and calcified components, with extension into the inferomedial orbit. Tissue biopsy revealed a grade 2 chondrosarcoma of the conventional subtype. Endonasal wide local resection of the lesion was performed with clear margins. The patient had no functional sequelae and will undergo routine surveillance.

Transorbital Endoscopic Approach to the Foramen Rotundum for Infraorbital Nerve Stripping.

PURPOSE: To develop and evaluate a transorbital endoscopic approach to the foramen rotundum to excise the maxillary nerve and infraorbital nerve branch. METHODS: Cadaveric dissection study of 10 cadaver heads (20 orbits). This technique is predicated upon 1) an inferior orbital fissure release to facilitate access to the orbital apex and 2) the removal of the posterior maxillary wall to enter the pterygopalatine fossa (PPF). Angulations along the infraorbital nerve were quantified as follows: the first angulation was measured between the orbitomaxillary segment within the orbital floor and the pterygopalatine segment suspended within the PPF, while the second angulation was taken between the pterygopalatine segment and maxillary nerve as it exited the foramen rotundum. With refinement of the technique, the minimum amount of posterior maxillary wall removal was quantified in the final 5 cadaver heads (10 orbits). RESULTS: The mean distance from the inferior orbital rim to the foramen rotundum was 45.55 ± 3.24 mm. The first angulation of the infraorbital nerve was 133.10 ± 16.28 degrees, and the second angulation was 124.95 ± 18.01 degrees. The minimum posterior maxillary wall removal to reach the PPF was 11.10 ± 2.56 mm (vertical) and 11.10 ± 2.08 mm (horizontal). CONCLUSIONS: The transorbital endoscopic approach to an en bloc resection of the infraorbital nerve branch up to its maxillary nerve origin provides a pathway to the PPF. This is relevant for nerve stripping in the context of perineural spread. Other applications include access to the superior portion of the PPF in selective biopsy cases or in concurrent orbital pathology.

Idiopathic Granulomatous Orbital Inflammation with Right Frontal Nerve Involvement: A Case Report.

Introduction: Idiopathic granulomatous orbital inflammation is a rare subtype of orbital inflammatory disease, and frontal nerve involvement has not been previously described. Case Presentation: This is a case of a 75-year-old female who presented with a 4-week history of a right orbital mass, scalp dysaesthesia, and ptosis. Magnetic resonance imaging of the orbits confirmed a right supraorbital mass originating from the lacrimal gland with frontal nerve enlargement. Biopsy showed non-caseating granulomatous inflammation. Conclusion: The patient was treated with an intralesional triamcinolone injection and oral prednisolone and will continue to be monitored for development of systemic sarcoidosis.

Cutaneous squamous cell carcinoma radiographically mimicking infiltration into the lacrimal gland.

Cutaneous squamous cell carcinoma (SCC) is a common malignancy of the skin, with the potential for local invasion and metastasis. Here, we present a case series of two patients with SCCs, suggesting radiological infiltration of the lacrimal gland on magnetic resonance imaging. However, histopathological examination revealed lymphoplasmacytic infiltration of the lacrimal gland consistent with dacryoadenitis, with no evidence of SCC infiltration. Our cases highlight the potential for peritumoural inflammation to cause dacryoadenitis and radiologically mimic tumour infiltration into the lacrimal gland.

Varicella zoster virus meningoencephalitis presenting as orbital myositis - a case report.

The authors present a case of meningoencephalitis caused by Varicella zoster virus (VZV) infection, which initially manifested as orbital myositis followed by rapid progression to orbital apex syndrome, meningoencephalitis and death. There was no development of a cutaneous rash. An orbital biopsy demonstrated VZV infection, which was confirmed on a lumbar puncture. In this case, VZV meningoencephalitis was not responsive to steroid or antiviral therapy. This case highlights an atypical presentation of VZV with orbital myositis preceding intracranial involvement.

Recommendations for risk stratification of periocular squamous cell carcinoma.

Periocular squamous cell carcinoma is a common cutaneous malignancy with generally favorable outcomes; however, the periocular region is intrinsically a high-risk location, and there exist a subset of lesions with a propensity for poor outcomes. Orbital invasion, intracranial perineural spread, nodal and distant metastasis are feared complications. There are several staging systems for eyelid carcinoma and cutaneous squamous cell carcinoma, but the definition of high-risk lesions remains heterogeneous. It is unclear exactly which lesions can be safely deescalated, and which require nodal evaluation and adjuvant multimodal therapy. We seek to answer these questions by summarizing the literature on clinicopathologic variables, molecular markers, and gene profiling tests in periocular squamous cell carcinoma, with the extrapolation of data from the cutaneous squamous cell carcinoma literature. Standardized pathology reports with information on tumor dimensions, histological subtype and grade, perineural invasion, and lymphovascular invasion should become uniform. Integration with gene expression profiling assessments will individualize and improve the predictive accuracy of risk stratification tools to ultimately inform multidisciplinary decision-making.

Disseminated thrombosis of the internal jugular vein, superior ophthalmic vein and cavernous sinus as the primary manifestation of occult malignancy: a case report.

Internal jugular vein (IJV) thrombosis is a life-threatening condition most often associated with local risk factors such as head or neck infection or central venous catheterisation. Underlying malignancy is a rare but important aetiology to consider in patients presenting with spontaneous IJV thrombosis. We describe a case of necrotic cervical lymphadenopathy with thrombosis of the IJVs, cavernous sinuses and superior ophthalmic veins in a patient with metastatic squamous cell carcinoma, which was further complicated by an orbital compartment syndrome. The differential diagnosis of IJV thrombosis includes a range of infective, metastatic and thrombophilic pathologies. This case illustrates that, in the absence of an underlying precipitating factor, spontaneous IJV thrombosis should prompt further systemic investigations. Furthermore, patients with thrombotic events affecting the orbital venous drainage system should be monitored closely for signs of an acute orbital compartment syndrome.

Recurrence Following Globe Sparing Excision for Basal Cell Carcinoma with Anterior Orbital Invasion.

PURPOSE: Globe-sparing excision for periocular basal cell carcinoma (BCC) with orbital invasion has evident benefits, but the ensuing morbidity and characteristics of recurrence are not well elucidated. This study aims to describe the extent of visual morbidity following globe-sparing excision, and the clinicoradiological characteristics of tumor recurrence. METHODS: Multicentre retrospective case series. RESULTS: Eight patients were identified for inclusion in this series. Time to recurrence following globe-sparing excision ranged from 3 to 12 years. Seven patients (87.5%) presented with recurrent disease originating from the medial canthus. Clinical features at presentation included contracture ( n =4, 50.0%), upper lid ptosis ( n =3, 37.5%), a palpable mass ( n =2, 25.0%), and hypoesthesia ( n =2, 25.0%). Radiologically, tumor recurrence was predominantly characterized by isointense signals on T1 and T2-weighted sequences ( n =5, 62.5%) with moderate contrast enhancement. The most common histologic subtype in recurrent tumors was a mixed nodular and infiltrative growth pattern ( n =5, 62.5%). Perineural invasion was a feature in four (50%) cases. Salvage therapy in the form of exenteration was performed in seven cases. Vismodegib and adjuvant radiotherapy were provided for one case with surgically unresectable tumor recurrence. CONCLUSIONS: Globe-sparing excision for invasive periocular BCC can be complicated by late recurrence that develops rapidly despite silent neuroimaging for years. Early clinical signs are subtle. High-risk features predictive of recurrence include medial canthus location, mixed histological subtypes, and perineural invasion. Patients with such characteristics require lifelong clinical and imaging surveillance following globe-sparing excision.

Transorbital drillout to the cavernous sinus: an approach for squamous cell carcinoma with perineural spread. Illustrative cases.

BACKGROUND: This study describes a transorbital apical approach to the cavernous sinus, where the greater wing of sphenoid (GWS) and superior orbital fissure (SOF) are drilled out to access the interdural incision zone and lateral wall of the cavernous sinus. OBSERVATIONS: This was a retrospective series of 3 patients with periocular squamous cell carcinoma (SCC) and radiological evidence of perineural spread to the cavernous sinus. Following an orbital exenteration, the GWS was drilled to reach the lateral border of the SOF. The meningo-orbital band, a periosteal transition between the frontotemporal basal dura and periorbita, was incised to enter the lateral wall of the cavernous sinus. The relevant cranial nerves were biopsied to provide an accurate zonal classification of disease. LESSONS: The transorbital apical approach via the SOF provides a corridor of access to the cranial nerves within the lateral wall of the cavernous sinus. This technique was successfully performed on 3 patients with periocular SCC. One case had radiological evidence of intracavernous oculomotor nerve involvement, 1 patient demonstrated nasociliary nerve enlargement at the SOF, and another had frontal nerve involvement extending into the cavernous sinus. Cerebrospinal fluid leak occurred in 1 case addressed with fat packing and fascial closure.