Orbital Invasion Research Articles

Management of Frontoethmoidal Mucoceles With Orbital Extension: Is Primary Orbital Reconstruction Necessary?

Sinus mucoceles with orbital extension are often managed primarily by an otolaryngologist to relieve the sinus obstruction. In this study, a series of patients was examined to determine whether or not reconstruction of the orbit was necessary at the time of the initial sinus surgery from the perspective of an orbital surgeon often involved in the comanagement of orbital complications of sinus mucoceles. A retrospective interventional case series of 5 patients with frontoethmoidal mucoceles comanaged by otolaryngology and oculoplastics. We reviewed symptoms, clinical findings, course, and treatment of our patients and reviewed the literature regarding management options for frontoethmoidal mucoceles. In extended follow-up, clinical resolution of presenting orbital symptoms and radiographic evidence of reformation of the bony orbit was noted without the need for orbital reconstruction at the time of the initial sinus surgery for a sinus mucocele with orbital extension. A specific subset of sinus mucoceles with orbital extension may resolve by primary sinus surgery alone. A decision on the relative merits of orbital surgery at the time of primary sinus surgery should be made on a case-by-case basis.

Orbital Syndromes

The orbit is a complex anatomical structure with unique properties not observed in other regions of the body. Composed of seven bones, the orbit is filled by the eye, optic nerve, lacrimal gland, extraocular muscles, peripheral motor and sensory nerves, fat, arteries, and veins. All these structures are intimately related to one another within an intricate framework of connective tissue. A variety of traumatic, vascular, inflammatory, infectious, and neoplastic processes can affect the orbit and its structures. Aside from the many primary orbital diseases, systemic disorders and pathological processes from neighboring structures (eye, ocular adnexa, oral cavity, paranasal sinuses, and intracranial cavity) can involve the orbit. A careful history and complete physical examination, with special attention to the orbit and ocular adnexa, are required to identify subtle orbital abnormalities that otherwise could be overlooked or mistakenly contributed to a nonorbital process. This article reviews the pertinent orbital anatomy, discusses the clinical evaluation and manifestations of orbital syndromes, and highlights several important orbital syndromes germane to the neurologist, including thyroid-associated orbitopathy, nonspecific orbital inflammation (also known as inflammatory orbital pseudotumor), perineural orbital invasion of cutaneous squamous cell carcinoma, rhino-orbital-cerebral mucormycosis, and carotid-cavernous sinus fistula.

Oncocytic carcinoma of the nasal cavity with widespread lymph node metastases

The first case of oncocytic carcinoma which arose from the inferior turbinate of the nasal cavity with orbital invasion through the nasolacrimal canal and widespread lymph node metastases in the neck and face is reported here. The tumor metastasized rapidly, spread widely to the whole neck and face, and produced tumor emboli in the lymphatics and extranodal extension by rupture of the lymph node capsule in the absence of clinically palpable regional lymph node enlargement. We think the oncocytic carcinoma of the nasal cavity may be a high-grade malignancy tumor.

Selective Removal of Sebaceous Gland Carcinoma of the Lower Eyelid With Orbital Infiltration

A 74-year-old woman presented with a nodule on the right lower eyelid. An incisional biopsy was significant for sebaceous gland carcinoma, whereas magnetic resonance imaging revealed infiltration of the ipsilateral orbit, including the medial and lower rectus muscles. The lesion was selectively excised, including the infiltrated part of the lower lid and the intraorbital part of the tumor. Two years postoperatively, no recurrence or metastasis have been noted. Preoperative orbital imaging is mandatory in cases of sebaceous gland carcinoma because it can reveal clinically silent orbital invasion. In such cases, selective surgical excision may be used, avoiding orbital exenteration.

Conjunctival Melanoma 3 Years After Radiation and Chemotherapy for Retinoblastoma

An 8-month-old infant with advanced bilateral retinoblastoma was managed with enucleation of the right eye and chemotherapy and radiotherapy of the left eye. Three years after treatment, an ill-defined, nonpigmented mass was noted in the anophthalmic right socket. Excisional biopsy revealed an amelanotic melanoma of the conjunctiva with superficial orbital invasion. There has been no tumor recurrence during 20 months of follow-up.

Transcleral Orbital Invasion by a Radiation and Chemotherapy-Resistant Choroidal Metastasis of a Pulmonary Adenocarcinoma

To report transcleral orbital invasion by a radiation- and chemotherapy-resistant choroidal metastasis of a pulmonary adenocarcinoma. Interventional case report. A 51-year-old female was found to have choroidal metastasis from a previously unknown primary pulmonary adenocarcinoma. She underwent external beam radiation treatment with a total dose of 40 Gray (Gy), delivered in 20 fractions over 28 days, followed by adjuvant chemotherapy with gemcitabine and carboplatin. After completing two of the three scheduled chemotherapy cycles, the choroidal lesion was observed to have increased in size. A transvitreal aspiration biopsy of the choroidal lesion confirmed the diagnosis of metastatic pulmonary adenocarcinoma. Enucleation was performed for intractable pain. Histopathology revealed invasion through scleral emissary channels to the retrobulbar tissue and through the optic nerve substance itself. Despite appropriate external beam radiation therapy (EBRT) and chemotherapy, pulmonary adenocarcinoma metastases to the choroid can continue to grow and may invade the orbit transclerally.

Orbital exenteration for invasive skin tumours

Orbital exenteration aims at local control of disease invading the orbit that is potentially fatal or relentlessly progressive. Of all exenterations presenting to ophthalmologists, 40-50% are required for tumours in the eyelid or periocular skin. 99% of these are basal cell carcinomas and 4-6% each are squamous cell carcinomas or sebaceous gland carcinomas. Orbital invasion results in progressive fixation of the tumour to bone and reduced ocular motility. Perineural invasion of branches of the trigeminal nerve leads to numbness or pain, and that the facial nerve, to weakness. Biopsy identifies the cell type and the presence of perineural invasion. CT and MRI scanning help in the assessment of tumour spread within the orbit. Management should be in collaboration with an oncologist. Exenteration may be total-the removal of all orbital contents-or lid-sparing if the tumour is placed posteriorly. The socket may be allowed to heal by granulation or lined with a split skin graft or local flap. Complications may be seen following 20-25% of exenterations and include fistulae, tissue necrosis, exposed bone, and infection. Incomplete clearance of tumours occurs in about 38% of total exenterations and 17% of subtotal. The overall 5-year survival is 55-65%, but significantly worse if there was perineural spread. Facial prostheses may be mounted on glasses or secured with tissue glue or osseointegrated implants. Excellent cosmetic results can be achieved but many patients prefer to wear a patch.

Management of malignant and benign eyelid lesions

The management of benign and malignant eyelid neoplasms has been extensively examined. This paper offers an overview of the most common eyelid lesions and their management based on a review of the relevant bibliography. Recent studies have focused on the results of nonsurgical approaches for benign and premalignant lesions that are routinely surgically excised. In the malignant group, a consensus has been reached over the preferred method of tumor excision for basal cell carcinoma: Mohs surgery or complete surgical excision with frozen-section control of the margins offers the lowest tumor-recurrence rate. Important acquisitions have been made on squamous cells for which sentinel node biopsy may reveal early metastatic cancer. Intraepithelial tumor growth is a peculiar feature of sebaceous gland carcinoma that seems to indicate an increased risk for orbital invasion. Recent reports regarding the rare tumor, Merkel cell carcinoma, recommend a wide surgical excision with 5 mm margins; this may reduce the incidence of lymph-node metastasis, haematogenous spread and local recurrences. Although treatment of the most common benign and malignant tumours affecting the eyelids has not radically changed over the years, recent reports have significantly improved the standard of care for affected patients.

Surgical approaches used in the reconstruction of the eyelids after excision of malignant tumors

The choice of reconstructive technique for eyelid defects after malignant tumors was based on tumor location and defect size after tumor excision. Treatments included direct closure for defects measuring less than 25%, Tenzel flap for defects measuring 25-50%, lid-sharing procedures for defects measuring more than 50%, skin grafts for tumors affecting the inner canthus advancement flap for outer canthus tumors, and orbital exenteration for malignant tumors demonstrating orbital invasion.

Fibrous Histiocytoma of the Conjunctiva

To review the clinical features and course of six patients with fibrous histiocytoma (FH) of the conjunctiva. Retrospective, observational clinical case series. Chart review of six consecutive patients with unilateral cases of conjunctival FH was conducted. Clinical presentation, treatment, histopathologic condition, and follow-up information were recorded. The mean patient age was 37 years (median, 38 years; range, 12 to 72 years). There were five white patients, one black patient, five male patients, and one female patient. The tumor was present for a mean of three months (median, five months; range, one to 12 months) and was unilateral (one right eye, five left eyes). In all cases, the tumor was a tan, dome-shaped limbal mass in the conjunctival stroma with visible intrinsic vessels. The mean tumor basal dimension was 7 mm (median, 5 mm; range, 4 to 11 mm). Surgical resection was performed in all cases, and histopathologic study demonstrated benign FH in four cases and malignant FH in two cases. Those cases with benign FH showed no recurrence over nine months (median, eight months; range, three to 18 months). One patient with malignant FH showed recurrence and required repeat excision. The other patient with malignant FH was treated with plaque radiotherapy to maintain control. There was no evidence of orbital invasion or remote metastasis in any case over the mean follow-up period of 21 months (median, 10 months; range, three to 80 months). FH is a rare conjunctival tumor that can show benign or malignant features. Complete resection is advised. Malignant FH can demonstrate recurrence that necessitates wide resection and radiotherapy.

Fronto-ethmoid sinus mucocele: A case report

Background Mucoceles are epithelium-lined cavities in the paranasal sinuses filled with mucus. They develop because of scarring and obstruction of the sinus ostium, whether from chronic sinusitis, trauma, or surgery. They commonly erode the bony sinus wall and can have serious complications of brain and orbital invasion, with potential for abscess and rupture. Case report A 39-year-old woman had diplopia in left gaze and a hard nodule above the right eye for 1 year. She recently noted a bulging right eye, nasal congestion, and occasional headaches. She was 9 years post–sinus surgery. All aspects of the afferent optic nerve function were intact. Right-sided proptosis was evident as was a right adduction and supraduction deficit, prompting immediate imaging, both with a computed tomography scan and magnetic resonance imaging of the orbits. These results showed a large right ethmoid sinus mucocele, with bony erosion and orbital invasion, prompting an immediate surgical referral. Conclusion Mucoceles may arise from any of the paranasal sinuses and, because of the close proximity of these spaces to the orbit, may initially manifest with visual and ocular signs and symptoms. Therefore, eye care providers need to be aware of this entity and the need for immediate referral or workup if a mucocele is suspected.

Periorbita: Computed Tomography and Magnetic Resonance Imaging Findings

The periorbita has been regarded as the critical structure in decision of exenteration in the patients with paranasal (PNS) malignancies. The purpose of this study is to present the CT and MRI findings of the periorbita with PNS malignancies. Ten patients with PNS cancers were chosen for this study. PNS CT and MRI scans were performed on all patients. The imaging findings were reviewed retrospectively by consensus of two neuroradiologists. Assessment of the defect of the orbital bone and invasion of the orbital fat was made by CT and MRI. The signal intensity (SI), thickness, enhancement, and correlation with the orbital bone of the periorbita were analyzed on MRI. The intraoperative and pathologic reports were used as the gold standard for orbital invasion. In patients with bone defects, it was impossible to differentiate the periorbita from the mass on CT. The periorbita showed hypointensity on T2-weighted images compared with the SI of mass. On Gd-enhanced T1-weighted images, the periorbita showed less enhancement than the mass in nine patients. Six patients showed two hypointense layers (the outer bony wall and the inner periorbita) between the mass and orbital fat on T2-weighted images. The thickness of the periorbita was 0.79-1.4 millimeter. Orbital invasion was more conspicuous on T2-weighted coronal images than on the CT images. MRI was more conspicuous for detecting orbital invasion than CT. The mass beyond the thickened periorbita on T2-weighted images was considered to be a positive finding of orbital invasion.

Periorbital Zygomycosis (Mucormycosis) Treated With Posaconazole

To report on the successful treatment of periorbital zygomycosis (mucormycosis) with posaconazole, a broad-spectrum oral antifungal available for compassionate use. Interventional case report. Review of a medical record. A 22-year-old male undergoing induction chemotherapy for acute lymphoblastic leukemia presented with periorbital cellulitis attributable to Rhizopus. The patient was initially treated with liposomal amphotericin B, surgical debridement, and reversal of immune compromise. The patient was switched to posaconazole because of amphotericin side effects and lack of improvement. He took posaconazole for five months while undergoing additional cycles of chemotherapy. Despite recurrent profound neutropenia, the periorbital infection resolved, he tolerated reconstructive procedures, and he did not develop orbital invasion. His Rhizopus isolate was highly susceptible to posaconazole in vitro. Drug toxicities can limit the use of amphotericin in some patients with zygomycosis. Posaconazole shows promise as an alternative antifungal agent in the treatment of periorbital zygomycosis.

Orbital Invasion by Recurrent Maxillary Ameloblastoma

To describe 2 patients with orbital invasion by maxillary ameloblastoma, a rare odontogenic tumor that is not commonly encountered in ophthalmic practice. Retrospective, interventional case report. Two patients who were diagnosed with maxillary ameloblastoma several years ago sought treatment for new-onset ocular and orbital signs and symptoms. Clinical and radiological findings and outcome. In the first patient, tumor recurrence with orbital invasion was diagnosed, and the patient underwent a total orbital exenteration. No recurrence was noted after 18 months of follow-up. The second patient had intracranial involvement with orbital invasion and underwent an extensive resection through an intracranial approach. No recurrence was noted after a 6-month follow-up period. Although a slow-growing tumor, maxillary ameloblastoma can recur after surgical excision and can be locally aggressive; it can invade the orbit and result in significant ocular morbidity. Ophthalmologists should be aware of this tumor and should monitor these patients closely when orbital invasion is suspected.

Basal Cell Carcinoma Associated with Orbital Invasion: Clinical Features and Treatment Options

Basal Cell Carcinoma Associated with Orbital Invasion: Clinical Features and Treatment Options

Basal Cell Carcinoma Associated with Orbital Invasion

Basal Cell Carcinoma Associated with Orbital Invasion

Intraocular malignant teratoid medulloepithelioma in an adult: clinicopathological case report and review of the literature

To report a case of intraocular medulloepithelioma, an embryonal tumour with extremely rare presentation in adults. The case of a 44-year-old man with intraocular malignant teratoid medulloepithelioma, primarily diagnosed as intraocular teratoma, is described and the literature on this subject is reviewed. The patient presented with progressive proptosis caused by a tumour in the left eyeball. He had a 28-year history of loss of vision in the left eye. Histopathological examination of the enucleated eye demonstrated an intraocular teratoma. No adjuvant treatment was given. Six months later the patient presented with massive progression in the left orbit and intracranial invasion. Cisplatin-based chemotherapy was administered, but discontinued after two cycles due to poor tolerance and lack of response. At subsequent pathology review, a final diagnosis of malignant teratoid medulloepithelioma was made. Salvage radiotherapy (60 Gy in 30 fractions) resulted in partial response of the intracranial lesion. However, the patient died 6 months later due to intracranial tumour progression. Medulloepithelioma should be considered in the differential diagnosis of intraocular tumours in adults, especially in the case of coexisting, long-standing ocular symptoms. In some cases this disease is very aggressive.

A Case of Alveolar Rhabdomyosarcoma of the Ethmoid Sinus Invading the Orbit in an Adult

PurposeA case study and literature review of alveolar rhabdomyosarcoma (RMS) in an adult.MethodsA 48-year-old male patient presented at our clinic complaining of proptosis that had persisted for 2 weeks in his left eye. A computed tomography (CT) scan revealed a destructive soft-tissue mass in the left ethmoid sinus with invasion of the left orbit and compression of the medial rectus muscle. Endoscopic intranasal biopsy revealed alveolar RMS. Conservative debulking and orbital wall decompression were performed.ResultsImmunohistochemical testing was positive for desmin, S-100, and smooth muscle actin (SMA), supporting the diagnosis of RMS. Since ipsilateral cervical and spinal metastasis was detected, systemic treatment was administered simultaneously.ConclusionsAlthough rarely found in adults, RMS should be considered in the differential diagnosis of orbital tumors. Immunohistochemical analysis plays an important role in the definitive diagnosis of RMS.

Regression of invasive conjunctival squamous carcinoma in an HIV-positive patient on antiretroviral therapy

Case history of an African woman presenting with advanced HIV and a painful conjunctival lesion is presented. A conjunctival biopsy revealed invasive squamous cell carcinoma, with orbital invasion on computed tomography scan. She was commenced on antiretroviral therapy. She refused surgery to remove the eye and orbital contents (exenteration), and was referred to palliative care. Gradually, her immune status and ocular symptoms improved. At ophthalmic review, the tumour had apparently completely regressed. This unprecedented phenomenon may be due to antiretroviral therapy. Discussion covers conjunctival carcinoma and behaviour of HIV-related tumours with antiretroviral therapy. Antiretroviral drugs may offer a better alternative to disfiguring surgery in the future.

Orbital Invasion by Esthesioneuroblastoma

Esthesioneuroblastoma is a rare malignancy arising from the olfactory mucosa in the nasal fossa, which can invade the orbit producing ophthalmic symptoms and signs. The purpose of this study was to present a case series and review the literature to assess the characteristics of orbital involvement by esthesioneuroblastoma. Retrospective case series of all patients treated for biopsy-proven esthesioneuroblastoma at the Royal Adelaide Hospital between 1992 and 2004. Nine patients (5 male, 4 female) with a mean age of 50 years (range, 20 to 66 years) were reviewed. One case was classified as Kadish stage A, 1 as Kadish stage B, and 7 as Kadish stage C. Mean time from onset of symptoms to diagnosis was 17 months (range, 2 to 24 months). Radiologic orbital invasion was present in 4 cases and was associated with ophthalmic symptoms or signs in 3 of these cases. These included proptosis (2 cases), periorbital pain (1 case), decreased visual acuity (1 case), extraocular muscle restriction (1 case), and chemosis (1 case). One patient with symptoms secondary to orbital invasion was initially referred to and assessed by an ophthalmologist. All patients who had or went on to have development of orbital invasion had advanced disease at diagnosis. Treatment was surgical (9/9), with the addition of radiotherapy (6/9) and chemotherapy (2/9). Mean follow-up was 3.6 years (range, 0.5 to 8.5 years). Six of 9 patients had tumor recurrence. Two had metastasis. Five of 9 patients remained alive. Orbital invasion by esthesioneuroblastoma is not uncommon. It is important to be aware of this malignancy because a significant proportion of patients will present with ophthalmic signs and symptoms.