A case of concurrent silent sinus syndrome, thyroid eye disease, idiopathic orbital inflammatory syndrome, and dacryoadenitis.

This is a retrospective case description of a single female patient found to have concomitant bilateral silent sinus syndrome, bilateral thyroid eye disease, unilateral dacryoadenitis, and idiopathic orbital inflammation that presented as progressive unilateral right-sided proptosis. The spectrum of inflammatory orbital diseases can make discerning between different entities challenging, but more unique in this case was the simultaneous presentation with bilateral silent sinus syndrome, a rare entity in its own right. Identifying each of these concurrent disease processes is important to establishing a multidisciplinary treatment approach to address all the patient's orbital and peri-orbital pathology. We hope to highlight the clinical and radiographic findings unique to each of these entities and share our approach to treatment in this complex case.

IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients.

OBJECTIVES: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre. METHOD: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as 'definite' or 'possible' according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means of the IgG4-RD Responder Index (IgG4-RD RI). RESULTS: Forty-one patients (15 females, 26 males) were included in this study: 26 with 'definite' IgG4-RD and 15 with 'possible' IgG4-RD. The median age at diagnosis was 62 years. The median follow-up was 36 months (IQR 24-51). A history of atopy was present in 30% of patients. The pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Serum IgG4 levels were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids (GCs) to induce remission. IgG4-RD RI decreased from a median of 7.8 at baseline to 2.9 after 1 month of therapy. Relapse occurred in 19/41 patients (46%) and required additional immunosuppressive drugs to maintain long-term remission. Multiple flares occurred in a minority of patients. A single case of orbital pseudotumour did not respond to medical therapy and underwent surgical debulking. CONCLUSIONS: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.

Inflammatory myofibroblastic tumor of the orbit: a case report.

Inflammatory myofibroblastic tumor (IMT) is a neoplasm most commonly found in the abdominal-pelvic region, lung, and retroperitoneum. The tumor tends to affect soft tissues of children and young adults and can locally recur but rarely metastasizes. Histologically, the appearance is one of bland spindle cell proliferation with a prominent, chronic inflammatory infiltrate. This article describes 1 case of IMT found in the orbit that is presented with rapidly progressive painless proptosis. In the authors' review of the literature, they have only found 2 other case reports involving the orbit.

Lateral micro-orbitotomy as the technique of choice for orbital pseudotumor in IgG4-RD. Case report and review of the literature.

Immunoglobulin G4-related disease (IgG4-RD) is characterized by a systemic fibroinflammatory infiltrate that often involves the orbit in addition to other tissues. Thus it has to be considered in the differential diagnosis of orbital tumors. We report the clinical case of a 64-year-old woman who presented with right mydriasis, progressive proptosis and paralysis of the third cranial nerve of 1 year of evolution. Cranial MRI identified an intraconal lesion of the right orbit, located between the external and inferior rectus muscles and the optic nerve, and she was scheduled for surgery by transcranial approach with lateral micro-orbitomy. A satisfactory macroscopic excision was achieved with no remarkable complications and a definitive deferred histological result of pseudotumor by IgG4-RD. Follow-up for 24 months showed no tumor recurrence, and the patient clinically improved from ophthalmoplegia. This case highlights the efficacy of lateral orbitotomy in the etiologic diagnosis and successful therapeutic outcome of complex orbital lesions associated with IgG4-RD pseudotumor.

A clinicopathologic case study of two patients with pediatric orbital IgG4-related disease.

The purpose of this report is to describe the clinical, radiographica, and histopathologic findings in two pediatric patients with orbital IgG4-related disease.

Idiopathic sclerosing orbital inflammation--a case series study.

AIMS: It was the aim of this study to perform a review of the clinical features, treatments and outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) in Taiwan. METHODS: All patients with histologically confirmed ISOI from 1995 to 2007 at the National Taiwan University Hospital were included. We reviewed the medical charts to see the clinical presentation, treatment modalities, treatment efficacy and final clinical status of these patients. RESULTS: Six male and 3 female patients ranging in age from 33 to 82 years were included. The most common symptoms were pain and blurred vision (both 77.8%). Reduced visual acuity and proptosis were the most common signs (both 89%). Five patients received oral steroid treatment with/without pulse therapy combined with tissue removal, the response to which was good in 1 patient and partially effective in the other 4 patients. One patient received oral steroids and surgery, followed by radiotherapy, with only partial response. Patients with oral prednisolone or surgery only all had poor responses. CONCLUSION: ISOI is rare and also difficult to diagnose and manage. Early intervention with steroids/immunosuppressants combined with surgical debulking procedures may result in control or regression of this recalcitrant disease.

Granulomatous orbital inflammation associated with intraorbital titanium-impregnated acetate fiber.

Herein we report a 52-year-old man with subacute right-sided proptosis and diffuse intraconal enhancing abnormality on MRI. Orbital biopsy revealed granulomatous inflammation consistent with idiopathic orbital inflammatory syndrome (IOIS), or orbital pseudotumor. However, further examination under polarizing light microscopy also revealed acetate fiber fragments within the orbit. Prominent speckles within the acetate fibers were identified as titanium by Energy Dispersive X-ray Analysis (EDXA). Acetate impregnated with titanium (as a delustrant) is a common synthetic fiber used in textile and clothing manufacture. The mechanism for entrance into the orbit in this case is not known. Granulomatous idiopathic orbital inflammatory syndrome without local or systemic cause is an uncommon clinical entity, with less than 50 cases reported in the literature. Predominance of lacrimal gland (and thus superficial) involvement in granulomatous IOIS suggests the possibility of occult foreign body in such cases.

[Tumour-like lesions of the orbit - clinical symptoms, difficulties in differentiation and options for therapy]. / Klinische Symptomatik, differenzialdiagnostische Schwierigkeiten und Therapieoptionen bei tumorartigen Läsionen der Orbita.

Tumour-like lesions are very rare entity among orbital diseases. Between 1996 and 2007, 83 patients with orbital disorders were treated and three of them complained about different tumour-like lesions: giant cell granuloma, eosinophilic granuloma and fibrous dysplasia. Because of the considerable increase of functional disorders and tumour growth, all of the patients underwent surgical treatment. In this paper we describe the clinical symptoms, diagnostics and the methods of therapy for each of the lesions. The results shows that the preoperative neuro-ophthalmological examination as well as radiological imaging are necessary to assess the motility, visual field, vision, eye ball dislocation and to plan the surgical strategy. In terms of this descriptive character of the paper, we hope to submit some relevant information in order to improve the diagnostics and therapeutic procedures for tumour-like lesions of the orbit.

[Progressive axial exophthalmy--case report]. / Exoftalmie axiala progresiva--caz clinic.

INTRODUCTION: It is being presented the clinical case of a 67 year old male patient who has been under clinical observation and treatment for 10 years. The diagnosis rests upon clinical methods, imaging techniques (ocular-orbital examination, computerized tomography and magnetic resonance imaging) as well as post-operative histopathology reports. RESULTS: The clinical diagnosis was supported by the progressive decrease in visual acuity, painful, irreducible, non-pulsating axial progressive exophthalmia, associated with disorders of the ocular motility initially at the level of the RE, followed by bilateral involvement. The RE ocular-orbital ultrasound and the MRI examination point out a homogenous retro bulbar formation that caudally compresses and exceeds the optic-nerve. The mass lesion from the right orbit was afterwards partially removed by neurosurgical excision. The general treatment was initiated with steroidal and non-steroidal anti-inflammatory drugs in repetitive cures, under protection of antibiotics and anti-secretory drugs, the response to treatment being unfavorable. In time, the exophthalmia increased progressively, fact that imposed right de-compressive orbitotomy, with the surgical ablation of the lateral orbital wall. About 1 year after the neurosurgical intervention the initial clinical symptoms insidiously reinstalled throughout 3 - 4 years. The ultrasound, completed by the cranium CT and MRI examinations have identified the bilateral presence of myositis. DEBATES: Regardless of all the therapeutical means applied in time, not only the medical treatment (the systemic corticotherapy), but also the surgical one (with palliative effect), the patient's evolution was unfavorable; the axial exophthalmia persists, it is painful and irreducible. The evolution is aggravated also by the fact that both orbits have been affected; the specialty literature mentions cases with frequent unilateral involvement. CONCLUSIONS: In the case presented the axial exophthalmia is irreversible; it is determined by an inflammatory pseudotumor of both orbits, that represents a chronic inflammatory, idiopathic disease, with unpredictable clinical evolution. The diagnosis is usually one of exclusion, the complementary imaging examinations being necessary to rule out other pathologies of the orbit.

Sclerosing Orbital Inflammation Caused by Leishmania braziliensis.

Orbital biopsy of nonspecific orbital inflammation, commonly referred to as "orbital pseudotumor," typically shows a combination of polyclonal lymphocytes, plasmocytes, leukocytes, macrophages, and variable degrees of collagen deposition. Herein, we report a patient with a positive history of mucocutaneous leishmaniasis who presented with an orbital mass with a histological profile of idiopathic orbital inflammation. Immunohistochemical and molecular analysis of the orbital specimens demonstrated that the orbital inflammation was associated with the presence of antigens of Leishmania braziliensis and DNA from the parasite.

Endoscopic transnasal approach to medial orbital lesions.

OBJECTIVE: This case report presents our experience of endoscopic transnasal management of medial intra- and extraconal lesions. CASE REPORT: An endoscopic transnasal approach to intra-orbital lesions was used for nine patients. Four patients had intraconal lesions and five had lesions in the extraconal space. Post-operatively, seven patients reported an improvement in visual acuity and two reported stable vision. There were no complications of cerebrospinal leakage or diplopia. CONCLUSION: This case series demonstrated the safety and effectiveness of an endoscopic transnasal approach to managing intraconal and extraconal lesions. This minimally invasive technique should be considered a valid alternative for accessing orbital tumours, particularly those located in the medial compartment.

[Pseudo-tumoral aspergillus rhinosinusitis: About two cases]. / Aspergillose sinusienne invasive pseudo tumorale: à propos de deux cas.

INTRODUCTION: Pseudo-tumoral fongal rhinosinusitis is a rare and benign illness due to saprophyte germ, Aspergillus flavus. OBSERVATION: We reported two cases of invasive pseudo-tumoral fongal rhinosinusitis. CT scan helped with diagnosis and allowed for extension assessment. Complete surgical excision was done through external approach. Bacteriological examination evidenced the germ. DISCUSSION: Pseudo-tumoral invasive fongal rhinosinusitis remains a potentially serious pathology because of its local aggressiveness and its multiple extensions.

Fibrosing pseudotumor of the sella and parasellar area producing hypopituitarism and multiple cranial nerve palsies.

We present an unusual patient with a medical history of a fibrosing pseudotumor of the left orbit that had been stable for 8 years who presented with acute anterior hypophyseal failure. During the next 10-month period, sequential magnetic resonance scans showed a rapid growth of a plaque-like sellar and parasellar mass extending into the right cavernous sinus, right Meckel's cave, along the dural surfaces of the clivus, dens, and body of the second cervical vertebra. A transsphenoidal biopsy revealed sphenoid and intrasellar pseudotumor that invaded the adenohypophysis and had microscopic features identical to those of the previously excised orbital pseudotumor. Rapid growth of the pseudotumor continued despite a course of radiotherapy. Palsies of cranial nerves V and VI and of the sensory root of the cranial nerve VII developed on the right side. Steroid therapy was associated with improvement of the cranial nerve palsies. This is the first report of the sellar fibrosing pseudotumor producing not only anterior hypophyseal failure, but also cranial nerve dysfunction secondary to plaque-like extension into the cavernous sinus, Meckel's cave, and cranial base dura. This intracranial plaque-like extension of a fibrous pseudotumor corresponds to a hypertrophic intracranial pachymeningitis, which is a rare, previously described phenomenon associated to the syndrome of multifocal fibrosclerosis.

Conjunctival cul-de-sac reconstruction with radial forearm free flap in anophthalmic orbit syndrome.

Contracted eye socket is a constant cosmetic embarrassment to the patient. It not only renders patients unable to maintain an eye prosthesis, but it becomes a source of chronic discharge and irritation. Eye socket reconstruction with free skin, mucous membrane, cartilage, or dermis-fat usually remains unsatisfactory in many cases, due to secondary graft contracture. Traumatic injuries to the orbit and neighboring soft tissue frequently lead to a contracted eye socket. This condition results from the need for removal of the traumatized conjunctiva at the time of the enucleation, along with the traumatized eyeball, for satisfactory wound closure. In traumatic anophthalmos patients, a radial forearm free flap was used for conjunctival cul-de-sac reconstruction. Eye socket beds were developed as hinge-shaped flaps and used as lining for the upper and lower palpebrae. The authors conclude that the radial forearm flap is a useful alternative in the treatment of traumatic anophthalmos.

Risk factors associated with complications of orbital surgery in children.

PURPOSE: To assess the overall complication rate of orbitotomies performed in children and to determine the nature of complication to identify possible risk factors. METHODS: Thirty children <13 years underwent 33 orbitotomies for space-occupying lesions of the orbit or orbital decompression. A detailed evaluation of the surgical procedures and outcomes was conducted. Follow up averaged 2.5 years (range: 6 months to 5 years). RESULTS: The complication rate was 20% (2 of 10 cases) for anterior orbitotomies, 40% (8 of 20 cases) for primary lateral orbitotomies, and 100% (3 of 3 cases) for secondary orbitotomies. Dense amblyopia was noted in 6 of 9 patients. Major complications were frozen eyeball, diplopia, and ptosis. Less serious complications were enophthalmos, internal ophthalmoplegia, dry eye, and eyelid retraction. Six patients underwent extraocular surgery, levator muscle surgery, or both at a later date. CONCLUSION: Although the scope of complications is the same in children as in adults, the frequency of cystic benign lesions and small orbits in children can cause more frequent serious complications. Additionally, dense amblyopia of a complicated nature makes visual outcome unpredictable. The most significant risk factors identified were the need for a secondary procedure for recurring pathology and the presence of an intraconal lesion.

[Intraocular pseudotumor in an AIDS patient. Block excision, differential diagnosis, histology]. / Intraokularer Pseudotumor bei einem Aids-Patienten. Blockexzision, Differentialdiagnose, Histologie.

BACKGROUND: Intraocular pseudotumors are a rare event in Aids patients and often pose diagnostic problems. CASE REPORT: A 37-year-old patient who had had HIV seroconversion for 7 years was seen to developed progressively growing, multiple, disseminated, subretinal lesions OD > OS, accompanied by exudative retinal detachment and iritis. Since all etiological laboratory diagnostic efforts to detect an infectious, noninfectious and neoplastic systemic lesion failed, a diagnostic and curative therapeutic chorioretinal excisional biopsy specimen of the largest of the tumors (3 x 3 x 2 mm) was taken. The histological work-up demonstrated granulation tissue similar to an intraocular pseudotumor without signs of infection, malignancy or reactive lymphoid hyperplasia. This finding resulted in systemic corticosteroid treatment with complete resolution of the lesions in both eyes and no recurrences. CONCLUSIONS: An invasive diagnostic procedure in patients suffering from lesions of unknown cause resulting in the institution of an appropriate medical treatment may be beneficial for the integrity and vision of the respective eye.

[Surgical extraction of subretinal pseudotumors in age related macular degeneration. Clinical, morphologic and immunohistochemical results]. / Chirurgische Extraktion subretinaler Pseudotumoren bei altersbezogener Makuladegeneration (AMD). Klinische, morphologische und immunohistochemische Ergebnisse.

We extracted massive disciform subretinal lesions in eight eyes of seven patients with age-related macular degeneration via pars plana access. The lesions were 4-12 mm. All eyes had additional pathological findings such as PVR detachments, vitreous hemorrhage or acute submacular hemorrhage, sometimes in combination with each other. In seven eyes the preoperative visual function was no better than perception of hand movements, in 1 eye 1/35. The postoperative visual acuity was in 1 eye 0.3, in 6 eyes between 1/50 and 1/10 and without improvement in 1 eye. An intraocular silicone oil tamponade (highly purified, 5000 cs) was used in all cases. In two eyes the postoperative complication was central submacular fibrosis, and in two eyes with large retinotomies over more than two quadrants there were PVR reactions that needed reoperation in one eye. One patient was operated on bilaterally with final visual acuities of 1/35 and 1/10. The morphological structure of the extracted disciform lesions was characterized by a central fibrotic zone surrounded by attached hematomas, which were organized from the central tissue. In the central parts histology showed single layers of pigmented cells arranged along a basal membranelike PAS-positive band. Cell differentiation revealed macrophages, fibroblasts, myofibroblasts, T-lymphocytes, and vascular cells with immunohistochemical positive stainings for CD 68, cytokeratin, vimentin, alpha-actin, UCHL-1, and factor VIII. The lesions were supplied by a main choroidal vessel with branches into the periphery of the tissue. This feeder vessel can cause severe intraocular hemorrhage when it ruptures during surgical tissue extraction. Further immunohistochemistry showed the presence of cell adhesion molecules such as fibronectin, VLA-2, VLA-5, and VLA-6, the vitronectin receptor, 1CAM-1, LFA-1, and the PDGF receptor B. Their presence reflects mechanisms of growth regulation. The surgical extraction of massive submacular disciform lesions is part of the treatment of selected cases of end stages in age-related macular degeneration.

Magnetic resonance imaging of non-specific inflammatory granulation involving the skull base--two case reports.

A 55-year-old male presented with a 20-day history of nuchal pain and hypoglossal nerve paresis on the right. T2-weighted magnetic resonance (MR) imaging showed an isointense lesion with a small hypointense part in the hypoglossal canal and caudal clivus. A 66-year-old female presented with a long-standing history of left orbital pain. T2-weighted MR imaging showed a hypointense lesion occupying the orbital apex which on recurrence became isointense. The histological diagnosis of both lesions was non-specific inflammatory granulation. The T2-weighted MR imaging appearance of this entity is variable, and can change within the clinical course of the same lesion.