HMB-45 negative angiomyolipoma of the orbit: a case report and review of the literature.

BACKGROUND: Angiomyolipoma is a benign mesenchymal tumor composed of variable amounts of smooth muscle, adipose tissue and thick-walled blood vessels, and usually named PEComas (perivascular epithelioid cell tumors). PEComas share overlapping histopathological features with epithelioid cells along a perivascular distribution and characteristic immunohistochemistry with coexpression of myoid and melanocytic markers (HMB-45 /or Melan-A). We report the first case of primary orbital angiomyolipoma with negative melanocytic marker. CASE PRESENTATION: An 80-year-old Asian woman had a 2-year history of progressive swelling in the left upper eyelid. External examination revealed 3 cm of relative proptosis of the left eye and a palpable mass in the left superonasal orbit. Computed tomographic scan demonstrated a circumscribed, heterogeneous orbital mass. Excision biopsy was done and the histological finding demonstrated the orbital mass was composed of mature adipocytes, intermingled with spindle or oval-shaped cells, and accompanied by thick-walled blood vessels. Immunohistochemically, tumor cells were positive for CD34 and HHF-35, but negative for cytokeratin, HMB-45 and Melan-A. The diagnosis of angiomyolipoma was made. No recurrence was noted at 2-year follow-up. CONCLUSION: In our case, the HMB-45 negativity may be explained by the rarity of the epithelioid cells, and the HMB-45 positivity is often weaker or absent in spindle cells. Angiomyolipoma, although rare, should be added to the differential diagnosis of space-occupying orbital lesion.

The protective effect of antioxidants on orbital fibroblasts from patients with Graves' ophthalmopathy in response to oxidative stress.

PURPOSE: To investigate the biphasic effects of hydrogen peroxide (H2O2) on the orbital fibroblasts of patients with Graves' ophthalmopathy (GO) and the relation to antioxidants and proinflammatory cytokines. METHODS: Proliferation of cultured orbital fibroblasts from patients with GO and normal controls was evaluated in response to various concentrations of H2O2. The effect of low concentrations of H2O2 (6.25 µM) on the cellular proliferation and induction of intracellular proinflammatory cytokines, and reactive oxygen species of orbital fibroblasts were assessed. Protective effects of N-acetylcysteine and vitamin C on GO fibroblasts in response to 6.25 µM H2O2 stimulation were also investigated. RESULTS: When the GO fibroblasts were exposed to H2O2 at a concentration of 50 µM or above, significant cytotoxicity was observed. In contrast, lower concentrations of H2O2 (3.125-25 µM) increased the survival of GO fibroblasts with the peak cellular proliferation at 6.25 µM H2O2. However, this biphasic effect of H2O2 on the viability of orbital fibroblasts was not found in normal controls. In addition, 6.25 µM H2O2 led to significant elevation of the levels of transforming growth factor, beta 1, interleukin-1ß, and superoxide anion in GO fibroblasts, but no significant change in the normal controls. Pretreatment with N-acetylcysteine or vitamin C reversed the enhanced proliferation capacity and the induction of transforming growth factor, beta 1, interleukin-1ß and superoxide anion of GO fibroblasts in response to 6.25 µM H2O2. CONCLUSIONS: These findings revealed the biphasic effect of H2O2 on cellular proliferation of GO orbital fibroblasts. Importantly, a low level of H2O2 can stimulate proliferation of GO orbital fibroblasts and induce the production of proinflammatory cytokines, which can be inhibited by pretreatment with antioxidants. This provides a theoretical basis for the rational use of antioxidant in treating GO at an early stage.

Increased response to oxidative stress challenge in Graves' ophthalmopathy orbital fibroblasts.

PURPOSE: To investigate whether orbital fibroblasts from patients with Graves' ophthalmopathy (GO) are more responsive to oxidative stress. METHODS: Lipid peroxidation, oxidative DNA damage, reactive oxygen species (ROS) contents and activities of antioxidant enzymes were measured in cultured orbital fibroblasts from GO patients and age-matched normal controls in response to 200 µM hydrogen peroxide (H(2)O(2)). RESULTS: GO fibroblasts had increased basal levels of malondialdehyde (MDA), 8-hydroxy 2'-deoxyguanosine, superoxide anions, H(2)O(2), and manganese-dependent superoxide dismutase (Mn-SOD) activity, as well as decreased glutathione peroxidase (GPx) activity and the ratio between reduced (GSH) and oxidized glutathione (GSSG) compared with the orbital fibroblasts from normal subjects. After treatment of the cells with 200 µM H(2)O(2), the amplitude of increase in the intracellular levels of MDA (63% versus 26%), H(2)O(2) (24% versus 13%) and Mn-SOD activity (48% versus 23%) was exaggerated in GO fibroblasts compared with normal controls, respectively. In addition, treatment of GO fibroblasts with 200 µM H(2)O(2) led to a dramatic reduction of catalase activity (-59% versus -29%), GPx activity (-56% versus -13%), and GSH/GSSG ratio (-49% versus -21%), respectively. CONCLUSIONS: Elevated ROS and redox imbalance in GO orbital fibroblasts were exacerbated by H(2)O(2) as a result of exhaustion of GSH and compromise of antioxidant enzymes. Hypersensitivity to oxidative stress of GO orbital fibroblasts may play a role in the pathogenesis of GO.

Extra-major salivary gland pleomorphic adenoma of the head and neck: a 10-year experience and review of the literature.

Pleomorphic adenomas, or benign mixed tumors, make up 65% of all salivary gland tumors. They also can be found as solid tumors in other parts of the head and neck region, such as the auditory canal, the eyelids, and the orbital area. In this study, we investigated extra-major salivary gland pleomorphic adenomas of the head and neck region retrospectively at a tertiary care center. Between March 1998 and June 2009, 37 patients underwent primary surgery for extra-major salivary gland pleomorphic adenoma of the head and neck. The duration of symptoms, radiographic findings, operative procedures, and pathologic findings were documented. Of the 37 patients enrolled, 22 were male and 15 were female, with a median age of 57 years. Tumors were found in the soft palate, hard palate, nasopharynx, orbital area, trachea, buccal mucosa, cheek, nasal septum, upper lip, lower eyelid, and external auditory canal. Cellular variant of the pleomorphic adenoma was found in four patients, while the remaining patients presented with the classic variant. No myxoid subgroup was noted in our study. Carcinoma ex pleomorphic adenoma was observed only in one patient for whom radical surgery was performed. Twenty-eight patients (76%) had long-term follow-ups, with the average follow-up period being 4.5 years. Local recurrence was observed in three patients, and they underwent revision surgery during the follow-up period. Our results indicate that extra-major salivary gland pleomorphic adenomas are most commonly found in the soft palate. Wide excision was the treatment of choice, although its efficacy might be compromised with cosmetics and functional structures of the head and neck. Therefore, long-term follow-up of patients is necessary.

The Clinical Feature and Treatment Outcome of Ocular Melanoma: A 34-Year Experience in a Tertiary Referral Center.

Malignant melanoma can arise from melanocytes in various structures of the eye, orbit, and ocular adnexa. We reviewed the clinical features and long-term results of all subjects with histologically proved melanoma originating from any of the ocular and periocular structures in a tertiary referral center. Overall, 88 patients including 47 men were recruited. The tumor was primarily located in the uvea, followed by the conjunctiva, orbit, eyelid, and lacrimal sac. Patients with uveal melanoma were diagnosed at a relatively younger age (47.0 years), while those with orbital and eyelid melanomas were older at presentation (79.5 years and 78.5 years, respectively). The overall local recurrence rate was 9% at a median follow-up of 41.0 months, among which orbital and eyelid melanomas recurred most commonly. The overall mortality rate was 41% in a median duration of 27.2 months (IQR, 13-58 months) from diagnosis, with the highest for lacrimal sac melanoma, followed by melanoma of the orbit, uveal, conjunctiva, and eyelid. Despite prompt local control, the risk for metastasis and mortality was high. Therefore, efficient modalities for early diagnosis and treatment of ocular melanoma are necessary.

Comparison of Clinical Features and Treatment Outcome in Benign and Malignant Lacrimal Sac Tumors.

PURPOSE: To compare the clinical characteristics and treatment outcome between benign and malignant lacrimal sac tumors. METHODS: We retrospectively reviewed the medical records of all patients with pathologically confirmed lacrimal sac lesions from 1995 to 2018 in a tertiary medical center. RESULTS: Among 65 eligible cases, 46 (70.8%) were benign lacrimal sac tumors and 19 (29.2%) were malignant lacrimal sac tumors. Secondary malignancy from nasal or paranasal cancer accounted for 47% of malignant lacrimal sac tumors. The patient's mean age at the time of diagnosis was 60 years in the benign group and 48 years in the malignant group (p=0.03). The most common presenting symptoms were a palpable lump/mass and epiphora in both groups. Palpable mass extending above the medial canthal tendon was noted in 9% of the benign group and in 74% of the malignant group, respectively (p=0.03). The most common presenting symptoms were a palpable lump/mass and epiphora in both groups. Palpable mass extending above the medial canthal tendon was noted in 9% of the benign group and in 74% of the malignant group, respectively (p=0.03). The most common presenting symptoms were a palpable lump/mass and epiphora in both groups. Palpable mass extending above the medial canthal tendon was noted in 9% of the benign group and in 74% of the malignant group, respectively (p=0.03). The most common presenting symptoms were a palpable lump/mass and epiphora in both groups. Palpable mass extending above the medial canthal tendon was noted in 9% of the benign group and in 74% of the malignant group, respectively (. CONCLUSION: Although benign and malignant lacrimal sac tumors may present similar initial symptoms, timely diagnosis and intervention for malignant lacrimal sac lesions are important because they tend to be infiltrating tumors with a poor outcome.

[The experience of parents caring for adult children with HIV/AIDS].

Antiretroviral treatment dramatically reduces mortality and prolongs the life expectancy of HIV/AIDS patients in Taiwan. The stigma attached to AIDS, and conflicts within the family result in family stress and emotional distress. Consequently, parents of adult children with HIV/AIDS endure perpetual distress while caring for their adult children. The purpose of this study is to elucidate the experiences of seven parents caring for children over 18 years of age with HIV/AIDS. Hermeneutic phenomenological methodology was applied to reveal the trajectory of parents' caring experiences and to discover the hidden meanings of the phenomena. Data were collected using semi-structured in-depth interviews lasting from 1.5 to three hours. Interviews were tape-recorded and transcribed verbatim. Data were analyzed using thematic analysis with the concept of the hermeneutic circle. Four essential themes emerged, and were identified and clarified. The parents' caring experiences were: (1) We didn't expect our children's controversial illness, so we pretend to ignore the taboo; (2) We are suffering from shame, and our daily lives and interaction with others have changed; (3) We provide advice frequently to protect our children from pain, and try our best to take care of them in order to bring them better fortune; and (4) We feel helpless in the face of predestined causality, and wish we could bear the burden of sin for our children. Unconditional love and endless responsibility, furthermore, were the essential experiences of these parents. Our findings highlight the importance of patient-center nursing care for HIV/AIDS patients and for healthcare professionals to assist HIV affected families on related family stress throughout the illness trajectory.

Immunoglobulin G4-related ophthalmic disease.

Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized inflammatory disease of unknown etiology. It characterized by distinctive histopathological appearance of dense IgG4-positive lymphoplasmacytic infiltrates, storiform fibrosis, and obliterative phlebitis in one or more organs, simultaneously or subsequently. In cases of ocular adnexal involvement, unique clinicohistopathological features were delineated by recent studies, and IgG4-related ophthalmic disease (IgG4-ROD) is generally recognized as the disease name. A significant proportion of previous labeled idiopathic orbital inflammations and Mikulicz's disease are now consistent with a diagnosis of IgG4-ROD. Increasing studies have accumulated regarding its epidemiology, diagnosis, clinical features, treatment, and the association between lymphoma. In this review, we summarize our present understanding of IgG4-ROD.

Primary ductal adenocarcinoma of lacrimal gland: Two case reports and review of the literature.

A 64-year-old male presented with progressive proptosis of the left eye for 3 months. Orbital computed tomography (CT) demonstrated a 3.9 cm infiltrative mass over the superotemporal quadrant of the left orbit. Pathology of biopsy revealed a ductal adenocarcinoma of lacrimal gland with positive immunohistochemical staining for androgen receptor (AR), cytokeratin-7 (CK7), and gross cystic disease fluid protein 15 (GCDFP-15). The patient received orbital exenteration and adjuvant chemoradiotherapy. No recurrence or metastasis was noted 27 months after treatment. Another case was a 64-year-old male who came for proptosis of the right eye and diplopia for 3 weeks. Orbital CT revealed a 5 cm infiltrated right superotemporal orbital mass with destruction of the lateral and inferior orbital walls. Biopsy showed primary ductal adenocarcinoma of lacrimal gland with positive immunohistochemical staining for CK7, AR, and epidermal growth factor receptor. The patient underwent exenteration and concomitant chemoradiotherapy. However, lung and neck metastasis was noted 21 months after surgery. Collectively, 26 cases in the literature were reviewed. The mean age was 57 years old and male was prevalent (73%). Most immunohistological staining showed positive for AR (46%), CK7 (46%), Ki-67 (38%), and GCDFP-15 (35%). More than half of the patients developed metastasis and one-third of the patients died of disease. Early diagnosis, treatment, and long-term follow-up are required for this aggressive tumor.

Conjunctival papilloma: Clinical features, outcome, and factors related to recurrence.

PURPOSE: The purpose of the study was to evaluate the clinical features, treatment, and outcome of patients with conjunctival papilloma. MATERIALS AND METHODS: Twenty-two patients (22 eyes) with biopsy-proven conjunctival papilloma between January 2005 and January 2015 in a tertiary medical center were retrospectively reviewed. Clinical profiles, treatment, outcome, and factors related to recurrence were evaluated. RESULTS: There were 16 males (73%) and 6 females (27%), with a mean age of 47 years. The most common location of conjunctival papilloma was the caruncle (43%), followed by palpebral conjunctiva (29%), bulbar conjunctiva (14%), and fornix (14%). Recurrence developed in five patients (22.7%). The risk of postoperative recurrence was significantly related to the presence of bulbar conjunctival papilloma with corneal involvement (P = 0.043) and surgical excision alone (P = 0.039). One case with multiple recurrences developed nonkeratinizing carcinoma. Two young females developed conjunctival papilloma even after receiving human papillomavirus vaccinations. CONCLUSION: The recurrence of conjunctival papilloma is not uncommon, especially for those patients underwent surgical excision alone. Surgical excision with adjunctive therapy and long-term follow-up is rational for the treatment of conjunctival papilloma.

Oxidative stress change by systemic corticosteroid treatment among patients having active graves ophthalmopathy.

OBJECTIVES: To measure the 8-hydroxy-2'-deoxyguanosine (8-OHdG) level in patients having active Graves ophthalmopathy (GO) and to compare this oxidative stress biomarker and the clinical evolution of patients after systemic corticosteroid treatment. METHODS: In 8 euthyroid patients having active GO, we determined the 8-OHdG levels in urine before, during, and after intensive corticosteroid therapy. Clinical activity and ophthalmopathy index scores were assessed. Nine age- and sex-matched healthy volunteers served as control subjects. RESULTS: The mean 8-OHdG level was statistically significantly increased in patients having active GO compared with that of controls (17.47 vs 5.97 ng/mg of creatinine, P < .001). During and after maximal systemic corticosteroid treatment, patients had statistically significantly lower mean 8-OHdG levels (7.19 and 10.18 ng/mg of creatinine, respectively) compared with the mean level before treatment. These changes were accompanied by decreases in clinical activity and ophthalmopathy index scores. The urinary 8-OHdG levels were subsequently elevated in 2 patients having recurrent active GO when corticosteroid therapy was tapered or withdrawn. CONCLUSIONS: Oxidative stress may have a role in the pathogenesis of GO. Urinary 8-OHdG level can be used not only as a noninvasive biomarker of oxidative stress in patients having GO but also as an objective and quantitative parameter in the follow-up of patients during immunosuppressive treatment.

Lacrimal gland extranodal marginal zone B-cell lymphoma of MALT-type.

PURPOSE: To evaluate the clinical features and outcome of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma) in the lacrimal gland. DESIGN: Retrospective, noncomparative, observational case series in an academic referral setting. METHODS: A consecutive series of 13 histologically verified MALT lymphoma in the lacrimal gland at presentation was studied. Clinical characteristics, treatment, and prognosis were analyzed. RESULTS: Eight males and five females with a median age of 64 years and a median follow-up time of 48 months were included. All patients had no prior lymphoma and initially presented as MALT lymphoma in the lacrimal gland. Extraorbital involvement at diagnosis was noted in six patients (46.2%). Two patients had autoimmune disease, and both had Stage IV disease at presentation. Treatment consisted of surgical resection in one patient, radiotherapy in four, chemotherapy in four, and combined radiotherapy and chemotherapy in four. Complete remissions were obtained in eight patients (61.5%). Patients with bilateral disease (61.5%) had a higher rate of advanced-stage disease and a poor outcome. Recurrence was noted in two patients. At the last follow-up, eight patients were free of disease, three were alive with disease, one died of sepsis as a complication of chemotherapy, and one died of lymphoma. CONCLUSIONS: MALT lymphoma in the lacrimal gland has a high rate of extraorbital involvement and synchronous bilateral lacrimal gland involvement at presentation. The prognosis is relatively poor, especially in patients with advanced disease and bilateral involvement. Extensive staging and long-term follow-up are warranted for these patients.

Change of Serum IgG4 in Patients with Ocular Adnexal Marginal Zone B Cell Lymphoma Associated with IgG4-Related Ophthalmic Disease After Treatment.

PURPOSE: To investigate the change of serum IgG4 concentrations correlated with clinical evolution in patients with ocular adnexal marginal zone B cell lymphoma associated with IgG4-related ophthalmic disease (IgG4-ROD). METHODS: Three consecutive patients with histopathologically confirmed ocular adnexal marginal zone B cell lymphoma associated with IgG4-ROD were evaluated. Two patients received radiotherapy and 1 patient received steroid therapy. Treatment outcome was evaluated by clinical symptoms, radiologic examination, and change of serum IgG4 level in these patients. RESULTS: All patients had elevated serum IgG4 before treatment (462, 338, and 780 mg/dL respectively.) The 2 patients who received radiotherapy achieved complete remission and the serum IgG4 decreased to 345 and 92 mg/dL, respectively. The patient who underwent systemic steroid achieved partial remission and the serum IgG4 decrease to 161 mg/dL. CONCLUSION: Our study showed elevated serum IgG4 in all patients with ocular adnexal marginal zone B cell lymphoma associated with IgG4-ROD. In addition, the elevated serum IgG4 may decrease or keep stable after treatment, accompanied by improvement in clinical symptoms and reduction of lesions.

Retinoblastoma with spinal recurrence presenting as spinal cord compression.

Central nervous system (CNS) involvement is not rare in extraocular retinoblastoma, and it is not surprising to find it in view of its route of spread. However, although spinal recurrence presenting as spinal cord compression (SCC) is a form of CNS involvement, it is extremely rare. This report describes two patients with unilateral retinoblastoma with spinal recurrence presenting as SCC. The first patient developed erythematous swelling of the right foot and weakness of the bilateral lower limbs at 7 months after left enucleation. Examination revealed pitting edema of bilateral feet and muscle power of 2+ to 3+, with intact sensory function. The second patient developed weakness of the bilateral lower limbs, and defecative and urinary difficulty for 2 days at 8 months after left enucleation. Examination revealed pitting edema of bilateral feet and muscle power of 2+, with defective sensory function. Both patients received surgery and local irradiation after SCC. The first patient refused chemotherapy and survived only 4 months due to disease progression. The second patient received systemic and intrathecal chemotherapy, and survived 19.5 months without disease progression. Spinal recurrence with SCC should be suspected when leg weakness or bowel or bladder disturbance occurs in patients with retinoblastoma.

Preseptal and orbital cellulitis: a 10-year review of hospitalized patients.

BACKGROUND: Preseptal and orbital cellulitis range in severity from minor to potentially severe complications. The purpose of this study is to describe the clinical features of patients with preseptal or orbital cellulitis in one medical center in Taiwan, and to assess the effectiveness of treatments and the complications. METHODS: Patients admitted between 1996 and 2005 to Taipei Veterans General Hospital under the diagnosis of preseptal or orbital cellulitis were retrospectively reviewed. The demographics, administrative history, past history, clinical presentations, treatments, and complications were analyzed. RESULTS: In total, 94 patients fulfilling the diagnostic criteria for preseptal or orbital cellulitis were identified (67 had preseptal cellulitis, 27 had orbital cellulitis). While paranasal sinus disease was the most common predisposing cause in orbital cases, skin lesions in children and dacryocystitis in adults were the most common in preseptal cases. Microbiologic investigations showed variable results, but the most common pathogen isolated was Staphylococcus aureus. Cultures from eye swabs and local abscesses gave the highest positive yield. Blood cultures were taken in some patients, but the positive rate was extremely low. Treatments included intravenous antibiotics alone, or intravenous antibiotics combined with surgical drainage. Only one case had permanent ocular motility impairment after removal of the orbital foreign body. CONCLUSION: Despite the past history of potential morbidity and even mortality from orbital cellulitis, early diagnosis and prompt treatment with proper antibiotics and/or surgical intervention can achieve a good prognosis.

Needle decompression in a patient with vision-threatening orbital emphysema.

Orbital emphysema is a condition resulting from trapping of air in loose subcutaneous or orbital tissues from the paranasal sinuses. This condition commonly seen in patients with a history of periorbital trauma or surgery, especially following sneezing or nose blowing. It usually has a benign and self-limited course. However, the entrapped orbital air can cause a substantial increase in pressure with restricted ocular motility or vascular compromise and become severe enough to cause visual impairment. We herein present the case of a patient who developed severe orbital emphysema after blunt trauma followed by sneezing and was successfully treated with needle decompression of intraorbital air. Emergency needle decompression resulted in an improvement in vision and intraocular pressure.

Clinical features, microbiological profiles and treatment outcome of lacrimal plug-related canaliculitis compared with those of primary canaliculitis.

AIMS: To compare the clinical features and treatment outcome between lacrimal plug-related canaliculitis and primary canaliculitis. METHODS: Patients with plug-related canaliculitis and primary canaliculitis between 2007 and 2014 in a medical centre were collected. Charts were reviewed for clinical features, microbiological profiles, time lapse between plug insertion and symptom onset, type of plug and outcomes. RESULTS: Of 76 eligible cases collected, 13 were plug-related canaliculitis and 63 were primary canaliculitis. The most common presenting symptom was discharge in both groups (85% and 79%, respectively). The average time interval from plug insertion to symptoms onset was 5.5 years. Most canaliculitis developed in women, especially for plug-related canaliculitis, when compared with primary canaliculitis (100% vs 65.1%; p=0.015). The most common isolated microorganism was Pseudomonas aeruginosa in plug-related canaliculitis (46%) and Streptococcus in primary canaliculitis (28%), respectively. Isolation of Pseudomonas was significantly higher in plug-related canaliculitis than in primary canaliculitis (46% vs 12%; p=0.029). Most plug-related canaliculitis resolved after removal of plugs by canaliculotomy (12 cases, 93%). Most identified plug was SmartPlug (seven cases), followed by EaglePlug (two cases) and Herrick Lacrimal Plug (two cases). There was no recurrence in patients with plug-related canaliculitis, however, recurrence developed in seven patients (11%) with primary canaliculitis. CONCLUSIONS: In comparison with primary canaliculitis, plug-related canaliculitis appear to be more prevalent in women and show a different microbiological profile. Retrieval of infected plug by canaliculotomy and adequate antibiotics can achieve a good outcome. Long-term follow-up is required because canaliculitis may develop several years after plug insertion.

Ophthalmic manifestations of paranasal sinus mucoceles.

BACKGROUND: Paranasal sinus mucoceles are mucus-containing cysts caused by obstruction of the sinus orifice. Although such mucoceles originate in the paranasal sinuses, they are usually only associated with ophthalmic and not rhinologic symptoms. The purpose of this study was to demonstrate the presence of ophthalmic manifestations in patients with paranasal sinus mucoceles, and to discuss the possible mechanisms and prognosis for such manifestations. METHODS: Medical records for 41 patients with paranasal sinus mucoceles, who presented with ophthalmic complaints at our department over a 10-year period (1989-1999), were reviewed retrospectively. For analysis of the relationships between ophthalmic complaints and the affected paranasal sinuses, we considered the paranasal sinuses as anterior and posterior. RESULTS: Proptosis (n = 19), periorbital pain (7), and impairment of ocular mobility (5), were common manifestations associated with mucoceles in the anterior paranasal sinuses (25 patients), while blurred vision (n = 9) and impairment of ocular mobility (3) were frequently associated with mucoceles in the posterior paranasal sinuses (11 patients). Ophthalmic complaints resolved in 38 of 41 patients (92.7%) after operation. CONCLUSION: The clinical ophthalmic manifestations of paranasal sinus mucoceles correlated with the paranasal sinuses involved (i.e. anterior or posterior). Because of cranial nerve involvement, mucoceles in the posterior paranasal sinuses had a worse prognosis than those in the anterior paranasal sinuses.

Ocular adnexal IgG4-related disease: clinical features, outcome, and factors associated with response to systemic steroids.

PURPOSE: The aim of this study was to investigate clinical characteristics, outcome, and factors associated with response to systemic administration of steroids in patients with ocular adnexal immunoglobulin G4 (IgG4)-related disease. METHODS: This was a retrospective evaluation of 11 patients with histopathologically verified ocular adnexal IgG4-related diseases at a medical center in Taiwan between January 2006 and December 2012. Clinical features and outcome, including serial change of serum IgG4 and clinical factors related to response to steroids systemically were evaluated. RESULTS: Seven men and four women, mean age 54.5 years and mean follow-up of 33.5 months, were evaluated. Elevated serum IgG4 levels (>135 mg/dl) were observed in ten patients (91%). Lacrimal gland involvement was noted in eight (72.7%), followed by orbit, extraocular muscles, and eyelids. Seven patients (63.6%) had bilateral ocular lesions and eight (72.7%) had extraorbital involvement. Eight of ten patients who underwent systemic steroid treatment responded well in the early phase. Recurrence developed in five patients (45%), requiring repeat steroid therapy and adjunctive treatment. Median serum levels of IgG4 reduced from 540 to 101 mg/dl in ten patients after systemic corticosteroid administration. Patients with lower serum IgG4 and IgG4:IgG ratio at diagnosis were associated with poor response (p = 0.037). CONCLUSIONS: Ocular adnexal IgG4-related disease predominantly involved the lacrimal glands bilaterally and was usually associated with high serum IgG4 levels and multiple organ involvement. Most patients responded well to steroid therapy, which was accompanied by a marked decrease in serum IgG4.

Adjunctive Orbital Radiotherapy for Ocular Adnexal IgG4-related Disease: Preliminary Experience in Patients Refractory or Intolerant to Corticosteroid Therapy.

PURPOSE: To present the clinical outcomes of combined orbital radiotherapy and systemic corticosteroid for patients with refractory ocular adnexal IgG4-related disease. METHODS: We retrospectively reviewed 3 patients with histopathologically confirmed ocular adnexal IgG4-related disease who had been refractory or intolerant to corticosteroid therapy and treated with adjunctive orbital radiotherapy (2000 cGy; 10 fractions). Clinical improvement was assessed by monitoring the patient's ability to taper corticosteroid to discontinuation and by follow-up radiologic examination. RESULTS: All 3 patients had a favorable response to adjunctive radiotherapy with improvement of the clinical symptoms and radiologic abnormalities. Systemic corticosteroid was tapered and discontinued in all patients successfully. There were no adverse effects of treatment or recurrence after a mean follow-up of 19 months. CONCLUSION: Adjunctive radiotherapy can help to achieve stable disease and cessation of systemic corticosteroid in patients with refractory ocular adnexal IgG4-related disease.