IgG4-related disease in patients with idiopathic orbital inflammation.

BACKGROUND: To identify the prevalence of positive IgG4 immunostaining in orbital tissue among patients previously diagnosed with nongranulomatous idiopathic orbital inflammation (IOI) and to compare the clinical characteristics of patients with and without IgG4-positive cells. METHODS: A retrospective review of all patients with a histopathologic diagnosis of IOI was performed. Immunohistochemical staining was performed to identify IgG-positive cells and IgG4-positive cells. Multivariate analysis was performed using likelihood ratio-test logistic regression on the differences between IgG4-related disease (IgG4-RD) and non-IgG4-RD. RESULTS: Of the 45 patients included, 21 patients (46.7%) had IgG4-positive cells, with 52.4% being male and a mean age of 55.9 ± 13.4 years. Bilateral ocular adnexal involvement (adjusted odds ratio [aOR] = 9.45; P = 0.016) and infraorbital nerve enlargement (aOR = 12.11; P = 0.008) were frequently found in IgG4-RD patients. Complete remission occurred in 23.8% of IgG4-RD patients and 41.7% of non-IgG4-RD patients. IgG4-RD patients had more frequent recurrent disease than non-IgG4-RD patients. CONCLUSIONS: Nearly 50% of IgG4-RD patients were previously diagnosed with biopsy-proven IOI. IgG4-RD was more frequent in patients with bilateral disease and infraorbital nerve enlargement, showing the importance of tissue biopsy in these patients. Immunohistochemistry studies of all histopathology slides showing nongranulomatous IOI are highly recommended to evaluate for IgG4-RD.

Clinicopathologic Characteristics and Predictors Affecting Survival Outcome of Eyelid Malignancy.

The aim of this study was to determine the clinicopathologic characteristics and evaluate the prognostic factors in eyelid malignancies. This was a retrospective, comparative, case series of 70 patients with eyelid malignancies. The mean age at diagnosis was 72.0 years (range 30.5-93.0 years) with 64.3% female. The 2 most common histologic types were basal cell carcinoma (BCC) followed by sebaceous gland carcinoma (SGC). In total, 47.1% of the malignancies were located in the lower eyelid and the majority of tumor (T) category was T1. The authors assessed the disease-specific survival rates of 64 surgical patients using Kaplan Meier curves. Univariate and multivariate analyses identified the predictors associated with disease-specific survival. Of the 64 surgical patients, 4 had regional nodal metastasis (2 SGC, 1 squamous cell carcinoma [SCC], and 1 adenocarcinoma) and distant metastasis at diagnosis was in 3 patients (2 SGC and 1 malignant melanoma [MM]). Six patients died of disease during follow-up. The 5-year disease-specific survival in BCC, SCC, and SGC were 100%, 100%, and 64.8%, respectively. The predictive factors affecting worse disease-specific survival on multivariate models were T4 category (adjusted hazard ratio [aHR] 14.15, P = 0.022) and tumor recurrence (aHR 6.84, P = 0.045). In conclusion, BCC was the most common eyelid malignancy in southern Thailand followed by SGC. In this study, T4 category and tumor recurrence were the most important prognostic factors in eyelid malignancies.

Factors related to survival outcomes following orbital exenteration: a retrospective, comparative, case series.

BACKGROUND: Orbital exenteration is a disfiguring procedure that aims to achieve local control. It is commonly a part of the management of malignant orbital tumor which is a life-threatening condition. It is necessary to determine predictive factors associated with overall survival (OS) following orbital exenteration. METHODS: This was a retrospective, comparative, case series of 39 patients with malignant tumors who underwent orbital exenteration. Patient records were reviewed for age, clinical presentation, preoperative visual acuity (VA), tumor size, surgical margin, tumor invasiveness, recurrent disease, and status of distant metastasis. Kaplan-Meier curves were used to assess OS and event-free survival (EFS). The predictive factors related to OS were identified using multivariate analysis. RESULTS: The mean age was 62.9 years (range, 5.5 to 89.7 years), 68.4% presented with VA < 20/400. The mean size of all tumors was 32 ± 18 mm. Distant metastasis at diagnosis was reported in 11 patients (28.2%). Twenty-two patients died during follow-up. The median OS and EFS were 3.89 years and 3.01 years, respectively. The predictive factors for worse OS on multivariate analysis were preoperative VA < 20/400 (adjusted hazard ratio [aHR] 4.67, P = 0.003), tumor size larger than 20 mm (aHR 3.14, P = 0.022,) and positive distant metastasis at diagnosis (aHR 15.31, P <  0.001). CONCLUSIONS: The prognostic factors for poor survival outcome following orbital exenteration were a preoperative VA < 20/400, tumor size > 20 mm, and distant metastasis at diagnosis mostly due to patient negligence.

Indications and results in anophthalmic socket reconstruction using dermis-fat graft.

OBJECTIVES: To present the indications for a dermis-fat graft in anophthalmic socket reconstruction and evaluate the results of this procedure. DESIGN: Retrospective review, interventional case series. METHODS: In our study, there were 41 patients who received either primary or secondary dermis-fat graft between August 1, 2007 and July 31, 2012 at Songklanagarind Hospital. RESULTS: The type of dermis-fat graft used in our study consisted of both primary dermis-fat grafts (6/41) and secondary dermis-fat grafts (35/41). The major indications for dermis-fat graft were exposure (13/41), extrusion (11/41), and volume insufficiency with a shallow fornix (10/41), which accounted for 82.9% of the total cases. A total of 30 patients were able to wear the eye prosthesis over a mean follow-up time of 32.3±19.0 months. The success rate of the dermis-fat graft alone was 73.3%, while that of the simultaneous dermis-fat graft and mucous-membrane graft was 25%. CONCLUSION: The most common indications for a dermis-fat graft were extrusion and large exposure. Dermis-fat graft is an alternative implant type in complicated enucleation/evisceration patients, which has a good success rate.

Clinical presentation, treatment, and prognosis of periocular and orbital amyloidosis in a university-based referral center.

OBJECTIVE: The purpose was to present the demographic data, clinical presentation, and treatment options, and to evaluate prognosis, for periocular and orbital amyloidosis in patients at the Songklanagarind Hospital, Thailand. METHOD: This was a retrospective study that reviewed the clinical records of six patients who were diagnosed with periocular and orbital amyloidosis between January 1, 2002 and December 31, 2011. RESULTS: The series included six patients (five female and one male). The mean age was 39.7 years (range 15-79 years). There were five cases of unilateral lesion and one case of bilateral lesion. The most common symptom was a mass lesion (83.3%), followed by irritation (66.7%), bloody tears (16.7%), epiphora (16.7%), eye pain (16.7%), and ptosis (16.7%). Five cases had periocular involvement and one case had orbital involvement. Five cases were investigated to rule out systemic amyloidosis, and no systemic involvement was found in these cases. The median duration of symptoms was 6 months, but the mean was 31 months. The main treatment option was surgical excision. The mean follow-up time was 41 months. There was disease progression in 20% of cases after definitive treatment. CONCLUSION: Periocular and orbital amyloidosis presented with a variety of symptoms, depending on the location of the disease. A mass lesion was the most common symptom. The intent of the treatment modalities was to spare function.

Clinical presentation, aetiology and prognosis of orbital apex syndrome.

OBJECTIVES: To present the clinical features, determine the causes and evaluate the prognosis of orbital apex syndrome in patients of Songklanagarind Hospital. METHODS: A retrospective review was conducted of patients diagnosed with orbital apex syndrome between January 1, 2002 and December 31, 2008. RESULTS: In our series, the major cause of orbital apex syndrome was carotid-cavernous sinus fistula, for which 30 patients were diagnosed. After excluding those patients whose condition was caused by carotid-cavernous sinus fistula, 50 patients (58 eyes) were analyzed. The mean age of the patients was 47.60 ± 18.88 years (27 male patients and 23 female patients). The most common presenting symptom was blurred vision (86%). The median duration of the presenting symptom was 37.5 d. Of the 50 patients the most frequent aetiology of orbital apex syndrome was neoplasia (48%), of which lymphoma was the most common in this group. After treatment, the vision of 19.4% of the patients improved and proptosis improved by 68.4%. CONCLUSIONS: The most common presenting symptom of orbital apex syndrome was blurred vision. The most frequent aetiology of orbital apex syndrome of all patients was carotid-cavernous sinus fistula, while the next most frequent was neoplasm, of which lymphoma was the most common in this group. Patients had a good outlook, after treatment directed at the cause, with a modest improvement in vision and exophthalmos in more than half of patients.

Retrograde cavernous sinus thrombosis and orbital cellulitis secondary to meningitis in immunocompetence child.

A case of cavernous sinus thrombosis is usually a complication of severe orbital cellulitis and meningitis. The authors reported a retrograde cavernous sinus thrombosis and orbital cellulitis in an immunocompetence child, due to meningitis.

Rhino-orbital fungal infection: two cases report.

Rhino-orbital fungal infections are serious and life threatening complications of immunocompromised host. The authors reported two cases of rapid progressive proptosis and eyelid necrosis of immunocompromised patients who suffered from highly malignant T-celled lymphoblastic leukemia/lymphoma and congenital heart disease with multiple anomalies. Although early diagnosis was made and prompt treatments including medical and surgical interventions were performed, both patients died.

Results of nonendoscopic endonasal dacryocystorhinostomy.

BACKGROUND: Surgical scarring on the face and disrupted anatomy in the medial canthal area following external dacryocystorhinostomy (DCR) can be avoided by an endonasal approach. This study examined the outcome of direct visualization endonasal DCR, performed by young surgeons and residents. METHODS: A retrospective case series of 75 consecutive endonasal DCRs performed under direct visualization from July 2002 to July 2004 were reviewed. Surgery was performed by surgeons and residents who had received no special training in the procedure. Full success was defined as no symptoms of tearing after surgery and anatomical patency with fluorescein flow on nasal endoscopy or patency to lacrimal syringing. Partial success was defined as a tearing decrease compared with prior to surgery and with anatomical patency, and failure was defined as no significant improvement in persistent tearing. The average follow-up duration was 26.83 ± 16.26 (range 6-55) months. RESULTS: Seventy-five DCRs were performed on 63 patients (four male, 59 female) of mean age 49.44 ± 16.63 (range 21-85) years. The surgery was successful in 54/75 eyes (72%), 37/54 eyes (68.5%), and 30/42 eyes (71.4%) at 6, 12, and 24 months, respectively. Partial success was achieved in 13/75 (17.3%), 9/54 (16.7%), and 9/42 (21.4%), and the failure rates were 10.7%, 14.8%, and 7.1% at 6, 12, and 24 months, respectively. The overall functional success with this technique was 74.7% and the overall anatomical patency was 92.0%. There were no serious complications arising from the surgery; three minor complications were documented, ie, an incorrectly placed silicone tube in the lower canaliculus, tube prolapse, and postoperative bleeding which needed nasal packing and eventually a developed retention cyst in the nasal cavity. CONCLUSION: Endonasal DCR under direct visualization is a simple technique with minimal complications and a low learning curve, without the necessity for expensive instruments.

Endogenous Klebsiella panophthalmitis: atypical presentation.

Endogenous endophthalmitis caused by K. pneumoniae was considered to be a rare cause of endogenous endophthalmitis. Most of the patients had prior medical conditions, and diabetes is the most common underlying condition. Patients with a K. pneumoniae liver abscess are at risk of developing endogenous Klebsiella pneumoniae endophthalmitis (EKE). Clinical manifestation of K. pneumoniae endophthalmitis are unique with posterior chamber becomes full of pus while anterior segment seem quiet. The authors presented a case of extremely rare presentation of endogenous bacterial endophthalmitis, who presented with choroidal mass. After receiving pars plana vitrectomy and intravitreal antibiotic injection, clinical condition improved. Despite aggressive treatment, the final visual outcome was generally poor.

Isolated orbital neurofibroma in NF-1 negative patients.

Isolated orbital neurofibroma unassociated with systemic neurofibromatosis is relatively rare and may be difficult to clinically differentiate from other orbital tumors. The authors report a case of isolated orbital neurofibroma without neurofibromatosis type 1.

Anthropometry of eyelid and orbit in four southern Thailand ethnic groups.

OBJECTIVE: To study the basic eyelid and orbital measurement in the four main ethnic groups of southern Thailand. MATERIAL AND METHOD: The basic eyelid and orbital surface anatomy of 101 normal subjects aged 20-40 years old were measured in the four ethnic groups of which the majority of people in southern Thailand belong to: Thai, Chinese, Thai-Malay, and Thai-Chinese. RESULTS: Of the 101 subjects, 51 were male and 50 were female. Each ethnic group had at least 12 normal subjects. Male-female data were analyzed separately and compared between groups. The palpebral fissure heights in Thai, Chinese, Thai-Malay, and Thai-Chinese males were 9.5, 9.0, 10.2 and 9.6 mm respectively, which demonstrated statistically significant differences between Thai-Malay versus Thai, and Thai-Malay versus Chinese. The palpebral fissure lengths were 30.4, 29.8, 30.5 and 30.5 mm, but without statistically significant differences. The marginal reflex distances were 3.2, 2.8, 3.7 and 3.1 mm respectively with a statistically significant difference only between Thai-Malay versus Chinese. The levator functions were 15.2, 15.2, 15.3 and 15.2 mm. The upper lid creases were 7.1, 4.0, 6.6, and 4.4 mm, statistically significantly different in Thai versus Chinese, Thai versus Thai-Chinese and Chinese versus Thai-Malay. The Hertel exophthalmometer measurements were 15.4, 16.3, 16.6 and 15.9 mm without statistically significant differences. The female measurements were overall similar to the male measurements, with some parallel differences between the groups. The eyebrow position in this age group was mostly at and above the orbital rim in both genders and all ethnic groups. An absence of upper lid crease and an epicanthal fold were found in significantly greater numbers in the Chinese group compared to the others, while parallel lid crease was significantly found in greater numbers in the Thai-Malay group than in the others. CONCLUSION: Different eyelid characteristics in diferent ethnic groups are an important feature to note when planning for eyelid surgery. As there is a wide range of ethnic groups in Asia, with many differences in eyelid characteristics, it is important for the ophthalmologist to be aware of and understanding these anatomical variations in clinical assessment and treatment in Asians.

Evaluating the effects of ice application on patient comfort before and after botulinum toxin type A injections.

OBJECTIVE: To evaluate the efficacy of ice application in reducing pain during botulinum toxin type A injections. DESIGN: Prospective, randomized, single-blind clinical study. MATERIAL AND METHOD: Sixty patients who underwent botulinum toxin A treatment on their faces were included in the present study, divided into three groups, group 1 had ice applied 5 minutes before the injection, group 2 ice was applied 5 minutes after the injection, and group 3 served as a control, receiving an injection without ice application. A numeric pain distress scale was used to assess pain intensity. Any complications arising from the injections were also recorded. RESULTS: Sixty patients were studied between July 2007 and March 2008. The ages ranged from 32 to 76 years (mean, 58.15 +/- 10.51 years) and 49 (81.7%) were female. Forty-five (75.0%) had hemifacial spasm and fifteen (25.0%) had blepharospasm. The numeric pain distress scale was completed immediately after the injections and the results were 3.55 +/- 1.85, 5.15 +/- 2.81 and 6.1 +/- 2.63 in groups 1, 2, and 3 respectively. The ratings indicated that pain was significantly reduced in group 1 compared to group 3 (p = 0.005), but there was not a significant difference between groups 1 and 2 (p = 0.109) or between groups 2 and 3 (p = 0.448). There was a significant difference in the rate of bleeding after the injections between groups 1 and 2 (p = 0.0472), but no significant difference between groups 1 and 3 (p = 0.106) and groups 2 and 3 (p=1). CONCLUSION: Using an ice application 5 minutes before or after injection showed no difference but both significantly reduce pain compared to without ice application, while 5-minute application before injection had significantly decreased the bleeding compared to both 5 minutes after injection and without ice application. The ice application is helpful in pain relief whereas the before-injection has lesser bleeding than after-injection modality.

Bilateral septic cavernous sinus thrombosis following the masticator and parapharyngeal space infection from the odontogenic origin: a case report.

Neglect of odontogenic infections can have serious consequences. If they spread through fascial planes and intracranially they can cause an abscess, orbital cellulitis, and eventually cavernous sinus thrombosis. The authors report a case of rapid progressive bilateral orbital cellulitis and cavernous sinus thrombosis that originated from dental caries. Septic cavernous sinus thrombosis is a medical emergency. Early recognition and prompt treatments direct to the underlying sources of infection are crucial. Broad-spectrum intravenous antibiotics are the mainstay of treatment to reduce morbidity and mortality from this lethal condition. Management should be based on early diagnosis and prompt management with intravenous broad-spectrum antibiotics and surgical intervention.

Orbital metastasis of renal cell carcinoma masquerading as cysticercosis.

Metastasis to the orbital soft tissues is relatively uncommon. We report a rare case of renal cell carcinoma with orbital metastasis as the first clinical manifestation. A 48-year-old-man presented with left proptosis and complete ptosis of three weeks duration. Radiological examination revealed a left intraconal heterogeneous cyst-like lesion with rim enhancement immediately deep to the left superior rectus muscle. Diagnosed as having orbital cysticercosis, he was prescribed oral albendazole and prednisolone. But there was no clinical improvement. An incisional biopsy performed showed metastatic poorly differentiated carcinoma. The patient complained of backache and weight loss in the interim. Magnetic resonance imaging (MRI) of the spine showed extensive vertebral metastasis to the thoracic and lumbosacral spine and the iliac bone, with an incidental detection of a large mass from the right kidney. Further MRI of abdomen and chest showed a large right renal mass presumed to be a renal cell carcinoma with extension into the right renal vein, intra-abdominal lymph nodes, and peritoneum. There were small nodules in the lung suggesting the possibility of pulmonary metastatic deposits. Renal cell carcinoma does not respond to chemotherapy, immunotherapy, or radiation; because of the disease's advanced stage, the patient received palliative treatment. There have been only two other reports in the literature of metastatic renal cell carcinoma in the orbit where the proptosis was the initial presenting feature similar to our case.

Refractive changes in epiblepharon.

PURPOSE: To study the prevalence of astigmatism in patients with epiblepharon and keratopathy and to determine if astigmatism was influenced by surgical correction. DESIGN: This is a retrospective review of 182 eyes of 91 patients who were diagnosed with significant epiblepharon at the National University Hospital, Singapore. METHODS: Demographic data, best-corrected visual acuity, refractive error at presentation and annually thereafter, presence of amblyopia, severity of keratopathy, and nature of surgical intervention if any were recorded. RESULTS: Mean age of the patients was 7.23 +/- 6.43 years. 52.2% of patients had astigmatism of 1 diopter (D) or more (range, -0.5 to -4.0 D), and the astigmatism was largely with-the-rule. There was no significant association between severity of keratopathy and astigmatism. Nine percent of patients had amblyopia after spectacle correction and all had significant astigmatism. A total of 70.3% of patients underwent surgery and mean time to surgery was 12.2 +/- 13.1 months after diagnosis. Mean age to surgery was 7.5 +/- 7.12 years. Comparison of pre- and postoperative astigmatism in patients younger than age 5 at the time of surgery showed no significant changes in astigmatism at one to two years of follow-up. CONCLUSIONS: There was high prevalence of astigmatism in patients with epiblepharon (52.2% had 1 D or more of astigmatism). A total of 9% of patients had amblyopia from astigmatism. Surgery did not seem to affect astigmatism especially in young children. Possibility of amblyopia from astigmatism must be borne in mind while treating children with epiblepharon.

Transitional cell carcinoma of the lacrimal sac.

Lacrimal sac tumor is a rare tumor, for which we do not know the exact incidence. Malignant tumors account for 70% of all cases. Epithelial tumors are the most common pathological type and squamous cell carcinoma is the most common of these, a transitional cell carcinoma is rare and has a poor prognosis. The authors report a rare case of transitional cell carcinoma of the lacrimal sac. A 57-year-old woman had tearing on her right eye. The pre-operative diagnosis was possible nasolacrimal duct obstruction with signs of dacryocele. Intraoperatively the authors found a lacrimal mass and a frozen section showed squamous papilloma with dysplasia. The tumor was completely removed and DCR was done. The subsequent pathological report of the right lacrimal sac was papillary transitional cell carcinoma, so medial maxillectomy and resection of the medial inferior orbit with ethmoidectomy were performed. She received radiation and has been tumor-free for 2 years.