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Aims: To evaluate the clinical efficacy of membrane tube implant made of expanded polytetrafluoroethylene (e-PTFE, Gore-Tex) membrane and silicone tube in treating refractory glaucoma. Methods: A retrospective chart review was performed on 43 eyes of 40 patients who underwent glaucoma tube shunt implant surgery using double layered e-PTFE membrane and silicone tube to treat refractory glaucoma. The surgeries were performed from May 1991 to September 1995, and the subjects were patients with terminal glaucoma without useful vision on the study eye. Results: The mean follow up period was 32.9 months. The Kaplan-Meier survival for intraocular pressure (IOP) control (IOP between 6 and 21mm Hg without significant complication) was 80.9% at 1 year, 73.9% at 2 years, and 62.2% at 3 years after surgery. After excluding three eyes of three patients who were dropped within 3 months after surgery and did not have any serious complication or problem in IOP control, the average preoperative IOP was 42.5 (SD 14.6) mm Hg and IOP on the last visit was 17.3 (10.2) mm Hg (p = 0.000, n = 40). The number of antiglaucoma medications before surgery (2.2 (0.6)) was reduced to 0.5 (0.8) on the last visit (p = 0.000). The IOP was controlled within the range of 6–21 mm Hg in 26 eyes (65.0%). In the remaining 14 eyes (35%), we could not control the IOP or additional surgery was needed to control the IOP or to treat severe complications. Two cases of endophthalmitis and three of phthisis were found as serious complications. The other complications were similar to those of other commercially available glaucoma implants. Conclusion: A comparable clinical result was obtained with this new implant as with the other commercially available implants. This implant with a thin and non-rigid reservoir has a potential to reduce some complications associated with the large volume and rigid consistency of the other implants, although it is not yet proved. This membrane tube implant may be considered as another substitute in the surgery of refractory glaucoma.

Purpose: To describe the CT findings of distensible orbital venous anomalies. Methods: We reviewed the records of 17 consecutive patients who met the imaging criteria for orbital venous anomalies. We analyzed the clinical data and the CT scans qualitatively and quantitatively. Results: Distensible venous anomalies usually occupied both intraconal and extraconal spaces of the orbit (65%). The contour of the mass was ovoid or fusiform in 3 of the orbits (18%) and tubular or lobular in 14 orbits (82%). The optic nerve was encased by the mass in 8 orbits. Changes in the adjacent bone were detected in 9 orbits. The average volume of the venous anomalies was 1.1 mL in the axial scan and 5.7 mL in the coronal scan. The patients who showed clinically evident exophthalmos on the Valsalva maneuver had larger volumes than those who did not. Conclusions: The orbital structures adjacent to the distensible orbital venous anomalies were vulnerable to intermittent compression. CT scans, including both postcontrast axial and coronal images, were useful in demonstrating the presence of distensible venous anomalies, even in cases with no apparent exophthalmos on the Valsalva maneuver.

Mass lesions of the lacrimal fossa primarily consist of inflammatory and benign and malignant neoplastic disorders of the lacrimal gland and cysts. The most common benign and malignant epithelial tumors of the gland are the pleomorphic adenoma and adenoid cystic carcinoma, respectively. Other tumors, including lymphoma, acinic cell carcinoma, oncocytoma, sebaceous carcinoma, solitary fibrous tumor, hemangioma, and Warthin’s tumor may be encountered in this region. Common inflammatory and structural pathologies such as sarcoidosis, Sjogren’s disease, Wegener’s granulomatosis, and dermoid cysts and dacryops are also covered in this chapter. The morphology, clinical aspects, and treatment of the lesions of this area are reviewed in this chapter.

A 19-year-old woman who underwent conjunctivodacryocystorhinostomy with Medpor-Coated Tear Drain 2 years ago presented with diplopia on left gaze for 4 months. Limitation of extraocular movement of OS on left gaze was observed on duction test. The forced duction test revealed restriction of the left medial rectus muscle. Orbital MRI demonstrated an enhancing soft tissue lesion surrounding the tube in inferomedial aspect of left orbit. Removal of the tube and adhesiolysis were performed. Histologic findings were consistent with a chronic inflammation with fibrosis. After surgery, limitation of extraocular movement and diplopia were completely resolved. Jones tube coated with a thin layer of porous polyethylene allows the ingrowth of fibrovascular tissue into the coating, decreasing the probability of tube extrusion, but can also accelerate fibrotic changes around the tube causing restrictive strabismus.

We sought to describe the histopathologic features of lacrimal ductular cysts with or without inflammation and speculate on the role of cilia in their pathogenesis. A retrospective study of 15 patients referred for lateral canthal subconjunctival cysts from 2001 to 2012 was conducted. All patients underwent cyst removal and a total histopathological examination was done. Patients were classified into 2 groups and analyzed according to their clinical and histopathologic characteristics. Fourteen showed unilateral disease and one had bilateral disease, with a mean age of 42.8 years. Six patients had mild discomfort or no symptoms, and the remaining 9 of 15 patients presented with a symptomatic mass with conjunctival discharge and injection. Two layers of ductal epithelium constituted the whole wall of the cyst in the seven non-inflammatory cysts, three of which showed cilia in the lumen. The presence of stratified columnar epithelium with goblet cells was a peculiar feature in the lining of the nine inflammatory cases, seven of which showed cilia shafts with inflammatory cells and concrete material in the lumen. Pilosebaceous units were not evident in any of the specimens on pathologic examination. Cilia entrapped in the lacrimal ductule may play a role in the formation of lacrimal ductular cysts with or without inflammation. Cilia falling from the eyelid may be caught in the conjunctival fornix then shift toward the horizontal lacrimal ductule in the lateral canthus. It may then serve as a nidus to provoke lacrimal ductular inflammation or cyst formation.

Canaliculocele is an extremely rare cause of a medial canthal mass. The authors report 2 cases in which painless medial canthal masses occurred. A 77-year-old man presented with a nontender, firm mass in the medial canthal area. This mass was located at the common canalicular level and was observed to obstruct lacrimal outflow. It was determined to be filled with mucinous material and was connected with the canaliculi. A 40-year-old woman had a fluid-filled globular cyst on her lower eyelid. The cyst was found to consist of dilated inferior canaliculus wall from the punctum to the common canaliculus. On histopathologic examination, the 2 cases shared a common feature: the walls were composed of nonkeratinized stratified squamous epithelium, which was compatible with the canaliculus. Canaliculocele should be considered in the differential diagnoses of a medial canthal mass.

A 77-year-old man was referred to the authors with an impression of recurrent chalazion of the left lower eyelid. He had suffered from multiple myeloma and had undergone chemotherapy for 11 months. A 2.0 x 0.8 cm, firm, tender, oval-shaped, ulcerated mass with an accompanying black crust on its surface, and erythematous swelling around it was observed. Evaluation of an eyelid biopsy specimen revealed neoplastic plasma cells with eccentric nuclei and monoclonality for lambda light chain, which the authors report as the first case of metastatic multiple myeloma presenting as a rare necrotizing eyelid lesion.

A 67-year-old woman was referred with the diagnosis of bilateral orbital tumors that were incidentally found on brain MRI. Fundoscopic examination showed bilateral optic disc colobomas and orbital CT showed bilateral cystic masses communicating with the optic disc coloboma. The patient has been examined at regular intervals for 2 years with no change in her masses or visual status. She has normal visual acuity and normal axial lengths.

A 34-year-old woman presented with progressive proptosis of the right eye for 2 years. MRI revealed a well-demarcated mass in the right intraconal space, which was isointense on T(1)- and hyperintense on T(2)-weighted images with bright uniform enhancement. The mass was resected through a lateral orbitotomy. Histopathologic examination revealed cords and clusters of spindle and epithelioid cells embedded in a myxoid matrix, with vacuolated eosinophilic cytoplasm and round nuclei. The tumor cells were diffusely positive for epithelial membrane antigen and vimentin. These findings helped confirm the diagnosis of chordoid meningioma, a rare variant of meningioma with a greater risk of recurrence and aggressive growth. Many of the clinical manifestations and imaging findings were indistinguishable from those of cavernous hemangiomas, solitary fibrous tumors, neurofibromas, and other variants of meningiomas. Given the prognostic significance of chordoid meningioma, extra care and attention should be given during surgery, histopathologic assessment, and follow-up.

* BACKGROUND AND OBJECTIVE: Despite the high success rate of external dacryocystorhinostomy (DCR), recurrent tearing after DCR can be troublesome. The authors performed transcanalicular revision in 6 patients with failed DCR. * PATIENTS AND METHODS: With the use of a continuous wave Nd:YAG laser with a sclerostomy probe, the internal ostium was reopened by a transcanalicular approach. The authors applied 0.4 mg/ml of mitomycin-C around the opening for 5 minutes intranasally and inserted a silicone tube as a stent. * RESULTS: A total of 7 operations were performed in 6 patients. The operation was successful after the first revision in 5 of the 6 patients, but 1 of the patients required a second procedure. * CONCLUSIONS: The transcanalicular laserassisted revision has several advantages. It is simple and fast, skin incision is avoided, there is good hemostasis, it is less traumatic, and there is less postoperative morbidity. [Ophthalmic Surg Lasers 1998;29:451-455.]

An 11-year-old girl presented with extremely large and slightly asymmetric breasts that had developed over a 1-year period. The condition was diagnosed as multiple bilateral juvenile fibroadenomas on the operative field and successfully treated with central pedicle breast reduction using a vertical and short horizontal scar design. Breast function and aesthetic appearance were preserved with no reported recurrence 5 years after surgery.

Purpose: Adult xanthogranulomatous disease of the orbit including adult-onset asthma and periocular xanthogranuloma is rare. To the best of the author’s knowledge, adult-onset asthma and periocular xanthogranuloma have not been previously reported in Korea. Case summary: A 75-year-old man presented with a 15-year history of progressive bilateral yellowish eyelid mass. He had a history of adult-onset asthma diagnosed 13 years ago. On ocular examination, there were firm yellowish bilateral eyelid masses and ptosis markedly on the right eye. Exophthalmos was noted in the right eye. There were exotropia, hypertropia, and gaze restriction of the right eye. Orbit magnetic resonance imaging demonstrated an ill-defined bilateral infiltrative orbital mass extending to the lid, lacrimal gland, and sinuses. A right orbital mass extended to the posterior orbit. Enlargement of extraocular muscles was also noted in the right eye. Left upper eyelid mass debulking surgery was performed. A combination therapy of azathioprine and prednisolone was started. Mass debulking surgeries of the right upper and lower eyelids were performed. Conclusions: Adult-onset asthma and periocular xanthogranuloma is known as a refractory disease. Combination therapy with azathioprine and steroid therapy with mass debulking surgery is a good treatment option for adult-onset asthma and periocular xanthogranuloma. J Korean Ophthalmol Soc 2016;57(9):1465-1471

The goal of this study was to investigate the safety and efficacy of multisession Gamma Knife surgery (GKS) in the treatment of benign orbital tumors.Twenty-three patients who retained their vision despite having tumors touching their optic nerve were treated with multisession (4-fraction) GKS. The median tumor volume was 2800 mm(3) (range 211-10,800 mm(3)), and the median cumulative margin dose was 20 Gy (range 18-22 Gy).The median clinical follow-up duration in these patients was 38 months (range 9-74 months). No patient experienced tumor progression in this study. In particular, a higher degree of tumor shrinkage was found in the 7 patients with cavernous hemangiomas than in patients with other types of lesions (p0.05). Of the 23 patients whose preoperative vision was preserved, 11 showed improvement in visual acuity and/or visual field and 12 showed stable visual acuity. No GKS-related adverse events were noted during or after treatment.Multisession radiosurgery using the Gamma Knife may be a good strategy for tumors in direct contact with the optic nerve. A cumulative margin dose of up to 22 Gy delivered in 4 sessions is safe for preservation of visual function with a high probability of tumor control.

Lateral canthal reconstruction performed to lengthen the horizontal palpebral fissure for aesthetic purposes is becoming more prevalent in Far East Asian countries. The lacrimal ductule fistula has become a complication of this procedure.A retrospective medical record review was performed to analyze four patients (one man and three women) who presented with tearing from a lacrimal ductule fistula after cosmetic lateral canthoplasty for lengthening of a horizontal palpebral fissure. The average age of the patients was 27.3 years (range 21-32 years). The review analyzed patient data from previous surgeries, eye examinations, photographs, operative records, and pathology records.All the patients experienced tearing from a lacrimal ductule fistula after cosmetic lateral canthoplasty performed by four different plastic surgeons. Eye examination demonstrated tearing through one or more lacrimal ductule fistulas in scar tissue of the lateral canthal area. To relieve this symptom, two patients underwent excision of the lacrimal ductule and lateral canthal reconstruction. The symptom subsided postoperatively without recurrence. Pathologic examination performed for one patient showed the lacrimal ductule in the surgical specimen.Lacrimal ductule fistula or exteriorization of the lacrimal ductule can complicate cosmetic lateral canthoplasty if precautions are not taken in the assessment of the patient or in the performance of the procedure. Excision is required to reconcile the symptomatic lacrimal fistula, whereas canthal reconstruction is needed to remedy the disfiguring lateral canthus to achieve appropriate canthal shape and position.

Purpose: To evaluate inferior oblique (IO) underaction related to orbital floor fracture and its management. Methods: We retrospectively assessed 137 patients with orbital floor fractures who had undergone surgical repair between July 2003 and August 2009. Review of clinical data, which included photographs and radiologic findings, was performed. IO underaction was diagnosed based on anomalous head position and which was confirmed with the Hess test and limitation of duction and version in the nine diagnostic positions of gaze. Results: Twelve patients demonstrated IO underaction pattern (8.8%); 3 patients presented preoperatively and 9 patients presented postoperatively. All the patients showed IO underaction pattern in the Hess test and head tilt position. The median age was 9.5 years (range, 6–24 years), and all the patients were male. Of 12 patients, 10 (85%) presented with nausea and vomiting symptoms, 2 (17%) infraorbital hypoesthesia, and 3 (25%) pupillary dilatation. On the basis of the CT scans, all patients had trap door fractures with soft tissue entrapment. The IO underaction recovered spontaneously within 2 months without any treatment. Conclusion: Head tilt towards the injured side can be a warning sign of IO underaction in orbital floor fracture, especially pre- or postoperatively in the paediatric population. Physicians managing paediatric orbital fracture should be aware of this transient complication.

Schizophyllum commune, a basidiomycetous fungus, is a rare cause of mycotic disease in humans. We describe the first case of sino-orbital infection caused by S. commune in an immunocompetent woman who presented with maxillary sinusitis and inferior orbital tumor. Identification of the organism was confirmed by rRNA sequencing.

To describe a medial epicanthoplasty technique using the skin redraping method and review the surgical outcome in patients with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES).Retrospective, noncomparative, interventional case series with the description of a new surgical technique.Sixteen consecutive Asian patients with BPES.The charts of patients with BPES who underwent medial epicanthoplasty using the skin redraping method were reviewed retrospectively. Preoperative and postoperative inner intercanthal distance (IICD), interpupillary distance (IPD), horizontal palpebral fissure length (HPFL), and visibility of the scar were measured. The ratio of the IICD to IPD (IICD ratio) was calculated.Postoperative improvement in IICD ratio and the visibility of the surgical scar.The preoperative median IICD ratio was 1.65 (range, 1.49-1.83) and decreased to 1.27 (range, 1.02-1.48) postoperatively. The median reduction in IICD ratio was 21.7% (range, 16.7%-38.2%) (P0.001, Wilcoxon signed-rank test). Fourteen patients (87.5%) had no visible scarring or scarring only visible under close inspection. Two patients (12.5%) had a more apparent scar, but no patient had severe scarring that required revision.Medial epicanthoplasty using the skin redraping method is an effective technique in the treatment of epicanthus inversus and telecanthus in patients with BPES, with excellent cosmetic outcomes.

Objective The orbital apex is an important anatomic landmark that hosts numerous critical neurovascular structures. Tumor resection performed at this complex region poses a therapeutic challenge to orbital surgeons and often is associated with significant visual morbidity. This article reports the efficacy and safety of multisession gamma knife radiosurgery (GKRS) in benign, well-circumscribed tumors located at the orbital apex. Design Retrospective interventional case series. Participants Five patients with visual disturbances resulting from a benign, well-circumscribed orbital apex tumor (3 cases of cavernous hemangioma and 2 cases of schwannoma). Methods Each patient treated with GKRS with a total radiation dose of 20 Gy in 4 sessions (5 Gy in each session with an isodose line of 50%) delivered to the tumor margin. Main Outcome Measures Best-corrected visual acuity, visual field changes, orbital imaging, tumor growth control, and side effects of radiation. Results All patients demonstrated improvement in visual acuity, pupillary responses, color vision, and visual field. Tumor shrinkage was observed in all patients and remained stable until the last follow-up. No adverse events were noted during or after the radiosurgery. None of the patients experienced any radiation-related ocular morbidity. Conclusions From this experience, multisession GKRS seems to be an effective management strategy to treat solitary, benign, well-circumscribed orbital apex tumors. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

We report a case of intraocular lymphoma in a 65-year-old man, 15 months after cardiac transplantation. On Magnetic Resonance (MR) images, the iris and the anterior chamber of the right eye were found to be involved with an enhancing soft-tissue lesion. To our knowledge, this is the first case of post-transplantation intraocular lymphoma evaluated with MR imaging.

Background Autologous fat injection into the face is a commonly used technique in plastic surgery. Autologous fat is claimed to be a safe soft-tissue filler without a foreign body reaction. This article reports on several cases of periorbital lipogranuloma after autologous fat injection for facial augmentation. Methods This was a retrospective, non-comparative and interventional case series. The authors identified nine patients with periorbital lipogranuloma. All patients had a history of previous autologous fat injection into the face by different plastic surgeons. A medical record review was performed for clinical history, radiological and histopathological studies, and treatment. Results Nine patients developed periorbital granuloma after autologous fat injection for facial augmentation. Eight of the patients had received two injections of autologous fat, and seven patients had undergone a second injection with their cryopreserved fat tissue. Erythematous swelling of the upper eyelid was refractory to antibiotic and steroid treatment, and surgery was required to resolve the inflammatory reactions in six cases. Histopathological evaluation revealed features of a lipogranulomatous reaction, with collection of histiocytes and foreign-body type giant cells around variable-sized microcysts containing lipid materials. Conclusions Lipogranuloma can develop in the eyelid following autologous fat injection to the forehead or glabella. Clinicians should be aware of this potential complication of a granulomatous reaction following autologous fat injection. Techniques for the injection and preservation of autologous fat should be refined.

To describe the anatomic characteristics of structures adjacent to the lacrimal sac fossa and the standardized osteotomy method for endonasal dacryocystorhinostomy in Asians.Retrospective noncomparative observational case series study.One hundred fifty-two eyes of 76 patients who had normal orbit computed tomographic scans were enrolled. The relationships of the uncinate process, the operculum of the middle turbinate, and the agger nasi cell to the lacrimal sac fossa were analyzed. Bone thickness and proportion of the frontal process of the maxilla in the lacrimal sac fossa was also evaluated.The uncinate process was attached to the lacrimal sac fossa in 152 cases (100%) at the lower level of the fossa. The operculum of the middle turbinate was attached to the lacrimal sac fossa in 142 cases (93.4%), with wide positional variation. The agger nasi cell was adjacent to the lacrimal sac fossa in 118 cases (77.6%). The descent of the operculum of the middle turbinate relative to the lacrimal fossa had a strong correlation with the descent of the agger nasi cell (P.001). The height and length of the nasal bone had a significant negative correlation with the thickness of the frontal process of the maxillary bone (P.001).The uncinate process, operculum of the middle turbinate, and agger nasi cell should be removed for sufficient osteotomy in a significant portion of Asian patients during dacryocystorhinostomy. A thick frontal process of the maxilla can be anticipated for the patient with a low nasal bridge. Further studies on the variation in intranasal procedures and the success rate of endonasal dacryocystorhinostomy need to be established.

A 47-year-old woman presented with a growing mass on the lateral rim of orbit. Orbital CT revealed a well-circumscribed soft tissue mass in the right lateral orbit, with focal hyperostosis of the adjacent zygomatic bone. MRI showed a lesion of mixed T1-signal intensity, which became hypointense after fat suppression. The lesion was excised, and the diagnosis of orbital fibrolipoma was made by histopathologic examination. There was no evidence of tumor after 12 months of follow-up. Orbital fibrolipoma is a rare variant of lipoma, with only 1 case described previously. It should be considered in the differential diagnosis of orbital mass.

A healthy 25-year-old man who received a calcium hydroxylapatite filler injection for nose augmentation by a dermatologist suddenly developed blepharoptosis and orbital pain on the right side, associated with progressive visual disturbance of the right eye. Patchy necrosis at the nose and glabella, limitations of extraocular movements, and anterior segment ischemia, as evidenced by conjunctival injection, chemosis, corneal edema, dilated pupil, hyphema, and hypopyon, were noted. Orbital CT demonstrated linear deposits of a similar density to bone in the right medial orbit and eyelid, suggestive of multiple emboli along the conjunctival vessels. A provisional diagnosis of ocular ischemia and ischemic oculomotor nerve palsy secondary to vascular embolization was made. After 3 months, visual acuity, all intraocular inflammation, oculomotor nerve palsy, and skin necrosis resolved completely except for a dilated pupil.

Purpose To describe the outcome of the reverse modified Hughes procedure for the reconstruction of extensive full-thickness defects of the upper eyelid. Methods Seventeen cases of full-thickness extensive upper eyelid defects were reconstructed using the reverse modified Hughes procedure. Extensive defects were characterized as those with a width of 80% or more of the eyelid after tumor excision. Posterior lamella reconstruction was achieved using a pedicled tarsoconjunctival flap from the donor lower eyelid. For anterior lamella reconstruction, the orbicularis oculi muscle superior to the defect was mobilized and transposed over the tarsoconjunctival flap. A cutaneous advancement flap using redundant skin adjacent to the defect or a free postauricular skin graft in cases of insufficient lax skin was used to cover the orbicularis oculi muscle flap. The tarsoconjunctival pedicle was divided 3 to 8 weeks after the primary operation. Results Seventeen patients from 40 to 81 years of age were included in the study. The underlying diagnoses included sebaceous gland carcinoma (13), squamous cell carcinoma (3), and Merkel cell carcinoma (1). Follow-up of 6 to 80 months revealed no cases of flap ischemia or identifiable necrosis after division. The functional and aesthetic outcomes were generally satisfactory throughout follow-up. Postoperative complications included epithelial keratopathy (4), lagophthalmos (3), upper eyelid entropion (2), granuloma formation (2), and lower eyelid entropion (1). Conclusions The reverse modified Hughes procedure with orbicularis muscle mobilization was successful in repairing extensive full-thickness upper eyelid defects greater than 80% of the eyelid width. The mobilization of the orbicularis oculi muscle, with its robust vascular supply, enhances the viability and cosmetic appearance of the reconstructed eyelid.

To investigate the expression of matrix metalloproteinase (MMP)-2, MMP-9, and tissue inhibitor of matrix metalloproteinase (TIMP)-1 in conjunctival melanomas and their correlations with clinicopathologic parameters and prognosis.Fourteen conjunctival melanoma tissue samples and nine conjunctival nevus tissue samples were stained immunohistochemically for MMP-2, MMP-9, and TIMP-1. Association of MMP-2, MMP-9, and TIMP-1 expression in melanoma tissues with clinical progression in terms of metastasis, recurrence, mitotic index, thickness, base diameter, and invasion depth was analyzed.In the melanoma group, 78.6% of samples showed a positive reaction for MMP-2, 85.7% for MMP-9, and 100% for TIMP-1. In the nevus group, 11.1% showed a positive reaction for MMP-2, 66.7% for MMP-9, and 100% for TIMP-1. MMP-2 expression was significantly more induced in conjunctival melanoma than in benign nevi (P = 0.002). In conjunctival melanoma, MMP-9 expression was higher in tumors1.5 mm thick (P = 0.026) and TIMP-1 expression was higher in recurrent cases (P = 0.03). There was no significant correlation between the expression and metastasis during the follow-up period (mean, 5 years).MMP-2, MMP-9, and TIMP-1 were expressed in the majority of conjunctival melanomas, and MMP-2 might play a role in the development and clinical behavior of conjunctival melanoma.

Purpose: To report 4 rare cases of periocular presumed tuberculosis that developed after eyelid and lacrimal drainage surgery. Methods: Retrospective, interventional case series. Review of clinical, laboratory, photographic, and radiologic records of patients with presumed tuberculous inflammation after periocular procedures. Results: Four patients had periocular presumed tuberculosis after blepharoplasty, dacryocystorhinostomy, and intracanalicular lacrimal plug insertion. Diagnosis was made by characteristic histopathologic findings, acid-fast bacilli staining, polymerase chain reaction results, or response to therapeutic trial. All of the patients underwent antituberculosis chemotherapy for a minimum of 9 months and all recovered. Ectropion and eyelid retraction complicated 2 cases. Conclusions: Unresponsiveness of chronic inflammation to conventional treatment after periocular procedures should arouse the clinical suspicion of localized tuberculosis, particularly in endemic areas or in patients who have a tuberculosis history or recent exposure. For presumed eyelid tuberculosis without evidence of Mycobacterium tuberculosis, the clinical diagnosis and therapeutic trials could be considered cautiously especially in countries with an intermediate or high tuberculosis burden. Early diagnosis and appropriate treatment can prevent the sequelae of tuberculosis infection.

The authors report 3 cases of orbital dermoid cysts presenting with subconjunctival fat droplets. Three female patients were evaluated for conjunctival injection and yellowish granular material lying under the bulbar conjunctiva in the left eye. Slit-lamp examination showed a number of subconjunctival fat droplets in the involved eyes. Orbital CT and MRI in each case revealed a cystic mass located in the superotemporal aspect of the left orbit. The masses were excised via lateral orbitotomy in 2 patients, and a conjunctival biopsy of the granular material was performed in 1 patient. The cystic masses were histopathologically diagnosed as dermoid cysts with findings of lipogranuloma that suggested a previous rupture of the cyst itself. Examination of the conjunctival specimen also suggested lipogranuloma. Orbital dermoid cysts should be considered in patients presenting with subconjunctival fat droplets.

Objective This study was performed to investigate the radiological and functional outcomes of patients with orbital tumors treated by gamma knife radiosurgery (GKS). Patients and methods Fifteen patients with orbital tumors (7 meningiomas, 3 cavernous hemangiomas, 2 schwannomas, 2 metastatic tumors and 1 adenoid cystic carcinoma of the lacrimal gland) were treated. Seven patients with preserved vision and tumors located near the optic nerve were treated with multisession (3 or 4 fractions) radiosurgery. The mean tumor volume was 3695 mm 3 (737–13,300). The median marginal dose was 14 Gy (13–20) in single-session radiosurgery, and the median cumulative marginal dose was 20 Gy (15–20) in multisession radiosurgery. Results After a mean follow-up of 20.9 months (6–50), tumor control was confirmed in 12 of 15 patients. Three patients with malignant lesions had to undergo another operation due to tumor progression. Of the 13 patients whose preoperative vision was preserved, 6 patients showed improvement in visual acuity and/or visual field, 4 patients showed no change in vision, and 3 patients showed deterioration (2 related to tumor progression). Conclusions As with intracranial tumors with similar pathologies, GKS may be an effective treatment option for orbital tumors. Multisession radiosurgery may be a good strategy for increasing the possibility of visual function preservation in selected cases in which the lesion is adjacent to the optic apparatus.

To report on the characteristics of patients with orbital venous anomaly presenting with acute orbital hemorrhage. We retrospectively reviewed the clinical and radiologic findings of a case series of eight orbital venous anomaly patients who presented with orbital hemorrhage. Charts of patients diagnosed as having orbital venous anomaly who initially presented with orbital hemorrhage were selected. The eight patients with orbital venous anomaly (one man and seven women) ranged in age from 7 to 66 years (median, 22 years). The average duration of symptoms was 7 days (range, 1–30 days). All eight patients were totally asymptomatic prior to their acute presentations. Orbital computed tomography (CT) scans showed a well-demarcated ovoid mass in all eight patients. Five patients were referred with a suspicion of orbital tumor, and one underwent a lateral orbitotomy for a biopsy. In six patients, the presence of an orbital hemorrhage was identified by typical findings on orbital magnetic resonance imaging (MRI). After resolution of the hemorrhage, all patients evidenced a distensible orbital venous anomaly in an imaging study. An orbital venous anomaly presenting with sudden orbital hemorrhage can be mistaken for other orbital tumors. In patients with acute orbital process, orbital MRI for detecting hemorrhage and a follow-up CT scan after resolution of the hemorrhage are needed to prevent unnecessary surgical intervention.

Objectives To evaluate the clinical features, treatments, and outcomes of patients with ocular lymphoproliferative disease classified according to the World Health Organization classification and to determine prognostic factors of this disease in South Korea. Methods Between March 1, 1995, and December 31, 2005, 128 cases of patients with ocular lymphoid tumors treated at the Samsung Medical Center were retrospectively reviewed. Results The mean patient age was 46.3 years (range, 1-87 years). The major histopathologic subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma in 96 patients (75.0%), lymphoid hyperplasia in 11 (8.6%), diffuse large B-cell lymphoma in 6 (4.7%), and mantle cell lymphoma in 4 (3.1%). Ocular lymphoproliferative lesions were located in the conjunctiva (53 patients), the eyelid (33 patients), and the orbit (42 patients). Twenty patients had tumor relapses (15.6%), and 9 died of lymphoma during follow-up (7.0%). Regarding the analysis of prognostic factors, most patients with MALT lymphoma evidenced local disease, required local treatment, and exhibited a superior prognosis. Conclusions Lymphomas of the MALT type constitute most ocular adnexal lymphoproliferative diseases and occur more frequently in South Korea than in Western countries. Patients with MALT lymphoma have favorable outcomes compared with patients with other types of lymphoma.

An 11-month-old girl was referred to us with enophthalmos and restriction of ocular movement in the right eye. There was restriction of supraduction and adduction with synergistic divergence on left gaze. MRI revealed a multiloculated cystic mass in the right inferior orbit. There was an atrophic and misinserted inferior rectus muscle and only a vestigial right medial rectus muscle. Excisional biopsy of the orbital cystic mass was performed. Microscopic examination disclosed that the mass was brain tissue composed of arachnoid tissue, brain parenchyma, choroid plexus, and dura mater. Our report describes a rare case of ectopic brain in the orbit that was combined with anomalies of the extraocular muscles.

The genetic changes in marginal zone B cell lymphomas (MZBCL) vary according to the anatomical region. This study aimed to investigate genomic aberrations in ocular MZBCL and to compare them with those of tumors from other anatomical sites. The study population comprised 24 cases of primary ocular MZBCL, 11 pulmonary MZBCL, and seven nodal MZBCL. For array CGH, fresh tumor tissues were analyzed with a genome-wide scanning array containing 2,304 BAC/PAC clones which cover the whole human genome at a resolution of 1.3 Mb. FISH analysis for MALT1 gene alteration was performed for ocular and nodal MZBCL and RT-PCR for the detection of API2-MALT1 transcripts was performed for pulmonary MZBCL. The recurrent genomic alterations in ocular MZBCL were losses of chromosome bands 6q23.3 (9/24, 38%), 7q36.3 (2/24, 8%), and 13q34 (2/24, 8%), and gains of chromosomes 3 (9/24, 38%), and 15 (4/24, 16%), and chromosome arms 18q (4/24, 16%), and 6p (2/24, 8%). The t(11;18)(q21;q21) was not detected. The genomic alterations of pulmonary MZBCL included recurrent loss of 18q21 (2/11, 19%). A t(11;18)(q21;q21) fusion transcript was detected in five out of eight cases (63%). Nodal MZBCL showed neither recurrent genome alterations nor any change in MALT1 gene copy number. In conclusion, the array CGH profile of ocular MZBCL is distinct from those of pulmonary and nodal MZBCL. Deletion of chromosome band 6q23.3 in ocular MZBCL is a novel finding and may constitute a crucial genetic alteration in the pathogenesis of ocular MZBCL. © 2007 Wiley-Liss, Inc.

Dear Editor, Two cases with epiphora and medial canthal mass showed a cyst within the lacrimal sac that compressed the lacrimal drainage system. We describe a new lacrimal sac anomaly of lacrimal intrasaccal cyst, which has not yet been reported. A 31-year-old woman was referred for epiphora in her left eye for 17 years. She also presented with a 10-year history of a palpable mass below the medial canthal tendon. The lacrimal system was patent on irrigation. In computed tomographic (CT) dacryocystography (DCG), a soft ovoid mass of 25 mm was found in the lacrimal sac fossa, and the lacrimal passage was compressed and deviated anteriorly (Fig. 1A and 1B). DCG revealed a patent but laterally deviated lacrimal drainage passage (Fig. 1C). After subciliary incision, an enlarged lacrimal sac was identified and dissected. After lacrimal sac incision, serous fluid gushed out from a blind sac on the periosteal side of the lacrimal sac. The internal common punctum and the lateral lacrimal sac wall were identified after excision of the lateral wall of the cyst. The cyst and the lacrimal sac shared a common wall along the entire length between them. External dacryocystorhinostomy was performed after cyst excision. Histopathologic study revealed a cyst wall lined with pseudostratified columnar and cuboidal epithelium, supporting the finding of the lacrimal sac mucosa (Fig. 1D). The patient has now been free of symptoms for five years. Fig. 1 (A,B) Computed tomographic dacryocystography (DCG) demonstrates an ovoid soft tissue mass (arrowhead) anterior to the lacrimal passage (arrow) in the lacrimal sac fossa (case 1). (C) DCG reveals a filling defect in the lacrimal sac without passage disturbance ... A 39-year-old man was referred for epiphora of two-year duration. A soft mass was found just above the left medial canthal tendon. In the CT-DCG, a 16 mm-sized soft tissue mass was identified with pressure remodeling of the adjacent bone (Fig. 1E). The DCG revealed a deviated and blocked lacrimal sac on the lateral side of a space occupying filling defect (Fig. 1F). The lateral wall of the cystic mass shared a common wall with the lacrimal sac. On histopathologic examination of the common wall, both sides showed the same features of pseudostratified columnar epithelium with squamous dysplasia. The patient has now been free of symptoms for five years. A lacrimal intrasaccal cyst is regarded to be a different clinical entity from a lacrimal sac cyst or a diverticulum because the latter anomalies are located outside of the lacrimal sac. Lacrimal diverticulum forms a mass attached and connected to the lacrimal sac with a narrow neck [1,2]. Lacrimal sac cyst has been described as a separated orbital cystic mass having lacrimal sac epithelium, which is near to or remote from the lacrimal sac [1,2]. Embryologically, the nasolacrimal apparatus develops from the surface epithelial core and canalization occurs at three months gestation throughout the length of the nasolacrimal apparatus [3]. Though an acquired condition from lacrimal sac inflammation cannot be excluded, lacrimal intrasaccal cyst can be considered to develop from an extra core of cells budding off the original core like other congenital lacrimal drainage system anomalies. Takahashi et al. [4] first described the lacrimal sac septum. They incidentally identified it in a cross section of the lacrimal sac, which shared the same epithelial lining with the lacrimal sac. It was also regarded to result from developmental anomalies in embryogenesis. Although there was no description of proximal or distal features of the septum, the case might represent an asymptomatic lacrimal intrasaccal cyst without cyst expansion. Mansour et al. [5] reported a supernumerary blind lacrimal sac that was connected to the supernumerary puncta and canaliculi, resulting in compression of the lacrimal drainage pathway. The entity was different from our cases because there was no supernumerary puncta or canaliculi connected to the cyst. Lacrimal intrasaccal cysts can demonstrate different clinical courses such as symptom-free if it is not expansile or cystic mass formation with or without nasolacrimal duct obstruction. Presenting symptoms cannot differentiate the lesion and imaging studies of CT-DCG and DCG are mandatory for diagnosis. If a thick membrane is found between the "lacrimal sac cavity" and internal punctum during dacryocystorhinostomy, possibility of intrasaccal cyst should be considered. Endonasal marsupialization or dacryocystorhinostomy could be one of the treatment options according to the obstructive signs. Recognition of this abnormal configuration of the lacrimal sac might be helpful in differential diagnosis and treatment of patients with medial canthal mass with epiphora, though it is a rare entity.

Purpose To report a rare case of craniodiaphysial dysplasia (CDD) that resulted in a profound loss of vision in both eyes. Design Observational case report. Methods A 2-year-old girl presented with midfacial anomaly. Marked thickening and sclerosis in the calvaria and facial bones were detected on the plain x-rays, which were compatible with CDD. Two years later, she visited our clinic because of visual loss in both eyes. Results The visual acuity was light perception in both eyes. The optic disk swelling with temporal pallor was observed in her both eyes. Orbital computed tomography scan revealed near-total obliteration of the optic canal in both eyes. Conclusions CDD is a severe bone disorder characterized by massive generalized hyperostosis and sclerosis, especially involving the facial bones. Bony encroachment on the cranial foramina causes optic nerve compression, and this may lead to progressive visual impairment and ultimately to blindness.

Purpose: To present a case of inflammatory myofibroblastic tumor in the anterior orbit and to describe its clinical features, diagnosis, and management. Methods: Case report and literature review. Results: A 10-year-old boy presented with diplopia and limited ocular motility in his right eye secondary to a subconjunctival mass in the right supranasal side. Incisional biopsy and debulking were performed. Histopathologic examination showed the proliferation of spindle-shaped myofibroblasts that were immunoreactive for smooth muscle actin and vimentin and infiltrate of inflammatory cells. Conclusions: We believe this is the first case of an inflammatory myofibroblastic tumor found localized in the orbit.

Purpose To review the clinical, radiological, and histopathologic features in 8 patients with natural killer/T-cell lymphoma (NKTL) involving the orbit and/or ocular adnexa, and to describe the responses of these patients to various treatment regimens. Design Retrospective observational case series. Participants Eight patients (5 male, 3 female) with NKTL involving the orbit and/or ocular adnexa were identified from 1999 through 2005. The mean age at presentation was 45 years (range, 26–65). Methods We retrospectively identified patients with NKTL of the ocular adnexa treated in the authors' medical centers from 1999 through 2004 using computerized diagnostic index retrieval. The clinical records and radiologic studies were analyzed to define modes of presentation and progression, response to therapy, and areas of anatomic involvement. Histopathologic findings, including the presence of CD3, CD56, and Epstein–Barr virus–encoded mRNA in each patient, were reviewed. Main Outcome Measurements Time of survival from presentation to last known follow-up and tumor-related death. Results Four of the 8 patients (50%) with NKTL involving the orbit or ocular adnexa had systemic involvement at presentation. Five of the 8 patients (62.5%) had concurrent sinonasal involvement, whereas 3 (37.5%) had orbital involvement alone. All lesions demonstrated CD3, CD56, and/or Epstein–Barr virus positivity on immunopathology studies. Therapy consisted of various chemotherapeutic regimens typically employed in the treatment of non-Hodgkins lymphoma, steroids, surgical intervention, and radiation. Seven (87.5%) patients died 5 weeks to 13 months after presentation, and 1 (12.5%) is alive without disease (5-year follow-up). Conclusions Natural killer/T-cell orbital lymphoma is a rare Epstein–Barr virus–associated neoplasm that may occur with or without associated sinonasal involvement. Our series, the largest cohort reported to date, demonstrates the high lethality of this condition despite aggressive conventional therapy, suggesting that new treatment options should be considered early in the course of treatment of patients with this disorder.

Primary lid tuberculosis after lid surgery is a very rare condition and is likely caused by the introduction of bacilli through epithelial injury. Secondary infection, due to direct hematogenous spread or contiguous spread from adjacent structures are more common presentations of lid tuberculosis. The authors experienced a case of primary lid tuberculosis occurring in a 19 year old female after blepharoplasty for making a eyelid crease. Her upper lid skin showed a reddish and non-tender mass lesion measured 3x1 cm, which was diagnosed as the tuberculosis through typical histopathological findings (caseous necrosis), acid-fast bacilli stain and PCR, and treated with anti-tuberculosis medications.

To describe early clinical results with the porous polyethylene smooth surface tunnel (SST) enucleation implant.Uncontrolled, prospective interventional case series of patients undergoing enucleation with placement of the SST implant. This implant consists of a porous polyethylene sphere with a smooth anterior surface containing pre-drilled tunnels to facilitate direct suturing of the rectus muscles to the implant without use of an implant wrap. Postoperatively, socket healing was assessed, and prosthesis and socket motility were evaluated by the surgeon using an ordinal scale (0 = no motility to 4 = excellent motility).Thirty patients received the SST implant, with a mean follow-up of more than 23 months. Two cases of exposure occurred and were managed surgically without the need for explantation. Mean socket motility was 3.1 on a 0 to 4 ordinal scale, with mean prosthesis motility of 2.8.The SST implant provides satisfactory socket motility and is generally well tolerated in the anophthalmic socket without the need for wrapping material.

A 48-year-old man presented with an enlarging mass of the right upper eyelid over a 1-month period. Nine years prior, the patient was diagnosed with adenoid cystic carcinoma (ACC) of the right maxillary sinus and underwent a total maxillectomy followed by local radiotherapy. Over the previous year, the patient had been treated with chemotherapy and radiotherapy for multiple organ metastases, including the spine, chest, and oral cavity. Ophthalmic examination revealed a white, round mass on the tarsal conjunctiva of the right upper eyelid, measuring approximately 1 × 1 cm, and 2 palpable subcutaneous masses near the inferior orbital rim. Excisional biopsies of the masses were performed, and the histopathological findings were consistent with ACC with solid and cribriform patterns. Herein, the authors describe a case of metastatic ACC of the eyelid, which has not been described in the literature to their knowledge.

Malignant peripheral nerve-sheath tumors (MPNSTs) are rare, poorly defined spindle cell sarcomas, and they are accompanied by heterologous elements in some cases. Among them, a fibroblastic element is one of the rarest and has been mentioned in some organs in several publications, but not in the orbit. The authors report a case of fibroblastic low-grade MPNSTs in the orbit. The mass was removed surgically, and the patient has been under close observation without evidence of disease recurrence for 2 years.

Purpose To investigate the clinical outcome of a frontalis sling using preserved fascia lata in the treatment of vision-obscuring congenital ptosis in patients less than 2 years of age. Design Retrospective, interventional case series. Methods The study was conducted in an institutional setting and included 82 patients with visual axis–obscuring congenital ptosis. All patients underwent frontalis sling surgery with preserved fascia lata between November 1994 and December 2008, and had a minimum follow-up of 6 months. Visual and surgical outcomes were assessed by reviewing clinical photographs and medical charts. Surgical outcomes were defined as good, fair, or poor, based on the postoperative lid level. Results The mean age at surgery was 15.3 ± 4.8 months. After a mean follow-up of 54.8 ± 41.4 months, 64 patients (78.0%) showed good or fair surgical outcomes. Reoperation was performed in 16 of the patients with poor or fair results. Amblyopia was treated postoperatively in 75 of the 82 patients (91.5%) and had improved in 65 patients at the last follow-up. Postoperative entropion was identified in 5 cases, all of which were treated successfully. No other significant complications occurred. Conclusions For patients younger than 2 years of age, preserved fascia lata may be an appropriate substitute for autogenous fascia lata in frontalis sling surgery. Its long-term stability may enable a permanent effect in a certain proportion of patients, and may not require secondary surgery, which is common with synthetic materials.